A63-07 Atrial Septostomy and Percutaneous Reverse Potts Shunt While on V-A ECMO as Salvage Therapies for Right Ventricular Failure Secondary to Severe Portopulmonary Hypertension: A Case Report
Abstract
Cardiogenic shock from right ventricular (RV) failure secondary to severe pulmonary hypertension carries high mortality. Treatment includes pulmonary vasodilators, volume removal, vasopressor and inotropic support, and consideration of mechanical circulatory support, though these therapies may prove inadequate. In patients with suprasystemic pulmonary arterial (PA) pressures despite maximally tolerated pulmonary vasodilators, procedures such as balloon atrial septostomy (BAS) and reverse Potts shunt (connection between the left main PA and descending aorta) have shown benefit, though experiences in adults and critically ill patients are limited.
A 33-year-old male with a history of liver failure requiring liver transplantation at age 6 complicated by portopulmonary hypertension on ambrisentan and tadalafil presented with fatigue and dyspnea. He was found to be in cardiogenic shock secondary to RV failure and was cannulated to venoarterial extracorporeal membrane oxygenation (V-A ECMO). Treatment with ambrisentan, inhaled nitric oxide, and IV epoprostenol was initiated, and Sotatercept 0.7mg/kg was given off-label. Attempts to wean V-A ECMO were complicated by hypotension and rising central venous pressure (CVP) requiring escalating vasopressors and inotropes. The patient was deemed ineligible for lung transplantation at multiple institutions. Given inability to wean from ECMO, BAS was performed. Hypotension persisted with subsequent ECMO weaning trials, however CVP did not…
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