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 syndrome,that is contraindication of surgery. What about patients with Moderate to severe PAH who do not have criteria of Eisenmenger 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 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 syndrome (90 patients), left- to- right shunt with PAH (48 patients), PAH with small defect (10 patients) and PAH after defect correction (44 patients). One, 5, 10, and 20 year survival was estimated for each group. Findings showed that patients with history of surgery in presence of PAH have worst outcome. And patients with Eisenmenger syndrome and those with PAH- associated with left- to- right shunt have 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 I present a young girl with history of CHD with PAH. She had been operated several years ago. When I admitted her, she was in a bad condition, with Functional class IV. Echo study showed severe PAH. Treatment of PAH with specific target PAH drugs started immediately, but these treatment were too late and patient expired in the hospital. Finally, I would like to pose the question”Wouldn’t it be better if the patient had not undergone the surgery in the first place”.