Pulmonary hypertension associated with lung diseases

Area of Science:

• Cardiology and Pulmonology
• Respiratory Medicine
• Pulmonary Vascular Disease

Background:

• Pulmonary hypertension (PH) is common in chronic lung disease (CLD) and linked to poorer patient outcomes.
• Understanding the heterogeneity of CLD-PH requires detailed phenotyping of both the lung disease and the severity of PH.
• Current diagnostic and management strategies for CLD-PH need refinement due to varied patient populations and treatment responses.

Purpose of the Study:

• To summarize current data on PH in chronic lung diseases like COPD and ILD.
• To provide recommendations for classifying CLD-PH phenotypes.
• To outline an approach for the diagnosis and management of patients with CLD-PH.

Main Methods:

• Systematic review and synthesis of existing epidemiological and clinical trial data.
• Analysis of uncontrolled studies and one phase 3 randomized controlled trial.
• Focus on specific CLD phenotypes, including COPD and interstitial lung disease (ILD).

Main Results:

• PH in CLD is associated with worse prognosis, necessitating accurate phenotyping.
• Treatment benefits for PH in CLD are suggested in fibrotic ILD but may yield adverse outcomes in COPD-PH.
• The heterogeneity of CLD-PH underscores the need for phenotype-specific management.

Conclusions:

• A systematic diagnostic approach is recommended for suspected PH in CLD patients.
• Developing phenotype-specific treatment algorithms is essential given the expanding therapeutic options for pulmonary arterial hypertension.
• Tailored management strategies are required for different CLD-PH phenotypes to improve patient outcomes.