RESUMO
BACKGROUND AND OBJECTIVES: Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality among patients with congenital heart disease (CHD). Earlier diagnosis of PAH would be of great benefit for the estimation of the CHD, the grasp of the indications for surgery and prognosis. PATIENTS AND METHODS: We assessed the diagnostic accuracy of Doppler echocardiography (D-ECHO) in detecting PAH in patients with CHD and the value of estimation about ventricular morphology and function of PAH-CHD patients. 123 CHD patients evaluated for suspected PAH were prospectively recruited. D-ECHO was performed and estimated right ventricular systolic pressure (RVSP) was measured to screen for PAH. Subsequently, pulmonary hemodynamic parameters were measured by right heart catheterization (RHC) for definitive diagnosis of PAH. RESULTS: RHC identified 66/123 (54%) patients with PAH. The noninvasive cut-point was: estimated right ventricular systolic pressure (RVSP) > 36.5 mm Hg by D-ECHO. D-ECHO classified 107 subjects correctly (sensitivity 89.4%, specificity 84.2%). The area under receiver-operating characteristic curve (AUC) was 0.96 for D-ECHO. A positive significant correlation (r = 0.853, p < 0.01) was found between RVSP measured by D-ECHO and systolic pulmonary arterial pressure (sPAP) measured by RHC. In addition, D-ECHO showed higher RVSP, left ventricular internal diameter (LV), right atrial diameter (RA), right ventricular internal diameter (RV), left ventricular end-diastolic diameter (EDD), left ventricular diastolic end-diastolic volume (EDV) and mitral velocity A wave (AMV) values in the PAH-CHD group than in the CHD group (p < 0.05). CONCLUSIONS: D-ECHO is not only an important noninvasive diagnostic technique for PAH-CHD patients, but also a tool which can indicate the ventricular remodeling and diastolic dysfunction induced by PAH to some extent.