Mohsen Amr, Rahman Faraaz, Ikram Sohail
University of Louisville, Department of Cardiology, 550 South Jackson Street, ACB, 3rd floor, Louisville, KY 40202 USA.
J Invasive Cardiol. 2013 Dec;25(12):E212-3.
Double-chambered right ventricle (DCRV) causing right ventricular outflow tract (RVOT) obstruction, is an uncommon cardiac disease especially when first diagnosed in adults. Diagnosis can be challenging when it is asymptomatic. We report a 40-year-old man who was referred to cardiology for work-up of a heart murmur that was found on a routine physical examination prior to obtaining a pilot's license. A transesophageal echocardiogram (TEE) revealed a DCRV and a small perimembranous ventricular septal defect (VSD) with left-to-right shunt and severe RVOT obstruction. Right heart catheterization showed an estimated gradient of 135 mm Hg across the two chambers due to subpulmonary stenosis. The pressure in the proximal right ventricular chamber was 160/25 mm Hg and 25/17 mm Hg in the distal chamber. Surgical correction was successfully performed. A postoperative TEE showed no residual gradients across the RVOT. DCRV has been reported as a rare disease in adults. It is difficult to diagnose DCRV using the transthoracic echocardiogram in adults. However, subcostal windows can offer better visualization of subpulmonary outflow tract compared to the parasternal and apical windows. TEE remains the main non-invasive modality for diagnosing DCRV in adults. Cardiac magnetic resonance imaging and invasive testing can provide further information to confirm the diagnosis and guide therapeutic decisions.
双腔右心室（DCRV）导致右心室流出道（RVOT）梗阻，是一种罕见的心脏病，尤其是首次在成人中诊断时。当它无症状时，诊断可能具有挑战性。我们报告一名40岁男性，他因在获得飞行员执照前的常规体检中发现心脏杂音而被转诊至心脏病科。经食管超声心动图（TEE）显示为双腔右心室和一个小的膜周部室间隔缺损（VSD），伴有左向右分流和严重的右心室流出道梗阻。右心导管检查显示由于肺下狭窄，两个腔室之间的估计压差为135 mmHg。近端右心室腔压力为160/25 mmHg，远端腔室压力为25/17 mmHg。成功进行了手术矫正。术后TEE显示右心室流出道无残余压差。双腔右心室在成人中已被报道为罕见疾病。在成人中使用经胸超声心动图很难诊断双腔右心室。然而，与胸骨旁和心尖窗相比，肋下窗可以更好地显示肺下流出道。TEE仍然是成人诊断双腔右心室的主要非侵入性检查方法。心脏磁共振成像和侵入性检查可以提供进一步信息以确诊并指导治疗决策。
