Multilevel obstruction of left ventricular outflow tract in Shone complex adult

摘要

Shone complex is a congenital cardiac malformation including a supravalvular mitral ring, parachute mitral valve, subaortic stenosis, and coarctation of the descending aorta. We encountered a patient with Shone anomaly without mitral involvement, who was successfully treated for severe and multilevel obstruction of the left ventricular outflow tract 44 years after a first operation. A 57-year-old woman with a history of surgical coarctation repair at 13-years old, presented with dyspnea on exertion. Transthoracic echocardiography showed an anomalous crescent-shaped membrane and a prominent septal bulge inducing flow turbulence, as well as severe bicuspid aortic stenosis (valve area 0.6 cm, mean gradient 60mmHg). There was no abnormality of the mitral valve. Cardiac computed tomography demonstrated a membrane obstructing the left ventricular outflow tract, located 7 mm under a bicuspid aortic valve and above a prominent septal bulge.