Division of Cardiology, Department of Pediatrics, British Columbia Children's Hospital and University of British Columbia, Vancouver, BC, Canada.
Ultrasound Obstet Gynecol. 2012 Nov;40(5):536-41. doi: 10.1002/uog.11126.
Tetralogy of Fallot with absent pulmonary valve syndrome (TETAPV) is reported in obstetric literature to have an extremely poor prognosis. We sought to determine the clinical outcome associated with TETAPV and whether prenatal diagnosis confers a poor prognosis.
All cases of TETAPV diagnosed in British Columbia between 1980 and 2009 were reviewed and grouped according to time of diagnosis, either prenatal or postnatal. The groups were compared with respect to mortality, respiratory problems, number of interventions and functional capacity at last follow-up.
Eight and 11 patients were included in the prenatally and postnatally diagnosed groups, with overall long-term survival of 71% and 82%, respectively. There was no significant difference in mortality, frequency of preoperative intubation, number of interventions or functional capacity between groups.
From a population-based retrospective analysis of TETAPV cases identified over three decades it is concluded that the prognosis for TETAPV is better than that previously reported in the obstetric literature. This information should be used to guide prenatal counseling.
在妇产科文献中，报道法洛四联症伴肺动脉瓣缺如综合征（TETAPV）的预后极差。我们试图确定与 TETAPV 相关的临床结果，以及产前诊断是否预示不良预后。
回顾了 1980 年至 2009 年在不列颠哥伦比亚省诊断的所有 TETAPV 病例，并根据诊断时间（产前或产后）进行分组。比较两组死亡率、呼吸问题、干预次数和最后随访时的功能能力。
分别有 8 例和 11 例患者被纳入产前和产后诊断组，两组的长期生存率分别为 71%和 82%。两组之间死亡率、术前插管频率、干预次数或功能能力均无显著差异。
通过对三十多年来基于人群的 TETAPV 病例回顾性分析，得出 TETAPV 的预后优于妇产科文献中的既往报道。这些信息应被用于指导产前咨询。
