Episodes Archives - Cardionerds

CardioNerds (Amit Goyal & Daniel Ambinder) join Penn cardiology fellows (Brian McCauley, Norrisa Haynes, and Mahesh Vidula) for a rooftop picnic in sunny Philadelphia! They discuss an informative case of peripartum cardiomyopathy with cardiogenic shock. Program director Dr. Frank Silvestry provides the E-CPR segment and a message to applicants. Johns Hopkins internal medicine resident Colin Blumenthal with mentorship from University of Maryland cardiology fellow Karan Desai. 

Jump to: Patient summary – Case figures & media – Case teaching – References – Production team

The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.

We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director.

CardioNerds Case Reports Page
CardioNerds Episode Page
CardioNerds Academy
Subscribe to our newsletter- The Heartbeat
Support our educational mission by becoming a Patron!
Cardiology Programs Twitter Group created by Dr. Nosheen Reza

Two weeks postpartum, a woman in her mid 20s, G1P1, with no past medical history presented following a tonic-clonic seizure. Prior to this, she had been experiencing 1 week of worsening dyspnea and lower extremity edema. Initial work-up revealed a left MCA stroke and she underwent thrombectomy. Limited TTE found LVEF <20% and a LV apical thrombus; she was started on milrinone due to concern for cardiogenic shock and transferred to the University of Pennsylvania. 

Upon arrival, she was found to be hypotensive and tachycardic. Exam was notable for elevated JVP, +S3, LE edema and R sided hemiparesis. Labs showed multiorgan injury, elevated NT-proBNP and elevated lactate. EKG demonstrated sinus tachycardia with no ST-T changes. Formal TTE showed severely dilated LV with EF 10%, diffuse LV hypokinesis, and confirmed a large LV apical thrombus. A pulmonary artery catheter was placed for tailored therapy and found elevated L-sided > R-side filling pressures with low cardiac index despite inotropes. Cardiac power output (CPO) was severely decreased with borderline pulmonary artery pulsatility index (PAPI), corroborating left > right heart failure. Patient ultimately required a durable left ventricular assist device (LVAD). Over the course of 9 months her guideline directed medical therapy (GDMT) was titrated and her intrinsic cardiac function and symptoms improved. Her EF improved to 35-40% and she tolerated an LVAD weaning protocol, so her LVAD was ultimately explanted! She is currently doing well on GDMT alone!  

• CXR: Mild interstitial edema, +ET tube
• ST (131), LAD, nonspec T wave flattening, nl intervals

1. How do we define Peripartum Cardiomyopathy?  

• Diagnosis is made by the development of heart failure towards the end of pregnancy or in the months following delivery (~5 months postpartum), no other identifiable cause of HF, and demonstration of LV systolic dysfunction with LVEF typically less than 45% with or without dilation. 
• Risk factors include history of pre-eclampsia, hypertension, cocaine use, multifetal pregnancies, older maternal age, and African descent.    
• Keep a broad differential diagnosis for new onset heart failure in the peripartum period. The differential includes pre-existing cardiomyopathy, valvular disease or congenital cardiomyopathy unmasked by the hemodynamic changes of pregnancy (see CNCR episode 48 for more on the hemodynamic changes of pregnancy!). Other differentials should include ischemia/spontaneous coronary artery dissection (SCAD), stress-induced cardiomyopathy, CM due to systemic disease (e.g. sepsis, rheumatologic disease), myocarditis and tachycardia-induced CM. 

2. The team used invasive hemodynamics to guide shock management. Why is a PAC helpful?  

• Despite data from the ESCAPE trial, a pulmonary artery catheter (PAC) can be very useful in the diagnosis and management of cardiogenic shock. Remember, the ESCAPE trial included chronic HF patients in whom there was clinical uncertainty on whether a PAC may be useful. Further, any patient on milrinone was excluded. Cardiac power output (CPO) and pulmonary artery pulsatility index (PAPi) are some of the parameters obtained from a PAC that can guide cardiogenic shock therapy and need for mechanical circulatory support (MCS). 
• Resting CPO = CO x MAP / 451. CPO measures the “pumping power” of the LV and correlates with end-organ perfusion. In the SHOCK registry, CPO <0.53 watts highly correlated with ↑ in-hospital mortality. CPO can also help identify patients ready for LVAD weaning trials and/or explantation. As with most cardiac parameters, the trends matter as much as the absolute values!    
• PAPi: PA(Systolic) – PA(diastolic) / RA(mean). PAPi was initially developed as a marker severe RV dysfunction in acute inferior wall myocardial infarction and those undergoing LVAD placement. It measures the ratio of the PA pulse pressure to a given preload (RA pressure). It is hard to apply PAPi thresholds across different clinical situations, but we can generally use less than 1-1.75 as a predictor of worsening right-sided function.   

3. How should we approach “typical HF” management in peripartum cardiomyopathy?  

• Though many aspects of the acute management of peripartum cardiomyopathy are similar to typical heart failure and/or cardiogenic shock management, there are a few key differences.  
• Remember, ACEI/ARBs/ARNI and spironolactone are contraindicated during pregnancy. ACE-I have demonstrated safety data while breastfeeding, but ARNIs and ARBs have not been studied robustly.   
• The decision to pursue an ICD in patients with PPCM needs special attention to the natural history of PPCM. Many of these patients will have recovery of LV function in 3-12 months, thus deferring primary prevention ICD while titrating GDMT for ~6 months may be appropriate. There may also be a role for a wearable cardioverter/defibrillators in those with severe LV dysfunction as a bridge to recovery or until an implantable ICD is indicated.  
• Consider early mechanical support in patients deteriorating on medical therapy (including inotropes) alone.  

4. What are some considerations unique to peripartum cardiomyopathy management?  

• The prothrombotic state of pregnancy and stasis within a weak ventricle both increase the risk of LV thrombus and a subsequent cardio-embolic event. Therefore, anticoagulation should be considered in PPCM patients with a severely reduced LVEF (EF < 30% by AHA vs ≤35% by ESC) during late pregnancy and 6 to 8 weeks postpartum. LMWH does not cross the placenta and is preferred during pregnancy. Both warfarin and LMWH may be used during lactation, while the direct oral anticoagulants have not been studied either during pregnancy or lactation and should be avoided.  
• Bromocriptine, a dopamine agonist, reduces prolactin levels (a possible culprit in the development of PPCM). However, data is conflicting on benefit, and thus this therapy should be considered investigative. It is pro-thrombotic, and so it is recommended for patients to be on anticoagulation if bromocriptine is used.  
• Prior to delivery, multidisciplinary discussions should occur around timing and method of delivery for patients diagnosed with PPCM during pregnancy. During delivery, remember that there are several hemodynamic changes (See Ep #48 – AS Complicating Pregnancy!). These include increased preload from placental auto-transfusion and relief of IVC compression.   
• The 2018 ESC guidelines suggest avoiding breastfeeding in patients with severe HF because of the high metabolic demands of lactation and the induced prolactin may contribute to worsening HF. However, some small studies suggest in clinically stable patients, breastfeeding does not worsen HF symptoms or LVEF. Considerations regarding possible risk must be counterbalanced with the known benefits of breastfeeding for infants and mothers.   

5. How do we approach long-term management in patients with peripartum cardiomyopathy?  

• Providers should engage in shared decision-making about future pregnancies in patients with recovered EF (>50%) as they are at higher risk of future recurrence. The 2018 ESC guidelines advise against pregnancy if EF has not recovered to >50-55%.   
• Contraception counseling should be done early: before discharge or at the time of diagnosis. Patients should avoid estrogen-containing methods especially in the early postpartum period as thromboembolism risk is high.  
• If a patient plans on another pregnancy, teratogenic HF medications, like ACE/ARB/ARNI and spironolactone, must be stopped prior to stopping contraception. Following cessation of these GDMT medication, follow-up echocardiography to ensure LV functional stability should be performed after at least 3 months off therapy. The patient should be monitored very closely during a subsequent pregnancy for changes in symptoms and followed with serial echocardiograms and NT-proBNP levels during pregnancy and thereafter.   

References

• Davis MB, Arany Z, McNamara DM et al. Peripartum Cardiomyopathy: JACC State-of-the-Art Review. J Am Coll Cardiol. 2020;75(2):207-221. doi:10.1016/j.jacc.2019.11.014 
• Korabathina R, Heffernan KS, Paruchuri V, et al. The pulmonary artery pulsatility index identifies severe right ventricular dysfunction in acute inferior myocardial infarction. Catheter Cardiovasc Interv. 2012;80(4):593-600. doi:10.1002/ccd.23309 
• Kang G, Ha R, Banerjee D. Pulmonary artery pulsatility index predicts right ventricular failure after left ventricular assist device implantation [published correction appears in J Heart Lung Transplant. 2017 Nov;36(11):1272]. J Heart Lung Transplant. 2016;35(1):67-73. doi:10.1016/j.healun.2015.06.009 
• Kochav SM, Flores RJ, Truby LK, Topkara VK. Prognostic Impact of Pulmonary Artery Pulsatility Index (PAPi) in Patients With Advanced Heart Failure: Insights From the ESCAPE Trial. J Card Fail. 2018;24(7):453-459. doi:10.1016/j.cardfail.2018.03.008 
• Lim HS, Gustafsson F. Pulmonary artery pulsatility index: physiological basis and clinical application. Eur J Heart Fail. 2020;22(1):32-38. doi:10.1002/ejhf.1679 
• Fincke R, Hochman JS, Lowe AM, et al. Cardiac power is the strongest hemodynamic correlate of mortality in cardiogenic shock: a report from the SHOCK trial registry. J Am Coll Cardiol. 2004;44(2):340-348. doi:10.1016/j.jacc.2004.03.060 
• Mendoza DD, Cooper HA, Panza JA. Cardiac power output predicts mortality across a broad spectrum of patients with acute cardiac disease. Am Heart J. 2007;153(3):366-370. doi:10.1016/j.ahj.2006.11.014 

• Bibin Varghese, MD
• Rick Ferraro, MD
• Tommy Das, MD
• Eunice Dugan, MD
• Evelyn Song, MD
• Colin Blumenthal, MD
• Karan Desai, MD
• Amit Goyal, MD
• Daniel Ambinder, MD

CardioNerds (Amit Goyal & Daniel Ambinder) join Northwestern University cardiology fellows (Sarah Hale, Sarah Chuzi, and Graham Lohrmann) for burgers and a great case by the Chicago River! They discuss a fascinating case of arrhythmogenic desmoplakin cardiomyopathy. Dr. Lisa Wilsbacher provides the E-CPR and program director Dr. Benjamin Freed provides a message for applicants.  Episode notes were developed by Johns Hopkins internal medicine resident Richard Ferraro with mentorship from University of Maryland cardiology fellow Karan Desai. 

Jump to: Patient summary – Case figures & media – Case teaching – References – Production team

• Episode graphic by Dr. Carine Hamo

The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.

We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director.

CardioNerds Case Reports Page
CardioNerds Episode Page
CardioNerds Academy
Subscribe to our newsletter- The Heartbeat
Support our educational mission by becoming a Patron!
Cardiology Programs Twitter Group created by Dr. Nosheen Reza

A male in his early 40s presented for second opinion regarding multiple ICD shocks. 10 years prior he was diagnosed with a “weak heart,” thought secondary to a viral illness and a dual-chamber ICD was placed at that time. He noted shocks occurring for the first time 5 years prior, at which time amiodarone was started. They recurred two years prior, when he was diagnosed with paroxysmal atrial fibrillation. Finally, he was hospitalized one month before presentation with multiple ICD shocks and was found to have high defibrillation thresholds (DFTs) and amiodarone was stopped. He  then presented for a second opinion from the Northwestern CardioNerds! 

The patient had been doing well on GDMT and had NYHA Class I symptoms (Enjoy Ep #13 – Approach to GDMT). He did note a family history of a cousin with “cardiac issues” and did not know his father’s family history. Physical exam demonstrated bradycardia and ECG demonstrated an a-paced, v-sensed rhythm at 50 bpm. TTE demonstrated a moderately dilated LV with LVEF 30%, globally reduced LV function and multiple wall motion abnormalities without a vascular distribution.  PET-CT was performed which showed diffuse uptake and high-intensity signal at the inferolateral and basal anterior walls. Cardiac MRI showed diffuse circumferential epicardial delayed enhancement with associated diffuse, enhancing thickening of the pericardium favoring inflammatory versus fibrotic process. Patient was initially diagnosed with cardiac sarcoid and started on prednisone and weekly methotrexate.  

On return of genetic testing, patient found to have a pathogenic variant for desmoplakin gene, and it was felt his cardiomyopathy was secondary to desmoplakin Left Dominant Arrhythmogenic Cardiomyopathy (LDAC, or left-dominant ARVC) presenting with inflammatory myocardial injury. On follow up the patient remained listed for transplant, and DFTs improved off amiodarone.  

• CXR
• ECG
• Cardiac MRI
• PET CT (Cardiac Sarcoid Protocol)

• Created by Dr. Karan Desai

1. We started the case talking about DFTs. What are DFTs?! 
   • Defibrillation Thresholds (DFTs) are the minimal amount of energy required to return a patient to sinus rhythm that is in a cardiac dysrhythmia.  
   • Most modern ICD leads have thresholds less than 15 joules and typically less than 10 joules with biphasic shocks. High DFT thresholds tend to be defined as >25J or a safety margin of <10J. 
   • DFT testing is not routinely recommended during implantation of left-sided transvenous devices; however, in patients undergoing right-sided transvenous ICD or ICD pulse generator changes, DFT testing is a reasonable approach. Patients undergoing subcutaneous ICD placement should generally have DFT testing. 
   • Contraindications to DFT testing include acute LV thrombus, atrial fibrillation/flutter without adequate anticoagulation, severe aortic stenosis, recent stroke or TIA, or hemodynamic compromise as DFT itself can cause hypotension and/or CVA.   
     
2. What are the major causes of high DFTs? 
   • First there are myocardial factors. This includes pathology that affects the current density through the myocardium. Conditions like hypertrophic cardiomyopathy, inflammatory cardiomyopathy, or significant LV dilation can lead to high DFTs.    
   • The second set of factors are extra-cardiac causes that lead to increased impedance or resistance in the counter coil, such as high BMI or medications that lead to electrical imbalances such as amiodarone, which is a common cause of increased DFTs. However, the increase in DFT caused by amiodarone can be small and routine DFT testing in patients is not recommended.   
   • The last cause is a device factor, such as a lead fracture or a mal-positioned lead.  
     
3. In the case, we used MRI and PET. What are their role in Cardiomyopathy? 
   • Cardiac MRI (CMR) has transformed our ability to assess cardiomyopathies, specifically by accurately defining chamber size & function, characterizing myocardial tissue, and determining ischemia & viability. The specific pattern of late gadolinium enhancement (LGE) can help us differentiate between ischemic and non-ischemic etiologies and specific cardiomyopathies have characteristic patterns on MRI. Further, MRI can reliably identify edema, inflammation, and fatty replacement. CMR can provide a wealth of information in a variety of disease processes. Enjoy Ep #33 – CMR!
   • When evaluating an unexplained cardiomyopathy, FDG-PET can be useful in identifying active myocardial inflammation. 18F-FDG is a glucose analogue that can differentiate activated macrophages in areas of inflammation from normal myocytes if there is appropriate suppression of normal physiologic myocardial glucose uptake (I.e., Ketogenic Diet). This can be especially useful in Cardiac Sarcoidosis. Note, if there is global myocardial uptake, without diffuse perfusion defects, it may be a false positive scan in the setting of inadequate prep!  
   • For more on evaluation of heart failure, enjoy Ep #12 – Eval of New Onset HF & CPS Ep #48 – HFrEF. 
     
4. When should we consider a genetic cause to cardiomyopathy? 
   • If a family history suggests a genetic predisposition to cardiomyopathy, a cardiomyopathy seems out of proportion to an identified ischemic or non-ischemic cause, a patients presents with a cardiomyopathy at a young age, or if multi-modal imaging has not revealed a clear cause of a cardiomyopathy, genetic testing would be appropriate. 
   • Various studies have indicated that 30 to 50% of unexplained cases of (DCM) may have a genetic component. A detailed, at least three-generation family history should be obtained when initially evaluating a dilated CM, as most genetic cardiomyopathies are autosomal dominant with variable penetrance.  
   • Genetic counseling is key prior to genetic testing given complexities including interpretation of potentially confounding results and contextualizing results for relatives. 
     
5. Our patient’s final diagnosis was “Arrhythmogenic Desmoplakin Cardiomyopathy”…what’s that?! 
   • Arrhythmogenic RV Cardiomyopathy is a familial cardiomyopathy which usually affects the RV via fibrous or fibro-fatty replacement of normal myocardium. This predisposes patients to sudden cardiac death (SCD), ventricular arrhythmias, and heart failure.  
   • ARVC classically displays autosomal dominant inheritance from mutations in genes encoding desmosomal proteins affecting the cell-to-cell junction: desmoplakin (DSP), plakophilin 2 (PKP2), desmoglein 2 (DSG2), and desmocollin 2 (DSC2). Rarely, genes unrelated to cell-to-cell junction may be involved.  
   • Clinically we have noted a “Left-Dominant Arrhythmogenic Cardiomyopathy” (LDAC), with similarities to classic ARVC, but affecting predominantly the LV.  
   • Genotype-phenotype studies are shedding light on these “Arrhythmogenic Cardiomyopathies”. DSP mutations affect predominantly the LV (causing LDAC) whereas PKP2 mutations affect predominantly the RV (causing ARVC); these are distinct entities with key differences in presentation, progression, and markers of SCD risk (see Smith et al., Circulation 2020 reference for more!). Pertinent to our case, a subset of patients with DSP cardiomyopathy will have evidence of myocardial inflammation on FDG-PET and will are initially misdiagnosed as a myocarditis or sarcoidosis. 

References

• Smith, E. D., Lakdawala, N. K., Papoutsidakis, N. et. al. (2020). Desmoplakin Cardiomyopathy, a Fibrotic and Inflammatory Form of Cardiomyopathy Distinct from Typical Dilated or Arrhythmogenic Right Ventricular Cardiomyopathy. Circulation. 141(23), 1872-1884 
• Crawford, T.C (2020). Desmoplakin Cardiomyopathy. https://www.acc.org/latest-in-cardiology/journal-scans/2020/06/11/15/43/desmoplakin-cardiomyopathy 
• Shulka, H. H., Flaker, G. C., Jayam, V. et. al. (2003). High defibrillation thresholds in transvenous biphasic implantable defibrillators: clinical predictors and prognostic implications. Pacing and clinical electrophysiology, 26(1p1), 44-48. 
• Russo, A. M., Sauer, W., Gerstenfeld, E. P., Hsia, H. H., Lin, D., Cooper, J. M., … & Patel, V. (2005). Defibrillation threshold testing: is it really necessary at the time of implantable cardioverter-defibrillator insertion?. Heart Rhythm, 2(5), 456-461. 
• Wilkoff, B. L., Fauchier, L., Stiles, M. K. et. al.(2016). 2015 HRS/EHRA/APHRS/SOLAECE expert consensus statement on optimal implantable cardioverter-defibrillator programming and testing. Ep Europace, 18(2), 159-183. 

• Bibin Varghese, MD
• Rick Ferraro, MD
• Tommy Das, MD
• Eunice Dugan, MD
• Evelyn Song, MD
• Colin Blumenthal, MD
• Karan Desai, MD
• Amit Goyal, MD
• Daniel Ambinder, MD

CardioNerds (Amit Goyal & Daniel Ambinder) join UT-Austin cardiology fellows (Priya Kothapali, Sergio Montano, Travis Benzing, and Michael Grzeskowiak) for a speedboat adventure on Lake Travis! They discuss a fascinating case of Suicide LV post-TAVR. Dr. Mark Pirwitz provides the E-CPR and program director Dr. Clay Cauthen provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Evelyn Song with mentorship from University of Maryland cardiology fellow Karan Desai. 

Jump to: Patient summary – Case figures & media – Case teaching – References – Production team

The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.

We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director.

CardioNerds Case Reports Page
CardioNerds Episode Page
CardioNerds Academy
Subscribe to our newsletter- The Heartbeat
Support our educational mission by becoming a Patron!
Cardiology Programs Twitter Group created by Dr. Nosheen Reza

A woman in her early 70s, with a history of CAD s/p PCI to LAD & RCA with DES six months prior, to presentation, paroxysmal atrial fibrillation s/p ablation, type 2 diabetes mellitus, hypertension, prior TIA, and severe symptomatic AS was admitted for elective TAVR. She underwent successful implantation of a 29mm Medtronic Evolut Pro valve via left common femoral artery access. Post-valve deployment and following protamine administration for heparin reversal, course was complicated by hypotension with PEA arrest requiring CPR for 4 minutes. Intra-op TEE and angiogram showed a well-seated prosthetic valve with trace paravalvular leak and no evidence of acute aortic regurgitation, significant paravalvular leak, pericardial effusion, coronary obstruction, aortic dissection, or access site complications. She was treated for suspected Protamine reaction with high dose steroids & epinephrine. However, she remained hypotensive with MAP in the 50s on high dose Epinephrine, Norepinephrine, and Vasopressin. Hemodynamics by pulmonary artery catheter demonstrated CVP 7, mPA 26, PCWP 18 mmHg and CO/CI 2.8 L/min and 1.3 L/min/m2. Her lactate was elevated at 5.92 mmol/L and EKG demonstrated normal sinus rhythm. Bedside TTE in the ICU showed hyperdynamic LV function with LVEF 70% and near-complete mid to distal cavity obliteration with significant intracavitary gradient. She was diagnosed with post-TAVR suicide LV and managed with aggressive volume resuscitation and rapid wean of Epinephrine/Norepinephrine with improvement in her hemodynamics. She was eventually extubated and discharged on beta-blocker therapy.

• A. ECG
• B. CXR

A. ECG: Normal sinus rhythm, no evidence of AV block, no ST segment elevation or depression.
B. CXR: Pulmonary vascular congestion, no pneumothorax, ETT at level of carina, PAC in appropriate position

• Created by Dr. Karan Desai
• Graphic by Dr. Carine Hamo

1. Remind all CardioNerds – how do we define Severe Aortic Stenosis? 

• By TTE, severe AS is defined as a mean gradient >40 mmHg, a peak velocity >4 m/s, an aortic valve area <1 cm2 and dimensionless index < 0.25. But remember, in HF patients decreased trans-aortic valve flow secondary to LV disease can make the diagnosis challenging! 
• In HFrEF, patients with severe AS by aortic valve area (AVA) may not meet velocity or gradient criteria in one of four contexts: AVA measurement error, pseudo-severe AS, poor contractile reserve, or true severe Low Flow, Low Gradient AS (LFLG AS). 
• Similarly, in HFpEF patients, disproportionately low flow or gradient AS may occur due to AVA measurement error or from true severe “paradoxical” LFLG due to decrease stroke volume (stroke volume index <35 mL/m2). 
• Low gradient AS is a critical subset of patients with generally with a comparatively worse clinical trajectory marked by increased heart failure hospitalizations and mortality compared with high gradient AS. 
• Enjoy the aortic stenosis series (Ep #1-2) and related figure for a deeper dive! 

2.     This patient received a TAVR for her severe AS. What are the major complications? 

• Generally there are five relatively important post-TAVR complications – termed the “Big 5” – that contribute to long-term morbidity. These include disabling stroke, acute kidney injury, moderate/severe paravalvular leak, vascular and bleeding complications, and conduction abnormalities with possible need for PPM. 
• Modern TAVR valves include mechanisms to reduce paravalvular leak (i.e., an outer skirt or a porcine pericardial tissue wrap or cuff), but may lead to more conduction abnormalities 

3.     This case covered Suicide LV. What is the pathophysiology behind this? 

• Severe AS leads to LV hypertrophy and chronic pressure overload. When the fixed obstruction is relieved, the increased contractility of the LV is “unmasked” and can lead to dynamic LV cavity obstruction or obliteration.  
• When this compromises cardiac output, these labile hemodynamics are termed “suicide LV”.   

4.     What are risk factors for developing Suicide LV post-AVR? 

• The major risk factors for Suicide LV with dynamic intracavitary gradient include small LVOT and LV size, hyperdynamic and elevated LVEF, asymmetric septal hypertrophy with an elevated interventricular septal to posterior wall thickness ratio, high valve gradients prior to AVR and small LV wall mass. 

5.     What should be my basic approach to Suicide LV management? 

• The management of suicide LV is similar to that of Hypertrophic Obstructive Cardiomyopathy (see Ep#3) and related figure for a refresher! 
• The basics include increasing preload with intravenous fluids, increasing afterload to reduce intra-cavitary gradients (phenylephrine tends to be the vasoactive of choice), avoiding inotropes (e.g., digoxin or beta-agonist infusions) while considering negative inotropes (e.g., beta blockers), and maintaining AV synchrony to ensure adequate LV preload 

References

• Evaluation of Shock Following TAVR – ACC  
• Big 5 Complications post-TAVR – JACC IC 2019 
• Suh 2010 – Suicide LV following TAVI 

• Bibin Varghese, MD
• Rick Ferraro, MD
• Tommy Das, MD
• Eunice Dugan, MD
• Evelyn Song, MD
• Colin Blumenthal, MD
• Karan Desai, MD
• Amit Goyal, MD
• Daniel Ambinder, MD

CardioNerd (Amit Goyal) join Washington University in St. Louis cardiology fellows (Adam Lick, Manny Rivera Maza, and Sam Lindner) for some amazing local St. Louis craft brews! They discuss a fascinating case of  wild-type aTTR cardiac amyloid. Prior to meeting up with the group, Amit bumps into Rachita Navara: a Wash U #FIT, aspiring electrophysiologist, & a rock star of the band “The Pacemakers” (be sure to check out their performance at the end of the episode!) who shares thoughts about the program and her cutting edge contributions to the field of EP. Dr. Katie Zhang provides the E-CPR and program director Dr. Andy Kates provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Colin Blumenthal with mentorship from University of Maryland cardiology fellow Karan Desai.  

Jump to: Patient summary – Case figures & media – Case teaching – References – Production team

The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.

We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director.

CardioNerds Case Reports Page
CardioNerds Episode Page
CardioNerds Academy
Subscribe to our newsletter- The Heartbeat
Support our educational mission by becoming a Patron!
Cardiology Programs Twitter Group created by Dr. Nosheen Reza

A man in his early 70s, with a history of hypertension, a bicuspid aortic valve, chronic kidney disease and carpal tunnel syndrome presents with two weeks of worsening dyspnea on exertion. At baseline, he is an avid cyclist and noticed he can now only bike ½ mile when before he could bike extended distances. In addition, he noted abdominal swelling and palpitations. Vitals signs showed mild tachycardia, irregularly irregular rhythm, and no clear evidence of volume overload. Labs demonstrated acute on chronic kidney disease, an elevated NT-proBNP and elevated troponin. ECG demonstrated atrial flutter with variable conduction block. TTE demonstrated marked concentric left ventricular hypertrophy with preserved ejection fraction, biatrial enlargement, reduced global longitudinal strain with apical sparing, and bicuspid aortic valve with moderate aortic stenosis. Further diagnostics revealed normal serum kappa/lambda light chains and PYP scan was positive. Patient underwent EMB which demonstrated ATTR amyloid deposition and genetic screening did not show mutations commonly associated with hereditary ATTR. 

• A
• B
• C
• D

A. CXR: Stable mild enlargement of the cardiac silhouette; mildly tortuous aorta. Lung fields are clear, no pneumonia, pleural effusions, or pneumothorax.
B. AV continuous wave Doppler
C. Strain Image
D. Tc-99 PYP Scan

• Created by Dr. Karan Desai
• Created by Dr. Carine Hamo (updated 9.2020)

1. Cardiac amyloidosis can have a range of cardiac and extra-cardiac findings as amyloid fibrils can deposit in many different tissues, depending on the amyloid protein involved. The first step in diagnosis is maintaining a high index of suspicion. 
   1. The presence of prominent right-sided HF symptoms, low voltage on ECG, biatrial enlargement, ventricular hypertrophy, conduction disease, and pericardial effusion should prompt a high suspicion for cardiac amyloid. These are typical features of infiltrative or storage restrictive cardiomyopathies. PEARL: ATTR CM can cause asymmetric LVH and is an important HCM phenocopy! 
   2. Remember, amyloidosis is a systemic disease and extra-cardiac findings are common. ATTR amyloid has a predilection for the musculoskeletal system (including bilateral carpal tunnel syndrome, lumbar spinal stenosis, and biceps tendon rupture) and peripheral nerves. Conversely, AL amyloid is widely deposited outside the CNS an can result in protean manifestations, including periorbital ecchymoses from vascular fragility, macroglossia, and visceral organ involvement (including nephrotic syndrome, hepatic infiltration, and gut amyloid). 
      
2. When considering cardiac amyloid, there are four primary etiologies: 
   1. Wild-Type (non-hereditary) ATTR amyloidosis is caused by deposition of misfolded transthyretin proteins. It is an under-recognized cause of HFpEF, with incidence increasing with age. 
   2. Hereditary ATTR is caused by a genetic mutation that leads to instability of the transthyretin tetramer. The most common mutation is the Val30Met variant. Specific mutations tend to have templated organ manifestations, natural history, and prognosis.  
   3. AL amyloidosis is caused by deposition of light-chains from a clonal plasma cell dyscrasia. 
   4. AA amyloidosis is a rare form of cardiac amyloid caused by deposition of the acute phase reactant serum amyloid A protein due to a chronic inflammatory process. 
      
3. Evaluating for AL amyloid with appropriate lab workup is crucial. “Missing AL amyloid is like missing a STEMI” – Dr. Paul Cremer (Episode #8)! Workup should include SPEP, UPEP, serum/urine immunofixation, and serum kappa/lambda free light chains to maximize sensitivity. Note: SPEP and UPEP alone are insensitive and inadequate! 
   
4. Like many cardiac diseases, multimodal diagnostics are key in the diagnosis of cardiac amyloid 
   1. ECG: Low voltage, pseudoinfarct pattern, variable conduction disease, atrial arrhythmias. PEARL: 10% of patients with cardiac amyloid may have high voltages on ECG. LVH on imaging out of proportion to EKG voltages is a red flag! 
   2. TTE: We have discussed typical features of an infiltrative or storage restrictive cardiomyopathy previously , including marked LVH with normal LV volumes and bi-atrial enlargement. Other features include: RV hypertrophy, thickened valve leaflets, thick interatrial septum, speckled appearance of the myocardium, and small pericardial effusion. Bi-atrial enlargement may lead to functional MR and TR as well as atrial arrhythmias. Mitral inflow pattern and tissue doppler will show varying degrees of diastolic dysfunction depending on stage. Systolic function may be borderline and progressively decline in “burned-out” disease. Reduced global longitudinal strain with apical sparing may help differentiate cardiac amyloid from hypertensive heart disease. There is an overlap with aortic stenosis which will frequently manifest as a low flow and/or low gradient phenotype due to reduce stroke volumes. 
   3. Cardiac MRI: There are characteristic findings of early subendocardial late gadolinium enhancement (LGE) and later transmural LGE with abnormal blood-pool-to-myocardial nulling (an MRI technique to accentuate pathology).  
   4. PYP scan: >99% sensitivity for cardiac ATTR amyloid. If monoclonal gammopathy is excluded, the positive predictive value is 100%. 
   5. RHC +/- EMBx: the hemodynamic profile is that of restrictive cardiomyopathy with elevated filling pressures, blunted x descent, and steep y descent. There may be diastolic pressure equalization. If EMBx is pursued, pathology will reveal amyloid protein as a salmon-pink color when congo red stain is applied and when placed under polarized light the amyloid proteins have an apple-green birefringence. Amyloid fibrils are seen with electron microscopic study.  Mass spec for protein identification. 
      
5. Cardiac amyloid can be very difficult to treat with typical HF regimens, as beta blockers and ACE-I/ARB can lead to excessive hypotension in the setting of autonomic neuropathy and restrictive hemodynamics.  For TTR amyloid, there are treatment options like tafamidis (a transthyretin protein stabilizer) which can reduce mortality considerably in selected patients.  Investigational RNA-targeted therapies (i.e., patisiran) are increasing the options for treating TTR, but cost remains a major barrier to care. For AL amyloid, a multidisciplinary cardio-oncology team is vital to coordinate chemotherapy and cardiovascular care. Heart transplant +/- bone marrow transplant may be options in advanced stages of disease. The challenges of managing advanced disease highlight the importance of early recognition.  

References

• TTR Ca Review – JACC 2019 
• Amyloid CM Case – JACC Case Reports 2019 
• CardioNerds Amyloid Page 
• Strain Imaging Echocardiography: What Imaging Cardiologists Should Know 
• Tafamidis Treatment for Patients with Transthyretin Amyloid Cardiomyopathy 

• Bibin Varghese, MD
• Rick Ferraro, MD
• Tommy Das, MD
• Eunice Dugan, MD
• Evelyn Song, MD
• Colin Blumenthal, MD
• Karan Desai, MD
• Amit Goyal, MD
• Daniel Ambinder, MD

CardioNerds (Amit Goyal & Daniel Ambinder) join Temple Cardiology Fellows (Anika Vaidy and Anne- Sophie LaCharite-Roberge) in Philadelphia, PA! They discuss a fascinating case of pulmonary hypertension secondary to Chronic Thromboembolic Pulmonary Hypertension (CTEPH) associated with May Thurner syndrome and large uterine fibroids. Dr. Vaidya provides the E-CPR and message to applicants. Episode notes were developed by Johns Hopkins internal medicine resident, Eunice Dugan, with mentorship from University of Maryland cardiology fellow Karan Desai. 

“To study the phenomena of disease without books is to sail an uncharted sea, while to study books without patients is not to go to sea at all.” – Sir William Osler. CardioNerds thank the patients and their loved ones whose stories teach us the Art of Medicine and support our Mission to Democratize Cardiovascular Medicine.

Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values.

Jump to: Patient summary – Case figures & media – Case teaching – Educational video – References – Production team

The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.

We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director.

CardioNerds Case Reports Page
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Cardiology Programs Twitter Group created by Dr. Nosheen Reza

A woman in her late 40s with history of iron deficiency anemia, uterine fibroids, and recurrent DVTs/PEs for the past 6 years despite different anticoagulation regimens, presented with syncope and progressive dyspnea on exertion. Family history was negative for DVT/PE or hypercoagulable states. On exam, she was euvolemic. Labs were significant for anemia, a normal pro-BNP, and a negative extensive hypercoagulable workup. TTE showed interventricular systolic septal flattening, right ventricular outflow tract pulse wave doppler with mid-systolic notch, and shortened acceleration time consistent with elevated pulmonary artery pressure. A VQ scan showed bilateral right greater than left mis-matched perfusion defects. CT angiogram showed right greater than left chronic mural thrombus, correlating with pulmonary angiogram which showed severe proximal and mid-vessel disease on the right and distal disease on the left. RHC corroborated the diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) with mean pulmonary artery pressure of 41 and PVR of 5.2 Woods Units (WU).  

To determine the etiology of her recurrent clots, a lower extremity venogram was performed and showed 80% stenosis of her left common iliac vein by the overlying right common iliac artery confirming May-Thurner syndrome. Lower extremity venogram also showed severe proximal stenosis of right iliac vein thought to be due to large uterine fibroids. Given her severe proximal and mid-vessel clot burden, she underwent pulmonary thromboendarterectomy with a subsequent drop in pulmonary vascular resistance to 1 WU.  The etiology of DVTs and CTEPH was determined to be external compression related to both May-Thurner syndrome and uterine fibroids. To prevent future thromboembolic events, she underwent stenting of her left common iliac vein and hysterectomy. With these interventions, RV function returned to normal, and her symptoms completely resolved! 

Figue Legend:
A. CXR
B. ECG
C. TTE: Interventricular systolic septal flattening, RVOT pulse wave Doppler with mid-systolic notch and shortened acceleration time, consistent with elevated PVR, RV:LV ratio 1.3, consistent with moderate RV enlargement, The RV is apex-sharing with an open apical angle
D. VQ Scan: Multiple b/l perfusion defects, R > L, V scan normal
E. CT Angiogram: 1) Enlarged R main PA2) large proximal chronic mural thrombus with minimal vessel count throughout R side, 3) Segmental LLL lining thrombus
F. Pulmonary angiogram significant for severe proximal and mid-vessel disease in the right segmental arteries. Patient also had severe distal disease in the left sub segmental disease (not shown).
G. Pulmonary Thromboendarterectomy (PTE)
H. LE Venogram: LEFT CIV  > 80% stenosis as a result of compression from an overlying right common iliac artery. This is consistent with May-Thurner syndrome. RIGHT EIV (not shown)– Significant proximal stenosis
I. Status Post left iliac vein stent
J. ECG: New typical atrial flutter

References

CardioNerds (Amit Goyal & Carine Hamo) discuss the past, present, and future of Women’s Heart Health & Women in Cardiology with Dr. Nanette Wenger, Professor of Medicine in the Division of Cardiology at the Emory University School of Medicine. Dr. Wenger is a true leader in the field of women’s heart health and a strong proponent for women in cardiology and medicine. Her passion, dedication, and advocacy have inspired countless trainees to carry this torch and continue to build on her truly impactful work. Special introduction by Dr. Martha Gulati and birthday wishes to Dr. Wenger by the entire CardioNerds Team! Special thanks to Dr. Kimberly Manning for her invaluable mentorship.

The Cardionerds CV prevention series  includes in-depth deep dives on so many prevention topics including the ABCs of prevention, approach to obesity, hypertension, diabetes mellitus and anti-diabetes agents, personalized risk and genetic risk assessments, hyperlipidemia, women’s cardiovascular prevention, coronary calcium scoring and so much more!

CardioNerds Episode Page
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CardioNerds Women’s Cardiovascular Health Page
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We are truly honored to be producing the Cardionerds CVD Prevention Series in collaboration with the American Society for Preventive Cardiology! The ASPC is an incredible resource for learning, networking, and promoting the ideals of cardiovascular prevention! This series is kicked off by a message from Dr. Amit Khera, President of the American Society for Preventive Cardiology and President of the SouthWest Affiliate of the American Heart Association.

1. Wenger NK (2005) Women in cardiology: The US experience. Heart.

2. Douglas PS, Rzeszut AK, Noel Bairey Merz C, Duvernoy CS, Lewis SJ, Walsh MN, Gillam L (2018) Career preferences and perceptions of cardiology among us internal medicine trainees factors influencing cardiology career choice. JAMA Cardiol.

3. Wenger NK, Speroff L, Packard B (1993) Cardiovascular Health and Disease in Women. N Engl J Med.

4. Burgess S, Shaw E, Zaman S (2019) Women in Cardiology. Circulation.

Dr. Nanette Wenger is Professor of Medicine in the Division of Cardiology at the Emory University School of Medicine. Dr. Wenger received her medical degree from Harvard Medical School in 1954 as one of their first female graduates followed by training at Mount Sinai Hospital where she was the first female to be chief resident in the cardiology department. She is among the first physicians to focus on heart disease in women with an expertise in cardiac rehabilitation and geriatric medicine.

Dr. Wenger has received numerous awards including the Distinguished Achievement Award from the Scientific Councils of the American Heart Association and its Women in Cardiology Mentoring Award, the James D. Bruce Memorial Award of the American College of Physicians for distinguished contributions in preventive medicine, the Gold Heart Award, the highest award of the American Heart Association, a Lifetime Achievement Award in 2009 and the Inaugural Bernadine Healy Leadership in Women’s CV Disease Distinguished Award, American College of Cardiology. She chaired the U.S. National Heart, Lung, and Blood Institute Conference on Cardiovascular Health and Disease in Women, is a Past President of the Society of Geriatric Cardiology and is past Chair, Board of Directors of the Society for Women’s Health Research. Dr. Wenger serves on the editorial boards of numerous professional journals and is a sought-after lecturer for issues related to heart disease in women, heart disease in the elderly, cardiac rehabilitation, coronary prevention, and contemporary cardiac care. She is listed in Best Doctors in America.

• Carine Hamo, MD
• Amit Goyal, MD

CardioNerds (Amit Goyal & Dan Ambinder) join Lankenau Medical Center cardiology fellows (Gwen McNeill and Shaung Ooi) for some Philly cheesesteaks! They discuss a fascinating case of Embolic Acute Coronary Syndrome from PFO and Pulmonary Hypertension. Dr. John Clark provides the E-CPR and program director Dr. Jeanine Romanelli provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Tommy Das with mentorship from University of Maryland cardiology fellow Karan Desai. 

Jump to: Patient summary – Case figures & media – Case teaching – Educational video – References – Production team

The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.

We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director.

CardioNerds Case Reports Page
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Support our educational mission by becoming a Patron!
Cardiology Programs Twitter Group created by Dr. Nosheen Reza

A woman in her early 40s with history of tobacco and prior methamphetamine use presented with acute onset chest pain. She was found to have an elevated troponin, anterior T wave inversions, and apical akinesis on TTE. Coronary angiography showed a coronary embolism in the mid-distal LAD. Attempts to wire the lesion led to distal embolization of the clot, and IVUS showed no underlying arteriosclerosis.  

To investigate the etiology of the coronary embolus, a repeat TTE with agitated saline was done that suggested a PFO with right to left flow, as well as decreased RV function with an estimated RVSP of 70 mmHg. The clinical picture was that of a paradoxical coronary embolus, arising from the right (venous) side traveling to the left (arterial) side via a PFO. PFO closure was discussed but not performed given severe pulmonary hypertension with Right to Left shunt. In this circumstance, the PFO functions as a “pop-off valve” for the overloaded RV; closing it risks precipitating acute RV overload and failure. A RHC showed a PA pressure of 70/24 mmHg with mPAP of 40 mmHg, PCWP 5 mmHg, and PVR of 11 woods units. Given concern for idiopathic PH, a vasodilator challenge was done which did not show reactivity, and she was started on ambrisentan and sildenafil. Ultimately, the etiology of her pulmonary hypertension was felt to be due to PAH from prior methamphetamine use vs. idiopathic PAH. On follow-up, her PA pressures and RV function had greatly approved, allowing for safe and successful PFO closure in an attempt to prevent future emboli.  Final diagnosis: Embolic Acute Coronary Syndrome from PFO & Pulmonary Hypertension.

• Before treatment: short axis
• Before treatment: apical 4
• Before treatment: RVSP
• After treatment: Short axis
• After treatment: Apical 4
• After treatment: RVSP

1. Coronary emboli are an uncommon cause of myocardial infarction. We can think of the etiology of coronary emboli in three major categories: Direct, Paradoxical, or Iatrogenic. 
   1. Direct emboli originate from the left side of the heart: sources include clot (from atrial appendage, apical thrombus), valvular lesion (vegetation, thrombus, fibroelastoma), or left sided cardiac mass (atrial myxoma, rhabdomyosarcoma). 
   2. Paradoxical emboli originate from the right side or systemic venous circulation, and pass from right to left through an atrial septal defect, patent foramen ovale, or pulmonary arteriovenous malformation. 
   3. Iatrogenic emboli occur following procedures such as valve replacement or PCI. Note that iatrogenic is the most common etiology! 
      
2. PFOs are present in up to 25% of adults, but are usually clinically insignificant. However, there is increasing evidence that PFO closure is moderately beneficial compared to antiplatelet therapy alone in patients less than 60 years old with cryptogenic, non-lacunar ischemic stroke. PFO closure may particularly benefit those with a large right-to-left shunt or an associated atrial septal aneurysm.
   
3. Remember closing an ASD in the setting of significant pulmonary hypertension can lead to decompensation, as the interatrial connection may be serving as a “pop-off” valve to decompress the RV and maintain cardiac output in the setting of high PA pressures! Specifically, ASD closure is generally contraindicated if the PA systolic pressure is > 2/3 systolic blood pressure, pulmonary vascular resistance > 2/3 systemic vascular resistance, or if  a net right-to-left shunt is present. 
   
4. Recall that the hemodynamic definitions of pulmonary hypertension changed in 2019. A mean pulmonary artery pressure of 20 or greater confirms a diagnosis of pulmonary hypertension. A PCWP ≤15 mmHg with pulmonary vascular resistance ≥3 woods units suggests pre-capillary pulmonary hypertension, while a PCWP >15 with pulmonary vascular resistance <3 woods units suggests isolated post-capillary PH.  
   
5. During a right heart catheterization, a pulmonary vasodilator challenge can be done to identify vasoreactive patients who may respond to calcium channel blockers. This is typically recommended for idiopathic and hereditary PAH. A positive response is defined as a drop in mean PAP to <40 mmHg, with a decrease of at least > 10 mmHg, with unchanged or increased cardiac output. These patients can be started on amlodipine or nifedipine, but should be followed closely as they may not always remain vasoreactive and clinical deterioration is possible! 

References

• P

• Raphael, C. E., Heit, J. A., Reeder, G. S. et al. (2018). Coronary Embolus: An Underappreciated Cause of Acute Coronary Syndromes. JACC. Cardiovascular interventions, 11(2), 172–180.  
• Mojadidi, M. K., Zaman, M. O., Elgendy, I. Y. et al. (2018). Cryptogenic Stroke and Patent Foramen Ovale. Journal of the American College of Cardiology, 71(9), 1035–1043.  
• Silvestry, F. E., Cohen, M. S., Armsby, L. B. et al. (2015). Guidelines for the Echocardiographic Assessment of Atrial Septal Defect and Patent Foramen Ovale: From the American Society of Echocardiography and Society for Cardiac Angiography and Interventions. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography, 28(8), 910–958. 
• Simonneau, G., Montani, D., Celermajer, D. S. et al. (2019). Haemodynamic definitions and updated clinical classification of pulmonary hypertension. The European respiratory journal, 53(1), 1801913. 
• Stout, K. K., Daniels, C. J., Aboulhosn, J. A. et al. (2019). 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation, 139(14), e637–e697.  

• Bibin Varghese, MD
• Rick Ferraro, MD
• Tommy Das, MD
• Eunice Dugan, MD
• Evelyn Song, MD
• Colin Blumenthal, MD
• Karan Desai, MD
• Amit Goyal, MD
• Daniel Ambinder, MD

CardioNerds (Amit Goyal & Dan Ambinder) discuss a case of hereditary restrictive cardiomyopathy with Duke University cardiology fellows Navid Nafissi and Sipa Yankey, and Marat Fudim, an advanced heart failure attending. E-CPR is provided by Dr. Richa Agarwal, fellowship program director of advanced heart failure and cardiac transplantation at Duke University with a final message by fellowship director Dr. Anna Lisa Crowley. Episode notes were developed by Johns Hopkins internal medicine resident Colin Blumenthal with mentorship from University of Maryland cardiology fellow Karan Desai.  

Jump to: Patient summary – Case figures & media – Case teaching – Educational video – References – Production team

The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.

We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director.

CardioNerds Case Reports Page
CardioNerds Episode Page
CardioNerds Academy
Subscribe to our newsletter- The Heartbeat
Support our educational mission by becoming a Patron!
Cardiology Programs Twitter Group created by Dr. Nosheen Reza

A 69 yo M with history of atrial fibrillation presents with 5 months of progressive HF symptoms, now NYHA class IV. He was found to be grossly volume overloaded, tachycardic in atrial fibrillation, and hypoxic. CXR demonstrated significant cardiomegaly, and labs indicated new normocytic anemia with evidence of hepatic dysfunction and an elevated NT proBNP. TTE demonstrated massive bi-atrial enlargement, preserved ejection fraction, filling pattern consistent with grade III diastolic dysfunction, and torrential TR. The echocardiogram did not have evidence of constrictive pericarditis and agitated saline study showed Right to Left shunt through a likely PFO. MRI to evaluate for infiltrative cardiomyopathy did not show late gadolinium enhancement (LGE). RHC demonstrated findings consistent with restrictive cardiomyopathy including equalization of diastolic pressures, square root sign, and concordance of RV and LV pressures. PYP scan evaluating for TTR amyloid was negative and laboratory workup did not suggest AL amyloid, Fabry’s, Hemochromatosis, or storage disease. Patient’s symptoms remained refractory and thus eventually underwent successful OHT. Genetic testing eventually revealed missense mutation in MYBPC3 – revealing an inherited cause of restrictive CM for the patient! 

• Chest X-ray
• -Rate-controlled A fib with frequent PVCs, LPFB, non-specific ST changes

1. When thinking about the etiology of a restrictive cardiomyopathy, we can organize the causes into four major categories: 
   1. Infiltrative (e.g., amyloidosis, sarcoidosis) 
   2. Storage diseases (e.g., hemochromatosis, Fabry’s) 
   3. Non-infiltrative (e.g., idiopathic, diabetic cardiomyopathy, genetic causes) 
   4. Endomyocardial (e.g., endomyocardial fibrosis, hypereosinophilic syndrome) 
      
2. On examination, patients with restrictive cardiomyopathy may have prominent right-sided symptoms, including hepatomegaly, ascites, and significant peripheral edema. On exam, neck veins may demonstrate a steep y descent, and cardiac auscultation may have a +S4, and murmurs of tricuspid and mitral regurgitation.
   
3. A multimodal imaging workup is recommended for evaluating RCM.  
   1. TTE: Typically demonstrates normal LVEF, normal chamber volumes, biatrial enlargement, and restrictive diastolic filling patterns (.e.g, E/A > 2, E/e’ > 14, decreased mitral deceleration time < 150 ms). We can see increased wall thickness with infiltrative processes and storage diseases.
   2. cMRI: specific patterns of Late Gadolinium Enhancement may indicate certain pathology (e.g., amyloid may demonstrate diffuse subendocardial, heterogeneous, or transmural signal). T2 signal can identify inflammation and quantify iron. 
   3. PYP scan: It has a >99% sensitivity for cardiac ATTR amyloid. If monoclonal gammopathy excluded, PPV 100%. 
      
4. Invasive hemodynamics can show physiology consistent with RCM. This includes square root sign, equalization of diastolic pressures, and concordance during respiration of LV/RV pressure changes. In contrast, constrictive physiology will yield discordant respirophasic LV/RV pressure changes. Endomyocardial biopsy may be necessary to identify etiology but the yield for patchy diseases, like sarcoid, can be low. 
   
5. Patients with end-stage RCM can be difficult to manage medically, especially as they become refractory to diuretics. They poorly tolerate antihypertensive agents (due to inability to augment limited stroke volume), heart rate lowering medication (due to low SV, they are dependent on HR to maintain CO which is HR x SV), and have a very limited optimal preload window (need high filling pressures to fill the stiff ventricles but also have symptoms of fluid overload). Due to anatomic considerations, patients are rarely candidates for durable left ventricular assist devices and often require orthotopic heart transplant. However, patients with RCM have higher waitlist mortality and longer wait times, in part due to lower utilization of MCS.   

1/17#CNCR and food trucks; what could be better!

Another amazing @Cardionerds case w/ @NavidNafissi, @FudimMarat and @sipa_yankey from @DukeCardFellows

Catch up on the episode here: https://t.co/wA70i64jEb

Here’s a brief re-cap with some of the?learning

First a synopsis… pic.twitter.com/jS264g9mTh

References

• Spectrum of Restrictive & Infiltrative CM – Part 1 article 
• Spectrum of Restrictive & Infiltrative CM – Part 2 article 
• Spectrum of Restrictive & Infiltrative CM – Part 1 notes 
• Spectrum of Restrictive & Infiltrative CM – Part 2 notes 
• Restrictive CM – Circ Res 2017 
• Echocardiography Diagnostic Criteria for Constriction 
• Differentiation of Constriction and Restriction: Complex Cardiovascular Hemodynamics 
• Constrictive Pericarditis Versus Restrictive Cardiomyopathy? 
• Does Survival on the Heart Transplant Waiting List Depend on the Underlying Heart Disease? 
• Genetic Counseling in Inherited Cardiomyopathies 
• 2016 ASE Echo Guidelines 

• Bibin Varghese, MD
• Rick Ferraro, MD
• Tommy Das, MD
• Eunice Dugan, MD
• Evelyn Song, MD
• Colin Blumenthal, MD
• Karan Desai, MD
• Amit Goyal, MD
• Daniel Ambinder, MD

CardioNerds (Amit Goyal & Daniel Ambinder)  join Allegheny Health Network cardiology fellows (Adnan Khalif, Mahathi Indaram, Kushani Gajjar, and Michael Nestasie) for a lovely Pittsburgh hike and discuss a fascinating case of platypnea-orthodeoxia secondary to a PFO. Dr. Farhan Katchi provides the E-CPR and Program director Dr. Craig Alpert provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Richard Ferraro with mentorship from University of Maryland cardiology fellow Karan Desai. 

Jump to: Patient summary – Case figures & media – Case teaching – Educational video – References – Production team

The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.

We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director.

CardioNerds Case Reports Page
CardioNerds Episode Page
CardioNerds Academy
Subscribe to our newsletter- The Heartbeat
Support our educational mission by becoming a Patron!
Cardiology Programs Twitter Group created by Dr. Nosheen Reza

A 64 y/o female with a history of venous and arterial embolism on anticoagulation, known PFO, and obesity presented after a fall. There was no loss consciousness, pre-syncopal symptoms, chest pain, aura, weakness, or palpitations. She had no recent preceding illness. When she arrived in the ED she was hypoxic to 87% on ambulation on room air and required 4L of nasal cannula O2 supplementation. The AGH CardioNerds were consulted!  

On examination, the team noted that upon sitting up the patient would desaturate to the mid 80% but when lying down oxygen saturation would improve to 95%! Her ECG demonstrated a RBBB and no acute ST-T changes. TTE was obtained and showed normal LV and RV function and size, no valvular disease, and a likely PFO on an agitated saline study. Cardiac MRI revealed mild RV hypertrophy and Qp:Qs of 0.8 (right/pulm cardiac output < left/systemic cardiac output, indicating right to left shunting).  RHC showed normal right-sided pressures. A right femoral vein bubble study  was done showing torrential right to left shunting! She underwent PFO closure and her platypnea-orthodexia resolved; she was discharged on room air. 

1. Platypnea-orthodeoxia syndrome (POS) is when dyspnea (Platypnea) and hypoxia (Orthodeoxia) are present in the upright position, but improve upon lying flat. One cause is ARLIAS, or Acute Right-to-Left Inter-Atrial Shunting, which requires an anatomic component (I.e., ASD or PFO) and a functional component (I.e., PH, PE, RV failure) for Right-to-Left shunting to occur 
   
2. A PFO, or patent foramen ovale is a common congenital defect and typically will not lead to hypoxia unless there is right to left shunting. Typically flow is left to right due to an LA to RA pressure gradient and lower compliance of the RV.  
   
3. Remember when evaluating for a PFO with agitated saline, the timing of the appearance of bubbles is important!  Early appearance of bubbles (seen in less than 5 beats of the cardiac cycle, think about intra-cardiac shunt. Delayed opacification (> 5 to 6 beats) occurs when the bubbles slowly build in the LV with each successive beat as they circulate to the LV and suggests extra-cardiac shunt.  
   
4. Cardiac MRI is helpful in quantifying the shunt fraction (Qp:Qs).  This is the ratio of pulmonary flow (Qp) to systemic flow (Qs), where hemodynamically significant Left-to-Right shunt is > 1.5, especially when we see RA/RV dilation. Qp/Qs < 1.1 indicates net R to L shunt. Other ways to measure a Qp:Qs are echocardiogram (less accurate) and right heart catheterization.  
   
5. If there remains clinical concern for PFO with right to left shunting without evidence of a clear functional cause clinically or by catheterization (I.e., no evidence of high PA pressures, pericardial effusion, constrictive pericarditis), consider a right femoral vein bubble study. Upper extremity agitated saline enters the RA via the SVC; however, patients with prominent eustachian valve at the IVC can have blood flow directed towards the interatrial septum. This will be demonstrated on femoral vein bubble study! 

CASE TWEETORIAL! We're going to talk about PLATYPNEA- ORTHODEOXIA! 

Here's an early preview of what we'll be discussing from the @cardionerds case report with Allegheny General Hospital!https://t.co/goIUEylJYH

References

• Cheng, T. O. (2002). Mechanisms of platypnea-orthodeoxia: what causes water to flow uphill. Circulation, 105(6), e47. 
• Tobis, J. M., & Abudayyeh, I. (2016). Platypnea-orthodeoxia syndrome: an overlooked cause of hypoxemia. 
• Sanikommu, V., Lasorda, D., & Poornima, I. (2009). Anatomical factors triggering platypnea‐orthodeoxia in adults. Clinical Cardiology: An International Indexed and Peer‐Reviewed Journal for Advances in the Treatment of Cardiovascular Disease, 32(11), E55-E57. 

• Bibin Varghese, MD
• Rick Ferraro, MD
• Tommy Das, MD
• Eunice Dugan, MD
• Evelyn Song, MD
• Colin Blumenthal, MD
• Karan Desai, MD
• Amit Goyal, MD
• Daniel Ambinder, MD

CardioNerds (Amit & Dan)  join Vanderbilt University cardiology fellows (Tara Holder, Majd El-Harasis, and Amar Parikh) for a Sunday morning brunch, Nashville style! They discuss an enthralling case of bicuspid aortic valve with critical aortic stenosis complicating pregnancy. Program director Dr. Julie Damp provides the E-CPR and a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Tommy Das with mentorship from University of Maryland cardiology fellow Karan Desai. 

Jump to: Patient summary – Case figures & media – Case teaching – Educational video – References – Production team

The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.

We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director.

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A 22yo transgender F2M man (G1P0000) at 32w5d was found to have a late-peaking systolic ejection murmur on a regularly scheduled OB visit. He reported recent left-sided exertional chest pain and intermittent lightheadedness, as well as a history of a childhood heart murmur. TTE showed a bicuspid aortic valve (LCC-NCC fusion) with severe aortic stenosis (peak velocity 4.83 m/s, mean gradient of 56 mmHg, AVA at 0.5 cm2 and Dimensionless Index at 0.15). Furthermore, there was preserved ejection fraction and no associated aortopathy.   

Following a syncopal episode, the patient was admitted for cardiac optimization prior to delivery. With shared decision making, he ultimately delivered via cesarean section prior to valvular intervention. Post-partum, he underwent balloon aortic valvuloplasty with improvement in mean aortic gradient to 27 mmHg and trace aortic insufficiency. He was asymptomatic at 5 months post-procedure with similar gradients across the aortic valve on TTE.   

• Chest – X ray
• Final gradients: Peak velocity 4.83 m/s Mean >50 (56) mmHg AVA 0.5 (AVAi=0.27) DOI=0.15

1. Hemodynamics change dramatically during pregnancy. Cardiac output increases by 30-50% during pregnancy due to: ↑ SV, ↑HR, ↓SVR.  
   
2. Immediately post-partum, patients with existing valvular lesions are at high risk of heart failure! Hemodynamic changes peripartum are unpredictable.  
   • ↑Preload: relief of IVC compression, auto-transfusion of 300-500mL blood from placenta with each uterine contraction, and intravenous fluids and/or blood products. 
   • ↓Preload: hemorrhage 
   • ↑Afterload: SBP & DBP increase with each uterine contraction 
   • ↓Afterload: systemic vasodilation from epidural and spinal analgesia 
   • ↑CO: by up to 30% in the first stage of labor and up to 80% in the immediate post-partum period. 2/2 ↑SV. 
     
3. Most pregnant patients with symptomatic AS can be managed medically, with balloon aortic valvuloplasty reserved for patients with refractory symptoms. TAVR and SAVR may be considered, ideally reserved for the 2nd trimester. Spontaneous vaginal delivery carries a lower risk than c-section due to smaller shifts in blood volume, decreased bleeding, and avoidance of preload-shifting anesthetic agents.  
   
4. In a woman of child-bearing age with severe symptomatic AS who is planned for AVR, shared decision making is key in choice of valve! A mechanical valve is more durable than a bioprosthetic valve, but requires anticoagulation that could complicate a future pregnancy. Alternatives include a Ross procedure (replacing the aortic valve using the patient’s own pulmonic valve – “pulmonary autograft” – and a cadaveric pulmonic valve is placed in the pulmonic position – “pulmonary allograft”), Ozaki procedure (recreating a new aortic valve from the patient’s pericardium), performing a bioprosthetic AVR with risk of earlier degeneration, and performing a TAVR with plans for a surgical AVR later. Decision making is complex and warrants a multidisciplinary team accounting for patient preferences.  
   
5. The modified World Health Organization (WHO) classification stratifies the risk of pregnancy in women with cardiovascular disease. Pregnancy is contraindicated in women with WHO group IV lesions: 
   • Pulmonary artery hypertension 
   • Severe systemic ventricular dysfunction (EF <30% or NYHA III-IV) 
   • Systemic RV with moderate or severely decreased ventricular function 
   • Previous peripartum cardiomyopathy with residual ventricular impairment 
   • Severe mitral stenosis or severe symptomatic aortic stenosis 
   • Severe aortic dilation (>45mm in Marfan, >50mm a/w bicuspid aortic valve) 
   • Severe (re)coarctation 
   • Vascular Ehlers-Danlos syndrome 
   •  Fontan with any complication  

Did you catch the #CNCR brunch between @cardionerds and @vumccardsfit? What a great discussion of cardio-obstetrics and valvular disease over (virtual) chicken and waffles!

Listen to the episode here: https://t.co/KqpBx6FNDM

Case synopsis and learning points below: pic.twitter.com/a1S3kGK1ON

References

• Nishimura, R. A., Otto, C. M., Bonow, R. O. et al. (2017). 2017 AHA/ACC Focused Update of the 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation, 135(25), e1159–e1195.  
• Sharma, G., Lindley, K., & Grodzinsky, A. (2020). Cardio-Obstetrics: Developing a Niche in Maternal Cardiovascular Health. Journal of the American College of Cardiology, 75(11), 1355–1359.  
• Elkayam, U., Goland, S., Pieper, P. G. et al. (2016). High-Risk Cardiac Disease in Pregnancy: Part I. Journal of the American College of Cardiology, 68(4), 396–410.  
• Orwat, S., Diller, G. P., van Hagen, I. M. et al (2016). Risk of Pregnancy in Moderate and Severe Aortic Stenosis: From the Multinational ROPAC Registry. Journal of the American College of Cardiology, 68(16), 1727–1737. 
• Mazine, A., El-Hamamsy, I., Verma, S. et al. (2018). Ross Procedure in Adults for Cardiologists and Cardiac Surgeons: JACC State-of-the-Art Review. Journal of the American College of Cardiology, 72(22), 2761–2777.  
• European Society of Gynecology (ESG), Association for European Paediatric Cardiology (AEPC), German Society for Gender Medicine (DGesGM), Regitz-Zagrosek, V., Blomstrom Lundqvist, C., Borghi, C. et al. (2011). 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, 32(24), 3147–3197.  

• Bibin Varghese, MD
• Rick Ferraro, MD
• Tommy Das, MD
• Eunice Dugan, MD
• Evelyn Song, MD
• Colin Blumenthal, MD
• Karan Desai, MD
• Amit Goyal, MD
• Daniel Ambinder, MD