Massin M
Département Universitaire de Pédiatrie, CHR Citadelle, Liège, Belgique.
Ann Cardiol Angeiol (Paris). 1998 Oct;47(8):579-81.
The double-chambered right ventricle (DCRV) is a rare cardiac malformation, often associated with ventricular septal defect (VSD).
The authors describe a case of DCRV in a child who underwent surgical repair of perimembranous VSD at the age of 7 months because of systemic pulmonary artery hypertension and growth retardation. At this time, no pressure gradient was measured within the right ventricle and no muscle proliferation was observed on echocardiography or right ventriculography. Postoperative follow-up revealed hypertrophy of the moderator band accompanied by progressive development of a right midventricular pressure gradient, which reached 60 mm Hg at the age of 3 1/2 years. The child was operated after invasive confirmation of the diagnosis of DCRV and the haemodynamic data. The child is now 6 years old and no restenosis has been observed.
DCRV is usually associated with VSD, but the course of the two diseases can be dissociated. The diagnosis of DCRV must be considered in any child with a history of VSD, even after surgical or spontaneous closure, when clinical examination reveals a systolic ejection murmur.
双腔右心室（DCRV）是一种罕见的心脏畸形，常与室间隔缺损（VSD）相关。
作者描述了一名儿童DCRV病例，该患儿因系统性肺动脉高压和生长发育迟缓，于7个月大时接受了膜周部室间隔缺损的手术修复。此时，右心室内未测到压力阶差，超声心动图或右心室造影未观察到肌肉增生。术后随访显示节制束肥厚，伴有右心室中部压力阶差逐渐发展，在3.5岁时达到60 mmHg。在通过侵入性检查确诊DCRV并获取血流动力学数据后，该患儿接受了手术。患儿现6岁，未观察到再狭窄。
DCRV通常与VSD相关，但这两种疾病的病程可能不同。对于任何有室间隔缺损病史的儿童，即使在手术或自然闭合后，若临床检查发现收缩期喷射性杂音，都必须考虑DCRV的诊断。
