Pulmonary Hypertension Registry: a single-center experience in Egypt

Abstract

Introduction Pulmonary hypertension is a life-threatening progressive disease that eventually leads to disability and death. Aim To establish the first single-center registry for pulmonary hypertension in Egypt, Kasr Al-Ainy Hospitals, Cairo University, to characterize the pattern of disease burden in terms of survival. Patients and methods Patient enrollment was performed over 2 years with a 5-year follow-up, and the following were recorded: demographic data, clinical presentation, hemodynamic changes, and the 1-, 3-, and 5-year survival rates in groups I and IV, who were potentially treatable. Results A total of 104 patients were enrolled, 65 patients completed the investigational panel, including right heart catheterization. A total of 54 (83.1%) patients belonged to group I, and 11 (16.9%) patients belonged to group IV. Idiopathic pulmonary arterial hypertension was present in 38.5% of the patients, bilharzial pulmonary arterial hypertension in 15.4%, and connective tissue diseases in 18.5%. The mean age was 37.8±12 years; the male : female ratio was 1 : 2.4. Of the patients, 85% presented with functional class II or III disease, and the mean 6-min walk distance was 256±98 m. The hemodynamic parameters were: mean pulmonary artery pressure: 47±10 mmHg; cardiac index: 2.29±0.5; pulmonary vascular resistance: 11±4; and mixed venous oxygen saturation: 59.6±7. Conclusion Idiopathic pulmonary arterial hypertension is the most common cause of pulmonary arterial hypertension in Egypt. Patients with connective tissue diseases have delayed referrals, leading to poor hemodynamic parameters and survival. Bilharziasis is not currently common. The 1-, 3-, and 5-year overall survival rates of group I were 90.9, 81.5, and 75.93%, respectively.

Keywords

• egypt
• pulmonary hypertension
• registry
• right heart catheterization
• survival