Importance of early management of pulmonary artery hypertension among Adult with Congenital Heart Disease (ACHD)

Abstract

Background Pulmonary artery hypertension (PAH) is a significant risk factor in patients with congenital heart disease. If left untreated, PAH can lead to reduced quality of life and functional class due to pulmonary vascular damage and right ventricular failure (RV).The prevalence of PAH in ACHD is estimated to be between 5% and 10%. In severe cases of PAH, treatment options are generally limited to palliative measures or heart–lung transplant. Early treatment of PAH improves quality of life and may reduce utilization of acute care facilities. Methods The King Abdulaziz Echocardiography database was used to search for patients with ACHD over age 12. A sample of 378 patients meeting these criteria were selected for review. Demographic and echocardiography data were extracted which include first and last pulmonary artery pressure, LV ejection fraction and RV function. Results Forty percent of all patients had PAH (as defined more than peak 39 mm Hg by Echo) in comparison with other international reports which is only 5–10%. Sixty percent of patients with PAH had cardiac surgery, 20% had significant residual defect. Twelve percent of patients were reported to have moderate or severe RV dysfunction as a result of PAH. Conclusions Impaired RV function is one of the most common complications of PAH. If PAH is treated early enough, RV failure and pulmonary vascular damage may be avoided. Most of our patients with PAH (88%) presented with normal or mildly impaired RV function. They are good candidates for PAH preventive treatment.