双腔右心室的病理特征和治疗问题

DOUBLE-CHAMBERED RIGHT VENTRICLE: ITS PATHOANATOMIC CHARACTERISTICS AND TREATMENT PROBLEMS

近10年来,外科治疗双腔右心室(DCRV)52例,占同期先天性心脏病总数的2.23％(52/1881)。男32例,女20例。年龄4.5～30岁。单纯性DCRV 1例,合并其它先天性心脏畸形51例,以室间隔缺损(VSD)最多见(50例),其次是肺动脉瓣狭窄(6例)、主动脉瓣脱垂(4例)、房间隔缺损(2例)和主动脉瓣下狭窄等。DCRV和其它心内畸形经右室切口修复47例,右室加右房切口2例;经右房切口2例,经肺动脉切口1例,均仅修复VSD而遗漏了DCRV。术后并发症有心律紊乱和低心排综合征。死亡3例,手术死亡率5.8％。根据右室异常肌束及其引起梗阻的特点,将本组52例DCRV分为2种类型:纤维肌隔型(24例,占44.7％)和肌束裂隙型(28例,占55.3％),不论何种类型,都不仅有室间隔侧的异常肌束肥厚,同时有壁侧的心室漏斗皱襞肥厚,少数病例心室漏斗皱襞的肥厚程度远较隔侧的异常肌束肥厚显著。

In the past ten years, 52 patients with double-chambered right ventricle (DCRV) were operated on in Xijing Hospital. They constituted 2.2% (52/1881) of all patients with congenital heart diseases. Of them, there were 32 males and 20 females, whose ages ranged from 4.5 to 30 years old. In one patient there was pure DCRV, and the remaining 51 patients DCRV was associated with other congenital cardiac anomalies, in which VSD was the most common (50), followed in order by pulmonary stenosis (6), aortic valve prolapse (4), atrial septal defect (2), and subaortic stenosis (1). The repair of DCRV and other intracardiac defects was done through the right ventricolotomy in 47 cases and through the right ventricolotomy plus atriotomy in 2 cases. In another 2 cases the operation was done via the right atriotomy and 1 case pulmonary arteriotomy, and only VSDs were closed, but DCRVs were missed. The major postoperative complications included cardiac arrhythmia (11) and low output syndrome (8). Three patients died, with the operative mortality of 5.8%. In this series, according to characteristics of the anomalous muscle bundle and its resulting obstruction, we divided 52 case DCRVs into two types: fibromuscular diaphragmatic type (24 cases, 44.7%) and muscular bundle-gap type (28 cases, 55.7%). In both types, there were not only hypertrophied anmalous muscle bundles on the septal side, but also hypertro-phied ventricoinfundibulum fold on the parietal side. In a few cases, the ventricoinfundibulum fold was more hypertrophic than the anomalous muscle bundle on the septal side.