Congenital Heart Disease with Pulmonary Hypertension; Surgery or Medical Treatment?

Pulmonary arterial hypertension (PHA) is a frequent complication of congenital heart disease (CHD), most commonly in systemic-to-pulmonary shunt lesions. In patients with an uncorrected left-to right shunt, PAH will end up to Eisenmenger’s syndrome, which is contraindication of surgery. What about patients with moderate to severe PAH, who do not have criteria of Eisenmenger’s syndrome; immediate cardiac repair is preferred or medical treatment? Heart catheterization and evaluation of pulmonary artery reactivity test or lung biopsy might be helpful to find presence of Eisenmenger’s syndrome. Although these guidelines are not always helpful or possible; look at the following article will show how important this subject is. 192 patient with CHD and PAH were evaluated in 4 groups, Eisenmenger’s syndrome (90 patients), left-to-right shunt with PAH (48 patients), PAH with small defect (10 patients) and PAH after defect correction (44 patients). 1-, 5-, 10-, and 20-year survivals were estimated for each group. Findings showed that patients with history of surgery in presence of PAH had worst outcome; and patients with Eisenmenger’s syndrome and those with PAH-associated left-to-right shunt had best prognosis. One of the reasons of this result is presence of a cardiac defect which allows a pulmonary-to-systemic shunt which can maintain the cardiac output in the end stage of disease. Finally, we present a young girl with history of CHD with PAH. She had been operated several years ago. When admitted, she was in a bad condition, with functional class of IV. Echocardiography showed severe PAH. Treatment of PAH started with specific target PAH drugs immediately, but these treatments were too late and patient expired in the hospital. Finally, we would like to pose the question ”Would not it be better if the patient had not undergone the surgery in the first place?”.