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

Abstract

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.

Neonatal Blues: Cyanosis and Failure to Thrive in a Newborn

“…We believe that this infant is the first reported neonate with a VSD, DOMV, and SMR to undergo successful surgical repair. A prior case of a child diagnosed at 4 years of age was complicated by severe pulmonary hypertension, leading to early postoperative mortality [8]. We are unaware of other cases with these three independently acyanotic lesions cumulatively resulting in cyanosis and heart failure during early infancy.…”

Perioperative Anesthetic Management for Surgical Repair of an Adult with Supracardiac Total Anomalous Pulmonary Venous Communication and Pulmonary Hypertension: A case report and Mini Review

“…It improves ventilation/perfusion matching, decreases the intrapulmonary shunt fraction, and often increases the systemic arterial saturation [39]. If the above measures fail in improving the clinical status then other options may be considered, such as atrial septostomy, Cavo-pulmonary diversion, pulmonary artery balloon counter pulsations, IABP, RVAD, ECMO, and in desperate situation, if facilities exist then plan for heart and lung transplant [26,40,41,42] (Table 1).…”

“…Whereas hypoxia, hypercarbia, and pulmonary embolism are other causes of PAH, and these can appear before, during, or after CPB[26,27,28]. The PHC has been reported to be associated with significant mortality.…”