Vázquez C, Azcuna I, Pastor E, Labairu M, Cabrera A
An Esp Pediatr. 1980 Nov;13(11):953-64.
Total anomalous pulmonary venous drainage is one of the cardiac malformations requiring early surgical correction. In our series of nine cases, 89% were under three months of age and none above six months. Clinically, two types were present: without obstruction and with obstruction to the pulmonary venous flow. In the non obstructive group, all had mild cyanosis, heart failure, cardiomegaly and systolic murmur. In the obstructive group, they had severe cyanosis, heart failure, small heart and pulmonary fields with a "ground glass" or mottled appearance on chest X-ray due to hypertension and edema. The second heart found was split in both groups. An increased right sided O2 saturation and pulmonary hypertension are due to the total anomalous pulmonary venous connection to the right atrium. Right atrium pressures were superior than left atrium pressures in two patients, requiring atrioseptostomy in one patient. Total surgical correction was performed in three patients, with one patient surviving; this patient is well one year after operation. It is noteworthy in our series the early symptomatology. Symptoms begun at birth in 77% of the cases. It was striking as well the absence of cases with drainage to the right atrium or the coronary sinus.
完全性肺静脉异位引流是需要早期手术矫正的心脏畸形之一。在我们的9例病例系列中，89%的患儿年龄在3个月以下，无1例超过6个月。临床上存在两种类型：无梗阻型和肺静脉血流梗阻型。在无梗阻组，所有患儿均有轻度发绀、心力衰竭、心脏扩大和收缩期杂音。在梗阻组，患儿有严重发绀、心力衰竭、心脏小，胸部X线显示肺野呈“毛玻璃”样或斑点状，这是由于高血压和水肿所致。两组患儿均发现第二心音分裂。右侧氧饱和度增加和肺动脉高压是由于肺静脉完全异位连接至右心房所致。2例患者右心房压力高于左心房压力，其中1例患者需要进行房间隔造口术。3例患者进行了完全手术矫正，1例患者存活；该患者术后1年情况良好。在我们的病例系列中，早期症状值得注意。77%的病例在出生时即开始出现症状。同样引人注目的是，没有引流至右心房或冠状窦的病例。
