Pediatric Pulmonary Hypertension in Left-Sided Heart Disease

Pulmonary hypertension (PH) from left-sided heart disease (group-II PH) is an increasingly recognized cause of PH in pediatrics. Group-II PH can result from obstruction at any level of the left heart, and can progress over time. Management can be particularly difficult, as targeted PH therapy in the setting of a fixed obstruction carries a risk of pulmonary vascular congestion and pulmonary edema. Based on existing evidence, the use of pulmonary vasodilators in group II PH is not recommended, and management centers around early identification and correction of the underlying left-sided lesion. In this review, we highlight the pathophysiology of group-II PH, the diagnostic evaluation of left heart pathology, and a general approach to both medical and surgical management, with particular attention to relevant left-sided lesions. Group-II PH is a multifaceted and progressive disease process that poses a difficult challenge to clinicians, and requires a thoughtful and individualized approach to management.