Echocardiographic Evaluation During BiVAD Insertion with Trans-atrial Cannulation in a Patient with Shone's Complex

Shone's complex encompasses a spectrum of left-sided obstructive lesions, such as a parachute mitral valve, supravalvular mitral ring, left ventricular outflow tract obstruction, and coarctation of the aorta. This complex of layered left-sided obstructive lesions may ultimately precipitate progressive cardiomyopathy and pulmonary hypertension. In this case, a 16-year-old male with a history of incomplete Shone's complex (parachute mitral valve, bicuspid aortic valve with moderate aortic stenosis, and hypoplastic aortic arch with neonatal arch repair) presented to our institution in acute combined systolic and diastolic biventricular failure, requiring biventricular device placement as bridge to transplantation. Echocardiographic evaluation revealed small left-sided structures typical of incomplete Shone's complex and biventricular failure. Small left-sided structures and reduced left ventricular volume prohibitive of a ventricular cannulation resulted in the utilization of a trans-atrial inflow cannulation technique for the left ventricular assist device. This case highlights the importance of recognizing the potential contribution of sequential stenosis to the development of diastolic dysfunction, left atrial hypertension, and the progression towards pulmonary hypertension, ultimately leading to decompensation. Furthermore, intraoperative echocardiography allowed continuous monitoring of the integrity of the atrial septum around the site of the septectomy and left atrial inflow cannula to facilitate optimal left-sided drainage.