Papillary muscle rupture is a fatal complication with a high operative mortality rate of 13–40%. Most patients experience cardiogenic shock and hypoxia due to pulmonary edema caused by severe mitral regurgitation. Although preoperative stabilization using a mechanical assist device potentially improves surgical outcomes, an appropriate strategy has not yet been established. ECPELLA is a useful mechanical circulatory support (MCS) strategy, combining venoarterial extracorporeal membrane oxygenation and Impella®, and potentially increases cardiac output and tissue perfusion and improves left ventricular distension and oxygenation in patients with refractory cardiogenic shock. Herein, we present our surgical technique involving the successful management of ECPELLA during surgery in patients with papillary muscle rupture.

We report about a 78-year-old woman with severe aortic valve stenosis (AS). She had heavily calcified and thickened aortic valve leaflets with black pigmentation. After excision of the aortic valve leaflets, biological aortic valve replacement (AVR) was performed. Alkaptonuria was diagnosed and confirmed based on increased urinary excretion of homogentisic acid. She has had no cardiovascular system-related symptoms for 3 years and no evidence of structural valve deterioration. Data on the long-term outcomes of AVR associated with structural valve deterioration that could regulate prosthetic valve selection in alkaptonuria are limited. Therefore, further research on the natural evolution of AS and the rate of structural valve deterioration after AVR is needed to provide an optimal prosthesis for these patients.