Hofbeck M, Sunnegårdh J T, Burrows P E, Moes C A, Lightfoot N, Williams W G, Trusler G A, Freedom R M
Department of Paediatrics, Hospital for Sick Children, Toronto, Ontario, Canada.
Am J Cardiol. 1991 Apr 1;67(8):737-43. doi: 10.1016/0002-9149(91)90532-p.
This study reviews the clinical course of 104 consecutive patients with pulmonic valve atresia and ventricular septal (VSD) defect who were diagnosed in the first year of life and followed for a mean period of 4.95 years (range 2 days to 13.75 years). Specific attention was paid to the nature of the pulmonary blood supply and to its influence on patient outcome. Confluent pulmonary arteries supplied by a single ductus arteriosus were present in 72 patients (69%, group I), whereas 32 patients (31%, group II) had a pulmonary blood supply that was partially or exclusively dependent on systemic collateral arteries. An estimate of the probability of survival for 10 years was 69% in the entire cohort, with no different between patients in group I and group II. Definitive surgical repair was performed in 33 of 72 group I patients (46%), compared with 5 of 32 group II patients (16%). Arborization and distribution abnormalities of the pulmonary arteries as well as intrapulmonary stenoses that were exclusively present in patients with systemic collateral arteries (p less than 0.00001) accounted for the significantly lower probability of undergoing corrective surgery in group II patients.
本研究回顾了104例出生后第一年内确诊的肺动脉瓣闭锁合并室间隔缺损患者的临床病程，平均随访4.95年（范围2天至13.75年）。特别关注了肺血供的性质及其对患者预后的影响。72例患者（69%，I组）存在由单一动脉导管供血的融合肺动脉，而32例患者（31%，II组）的肺血供部分或完全依赖体循环侧支动脉。整个队列10年生存率估计为69%，I组和II组患者之间无差异。72例I组患者中有33例（46%）进行了确定性手术修复，而32例II组患者中有5例（16%）进行了手术修复。仅在体循环侧支动脉患者中出现的肺动脉分支和分布异常以及肺内狭窄（p<0.00001），是导致II组患者接受矫正手术概率显著降低的原因。
