Qureshi Ataul, Awuor Stephen, Martinez Mathew
Lehigh Valley Health Network, Allentown, PA, USA.
Lehigh Valley Health Network, Allentown, PA, USA.
Heart Lung Circ. 2015 Jan;24(1):e7-e10. doi: 10.1016/j.hlc.2014.08.003. Epub 2014 Aug 23.
Subaortic stenosis (SAS) is a rare entity in adults with an unclear aetiology and variable clinical presentations and outcomes. SAS typically tends to occur in the first decade of life either as an isolated lesion or in association with other congenital heart diseases. The clinical presentation of SAS can closely mimic hypertrophic cardiomyopathy (HCM) with obstructive physiology. We present two cases of SAS in adults that were initially presumed to be HCM. The patients were in their late forties and were referred to HCM clinic for further evaluation. Careful review of the transthoracic echocardiogram was indicative for the presence of possible subaortic membrane. These patients underwent subsequent imaging studies that completed the diagnosis for SAS and thereafter had successful surgical resection of the subaortic membrane. Subaortic stenosis remains a rare and clinically challenging diagnosis in the adult population. Often a combination of imaging modalities is needed to distinguish SAS from HCM with obstruction. It is critical to make the appropriate diagnosis as the treatment options are vastly different from the SAS and HCM with obstruction as well as the implications of a diagnosis of HCM with regards to risk of sudden death and family screening.
主动脉瓣下狭窄（SAS）在成人中是一种罕见疾病，其病因不明，临床表现和预后各异。SAS通常倾向于在生命的第一个十年出现，可为孤立性病变或与其他先天性心脏病相关。SAS的临床表现可与具有梗阻性生理学特征的肥厚型心肌病（HCM）极为相似。我们报告两例成人SAS病例，最初均被误诊为HCM。患者均为四十多岁，因进一步评估被转诊至HCM诊所。仔细回顾经胸超声心动图提示可能存在主动脉瓣下隔膜。这些患者随后接受了进一步的影像学检查，确诊为SAS，之后成功进行了主动脉瓣下隔膜切除术。主动脉瓣下狭窄在成人中仍然是一种罕见且具有临床挑战性的诊断。通常需要多种影像学检查手段相结合，以区分SAS与梗阻性HCM。做出正确诊断至关重要，因为SAS和梗阻性HCM的治疗选择截然不同，而且HCM诊断对猝死风险和家族筛查也有影响。
