• Medicina · Sep 2020

    Segmental Pulmonary Hypertension in Children with Congenital Heart Disease.

    • Bibhuti B Das, Benjamin Frank, and Dunbar Ivy.
    • Department of Pediatric Cardiology, Baylor College of Medicine, Texas Children's Hospital Austin Specialty Care, Austin, TX 78759, USA.
    • Medicina (Kaunas). 2020 Sep 24; 56 (10).

    AbstractSegmental pulmonary hypertension is a complex condition in children that encompasses many congenital heart diseases including pulmonary atresia with ventricular septal defect, hemitruncus/truncus arteriosus with branch pulmonary artery stenosis, unilateral absent pulmonary artery, and several post-tricuspid shunt lesions. Multimodality imaging is required to confirm and assess pulmonary vascular disease in subjects with major aorto-pulmonary collaterals. We describe 3 children with complex congenital heart defects who have a variable degree of segmental pulmonary hypertension and discuss management strategies and the role of interventional and/or pulmonary hypertension targeted therapies.

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