Abstract
The World Pulmonary Hypertension Symposium in 2013 (Nice, France) introduced a new entity in the classification for pediatric and adult patients called “segmental pulmonary hypertension (PH).”1 Segmental PH was described in the international 2015 guidelines as PH “observed in discrete lung areas perfused by aortopulmonary collaterals in congenital heart diseases such as pulmonary or tricuspid atresia,”2 while the proceedings of the Nice World Symposium1 defined this as “PH in one or more lobes of one or both lungs.” Others have defined segmental PH more broadly as PH that does not follow a homogeneous distribution, with some parts of the pulmonary vasculature being exposed to higher pressures than others.3 This entity was included under the umbrella of World Heart Organization group 5 (PH caused by unclear or multifactorial mechanisms), because little is known about its pathophysiology and response to pulmonary arterial hypertension (PAH) therapies.1, 2 Segmental PH is most commonly encountered in patients with congenital heart disease (CHD) and carries notable similarities to PAH (Group 1.4.4, PAH associated with CHD) and group 4 of the PH classification (Group 4.2.4 PH in patients with congenital pulmonary artery [PA] stenoses), yet there is no systematic description of the broad spectrum of conditions encompassed by this entity or its distinct pathophysiological features and how these may affect management. We present herewith a consensus statement on segmental PH, including a working definition, range of conditions that may be classified under this entity, description of pathophysiology in terms of pulmonary vasculature, cardiovascular anatomy, and management principles.
Published Version (
Free)
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call