Chantepie A, Marchand M, Dupre J M, Raynaud P, Laugier J
Département de Pédiatrie, CHU Clocheville, Tours.
Arch Fr Pediatr. 1988 Jan;45(1):41-4.
A case of pulmonary valve agenesis with ventricular septal defect and large patent ductus arteriosus is reported in a neonate. Heart failure occurred at 10 days of life; clinical examination showed continuous murmur. Echocardiography and angiocardiography visualized a major dilatation of the pulmonary artery. At cardiac catheterization there was an important left to right shunt through a large ductus, and pulmonary hypertension. There was also marked pulmonic regurgitation. Because of respiratory distress, ligation of ductus arteriosus was performed at 5 weeks of life. Five years later the patient is still in good condition. Patent ductus arterious is rarely associated with pulmonary valvular agenesis and ventricular septal defect. Our case is not explained by the fetal circulation theory described in this heart malformation.
本文报道了一例患有室间隔缺损和大型动脉导管未闭的新生儿肺动脉瓣发育不全病例。患儿出生10天时出现心力衰竭；临床检查显示有连续性杂音。超声心动图和心血管造影显示肺动脉明显扩张。心导管检查发现通过大型动脉导管存在大量从左向右分流以及肺动脉高压。同时还存在明显的肺动脉反流。由于呼吸窘迫，患儿在出生5周时进行了动脉导管结扎术。5年后，该患者状况良好。动脉导管未闭很少与肺动脉瓣发育不全和室间隔缺损相关。我们的病例无法用这种心脏畸形所描述的胎儿循环理论来解释。
