BACKGROUND: Pulmonary arterial hypertension (PAH) is a recognized complication of congenital heart defects with chronic left-to-right shunting, and the status of the pulmonary vascular bed often is the principal determinant of the clinical manifestations, course, and feasibility of surgical treatment as well as in most instances, it is reversible if detected early and the shunt is corrected. In some instances, however, pulmonary hypertension develops very rapidly at the early stages of the disease and precludes any surgical correction.
This study was conducted to: (1) determine the prevalence of pulmonary arterial hypertension in congenital heart disease specifically with left-to-right shunts; (2) determine if surgery and other medical treatment would improve the pulmonary hypertension; and (3) describe the clinical profile of patients with pulmonary hypertension secondary to congenital heart disease.
A total of 252 patients aged 2 months to 19 years were included In this study. Most of them (59.5 percent) were females. The cardiac lesions seen in this study included 22 ASD (8.7 percent), 99 VSD (39.3 percent), and 131 PDA (52 percent). Among these subjects, 30.2 percent had mild pulmonary hypertension, 51.6 percent with moderate pulmonary hypertension, and 18.3 percent with severe pulmonary hypertension as measured by 2D-echocardiography. There were only 37 patients (15 percent) who underwent cardiac catheterization. Pulmonary artery pressure (PAP) had a mean value of 48.91 +/- 13.83 mmHg by 2D-echocardiography and 78.84 +/- 17.88 mmHg by cardiac catheterization. Pulmonary artery pressure decreased after definitive cardiac surgery as seen when PAP was measured by 2D-echocardiography at different periods of time namely: (1) 2 wks to 1 month post-operative, (2) 6 months to 1 year post-operative, and (3) beyond 1 year after surgery. However, no normalization of the PAP was noted. Other medications given were vasodilators, anticoagulants and oxygen. Of the 252 patients, 163 (65 percent) had pulmonary function test. Fifty-six percent (92 of 163) showed a restrictive ventilatory defect and 34 percent (55 of 163) had an obstructive ventilatory defect. Comparing the presence of PAH and lung function, there was no correlation.
1) To determine the prevalence of pulmonary arterial hypertension in acyanotic congenital heart disease with left-to-right shunt.
2) To describe the clinical profile of patients with pulmonary hypertension secondary to acyanotic congenital heart disease with left-to-right shunt.
3) To determine if surgery can reverse or decrease the degree of pulmonary hypertension.
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