Abstract
Segmental 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.
Highlights
The segmental pulmonary hypertension (PH) is defined as pulmonary vascular disease (PVD) in one or more but not all segments of the lung
The calculation of pulmonary vascular resistance (PVR) is impossible as the major aorto-pulmonary collaterals (MAPCAs) originating from aorta have the Evaluation of segmental PH in subjects with MAPCAs is best done using a multimodality approach
Segmental PH is a complex condition that encompasses a wide range of congenital heart diseases (CHD) and its management requires tertiary expertise both in CHD and PH
Summary
The segmental pulmonary hypertension (PH) is defined as pulmonary vascular disease (PVD) in one or more but not all segments of the lung. Each hypertensive segment may present with different degrees of PVD due to variable blood flow, local pressures, and different sources. A consensus statement to define and classify segmental PH is published in 2018 [1]. Segmental PH is commonly encountered in patients with congenital heart diseases (CHD) such as tetralogy of Fallot (TOF). In segmental PH due to pulmonary atresia with VSD or truncus arteriosus, the right ventricle (RV) hypertrophy, dilation or dysfunction can result from large VSD and resultant systemic RV pressure.
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