Chronic thromboembolic pulmonary hypertension.

Area of Science:

• Cardiology
• Pulmonology
• Vascular Medicine

Background:

• Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, potentially fatal complication of acute pulmonary embolism.
• The exact epidemiology, mechanisms of thrombotic material nonresolution, and arterial scarring in CTEPH remain largely unknown.
• CTEPH is currently underdiagnosed and undertreated, presenting diagnostic challenges due to nonspecific symptoms like exertional dyspnoea.

Purpose of the Study:

• To summarize the current understanding of CTEPH diagnosis and treatment.
• To highlight the importance of specialized centers for managing CTEPH.
• To review the available treatment options for CTEPH patients.

Main Methods:

• Diagnosis involves right heart catheterization and imaging (V/Q scan, CTPA, conventional angiography).
• Operability assessment by a multidisciplinary team is critical for treatment planning.
• Treatment strategies include pulmonary endarterectomy (PEA), medical therapy, and balloon angioplasty.

Main Results:

• Pulmonary endarterectomy (PEA) is the only curative treatment, significantly improving hemodynamics, symptoms, and life expectancy.
• PEA enables many patients to lead unrestricted lives on anticoagulation therapy.
• Medical therapy and balloon angioplasty are emerging options for inoperable or non-surgical candidates.

Conclusions:

• CTEPH diagnosis and management require specialized expertise and are best handled by experienced CTEPH centers.
• Prompt diagnosis and appropriate treatment are crucial for improving outcomes in CTEPH patients.
• Ongoing research is needed to further elucidate CTEPH mechanisms and refine treatment strategies.