Datt Vishnu, Khurana Priyanka, Aggarwal Saket, Mishra Smita, Sujith C N, Virmani Sanjula
Department of Anaesthesiology and Intensive Care and Cardiothoracic and Vascular Surgery and Cardiology, GB Pant Hospital (GIPMER) and Jaypee Hospital, New Delhi, India.
Department CTVS, GB pant Hospital (GIPMER), New Delhi, India.
Ann Card Anaesth. 2019 Apr-Jun;22(2):215-220. doi: 10.4103/aca.ACA_123_18.
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.
双孔二尖瓣（DOMV）是一种罕见的先天性异常，其特征为二尖瓣具有单个纤维环和两个孔口，或极少情况下为两个孔口且有两个独立的二尖瓣环开口于左心室。我们首次报告了一例患有双孔二尖瓣合并二尖瓣上环形结构（SMR）、主动脉瓣下隔膜（SAM）、大于50%主动脉骑跨的大型室间隔缺损（VSD）以及严重肺动脉高压（PAH）的患者。该患者在体外循环（CPB）下同时进行了主动脉瓣下隔膜和二尖瓣上环形结构切除术，并关闭了室间隔缺损。然而术后，患者从手术室（OR）转入心脏重症监护病房后立即出现了不可逆的致命性肺动脉高压危象（PHC）。该肺动脉高压危象对静脉注射和吸入米力农、硝酸甘油以及静脉注射肾上腺素、多巴酚丁胺、去甲肾上腺素、血管加压素、卵圆孔未闭（PFO）和体外循环支持均无效。本文讨论了双孔二尖瓣的处理及围手术期肺动脉高压的治疗。
