J Korean Med Sci.  2014 Apr;29(4):604-608. 10.3346/jkms.2014.29.4.604.

A Case of Pulmonary Arterial Hypertension Associated with Congenital Extrahepatic Portocaval Shunt

• Yi JE
• Jung HO
• Youn HJ
• Choi JY
• Chun HJ
• Lee JY

• KMID: 1774470
• DOI: http://doi.org/10.3346/jkms.2014.29.4.604

Abstract

Congenital extrahepatic portocaval shunt (CEPS) is a rare anomaly of the mesenteric vasculature in which the intestinal and splenic venous drainage bypasses the liver and drains directly into the inferior vena cava, the left hepatic vein or the left renal vein. This uncommon disease is frequently associated with other malformations and mainly affects females. Here we report a case of pulmonary arterial hypertension associated with CEPS (Abernethy type 1b shunt) in a 20-yr-old man who was incidentally diagnosed during evaluation of multiple nodules of the liver. The patient was treated by inhalation of iloprost (40 microg/day) with improved condition and walking test. Physicians should note that congenital portocaval shunt may cause pulmonary hypertension.

Keyword

Congenital Extrahepatic Portocaval Shunt; Pulmonary Arterial Hypertension; Heart Septal Defect, Ventricular; Multifocal Nodular Regenerative Hyperplasia of the Liver

MeSH Terms

Echocardiography, Doppler
Humans
Hypertension, Pulmonary/*diagnosis/drug therapy
Iloprost/therapeutic use
Liver/blood supply/radiography
Magnetic Resonance Imaging
Male
Thoracic Arteries/ultrasonography
Tomography, X-Ray Computed
Vasodilator Agents/therapeutic use
Vena Cava, Inferior/*abnormalities/ultrasonography
Young Adult
Iloprost
Vasodilator Agents

Figure

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  Fig. 1 Chest X-ray showed cardiomegaly and increased vascularity of the proximal portion of both pulmonary arteries.

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  Fig. 2 Two-dimensional echocardiography, showing D-shpaed left ventricle during diastole phase in parasternal short axis view (arrowhead) (A) and transthoracic Doppler echocardiography, showing tricuspid regurgitation with maximal pressure gradient (54.8 mmHg) (B).

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  Fig. 3 Right heart catheterization, showing the systolic pulmonary artery pressure was 52-63 mmHg, and the mean pulmonary artery pressure was 44 mmHg (A). The estimated mean pulmonary capillary wedge pressure was 7 mmHg (B).

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  Fig. 4 Cardiac magnetic resonance imaging, showing multiple hyperintense masses adjacent to the heaptic parenchyma. There was a central stellate scar that was hypointense on the T1 images (arrowheads) (A) and hyperintense on the T2 images (arrows) (B).

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  Fig. 5 Coronal CT angiogram images illustrated the course of the extrahepatic PV. The left PV (arrows) and left hepatic vein (arrowhead) joined (A) and drained directly into the suprahepatic IVC (asterisk) (B). PV, portal vein; IVC, inferior vena cava.

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  Fig. 6 Portal venography (A) and two-dimensional echocardiography (B) showed the end-to-side communication between the left PV and IVC with obliteration of the right PV (Abernethy type 1b). IVC, inferior vena cava; PV, portal vein; SMV, superior mesenteric vein; Splenic v., Splenic vein.

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