Children's' Heart Center, Second Floor, Kokilaben Dhirubai Ambani Hospital and Research Center, Rao Achyut Rao Patwardhan Marg, Four Bunglows, Andheri West, Mumbai, Maharashtra, 400053, India.
Department of Pediatrics, Bhaktivednata Hospital and Research Institute, Bhaktivendanta Swami Marg, Srishti Sector 1, Mira Road, Thane, 401107, India.
Indian Heart J. 2021 Jan-Feb;73(1):99-103. doi: 10.1016/j.ihj.2020.12.015. Epub 2020 Dec 30.
Abernethy malformation (congenital extrahepatic portosystemic shunt) is a rare anomaly of the splanchnic venous system. Though rare, it is an important cause of pulmonary artery hypertension (PAH) which is often missed. All patients with PAH should be carefully evaluated for presence of Abernethy malformation before labelling them as Idiopathic PAH.
This is a retrospective analysis of prospectively collected data. We reviewed the data of all patients referred to our center for evaluation of PAH. 10 patients were diagnosed to have an extrahepatic portocaval malformation. We reviewed their presentation, diagnosis, catheterization data, intervention and their outcome along with review of literature.
10/104 patients with pulmonary hypertension and no intra or extracardiac shunt were found to have extrahepatic portocaval shunt (EHPCS). 3 patients had EHPCS type 1 and 7 had type 2 EHPCS. 6/7 patient with EHPCS type 2 underwent closure of the shunt. There was no procedure related complication. There was one death 3 months post procedure and one patient who was advised surgical closure was lost to follow up. Closure of the shunt resulted in normalization of the pulmonary artery pressures in 4/5 patients.
Congenital portosystemic malformations form an important and potentially treatable cause of pulmonary hypertension.
Abernethy 畸形（先天性肝外门体分流）是一种罕见的内脏静脉系统畸形。尽管罕见，但它是肺动脉高压（PAH）的一个重要原因，而这种情况常常被漏诊。所有患有 PAH 的患者在被标记为特发性 PAH 之前，都应仔细评估是否存在 Abernethy 畸形。
这是一项前瞻性收集数据的回顾性分析。我们回顾了所有转诊至我们中心评估 PAH 的患者的数据。有 10 名患者被诊断为存在肝外门腔静脉分流。我们回顾了他们的临床表现、诊断、导管数据、干预措施及其结果，并同时回顾了文献。
在 104 例患有肺动脉高压且无心脏内或心脏外分流的患者中，发现 10 例存在肝外门腔静脉分流（EHPCS）。3 例为 EHPCS 1 型，7 例为 EHPCS 2 型。EHPCS 2 型的 7 例患者中有 6 例接受了分流关闭术。无手术相关并发症。术后 3 个月有 1 例死亡，1 例建议行手术闭合的患者失访。5 例患者中有 4 例在关闭分流术后肺动脉压恢复正常。
先天性门体分流畸形是肺动脉高压的一个重要且潜在可治疗的原因。
