Pulmonary Hypertension Diagnosis and Management

Introduction to Pulmonary Hypertension

The European Respiratory Society defines pulmonary hypertension as mean pulmonary arterial pressure ≥25 mmHg at rest measured by right heart catheterization 3
The World Health Organization (WHO) Functional Classification stratifies patients based on physical activity limitations and symptoms, with Class I representing no limitation and Class IV representing inability to perform any activity without symptoms 1, 2

Pre-capillary PH requires pulmonary artery wedge pressure ≤15 mmHg and pulmonary vascular resistance >3 Wood units 3
Post-capillary PH is defined by pulmonary artery wedge pressure >15 mmHg 3
The European Respiratory Society defines five clinical groups of pulmonary hypertension, including pulmonary arterial hypertension (PAH), PH due to left heart disease, PH due to lung diseases and/or hypoxia, chronic thromboembolic PH (CTEPH), and PH with unclear/multifactorial mechanisms 3, 4

The European Respiratory Society identifies specific parameters that determine better versus worse prognosis, including WHO Functional Class, 6-minute walk test distance, peak oxygen consumption, and BNP/proBNP levels 1, 2
Better prognosis indicators include no clinical evidence of right ventricular failure, slow rate of symptom progression, and WHO Functional Class I-II 1, 2
Worse prognosis indicators include clinical evidence of right ventricular failure, rapid symptom progression, and WHO Functional Class III-IV 1, 2

Right heart catheterization is essential for accurate diagnosis and classification of all PH 1, 2
Echocardiographic diagnosis of "PH likely" with symptoms requires right heart catheterization (Class I recommendation) 1, 2
The European Respiratory Society recommends specific assessments at baseline and every 3-6 months, including WHO Functional Class assessment, 6-minute walk test, BNP/proBNP levels, echocardiography, and right heart catheterization 8

The American Thoracic Society and the European Respiratory Society recommend targeted therapies for PAH, including phosphodiesterase-5 inhibitors, endothelin receptor antagonists, prostacyclin analogues, and soluble guanylate cyclase stimulators 3, 6
Long-term oxygen therapy is indicated when arterial oxygen pressure is consistently <8 kPa (60 mmHg) 8
Surgical pulmonary endarterectomy is the treatment of choice for eligible CTEPH patients 3