Published online March 24, 2008
PEDIATRICS Vol. 121 No. 4 April 2008, pp. e892-e899 (doi:10.1542/peds.2006-3411)
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ARTICLE

Pulmonary Hypertension in Patients With Congenital Portosystemic Venous Shunt: A Previously Unrecognized Association

Takuro Ohno, MD, PhDa, Jun Muneuchi, MDa, Kenji Ihara, MD, PhDa, Tetsuji Yuge, MDb, Yoshiaki Kanaya, MDa, Shigeo Yamaki, MD, PhDc and Toshiro Hara, MD, PhDa

a Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
b Department of Pediatrics, Kyushu Kouseinenkin Hospital, Fukuoka, Japan
c Japanese Research Institute of Pulmonary Vasculature, Miyagi, Japan

BACKGROUND. Pulmonary arterial hypertension has been reported to be observed in association with acquired portal hypertension. However, the contribution of congenital anomalies occurring in the portal system to the development of pulmonary arterial hypertension remains to be elucidated.

METHODS. Nine patients with congenital portosystemic venous shunt were studied from January 1990 through September 2005.

RESULTS. Patent ductus venosus was detected in 5 patients, including 3 patients with an absence of the portal vein. The presence of either a gastrorenal or splenorenal shunt was evident in another 4 patients. Six patients had a history of hypergalactosemia with normal enzyme activities, as seen during neonatal screening. Six (66.7%) of the 9 patients were identified to have clinically significant pulmonary arterial hypertension (mean pulmonary artery pressure: 34–79 mm Hg; pulmonary vascular resistances: 5.12–38.07 U). The median age at the onset of pulmonary arterial hypertension was 12 years and 3 months. Histologic studies of lung specimens, which were available in 4 of the 9 patients with congenital portosystemic venous shunt, showed small arterial microthrombotic lesions in 3 patients. This characteristic finding was recognized even in the congenital portosystemic venous shunt patients without PAH.

CONCLUSIONS. This study demonstrated thromboembolic pulmonary arterial hypertension to be a crucial complication in congenital portosystemic venous shunt, and this pathologic state may be latently present in patients with pulmonary arterial hypertension of unknown etiology.

Key Words: congenital portosystemic venous shunt • hypergalactosemia • microthrombosis • pulmonary arterial hypertension

Abbreviations: CPSVS—congenital portosystemic venous shunt • PAH—pulmonary arterial hypertension • IPAH—idiopathic pulmonary arterial hypertension • PA—pulmonary artery • MPAP—mean pulmonary artery pressure • NO—nitric oxide • VSD—ventricular septal defect • 5-HT—5-hydroxytryptamine

Accepted Sep 18, 2007.


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