Pulmonary Arterial Hypertension | ||||||
Definition | Treatment (CHD) | Medications |
Introduction
Pulmonary hypertension (PH) is defined as a mean pulmonary artery pressure (mPAP) > 20 mmHg in children > 3 months age, at sea level. The etiology includes cardiac, pulmonary, connective tissue, pulmonary veno-occlusive diseases. Pulmonary arterial hypertension (PAH) is thought to result from an imbalance in the amount and ratio of vasoactive substances including prostacyclin, nitric oxide, and endothelin-1. In many instances the triggers for PAH are unknown but there is a subgroup with identified genetic abnormalities which may be inherited.
Further Reading
- 2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT. J Heart Lung Transplant 2019;38:879-901
- Pediatric Pulmonary Hypertension. Special Edition Cardiovascular Diagnosis and Therapy 2021;11 (4): 1023-1199
Last Updated: April 2023