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.

AHA Scientific Sessions 2025 took place November 7–10 in New Orleans, LA — one of the premier annual gatherings in cardiovascular science and education.

It was an incredible opportunity to connect with colleagues, hear cutting-edge research, and contribute to the ongoing conversations shaping the future of cardiovascular care.

We’re grateful to everyone who joined us in New Orleans and made this year’s meeting so impactful.

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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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
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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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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?

How should we approach the management of chronic right ventricular failure?

When should bioprosthetic valve obstruction be suspected and what is the differential for this? What is the initial workup?

What are the indications for management of valve obstruction? What are the primary management strategies?

What is the role of right heart catheterization in valvular obstructive shock?

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.

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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.

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.

US Cardiology Review is now the official journal of CardioNerds! Submit your manuscript here.

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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.

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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What should we know about atrial arrhythmias in a Fontan patient?

How should we manage atrial arrhythmias in a Fontan patient?

How should we approach catheter ablation in a Fontan patient?

How should we approach anticoagulation in Fontan patients?

What arrhythmias are associated with patients who are born with Ebstein’s anomaly and when should we pursue EP study and/or catheter ablation?

In which ACHD patients should we consider the use of Holter monitor when they are asymptomatic?

How should we approach ICD implant for primary sudden cardiac death prevention in patients who are born with Tetralogy of Fallot (ToF)?

What are the technical considerations that ACHD patients warrant when having an ICD/PPM implanted compared to the general population?

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/

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
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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)?

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.

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.

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/

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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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.

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.

CardioNerds Case Reports Page
CardioNerds Episode Page
CardioNerds Academy
Cardionerds Healy Honor Roll

CardioNerds Journal Club
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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.

CardioNerds (Amit Goyal and Daniel Ambinder), ACHD series co-chairs Dr. Dan Clark and Dr. Josh Saef, and ACHD FIT lead Dr. J.D. Serfas (Duke University) and Cardiology Fellow Dr. Victoria Thomas (Vanderbilt University) join ACHD experts Dr. Jamil Aboulhosn (Professor of Medicine at UCLA and the director of the Ahmanson/UCLA Adult Congenital Heart Disease Center) and Dr. Joanna Ghobrial, Medical and Interventional Director of the Adult Congenital Heart Disease Center at Cleveland Clinic. They discuss common ACHD pathologies that benefit from interventional cardiology procedures such as transcatheter pulmonic valve replacement (TPVR) and share new advancements in transcatheter approaches to correct sinus venosus defects. They end with a brief discussion on how to become an adult cardiology interventionalist that performs ACHD interventions. Episode notes were drafted by Dr. Victoria Thomas. Audio editing by CardioNerds Academy Intern, student doctor Akiva Rosenzveig. 

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

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

Disclosures: None

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1. When considering a patient for TPVR there are 3 types of landing zones for pulmonic valves in ACHD patients:   

2. What are some of the more severe complications to consider when talking to an ACHD patient about a TPVR?  

3. What are some of the hemodynamic measurements one would want to pay attention to in a patient with a Fontan heart?   

4. When considering closing a fenestration of a Fontan circuit, what are the measurements that one would want to consider? 

5. What are the technical considerations to consider when occluding a fenestration in a Fontan circuit?  

6. How does a transcatheter intervention help a sinus venosus defect? 

7. What are some of the concerns to consider when closing a sinus venosus defect via a transcatheter approach? 

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/