Congenital Heart Disease Archives - Cardionerds

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Congenital Heart Disease Archives - Cardionerds episodes

June 10454. ACHD Surgery 101: Thinking Like a Surgeon with Elizabeth Stephens
CardioNerds (Drs. Rawan Amir, Tripti Gupta, and Alysha Joseph) discuss the fundamentals of adult congenital heart disease (ACHD) surgery with Dr. Elizabeth Stephens. Audio editing by CardioNerds academy intern, Grace Qiu.
Using a case of a young adult undergoing a Ross procedure, the episode walks through what happens in the operating room—from induction and intraoperative transesophageal echocardiography (TEE) to cardiopulmonary bypass (CPB), myocardial protection, and surgical repair. The discussion highlights key concepts including cardioplegia, cross-clamp and bypass times, hypothermic circulatory arrest, and the complexity of redo sternotomy. This episode provides learners with a practical framework to interpret operative reports, anticipate postoperative physiology, and better collaborate with surgical teams.
This episode was produced by the CardioNerds ACHD Council and planned by Dr. Rawan Amir.
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Pearls
“LV distension kills patients.”
Preventing left ventricular distension with appropriate venting and awareness of aortic insufficiency is critical to intraoperative safety.
TEE can change the surgical plan in real time.
Findings such as underestimated aortic regurgitation, mitral pathology, or a PFO may directly alter cannulation and cardioplegia strategy.
Cross-clamp time = myocardial ischemic time; bypass time = systemic stress.
Both are key predictors of postoperative complications including renal injury, bleeding, and ventricular dysfunction.
Redo sternotomy risk is driven by anatomy, not just number.
Aorta adherent to the sternum, conduit position, and chamber pressurization define risk more than the number of prior surgeries.
Think longitudinally—ACHD surgery is lifetime planning.
Surgical materials and strategies must account for future interventions, especially in younger patients.
Notes:
Notes drafted by Dr. Alysha Joseph, aided by generative artificial intelligence.
What are the key steps in congenital cardiac surgery from incision to closure?
Preoperative planning is multidisciplinary, involving surgeon, anesthesia, cardiology, and ICU teams; high-risk inductions (e.g., critical AS, Williams syndrome) are identified early
TEE is performed immediately after induction to reassess anatomy and may reveal new findings (e.g., underestimated AI, mitral disease, PFO)
Median sternotomy is performed, followed by creation of a pericardial well to optimize exposure
Heparin is administered prior to cannulation; arterial and venous cannulas are placed for initiation of CPB
Cross-clamp is applied and cardioplegia delivered to arrest the heart, allowing a still and protected operative field
Surgical repair (e.g., Ross procedure) is performed, followed by de-airing, cross-clamp removal, and reperfusion
Patient is weaned from bypass with TEE reassessment, hemostasis achieved, and chest closed
What is cardioplegia and how is it delivered?
Cardioplegia is a potassium-rich solution that arrests myocardial activity and reduces metabolic demand
Most commonly used solution in the U.S. is Del Nido cardioplegia, originally developed for pediatric myocardium
Delivery strategies include:
Antegrade (via aortic root) – standard approach
Ostial (direct coronary delivery) – used when aortic root cannot be relied upon
Retrograde (via coronary sinus) – useful in severe AI or coronary disease
NOTE: Severe aortic regurgitation can impair antegrade delivery and requires alternative strategies and LV venting
What do cross-clamp time and bypass time represent clinically?
Cross-clamp time = duration of myocardial ischemia while the heart is arrested
Bypass time = total duration on CPB, reflecting systemic exposure to non-physiologic circulation
Prolonged cross-clamp time (>2–3 hours) increases risk of myocardial dysfunction, especially with poor baseline function
Longer bypass time is associated with increased risk of renal injury, coagulopathy, and bleeding
These metrics often reflect both case complexity and intraoperative challenges
What is hypothermic circulatory arrest (HCA) and when is it used?
HCA involves complete cessation of blood flow to allow a bloodless surgical field
Typically used in complex aortic arch repairs
Patients are cooled to ~18°C to reduce metabolic demand and protect organs
Duration is ideally limited to <30 minutes to minimize neurologic injury
Adjuncts include:
Antegrade cerebral perfusion (ACP) – provides targeted brain perfusion
Retrograde cerebral perfusion (RCP) – less effective for oxygen delivery
What makes redo congenital cardiac surgery high risk?
Re-entry risk depends on anatomical relationships:
Aorta adherent to sternum (especially midline) poses high risk of catastrophic bleeding
RVOT conduits or pressurized chambers near sternum increase injury risk
Loss of peripheral vascular access from prior procedures limits bailout options
Accumulated comorbidities (renal, hepatic dysfunction) increase perioperative risk
Diastolic dysfunction and ventricular impairment complicate weaning from bypass
Complexity of planned repair and institutional/surgeon experience significantly influence outcomes
What does “venting the ventricle” mean and why is it important?
Venting refers to decompression of the left ventricle using a cannula (often via right superior pulmonary vein)
Prevents LV distension, which can impair myocardial protection and lead to hemodynamic collapse
Particularly important in the presence of aortic insufficiency or inadequate forward flow
Failure to adequately vent can result in arrhythmias, poor recovery, and adverse outcomes
What materials are used in congenital surgery and how do they impact long-term care?
Common patch materials include bovine pericardium (durable, non-stretch), Dacron, Gore-Tex, and autologous pericardium
Conduits (e.g., homografts, Contegra, Hancock) are used to connect cardiac structures and often contain valves
Most materials do not grow with the patient and are prone to calcification over time
Surgical decisions must consider future transcatheter or surgical interventions
Limited availability of certain graft sizes (e.g., pulmonary homografts) impacts real-world decision-making
References:
1. Salis, S. et al. Cardiopulmonary bypass duration is an independent predictor of morbidity and mortality after cardiac surgery. J Cardiothorac Vasc Anesth. 2008;22(6):814-822. doi:10.1053/j.jvca.2008.08.004
2. Al-Sarraf, N. et al. Cross-clamp time is an independent predictor of mortality and morbidity in low- and high-risk cardiac patients. International journal of surgery (London, England). 2011; 9(1):104–109. https://doi.org/10.1016/j.ijsu.2010.10.007
3. Weiland, A. P. et al. Physiologic principles and clinical sequelae of cardiopulmonary bypass. Heart & lung : the journal of critical care. 1986;15(1):34–39.
4. Park, C. B. et al. Identifying patients at particular risk of injury during repeat sternotomy: analysis of 2555 cardiac reoperations. The Journal of thoracic and cardiovascular surgery. 2010;140(5):1028–1035. https://doi.org/10.1016/j.jtcvs.2010.07.086
5. Morales, D. L. et al. Repeat sternotomy in congenital heart surgery: no longer a risk factor. The Annals of thoracic surgery. 2008; 86(3):897–902. https://doi.org/10.1016/j.athoracsur.2008.04.044
6. Francica, A. et al. Cardioplegia between Evolution and Revolution: From Depolarized to Polarized Cardiac Arrest in Adult Cardiac Surgery. Journal of clinical medicine. 2021;10(19):4485. https://doi.org/10.3390/jcm10194485
7. Ghia, S. et al. Hypothermic Circulatory Arrest in Adult Aortic Arch Surgery: A Review of Hypothermic Circulatory Arrest and its Anesthetic Implications. Journal of cardiothoracic and vascular anesthesia. 2023; 37(12): 2634–2645. https://doi.org/10.1053/j.jvca.2023.08.139
8. Peivandi, A. D. et al. Grafts and Patches: Optimized but Not Optimal Materials for Congenital Heart Surgery. Pediatric cardiology. 2023;44(5):996–1002. https://doi.org/10.1007/s00246-023-03153-6
43 min
September 28, 2025 429. Walking Both Paths: A Physician and Patient in Adult Congenital Heart Disease with Dr. Leigh Reardon
CardioNerds (Dr. Abby Frederickson, Dr. Claire Cambron, and Dr. Rawan Amir) are joined by Dr. Leigh Reardon for a powerful conversation on navigating adult congenital heart disease as both a patient and provider. Dr. Reardon shares his personal journey with congenital heart disease and how it shaped his path to becoming an expert in the field himself. The discussion highlights patient-centered perspectives, barriers to care within the healthcare system, and the importance of advocacy and empathy.
This episode was planned by the CardioNerds ACHD Council.
Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values.
US Cardiology Review is now the official journal of CardioNerds! Submit your manuscript here.
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44 min
April 23, 2025 416. Hearts Over Time: Navigating Grief and Growth in Adult Congenital Cardiology
CardioNerds ACHD Council members Dr. Rawan Amir and Dr. Claire Cambron lead a profound conversation with ACHD faculty Dr. Allison Tsao, Dr. Jill Steiner, and Dr. Katherine Salciccioli. Together, they explore the emotional and professional challenges that ACHD providers face across the lifespan of congenital heart disease. Topics discussed include navigating challenging case scenarios, empowering patients through tough decisions, leveraging multi-subspecialty expertise, celebrating the successes, preparing for and grieving loss, and more.
This episode was planned by the CardioNerds ACHD Council.
Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values.
US Cardiology Review is now the official journal of CardioNerds! Submit your manuscript here.
CardioNerds Adult Congenital Heart Disease Page
CardioNerds Episode Page
CardioNerds Academy
Cardionerds Healy Honor Roll
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Subscribe to The Heartbeat Newsletter!
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29 min
May 14, 2024371. Case Report: The Curious Case of Obstructive Cardiogenic Shock – Maine Medical Center
CardioNerds Dr. Josh Saef and Dr. Tommy Das join Dr. Omkar Betageri, Dr. Andrew Geissler, Dr. Philip Lacombe, and Dr. Cashel O’Brien from the Maine Medical Center in Portland, Maine to enjoy an afternoon by the famous Portland headlight. They discuss a case of a patient who presents with obstructive cardiogenic shock. Dr. Bram Geller and Dr. Jon Donnelly provide the Expert CardioNerd Perspectives & Review segment for this episode. Dr. Maxwell Afari, the Maine Medical Center cardiology fellowship program director highlights the fellowship program. Audio editing by CardioNerds Academy Intern, student doctor Tina Reddy.
This is the case of a 42 year-old woman born with complicated Tetralogy of Fallot repair culminating in a 29mm Edwards Sapiens (ES) S3 valve placement within a pulmonary homograft for graft failure who was admitted to the cardiac ICU for progressive cardiogenic shock requiring vasopressors and inotropic support. Initial workup showed lactic acidosis, acute kidney injury, elevated NT-proBNP, and negative blood cultures. TTE showed at least moderate biventricular systolic dysfunction. She was placed on furosemide infusion, blood cultures were drawn and empiric antibiotics initiated. Right heart catheterization demonstrated elevated right sided filling pressures, blunted PA pressures with low PCWP, low cardiac index, and low pulmonary artery pulsatility index. Intracardiac echocardiography (ICE) showed a large mass within the ES valve apparatus causing restrictive valve motion with a low gradient across the pulmonic valve in the setting of poor RV function. Angiography revealed a large filling defect and balloon valvuloplasty was performed with immediate hemodynamic improvement. Blood cultures remained negative, she was gradually weaned off of inotropic and vasopressor support, and discharged. Despite empiric treatment for culture negative endocarditis and ongoing anticoagulation, she was readmitted for recurrent shock one month later at which time the pulmonic mass was revisualized on ICE. A valve-in-valve transcatheter pulmonary valve (29mm ES S3) was placed to compress what was likely pannus, with an excellent hemodynamic result and no visible mass on ICE.
“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.
US Cardiology Review is now the official journal of CardioNerds! Submit your manuscript here.
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Case Media
Pearls – Obstructive Cardiogenic ShocK
Tetralogy of Fallot is the most common cyanotic defect and can lead to long term complications after surgical repair including chronic pulmonary insufficiency, RV dysfunction, residual RVOT obstruction and branch pulmonary artery stenoses.
Chronic RV failure may be more indicative of a structural defect and therefore require interventional or surgical management.
Valve thrombosis, infective endocarditis and obstructive pannus formation should be considered in the differential of a patient with obstructive shock with a prosthetic valve.
Bioprosthetic pulmonic valve obstruction may be effectively managed with balloon valvuloplasty in patients who present in acute extremis but TCPV will likely provide a more lasting result.
While valvular gradients are typically assessed via echocardiography, invasive hemodynamics can serve as a critical adjunctive tool in its characterization.
Show Notes – Obstructive Cardiogenic ShocK
Notes were drafted by Drs. Omkar Betageri, Philip Lacombe, Cashel O’Brien, and Andrew Geissler.
What are the common therapies and management for Tetralogy of Fallot?
Tetralogy of Fallot is the most common cyanotic defect in children beyond the age of one year
Anatomic Abnormalities: Anterior and Superior deviation of the conal septum creating a SubAo VSD and encroachment on the RVOT. The Ao follows the conal septum anteriorly to override the VSD and RVH is a consequence of an RV chamber that is at systemic pressure.
The need for medical intervention is dependent on the degree of RVOT obstruction, pulmonary regurgitation, and/or peripheral pulmonary artery obstruction.
Many patients are minimally cyanotic in newborn period and clinical follow-up with elective surgical correction between 3 months and 6 months of age is a preferred approach.
Total surgical correction typically involves placing patients on cardiopulmonary bypass, relieving right sided obstruction (sometimes utilizing trans-annular patch), and connecting left ventricular blood flow with aortic, with VSD closure.
Hypercyanotic “Tet” spells can occur in the neonatal period when there is an acute muscular spasm in the RVOT, fall in SVR or increase in PVR to facilitate right to left shunting at the VSD. It is managed medically with a stepwise approach placing the patient in knee-chest position, administer oxygen, IV fluids and a dose of narcotics, IV beta blocker, IV phenylephrine, ECMO
Long term complications post-surgical repair include chronic pulmonary insufficiency, RV dysfunction, residual RVOT obstruction, peripheral PA obstruction, aortic root dilation, endocarditis, arrhythmias, and sudden cardiac death.
How should we approach the management of chronic right ventricular failure?
Briefly, RV failure can result in chamber dilatation, bowing of the interventricular septum to the LV, impairing LV stroke volume, and ultimately causing hemodynamic collapse. This is the frequently referenced “RV spiral”
The primary step in management of RV cardiogenic shock should be to identify and reverse the primary etiology
While patients in acute RV failure may respond well to therapies such as fluid resuscitation, inotropes and vasopressors, or inhaled nitric oxide, patients with chronic RV failure (such as in our case) are more likely to have structural defects driving their decompensation
Chronic RV failure can result from etiologies related to preload (ASD, tricuspid and pulmonic insufficiency), afterload (pulmonic or pulmonary artery stenosis, pulmonary vascular disease, and left heart disease) or contractility (RV myopathy or ARVC).

VSD is not volume loading to RV and Eisenmenger’s would be a pressure load b/c of increased PVR and actually volume unload the RV with R to L VSD shunting.
In acute stabilization, it is reasonable to utilize methods used to manage acute RV failure, with the understanding that interventional/surgical management will ultimately be necessary for definitive management
When should bioprosthetic valve obstruction be suspected and what is the differential for this? What is the initial workup?
Obstruction should be suspected in any patient with a history of prosthetic valve placement who presents with a new murmur, new history of exercise intolerance, syncope/presyncope, or evidence of heart failure on exam.
Differential diagnosis includes pannus ingrowth, thrombus, and vegetation. The anterior location of a surgically replaced RVOT/PV also makes it susceptible to compressive forces from the chest wall anteriorly and dilated Ao posteriorly, particularly in growing children. For bioprosthetic valves pannus is more common than acute thrombosis. Age of the valve, risk factors for infection, and anticoagulation status are clinical signs that may help differentiate.
Diagnosis is typically made by transthoracic echocardiology (TTE): Increased gradients across the PV, RV hypertrophy, dilatation or dysfunction, increasing TR volume with elevated gradients from RV to RA.
Cardiac CT or CMRI can also be helpful in characterizing the stenosis (discreet or long-segment, singular or stenoses in series).
Invasive hemodynamic assessment in the catheterization laboratory can be especially helpful to more specifically characterize the degree and location of the obstruction as well as perform intervention (balloon, stent placement, TCPV) to relieve the obstruction. It is not uncommon that distal pulmonary artery stenting is required at the time of ballooning of the valve or valve-in-valve TCPVR.
Lab testing sometimes performed includes INR, hemoglobin level, hemolysis labs, NT-BNP. If acute thrombosis of the bioprosthetic valve is diagnosed, a hypercoaguable work-up to include familial thrombophilia should be performed
FDG PET may be an adjunctive tool to help evaluate for prosthetic valve endocarditis by detection of acute inflammation.

What are the indications for management of valve obstruction? What are the primary management strategies?
As previously discussed the differential diagnosis of bioprosthetic valve obstruction includes BPVT, pannus, and vegetation. Valve intervention is indicated for symptomatic severe stenosis and treatment strategy depends on the underlying cause.
BPVT: If stable, can trial anticoagulation therapy. If unstable immediate therapy is required which requires surgery or fibrinolytics.
Pannus: Severe stenosis caused by soft tissue overgrowth with degeneration and calcium requires stenting open the obstructive valve in the catheterization laboratory followed by a valve-in-valve TCPV placement. It is increasingly rare that an obstructed bioprosthetic PV cannot be managed in this fashion and surgical PVR is needed. Medical therapy is ineffective in treating the underlying cause although can treat consequences from heart failure.
Vegetation: Endocarditis with vegetation causing severe stenosis is usually treated with surgical intervention. Decisions regarding surgical indications for endocarditis are complex and a trial of medical therapy is reasonable in less severe disease.
For native pulmonic valve obstruction many patients are amenable to balloon valvuloplasty and recurrent stenosis is rare. Patients are usually left with some degree of pulmonic insufficiency. Balloon valvuloplasty alone is rarely an effective long-term solution for bioprosthetic valve stenosis.
What is the role of right heart catheterization in valvular obstructive shock?
Typically valve gradients are obtained by TTE in a parasternal short axis view but hemodynamic gradients measured in the cath lab can provide supportive information
Additionally right heart catheterization can assist in the characterization of the degree of shock (through calculation of cardiac indices, pulmonary artery pulmonary index, and cardiac power output).
In pulmonary stenosis, the narrowed valve creates a pressure differential between the RV and PA such that a peak pressure difference RVSP-PASP >64 mmHg is considered severe or mean difference of >35 mmHg
As in this case, catheterization also allows for therapeutic intervention either through balloon valvuloplasty or TCPVP.
References – Obstructive Cardiogenic ShocK
Egbe AC, Pislaru SV, Pellikka PA, et al. Bioprosthetic Valve Thrombosis Versus Structural Failure: Clinical and Echocardiographic Predictors. J Am Coll Cardiol. 2015;66(21):2285-2294. doi:10.1016/j.jacc.2015.09.022
McElhinney DB, Zhang Y, Levi DS, et al. Reintervention and Survival After Transcatheter Pulmonary Valve Replacement. J Am Coll Cardiol. 2022;79(1):18-32. doi:10.1016/j.jacc.2021.10.031
Nishimura RA, Carabello BA. Hemodynamics in the cardiac catheterization laboratory of the 21st century. Circulation. 2012;125(17):2138-2150. doi:10.1161/CIRCULATIONAHA.111.060319
Otto, Catherine M et al. “2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.” Circulation vol. 143,5 (2021): e35-e71. doi:10.1161/CIR.0000000000000932
Rao PS. Management of Congenital Heart Disease: State of the Art-Part II-Cyanotic Heart Defects. Children (Basel). 2019;6(4):54. Published 2019 Apr 4. doi:10.3390/children6040054
Stout, Karen K et al. “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.” Journal of the American College of Cardiology vol. 73,12 (2019): 1494-1563. doi:10.1016/j.jacc.2018.08.1028
VARC-3 WRITING COMMITTEE et al. “Valve Academic Research Consortium 3: updated endpoint definitions for aortic valve clinical research.” European heart journal vol. 42,19 (2021): 1825-1857. doi:10.1093/eurheartj/ehaa799
Arrigo, M., Huber, L. C., Winnik, S., Mikulicic, F., Guidetti, F., Frank, M., Flammer, A. J., & Ruschitzka, F. (2019). Right ventricular failure: Pathophysiology, diagnosis and treatment. Cardiac Failure Review, 5(3), 140–146. https://doi.org/10.15420/cfr.2019.15.2
Kanwar, M. K., Everett, K. D., Gulati, G., Brener, M. I., & Kapur, N. K. (2022). Epidemiology and management of right ventricular-predominant heart failure and shock in the cardiac intensive care unit. European Heart Journal. Acute Cardiovascular Care, 11(7), 584–594. https://doi.org/10.1093/ehjacc/zuac063
51 min
November 30, 2023347. Case Report: Heartmate 3 with a Side of Mustard – Medical University of South Carolina
CardioNerds (Dr. Josh Saef and Dr. Sumeet Vaikunth) join Dr. Sheng Fu, Dr. Payton Kendsersky, and Dr. Aniqa Shahrier from the Medical University of South Carolina for some off-shore fishing. They discuss the following featuring a patient with D-TGA and Eisenmenger’s syndrome treated with a Heartmate 3. Expert commentary was provided by Dr. Brian Houston. The episode audio was edited by student Dr. Adriana Mares.
A 39-year-old woman with a history of D-transposition of the great arteries (D-TGA) with prior atrial switch repair (Mustard) was admitted from the clinic with cardiogenic shock. She underwent right heart catheterization which demonstrated elevated biventricular filling pressures and low cardiac index. An intra-aortic balloon pump was placed, and the patient was evaluated for advanced therapies. A liver biopsy showed grade 3 fibrosis, which, in combination with her shock state, made her a high-risk candidate for isolated heart or combined heart-liver transplantation. After a multi-disciplinary discussion, the patient underwent a Heartmate III left ventricular assist device (LVAD) implant in her systemic right ventricle. Although she did well post-operatively, she was admitted after a month with recurrent cardiogenic shock, with imaging showing her inflow cannula had become perpendicular to the septum. The patient and family eventually decided to pursue comfort measures, and the patient passed.
“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.
US Cardiology Review is now the official journal of CardioNerds! Submit your manuscript here.
CardioNerds Case Reports Page
CardioNerds Episode Page
CardioNerds Academy
Cardionerds Healy Honor Roll
CardioNerds Journal Club
Subscribe to The Heartbeat Newsletter!
Check out CardioNerds SWAG!
Become a CardioNerds Patron!
Case Media – D-TGA and Eisenmenger’s syndrome treated with a Heartmate 3
Pearls – D-TGA and Eisenmenger’s syndrome treated with a Heartmate 3
Early diagnosis of cyanotic congenital heart disease is paramount for treatment and prevention of future complications.
Adult congenital heart disease requires a multi-disciplinary team for management in consultation with an adult congenital cardiology specialist.
Eisenmenger syndrome is related to multiple systemic complications and has a high rate of mortality.
Advancement in PAH medical management can offer noninvasive treatment options for some patients.
Transthoracic echocardiography is the cornerstone for diagnosis. Other modalities (e.g. cardiac CT, cardiac MRI, invasive catheterization) can aid in diagnosis and management.
Pearls – D-TGA and Eisenmenger’s syndrome treated with a Heartmate 3
While advances in pediatric surgery have allowed many patients born with congenital heart disease to survive into adulthood, adult congenital heart disease (ACHD) patients are complex and prone to numerous adverse sequalae including arrhythmias, heart failure, valvular disease, and non-cardiac organ dysfunction.
Heart failure can be a challenging presentation in ACHD patients due to a longstanding history of clinical compensation.
Their unique and complex anatomy, as well as highly variable clinical presentation, present unique challenges when it comes to advanced heart failure options such as durable left ventricular assist devices (LVAD) or transplantation.
While durable LVAD implantation is possible in patients with systemic right ventricles, anatomic compatibility is paramount and poses ongoing challenges in their management.
Goals of care discussions should be had early, as options for treatment may be limited.
Show Notes – D-TGA and Eisenmenger’s syndrome treated with a Heartmate 3
What are some common sequelae in ACHD patients?
ACHD patients are a heterogeneous population, but atrial tachycardias are extremely frequent in this patient population, often due to re-entrant pathways around surgical suture lines. These can often be treated with radiofrequency ablation while paying close attention to their challenging anatomy. Heart failure is also extremely common (up to 40% incidence) but has variable incidence dependent on the specific anatomy. Valvular heart disease, including infective endocarditis as well as non-cardiac organ dysfunction, are also important contributors to the overall prognosis of ACHD patients.
How does heart failure present in ACHD patients?
Heart failure presentations in ACHD patients tend to be subacute and insidious, as patients often have become accustomed to their symptoms. They are often unable to identify clear exercise limitations due to the slow, subacute nature of symptoms. However, acute presentations and shock can also be seen. Heart failure is the leading cause of death in ACHD patients.
What are the challenges for advanced therapies in the ACHD population?
First and foremost, risk stratification for these patients is often difficult due to often unreliable self-reporting of symptoms. Thus, early recognition becomes key, but even then, may not be enough. Several anatomic and physiological challenges remain. ACHD patients have often undergone multiple cardiac surgeries, increasing the surgical risk of redo sternotomies with severe adhesions. Due to the longstanding nature of their disease, ACHD patients often develop irreversible pulmonary hypertension (making transplant prohibitive) or end-organ dysfunction secondary to right heart failure (necessitating dual organ transplant).
Is durable LVAD a feasible option for patients with systemic right ventricles?
Isolated case reports demonstrate the feasibility of the off-label use of durable LVADs in systemic right ventricles. The complex anatomy of these patients has led to reports of alternative implant sites for the inflow cannula to minimize obstruction. Alternative surgical approaches, such as lateral thoracotomy, have also been described to try to avoid the adhesions that are often seen in these patients as a result of multiple prior cardiac surgeries. While the surgery is technically feasible, long-term data is not available, and this remains a “bail-out” therapy with current-generation LVAD designs.
What is the role of goals of care discussions in ACHD patients?
Advanced care planning is rarely discussed in routine clinic visits, and most providers wait until the condition has deteriorated significantly. Most ACHD patients want to discuss advanced care early before the disease progresses, but some do not, so it is important to ask about patient preferences. The majority of ACHD patients prefer to have advanced care discussions with their ACHD provider due to an already established and trusting relationship.
References
Gatzoulis MA, Webb GD, F. DPE, Hornung T, O’Donnell C. Transposition of the Great Arteries. In: Diagnosis and Management of Adult Congenital Heart Disease: Expert Consult – Online and Print. 3rd ed. Elsevier; 2018:513-527.
Stout KK, Daniels CJ, Aboulhosn JA, et al. 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. Circulation. 2019;139(14).
Schwerzmann M, Goossens E, Gallego P, et al. Recommendations for advance care planning in adults with congenital heart disease: A position paper from the ESC Working Group of adult congenital heart disease, the Association of Cardiovascular Nursing and Allied Professions (ACNAP), the European Association for Palliative Care (EAPC), and the International Society for Adult Congenital Heart Disease (ISACHD). European Heart Journal. 2020;41(43):4200-4210. doi:10.1093/eurheartj/ehaa614
Khairy P, Clair M, Fernandes SM, et al. Cardiovascular outcomes after the arterial switch operation for D-transposition of the great arteries. Circulation. Jan 22 2013;127(3):331-9. doi:10.1161/CIRCULATIONAHA.112.135046
Piran S, Veldtman G, Siu S, Webb GD, Liu PP. Heart failure and ventricular dysfunction in patients with single or systemic right ventricles. Circulation. Mar 12 2002;105(10):1189-94. doi:10.1161/hc1002.105182
Zandstra TE, Palmen M, Hazekamp MG, Meyns B, Beeres SLMA, Holman ER, Kiès P, Jongbloed MRM, Vliegen HW, Egorova AD, Schalij MJ, Tops LF. Ventricular assist device implantation in patients with a failing systemic right ventricle: a call to expand current practice. Neth Heart J. 2019 Dec;27(12):590-593.
Burchill LJ. Heart transplantation in adult congenital heart disease. Heart. 2016;102:1871–1877. doi: 10.1136/heartjnl-2015-309074.
1 hr 9 min
November 22, 2023345. Case Report: A Case of Unrepaired Congenital Heart Disease – University of Chicago – Northshore University
CardioNerds (Dr. Josh Saef, Dr. Agnes Koczo) join Dr. Iva Minga, Dr. Kifah Hussain, and Dr. Kevin Lee from the University of Chicago – NorthShore to discuss a case of unrepaired congenital heart disease that involves D-TGA complicated by Eisenmenger syndrome. The ECPR was provided by Dr. Michael Earing. Audio editing by Dr. Akiva Rosenzveig.
A 25-year-old woman with an unknown congenital heart disease that was diagnosed in infancy in Pakistan presents to the hospital for abdominal pain and weakness. She is found to be profoundly hypoxemic, and an echocardiogram revealed D-transposition of the great arteries (D-TGA) with a large VSD. As this was not repaired in childhood, she has unfortunately developed Eisenmenger syndrome with elevated pulmonary vascular resistance. She is stabilized and treated medically for her cyanotic heart disease. Unfortunately given the severity and late presentation of her disease, she has limited long-term options for care. CardioNerds discuss the diagnosis of D-TGA and Eisenmenger’s syndrome, as well as long-term management and complications associated with this entity.
“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.
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Case Media – Unrepaired Congenital Heart Disease
Pearls – Unrepaired Congenital Heart Disease
Early diagnosis of cyanotic congenital heart disease is paramount for treatment and prevention of future complications.
Adult congenital heart disease requires a multi-disciplinary team for management in consultation with an adult congenital cardiology specialist.
Eisenmenger syndrome is related to multiple systemic complications and has a high rate of mortality.
Advancement in PAH medical management can offer noninvasive treatment options for some patients.
Transthoracic echocardiography is the cornerstone for diagnosis. Other modalities (e.g. cardiac CT, cardiac MRI, invasive catheterization) can aid in diagnosis and management.
Show Notes – Unrepaired Congenital Heart Disease
Cyanotic congenital heart disease is often diagnosed in infancy and timely treatment is paramount. As these diseases progress over time, pulmonary over-circulation often pulmonary hypertension (PH), elevated pulmonary vascular resistance, and Eisenmenger syndrome will develop, which preclude definitive treatment. For D-TGA, before PH develops, there are surgical options such as the arterial switch procedure that can treat the disease. Unfortunately, once Eisenmenger syndrome develops, there are multiple systemic complications including hyperviscosity, thrombosis, bleeding, kidney disease, iron deficiency, arrhythmias, etc. that can occur. Management requires a multi-disciplinary team including an adult congenital cardiology specialist, but mortality rates remain high, with median survival reduced by 20 years, worse with complex cardiac defects. Bosentan is a first line treatment for patients with Eisenmenger syndrome, with PDE-5 inhibitors as a second line either by themselves or in combination with bosentan. Data are currently limited for latest-generation PH treatments in Eisenmenger syndrome and further study is still underway.
References
Ferencz C. Transposition of the great vessels. Pathophysiologic considerations based upon a study of the lungs. Circulation. 1966 Feb;33(2):232-41.
Arvanitaki A, Gatzoulis MA, Opotowsky AR, Khairy P, Dimopoulos K, Diller GP, Giannakoulas G, Brida M, Griselli M, Grünig E, Montanaro C, Alexander PD, Ameduri R, Mulder BJM, D’Alto M. Eisenmenger Syndrome: JACC State-of-the-Art Review. J Am Coll Cardiol. 2022 Mar 29;79(12):1183-1198.
Earing MG, Webb GD. Congenital heart disease and pregnancy: maternal and fetal risks. Clin Perinatol. 2005 Dec;32(4):913-9, viii-ix
Østergaard L, Valeur N, Ihlemann N, Bundgaard H, Gislason G, Torp-Pedersen C, Bruun NE, Søndergaard L, Køber L, Fosbøl EL. Incidence of infective endocarditis among patients considered at high risk. Eur Heart J. 2018 Feb 14;39(7):623-629
Opotowsky AR, Moko LE, Ginns J, Rosenbaum M, Greutmann M, Aboulhosn J, Hageman A, Kim Y, Deng LX, Grewal J, Zaidi AN, Almansoori G, Oechslin E, Earing M, Landzberg MJ, Singh MN, Wu F, Vaidya A. Pheochromocytoma and paraganglioma in cyanotic congenital heart disease. J Clin Endocrinol Metab. 2015 Apr;100(4):1325-34.
Jaïs X, D’Armini AM, Jansa P, Torbicki A, Delcroix M, Ghofrani HA, Hoeper MM, Lang IM, Mayer E, Pepke-Zaba J, Perchenet L, Morganti A, Simonneau G, Rubin LJ; Bosentan Effects in iNopErable Forms of chronIc Thromboembolic pulmonary hypertension Study Group. Bosentan for treatment of inoperable chronic thromboembolic pulmonary hypertension: BENEFiT (Bosentan Effects in iNopErable Forms of chronIc Thromboembolic pulmonary hypertension), a randomized, placebo-controlled trial. J Am Coll Cardiol. 2008 Dec 16;52(25):2127-34.
Gatzoulis MA, Landzberg M, Beghetti M, Berger RM, Efficace M, Gesang S, He J, Papadakis K, Pulido T, Galiè N; MAESTRO Study Investigators. Evaluation of Macitentan in Patients With Eisenmenger Syndrome. Circulation. 2019 Jan 2;139(1):51-63.
McLaughlin VV, Gaine SP, Howard LS, Leuchte HH, Mathier MA, Mehta S, Palazzini M, Park MH, Tapson VF, Sitbon O. Treatment goals of pulmonary hypertension. J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D73-81.
Stoica SC, McNeil KD, Perreas K, Sharples LD, Satchithananda DK, Tsui SS, Large SR, Wallwork J. Heart-lung transplantation for Eisenmenger syndrome: early and long-term results. Ann Thorac Surg. 2001 Dec;72(6):1887-91.
1 hr 1 min
October 25, 2023339. ACHD: Electrophysiology in ACHD with Dr. Frank Fish
CardioNerds (Amit Goyal and Daniel Ambider) ACHD series co-chair Dr. Daniel Clark (Vanderbilt University), cardiology FIT lead Dr. Stephanie Fuentes (Houston Methodist Hospital), and Dr. Frank Fish, a Pediatric Electrophysiologist and the Director of the Pediatric Electrophysiology (EP) Lab at Monroe Carrell Jr Children’s Hospital at Vanderbilt University. He is a board certified Adult Congenital Heart Disease (ACHD) physician and has a wealth of experience performing EP procedures in adults living with congenital heart disease. Audio editing was performed by student Dr. Shivani Reddy.
In this episode, we discuss key concepts and management of electrophysiologic issues that we can encounter when caring for adults with congenital heart disease. Arrythmias in adults with congenital heart disease can be intrinsic due to the defect itself or as a consequence of the interventions that they have undergone to palliate and/or repair these defects. The complex anatomy of these patients and the years of pressure and volume load make them not only exquisitely hemodynamically sensitive to arrhythmias (that may otherwise not be of much consequence to the general population) but they also make interventions (catheter ablation or device implant) complex. We therefore embark in a case-based discussion of patients with ACHD (Fontan circulation, Ebstein’s anomaly and Tetralogy of Fallot) in an effort to highlight the presentation of arrythmias and the management strategy in this very important group of patients.
Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values.
US Cardiology Review is now the official journal of CardioNerds! Submit your manuscript here.
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
CardioNerds Adult Congenital Heart Disease Page
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Pearls – Electrophysiology in ACHD
Patients with Fontan circulation have a high risk of developing atrial (and ventricular) arrhythmias and they are highly sensitive to the hemodynamic consequences that these arrythmias ensue. The goal of therapy then should be to achieve sinus or atrial paced rhythm. Rate control should NOT the goal.
Patients with Ebstein’s anomaly have high arrhythmic potential. They can have multiple accessory pathways (especially right sided) which can in turn be associated with sudden cardiac death. We should have low threshold for EPS +/- catheter ablation in patients with WPW pattern.
Patients with Tetralogy of Fallot have a unique risk for SCD that warrant ICD implant apart from the standard criteria (LVEF <=35% and NYHA II-III symptoms). This involves the pump (RV dilation/dysfunction), electricity (QRSd>180 ms) and surgical repair approach.
Patient’s anatomy is the major consideration when implanting devices (PPM/ICD). We ought to assess for residual intracardiac shunt at the atrial level and consider closing if feasible prior to placing a device. CRT has merit in systemic LV but less so in systemic RV.
Notes- Electrophysiology in ACHD
What should we know about atrial arrhythmias in a Fontan patient?
Intraatrial re-entrant tachycardia (IART) is slower than typical atrial flutter with atrial rates generally <300 bpm. It does not have typical flutter waves and when it is slow enough, it may be confused with sinus tachycardia. A simple maneuver to help discern between sinus tachycardia or AT/AFl is to stand the patient up. If there’s no variability in heart rate, then it is less likely for it be sinus. With intact AV conduction, atrial flutter or IART can conduct 1:1.
IART is usually a harbinger of issues among patients with Fontan circulation. These patients are dependent on passive pulmonary blood flow. So, loss of AV synchrony in these patients may raise left atrial pressures, impede forward flow and subsequently compromise cardiac output. Tachycardia reduces diastolic filling, thus further limiting systemic venous return. IART can be associated with systemic illness, worsening pump function, obstruction in the Fontan circuit, etc. Once it’s present, it warrants full investigation into the trigger and a management plan to prevent recurrence.
How should we manage atrial arrhythmias in a Fontan patient?
The goal of therapy is to achieve sinus (or A-paced) rhythm. Rate control should not be the goal.
If hemodynamically unstable-> DCCV
Chemical options include ibutilide but would use with caution if one does not know sinus function as post conversion bradycardia can put patient at risk for torsades. Amiodarone or Procainamide are helpful in patients that are requiring frequent cardioversions acutely. However, would avoid amiodarone if patients are heading to EP lab for catheter ablation as this medication affects defibrillation thresholds.
How should we approach catheter ablation in a Fontan patient?
Catheter ablation should be considered in centers with expertise with managing ACHD patients.
Operators should understand patient’s anatomy, ventricular function and systemic issues such as renal or hepatic function. Prior to intervening, venous/arterial access issues ought to be assessed. In cases where there’s femoral vein or IVC occlusion, trans-hepatic approach can be considered. Presence of atrial thrombi ought to be assessed prior to ablation as well. Given slower atrial rate leading to less degree of stasis compared to Afib, left atrial thrombi are less common.
When ablating, first assess the Fontan itself (lateral tunnel in this case) as it can become dilated overtime and this leads to an area of scar that can be source of arrythmias. If Fontan is not the source, then one has to go through it to reach the atria. In extra-cardiac Fontan, this may require the use of a radiofrequency-powered needle to cross the conduit and thereby reach the atria. A retrograde approach from the aorta, into the systemic RV, and then into the atria is much less ideal given that this would entail placing catheters in the systemic circulation for prolonged periods of time, raising thromboembolic risk, not to mention the trajectory that is required with this approach.
How should we approach anticoagulation in Fontan patients?
CHADS2VASC for CVA prevention risk stratification is not as heavily applicable in this patient population as compared to the general population.
The Fontan circuit is highly susceptible to thrombosis. Each element of Virchow’s triad is present in patients with Fontan circulation: (1) Venous stasis – from the passive pulmonary flow and venous hypertension necessary to maintain this forward flow, (2) Activation of blood coagulation – underlying liver disease, prostheses, and inflammation may all play a role in making these patients hypercoagulable, and (3) Venous damage – surgical reconstruction of the venous tree and chronic venous hypertension lead to venous damage. Together, all these factors increase the risk of thrombosis. Let’s not forget that there are often residual right-to-left shunts that present opportunities for paradoxical emboli to the systemic circulation. So, not only is clotting more common, the stakes are often higher in this population.
All adults with Fontan palliation are on at least anti-platelets (aspirin) and many on systemic anticoagulation. Special circumstances that prompt consideration of systemic anticoagulation include prior clots in the Fontan circuit, atrial arrhythmias, thrombi in hypoplastic chambers when there are systemic communications or if they have other indications like: DVT/PE, mechanical valve prosthesis.
What arrhythmias are associated with patients who are born with Ebstein’s anomaly and when should we pursue EP study and/or catheter ablation?
Approximately 1/3 of adults with Ebstein’s anomaly have multiple accessory pathways (particularly right sided) and atrial tachyarrhythmias are very common in these patients.
In the setting of ventricular preexcitation (Wolf Parkinson White syndrome), atrial tachyarrhythmias may expose the patient to a higher risk of lethal ventricular arrhythmias. This is why we don’t give nodal blockers to WPW patients in atrial fibrillation, as this can precipitate 1:1 conduction down the accessory pathway. In patients with Ebstein’s anomaly, without WPW, EKG shows right bundle branch block.
Ventricular myocardium that lies between the true AV groove and displaced tricuspid valve is thin-walled and unhealthy thus prone to ventricular tachycardia
The 2018 ACC/AHA ACHD guidelines give EPS with or without catheter ablation a IIA indication for diagnostic evaluation of adults with Ebstein anomaly and ventricular preexcitation (WPW pattern), even without SVT. Likewise, these guidelines give a IIA indication for EPS +/- CA prior to surgical intervention on the tricuspid valve regardless of a history of preexcitation or SVT.
In which ACHD patients should we consider the use of Holter monitor when they are asymptomatic?
To screen for abrupt loss of pre-excitation in patients with Ebstein’s anomaly.
In Fontan patients to screen for junctional rhythm as this loss of AV synchrony would lead to long term hemodynamic consequences.
In patients with tetralogy of Fallot, to identify patients with non-sustained VT
In patients with D-TGA and atrial switch, to screen for sinus node dysfunction
In patients with CC-TGA, to screen for AV conduction abnormalities.
How should we approach ICD implant for primary sudden cardiac death prevention in patients who are born with Tetralogy of Fallot (ToF)?
In general, primary prevention ICD for ToF is indicated for patients meeting standard criteria (LVEF <=35% and NYHA II-III symptoms). There have been many studies to better understand SCD risk among patients living with ToF. We can think of the risks in 3 categories: (1) The pump – how hypertrophied, dilated, and/or weak is the RV? Is RV dilatation so bad it is affecting the LV? If so, this confers a greater risk of SCD. (2) Electrical – has the patient had prior VT/VF (are we talking secondary prevention?)? How wide and fractionated is the QRS? We think of QRSd >=180 msec as a cut-point for increased risk and there’s new data from France last year that a fractionated QRS may matter even more (DAI-T4F registry). (3) Surgical/RVOT – what was the original repair? Did they use an LV vent or a RV ventriculotomy? Was there a transannular patch or RVOT aneurysm post-operatively? What’s the current status of the pulmonary valve?
What are the technical considerations that ACHD patients warrant when having an ICD/PPM implanted compared to the general population?
Patient’s anatomy is the major consideration when implanting devices. We need to first assess if there’s remaining intracardiac shunting at the atrial level and consider closure prior to implanting a device.
CRT has merit in systemic LV. CRT in systemic RV like patients with D-TGA with atrial switch has less merit and is more technically challenging as it may require epicardial lead.
References – Electrophysiology in ACHD
Stout KK et al. 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. Circulation.2019;139:e698-e800. Doi:10.1161/CIR. 0000000000000603
Khairy P et al. PACES/HRS Expert consensus Statement on the recognition and management of arrhythmias in adult congenital heart disease. Heart Rhythm.2014(11): e102-e165.
Baumgartner H et al. 2020 ESC Guidelines for the management of adult congenital heart disease, European Heart Journal. 2021; 42:563-645 doi:10.1093/eurheartj/ehaa554
Walsh EP, Cecchin F. Arrhythmias in adult patients with congenital heart disease. Circulation. 2007 Jan 30;115(4):534-45.
Waldmann et al. Long-Term Follow-Up of Patients With Tetralogy of Fallot and Implantable Cardioverter Defibrillator: The DAI-T4F Nationwide Registry. Circulation. 2020 Oct 27;142(17):1612-1622. doi:10.1161/CIRCULATIONAHA.120.046745.Epub 2020 Oct 1.
Meet Our Collaborators!
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/
1 hr 7 min
August 24, 2023328. ACHD: Eisenmenger Syndrome with Dr. Alexander Sasha Opotowsky
Eisenmenger syndrome is an end-stage complication of congenital heart disease that occurs when a left to right shunt causes pulmonary over-circulation, leading to vascular remodeling, increased vascular resistance, and ultimately even shunt reversal. Aside from cardiac complications, this pathology has unique complications secondary to chronic cyanosis. In this episode of CardioNerds co-founder Dr. Amit Goyal, ACHD series co-chair Dr. Josh Saef, and Dr. Khaled Tuwairqi (ACHD cardiologist at King Faisal / Elite Hospitals) join Dr. Alexander (Sasha) Optowsky (Director of the Adult Congenital Heart Disease Program at Cincinnati Childrens) to discuss diagnosis and management of Eisenmenger syndrome. Show notes were drafted by Dr. Anna Scandinaro and episode audio was edited by CardioNerds Academy Intern Dr. Akiva Rosenzveig.
Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values.
US Cardiology Review is now the official journal of CardioNerds! Submit your manuscript here.
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
CardioNerds Adult Congenital Heart Disease Page
CardioNerds Episode Page
CardioNerds Academy
Cardionerds Healy Honor Roll
CardioNerds Journal Club
Subscribe to The Heartbeat Newsletter!
Check out CardioNerds SWAG!
Become a CardioNerds Patron!
Pearls – Eisenmenger Syndrome
First described in 1897 by Victor Eisenmenger, Eisenmenger syndrome is a long-term complication of unrepaired left to right shunts, resulting from pulmonary vascular remodeling and pulmonary hypertension. This eventually leads to reversal of the shunt, with right to left flow causing cyanosis.
Evaluation for Eisenmenger syndrome should include a comprehensive history, physical exam, ECG, echocardiogram, cardiac catheterization, and laboratory work to identify multi-system complications of cyanosis and secondary erythrocytosis.
The most definitive means to diagnose Eisenmenger syndrome in a patient with a prior left-to-right shunt lesion is with a right heart cardiac catheterization showing right to left shunting (Qp:Qs < 1).
Eisenmenger syndrome is a multi-organ disease and many manifestations occur due to secondary erythrocytosis. Prevention and treatment of these complications are the major goals of care in this population. Complications of Eisenmenger syndrome include gout, bilirubin gallstones, stroke, paraganglioma/pheochromocytoma, thrombophilia, retinal changes, hypertrophic osteoarthropathy, and kyphoscoliosis.
Emergency non-cardiac complications of Eisenmenger syndrome include cerebral abscess and hemoptysis.
Pregnancy is contraindicated in Eisenmenger syndrome due to high maternal and fetal mortality.
Notes- Eisenmenger Syndrome
1. How does Eisenmenger syndrome develop? Does everyone with a left-to-right shunt develop it? Can it develop as an iatrogenic complication?
The pulmonary vasculature is not used to seeing the increased flow it receives in the context of a left to right shunt. Over time this leads to an increase in pulmonary vascular resistance and pulmonary hypertension. When pulmonary pressures exceed systemic pressures, this causes shunt reversal with right to left shunting causing deoxygenated blood to cross from right side of the heart to the left side bypassing the lungs and causing cyanosis.
The process of developing Eisenmenger syndrome is chronically progressive and so adaptive changes have time to occur. Not all persons with unrepaired shunts will develop Eisenmenger syndrome and it is not completely understood why these differences exist.
Eisenmenger syndrome can be acquired by the creation of palliative aorto-pulmonary shunts which cause pulmonary over-circulation with elevated PVR. This is less commonly seen now, due to earlier corrective repairs occurring at younger ages instead of palliative repairs with plans to perform corrective repairs at older ages. This could be seen in a patient who had a palliative repair in 1960-1980s and was lost to follow up. Eisenmenger syndrome is uncommon in Blalock-Taussig-Thomas (BTT) shunts (especially classic BTT) due to their small size and relatively limited flow.
2. What must-not miss conditions may mimic Eisenmenger syndrome?
Large vessel obstruction, specifically bilateral pulmonary artery stenosis and double chamber right ventricle (gradient through the RVOT leading to R->L shunt causing cyanosis), may mimic Eisenmenger syndrome. Their management and natural history differ from Eisenmenger syndrome and can be reversed with treatment.
3. How should one initiate a workup for Eisenmenger syndrome?
History – ask about symptoms of dyspnea, decreased exercise tolerance, and cyanosis. It is important to establish a detailed surgical history.
Physical exam – specifically assess for the presence (or absence) of a murmur, loud P2 suggestive of pulmonary hypertension, and differential cyanosis (suggestive of PDA – hint see Episode 263 for more information on PDAs and Eisenmenger syndrome). Additional Pearl: take blood pressure on opposite side of a BTT shunt, as a BTT can affect the BP!
EKG – look for evidence of right ventricular hypertrophy: RAD, RVH.
CXR – may show prominent pulmonary trunk.
Laboratory evaluation – should include CBC, iron panel, uric acid, CMP, and BNP to evaluate for signs of secondary erythrocytosis.
Echocardiogram – define the anatomy, calculate right ventricular systolic pressure, pulmonary artery diastolic pressure from pulmonary regurgitation jet, and flow across RVOT and LVOT for estimate of Qp/Qs.
Cardiac cath – measure pressures and saturations, calculate Qp/Qs, assess response to vasodilator challenge, calculate CO by Fick (not thermodilution assumptions for thermodilution are not met with shunts), and rule out mimics.
Remember: You must have had a right heart cardiac catheterization to diagnose someone with Eisenmenger syndrome. When reviewing external data, it is always important to interpret the primary data yourself to ensure the right diagnosis and management.
4. What are some multi-organ complications that can be seen in Eisenmenger syndrome secondary to cyanosis and secondary erythrocytosis (kidney’s response to cyanosis)?
Gout due to over production and decreased excretion of uric acid
Bilirubin gallstones due to high RBC turnover
Stroke due to paradoxical emboli
Paraglioma/pheochromocytoma due to mutations in the hypoxia pathway – VHL, succinate dehyrdrogenase, hypoxia inducible factor- curable cause of deterioration, should be investigated for in a patient with Eisenmenger syndrome and hypertension
Thrombophilia
Retinal changes
Hypertrophic osteoarthropathy
Kyphoscolisosis.
5. What is a life-threatening emergency that should be considered if a patient with Eisenmenger syndrome presents with neurologic symptoms? How about hemoptysis?
In a patient with Eisenmenger syndrome presenting with neurologic symptoms there should be a high index of suspicion for cerebral abscess. Chronic hypoxemia leads to secondary erythrocytosis leading to hyperviscosity in the central venous blood, this causes a favorable environment for bacterial growth.
Hemoptysis is not uncommon in this population and is a life threatening complication of Eisenmenger syndrome which requires a broad but direct differential to treat emergent cases quickly. The differential diagnosis includes pulmonary infections (Eisenmenger syndrome can cause decreased immune function), pulmonary vasoocclusive disease, aorto-pulmonary collaterals, as well as pulmonary artery in-situ thrombosis. Obtaining a Chest CTA is a high yield clinical tool to help establish this diagnosis. Interventional colleagues will be instrumental in treatment of many of these complications. Hemoptysis and iatrogenic surgical death used to be a high cause of mortality; this has decreased in recent years.
6. What steps can be taken to minimize operative risk in patients with Eisenmenger syndrome? Do we use routine phlebotomy in this population?
Surgery should be performed with an experienced cardiac anesthesiologist. Operative risk in Eisenmenger syndrome is lower than in pulmonary arterial hypertension as patients with Eisenmenger syndrome are able to increase their cardiac output by increasing right to left shunting at the expense of their oxygenation. Bubble filters should be used as available to minimize risk of air and paradoxical embolism. For non-urgent operations, exchange transfusions before surgery can improve clotting function. Coagulations labs are affected by high hematocrit (less plasma = fewer clotting factors); you will need to adjust citrate in the tube for an accurate PT/PTT.
Routine phlebotomy is no longer used in this population in the absence of moderate to severe hyperviscosity symptoms. Instead, the focus has shifted to iron deficiency. Repleting iron can improves symptoms of hyperviscosity, increases exercise capacity, and reduces stroke risk.
7. What is “Treat to Close” and should it be used in Eisenmenger syndrome?
The new area of “Treat to Close” is an evolving area without much data to support its practice. There is a need for randomized clinical trials and further investigation to clarify its use. Indications for shunt closure can be found in the 2018 AHA/ACC and the 2020 ESC Guidelines for the Management of Adults with Congenital Heart Disease. It is contraindicated to close a shunt when Eisenmenger syndrome has developed.
8. Is it safe for someone with Eisenmenger syndrome to become pregnant?
Among the few cardiac conditions where pregnancy is contraindicated are pulmonary arterial hypertension and Eisenmenger syndrome. In a pregnant woman with a resting oxygen saturation of less than 90%, babies are born small for gestational age, and most are born premature. In a pregnant woman with a resting oxygen saturation of less than 85%, less than 10% of babies are born alive. The maternal death rate is approximately 30% in these patients. Of note, some PAH medications are contraindicated in pregnancy.
9. Do patients with Eisenmenger syndrome receive heart transplants?
Transplantation in Eisenmenger syndrome requires both heart and lung transplant. The number of heart-lung transplant for congenital heart disease is a small proportion and even less so for Eisenmenger syndrome. Transplantation is complicated in patients with Eisenmenger syndrome due to the multi-organ system dysfunction that is often present.
References – Eisenmenger Syndrome
Opotowsky, Alexander R., et al. “Pheochromocytoma and paraganglioma in cyanotic congenital heart disease.” The Journal of Clinical Endocrinology & Metabolism 100.4 (2015): 1325-1334.
Silversides, Candice K., et al. “Pulmonary thrombosis in adults with Eisenmenger syndrome.” Journal of the American College of Cardiology 42.11 (2003): 1982-1987.
Baumgartner, Helmut, et al. “2020 ESC Guidelines for the management of adult congenital heart disease: the Task Force for the management of adult congenital heart disease of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Adult Congenital Heart Disease (ISACHD).” European heart journal 42.6 (2021): 563-645.
Stout, Karen K., et al. “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.” Journal of the American College of Cardiology 73.12 (2019): e81-e192.
Regitz-Zagrosek, Vera, 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 (ESC).” European heart journal 39.34 (2018): 3165-3241.
Meet Our Collaborators!
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/
1 hr 16 min
May 23, 2023300. Case Report: A Presentation of Heart Failure and Heart Block with Elusive Genetic Origins – Cambridge University
CardioNerds (Drs. Amit Goyal and Dan Ambinder) join Dr. Mina Fares, Dr. Johannes Bergehr, and Dr. Christina Peter from Cambridge University Hospitals in the UK. They discuss a case involving a man man in his 40’s presented with progressive heart failure symptoms. He has extensive background cardiac history including prior episodes of myocarditis and complete heart block status post permanent pacemaker implantation. Ultimately a diagnosis of Danon disease is made. Dr. Sharon Wilson provides the E-CPR for this episode. Audio editing by CardioNerds Academy Intern, Hirsh Elhence.
“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.
CardioNerds is collaborating with Radcliffe Cardiology and US Cardiology Review journal (USC) for a ‘call for cases’, with the intention to co-publish high impact cardiovascular case reports, subject to double-blind peer review. Case Reports that are accepted in USC journal and published as the version of record (VOR), will also be indexed in Scopus and the Directory of Open Access Journals (DOAJ).
Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values.
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Case Summary – A Presentation of Heart Failure and Heart Block with Elusive Genetic Origins – Cambridge University
A man in his 40s with a history of cardiac issues, including prior myocarditis and complete heart block, presented with progressive heart failure symptoms. Extensive cardiac investigations were conducted, revealing dilated left ventricle, mild to moderate left ventricular systolic dysfunction, normal coronaries, infero-lateral late gadolinium enhancement on cardiac MRI, and low-level uptake on PET-CT. Differential diagnosis included worsening underlying cardiomyopathy, recurrent myocarditis, tachycardia-related cardiomyopathy, pacemaker-induced LV dysfunction, and sarcoidosis. The patient’s condition improved with heart failure medications, and cardiac MRI showed a mildly dilated left ventricle with moderate systolic dysfunction and active inflammation in the anterior wall. Further evaluation indicated a family history of hereditary cardiomyopathy, and the patient exhibited phenotypic features such as early-onset heart disease, arrhythmias, family history of cardiomyopathy, learning problems, intellectual disability, and mild proximal myopathy. Genetic testing confirmed a LAMP2 mutation, leading to the diagnosis of Danon disease.
Case Media – A Presentation of Heart Failure and Heart Block with Elusive Genetic Origins – Cambridge University
Show Notes -A Presentation of Heart Failure and Heart Block with Elusive Genetic Origins – Cambridge University
References –
Danon, M. J., Oh, S. J., DiMauro, S., Miranda, A., De Vivo, D. C., & Rowland, L. P. (1981). Lysosomal glycogen storage disease with normal acid maltase. Neurology, 31(1), 51-7.
Nishino, I., Fu, J., Tanji, K., Nonaka, I., & Ozawa, T. (2000). Mutations in the gene encoding LAMP2 cause Danon disease. Nature, 406(6798), 906-10.
Tanaka, K., Nishino, I., Nonaka, I., Fu, J., & Ozawa, T. (2000). Danon disease is caused by mutations in the gene encoding LAMP2, a lysosomal membrane protein. Nature, 406(6798), 902-6.
Maron, B. J., Haas, T. S., Ackerman, M. J., Ahluwalia, A., Spirito, P., Nishino, I., … & Seidman, C. E. (2009). Hypertrophic cardiomyopathy and sudden death in a family with Danon disease. JAMA, 301(12), 1253-9.
Hashem, S., Zhang, J., Zhang, Y., Wang, H., Zhang, H., Liu, L., … & Wang, J. (2015). AAV-mediated gene transfer of LAMP2 improves cardiac function in Danon disease mice. Stem cells, 33(11), 2343-2350.
Chi, L., Wang, H., Zhang, J., Zhang, Y., Liu, L., Wang, J., … & Hashem, S. (2019). CRISPR/Cas9-mediated gene editing of LAMP2 in patient-derived iPSCs ameliorates Danon disease phenotypes. Proceedings of the National Academy of Sciences, 116(4), 556-565.
57 min
May 10, 2023297. Case Report: A Sinister Cause of Sudden Cardiac Death – University of Washington
CardioNerds (Daniel Ambinder) join Dr. Tomio Tran, Dr. Vid Yogeswaran, and Dr. Amanda Cai from the University of Washington for a break from the rain at the waterfront near Pike Place Market. They discuss the following case: A 46-year-old woman presents with cardiac arrest and was found to have cor triatriatum sinistrum (CTS). CTS is a rare congenital cardiac malformation in which the left atrium is divided by a fenestrated membrane, which can restrict blood flow and cause symptoms of congestive heart failure. Rarely, the condition can present in adulthood. To date, there have been no cases of sudden cardiac death attributed to CTS. Dr. Jill Steiner provides the E-CPR for this episode. Audio editing by CardioNerds Academy Intern, student doctor Akiva Rosenzveig.
“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.
CardioNerds is collaborating with Radcliffe Cardiology and US Cardiology Review journal (USC) for a ‘call for cases’, with the intention to co-publish high impact cardiovascular case reports, subject to double-blind peer review. Case Reports that are accepted in USC journal and published as the version of record (VOR), will also be indexed in Scopus and the Directory of Open Access Journals (DOAJ).
Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values.
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Case Media – A Sinister Cause of Sudden Cardiac Death – University of Washington
A 40-year-old woman with a history of recurrent exertional syncope had sudden loss of consciousness while kissing her partner. The patient received bystander CPR while 911 was called. EMS arrived within 10 minutes of the call and found the patient apneic and unresponsive. Initial rhythm check showed narrow complex tachycardia at a rate of 136 BPM. ROSC was eventually achieved. A 12-lead ECG showed that the patient was in atrial fibrillation with rapid ventricular rate. The patient was intubated and brought to the emergency department. The patient spontaneously converted to sinus rhythm en route to the hospital.
In the emergency department, vital signs were remarkable for hypotension (76/64 mmHg) and sinus tachycardia (110 BPM). The physical exam was remarkable for an inability to follow commands. Laboratory data was remarkable for hypokalemia (2.5 mmol/L), transaminitis (AST 138 units/L, ALT 98 units/L), acidemia (pH 7.12), and hyperlactatemia (11.2 mmol/L). CT scan of the chest revealed a thin membrane within the left atrium. Transthoracic echocardiogram showed normal biventricular size and function, severe tricuspid regurgitation, pulmonary artery systolic pressure of 93 mmHg, and the presence of a membrane within the left atrium with a mean gradient of 25 mmHg between the proximal and distal left atrial chambers. Vasopressors and targeted temperature management were initiated.
The patient was able to be re-warmed with eventual discontinuation of vasopressors, however she had ongoing encephalopathy and seizures concerning for hypoxic brain injury. There was discussion with the adult congenital heart disease team about next steps in management, however the patient was too sick to undergo any definitive treatment for the intracardiac membrane within the left atrium. The patient developed ventilator associated pneumonia and antibiotics were initiated. The patient ultimately developed bradycardia and pulseless electrical activity; ROSC was unable to be achieved, resulting in death.
Autopsy was remarkable for the presence of a fenestrated intracardiac membrane within the left atrium and lack of other apparent congenital heart defects. There was right ventricular hypertrophy and pulmonary artery intimal thickening with interstitial fibrosis suggestive of pulmonary hypertension. There were bilateral acute subsegmental pulmonary emboli present. The cause of death was declared to be arrhythmia in the setting of pulmonary hypertension and right sided heart failure caused by cor triatriatum sinistrum with a significant contribution from acute subsegmental pulmonary emboli.
Case Media – A Sinister Cause of Sudden Cardiac Death – University of Washington
Pearls – A Sinister Cause of Sudden Cardiac Death – University of Washington
In a patient presenting with syncope, the following feature may indicate an underlying cardiac etiology: exertional syncope, sudden syncope without a prodrome, structural heart disease, advanced age, and family history of sudden cardiac or unexplained death.
Cor triatriatum sinistrum is diagnosed by CT, echocardiography, or MRI and is often found incidentally in adults.
Acute management of CTS is similar to mitral stenosis and consists of 1) careful volume management to avoid both hypovolemia and hypervolemia, and 2) avoidance of tachycardia to allow for adequate LV filling during diastole. Surgical resection of the membrane is definitive.
A mean gradient of ≥ 8 mmHg across a CTS membrane is hemodynamically significant and should prompt surgical evaluation for membrane resection.
Show Notes -A Sinister Cause of Sudden Cardiac Death – University of Washington
Syncope
Loss of consciousness due to transient decrease in cerebral blood flow
Differential
Orthostatic
Neurally mediated
Cardiogenic
Mimickers of syncope: seizures, head trauma causing loss of consciousness, hypoglycemia
Red flag symptoms of cardiogenic syncope
Advanced age
Exertional or while lying down
Palpitations prior to event
Structural heart disease
Family history of unexplained or sudden cardiac death
Structural heart disease etiologies
Generally left sided and causes obstruction to blood flow
Valvular stenoses
Hypertrophic cardiomyopathy
Cardiac tumors
Arrhythmia etiologies
Can be caused by any brady- or tachyarrhythmia, especially in the setting of structural heart disease
Most commonly
Sinus node disease
High degree heart block
Ventricular arrhythmia
Pulse pressure
Difference between systolic and diastolic pressure
Normal ~ 40 mmHg
Narrow – <25% of systolic blood pressure
Wide – >100 mmHg
Wide pulse pressure etiologies
Physical conditioning (normal variant)
Aortic regurgitation
Severe iron deficiency anemia
Hyperthyroidism
Arteriosclerosis
Shunting from arteriovenous fistulas
Narrow pulse pressure etiologies – indicates low stroke volume/cardiac output
Heart failure
Hypovolemia
Blood loss
Valvular stenosis
Cardiac tamponade
Pulmonary embolism
Cor triatriatum sinister
Presence of a membrane within the left atrium that divides the left atrium into 2 chambers
Pathophysiology
Theorized to be caused by misincorporation of the pulmonary veins within the left atrium causing a membrane within the left atrium
If restrictive, sequelae include congestive heart failure, pulmonary hypertension, and right ventricular dysfunction
Often associated with other congenital heart disease (ASD, pulmonary venous return, mitral regurgitation)
Epidemiology
Among the rarest of all congenital heart disease (up to 0.4% of all congenital heart disease, but true incidence is unknown as many can be asymptomatic)
Found more often in infancy/childhood; often found incidentally in adults
Signs/symptoms
Many are asymptomatic
Over time, membrane may become fibrotic or calcified and cause significant obstruction
Infants/children – pulmonary congestion, respiratory infections, cyanosis, growth restriction
Adults – dyspnea, chest pain, palpitations, syncope
Thrombotic events are common likely from vascular injury and congestion/stasis
Atrial arrhythmia is associated with CTS, possibly from scarring of the membrane vs chronic elevation of left atrial pressure
Diagnosis
Made by imaging (CT chest/cardiac, echocardiography, MRI)
Primary competing differential diagnosis is supravalvular mitral ring
If intra-atrial membrane contains the left atrial appendage and pulmonary veins -> supravalvular mitral ring
Associated with Schone complex (supravalvular mitral ring, parachute mitral valve, subaortic stenosis, aortic coarctation
CTS rarely associated with Shone complex
If intra-atrial membrane contains only the pulmonary veins -> CTS
Can be found incidentally and cause technical issues in cardiac procedures that require transeptal atrial punctures
Quantify degree of restriction with echocardiography; gradients ≥ 8 mmHg are significant per AHA guidelines
Treatment
Acute:
Diuretics to treat congestion
Fluids to avoid hypovolemia due to preload dependence for cardiac output
Treat underlying causes of tachycardia to optimize diastolic filling
Treat tachyarrhythmia with anti-arrhythmis and AV nodal blockers
Chronic/definitive:
Surgical resection of the membrane offers a good and durable outcome
Low recurrence rate, residual gradients likely due to incomplete resection
Pulmonary hypertension
WHO classification
Group 1 – Pulmonary arterial hypertension (idiopathic, toxin induced, HIV, connective tissue disease, congenital heart disease
Congenital heart disease comprises a small portion of group 1, typically from shunt lesions
Group 2 – Left sided heart disease
Group 3 – Pulmonary disease
Group 4 – CTEPH
Group 5 – Unclear mechanisms (sickle cell, sarcoid, metabolic disease)
References –
Albassam OT, Redelmeier RJ, Shadowitz S, Husain AM, Simel D, Etchells EE. Did This Patient Have Cardiac Syncope?: The Rational Clinical Examination Systematic Review. JAMA. 2019;321(24):2448-2457. doi:10.1001/jama.2019.8001
Jegier W, Gibbons JE, Wigglesworth FW. Cortriatriatum: clinical, hemodynamic and pathological studies surgical correction in early life. Pediatrics. 1963;31:255-267.
Jha AK, Makhija N. Cor Triatriatum: A Review. Semin Cardiothorac Vasc Anesth. 2017;21(2):178-185. doi:10.1177/1089253216680495
Rudienė V, Hjortshøj CMS, Glaveckaitė S, et al. Cor triatriatum sinistrum diagnosed in the adulthood: a systematic review. Heart. 2019;105(15):1197-1202. doi:10.1136/heartjnl-2019-314714
Saxena P, Burkhart HM, Schaff HV, Daly R, Joyce LD, Dearani JA. Surgical repair of cor triatriatum sinister: the Mayo Clinic 50-year experience. Ann Thorac Surg. 2014;97(5):1659-1663. doi:10.1016/j.athoracsur.2013.12.046
Stout KK, Daniels CJ, Aboulhosn JA, et al. 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 [published correction appears in J Am Coll Cardiol. 2019 May 14;73(18):2361]. J Am Coll Cardiol. 2019;73(12):1494-1563. doi:10.1016/j.jacc.2018.08.1028
Yaroglu Kazanci S, Emani S, McElhinney DB. Outcome after repair of cor triatriatum. Am J Cardiol. 2012;109(3):412-416. doi:10.1016/j.amjcard.2011.09.029
47 min

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