ArticleIndian heart journal
Congenital extrahepatic portocaval malformation: Rare but potentially treatable cause of pulmonary hypertension.
Article in Indian heart journal. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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Who cites it
5 citing papers in PubMed, 8 citations in OpenAlex.
- Challenges and Strategies in Managing Pulmonary Arterial Hypertension Within the Indian Healthcare System: A Consensus Statement From the Pulmonary Vascular Research Institute India Taskforce.Pulmonary circulation · 2026Article
- Porto-Pulmonary Hypertension in Children: Insights From a National Registry.Pulmonary circulation · 2025Article
- Congenital Absence of the Portal Vein as a Rare Cause of Portopulmonary Hypertension-A Case Study Series.Medicina (Kaunas, Lithuania) · 2022Article
- Case report: Rare abernethy malformation with hepatopulmonary syndrome in a pediatric patient.Frontiers in pediatrics · 2022Article
- Pediatric pulmonary hypertension registry: Single-center experience from India.Annals of pediatric cardiologyArticle
Corrections and comments
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Authors and funding
8 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveAbernethy 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.
methodsThis 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.
results10/104 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.
conclusionCongenital portosystemic malformations form an important and potentially treatable cause of pulmonary hypertension.
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