Batlivala Sarosh P, Matoq Amr, Shahanavaz Shabana
Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
World J Pediatr Congenit Heart Surg. 2023 Jul;14(4):527-530. doi: 10.1177/21501351231162958. Epub 2023 Mar 27.
Microvascular pulmonary arteriovenous malformations (pAVMs) can lead to profound hypoxemia. "Hepatic factor" is postulated to play a role in their development. Certain patients with congenital heart disease are at particular risk to develop pAVMs, including those with heterotaxy syndromes and complex Fontan palliation. Ideally, an underlying cause is identified and corrected, although pAVMs may persist despite those interventions. We report a patient with heterotaxy syndrome s/p Fontan who had pAVMs that persisted despite Fontan revision with equal hepatic flow to both lungs. We employed a novel method to produce a diabolo configuration of a large covered stent to restrict lung flow while maintaining the potential for future dilation.
微血管性肺动静脉畸形（pAVM）可导致严重低氧血症。推测“肝脏因子”在其发展过程中起作用。某些先天性心脏病患者发生pAVM的风险特别高，包括那些患有内脏异位综合征和复杂Fontan姑息手术的患者。理想情况下，应找出并纠正潜在病因，尽管进行了这些干预措施，pAVM仍可能持续存在。我们报告了一名患有内脏异位综合征且接受过Fontan手术的患者，其pAVM尽管进行了Fontan手术矫正，使两肺肝血流相等，但仍持续存在。我们采用了一种新颖的方法，制作了一个大型覆膜支架的空竹状结构，以限制肺血流，同时保留未来扩张的可能性。
