Pulmonary hypertension in patients with congenital portosystemic venous shunt: a previously unrecognized association.
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.
Ohno
Takuro
T
Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan.
Muneuchi
Jun
J
Ihara
Kenji
K
Yuge
Tetsuji
T
Kanaya
Yoshiaki
Y
Yamaki
Shigeo
S
Hara
Toshiro
T
D000293:Adolescent; D000792:Angiography; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D004452:Echocardiography; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006975:Hypertension, Portal / Q000151:congenital / Q000175:diagnosis / Q000209:etiology*; D006976:Hypertension, Pulmonary / Q000151:congenital / Q000175:diagnosis / Q000209:etiology*; D015994:Incidence; D007223:Infant; D008102:Liver Circulation; D008111:Liver Function Tests; D008297:Male; D011168:Portal System / Q000002:abnormalities*; D011169:Portal Vein / Q000002:abnormalities*; D011172:Portography; D011379:Prognosis; D012189:Retrospective Studies; D018570:Risk Assessment