Evaluation of Pulmonary Hypertension In First Degree Relatives Of Patients With Primary Pulmonary Hypertension

Background: Pulmonary hypertension (PH) was defined for the first time in 1951 as primary pulmonary hypertension (PPH). Some studies emphasized on the role of genetics in the development of pulmonary hypertension in family members of affected patients. So, in this study we evaluated the prevalence of pulmonary hypertension in first degree family of patients with documented PPH. Methods: In this cross-sectional analytic study 51 subjects (first relatives of patients with PPH, including parents, sisters, brothers and children were enrolled. Echocardiography and EST was done and the pulmonary artery pressure (PAP) before and after the test was compared. Results: A total of 27 subjects, 17 women and the 10 men were evaluated in 3 groups and based on PAP before exercise, 22 (81.5%) of participants had normal PAP and 5 (18.5%) were in intermediate group. After the exercise test 55.5% were in normal group, 33.3% in intermediate group and 11.1% had high PAP. Conclusion: We recommend to screen and evaluate the first degree relatives of these patients as high risk group.