[Pulmonary hypertension associated with connective tissue diseases]

Area of Science:

• Cardiology
• Rheumatology
• Pulmonology

Background:

• Pulmonary arterial hypertension (PAH) is a significant complication in connective tissue diseases (CTDs).
• Understanding the characteristics of PAH in CTD patients is crucial for managing complications.

Purpose of the Study:

• To delineate the clinical and echocardiographic features of patients diagnosed with pulmonary hypertension (PH) linked to CTDs.
• To identify differences in these characteristics between severe and non-severe PH cases.

Main Methods:

• A retrospective, observational study analyzed 35 patients with PH associated with CTDs.
• PAH was defined by echocardiography as pulmonary artery systolic pressure ≥ 40 mmHg.
• Patients were categorized into non-severe (40-64 mmHg) and severe (≥ 65 mmHg) PH groups.

Main Results:

• Diffuse scleroderma was the most frequent CTD (46%), with 89% of patients being female.
• Echocardiography revealed higher rates of right ventricular dilatation (85.7% vs 52.3%), hypertrophy (42.8% vs 0%), and hypokinesia (71.4% vs 9.5%) in severe PH patients.
• Pulmonary restriction was observed in 51% of patients, with no significant differences in gas exchange.

Conclusions:

• Severe PAH in CTD patients is strongly associated with echocardiographic evidence of right ventricular remodeling.
• Specifically, dilated, hypertrophic, and hypokinetic right ventricles are more common in severe cases of PAH associated with CTDs.