Vargas F J, Kreutzer G O, Pedrini M, Capelli H, Rodriguez Coronel A
J Thorac Cardiovasc Surg. 1986 Nov;92(5):908-12.
Since 1964, 77 patients underwent repair for tetralogy of Fallot with subarterial ventricular septal defect. Median age at operation was 5 years. Cyanosis was commonly mild, and hypoxic episodes were infrequent. Accordingly, only 12 patients (15.58%) needed either palliative or corrective operations before the age of 2 years. Characteristic angiographic and echocardiographic features were observed, which allowed this entity to be differentiated from either classic tetralogy or other types of double-outlet right ventricle. The earliest series of operations (in which no right ventricular outflow patch was used) was followed by a prohibitive mortality (50%). Residual subpulmonary obstruction was the cause of all of these deaths. In a second series of patients, a transannular patch was frequently used (79.59%), with the rationale that closure of the ventricular septal defect would make restrictive the deficient infundibulum of these patients. In a later series, we observed that most of the patients had a nonrestrictive pulmonary anulus (80%); the patch therefore was limited to the ventriculotomy. In the absence of pulmonary annular hypoplasia, the need for patching of the right ventricular outflow tract in this entity has to be confined to preventing the subpulmonary obstruction induced by closure of the ventricular septal defect. A considerable improvement in the results followed our first series (4.3% mortality). Follow-up of the survivors (mean 7 years) has been satisfactory.
自1964年以来，77例患有动脉下室间隔缺损的法洛四联症患者接受了修复手术。手术时的中位年龄为5岁。紫绀通常较轻，缺氧发作很少见。因此，只有12例患者（15.58%）在2岁之前需要进行姑息性或矫正性手术。观察到了特征性的血管造影和超声心动图特征，这使得该病症能够与经典法洛四联症或其他类型的右心室双出口相鉴别。最早的一系列手术（未使用右心室流出道补片）之后死亡率极高（50%）。所有这些死亡均由残余的肺下梗阻所致。在第二组患者中，经常使用经环补片（79.59%），其理由是关闭室间隔缺损会使这些患者原本不足的漏斗部变得狭窄。在后来的一组患者中，我们观察到大多数患者的肺动脉环无狭窄（80%）；因此，补片仅限于心室切开处。在没有肺动脉环发育不全的情况下，对于该病症，右心室流出道补片的需求必须局限于预防因室间隔缺损关闭所导致的肺下梗阻。在我们的第一组手术之后，结果有了显著改善（死亡率为4.3%）。对幸存者的随访（平均7年）情况令人满意。
