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- W2063156718 abstract "A 31-year-old man presented with a 2-year history of progressive exertional dyspnoea and chest pain. He had severe pulmonary hypertension with a mean pulmonary artery pressure of 53 mm Hg. Magnetic resonance imaging (figure, left; T1-weighted 3D echo sequence with contrast), showed the dilated central pulmonary vessels and a persisting left caval vein (arrow) draining into a dilated coronary sinus. Spiral computed tomography (figure, right) showed the liver in midline position, a right-sided hypoplastic spleen (arrow) and the small bowel almost confined to the right abdomen. Abdominal angiography showed complex vascular malformations including bilateral aneurysms of the renal arteries, and an inferior mesenteric vein flowing into the superior mesenteric vein, which drained into the inferior vena cava. In combination with hypoplastic portal and hepatic veins, this anatomy caused a portosystemic shunt with elevated blood ammonia concentrations. This is a rare case with features of heterotaxy syndrome in a young adult, complicated by primary pulmonary hypertension." @default.
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- W2063156718 date "2003-03-01" @default.
- W2063156718 modified "2023-09-24" @default.
- W2063156718 title "Situs inversus and severe pulmonaryhypertension" @default.
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- W2063156718 doi "https://doi.org/10.1016/s0140-6736(03)12708-0" @default.
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