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.

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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

1. 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.
   
2. Heart failure can be a challenging presentation in ACHD patients due to a longstanding history of clinical compensation.
   
3. 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.
   
4. While durable LVAD implantation is possible in patients with systemic right ventricles, anatomic compatibility is paramount and poses ongoing challenges in their management.
   
5. 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

1. 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.
   
2. 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).
   
3. 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
   
4. 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
   
5. 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
   
6. 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.
   
7. Burchill LJ. Heart transplantation in adult congenital heart disease. Heart. 2016;102:1871–1877. doi: 10.1136/heartjnl-2015-309074.