Résumé
The main objective of this study is to describe patients' clinical characteristics and physiological and hemodynamic parameters at the time of diagnosis in a pulmonary hypertension center in Saudi Arabia. This study reports the results from a single pulmonary hypertension specialized center in Riyadh, Saudi Arabia, namely Prince Sultan Medical Military City/Cardiac Center [PSMMC and CC]. Both newly diagnosed [incidence] and referred [prevalence] cases of pulmonary arterial hypertension are included. All characteristics, including clinical, physiological, and hemodynamic parameters at the time of diagnosis are described. A total of 107 patients were identified as having pulmonary arterial hypertension as diagnosed by right heart catheterization. The mean age at diagnosis was 36 [ +/- 9] years, and there was a female preponderance of 62.6%. The mean duration between symptom onset and diagnosis was 27.8 [ +/- 9.0] months. At the time of enrollment, 56.1% of patients were in functional class III and 16.8% were in functional class IV. Fifty five patients [51.4%] were diagnosed as idiopathic pulmonary arterial hypertension, 29 patients [27.1%] as congenital heart disease associated with pulmonary arterial hypertension, 16 patients [15.0%] as connective tissue diseases associated with pulmonary arterial hypertension, 4 patients [3.7%] as heritable pulmonary arterial hypertension, and 3 patients [2.8%] as portopulmonary hypertension. This data highlights the current situation of pulmonary arterial hypertension in Saudi Arabia. Our patients are much younger than patients described in other international registries but still detected as late in the course of the disease. A majority of patients displays severe functional and hemodynamic compromise