90. Case Report: Atrioesophageal Fistula (AEF) Formation after Pulmonary Vein Isolation – Thomas Jefferson University Hospital

CardioNerds (Amit Goyal) joins Thomas Jefferson cardiology fellows (Jay Kloo, Preya Simlote and Sean Dikdan – host of the Med Lit Review podcast) for some amazing craft beer from Independence Beer Garden in Philadelphia! They discuss a fascinating case of atrioesophageal fistula (AEF) formation after pulmonary vein isolation (PVI). Dr. Daniel Frisch provides the E-CPR and program director Dr. Gregary Marhefka provides a message for applicants. Johns Hopkins internal medicine resident Colin Blumenthal with mentorship from University of Maryland cardiology fellow Karan Desai.  

Jump to: Patient summaryCase mediaCase teachingReferences

CardioNerds (Amit Goyal) joins Thomas Jefferson cardiology fellows (Jay Kloo, Preya Simlote and Sean Dikdan - host of the Med Lit Review podcast) for some amazing craft beer from Independence Beer Garden in Philadelphia! They discuss a fascinating case of atrioesophageal fistula (AEF) formation after pulmonary vein isolation (PVI). Dr. Daniel Frisch provides the E-CPR and program director Dr. Gregary Marhefka provides a message for applicants. Johns Hopkins internal medicine resident Colin Blumenthal with mentorship from University of Maryland cardiology fellow Karan Desai.
Episode graphic by Dr. Carine Hamo

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

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

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

Cardionerds Cardiology Podcast Presents CardioNerds Case Report Series

Patient Summary

A mid 60s male with relevant PMHx of paroxysmal atrial fibrillation presents to the ED with altered mental status after one week of mild chest pain. Given the long history of atrial fibrillation refractory to rate and rhythm control with diltiazem and flecainide, he underwent a pulmonary vein isolation 21 days prior to arrival. In the ED, T 39.4 and patient had a witnessed seizure requiring intubation for airway protection. Signs of hypoperfusion on labs, but white blood cell count not elevated. LP negative, but blood cultures positive for strep agalactiae. CT head with multiple tiny foci of intravascular air throughout the brain with MRI consistent with multiple areas of acute infarction. CTA of chest then obtained, which was notable for a small focus of air tracking along the esophagus. Taken together, findings most c/w atrial esophageal fistula causing sepsis and air emboli. Patient underwent surgical repair of left atrium and esophagus with a good outcome. 


Case Media

A. ECG: Normal sinus rhythm HR 105 bpm
B. CXR
C. CT head: Multiple tiny foci of air throughout bilateral cerebral hemispheres. Appearance is most suggestive of intravascular air, although it is unclear if it is venous, arterial or both.
D. MRI: 1. Restricted diffusion in bilateral cortical watershed zones, as well as in the posterior medial left cerebellar hemisphere, most consistent with recent infarctions.
E. CT Chest: A small focus of air tracking along the left mainstem bronchus anterior to the esophagus, may represent a small amount of pneumomediastinum versus air in an outpouching of the esophagus. No air tracking more cranially along the mediastinal soft tissues. No definite soft tissue defect in the esophagus.
F. Surgical repair of LA & Esophagus


Episode Schematics & Teaching

Coming soon!


The CardioNerds 5! – 5 major takeaways from the #CNCR case

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References

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CardioNerds Case Reports: Recruitment Edition Series Production Team

89. Case Report: Cardiac Arrest associated with Mitral Valve Prolapse with Mitral Annular Disjunction – Oregon Health & Science University

CardioNerds (Amit Goyal & Daniel Ambinder) join Oregon Health & Science University cardiology fellows (Miranda Merrill, Timothy Simpson, Kris Kumar, and Stacey Howell) for a riverside chat at the Portland waterfront! They discuss a case of cardiac arrest associated with mitral valve prolapse (MVP) with mitral annular disjunction (MAD). Dr. Punag Divanji provides the E-CPR and program director Dr. Hind Rahmouni provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident, Eunice Dugan, with mentorship from University of Maryland cardiology fellow Karan Desai.  

Jump to: Patient summaryCase mediaCase teachingReferences

CardioNerds (Amit Goyal & Daniel Ambinder) join Oregon Health & Science University cardiology fellows (Miranda Merrill, Timothy Simpson, Kris Kumar, and Stacey Howell) for a riverside chat at the Portland waterfront!  They discuss a case of cardiac arrest associated with mitral valve prolapse (MVP) with mitral annular disjunction (MAD). Dr. Punag Divanji provides the E-CPR and program director Dr. Hind Rahmouni provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident, Eunice Dugan, with mentorship from University of Maryland cardiology fellow Karan Desai.
Episode graphic by Dr. Carine Hamo

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

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

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

Cardionerds Cardiology Podcast Presents CardioNerds Case Report Series

Patient Summary

Coming soon!


Case Media

A. CXR
B. Rhythm Strips – ventricular fibrillation
C. ECG: 1st degree AVB (PR ~ 215), borderline RAD, Qtc ~460 msec, slight ant. convexity with inferior terminal T wave
D: TTE
E: TTE with Pickelhaube Spike seen in mitral valve prolapse
F-G: Cardiac MRI

TTE 1
TTE 2
TTE 3
Cardiac MRI

Episode Schematics & Teaching

Coming soon!


The CardioNerds 5! – 5 major takeaways from the #CNCR case

Coming soon!


References

Coming soon!


CardioNerds Case Reports: Recruitment Edition Series Production Team

87. Case Report: Giant Coronary Aneurysm Presenting with Heart Failure – University of Hawaii

Aloha! CardioNerds (Amit Goyal & Karan Desai)  join University of Hawaii cardiology fellows (Isaac Mizrahi, Nath Limpruttidham, Nishant Trivedi, and Shana Greif) for some shaved iced on the Big Island’s north shore! They discuss a fascinating case of a patient presenting with decompensated heart failure found to have a giant coronary aneurysm. Program director Dr. Dipanjan Banerjee provides the E-CPR as well as a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Tommy Das with mentorship from University of Maryland cardiology fellow Karan Desai.  

Jump to: Patient summaryCase mediaCase teachingReferences

Aloha! CardioNerds (Amit Goyal & Karan Desai)  join University of Hawaii cardiology fellows (Isaac Mizrahi, Nath Limpruttidham, Nishant Trivedi, and Shana Greif) for some shaved iced on the Big Island's north shore! They discuss a fascinating case of a patient presenting with decompensated heart failure found to have a giant coronary aneurysm. Program director Dr. Dipanjan Banerjee provides the E-CPR as well as a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Tommy Das with mentorship from University of Maryland cardiology fellow Karan Desai.
Episode graphic by Dr. Carine Hamo

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

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

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

Cardionerds Cardiology Podcast Presents CardioNerds Case Report Series

Patient Summary

Coming soon!


Case Media

A. CXR
B. ECG: atrial fibrillation with RVR, left axis deviation, poor r wave progression
C. Wide complex tachycardia
D. CT chest demonstrating giant aneurysm

TTE
Coronary Angiography

Episode Schematics & Teaching

Coming soon!


The CardioNerds 5! – 5 major takeaways from the #CNCR case

Coming soon!


References

  1. Thibodeau, J. T., & Drazner, M. H. (2018). The Role of the Clinical Examination in Patients With Heart Failure. JACC. Heart failure, 6(7), 543–551.  
  2. Abou Sherif, S., Ozden Tok, O., Taşköylü, Ö., et al. (2017). Coronary Artery Aneurysms: A Review of the Epidemiology, Pathophysiology, Diagnosis, and Treatment. Frontiers in cardiovascular medicine, 4, 24. 
  3. Kawsara, A., Núñez Gil, I. J., Alqahtani, F., et al. (2018). Management of Coronary Artery Aneurysms. JACC. Cardiovascular interventions, 11(13), 1211–1223.  
  4. Newburger, J. W., Takahashi, M., & Burns, J. C. (2016). Kawasaki Disease. Journal of the American College of Cardiology, 67(14), 1738–1749.  
  5. McCrindle, B. W., Rowley, A. H., Newburger, J. W., et al. (2017). Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. Circulation, 135(17), e927–e999.  

CardioNerds Case Reports: Recruitment Edition Series Production Team

86. Case Report: Histoplasmosis Pericarditis Complicated by Cardiac Tamponade – Georgetown University

CardioNerds (Amit Goyal & Daniel Ambinder) join Georgetown University/Washington Hospital Center cardiology fellows (Nitin Malik, AJ Grant, and Tsion Aberra) for some fresh Maryland blue crab cakes at the Georgetown waterfront in Washington, DC. They discuss a rare case of histoplasmosis pericarditis complicated by cardiac tamponade. Dr. Patrick Bering provides the E-CPR and program director Dr. Gaby Weissman provides a message for applicants. Johns Hopkins internal medicine resident Colin Blumenthal with mentorship from University of Maryland cardiology fellow Karan Desai.  

Jump to: Patient summaryCase mediaCase teachingReferences

CardioNerds (Amit Goyal & Daniel Ambinder) join Georgetown University/Washington Hospital Center cardiology fellows (Nitin Malik, AJ Grant, and Tsion Aberra) for some fresh Maryland blue crab cakes at the Georgetown waterfront in Washington, DC. They discuss a rare case of histoplasmosis pericarditis complicated by cardiac tamponade. Dr. Patrick Bering provides the E-CPR and program director Dr. Gaby Weissman provides a message for applicants. Johns Hopkins internal medicine resident Colin Blumenthal with mentorship from University of Maryland cardiology fellow Karan Desai.
Episode graphic by Dr. Carine Hamo

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

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

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

Cardionerds Cardiology Podcast Presents CardioNerds Case Report Series

Patient Summary

Coming soon


Case Media

A. Left: Admission chest x-ray (PA film), which was overall unremarkable. Right: Chest x-ray from hospital day 12 – which revealed pulmonary edema with bilateral perihilar haziness, increased prominence of pulmonary vascularity, and small-moderate bilateral pleural effusions. Note increased size of cardiac silhouette. At the corresponding time, pericardial effusion (without tamponade) had been diagnosed.
B. EKG: Sinus tachycardia and low-voltage QRS complexes.
C. CT abdomen/pelvis on hospital day 14. Free air noted within the abdomen (left). Moderate pericardial effusion also incidentally appreciated (right).
D. Pulse-Wave Doppler of mitral inflow. Flow variation is present, but variation is less than <30%.
E. (A) Small bowel resection showing focal mucosal ulceration, serositis, and formation of a granuloma. (B) Transmural inflammation seen on small bowel resection. (C) Pathology of ileocecectomy showing focal histoplasmosis characterized by intracytoplasmic yeast-like forms (black circles)

Parasternal short axis view on echocardiogram showing a moderate pericardial effusion without diastolic septal flattening.
Apical view showing profound tachycardia but without chamber collapse. Ejection fraction was moderately reduced.
Parasternal short axis view on echocardiogram showing a moderate pericardial effusion with intermittent septal flattening.
Apical view showing early diastolic RV chamber collapse. 

Episode Schematics & Teaching

Coming soon!


The CardioNerds 5! – 5 major takeaways from the #CNCR case

Coming soon!


References

Coming soon!


CardioNerds Case Reports: Recruitment Edition Series Production Team

84. Case Report: Hypertrophic Cardiomyopathy with Superimposed Stress Cardiomyopathy – Brown University

CardioNerds (Amit Goyal & Daniel Ambinder) join Brown University cardiology fellows (Greg Salber, Vrinda Trivedi, and Esseim Sharma) for a gorgeous coastal boat ride in Providence, RI. They discuss an educational case of hypertrophic cardiomyopathy with superimposed stress cardiomyopathy. Dr. Katharine French provides the E-CPR and program director Dr. Raymond Russell provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Evelyn Song with mentorship from University of Maryland cardiology fellow Karan Desai.  

Jump to: Patient summaryCase mediaCase teachingReferences

CardioNerds (Amit Goyal & Daniel Ambinder) join Brown University cardiology fellows (Greg Salber, Vrinda Trivedi, and Esseim Sharma) for a gorgeous coastal boat ride in Providence, RI. They discuss an educational case of hypertrophic cardiomyopathy with superimposed stress cardiomyopathy. Dr. Katharine French provides the E-CPR and program director Dr. Raymond Russell provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Evelyn Song with mentorship from University of Maryland cardiology fellow Karan Desai.
Episode graphic by Dr. Carine Hamo

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

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

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

Cardionerds Cardiology Podcast Presents CardioNerds Case Report Series

Patient Summary

Coming soon!


Case Media

A. ECG 2 weeks prior to current presentation
B. Current ECG
C. CXR
D. M mode though the mitral valve demonstrating systolic anterior motion of the mitral valve
E. LVOT CW Doppler tracings with a peak velocity ~ 5 m/s

Coronary angiography – 1
Coronary angiography – 2
TTE – 1
TTE – 2
TTE – 3
TTE – 4
Cardiac MRI

Episode Schematics & Teaching


The CardioNerds 5! – 5 major takeaways from the #CNCR case

Coming soon!


References

Coming soon!


CardioNerds Case Reports: Recruitment Edition Series Production Team

82. Case Report: L-TGA with Double Inlet LV post-Fontan complicated by VF Arrest – Stanford University

CardioNerds (Amit Goyal & Daniel Ambinder) join Stanford cardiology fellows (Pablo Sanchez, Natalie Tapaskar, Jimmy Tooley) for tacos while enjoying the sunshine on the Stanford Oval! They recount the story of a man with adult congenital heart disease (ACHD): L-TGA (levo-transposed great arteries) with double inlet LV post-Fontan complicated by VF arrest. Dr. Christiane Haeffele provides the E-CPR and program director Dr. Joshua Knowles provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Evelyn Song with mentorship from University of Maryland cardiology fellow Karan Desai and Cleveland clinic cardiology fellow Josh Saef.

CardioNerds (Amit Goyal & Daniel Ambinder) join Stanford cardiology fellows (Pablo Sanchez, Natalie Tapaskar, Jimmy Tooley) for Tacos while enjoying the sunshine on the Stanford Oval! They discuss a meaningful case of Adult Congenital Heart Disease (double inlet LV with levo-transposed great arteries (L-TGA) s/p single ventricle palliation to a Fontan (fenestration now closed) complicated by VF arrest. Dr. Christiane Haeffele provides the E-CPR and program director Dr. Joshua Knowles provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Evelyn Song with mentorship from University of Maryland cardiology fellow Karan Desai.

Jump to: Patient summaryCase mediaCase teachingReferences


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

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

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

Cardionerds Cardiology Podcast Presents CardioNerds Case Report Series

Patient Summary

A man in his mid-30s with past medical history notable for L-TGA (levo-transposed great arteries) with double inlet LV s/p Fontan palliation was playing golf when he suddenly collapsed.  EMS arrived after three minutes of bystander CPR. An AED indicated the patient had suffered a VF arrest. ROSC was achieved after 1 round of Epi and 1 shock delivered. He was intubated and started on targeted temperature management protocol. Home medications were  notable for digoxin 0.25mg daily, sotalol 120mg BID, and warfarin 5mg daily. Initial labs were notable for Na 127, K 5.4, Cr 1.0 (unknown baseline), INR 4.5, Lactate 4.6, Troponin-I 0.532, VBG 7.06/61, and random Digoxin level 2.7.  EKG showed AV sequential pacing at a rate of 70 bpm. QTc prolonged at 571ms. No ischemic ST changes. Device interrogation showed sustained VT for 5 minutes prior to external shock. No internal shock was delivered. He was initially stabilized and his acidosis and hyperkalemia were corrected. Course was complicated by hemoptysis due to alveolar hemorrhagic and he was given concentrated prothrombin complex to reverse his coagulopathy. He eventually stabilized, and a formal TTE was obtained which showed a hypoplastic RV, single dilated LV with an akinetic posterior wall and hypokinetic lateral wall, all similar to his prior TTE in 2019. No obstruction noted at the IVC/Fontan anastomotic site. Coronary angiogram performed after his kidney function improved also did not show any significant obstructions or coronary anomalies. After multidisciplinary discussion, his VF arrest was attributed to a combination of prior ventricular fibrosis/scar, suspected digoxin toxicity, sotalol, dehydration, and renal failure. He had a subcutaneous ICD lead placed and was ultimately discharged home. 


Case Media

A. CXR
B. ECG


Episode Schematics & Teaching

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The CardioNerds 5! – 5 major takeaways from the #CNCR case

  1. What’s Transposition of the Great Arteries (TGA)? 
    • TGA is defined by a nontraditional ventricle-arterial relationship so that the aorta arises from the morphological RV and the pulmonary artery (PA) arises from the morphological LV 
    • There are two types of TGA, L or levo and D or dextro. 
      • D (rightward)-TGA: Systemic venous return flows into RA -> RV -> delivered to systemic circulation via Aorta, bypassing the lungs. The pulmonary veins flow into the LA -> LV-> delivered to the pulmonary circulation via the PA. The result is two parallel systems that fail to deliver oxygenated blood to the systemic circulation. This is not compatible with life unless another defect such as ASD, VSD, or PDA is present (or created) to allow mixing of deoxygenated and oxygenated blood. Patients with D-TGA usually require arterial switch procedures within 1 month of life. A simplified way to understand this is to say that the great vessels are malpositioned, leaving patients with two parallel circulation. 
      • L (leftward)-TGA: Deoxygenated blood flows into RA -> morphologic LV in the traditional RV position -> PA -> Lungs->  LA -> morphologic RV in the traditional LV position -> aorta to deliver oxygenated blood to the body. A simplified way to understand this is to say that the ventricles are malpositioned, leaving patients with two circulations in series (normal) with the pumps in the wrong places. There’s no cyanosis at birth and patients may be completely asymptomatic for years. This is more rare than D-TGA and only occurs in ~7 per 100,000 births.  
        • The problem in L-TGA is that the morphologic RV, being the systemic ventricle, is not meant to withstand systemic afterload and can result in significant TR and ventricular dysfunction. Additionally, the majority of the cases (80%) are associated with an additional heart defect, such as VSD, pulmonary stenosis, RV hypoplasia, or DILV as in our case.   
  2. What’s Double Inlet Left Ventricle (DILV)? 
    • In DILV, both atrio-ventricular valves (mitral and tricuspid) lead into the LV and a large VSD is present to connect the LV and RV. DILV is very rare (~5 in 100,000 births). It coexists with L-TGA in about 65% of the cases. 
    • DILV leads to mixing of oxygenated and deoxygenated blood in the LV, resulting in inadequately oxygenated blood entering the systemic circulation and also over-circulation to the pulmonary system (increased Qp:Qs), resulting in LV congestion and heart failure over time.   
  3. What’s the Fontan procedure? 
    • The Fontan procedure is typically performed as a palliation procedure to direct flow of systemic venous return to the lungs without passing through a subpulmonic ventricle. It’s typically performed in patients with complex congenital heart disease with a single functioning ventricle such as tricuspid atresia, pulmonary atresia, hypoplastic left heart syndrome, and DILV. In these conditions, intracardiac mixing of oxygenated and deoxygenated blood leads to cyanosis and ventricular volume overload without surgical intervention. 
    • The early variation of the Fontan procedure connected the pulmonary arteries to the RA. However, this led to RA dilation and loss of contractility, resulting in decreased pulmonary blood flow and increased risk for thrombus formation and arrhythmias. More modern Fontan palliation procedures connect both vena cavae directly to pulmonary arteries (via an intra- or extra-cardiac conduit), bypassing the RA and RV completely. 
    • Elevated pulmonary pressure is an absolute contraindication for the Fontan procedure since there’s no ventricular contraction to pump blood through the lungs (it relies on passive flow). Therefore, the cavopulmonary Fontan circulation can’t be created at birth given the normal high pulmonary vascular resistance in newborns.  
    • The modern Fontan palliation sequence is performed in a staged fashion to allow the patient’s body to adapt to the different hemodynamic states and reduce overall surgical morbidity and mortality. 
      1. Stage 1: systemic-pulmonary shunt; performed during neonatal period 
      • An artificial shunt is placed between a major systemic central vessel, usually subclavian artery, and proximal pulmonary artery. The goal of this step is to provide dedicated pulmonary blood flow to allow adequate oxygen delivery to tissues and pulmonary arterial growth.  
      1. Stage 2: superior cavopulmonary connection (Glenn procedure); performed between 4-12 months 
      • Anastomosis is made between the SVC and proximal right PA. The previous systemic-pulmonary shunt is usually ligated. This allows priming of the pulmonary vasculature over time before completion of the Fontan circulation.  
      1. Stage 3: completion of the Fontan circulation; performed between ages 1-5. 
      • Different surgical techniques are used, but the common endpoint is  IVC anastomosis to the right PA. 
    • After the Fontan procedure, cardiac output is completely dependent on passive flow into the lungs. LV preload is central to Fontan physiology.  Dehydration, an increase in pulmonary vascular resistance and/or worsening LV stiffness (and hence LV filling) can lead to decreased cardiac output.   
  4. What are some complications associated with the Fontan procedure? 
    • RA dilatation was very common with the classic Fontan where atriopulmonary instead of cavopulmonary Fontan circulation was created, the RA is exposed to elevated pressure, leading to RA dilatation, thrombus formation, and arrhythmias. 
    • Ventricular failure usually develops after the first decade following completetion of the Fontan palliation. Patients will typically develop the classic symptoms of heart failure due to either HFrEF or HFpEF. Potential contributors to CHF include atrial tachycardia, valvular regurgitation, and volume-loading shunts. 
    • Atrioventricular valve (AVV) regurgitation can develop insidiously after the Fontan procedure and is a significant risk factor for long-term mortality post Fontan. AVV regurgitation can lead to volume overload, ventricular dilation, reduced ventricular contractility, and increased postcapillary and central venous pressures, compromising the Fontan circulation. Medical management of patients with AVV regurgitation post Fontan include diuretic therapy and afterload reduction. 
    • Protein-losing enteropathy (PLE) is the abnormal loss of serum proteins into the intestinal lumen and occurs in 5-12% of patients after a Fontan palliation. Its pathophysiology is incompletely understood, but thought to be due to chronic venous congestion-induced lymphatic insufficiency. PLE may lead to edema/ascites, growth failure, coagulopathy, decreased bone density, and lymphopenia. 
    • Plastic Bronchitis (PB) occurs in <5% of patients with Fontan and is characterized by production of thick, tenacious casts within the airway lumen. Similar to PLE, it’s believed to be due to spillage of protein-rich lymph through lymphatic-to-bronchial communications. Medical management includes diuretics, ARBs, and pulmonary vasodilators. 
    • Fontan associated liver disease (FALD) is a common complication after Fontan due to increased venous pressure, lymphatic overflow, and hepatic congestion. FALD spans the spectrum from liver fibrosis to cardiac cirrhosis and hepatocellular carcinoma. All patients with Fontan circulation should be counseled on avoiding hepatotoxins and undergo regular hepatic screening.   
  5. What are the common long-term complications of congenital heart disease (CHD)? 
    • With advances in cardiology and cardiac surgery, 85% of neonates with CHD survive into adult life. The four most common complications seen in ACHD include Heart failure, Endocarditis, Arrhythmias, and Pulmonary Hypertension, or H.E.A.P 
    • Heart failure is a major cause of morbidity and mortality in ACHD patients. The pathophysiology of HF in ACHD is multifactorial and includes chronic pressure/volume ventricular loading, persistent arrhythmias, longstanding cyanosis, myocardial fibrosis, or pulmonary vascular disease. Medical management typically includes diuretics and ARBs; however, guidelines for treatment of HF in ACHD patients are lacking because all the trials excluded ACHD patients. Ultimately heart transplantation should be considered in those with refractory HF. Given high prevalence of pulmonary hypertension and RV dysfunction, the use of VADs is limited in this population. 
    • Endocarditis is more prevalent in patients with ACHD compared to the general population. The increased risk of IE in this population is related to both the underlying congenital defect and previous surgical interventions with reconstructed anatomy. The most common site for IE is the LV outflow tract, regardless of previous surgery. IE should be suspected in all ACHD patients presenting with fever, night sweats, or new manifestation of HF. Patients with cyanotic heart disease, history of IE, prosthetic valve, or prosthetic material/devices should receive prophylactic antibiotics prior to any invasive dental procedures. 
    • Arrhythmias account for the majority of ED visits in ACHD patients. For patients older than 20 years, >50% will have atrial tachyarrhythmias. Other common arrhythmias include AV node disease, PVCs, and NSVTs. Sustained ventricular arrhythmias, typically due to prior ventriculostomy scars or ventricular fibrosis, is the most common cause of SCD in the ACHD population. Additionally, pacemaker implantation in ACHD patients requires thorough understanding of underlying anatomy. At times, epicardial pacing is needed (i.e. Fontan patients) due to inability to access cardiac chambers. Pulmonary hypertension (PH) is defined as mean PAP >/= 25 mmHg, similar as in the general population. About 5-10% of ACHD patients have PH and these patients are at a higher risk for hospitalization and death. The clinical classification of ACHD-related PH has four main clinical groups: Eisenmenger’s syndrome, PAH associated with systemic-to-pulmonary shunts, PAH with small defects, and PAH after corrective cardiac surgery.  

References

  1. Warnes C. A. (2006). Transposition of the great arteries. Circulation, 114(24), 2699–2709.  
  2. Ministeri, M., Alonso-Gonzalez, R., Swan, L., & Dimopoulos, K. (2016). Common long-term complications of adult congenital heart disease: avoid falling in a H.E.A.P. Expert review of cardiovascular therapy, 14(4), 445–462.  
  3. Rychik, J., et.al. American Heart Association Council on Cardiovascular Disease in the Young and Council on Cardiovascular and Stroke Nursing (2019). Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association. Circulation, CIR0000000000000696. Advance online publication.  
  4. Fredenburg, T. B., Johnson, T. R., & Cohen, M. D. (2011). The Fontan procedure: anatomy, complications, and manifestations of failure. Radiographics : a review publication of the Radiological Society of North America, Inc, 31(2), 453–463. 

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