Update on chronic thromboembolic pulmonary hypertension

Area of Science:

• Cardiology
• Pulmonology
• Vascular Medicine

Background:

• Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, underdiagnosed complication of acute pulmonary embolism.
• It involves unresolved thromboemboli causing pulmonary artery occlusion, classified as WHO Group 4 PH.
• The exact etiology of CTEPH remains uncertain.

Purpose of the Study:

• To provide an overview of CTEPH pathogenesis.
• To highlight current recommendations and recent advances in CTEPH evaluation and treatment.
• To emphasize the importance of recognizing CTEPH as a potentially curable condition.

Main Methods:

• Review of current understanding of CTEPH pathogenesis.
• Analysis of diagnostic evaluation strategies for PH.
• Summary of emerging and established treatment modalities for CTEPH.

Main Results:

• CTEPH is a distinct subtype of PH requiring specific diagnostic and treatment approaches.
• Pulmonary endarterectomy is a primary surgical option for eligible patients.
• Medical therapy and balloon pulmonary angioplasty are emerging treatment options for non-surgical candidates.

Conclusions:

• CTEPH is the only potentially curable form of PH, necessitating prompt recognition.
• Timely referral to expert centers is critical for optimal management.
• Effective treatment can prevent progressive vascular obstruction, right heart failure, and mortality.