Episodes Archives - Cardionerds

In this episode, CardioNerds (Amit Goyal), Cardio-OB series co-chair and UT Southwestern cardiology fellow, Dr. Sonia Shah, and episode lead fellow, Dr. Laurie Femnou (UT Southwestern) are joined by Dr. Michael Luna (UT Southwestern) to discuss cardiovascular interventions during pregnancy. We discuss practical considerations for performing coronary angiography and valvular interventions in the pregnant patient, the timing and indication of procedures, and ways to minimize radiation exposure to both mom and baby. Audio editing by CardioNerds Academy Intern, Hirsh Elhence.

This episode is made possible with support from Panacea Financial. Panacea Financial is a national digital bank built for doctors by doctors. Visit panaceafinancial.com today to open your free account and join the growing community of physicians nationwide who expect more from their bank. Panacea Financial is a division of Primis, member FDIC.

Pearls • Notes • References • Guest Profiles • Production Team

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1. Attempt should be made to manage pregnant patients with valvular disease with medical therapy, and cardiac interventions during pregnancy should be considered a last resort.
2. Ideally, procedures in the gravid patient should be performed after 20 weeks gestation to minimize fetal risk. 
3. Specific ways to minimize radiation to the pregnant patient and fetus in the catheterization lab include: using an abdominal shield, radial access for coronary procedures, proper positioning of the C-arm to avoid extreme angulation, using collimation, and minimizing fluoroscopic time, frame rate, and use of cine-acquisition. When appropriate, intracoronary imaging modalities (ie. IVUS or OCT) should also be considered.
4. Fetal monitoring should be performed during any cardiac intervention in the pregnant patient
5. Multidisciplinary involvement and contingency planning are critical for the success of any high-risk cardiac intervention in the pregnant patient.

“Meetings including all providers— our cardiac surgical colleagues, cardiac anesthesiologists, and our obstetrics team—in the care of [pregnant] patients has to be had well ahead of a cardiac procedure to plan every detail.”

1. What are special considerations for performing a balloon valvuloplasty in a pregnant patient with mitral stenosis?

•  In pregnant patients with severe mitral stenosis who cannot be adequately managed with medical therapy, percutaneous balloon mitral valvuloplasty (PMBV) is the treatment of choice given the high risk of morbidity and fetal loss with cardiac surgery.
• Ideally, procedures in the gravid patient should be performed after 20 weeks gestation to minimize risk to the fetus. 
• Assessment of valve anatomy and consideration of the Wilkin’s score are especially important in pregnant patients to minimize the risk of peri-procedural complications.
• PBMV should performed at experienced centers with cardiac surgery and MFM available.
• Complications of PBMV are rare but include atrial perforation, cardiac tamponade, arrhythmias, emboli, mitral regurgitation, hypotension and maternal death. Mechanical support should be readily available and a delivery strategy in place in case there is sudden maternal or fetal deterioration.

2. What are ways to minimize radiation exposure in the catheterization lab to the pregnant patient and fetus?

• The general principle for imaging during pregnancy is similar to imaging for the general population, with the goal of radiation exposure being as low as reasonably achievable (ALARA). The mean radiation exposure to the unshielded abdomen is 1.5 mGy, and <20% of this reaches the fetus. 
• Specific ways to minimize radiation to the pregnant patient and fetus in the catheterization lab include the following: using an abdominal shield, radial access for coronary procedures, proper positioning of the C-arm to avoid extreme angulation, using collimation, and minimizing fluoroscopic time, frame rate, and use of cine-acquisition. When appropriate, intracoronary imaging modalities (ie IVUS or OCT) should also be considered.

3. What should we know about coronary angiography and revascularization in the pregnant woman?

• According to a large US-based study of 859 patients presenting with acute myocardial infarction during pregnancy and the postpartum period, less than half of patients undergo cardiac catheterization. In stable, low-risk NSTEMI, a non-invasive approach should be considered per ESC guidelines. In pregnant patients who present with STEMI, PCI is the preferred revascularization strategy.
• Radial access should be used if possible, as femoral artery access involves direct pelvic radiation.
• In pregnancy-associated SCAD, enhanced vascular vulnerability should be considered when performing angiography, from obtaining access to engaging the coronary ostia.
• Fetal monitoring should be performed during any cardiac intervention in the pregnant patient

4. What is the role of mechanical support in the management of peripartum patients with cardiogenic shock?

• Urgent intervention with mechanical assist support, including intra-aortic balloon pump, impella, and VA-ECMO, may be necessary in rare circumstances. Prompt assessment of hemodynamics can be helpful to determine the level of support needed. Axillary access may be considered in patients with favorable anatomy.

Patel C, Akhtar H, Gupta S, Harky A. Pregnancy and cardiac interventions: What are the optimal management options? Journal of Cardiac Surgery, 2020. 35(7): 1589-1596.

Regitz-Zagrosek V, Lundqvist CB,  Borghi C, et al. ESC Guidelines on the management of cardiovascular diseases during pregnancy The Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC). European Heart Journal, 2011. 32, 3147–3197 doi:10.1093/eurheartj/ehr218

Dr. Michael Luna is an associate professor of cardiology at UT Soutwestern trained in interventional cardiology, with additional focused training in congenital heart disease. He specializes in adult congenital heart defects, heart valve disorders, and complex coronary artery disorders. Dr. Luna also serves as one of the supervising attendings in the Parkland Congenital Heart Disease Fellow’s Clinic.

Dr. Laurie Femnou Mbuntum is currently a general cardiology fellow at The University of Texas Southwestern. She completed her undergraduate degree at The University of Maryland Baltimore County. She then moved down South to complete residency at The University of Texas Southwestern where she stayed for cardiology fellowship where she is planning to stay for advanced training in interventional cardiology. She has a special interest in cardio obstetrics and figuring out ways to reduce cardiovascular maternal death. When not in the hospital, she loves spending time with her two boys and learning more about makeup artistry.

CardioNerds Rounds Co-Chair, Dr. Karan Desai, joins Dr. Michelle Kittleson (Director of Postgraduate Education in Heart Failure and Transplantation, Director of Heart Failure Research, and Professor of Medicine at the Smidt Heart Institute at Cedars-Sinai) to discuss challenging cases of hypertrophic cardiomyopathy. As a guideline author on the 2020 ACC/AHA Hypertrophic Cardiomyopathy Guidelines, Dr. Kittleson shows us how the latest evidence informs our management of HCM patients, while sharing many #Kittlesonrules and pearls on clinical care. Come round with us today by listening to the episodes now and joining future sessions of #CardsRounds!

This episode is supported with unrestricted funding from Zoll LifeVest. A special thank you to Mitzy Applegate and Ivan Chevere for their production skills that help make CardioNerds Rounds such an amazing success. All CardioNerds content is planned, produced, and reviewed solely by CardioNerds. Case details are altered to protect patient health information. CardioNerds Rounds is co-chaired by Dr. Karan Desai and Dr. Natalie Stokes. 

Speaker disclosures: None

Cases discussed and Show Notes • References • Production Team

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Case #1 Synopsis:

Two non-white brothers in their early 20s come to clinic to establish care. They have no cardiopulmonary symptoms, normal EKGs and normal echos, but there was a possible family history of HCM. Their mother had LV hypertrophy and underwent septal myectomy, but she could not afford genetic testing and was no longer in the patients’ lives. The path report suggested “myocyte hypertrophy without disarray or bundles of myocytes.” How would you advise these patients regarding screening and surveillance? Listen to #CardsRounds for the full details!

 Quotes from Case #1:

“Let’s take a walk down memory lane and let’s get to our evolution of understanding hypertrophic cardiomyopathy… [our understanding] follows the parable of the six blind men and the elephant. Each of the six blind man approached it from different angles, its tusk, its ear, its tail, and they all try to convince each other what an elephant is … because none of them can see the big picture.”

• Dr. Kittleson on the history of HCM and coming to a unifying diagnosis

 “The next time you are sitting there mashing your teeth because you have to memorize what the HCM murmur does squat to stand, Valsalva, or handgrip … remember you are standing on the shoulder of Giants. They [Drs. Braunwald and Morrow] pioneered surgical myectomy based on physical exam and cath lab findings”

• Dr. Kittleson on the physical exam guiding HCM management

Takeaways from Case #1

Before we round, we think it is important to get on the same page regarding the nomenclature around HCM.

1. Since the original characterization of hypertrophic cardiomyopathy (HCM) more than 60 years ago (see the Braunwald Chronicles for the origin stories!), different terms have been used to describe the disease. These include idiopathic hypertrophic subaortic stenosis, hypertrophic obstructive cardiomyopathy (HoCM), and “burnt out HCM” when heart failure develops.
2. The 2020 guideline committee recommended a common language to avoid confusion: since left ventricular (LV) outflow tract obstruction (LVOTO) occurs in >60% of patients over time, but one-third remain non-obstructive, the recommendation is t0 call the disease state HCM with or without outflow tract obstruction.
3. Dr. Kittleson added that when heart failure develops we should characterize the pathology as HCM with heart failure rather than “burnt out HCM.”

 Do we use HCM to describe any LV that has thick walls?

• Some clinicians will use HCM to describe all disease states that can lead to increased LV wall thickness, including those associated with systemic disorders such as RASopathies, mitochondrial myopathies, glycogen/lysosomal storage diseases, Fabry’s disease, hemochromatosis, Danon disease, and amyloidosis (especially in adults).
• However, the pathophysiologic mechanisms, genetic underpinnings, and treatment of these diseases are different. Secondary causes of left ventricular hypertrophy (LVH) – such as athlete’s heart and hypertensive disorders – can also cause confusion with identifying the correct terminology and diagnosis.
• As Dr. Kittleson (and the guidelines) made clear, the clinical definition of HCM is a disease state in which (1) the morphologic expression is restricted to the heart, characterized primarily by LVH in the absence of another systemic; (2) a metabolic or cardiac cause; (3) and for which a disease-causing sarcomere (or sarcomere-related) variant is identified or genetic etiology remains undetermined.
• See some of our original CardioNerds episodes to review the clinical manifestations, diagnosis and treatment of HCM!

Ok. We are with you on the terminology! But as you mentioned there are many disease states that can look like HCM. What are phenocopies?

1. As alluded to above, there are multiple genes that can lead to the phenotypic appearance of HCM, but rather these are “phenocopies” masquerading as HCM.
2. Dr. Kittleson noted that many of these phenocopies are diagnosed in childhood (e.g., glycogen storage diseases, Friedrich ataxia, Danon disease); however, for adult cardiologists, particular attention should be given to recognizing amyloidosis (see our Amyloidosis page).
3. We should remain alert for “LVH+” states: when we see left ventricular hypertrophy but also systemic signs including peripheral neuropathy, renal dysfunction, and skin changes, which could clue us into a systemic pathology like Amyloidosis. After all, Imitation is the sincerest form of flattery!
4. The classic pathologic findings of HCM include myocyte disarray (where individual cardiomyocytes vary in size, shape and form), abnormal intercellular connections, expansion of the interstitial compartment and areas of replacement fibrosis, and small vessel disease (in which intramural vessels are narrowed by medial hypertrophy).

So back to this case. It is possible the patients’ mother had a HCM phenocopy. And the pathology was not classic for HCM. What do the guidelines say regarding genetic screening in general?

1. It was not until the 1990s when DNA sequencing of HCM pedigrees led to the discovery of variants in genes coding for sarcomere proteins were co-inherited in patients with LVH. Thus, HCM since has been regarded as a monogenic cardiac disease.
2. The two most common genes with damaging variants are beta myosin heavy chain (MYH7) and myosin-binding protein C3 (MYBPC3).70% of variant-positive patients have gene variants in these genes; however, it is important to recognize that many patients with HCM are currently without any identifiable pathogenic genetic etiology to their disease (40 to 60% of patients depending on the study).
3. Thus, genetic testing is crucial to the diagnosis and management of HCM, especially to inform cascade testing in family member and preconception and prenatal genetic counseling. As has been discussed previously on the CardioNerds podcast, appropriate pre- and post-test genetic counseling is necessary to ensure patients and their families understand the medical, social, psychological, ethical and professional implications of having a genetic disease, underscoring the value of multidisciplinary HCM centers.

How do we apply the guidelines regarding genetic screening to our patients?

• As was done in this case, a detailed family history of at least three generations regarding HCM and SCD events should be taken. Typically, genetic testing for HCM is first done in the family member with clear phenotypic evidence of HCM – the index case. The 2020 ACC/AHA HCM guidelines nicely outline subsequent testing based on whether a variant is pathologic (Figure 1) Please remember to review the Guidelines yourself as it is a comprehensive text that provides key details not covered here!
• However, the index case could not afford genetic testing and the pathology available to us was not definitive for HCM. Postmortem testing for HCM-associated variants using blood or tissue samples collected at autopsy is possible, but access to a molecular autopsy and insurance coverage can vary significantly based on jurisdiction.
• Our patients (the brothers in their 20s) were arriving without genetics to inform their screening. In this circumstance we utilize Figure 2 (below) from the guidelines and would consider the index patient (the mother) phenotype negative. And since no variant was identified in the index patient, we would likely offer screening ECG/Echo (or cardiac MRI) to the brothers and follow up with clinical surveillance with imaging/ECG every 3-5 years.

How do we decide if a variant is pathogenic?

1. The pathogenicity of variants is based on American College of Medical Genetics and Genomics criteria and can actually change over time. As Dr. Kittleson noted, there are fewer quality genetic data in non-white HCM populations.
2. The five highest frequency variants for HCM in the Human Gene Mutation Database of the NHLBI Exome sequencing Project were more frequent in Black Americans vs. White Americans which could lead to false positive diagnoses of HCM in Blacks. Furthermore, a lack of diverse racial/ethnic control populations could lead to a misclassification of benign variants
3. As Dr. Kittleson noted on #CardsRounds, this again highlights the importance of multidisciplinary HCM clinics that have the expertise to periodically re-evaluate the pathogenicity of variants. If they a particular variant has been reclassified (either upgraded or downgraded), it would affect cascade testing and clinical surveillance in families.

Case #2 Synopsis:

In this second case on HCM #CardsRounds, we saw a young woman in her 20s who was seeking a 2nd opinion regarding primary prevention ICD. She was diagnosed with HCM three years ago after a murmur was discovered on exam. Her echo demonstrated a septal wall thickness of 1.9 cm, LVOT gradient at 23 mmHg at baseline and 126 mmHg with treadmill stress. She was eventually referred for myectomy where 11 grams of myocardium was resected and her gradients improved significantly. She did well for two years and then had multiple syncopal episodes in the setting of a GI illness. Her repeat echo showed a maximal wall thickness of 2.6 cm, no LVOT obstruction at rest or provocation. She was referred for an ICD but wanted a 2nd opinion. You discover she does have a likely pathologic variant in the MYH7 gene, and both her mother and son have the variant. Her mother had NSVT and so will be receiving an ICD. She had a maternal half-brother who passed away from possible overdose but autopsy was suggestive of HCM (with genetic testing still in the works). She underwent a 7-day Holter with no NSVT. Just before this visit she had an MRI which suggested 8-12% late gadolinium enhancement, a hypertrophied septum and LV apical wall thinning (likely not a true aneurysm).

Case #2 Quotes:

“Let’s talk about Sudden Death risk stratification in HCM, or basically, how do you predict the future?”

• Dr. Kittleson reviewing the 2020 guidelines

 “If something doesn’t make sense. You go to the source! Speak to your whole team. Help me, help you, help the patient!”

• Dr. Kittleson On utilizing multidisciplinary HCM team to inform patient care

 “One fantastic component of the 2020 HCM guidelines is the explicit recommendation for shared decision-making with a full disclosure of risks, benefits, anticipated outcomes and the patient expresses their goals and concerns. But let us not forget, that shared decision-making is not an excuse to abdicate medical decision making. It is still your responsibility to provide a medical recommendation … and then work with the patient to see how that medical opinion fits in with the patient’s values and goals.”

• Dr. Kittleson on shared decision-making on ICD placement

 Takeaways Case #2

How do we make a decision for ICD in HCM patients? How do the new guidelines differ?

1. HCM is considered the most common cause of SCD in young people in North America, and thus much research has gone into defining the clinical features that would justify a primary prevention ICD in HCM patients.
2. There are important differences between the 2011 and 2020 guidelines in regards to clinical factors to consider, as outlined below.
3. Similar in Both Guidelines (Typically applying to Adults <60 years old*)
   1. A history of sudden cardiac death, ventricular fibrillation or sustained VT is a Class I indication for secondary prevention ICD in patients with HCM in both guidelines.
   1. Family history of SCD from HCM (Class IIa in 20211 and 2020) is defined as sudden death attributable (or likely attributable) to HCM in ≥ 1 first-degree or close relatives who are ≤ 50 years of age. Close relatives are generally considered to be second-degree relatives.
   1. Massive LVH (Class IIa in 2011 and 2020), in adults patients is defined as wall thickness ≥ 30 mm in any segment by echo or cardiac MRI
   1. Unexplained Syncope (Class IIa in 2011 and 2020) is defined as ≥ 1 unexplained episodes of syncope, unlikely to be of neurocardiogenic etiology or attributable to LVOTO
   1. Apical Aneurysm (Class IIa in 2011 and 2020) is defined as a discrete thin-walled dyskinetic or akinetic segment of the most distal portion of the LV
4. Differences in the Guidelines (Typically applying to Adults <60 years old*)
   1. Previously in the 2011 guidelines, the presence of abnormal BP response to exercise + either (1) genetic mutations and/or (2) LVOTO > 30 mmHg was considered a Class IIa indication for an ICD. These criteria have been removed from the 2020 guidelines.
   1. In the 2020 guidelines, there is a new emphasis on systolic dysfunction with EF < 50% by echo or cardiac MRI, and it is given a Class IIa indication for an ICD.
   1. NSVT – defined as frequent (≥3), longer (≥10 beats), and fast (≥200 bpm) occurring usually over 24 to 48 hours – was a Class IIa indication for an ICD in the 2011 guidelines and has been de-emphasized in the 2020 guidelines as a Class IIb.
   1. Extensive late gadolinium enhancement (LGE) on CMR is defined as diffuse and extensive LGE quantified or estimated as compromising ≥ 15% of the LV mass

 How often should we screen for clinical factors that would increase risk for SCD? What are some nuances we should be aware of?

1. As with most decisions in medicine, the patient should be at the center of the decision-making process regarding ICD. For some patients, they may want a quantifiable estimate of their SCD risk, which can be estimated with a 5-year SCD Risk Calculator. Of note, certain contemporary SCD risk markers are not included in these calculators, including systolic dysfunction, LGE on MRI, or LV apical aneurysm.
2. SCD risk assessment should occur at the initial visit and then be repeated every 1 to 2 years as part of the ongoing clinical surveillance of HCM. Of note, the data suggests a lower SCD event rate in older patients with HCM (>60 years of age), and thus the decision regarding continuing risk assessment in this population is individualized.
3. As noted above, several criteria are dependent on imaging – including LV wall thickness. This may be underestimated on echocardiography compared to cardiac MRI and MRI adds the additional component of assessing LGE

 Based on the above criteria, how would you advise our patient?

1. She does not meet a Class I indication for ICD.
2. In regards to Class IIa recommendations, she does not have massive LVH, unexplained syncope, likely does not have an apical aneurysm, and has a normal LVEF.
3. The family history is questionable given her half-brother’s death under unclear circumstances with morphologic evidence of HCM on autopsy. Given the concerning family history, she may have a Class IIa reason to pursue an ICD.
4. However, she also does not have NSVT and does not have extensive LGE – though this is in the setting of being post-surgical myectomy.
5. After shared-decision making, given the constellation of findings it would be reasonable to offer her an ICD.

Case #3 Synopsis:

A woman in her early 30s comes to an HCM Center of Excellence to establish care. She was diagnosed with HCM without obstruction one year prior. She initially presented with exertional dyspnea, and eventually MRI revealed HCM without obstruction, maximal wall thickness with 2.5 cm and no LGE. Her father passed away at age 50 from an “MI.” Her genotyping was negative. Soon after she was having recurrent episodes of syncope and a loop recorder was placed. She came for follow was now 4 months pregnant. Just before the visit she felt pre-syncopal and Loop revealed SVT at rates of 150s. Then at 6 months pregnant, patient had one 10-beat run of NSVT at rate of 180 bpm. How would you advise this patient?

 Case #3 Quotes:

“One of my most important ways of preventing burnout in medicine – is to phone a friend, to ask for help, to talk about cases with trusted colleagues.”

• Dr. Kittleson on how to approach complex cases

Case #3 Takeaways:

 How does HCM care differ in pregnant patients per the guidelines?

1. In most pregnant women with HCM, selected beta-blockers should be given for symptoms related to LVOTO or arrhythmias with monitoring of fetal growth (Class I). Note atenolol has some evidence of potential fetal risk.
2. In terms of delivery, Valsalva during labor has generally been shown to be well tolerated. Cesarean section should typically only be done for obstetric reasons or for emergency cardiac/maternal reasons. As discussed in our Cardio-Ob series, a delivery plan should be developed by a multi-disciplinary team by the end of the second trimester
3. Most antiarrhythmic medications are contraindicated during pregnancy because of the potential teratogenic effects, and typically many are not recommended for patients with HCM. Cardioversion can typically be performed safely in pregnancy with minimal risk to the fetus and thus may be preferred for restoring sinus rhythm in symptomatic pregnant women with HCM.

How would you advise this patient regarding ICD?

1. It appears she may have a Class IIa recommendation for ICD if you believe the family history is actually positive for sudden cardiac death, otherwise she likely does not have an indication for ICD. The NSVT was present but it was one episode, not at a fast rate (<200 bpm) and thus likely does not reach to the level of recommending an ICD. Furthermore, the syncope appears attributable to SVT rather than ventricular arrhythmia.
2. Implantation ICD for primary prevention can typically wait until after pregnancy

Figure 1:

Figure 2

1. Ommen SR, Mital S, Burke MA et al. 2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2020 Dec 22;142(25):e558-e631. doi: 10.1161/CIR.0000000000000937. Epub 2020 Nov 20. Erratum in: Circulation. 2020 Dec 22;142(25):e633. PMID: 33215931.
2. Maron BJ. Clinical Course and Management of Hypertrophic Cardiomyopathy. N Engl J Med. 2018 Aug 16;379(7):655-668. doi: 10.1056/NEJMra1710575. PMID: 30110588.
3. Manrai AK, Funke BH, Rehm HL et al. Genetic Misdiagnoses and the Potential for Health Disparities. N Engl J Med. 2016 Aug 18;375(7):655-65. doi: 10.1056/NEJMsa1507092. PMID: 27532831; PMCID: PMC5292722.
4. Gersh BJ, Maron BJ, Bonow RO, et al. 2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Developed in collaboration with the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol. 2011 Dec 13;58(25):e212-60. doi: 10.1016/j.jacc.2011.06.011. Epub 2011 Nov 8. PMID: 22075469.
5. Maron BJ, Casey SA, Olivotto I et al. Clinical Course and Quality of Life in High-Risk Patients With Hypertrophic Cardiomyopathy and Implantable Cardioverter-Defibrillators. Circ Arrhythm Electrophysiol. 2018 Apr;11(4):e005820. doi: 10.1161/CIRCEP.117.005820. PMID: 29625970.

• Karan Desai, MD
• Natalie Stokes, MD
• Amit Goyal, MD
• Daniel Ambinder, MD

CardioNerds (Amit Goyal and Daniel Ambinder) join CardioNerds Ambassadors Dr. Pablo Sanchez (FIT, Stanford University) and Dr. Christine Shen (FIT, Scripps Clinic) for a discussion with Dr. Bob Harrington (Interventional Cardiologist, Professor of Medicine, and Chair of the Department of Medicine at Stanford University) about diversity and inclusion in the field of cardiology. This episode discusses Dr. Harrington’s broader approach to mentorship, sponsorship, and allyship; and particularly how (and why) he used his position as the president of the American Heart Association to advocate against all-male panels, or “manels.” Listen to the episode to learn the background and motivations behind his evidence-based efforts to make Cardiology a more inclusive field. Special message by California ACC State Chapter President, Dr. Jamal Rana.

The PA-ACC & CardioNerds Narratives in Cardiology is a multimedia educational series jointly developed by the Pennsylvania Chapter ACC, the ACC Fellows in Training Section, and the CardioNerds Platform with the goal to promote diversity, equity, and inclusion in cardiology. In this series, we host inspiring faculty and fellows from various ACC chapters to discuss their areas of expertise and their individual narratives. Join us for these captivating conversations as we celebrate our differences and share our joy for practicing cardiovascular medicine. We thank our project mentors Dr. Katie Berlacher and Dr. Nosheen Reza.

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“If senior men don’t change the field, it’s not going to change. We have the senior positions. We have to change it.”

“You’re missing talent. You’re missing talent of the women who have decided not to go into Cardiology. I say to a lot of my male colleagues…don’t you care about the health of our specialty? Don’t you want the very best people going into it?”

“How great is that–to open up an artery in the middle of the night?… What could be better than that?…Why would you not want to be a cardiologist? Frankly, maybe the field is not so friendly to women…And that bothers me greatly because I love the specialty.”

“To those who have been given much, much is expected. That’s what people like me should do.”

What are the gender disparities in the field of Cardiology?

• 45.8% of residents and fellows in ACGME-accredited programs are women. 14.9% of cardiologists are women. 8% of interventional cardiologists are women [1]. 30.6% of male faculty were full professors, while 15.9% of female faculty are full professors [2].
• Men are more likely to be influenced by positive attributes of a field, while women are more likely to be influenced by negative attributes [3].
• 3% of percutaneous coronary interventions in the United States are performed by female operators [4].

What is mentorship, sponsorship, and allyship?

• A mentor provides advice and helps someone develop a skill.
• A sponsor is an advocate who helps someone secure career advancement opportunities.
• An ally partners with people, utilizing their power and influence to champion the rights of others [5].
• According to some studies, women report less sponsorship experiences than men. Additionally, in women it seems to translate less frequently into experiences that further their career (speaking engagements, serving on editorial boards, etc) [6].
• “I’m a mentor to a few people, I’m a sponsor to many, and I’m an ally to all.” – Dr. Bob Harrington

Why is a diverse cardiovascular workforce so important?

• Teams that are diverse pursue innovative and creative solutions.
• Medicine requires meaningful connections and having a physician with a common background enhances the patient-doctor interaction by a spectrum of constructive effects.
• Minority groups are less likely to be treated with effective cardiac medications [7].
• There continue to be barriers in clinical trials to include diverse and underrepresented patients [8].
• A diverse workforce of clinical scientists is crucial to promoting diversity in clinical trials, including understanding the problem, asking the right questions, and proposing solutions [9].

1. AAMC 2019. Physician Specialty Data Report. Accessed November 18, 2021.
2. Blumenthal DM, Olenski AR, Yeh RW, et al. Sex Differences in Faculty Rank Among Academic Cardiologists in the United States. Circulation. 2017;135(6):506-517. doi:10.1161/CIRCULATIONAHA.116.023520
3. Yong CM, Abnousi F, Rzeszut AK, et al. Sex Differences in the Pursuit of Interventional Cardiology as a Subspecialty Among Cardiovascular Fellows-in-Training [published correction appears in JACC Cardiovasc Interv. 2019 Apr 8;12(7):695]. JACC Cardiovasc Interv. 2019;12(3):219-228. doi:10.1016/j.jcin.2018.09.036
4. Wang TY, Grines C, Ortega R, et al. Women in interventional cardiology: Update in percutaneous coronary intervention practice patterns and outcomes of female operators from the National Cardiovascular Data Registry®. Catheter Cardiovasc Interv. 2016;87(4):663-668. doi:10.1002/ccd.26118
5. Sharma G, Narula N, Ansari-Ramandi MM, Mouyis K. The Importance of Mentorship and Sponsorship: Tips for Fellows-in-Training and Early Career Cardiologists. JACC Case Rep. 2019;1(2):232-234. Published 2019 Aug 21. doi:10.1016/j.jaccas.2019.06.007
6. Patton EW, Griffith KA, Jones RD, Stewart A, Ubel PA, Jagsi R. Differences in Mentor-Mentee Sponsorship in Male vs Female Recipients of National Institutes of Health Grants. JAMA Intern Med. 2017;177(4):580-582. doi:10.1001/jamainternmed.2016.9391
7. Tran HV, Waring ME, McManus DD, et al. Underuse of Effective Cardiac Medications Among Women, Middle-Aged Adults, and Racial/Ethnic Minorities With Coronary Artery Disease (from the National Health and Nutrition Examination Survey 2005 to 2014). Am J Cardiol. 2017;120(8):1223-1229. doi:10.1016/j.amjcard.2017.07.004
8. Clark LT, Watkins L, Piña IL, et al. Increasing Diversity in Clinical Trials: Overcoming Critical Barriers [published correction appears in Curr Probl Cardiol. 2021 Mar;46(3):100647]. Curr Probl Cardiol. 2019;44(5):148-172. doi:10.1016/j.cpcardiol.2018.11.002
9. Poppas A, Albert MA, Douglas PS, Capers Q 4th. Diversity and Inclusion: Central to ACC’s Mission, Vision, and Values. J Am Coll Cardiol. 2020;76(12):1494-1497. doi:10.1016/j.jacc.2020.08.019

• Dr. Gurleen Kaur
• Amit Goyal, MD
• Daniel Ambinder, MD

CardioNerds (Amit Goyal and Daniel Ambinder), are joined by guest host Dr. Alex Pipilas (CardioNerds Ambassader, Boston University), and Cleveland Clinic fellows, Dr. Gary Parizher, Dr. Ambreen Ali, and Dr. Tiffany Dong. They discuss a case of an 18-year-old man with Autism Spectrum Disorder presented with advanced nonischemic dilated cardiomyopathy. Due to anxiety, he was unable to tolerate right heart catheterization, and the initial evaluation for advanced heart failure therapies was deferred. With assistance from a multidisciplinary team, catheterization was successful, and he underwent cardiac transplantation. Faculty experts, Dr. Richard Dane Meredith (Cardiovascular Imaging, Mission healthcare), Dr. Julie Niezgoda (Congenital Cardiac Anesthesiologist, CCF), and Dr. Ran Lee (Critical Care Cardiology and Advanced HF/Transplant Cardiologist, CCF) provide the E-CPR for this episode. Audio editing by CardioNerds Academy Intern, Dr. Leticia Helms.

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1. Autism spectrum disorder should not be regarded as a contraindication to organ transplantation.
2. Respect for patient discomfort with procedures, and efforts to mitigate that discomfort, are essential.
3. A multidisciplinary team approach, especially one utilizing allied health support services, is important to provide care to adolescent patients with advanced organ dysfunction, particularly those with developmental disabilities.

Autism spectrum disorder (ASD) is a developmental disability characterized by impairments in social interaction and the presence of restricted, repetitive patterns of behaviors, interests, or activities (2). In 2016 the CDC estimated one in 54 children age 8 had ASD (3). Despite ASD’s prevalence, studies of organ transplantation in children and adolescents with developmental disabilities are lacking. Guidelines from the International Society for Heart Lung Transplantation indicate that heart transplantation cannot be recommended in patients suffering from severe cognitive-behavioral disabilities (4). However, the definition of “severe” is not clear, so the assessment of severity of a cognitive impairment, as well as whether the impairment constitutes a contraindication to organ transplantation, falls to healthcare providers on a case-by-case basis.

            Cardiac transplantation in a patient with ASD has been documented previously (5). Nonetheless our case represents an important example of advocacy for lifesaving care in patients with developmental disability. Without any one component of the team taking care of our patient, including physicians and allied healthcare providers, he would have died of refractory cardiogenic shock. However, with individualized care and a multidisciplinary combined effort, his providers were able to overcome the obstacles posed by his ASD and deliver indicated interventions.

1.  Baran, David A., et al. “SCAI clinical expert consensus statement on the classification of cardiogenic shock” Catheterization and Cardiovascular Interventions 94.1 (2019): 29-37.

2. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders. 5th edition. Arlington, VA: American Psychiatric Association, 2013.

3. Baio J, Wiggins L, Christensen D, et al. Prevalence of Autism Spectrum Disorder among children aged 8 years – Autism and Developmental Disabilities Monitoring Network, 11 Sites, United States, 2014. MMWR Surveillance Summaries 2018; 67:1-23.

4. Mehra M, Canter C, Hannan M, et al. The 2016 International Society for Heart Lung Transplantation listing criteria for heart transplantation: a 10-year update. J Heart Lung Transplant 2016; 35:1-23.

5. Bailey D, Schneider L, Maeda K, et al. Orthotopic heart transplant in a child with nonverbal autism. Austin J Autism & Relat Disabil 2016;2:1017.

6. Chen Y, Shlofmitz E, Khalid N, et al. Right Heart Catheterization-Related Complications: A review of the literature and best practices. Cardiol Rev 2020;28:36-41.

• Karan Desai, MD
• Amit Goyal, MD
• Daniel Ambinder, MD

Pregnancy is a hypercoagulable state associated with increased risk of thromboembolism. Managing anticoagulation during pregnancy has implications for both the mother and the fetus. CardioNerd Amit Goyal joins Dr. Akanksha Agrawal (Cardiology Fellow at Emory University), Dr. Natalie Stokes (Cardiology Fellow at UPMC and Co-Chair of the Cardionerds Cardio-Ob series), and Dr. Katie Berlacher (Program Director of the Cardiovascular Disease Fellowship and Director of the Women’s Heart Program at UPMC) as they discuss the common indications for anticoagulation and their management before, during, and after pregnancy. In this episode, we focus on management of pregnant patients with mechanical valves and venous thromboembolism.

Audio editing by CardioNerds Academy Intern, Dr. Maryam Barkhordarian.

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1. Pregnancy is a hypercoagulable state. Pregnancy-associated VTE is a leading cause of maternal morbidity and mortality.
2. The use of anticoagulation requires a balance between the risks and benefits to the mother and her fetus.
3. The agent of choice for anticoagulation during pregnancy depends on the indication, pre-pregnancy dose of vitamin K antagonist (VKA), and the trimester of pregnancy. For instance, patients with mechanical heart valves, warfarin is generally recommended in the first trimester if the daily dose is less than 5 mg and as the first option for all patients with mechanical valves in the 2nd and 3rd trimester. Use of direct oral anticoagulants (DOACs) has not been systematically studied, they do cross the placenta and their safety remains untested.
4. Warfarin crosses the placenta but is not found in breast milk. LMWH does not cross the placenta and is not found in breast milk. Thus, both these agents can be used by a lactating mother.

“[We] can’t highlight enough that good communication and documentation is vital in such situations” says Dr. Berlacher while discussing the role of a multidisciplinary team including cardiologists, obstetricians and fetal medicine physicians in taking care of a pregnant patient on anticoagulation.

“What I love about cardio-obstetrics is that we really can help women in a time that is so important in their life…this is one of the most memorable times in their life..” says Dr. Berlacher when asked what makes your heart flutter about cardio-obstetrics.

“Knowledge is power…not just for providers, but also for the patients” says Dr. Berlacher emphasizing the importance of clear communication between physicians and patients.

1. What makes pregnancy a hypercoagulable state?

• Pregnancy is a hypercoagulable state associated with higher risk of thromboembolic phenomenon. The three components of Virchow’s triad: hypercoagulability, stasis, and endothelial injury are all present during pregnancy. This leads to a 5-fold increased risk of venous thromboembolism (VTE) during pregnancy that persists for 12 weeks postpartum. The risk for VTE seems to be highest in the first 6 weeks postpartum, with a higher prevalence of clot in the left lower extremity.
• There are additional risk factors for developing VTE in the postpartum period besides pregnancy itself, and this includes but is not limited to preeclampsia, emergent c-section, hypertension, smoking, and postpartum infection.
• Choosing anticoagulant therapies during pregnancy involves a fine balance between the risks and benefits to both the mother and fetus. A multidisciplinary team involving the obstetrician, cardiologist, and maternal-fetal medicine team is critical to guide anticoagulation in pregnancy.

2. What are some of the common indications for anticoagulation during pregnancy?

• One of the most common indications for anticoagulation in pregnancy is valvular disease, and specifically mitral valve stenosis with atrial fibrillation or a prior embolic event.
• Patients with a mechanical heart valve will require anticoagulation during pregnancy. Patients with a bioprosthetic valve (surgical or transcatheter) are generally continued on low dose Aspirin; in the uncommon scenario of pregnancy in the first 3-6 months following implantation of a bioprosthetic valve, the decision to pursue anticoagulation is individualized.
• Other indications include acute VTE, atrial fibrillation, antiphospholipid syndrome, and inherited thrombophilias that may predispose a patient to developing VTE during pregnancy.

3. For mechanical heart valves, how do anticoagulation recommendations vary based on trimester?

• The European Society of Cardiology has divided various valvular heart diseases into 4 classes as per the modified World Health Organization classification of maternal cardiovascular risk, and having a mechanical valve falls under Class III where the maternal cardiac event rate varies between 19-27%. Such patients should get their care at expert centers for pregnancy and cardiac disease.
• Anticoagulation for mechanical valve during pregnancy varies with each trimester to balance the risks and benefits. During the first trimester, the period of organogenesis, the decision of whether to continue warfarin (a potential teratogenic) depends on the dose of warfarin. If a patient has been taking 5 mg/day of warfarin, the American Heart Association (AHA) recommends stopping warfarin and using alternate agents like LMWH/UFH (Class IIa).
• During the second and third trimesters, it is typically advised to continue warfarin until prior to the vaginal delivery when continuous infusion of UFH should be used as the anticoagulant agent of choice. Expert multidisciplinary teams are needed not only to guide these general recommendations, but to individualize the treatment based on patient preferences and specific factors (e.g., previous prosthetic valve thromboembolic complication).
• For a mechanical mitral valve replacement, 2014 AHA/CC guidelines recommend a goal INR of 3.0. For a mechanical aortic valve replaceent, the goal depends on the presence or absence of risk factors. In a patient with high-risk conditions like atrial fibrillation, previous thromboembolism, LV dysfunction, hypercoagulable condition, and older-generation mechanical valve, a goal INR of 3.0 (2.5 to 3.5), similar to MVR is recommended. However, if no high-risk features exist, then an INR goal of 2.5 (2.0 to 3.0) is recommended. Additionally, no additional bridging is required in the latter group of patients if their VKA therapy is interrupted for non-cardiac procedures. With certain AVR valves and no other risk factors (e.g. ON-X), a lower INR goal may be pursued.

4. What are the major differences between Warfarin, Heparin products, and DOACs in pregnancy and lactation?

• Warfarin crosses the placenta and has a dose-dependent relationship with adverse fetal outcomes (e.g., miscarriage, stillbirth, embryopathy). Warfarin’s teratogenic effects are also trimester-dependent with fetal bone and cartilage abnormalities occurring in the 1st trimester and CNS abnormalities (e.g., microencephaly, spasticity, hypotonia, optic atrophy) if teratogenic levels are reached in the 2nd and 3rd trimester. 
• When compared with LMWH and UFH, warfarin has the least maternal risk for those with mechanical heart valves, but lowest rates of livebirths. [4] LMWH does not cross the placenta and is associated with the highest number of livebirths. However, the challenges of using LMWH include its monitoring. Weight-based LMWH should be accompanied by peak anti-Xa levels drawn 4-6 hours post-dose to achieve a goal level of 1.0-1.2 U/ml.
• UFH is the preferred agent of choice at the time of delivery, since this is the highest period of bleeding for a pregnant woman. It is usually stopped 4-6 hours before delivery and restarted 4-6 hours after delivery if there is no bleeding.
• DOACs have not been studied in pregnant patients on a large scale. And the limited data present revealed a high miscarriage rate and possible embryopathy. There use is not recommended in pregnant women.
• Anticoagulants such as UFH, LMWH, warfarin, fondaparinux, or danaparoid are all recommended as safe options for breastfeeding women with indication for anticoagulation. DOACs are not recommended for lactating women.

5. What are the recommendations for VTE management during pregnancy?

• Venous thromboembolism (VTE) complicates ∼1.2 of every 1000 deliveries. Despite these low absolute risks, pregnancy associated VTE is a leading cause of maternal morbidity and mortality.
• Women with VTE on chronic anticoagulation are recommended to continue anticoagulation during (and after) pregnancy. Weight-based LMWH guided by Xa levels (to achieve a goal level of 1.0-1.2 U/ml) is the preferred agent, but warfarin (daily dose ≤5 mg) is an alternative.
• In patients with recent pulmonary embolism (PE), postpartum heparin treatment should be restarted 6 hours after a vaginal birth and 12 hours after a caesarean delivery if no significant bleeding has occurred. There should be subsequent overlap with VKAs for at least 5 days.
• In the absence of significant bleeding, VKAs may be started on the second day after delivery and continued for at least 3 months, or for 6 months if PE occurred late in pregnancy. The goal INR should be between 2 and 3.
• Anticoagulation decisions are complex and should be determined in collaboration with a multidisciplinary cardio-obstetrics team.

1. Alshawabkeh L, Economy KE, Valente AM . Anticoagulation During Pregnancy: Evolving Strategies With a Focus on Mechanical Valves. J Am Coll Cardiol 2016;68:1804-1813.
2. Regitz-Zagrosek V, Roos-Hesselink JW, Bauersachs J, et al. 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy. The Task Force for the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology. European Heart Journal. 2018, 39, 3165-3241.
3. Nishimura RA, Otto CM, Bonow RO et al. 2014 AHA/ACC Guideline for the Management of Patients with Valvular Heart Disease: Executive Summary. A Report of the AMerican College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation. 2014; 129:e521-e643.
4. Richardson A, Shah S, Harris C, McCulloch G, Antoun P. Anticoagulation for the Pregnant Patient with a Mechanical Heart Valve, No Perfect Therapy: Review of Guidelines for Anticoagulation in the Pregnant Patient. Case Rep Cardiol. 2017;2017:3090273.
5. Shannon M. Bates, Anita Rajasekhar, Saskia Middeldorp, Claire McLintock, Marc A. Rodger, Andra H. James, Sara R. Vazquez, Ian A. Greer, John J. Riva, Meha Bhatt, Nicole Schwab, Danielle Barrett, Andrea LaHaye, Bram Rochwerg; American Society of Hematology 2018 guidelines for management of venous thromboembolism: venous thromboembolism in the context of pregnancy. Blood Adv 2018; 2 (22): 3317–3359.
6. Lameijer H, Aalberts JJJ, van Veldhuisen DJ, Meijer K, Pieper PG. Efficacy and safety of direct oral anticoagulants during pregnancy; a systematic literature review. Thromb Res. 2018 Sep;169:123-127.

Katie Berlacher, MD, is a cardiologist and is certified in cardiovascular disease by the American Board of Internal Medicine and adult echocardiography by the National Board of Echo. She is the medical director of the Magee Women’s Heart Program, the program director of cardiovascular fellowship, and is an assistant professor of medicine at the University of Pittsburgh School of Medicine. She received her medical degree from The Ohio State University and completed her residency and fellowship at the University of Pittsburgh Medical Center.

Dr. Berlacher joined the UPMC Heart and Vascular Institute in 2012. Her clinical interests include women’s heart disease, including pregnancy and heart disease risks, as well as medical education. She has published numerous articles in peer-reviewed journals and is a member of the American College of Cardiology and the American Heart Association. She lives in the city and is an avid cyclist, boxer, and hiker.

Akanksha is a cardiology fellow at Emory University. She did her medical school from Maulana Azad Medical College, India, and Internal Medicine Residency at Einstein Medical Center, Philadelphia, where she did a year of chief residency as well. She is interested in cardio-obstetrics and advanced heart failure, and plans to pursue Advanced Heart Failure and Cardiac Transplant fellowship.

• Natalie Stokes, MD
• Sonia Shah, MD
• Amit Goyal, MD
• Daniel Ambinder, MD

The CardioNerds are thrilled to launch The Cardiac Critical Care Series! The series Co-Chairs – Dr. Mark Belkin (Advanced Heart Failure FIT, U Chicago), Dr. Yoav Karpenshif (FIT, U Penn), Dr. Eunice Dugan (CardioNerds Academy Chief Fellow and FIT, Cleveland Clinic), and Dr. Karan Desai (CardioNerds Academy Editor and FIT, U Maryland) – join CardioNerds Co-Founders, Amit Goyal and Daniel Ambinder to delve into high-yield topics in critical care cardiology.

We kickstart this series with one of the early pioneers and national leaders in cardiac critical care – Dr. Jason Katz, Director of Cardiovascular Critical Care and Co-Director of Mechanical Circulatory Support and the CICU at Duke University Medical Center.

In this episode, we learn about Dr. Katz’s career path and what motivated him to train in Critical Care Cardiology. He shares early struggles, notable changes in this field’s nascent period, and ongoing challenges in training and practice. We discuss collaboration with other cardiac and non-cardiac specialties and their importance in comprehensive care. Furthermore, we discuss how to advance critical care research, including the Critical Care Cardiology Trials Network and future randomized controlled trials to inform our practice and develop standardized protocols. In this small but rapidly growing field, we learn there is much to discover together. Audio editing by CardioNerds Academy Intern, Hirsh Elhence.

Claim free CME for enjoying this episode! Disclosures: None

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1. “I think it’s really important not to be afraid of change in order to potentially succeed […] it’s okay to not entirely know what you want to do.” – Dr. Katz when sharing his non-direct career path in a novel field.
2. “There is no greater team sport in cardiology than Cardiac Critical Care” and “I oftentimes think of the Critical Care Cardiologist as sort of the conductor that helps to orchestrate [the team]…” – Dr. Katz when discussing the importance of multidisciplinary teams and need to collaborate with other cardiac and non-cardiac sub-specialties.
3. Many general surgical or medical residency/fellowship graduates are not comfortable caring for patients in the critical care setting. There is a need to revamp critical care training without significantly prolonging training time in order to complement and enhance our current workforce to care for complex, critically ill cardiac patients.
4. “I don’t think there’s necessarily a one size fits all model, and I think we should be malleable or adaptable to the needs of our trainees and the needs of our patients.” – Dr. Katz when discussing training pathways in Critical Care Cardiology or combining Critical Care with other subspecialties like Interventional Cardiology or Advanced Heart Failure.
5. Dr. Katz suggests that when choosing a Critical Care Cardiology training program: “consider geography, the flexibility of the curriculum, the overall fellowship and social experience, and the clinical setting. Everything that’s really important to choosing a cardiology fellowship is more important in my mind than if they actually have a standardized, cardiac critical care pathway.”

1. What are some recent changes in the field of Critical Care Cardiology?

• Compared to even just a decade ago, there is a growing interest from medical students to young faculty in pursuing a career in critical care cardiology.
• At the same time there is evidence that the patient demographics in our CICUs are changing, including more multi-organ dysfunction and many non-cardiac diagnoses. In a recent paper from the Critical Care Cardiology Trials Network (CCCTN), the proportion of patients with a primary diagnosis of acute coronary syndrome was only approximately 32%. Following overall trends in heart failure, a greater proportion of CICU patients have heart failure and/or heart failure phenotype cardiogenic shock requiring clinicians to be facile with the spectrum of mechanical support devices.
• These demographic and workforce trends are requiring cardiologists to re-think how to best meet the needs of the modern CICU.

2. What continue to be some challenges in practicing Critical Care Cardiology?

• CICU patients are a heterogenous group with varying baseline characteristics, comorbidities, illness severity, and treatment responses. Since distinct pathophysiologic targets are lacking, challenges exist when devising strategies to improve outcomes. Similarly, these challenges extend to developing and executing research protocols to inform management.
• As Dr. Katz discussed on the episode, the lack of standardization, consistent terminology, and even who belongs in the CICU, remains a barrier to devising who should staff the CICU and how we train physicians to care for CICU patients.
• Dr. Katz described the cardiac intensivist as akin to the conductor of an orchestra working with multiple cardiac and non-cardiac subspecialists, and other professionals such as RNs, dieticians, physical therapy, social work, chaplaincy etc.  Understanding the roles of multidisciplinary members can help inform resource utilization and allocate costs.

3. What are some challenges in Critical Care Cardiology training?

• There continues to be a supply-demand mismatch when it comes to the growing clinical need and the availability of trained cardiac critical care clinicians.
• Many surgical or medical residency/fellowship graduating trainees do not feel comfortable caring for critically ill patients. Thus, Dr. Katz advocates for outside the box thinking to find novel ways to supplement current cardiovascular training – whether it be in general fellowship, advanced heart failure or interventional training – with adequate critical care training for interested trainees.
• Accomplishing this goal without excessive increases in numbers of years in training – and assuring competency and exposure to aspects of critical care medicine not seen in general cardiology training – may require creating more blended pathways
• Furthermore, the optimal timing of when to obtain critical care training remains unclear. As few integrated options currently exist, trainees most commonly pursue critical care training after general cardiology or subspecialty cardiology fellowship training. Without integrated options, however, there is concern that trainees may not maintain their cardiac clinical skills or competency in areas such as echocardiography during dedicated critical care medicine years.

4. What about combining critical care with other cardiac-subspecialities like Interventional Cardiology or Advanced Heart Failure?

• There isn’t a “one size fits all” model. Training pathways should be flexible to accommodate the needs and interests of trainees. Recent opinion pieces have suggested blending Advanced Heart Failure training with Critical Care Training in a preferably 5-year pathway.
• Regardless of who is staffing the CICU, optimal care requires continued collaboration – not just during admission but frequent revisitation of management plans with cardiac subspecialists to direct patient care.

5. What can we learn from our cardiac intensivist colleagues in Europe?

• Europe has a more established training pathway for cardiac critical care with a dedicated scientific symposium, credentialing pathway, and journal.
• Although we have different regulatory authorities and settings for care of delivery, with colleagues in Europe having been through the process of establishing this new field, we can learn from their struggles and successes.
• Furthermore, there are opportunities for multinational collaboration in research, training, and education.

Il’Giovine ZJ, Menon V. The Intersection of Heart Failure and Critical Care: The Contemporary Cardiac Intensive Care Unit and the Opportunity for a Unique Training Pathway. J Card Fail. 2021 Oct;27(10):1152-1155. doi: 10.1016/j.cardfail.2021.03.014. PMID: 34625134.

Katz J, Turer A, Becker R. Cardiology and the critical care crisis: a perspective. Journal of the American College of Cardiology. Published online 2007. doi:10.1016/J.JACC.2006.11.036

Bhatt AS, Berg DD, Bohula EA, et al. De Novo vs Acute-on-Chronic Presentations of Heart Failure-Related Cardiogenic Shock: Insights from the Critical Care Cardiology Trials Network Registry. J Card Fail. 2021 Oct;27(10):1073-1081. doi: 10.1016/j.cardfail.2021.08.014. PMID: 34625127; PMCID: PMC8514080.

Dr. Jason Katz is the Director of the Cardiac ICU, Mechanical Circulatory Support, and LVAD programs at Duke University. After completing his Internal Medicine residency at UT-Southwestern, he went on to complete a Cardiology Fellowship at Duke University, a Clinical Research Fellowship with the DCRI, and a finally Critical Care Fellowship at Duke. He has published over 100 articles across a range of topics within Cardiac Critical Care, including multiple reviews and statements addressing the role of, and training options for, Cardiac Intensivists. He is considered an early pioneer, and continues to be a leader, in this growing field, currently serving as the immediate past-President of the AHA Acute Cardiac Care Committee.

• Karan Desai, MD
• Dr. Mark Belkin
• Amit Goyal, MD
• Daniel Ambinder, MD

CardioNerds Tommy Das (Program Director of the CardioNerds Academy and cardiology fellow at Cleveland Clinic) and Rick Ferraro (Director of CardioNerds Journal Club and cardiology fellow at the Johns Hopkins Hospital) join Dr. Erin Michos (Associate Professor of Cardiology at the Johns Hopkins Hospital and Editor-In-Chief of the American Journal of Preventative Cardiology) for a discussion about the effect of DHA and EPA on triglycerides and why DHA/EPA combinations may have exhibited limited benefits in trials. This episode is part of the CardioNerds Lipids Series which is a comprehensive series lead by co-chairs Dr. Rick Ferraro and Dr. Tommy Das and is developed in collaboration with the American Society For Preventive Cardiology (ASPC).

Relevant disclosures: None

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1. The best intervention for heart disease is prevention! The InterHeart trial showed that 9 modifiable risk factors (dyslipidemia, smoking, hypertension, diabetes, abdominal obesity, dietary patterns, physical activity, consumption of alcohol, and psychosocial factors) predict 90% of acute myocardial infarction. So many acute events can be prevented1.
2. Atherosclerotic vascular disease events increase across a range of triglyceride levels, even from 50-200mg/dL. So even in a relatively normal range, lower triglycerides seem to be better. Over ¼ of US adults have triglycerides over 150.
3. While 8% of US adults take fish oil supplements, multiple meta-analyses have failed to show any benefit to the use of dietary omega-3 supplementation2. Dietary supplements these are not meant for medical use and are not studied or regulated as such!

1. What are DHA and EPA?

• DHA, or docosahexaenoic acid, and EPA, or eicosapentaenoic acid, are n-3 polyunsaturated fatty acids, also known as omega-3 fatty acids. These compounds have been of considerable interest for over two decades given observed association of high dietary omega-3 fatty acid intake with reduced cardiovascular events3. As both are important omega-3 fatty acids, trials on the benefits of DHA and EPA have often focused on the two compounds in combination.

2. What was the GISSI-Prevenzione Trial and why was it Important?

• GISSI-Prevenzione trial (Lancet 1999), was one of the earliest trials to study DHA and EPA4. In this trial, the authors evaluated the effect of omega-3 supplementation as a combination pill of DHA and EPA on cardiovascular events and death in patients with recent myocardial infarction (the last three months). Over a 3.5-year follow-up period, participants treated with DHA/EPA combination experienced a significant reduction in death, nonfatal MI, and stroke.
• As this was an early trial, patients were largely not on statins, as these were not supported at the time of study initiation (Only 5% were on cholesterol-lowering medications at baseline, and only 45% were on cholesterol-lowering therapy at study completion). The benefits seen in this trial may not extend to modern practice with patients on contemporary background therapy.
• The trial participants were also not representative of our modern patients for a variety of other reasons. 85% of participants in the trial were men. 42.2% of patients in EPA/DHA arm were current smokers, and 35.4% were prior smokers. Only 14.2% of patients had diabetes and 14.7% with BMI >30.
• Notably, the decrease in triglycerides in this trial was only 3%, implying that triglyceride lowering did not entirely explain the benefit in cardiovascular events seen.

3. What about the data after the GISSI-Prevensione Trial?

• After this positive trial for DHA/EPA in combination, subsequent trial data in support of DHA/EPA has been less robust.
• The Alpha Omega trial in 2010, ORIGIN in 2012, ASCEND in 2018, and VITAL in 2019 were all trials of DHA/EPA combinations versus placebo, and all exhibited no significant differences in cardiovascular events with DHA/EPA use5–8.
• The recent STRENGTH trial, published in 2020, also showed no reduction in cardiovascular events when taking DHA/EPA in combination (and as discussed in CardioNerds episode 136!)9. This remained the case upon sub-analysis of patients from the STRENGTH trial with the highest levels of serum EPA, who again exhibited no cardiovascular benefit.

4. Why then don’t DHA and EPA seem to work in combination?

• In short, we do not know.
• It seems that the beneficial effects of EPA – which as monotherapy has shown benefit across numerous trials (namely JELIS and REDUCE-IT) – are somehow offset by the combination with DHA, via a mitigation of anti-inflammatory processes or otherwise – this remains theoretical10,11.
• The EVAPORATE trial of purified EPA showed lower total plaque in participants taking EPA, suggesting a possible mechanism of effect12.

5. What About Dietary Omega-3 Supplements?

• 8% of US adults take fish oil supplements.
• Multiple meta-analyses have failed to show any benefit to the use of dietary omega-3 supplementation2.
• Note that the term “over the counter” is not correct when referring to these supplements! Over the counter refers to medications that are FDA regulated, just not prescription. Omega-3 supplements have minimal FDA oversight, which is perhaps another reason to avoid them.

1.     Yusuf S, Hawken S, Ôunpuu S, et al. Effect of potentially modifiable risk factors associated with myocardial infarction in 52 countries (the INTERHEART study): case-control study. The Lancet. 2004;364(9438):937-952. doi:10.1016/S0140-6736(04)17018-9

2.     Abbasi J. Another Nail in the Coffin for Fish Oil Supplements. JAMA. 2018;319(18):1851-1852. doi:10.1001/jama.2018.2498

3.     Bang HO, Dyerberg J, Hjørne N. The Composition of Food Consumed by Greenland Eskimos. Acta Medica Scandinavica. 1976;200(1-6):69-73. doi:10.1111/j.0954-6820.1976.tb08198.x

4.     GISSI-Prevenzione, Investigators. Dietary supplementation with n-3 polyunsaturated fatty acids and vitamin E after myocardial infarction: results of the GISSI-Prevenzione trial. The Lancet. 1999;354(9177):447-455. doi:10.1016/S0140-6736(99)07072-5

5.     Kromhout D, Giltay EJ, Geleijnse JM. n–3 Fatty Acids and Cardiovascular Events after Myocardial Infarction. N Engl J Med. 2010;363(21):2015-2026. doi:10.1056/NEJMoa1003603

6.     The ORIGIN Trial Investigators. n–3 Fatty Acids and Cardiovascular Outcomes in Patients with Dysglycemia. N Engl J Med. 2012;367(4):309-318. doi:10.1056/NEJMoa1203859

7.     The ASCEND Study Collaborative Group. Effects of n−3 Fatty Acid Supplements in Diabetes Mellitus. N Engl J Med. 2018;379(16):1540-1550. doi:10.1056/NEJMoa1804989

8.     Manson JE, Cook NR, Lee IM, et al. Vitamin D Supplements and Prevention of Cancer and Cardiovascular Disease. N Engl J Med. 2018;380(1):33-44. doi:10.1056/NEJMoa1809944

9.     Nicholls SJ, Lincoff AM, Garcia M, et al. Effect of High-Dose Omega-3 Fatty Acids vs Corn Oil on Major Adverse Cardiovascular Events in Patients at High Cardiovascular Risk: The STRENGTH Randomized Clinical Trial. JAMA. 2020;324(22):2268-2280. doi:10.1001/jama.2020.22258

10.   Bhatt DL, Steg PG, Miller M, et al. Cardiovascular Risk Reduction with Icosapent Ethyl for Hypertriglyceridemia. N Engl J Med. 2019;380(1):11-22. doi:10.1056/NEJMoa1812792

11.   Yokoyama M, Origasa H, Matsuzaki M, et al. Effects of eicosapentaenoic acid on major coronary events in hypercholesterolaemic patients (JELIS): a randomised open-label, blinded endpoint analysis. The Lancet. 2007;369(9567):1090-1098. doi:10.1016/S0140-6736(07)60527-3

12.   Budoff MJ, Bhatt DL, Kinninger A, et al. Effect of icosapent ethyl on progression of coronary atherosclerosis in patients with elevated triglycerides on statin therapy: final results of the EVAPORATE trial. European Heart Journal. 2020;41(40):3925-3932. doi:10.1093/eurheartj/ehaa652

Dr. Erin Donnelly Michos is an Associate Professor of Medicine at Johns Hopkins School of Medicine, with joint appointment in the Department of Epidemiology at the Johns Hopkins Bloomberg School of Public Health. She is the Director of Women’s Cardiovascular Health and the Associate Director of Preventive Cardiology with the Johns Hopkins Ciccarone Center for the Prevention of Cardiovascular Disease. Dr. Michos completed medical school at Northwestern University in Chicago, IL, and then completed both her Internal Medicine residency and Cardiology fellowship at the Johns Hopkins Hospital in Baltimore, MD.  She also completed her MHS in Cardiovascular Epidemiology at the Johns Hopkins Bloomberg School of Public Health. She has authored or co-authored over 300 manuscripts in peer reviewed journals and is an internationally known leader in preventive cardiology and women’s health. 

CardioNerds (Amit Goyal and Daniel Ambinder), join CardioNerds FIT Ambassador, Dr. Natasha Cuk and her co-fellows, Dr. Lily Stern, and Dr. Paul Marano from the Cedars-Sinai Cardiology Fellowship for some late afternoon smoothies on the beach. They discuss the case of a 46-year-old woman who presented with sudden cardiac arrest and was ultimately found to have a mobile intraluminal aortic thrombus adherent to a penetrating ulcer in the ascending aorta. This mobile thrombus was ultimately thought to be the cause of transient ischemia and the patient’s cardiac arrest. We discuss a differential for sudden cardiac arrest, initial management after resuscitated cardiac arrest, a differential for arterial thrombus, and review an illness script for penetrating atherosclerotic ulcers. Dr. Dominick Megna provides the provides the E-CPR for this episode. Audio editing by Dr. Gurleen Kaur (Director of the CardioNerds Internship).

This Case Report has been published in JACC Case Reports!

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46W no known PMH BIBA after sudden cardiac arrest. Normal trops, BNP, ECG, TTE. Extubated HD1 w/o any memory of the event.

S/p CCTA w clean cors but aorta with this 😳…

🧵1/ pic.twitter.com/jI89UXNUtq

1. After cardiac arrest, the initial ECG obtained after the return of spontaneous circulation can provide important information on the etiology of the arrest. The ECG can narrow our structured differential, for which one approach would be the following breakdown: acute ischemic events, structural heart disease, arrhythmogenic syndromes with no structural abnormality, and then non-cardiac causes such as drugs, toxins, trauma, metabolic arrangements
2. The risk vs benefit of emergent angiography after sudden cardiac arrest depends on balancing the potential benefit from revascularization in an acute ischemic event vs bleeding risks and potential delays in other aspects of care, particularly given that a large percentage of mortality is related to neurologic injury from the arrest, which would not be impacted by immediate angiography. The available randomized controlled trial evidence has not demonstrated a survival or neurologic outcome benefit to immediate angiography, and the decision depends on weighing the risk/benefit for each patient.
3. Due to high flow, a thrombus in the aorta should prompt an investigation for causes focused on the other two ‘points’ of Virchow’s triad (aside from stasis): endothelial injury and hypercoagulability.
4. A penetrating atherosclerotic ulcer (PAU) is a deep atherosclerotic lesion where there is a focal ulceration of the elastic lamina that extends through the medial layer of the aortic wall. These lesions are most commonly associated with extensive atherosclerosis, but can also occur related to inflammatory, infectious, or traumatic causes.
5. A PAU is a type of acute aortic syndrome and accounts for up to 8% of total acute aortic syndromes. It may present with a spectrum of symptoms, including as an incidental finding on cardiothoracic imaging or a severe chest and back pain, like an aortic dissection. While it is a subtype of aortic syndrome, PAU can also progress to become aortic dissection and rupture.

1. How might a post-ROSC ECG help determine the etiology of a sudden cardiac arrest?

During our case, we discussed a systematic approach to the differential diagnosis for sudden cardiac arrest. We broke down the causes into the buckets of:

• Acute ischemic events
• Structural heart disease
• Arrhythmogenic syndromes with no structural heart disease
• Non-cardiac causes such as drugs, toxins, trauma, and metabolic arrangements

The post-ROSC ECG can provide immediate information to help narrow our differential. Evidence of acute ischemia (e.g. STEMI) would provide a likely etiology and would direct immediate next steps. We can look at ECG features such as axis and conduction abnormalities to look for evidence of an underlying structural abnormality. We can also see features of arrhythmogenic syndromes without underlying structural defects, such a short or long QT or a Brugada pattern.

2. Should all patients who present with sudden cardiac arrest and for whom ROSC is achieved undergo immediate coronary angiography?

In considering the timing of coronary angiography after sudden cardiac arrest, clinical experience and the available evidence indicate a tension between:

1. The high pre-test probability for acute ischemic events as the etiology for VT/VF arrest, and possible improvement in post-ROSC outcomes from immediate coronary angiography (and revascularization).
2. A large portion of the morbidity and mortality associated with sudden cardiac arrest is driven by neurologic injury. Immediate angiography may expose patients to additional risk (delays in targeted temperature management, bleeding risk), without benefit to a patient’s ultimate outcome due to neurologic injury.

Observational data has suggested a benefit for immediate angiography after resuscitated sudden cardiac arrest, though there was concern for selection bias. Recently, there have been two randomized controlled trials that have investigated the role for immediate angiography after sudden cardiac arrest. These trials are the COACT and PEARL trials, published in 2019 and 2020, respectively. These trials each have their own limitations, though they did not demonstrate a benefit for immediate angiography on outcomes such as mortality or neurologic outcomes. There are multiple ongoing trials to provide further guidance.

The most recent AHA/ACC guideline on the topic recommends immediate coronary angiography for patients with STEMI on the post-ROSC ECG (Class I), and give a Class IIa recommendation that emergency coronary angiography is reasonable for selected patients (e.g. hemodynamically or electrically unstable) with sudden cardiac arrest of suspected cardiac origin without STEMI.

3. What is an illness script for penetrating atherosclerotic ulcers?

Definitions: A penetrating atherosclerotic ulcer (PAU) is a deep atherosclerotic lesion where there is a focal ulceration of the elastic lamina that extends through the medial layer of the aortic wall. PAU is a type of acute aortic syndrome (along with other disease processes such as aortic dissection, intramural hematoma). While a distinct entity, PAU can also progress and lead to intramural hematoma, dissection, and even aortic rupture.

Epidemiology: PAU accounts for 8% of total acute aortic syndromes. It is typically associated with extensive atherosclerosis in older adults (age > 65) and is most commonly found in the descending thoracic aorta. It is less commonly associated with infectious, inflammatory, or traumatic etiologies.

Clinical Presentation: PAU may present with a spectrum of symptoms, including as an incidental finding on cardiothoracic imaging or a severe chest and back pain, like an aortic dissection.

Diagnosis: PAU can be diagnosed by multiple imaging modalities, including computed tomography angiography, magnetic resonance imaging, and transesophageal echocardiography.

4. How are penetrating aortic ulcers treated?

Management of symptomatic penetrating aortic ulcers is similar to management of aortic dissection with indications for surgery including recurrent pain despite medical treatment, hemodynamic instability, aortic diameter enlargement to >55 mm, and significant periaortic hemorrhage. In asymptomatic patients who are hemodynamically stable, management is controversial–some centers support aggressive early surgical intervention while others opt for conservative medical management along with serial surveillance for aortic enlargement.

1. Lemkes JS, Janssens GN, van der Hoeven NW, Jewbali LSD, Dubois EA, Meuwissen M, Rijpstra TA, Bosker HA, Blans MJ, Bleeker GB, Baak R, Vlachojannis GJ, Eikemans BJW, van der Harst P, van der Horst ICC, Voskuil M, van der Heijden JJ, Beishuizen A, Stoel M, Camaro C, van der Hoeven H, Henriques JP, Vlaar APJ, Vink MA, van den Bogaard B, Heestermans TACM, de Ruijter W, Delnoij TSR, Crijns HJGM, Jessurun GAJ, Oemrawsingh PV, Gosselink MTM, Plomp K, Magro M, Elbers PWG, van de Ven PM, Oudemans-van Straaten HM and van Royen N. Coronary Angiography after Cardiac Arrest without ST-Segment Elevation. New England Journal of Medicine. 2019;380:1397-1407.
2. Kern KB, Radsel P, Jentzer JC, Seder DB, Lee KS, Lotun K, Janardhanan R, Stub D, Hsu CH and Noc M. Randomized Pilot Clinical Trial of Early Coronary Angiography Versus No Early Coronary Angiography After Cardiac Arrest Without ST-Segment Elevation: The PEARL Study. Circulation. 2020;142:2002-2012.
3. Callaway CW, Donnino MW, Fink EL, Geocadin RG, Golan E, Kern KB, Leary M, Meurer WJ, Peberdy MA, Thompson TM and Zimmerman JL. Part 8: Post–Cardiac Arrest Care. Circulation. 2015;132:S465-S482.
4. Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE, Eagle KA, Hermann LK, Isselbacher EM, Kazerooni EA, Kouchoukos NT, Lytle BW, Milewicz DM, Reich DL, Sen S, Shinn JA, Svensson LG and Williams DM. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the Diagnosis and Management of Patients With Thoracic Aortic Disease. Circulation. 2010;121:e266-e369.

• Karan Desai, MD
• Amit Goyal, MD
• Daniel Ambinder, MD

CardioNerds (Amit Goyal and Daniel Ambinder),  ACHD series co-chair Dr. Agnes Koczo (UPMC), and episode FIT lead, Dr. Natasha Wolfe (Washington University) join Dr. Ari Cedars   (Director of the Adult Congenital Heart Disease Program at Johns Hopkins) for a discussion about coarctation of the aorta.  

In this episode we discuss the presentation and management of unrepaired and repaired coarctation of the aorta in adults. We discuss the unique underlying congenital anatomy of coarctation and how that impacts physiology, clinical presentation, and diagnostic findings. We discuss the importance of long-term routine follow-up and screening of patients (including those who have been “repaired”) for common complications such as hypertension, re-coarctation, and aneurysm development. We end with a discussion of treatment options for coarctation and its complications.

Audio editing by CardioNerds Academy Intern, Dr. Maryam Barkhordarian.

The CardioNerds Adult Congenital Heart Disease (ACHD) series provides a comprehensive curriculum to dive deep into the labyrinthine world of congenital heart disease with the aim of empowering every CardioNerd to help improve the lives of people living with congenital heart disease. This series is multi-institutional collaborative project made possible by contributions of stellar fellow leads and expert faculty from several programs, led by series co-chairs, Dr. Josh Saef, Dr. Agnes Koczo, and Dr. Dan Clark.

The CardioNerds Adult Congenital Heart Disease Series is developed in collaboration with the Adult Congenital Heart Association, The CHiP Network, and Heart University. See more

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1. Coarctation of the aorta can occur as a discrete stenosis or as a long and hypoplastic hypoplastic aortic arch segment. Most commonly it is a discrete stenosis located at the insertion site of the ductus arteriosus just distal to the left subclavian artery.
2. Three quarters of patients with coarctation of the aorta also have a bicuspid aortic valve.
3. Hypertension is the most common long-term complication of coarctation of the aorta, whether repaired or unrepaired. Unrepaired coarctation is a rare cause of secondary hypertension in young adults with a difference in upper extremity and lower extremity BP by ≥ 20 mmHg. Systemic hypertension may not be consistently identifiable at rest in those with repaired coarctation, thus guidelines recommend ambulatory blood pressure monitoring or stress testing to identify hypertension with exertion.
4. Chest and brain imaging via CT or MRI should be done every 5-10 years to screen for other long-term complications including re-coarctation (rate ~11%), aortic aneurysm development (higher risk in those with concurrent bicuspid aortic valve), pseudoaneurysm, aortic dissection, and cerebral aneurysms.
5. Repair of coarctation or re-coarctation is indicated for patients who are hypertensive with a BP gradient ≥ 20 mmHg (Class I recommendation). Catheter-based stenting is the preferred approach when technically feasible.

1. What is the proposed embryologic origin of coarctation of the aorta?

• The aortic arch and its branches develop at 6-8 weeks fetal gestation. We all start with six aortic arches that go on to become the great arteries of the head and neck. The 4th arch forms the thoracic aortic arch and isthmus. The 6th arch persists as the proximal pulmonary arteries and ductus arteriosus. Thoracic aortic coarctation is therefore a manifestation of abnormal embryologic development of the 4th and 6th arches.
• There are two main theories regarding how aortic coarctation occurs.
• The first is the “ductus tissue theory”, which proposes that coarctation develops as the result of migration of ductal smooth muscles cells in the periductal aorta, with subsequent constriction and narrowing of the aortic lumen.
• The “hemodynamic theory” proposes that coarctation develops because of hemodynamic disturbances from other left sided congenital lesions that reduce the volume of blood flow through the fetal aortic arch – recall the “no flow, no grow” tenet.
• In all likelihood, both are true and play a role in aortic coarctation embryologic development.

2. What are the key features of aortic coarctation anatomy?

• Coarctation is, strictly speaking, a congenital narrowing of the aorta.
• Most commonly, this occurs at the insertion of the ductus arteriosus just distal to the left subclavian artery and is more of an isolated ridge or shelf that causes obstruction and narrowing.
• There are several other variants, however and include discrete thoracic lesions, long-segmental defects, tubular hypoplasia, and more rarely, coarctation located in the abdominal aorta.
• Other common congenital anomalies found in patients with aortic coarctation include supra- or subaortic stenosis, Shone complex (a series of left-sided obstructive lesions), and hypoplastic left heart syndrome. Enjoy Episode #121 for more on Shone Complex.
• Patients with Turner Syndrome and Williams Syndrome have high rates of aortic coarctation.

3. What are the hemodynamic consequences of aortic coarctation?

• While coarctation can be severe, it is often well tolerated in utero given ~2/3 of cardiac output flows through the ductus arteriosus into the descending thoracic aorta, bypassing the area of narrowing.
• After birth, however, increasing amounts of cardiac output must bypass the area of constriction. The severity of narrowing correlates with the amount of added afterload beyond what we would expect to see in normal hemodynamics. The consequences thus vary depending on this severity, from asymptomatic to mild hypertension to heart failure and overt cardiogenic shock.
• Compensatory mechanisms the heart can use to counteract this extra afterload are 1) hypertrophy and 2) increasing LV dimensions – neither of which are ideal for a young patient!
• The systemic circulation may compensate for the coarctation by developing collateral blood flow involving the intercostal, internal mammary, and scapular vessels.
• Upper extremity systemic arterial hypertension with a 20-mmHg gradient between the upper and lower extremities
• Delayed and diminished femoral arterial pulses, rarely with symptoms of claudication
• Systolic murmur at left sternal border with radiation to the back and possible thrill in suprasternal notch reflective of high flows through the coarct
• Possible continuous murmur from development of arterial collaterals

4. How might an adult with a history of repaired coarctation of the aorta present?

• 10-20% of those with repaired coarctation go on to develop hypertension in adulthood. This is an important risk factor in these patients for premature coronary artery disease, stroke, cardiomyopathy, and aneurysm (aortic and cerebral) rupture.
• Even in those with normal blood pressure at rest, many have hypertension that can be unmasked with exercise.
• ~11% of patients develop re-coarctation.
• Patients are at risk of developing aneurysm, aortic dissection, or pseudoaneurysms at the site of prior repair (risk is higher in those with concurrent bicuspid aortic valve).
• ~10% of patients with coarctation of the aorta develop cerebral aneurysms and in 5% of coarctation patients, the cause of death is ruptured cerebral aneurysm.

5. What are long-term considerations for coarctation of the aorta?

• In patients with unrepaired coarctation or re-coarctation with a BP gradient ≥ 20 mmHg (Class I recommendation) and hypertension, repair is indicated with transcatheter stenting preferred when technically feasible.
• In patients with repaired coarctation and normal BP at rest, guidelines recommend consideration of ambulatory blood pressure monitoring or exercise stress test to diagnose hypertension with exertion. If this is found, treatment with low dose anti-hypertensive is indicated.
• Routine chest and brain imaging via CT or MRI every 5-10 years is indicated to screen for long-term complications at the site of prior repair and for cerebral aneurysm development.
• Surgical management is indicated for ascending aorta aneurysm >50mm in diameter or those with rapid progression due to high risk of rupture or dissection.

1. Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2019 Apr 2;73(12):e81-e192. doi: 10.1016/j.jacc.2018.08.1029. Epub 2018 Aug 16. Erratum in: J Am Coll Cardiol. 2019 May 14;73(18):2361-2362. PMID: 30121239.
2. Baumgartner H, De Backer J, Babu-Narayan SV, Budts W, Chessa M, Diller GP, Lung B, Kluin J, Lang IM, Meijboom F, Moons P, Mulder BJM, Oechslin E, Roos-Hesselink JW, Schwerzmann M, Sondergaard L, Zeppenfeld K; ESC Scientific Document Group. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. 2021 Feb 11;42(6):563-645. doi: 10.1093/eurheartj/ehaa554. PMID: 32860028.
3. Kenny D, Hijazi ZM. Coarctation of the aorta: from fetal life to adulthood. Cardiol J. 2011;18(5):487-95. doi: 10.5603/cj.2011.0003. PMID: 21947983.
4. Nguyen L, Cook SC. Coarctation of the Aorta: Strategies for Improving Outcomes. Cardiol Clin. 2015 Nov;33(4):521-30, vii. doi: 10.1016/j.ccl.2015.07.011. Epub 2015 Aug 29. PMID: 26471817.
5. Horlick EM, McLaughlin PR, Benson LN. The adult with repaired coarctation of the aorta. Curr Cardiol Rep. 2007 Jul;9(4):323-30. doi: 10.1007/BF02938381. PMID: 17601399.

Adult Congenital Heart Association
Founded in 1998, the Adult Congenital Heart Association is an organization begun by and dedicated to supporting individuals and families living with congenital heart disease and advancing the care and treatment available to our community. Our mission is to empower the congenital heart disease community by advancing access to resources and specialized care that improve patient-centered outcomes. Visit their website (https://www.achaheart.org/) for information on their patient advocacy efforts, educational material, and membership for patients and providers

CHiP Network

The CHiP network is a non-profit organization aiming to connect congenital heart professionals around the world. Visit their website (thechipnetwork.org) and become a member to access free high-quality educational material, upcoming news and events, and the fantastic monthly Journal Watch, keeping you up to date with congenital scientific releases. Visit their website (https://thechipnetwork.org/) for more information.

Heart University
Heart University aims to be “the go-to online resource” for e-learning in CHD and paediatric-acquired heart disease. It is a carefully curated open access library of educational material for all providers of care to children and adults with CHD or children with acquired heart disease, whether a trainee or a practicing provider. The site provides free content to a global audience in two broad domains: 1. A comprehensive curriculum of training modules and associated testing for trainees. 2. A curated library of conference and grand rounds recordings for continuing medical education. Learn more at www.heartuniversity.org/

Dr. Ari Cedars is a cardiologist in Baltimore, Maryland. He also serves as director of the Adult Congenital Heart Disease Center at both the Johns Hopkins Heart and Vascular Institute and the Blalock-Taussig-Thomas Pediatric and Congenital Heart Center. Dr. Cedars is an associate professor of medicine and pediatrics at the Johns Hopkins University School of Medicine.

Dr. Natasha K. Wolfe is the adult congenital heart disease fellow at Washington University in St. Louis. She completed medical school at Vanderbilt University School of Medicine in Nashville. She went on to complete internal medicine residency training in their women’s health track program at the University of Pittsburgh Medical Center. She then moved to St. Louis where she completed her general cardiology fellowship at Washington University in St. Louis/Barnes-Jewish Hospital. She has academic interests in cardio-obstetrics, single ventricle physiology and clinical outcomes, and improving advanced therapy options for the ACHD population. When she is not in the hospital, you can find her outside playing cars and blocks with her adorable one-year old son.

• Amit Goyal, MD
• Daniel Ambinder, MD

CardioNerds (Amit Goyal and Daniel Ambinder), Cardio-OB series co-chair and University of Texas Southwestern Cardiology Fellow, Dr. Sonia Shah, and episode FIT lead and UT Southwestern Cardiology Fellow Dr. Laurie Femnou discuss valvular heart disease in pregnancy with cardio-obstetrics expert Dr. Uri Elkayam, Professor of Medicine and OB Gyn at the University of Southern California.

In this pearl-packed episode, we discuss the diagnosis, acute management, and long-term considerations of valvular heart disease in pregnancy. Through a series of cases, we review the physiologic changes in pregnancy that make certain valvular lesions well-tolerated, while others are associated with a much higher risk of peripartum complications. We also discuss which patients to consider referring for valvular intervention, the ideal timing, and which valvular interventions are safest in the peripartum period. We promise, you won’t want to miss this clinically high-yield episode with Dr. Elkayam, the father of cardio-obstetrics and an absolute legend in the field!

Audio editing by CardioNerds Academy Intern, Adriana Mares.

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1. Most women with severe valvular heart disease can be managed medically throughout pregnancy.
2. Right sided valvular lesions are generally better tolerated than left-sides lesions, and regurgitant lesions are generally better tolerated than stenotic lesions. However, the context and etiology of the valve dysfunction must be taken into consideration. Severe tricuspid valve regurgitation, for example, can be associated with a failing right ventricle and undiagnosed pulmonary hypertension.
3.  Changes in BNP, severity of symptoms, and right ventricular systolic pressure (RVSP) assessed by echocardiography can be helpful in differentiating normal pregnancy-related symptoms from symptoms due to hemodynamically significant valvular lesions.
4. Valvular interventions during pregnancy are safe when well-planned and performed by experienced operators, and they can significantly improve morbidity and mortality in women who remain symptomatic despite medical management.
5. A multidisciplinary team-based approach is important when managing patients with valvular heart disease during pregnancy.

• “We do not need to perform prophylactic valvular intervention in women prior to pregnancy if they do not meet criteria for intervention otherwise. A patient with regurgitant lesion will tolerate pregnancy well, provided that they are not                candidates for surgery already.”

• “Valvuloplasty during pregnancy is a great and effective procedure, but restenosis occurs. For women who desire future pregnancies, preconception evaluation is important to determine if valve intervention is indicated prior to conceiving.”

• What is the epidemiology of valvular heart disease in pregnancy?
  • Cardiovascular conditions affect up to 4% of pregnancies, with valvular heart disease being the most common cardiac pathology encountered during pregnancy worldwide.
  • In the developing world, rheumatic valve disease is still the most common etiology, with mitral valve most commonly affected, followed by the aortic valve.
  • In the developed world, congenital aortic valve pathology is most common.

• What are the hemodynamic effects of stenotic vs. regurgitant lesions during pregnancy?
  • In normal pregnancy, there is a significant drop in systemic vascular resistance as early as 5 weeks gestational age. This drop leads to a transient decrease in perfusion to the kidneys, causing an increase in fluid retention and expansion of plasma volume. At the same time, there is an increase in heart rate which becomes more pronounced later in the second trimester.
  • These changes ultimately lead to an increase in cardiac output. Patients with stenotic lesions have a fixed obstruction, and therefore can have a difficult time adjusting to these physiologic changes of pregnancy. In mitral stenosis for example, the increase in heart rate leads to less atrial emptying time in diastole, which leads to an underfilled left ventricle and increase in left atrial preload.
  • In contrast, regurgitation lesions are often better tolerated than stenotic lesions during pregnancy because of the ability of the cardiac chambers to dilate and accommodate the increase in plasma volume.
  • These rules are generally true, provided that the ventricular systolic function is preserved. A patient with functional mitral regurgitation secondary to a failing left ventricle may not tolerate the hemodynamic changes of pregnancy well.

• What is involved with preconception evaluation and valvular heart disease?
  • Preconception evaluation and counseling is recommended for all women with a history of heart disease to assess risk and modify them if indicated. If already pregnant, a complete risk assessment should be performed as soon as possible by the cardio-obstetrics team.
  • Women with a known or suspected valvular lesion should have a complete echocardiogram performed as part of their assessment. It is important to also assess functional status, as poor baseline functional status is associated with worse outcome during pregnancy. For women with severe valvular lesions and no symptoms at baseline, stress testing can be helpful in assessing functional capacity.
  • Modified WHO, CARPREG and ZAHARA are risk assessment tools that can be used during pregnancy to counsel patients. (See below)
  • Preconception evaluation is a good opportunity to review medications and stop potential teratogenic medications depending on the risks and benefits.

• What are the guidelines for the management of valve disease during pregnancy: medical vs invasive management?
  • Women who otherwise qualify for valve repair or replacement should be strongly considered for valve intervention prior to pregnancy.

Shared decision-making is important when it comes to evaluating therapeutic and interventional options for women who want to become pregnant with valve disease. Mechanical valve replacement, for example, is typically the most durable option for a young patient, but the need for systemic anticoagulation during pregnancy often makes it an unattractive option for some patients. Bioprosthetic valve is an alternative if trying to avoid systemic anticoagulation during pregnancy, with the understanding that patient will likely need reoperation in the future. For women with aortic valve dysfunction, a Ross procedure is another option. In this procedure, a patient’s own pulmonic valve is placed in the aortic position, and the pulmonic valve is replaced using a donor valve. The advantage of this is that no anticoagulation is necessary. It is a more complex surgery, but outcomes are good when performed by experienced operators. Balloon valvuloplasty can also be performed with good result, although restenosis can occur within months of initial procedure; women who undergo valvuloplasty should have repeat assessment immediately prior to conception to ensure that the valve is not re-stenosed.

For women who are already pregnant with persistent symptoms despite medical therapy, balloon valvuloplasty should be considered.

•  How should be monitor women with valvular heart disease during pregnancy?
  • The frequency of monitoring during pregnancy depends on the severity of disease and symptoms. Serial echocardiograms in the absence of symptoms are usually not required.
  • Echocardiograms can be helpful in estimating pulmonary pressures and left atrial pressures. Gradients across the valves in stenotic lesions are expected to increase due to the increase flow and management should not be guided based on this alone.
  • Obtaining cardiac biomarkers such as BNP early in pregnancy can help differentiate between normal symptoms of pregnancy versus hemodynamic compromise from valve disease.
  • Right heart catheterization can be helpful in cases where the symptoms are discordant with the objective data (echo and BNP) especially when result can affect major decision like cesarian section versus vaginal delivery. However, this should be done in expert hands given the increased risk of vascular complications during pregnancy.

• What is the morbidity and mortality associated with severe left sided valvular obstruction during pregnancy?
  • Women with severe left-sided valvular obstruction have the highest risk of morbidity and mortality during pregnancy. A recent meta-analysis of studies published between 1985 and 2019 of women treated in specialized centers in developed countries showed a mortality rate of 3% and 2% in severe mitral and aortic stenosis respectively. Heart failure was more common in mitral stenosis 37% as compared to 9% in AS. New or recurrent arrhythmia were reported in 16% of women with severe MS and 4% in women with severe AS.
  • Fetal outcomes were similarly worse than in the general population.
  • Women who remain symptomatic after initiation of adequate medical therapy should be considered for intervention, as they are in the highest risk group.
  • Valvuloplasty, if valve anatomy allows, to relieve the obstruction is preferred over surgery given high risk of morbidity and fetal loss with cardiac surgery. There are multiple tools used in the cardiac catheterization laboratory to reduce the risk and radiation exposure both to the mother and fetus.

2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease (Journal of the American College of Cardiology). March 3, 2014

Hameed, Afshan B. MD; Mehra, Anilkumar MD; Rahimtoola, Shahbudin H. MB, FRCP The Role of Catheter Balloon Commissurotomy for Severe Mitral Stenosis in Pregnancy. Obstetrics & Gynecology. 2009 – Volume 114 – Issue 6 – p 1336-1340 doi: 10.1097/AOG.0b013e3181bea92d

Ducas RA, Javier DA, D’Souza R, Silversides CK, Tsang W. Pregnancy outcomes in women with significant valve disease: a systematic review and meta-analysis. Heart. 2020;106(7):512-519. doi:10.1136/heartjnl-2019-315859

Elkayam U, Goland S, Pieper PG, Silversides CK. High-Risk Cardiac Disease in Pregnancy: Part II. J Am Coll Cardiol. 2016;68(5):502-516. doi:10.1016/j.jacc.2016.05.050

Dr. Elkayam is an internationally known expert in heart failure and heart disease in pregnancy. Dr. Elkayam did his medical training in Austria and Israel before completing his cardiology fellowships at Albert Einstein College of Medicine and  Cedars Sinai Medical Center. He is currently a dual professor of Medicine and OB Gyn at the University of Southern California, Former Chief of Cardiology at USC University Hospital, and Director of the USC Heart Failure Program. He has been involved in more than 100 self-initiated NIH and industry funded research projects, and has authored over 200 peer review articles and 80 book chapters.

Dr. Laurie Femnou Mbuntum is currently a general cardiology fellow at The University of Texas Southwestern. She completed her undergraduate degree at The University of Maryland Baltimore County. She then moved down South to complete residency at The University of Texas Southwestern where she stayed for cardiology fellowship where she is planning to stay for advanced training in interventional cardiology. She has a special interest in cardio obstetrics and figuring out ways to reduce cardiovascular maternal death. When not in the hospital, she loves spending time with her two boys and learning more about makeup artistry.

• Natalie Stokes, MD
• Sonia Shah, MD
• Amit Goyal, MD
• Daniel Ambinder, MD