Pulmonary arterial hypertension caused by congenital extrahepatic portocaval shunt: a case report

Kai-Yang Lin,Hui Chen,Ling Yu

doi:10.1186/s12872-019-1124-1

Abstract

BackgroundCongenital extrahepatic portocaval shunt (CEPS), also known as Abernethy malformation, is an extremely rare anomaly of the splanchnic venous system, especially when accompanied by pulmonary arterial hypertension.Case presentationWe report a case of a 15-year-old female who was diagnosed with CEPS (Abernethy type Ib) accompanied by pulmonary arterial hypertension. This case was incidentally identified during abdominal ultrasound examination and confirmed by mesenteric and splenic arteriography. During more than 4 years of follow-up, after receiving sildenafil (80 mg/day), the patient’s condition improved in the first year after discharge. However, one year later, the patient’s conditions start to deteriorate.ConclusionThis article presents a rare case of Abernethy malformation accompanied by pulmonary arterial hypertension, which can be diagnosed by using abdominal ultrasonography, portal vein computed tomography angiography or mesenteric and splenic arteriography. This malformation had limited treatment and poor prognosis.

Highlights

Congenital extrahepatic portocaval shunt (CEPS), known as Abernethy malformation, is an extremely rare anomaly of the splanchnic venous system, especially when accompanied by pulmonary arterial hypertension.Case presentation: We report a case of a 15-year-old female who was diagnosed with CEPS (Abernethy type Ib) accompanied by pulmonary arterial hypertension
This article presents a rare case of Abernethy malformation accompanied by pulmonary arterial hypertension, which can be diagnosed by using abdominal ultrasonography, portal vein computed tomography angiography or mesenteric and splenic arteriography
Since the first description by Abernethy in 1793 [2], fewer than 100 cases of CEPS have been reported, and only a few cases accompanied by pulmonary hypertension have been described [3,4,5,6,7]

Summary

Introduction

Congenital extrahepatic portocaval shunt (CEPS), known as Abernethy malformation, is an extremely rare anomaly of the splanchnic venous system, especially when accompanied by pulmonary arterial hypertension. Case presentation: We report a case of a 15-year-old female who was diagnosed with CEPS (Abernethy type Ib) accompanied by pulmonary arterial hypertension. This case was incidentally identified during abdominal ultrasound examination and confirmed by mesenteric and splenic arteriography. During more than 4 years of follow-up, after receiving sildenafil (80 mg/day), the patient’s condition improved in the first year after discharge. One year later, the patient’s conditions start to deteriorate

Findings

Discussion

Conclusion