Santos de Soto J, Castillo J, Alvarez Madrid A, Grueso J, Moruno A, Descalzo A
Servicio de Hemodinámica, Hospital Infantil Virgen del Rocío, Sevilla.
Rev Esp Cardiol. 1990 Jan;43(1):23-8.
Double-outlet right ventricle with mitral atresia is an uncommon anomaly with a few cases reported in the literature. We present 9 cases of this malformation that have been diagnosed by two-dimensional echocardiography (7 cases), cardiac catheterization (9 cases) and anatomical study (2 cases). We classify them into two groups according to whether or not they have associated pulmonary stenosis. The dominant symptoms were cyanosis and hypoxemia in the first group and cardiac insufficiency signs in the other. The left ventricle was hypoplastic in eight and normal in the one with tricuspid overriding. Six of the cases had ventricular septal defect. The great arteries were in normal relationship in 4 cases, with D-malposition in 3 cases and side-by-side in 2 cases. Rashkind atrial septotomy was performed in 5 patients, but was effective only in two. Palliative surgical treatment was performed on six of them. The actual survival rate is 44%.
右心室双出口合并二尖瓣闭锁是一种罕见的畸形，文献报道的病例较少。我们呈现9例经二维超声心动图（7例）、心导管检查（9例）及解剖学研究（2例）确诊的该畸形病例。根据是否合并肺动脉狭窄，我们将其分为两组。第一组主要症状为青紫和低氧血症，另一组为心功能不全体征。8例左心室发育不全，1例合并三尖瓣骑跨者左心室正常。6例有室间隔缺损。4例大动脉关系正常，3例为右位心，2例为并列关系。5例患者行Rashkind房间隔造口术，但仅2例有效。其中6例接受了姑息性手术治疗。实际生存率为44%。
