Pulmonary Hypertension: A Brief Guide for Clinicians

Area of Science:

• Cardiology
• Pulmonology
• Internal Medicine

Background:

• Pulmonary hypertension (PH) encompasses five clinical subgroups, impacting approximately 1% of the global population and frequently observed in heart failure patients.
• Cardiologists commonly encounter PH, necessitating awareness of its diverse etiologies and diagnostic pathways.

Purpose of the Study:

• To outline the classification, diagnosis, and management strategies for the five clinical subgroups of pulmonary hypertension.
• To emphasize the importance of accurate diagnosis and timely referral for specific PH types.

Main Methods:

• Initial assessment involves electrocardiography, chest radiography, pulmonary function tests, and echocardiography to estimate PH probability.
• Ventilation-perfusion scans are crucial for excluding chronic thromboembolic pulmonary hypertension (CTEPH) in relevant cases.
• Right-sided heart catheterization is essential for definitive diagnosis and classification of PH.

Main Results:

• Surgical pulmonary endarterectomy is the primary treatment for eligible CTEPH patients.
• Targeted therapies, including PDE5 inhibitors, guanylate cyclase stimulators, and prostacyclin analogues, are available for pulmonary arterial hypertension (PAH).
• Riociguat is the sole approved targeted therapy for inoperable or persistent/recurrent CTEPH; management of PH from left-sided heart disease focuses on the underlying condition.

Conclusions:

• Accurate diagnosis and classification of PH subgroups are critical for guiding appropriate treatment strategies.
• Referral to specialist centers is mandatory for patients with PAH or CTEPH.
• Treatment approaches vary significantly across PH subgroups, ranging from surgery to targeted pharmacotherapies and management of underlying causes.