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Effective shunt closure for pulmonary hypertension and liver dysfunction in congenital portosystemic venous shunt
Author(s) -
Uike Kiyoshi,
Nagata Hazumu,
Hirata Yuichiro,
Yamamura Kenichiro,
Terashi Eiko,
Matsuura Toshiharu,
Morihana Eiji,
Ohkubo Kazuhiro,
Ishii Kanako,
Sakai Yasunari,
Taguchi Tomoaki,
Ohga Shouichi
Publication year - 2018
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.23944
Subject(s) - medicine , portosystemic shunt , pulmonary hypertension , shunt (medical) , portal hypertension , pulmonary artery , gastroenterology , surgery , vascular resistance , bilirubin , hemodynamics , cardiology , cirrhosis
Objective Congenital portosystemic venous shunt (CPSVS) is a rare vascular malformation with a high risk of mortality from pulmonary arterial hypertension (PAH), but the treatment outcome of CPSVS closure remains elusive. Our aim was to investigate the clinical features and establish the optimal management of CPSVS with or without PAH. Methods Twenty‐four patients with CPSVS treated in Kyushu University Hospital between 1990 and 2015 were enrolled in this study. The patients were divided into a PAH group ( n = 9) and a non‐PAH group ( n = 15). Clinical characteristics and outcomes were evaluated. Results The first manifestation of CPSVS at diagnosis (28.5 [1‐216] months) was hypergalactosemia in 13 (54%) or PAH in six (25%) patients. PAH was the cause of all three deaths. The PAH group had higher levels of serum total bile acid, manganese, and total bilirubin, along with higher pulmonary vascular resistance index (PVRI) than the non‐PAH group (7.2 [5.1‐38.1] vs 1.2 [0.5‐3.3] unit/m 2 , P < 0.001). Five of nine PAH patients underwent CPSVS closure at a median of 38 months (range 21‐118) after PAH diagnosis. Pulmonary artery pressure improved after CPSVS closure with PAH‐specific therapy, but normal range was not achieved. CPSVS closure improved the hepatic synthetic function of four PAH patients. Eight of 15 non‐PAH patients who received CPSVS closure did not develop PAH for a median of 34.5 months (range 6‐164) after the procedure. Conclusions CPSVS closure with PAH‐specific therapy successfully controlled PAH. Early CPSVS closure may prevent the occurrence and progression of PAH with CPSVS.