Congenital heart disease combined with pulmonary hypertension

  In congenital heart disease with left-to-right shunts, especially large defects if left untreated, almost all patients end up with pulmonary hypertension. Pulmonary hypertension is sometimes treatable, but if it progresses to the final stage, Eisenmenger’s syndrome at this time, the long-term survival of the patient is not even satisfactory.  The measure we are taking now is to have a very thorough and careful comprehensive examination of the patient with congenital heart disease with pulmonary hypertension. A complete evaluation is performed, including cardiac catheterization, to see if the extent of pulmonary vascular disease makes surgery possible. Some patients with congenital heart disease combined with pulmonary hypertension are indicated for surgery after examination; some patients are not indicated for surgery after examination; and some patients in between are treated with medication for the chance of surgery and then have surgery. In children with congenital heart disease, surgery is generally recommended before the age of one to prevent irreversible pulmonary hypertension.