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One of the objectives of the Pulmonary Hypertension Associated with Congenital Heart Disease (PAH-CHD) Task Force is to bring up controversial issues to discussion. Two frequent problems in the management of children with PAH-CHD are: 1) to define the need for cardiac catheterization; 2) to decide which patients should undergo complete repair of the anomaly.
In developed countries, most children with congenital cardiac shunts are assigned to operation on the basis of noninvasive evaluation only. This is based on the assumption that patients can be considered as “at low risk” for immediate postoperative complications and late residual pulmonary hypertension if surgical treatment is offered early in life. Although this can be accepted as a “general rule”, early access to treatment may not be the reality in many underserved areas. Furthermore, young babies with certain anomalies (eg, truncus arteriosus, transposition of the great arteries and complete atrioventricular septal defect) may develop severe pulmonary hypertension early during the first months of life. Some patients with simple anomalies such as nonrestrictive ventricular septal defect or patent ductus arteriosus do not have congestive heart failure or failure to thrive in their clinical history, thus indicating increased pulmonary vascular resistance and absence of increased pulmonary blood flow.