Rao P S
Br Heart J. 1977 Mar;39(3):276-88. doi: 10.1136/hrt.39.3.276.
We have previously reported isolated cases of anatomical and functional closure of ventricular septal defects in tricuspid atresia. To study this phenomenon further, clinical, angiographic, and pathological findings in 20 consecutive cases of tricuspid atresia were reviewed. Sixteen cases were found to have normally related great arteries (type I) and 4 had transposition (type II). In 6 of these patients there was evidence of closure of a ventricular septal defect; in 3 this was complete and in the other 3, partial. Five of these ventricular septal defect closures occurred in type I patients and one among type II. Increasing cyanosis and polycythaemia and/or disappearance of a previously heard murmur were observed in all patients; these signs are more conspicuous in complete closure than in partial. The incidence of closure of ventricular septal defect in tricuspid atresia is 38 per cent and approximates to the incidence of spontaneous closure of isolated ventricular septal defects. Progressive muscular "encroachment" of the margins of ventricular septal defects with subsequent fibrosis and covering by endocardial proliferation is the most likely mechanism of closure in tricuspid atresia. The factors initiating the closure of ventricular septal defect remain unknown, but because of its occurrence in patients both with and without previous shunt operations, it is reasonable to assume that closure of ventricular septal defect in tricuspid atresia is not initiated or accelerated by these surgical shunts. Recent developments in surgical technique permit total surgical correction of tricuspid atresia at least in a physiological sense, but these operations can be successfully performed only in older children (over 8 years). Thus, palliation is essential in younger patients until they reach the age for total correction. A Blalock-Taussig shunt is recommended in preference to a Glenn anastomosis because the latter may leave the left pulmonary circuit without blood supply if the ventricular septal defect closes. In type II cases, a large and non-restrictive ventricular septal defect is essential for survival of the patient after the Fontan operation. For this reason, the size of the ventricular septal defect should be evaluated before and at the time of surgical correction. If the ventricular septal defect is small in a type II case, resection of the ventricular septum or a complete bypass of the ventricular septal defect and right ventricle is necessary at the time of the Fontan operation.
我们之前曾报道过三尖瓣闭锁患者室间隔缺损出现解剖学和功能性闭合的个别病例。为了进一步研究这一现象，我们回顾了连续20例三尖瓣闭锁患者的临床、血管造影及病理检查结果。发现其中16例患者的大动脉关系正常（I型），4例存在大动脉转位（II型）。在这些患者中，有6例存在室间隔缺损闭合的证据；其中3例完全闭合，另外3例部分闭合。这些室间隔缺损闭合病例中，5例发生在I型患者，1例发生在II型患者。所有患者均出现发绀加重、红细胞增多和/或之前听到的杂音消失；这些体征在完全闭合时比部分闭合时更明显。三尖瓣闭锁患者室间隔缺损闭合的发生率为38%，接近孤立性室间隔缺损自然闭合的发生率。室间隔缺损边缘逐渐出现肌肉性“侵入”，随后发生纤维化并被心内膜增生覆盖，这是三尖瓣闭锁中室间隔缺损闭合最可能的机制。启动室间隔缺损闭合的因素尚不清楚，但由于其在有或无既往分流手术的患者中均有发生，因此有理由推测三尖瓣闭锁患者室间隔缺损的闭合并非由这些外科分流手术启动或加速。外科技术的最新进展使得至少在生理意义上能够对三尖瓣闭锁进行完全外科矫正，但这些手术仅能在年龄较大的儿童（8岁以上）中成功实施。因此，对于较年幼的患者，在达到完全矫正年龄之前，姑息治疗至关重要。推荐优先采用Blalock-Taussig分流术而非Glenn吻合术，因为如果室间隔缺损闭合，后者可能会使左肺循环失去血液供应。在II型病例中，大型非限制性室间隔缺损对于Fontan手术后患者的生存至关重要。因此，在外科矫正前及矫正时均应评估室间隔缺损的大小。如果II型病例中的室间隔缺损较小，在Fontan手术时需要切除室间隔或完全绕过室间隔和右心室。
