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Case Reports
. 2019 Jun 13;19(1):141.
doi: 10.1186/s12872-019-1124-1.

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

Kai-Yang Lin  1 , Hui Chen  1 , Ling Yu  2
Affiliations
Case Reports

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

Kai-Yang Lin et al. BMC Cardiovasc Disord. .

Abstract

Background: Congenital 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 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. However, one year later, the patient's conditions start to deteriorate.

Conclusion: 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. This malformation had limited treatment and poor prognosis.

Keywords: Abernethy malformation; Congenital extrahepatic portocaval shunt; Pulmonary arterial hypertension.

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Conflict of interest statement

The author(s) declare no potential conflicts of interest with respect to the research, authorship, and/or publication of this case report.

Figures

Fig. 1
Fig. 1
Echocardiography shows an enlargement of the PA and its branches (a). Both RA and RV are significantly dilated, and the RV wall thickness is increased (b). PA: pulmonary artery; RPA: right pulmonary artery; LPA: left pulmonary artery; AO: aorta; RA: right atrium; RV: right ventricle; LA: left atrium; LV: left ventricle
Fig. 2
Fig. 2
Abdominal ultrasonography shows that the intrahepatic PV system is absent (arrows) (a), and the SPV is tortuous and dilated (b). PV: portal vein; HA: hepatic artery; SP: spleen; SPV: splenic vein
Fig. 3
Fig. 3
PV-CTA shows that the trunk and branches of PV are absent, the SV is widened, with the SMV joining the LRV and then draining directly into the IVC without passing through the liver (a). CTA also reveals the enlargement of the spleen and splenic veins (arrows) and heterogeneous density lesions in the liver nodules (arrows) (b). PV: portal vein; CTA: computed tomography angiography; SV: splenic vein; SMV: superior mesenteric vein; LRV: left renal vein; IVC: inferior vena cava
Fig. 4
Fig. 4
Mesenteric and splenic arteriography shows that the portal vein was absent, and the shunt between splenic and renal venous supply and the LRV and IVC can be seen in the delayed phase (a, b). LRV: left renal vein; IVC: inferior vena cava
Fig. 5
Fig. 5
Changes in important parameters in echocardiography. RVSP: right ventricle systolic pressure; RA: right atrium; RV-ID: right ventricle inner diameter
See this image and copyright information in PMC

References

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