Document Type : Original Article
Department of Pediatric Cardiology, Shahid Beheshti University of Medical Sciences, Tehran, IR Iran
Background: Pulmonary arterial hypertension (PAH) is a serious complication of unrepaired congenital left-to-right shunts. The final consequence is right ventricular (RV) systolic dysfunction and reversal of shunt. Objectives: The aim of this study was to evaluate the clinical course and paraclinical findings in a group of patients with PAH associated with congenital heart disease (PAH-CHD). We also compared the results obtained by echocardiographic technique used for assessment of RV systolic function (Tricuspid annular plane systolic excursion TAPSE) with parameters obtained by diagnostic cardiac catheterization. Patients and Methods: During a one-year period (2011-2012), all patients with confirmed diagnosis of PAH-CHD in pediatric cardiology ward of a teaching hospital enrolled in the study. They underwent through echocardiographic assessment and cardiac catheterization. Results: Forty-one patients (22 males, 19 females) with mean age of 5.5 ± 5.5 years (4 months to 18 years) entered in the study. Ventricular septal defect (VSD) was the most common (65.8%) underlying CHD. Patients ≤ 10 years old comprised 71, 8% of subjects. The higher level of Tricuspid regurgitation (TR) was correlated with higher mean pressure of pulmonary artery (PAP), and greater resistance ratio (Rp/Rs) (P = 0.001, and P = 0.044 respectively). Indexed TAPSE (TAPSE/body surface area) had a significant reverse correlation with mean PAP (P = 0.015), and Rp/RS (P = 0.002). There was a significant reverse correlation between indexed TAPSE and TR (P = 0.032). Conclusions: TAPSE is an easy echocardiographic measurement and reflected RV systolic function. Indexed TAPSE has good correlation with hemodynamic data obtained by cardiac catheterization. TAPSE index is recommended for evaluation of RV systolic function in pediatric patients with PAH-CHD.