Article
First locus for primary pulmonary vein stenosis maps to chromosome 2q.
European heart journal - 1 Oct 2009
van de Laar Ingrid, Wessels Marja, Frohn-Mulder Ingrid, Dalinghaus Michiel, de Graaf Bianca, van Tienhoven Marianne, van der Moer Paul, Husen-Ebbinge Margreet, Lequin Maarten, Dooijes Dennis, de Krijger Ronald, Oostra Ben A, Bertoli-Avella Aida M
Abstract excerpt
AIMS: Primary pulmonary vein stenosis (PVS) is a rare cardiac abnormality that exhibits a high morbidity and mortality rate. The disease is characterized by obstruction of the pulmonary venous blood flow owing to congenital hypoplasia of individual extra-pulmonary veins. We describe a consanguineous Turkish family with four affected siblings with primary PVS in association with prenatal lymphatic abnormalities....
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