Shahab Hunaina, Baqi Abdul, Askari Yusra, Aqeel Qurrat Ul Ain, Khandwala Kumail
Cardiology, Aga Khan University Hospital, Karachi, PAK.
Medicine, Aga Khan University Hospital, Karachi, PAK.
Cureus. 2019 Oct 6;11(10):e5850. doi: 10.7759/cureus.5850.
Subaortic membrane (SAM) is a discrete fibromuscular structure which causes left ventricular outflow tract obstruction and leads to the symptomatology of valvular aortic stenosis. It is known to be associated with other congenital cardiac defects in around 30% of cases. However, it has not been associated with chronic pulmonary thromboembolism in the past. We present a case of a middle-aged Pakistani man who presented with dyspnea and hemoptysis. He was found to have a SAM and severe pulmonary hypertension on transthoracic echocardiogram. A coronary angiogram revealed non-obstructive left main coronary artery disease. A computed tomography (CT) scan chest was done to evaluate the cause of severe pulmonary hypertension unexplained by SAM which revealed chronic pulmonary thromboembolism. Surgical resection was deferred due to high risk. Hence, he was kept on anticoagulation for pulmonary thromboembolism, and aspirin and a statin for non-obstructive coronary artery disease. Over the course of two months, his symptoms improved. This case highlights the importance of evaluating different causes of pulmonary hypertension in patients with SAM.
主动脉瓣下隔膜(SAM)是一种离散的纤维肌肉结构,可导致左心室流出道梗阻,并引发瓣膜性主动脉狭窄的症状。已知约30%的病例与其他先天性心脏缺陷有关。然而,过去它与慢性肺血栓栓塞症并无关联。我们报告一例中年巴基斯坦男性病例,该患者表现为呼吸困难和咯血。经胸超声心动图检查发现他患有SAM和严重肺动脉高压。冠状动脉造影显示左主干冠状动脉疾病无阻塞。进行胸部计算机断层扫描(CT)以评估SAM无法解释的严重肺动脉高压的原因,结果显示为慢性肺血栓栓塞症。由于手术风险高,手术切除被推迟。因此,他接受了针对肺血栓栓塞症的抗凝治疗,以及针对非阻塞性冠状动脉疾病的阿司匹林和他汀类药物治疗。在两个月的病程中,他的症状有所改善。该病例强调了评估SAM患者肺动脉高压不同病因的重要性。