Epidemiological and clinical characteristics of pulmonary artery hypertension (PAH) have never been described in Afro-Caribbean populations.

To describe the demographic, clinical, and hemodynamic characteristics of all patients with PAH followed in the dedicated Rare Diseases Centre for Pulmonary Hypertension of Martinique, and to compare them with those of the overall French Cohort.

A retrospective analysis of clinical and demographic data of all the adult patients referred for pulmonary hypertension in the Centre of Rare Diseases of the University Hospital of Martinique from January 2007 to November 2015.

During this 9 years period, 107 patients were diagnosed with pulmonary hypertension. Sixty-three of them had a pulmonary artery hypertension. Most of them were also women. Mean age at diagnosis was 56 years, and 71% of the patients were in III-IV WHO functional class. Mean pulmonary artery pressure and cardiac index at right heart catheterism were 43mmHg and 2.7 L/min/m², respectively. The most frequent causes were idiopathic pulmonary hypertension (28.8%), pulmonary artery hypertension associated with connective disease (16.7%), congenital cardiac diseases. The estimated prevalence was 113 per million inhabitants, 7,5 times higher than that of overall France. One-year survival was 84.1%. Compared to the French overall cohort, our patients were more likely to be women (sex ratio 3.9 versus 1.9, P=0.005) and older than 70 years at diagnosis (33.3% vs. 9.1%, P<0.001). A higher albeit non-significant frequency of connective tissue-associated pulmonary hypertension (22.7% versus 15.3%, P=0.07) was observed.

This first study of the epidemiological characteristics of pulmonary hypertension in an Afro-Caribbean region shows a high prevalence of disease, a diagnosis uncharacteristically done often in late stages of life, and a high frequency of connective tissue disorders.