Wisotzkey Bethany L, Magyar Dari L, Jones Thomas K, Boucek Robert J, Permut Lester C, Kemna Mariska S, Law Yuk M
Department of Pediatrics, Division of Pediatric Cardiology, Johns Hopkins All Children's Hospital, Saint Petersburg, FL, USA.
Department of Pediatrics, Seattle Children's Hospital, Seattle, WA, USA.
Clin Transplant. 2018 Feb;32(2). doi: 10.1111/ctr.13183. Epub 2018 Jan 12.
In single ventricle patients, aortopulmonary collaterals (APCs) and pulmonary arteriovenous malformations (PAVMs) following superior cavopulmonary shunt (CPS) can complicate orthotopic heart transplant (OHT) by cyanosis and hemoptysis. Although PAVMs can regress with the restoration of hepatic venous flow to the pulmonary circulation, the effects of hypoxemia on the "unconditioned" allograft are not known.
Two patients with significant PAVMs after CPS were cyanotic following OHT. One patient with predominantly unilateral left PAVMs had arterial saturation levels less than 70% despite pulmonary vasodilators and ventilation. A custom flow restrictor-covered stent was deployed in the pulmonary artery of the affected side, redirecting the blood flow to the contralateral lung, immediately improving cyanosis. When the PAVMs regressed, the flow restrictor stent was dilated to eliminate the constriction. The second patient with PAVMs had cyanosis and severe hemoptysis from APCs post-OHT. The APCs required an extensive coil embolization, while the cyanosis responded to oxygen and pulmonary vasodilators. Both recipients did well with gradual resolution of PAVMs within 8 months.
Despite cyanosis from right-to-left intrapulmonary shunting, allograft function recovered. Novel transcatheter interventions can play a role in patients with significant APCs or PAVM following cardiac transplantation.
在单心室患者中，上腔静脉-肺动脉分流术（CPS）后出现的主-肺动脉侧支血管（APC）和肺动静脉畸形（PAVM）可因发绀和咯血而使原位心脏移植（OHT）变得复杂。尽管PAVM可随着肝静脉血流恢复至肺循环而消退，但低氧血症对“未预处理”同种异体移植物的影响尚不清楚。
两名CPS后出现显著PAVM的患者在OHT后出现发绀。一名主要为左侧单侧PAVM的患者，尽管使用了肺血管扩张剂并进行了通气，动脉血氧饱和度仍低于70%。在患侧肺动脉中置入了一个定制的限流覆盖支架，将血流重定向至对侧肺，立即改善了发绀。当PAVM消退时，扩张限流支架以消除狭窄。第二名患有PAVM的患者在OHT后因APC出现发绀和严重咯血。APC需要广泛的弹簧圈栓塞，而发绀对氧气和肺血管扩张剂有反应。两名受者情况良好，PAVM在8个月内逐渐消退。
尽管存在右向左肺内分流导致的发绀，但同种异体移植物功能仍得以恢复。新型经导管干预措施可在心脏移植后患有显著APC或PAVM的患者中发挥作用。
