Perioperative management of a patient with double orifice mitral valve with supramitral ring with subaortic membrane with ventricular septal defect and severe pulmonary hypertension: Report of a rare case

Double-orifice mitral valve (DOMV) is an unusual congenital anomaly characterized by a mitral valve with a single fibrous annulus with two orifices or rarely two orifices with two separate mitral annuli opening into the left ventricle. We present a first report of a patient with a DOMV with supramitral ring (SMR), subaortic membrane (SAM), a large ventricular septal defect (VSD) with more than 50% aortic override, and severe pulmonary arterial hypertrophy (PAH). This patient underwent excision of the SAM, and SMR, with closure of the VSD together under cardiopulmonary bypass (CPB). However postoperatively, the patient developed an irreversible fatal pulmonary hypertensive crisis (PHC), immediately after transferring the patient to the cardiac intensive care unit from the operating room (OR). The PHC was refractory to intravenous and inhaled milrinone and nitroglycerine and intravenous adrenaline, dobutamine, norepinephrine, vasopressin, patent foramen oval (PFO), and CPB support. The management of DOMV and perioperative pulmonary hypertension is discussed.

Flail Subaortic Membrane Mimicking Left Ventricular Outflow Tract Obstruction in Hypertrophic Cardiomyopathy

A subaortic membrane is an uncommon cause for left ventricular outflow tract obstruction. Hypertrophic cardiomyopathy with dynamic left ventricular outflow tract obstruction would mask the presence of the subaortic membrane on transthoracic echocardiography and cause a false diagnosis. We report a patient with subaortic stenosis due to flail subaortic membrane misdiagnosed as obstructive hypertrophic cardiomyopathy on transthoracic echocardiography, identified on transesophageal echocardiography and cardiac catheterization.

A Case of Supraaortic Ridge and Subaortic Membrane Manifestated as Aortic Regurgitation

Supraaortic stenosis is a localized or diffuse congenital narrowing of the ascending aorta at the levels of superior margin of the sinus of Valsalva, just above coronary arteries. Hemodynamically, it is a usually manifestated as aortic stenosis especially when it combined with subaortic membrane. And there is no case report of supraaortic stenosis combined with subaortic membrane which has aortic valvular reurgitation due to secondary change of dilated sinus of Valsalva by supraaortic ridge. We have experienced a case of aortic regurgitation associated with supraaortic ridge combined with subvalvular aortic membrane.