Surgery is the mainstay of treatment for congenital heart disease, and the expected results can be classified as follows:

  • Curative: Patients with these conditions rarely have long-term sequelae after surgical correction in childhood. These conditions are patent ductus arteriosus, secundum atrial defect, and uncomplicated ventricular septal defect.

  • Reparative: Patients with these defects are improved after corrective surgery, yet have lifelong sequelae, and some proportion will have significant late impairment. These defects are aortic stenosis, atrioventricular canal, coarctation of the aorta, partial anomalous pulmonary venous return, pulmonary stenosis, tetralogy of Fallot, total anomalous pulmonary venous return, d-transposition of the great arteries, and l-transposition (also called congenital corrected transposition of the great arteries).

  • Palliative: Surgery in these patients (if done) does not fully correct the underlying defect, so they are likely to have significant lifelong impairment of function. These defects are Eisenmenger syndrome, hypoplastic left heart syndrome, malaligned atrioventricular canal with single ventricle repair, single ventricle, tricuspid atresia, and unrepaired cyanotic heart disease.

EPIDEMIOLOGY

Eight out of 1,000 infants are born with congenital heart disease. Of these, approximately 25 percent require immediate surgical or catheter-based intervention (Ferencz et al., 1993). Even with treatment, the lifespan of individuals with congenital heart disease is limited compared with their peers; 89.5 percent of individuals with congenital heart disease are alive at age 20, but for some diagnoses (e.g., truncus arteriosus and single ventricle), the survival is much poorer (Tennant et al., 2010). Survival in individuals with congenital heart disease who reach adulthood is reduced (Verheugt et al., 2010). Death is commonly due to heart failure or sudden death, and risk is increased by arrhythmia, endocarditis, myocardial infarction, and pulmonary hypertension.

In 2000, 1 million adults in the United States were living with congenital heart disease. An estimated half of them had relatively simple residual disease, one quarter had moderately complex residual disease, and one quarter had severe residual disease (Warnes et al., 2001). However, functional disability is not limited to those with “severe” disease, as even an individual with “simple” disease may be disabled due to a complication.



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