Senior Faculty Baylor College of Medicine Baylor College of Medicine BELLAIRE, Texas, United States
Background: Adults with congenital heart disease have complex cardiac anatomy which renders mechanical circulatory support challenging. Due to frequent loss to followup and severe physiologic disturbances from their underlying disease, they are at high risk for presentation to the emergency room in extremis with high subsequent mortality.
Methods: Case report of 42 yo male with d-transposition of the great arteries (d-tGA) palliated with a Mustard atrial switch following out-of-hospital cardiac arrest (OHCA). The patient had long standing RV dysfunction and suffered a witnessed arrest at work with bystander CPR provided by colleagues. At the initial hospital, he had ROSC which was lost with subsequent VF and underwent coronary angiography with no coronary disease and recurrent VF. He was then transferred to the Texas Children's Hospital (TCH) Adult Congenital Heart Disease (ACHD) program.
Outcome: At TCH, the patient was quickly cannulated onto VA ECMO despite complex venous drainage but his early hospital course was complicated by multi-system organ failure and persistent cardiogenic shock (CS) and coma. He was transitioned to percutaenous ventricular assist device (pVAD) to permit ECMO weaning awaiting neurologic prognostication. Over the course of 5 weeks, he awoke and was following commands, had renal recovery, was extubated but could not separate from pVAD due to persistent CS. He therefore underwent placement of a durable VAD as a potential bridge to transplantation vs destination therapy depending on prognosis. The patient has had ongoing functional recovery and is now being considered for cardiac transplant.
Conclusion: This case report underscores the unique nature of ACHD OHCA, often due to CHD-related heart failure or arrhythmia rather than acute coronary syndrome. Mechanical circulatory support of CS is challenging given the unique anatomy but is feasible with excellent outcomes at specialty centers with significant ACHD experience.
