Pulmonary Hypertension in Children - New Insights of Diagnosis and Management

Abstract

Pulmonary hypertension (PH) is a pathophysiological condition that includes multiple clinical situations and can complicate most cardiovascular and respiratory diseases1. Step by step diagnosis and reviewing contemporary treatment approaches would signifi cantly impact the prognosis of pediatric patients with PH. Management of children with PH requires a multidisciplinary team with experience. PH is frequently associated with cardiac and pulmonary diseases with chronic hypoxia, obstructive respiratory disorders, chest malformations, pneumonia, acute respiratory distress, and can also develop during late phases of cystic fi brosis, bronchial asthma, bronchiectasis. Updated defi nition of PH at the world symposium (HTAP, Nice, 2018) includes values of pulmonary artery pressure >20 mmHg and using pulmonary vascular resistance (PVR) indexed to the body surface to identify pre-capillary PH, PVR≥3 WU×m2 5. Cardiac catheterization represents the gold standard in diagnosing PH, being the most precise method of measuring the blood pressure (BP) in the pulmonary artery and offering valuable information about cardiac output, arterial pressure, and the response to pulmonary vasodilators2,3. The specifi c modern treatment with endothelin receptor inhibitors signifi cantly improves the disease’s clinical course and brings better parameters at instrumental investigations.