Shah M J, Rychik J, Fogel M A, Murphy J D, Jacobs M L
Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, 19104, USA.
Ann Thorac Surg. 1997 Apr;63(4):960-3. doi: 10.1016/s0003-4975(96)00961-7.
A high incidence of pulmonary arteriovenous malformations (PAVMs) has been reported in patients who have polysplenia and congenital heart disease after superior cavopulmonary anastomosis. Interruption of hepatic venous return to the pulmonary circulation is believed to potentiate the development of PAVMs. Surgical inclusion of hepatic flow in the pulmonary circulation may result in their resolution.
We reviewed 3 patients with congenital heart disease and polysplenia in whom PAVMs developed and who had subsequent hepatic vein inclusion in the pulmonary circulation.
Patients underwent superior cavopulmonary connection at a median age of 8 months. The PAVMs were diagnosed at a median duration of 8 months after operation (arterial saturation <75% in room air). Hepatic venous flow was included in the pulmonary circulation at operation. Resolution of PAVMs occurred at a median duration of 7 months after operation (arterial saturation >90% in room air).
Surgical inclusion of hepatic venous blood in the pulmonary circulation results in the resolution of PAVMs. Electively associating the hepatic veins with the pulmonary vasculature may prevent the development of PAVMs in patients who are at risk.
据报道，在多脾综合征且先天性心脏病患者接受上腔静脉-肺动脉吻合术后，肺动静脉畸形（PAVM）的发生率较高。肝静脉回流至肺循环的中断被认为会促进PAVM的发展。手术将肝血流纳入肺循环可能会使其消退。
我们回顾了3例先天性心脏病合并多脾综合征且发生PAVM并随后将肝静脉纳入肺循环的患者。
患者接受上腔静脉-肺动脉连接术的中位年龄为8个月。PAVM在术后中位时间8个月时被诊断（室内空气中动脉血氧饱和度<75%）。术中将肝静脉血流纳入肺循环。PAVM在术后中位时间7个月时消退（室内空气中动脉血氧饱和度>90%）。
手术将肝静脉血纳入肺循环可使PAVM消退。选择性地将肝静脉与肺血管系统相连可能会预防有风险患者发生PAVM。
