Objectives: Closure of isolated secundum atrial septal defect is generally recommended at the age of 4 to 5 years. However, there are children with isolated secundum atrial septal defect in whom early closure should be performed. We aimed to assess the underlying conditions that led to earlier closure in this special patient group and to analyze the outcome.
Methods and results: From January 1990 through August 2002, 24 infants with isolated secundum atrial septal defect underwent surgical closure within the first year of life. All children were symptomatic. Signs of pulmonary hyperperfusion, such as tachydyspnea, failure to thrive, recurrent respiratory infections, or heart failure, were present. Four infants required artificial ventilation. Ten patients had additional problems, such as prematurity with chronic lung disease, hepato-omphalocele and congenital diaphragmatic hernia, which were present in 1 patient each. Eleven patients had defined dysmorphic syndromes. All but 1 infant underwent preoperative invasive hemodynamic evaluation. Thirteen patients had pulmonary hypertension preoperatively. The follow-up time was 46 +/- 33 months (range, 4-125 months). At follow-up, pulmonary artery pressure proved to be normal in 11 of the 13 children who had pulmonary hypertension previously. One patient died of persistent pulmonary hypertension. Clinical performance, growth, and development improved in nearly all patients. All ventilator-dependent children could be weaned shortly after atrial septal defect closure.
Conclusions: If lungs are compromised, even a minor left-to-right shunt might be poorly tolerated in infancy. In these children early surgical closure of an isolated secundum atrial septal defect should be performed to support thrive and growth and to prevent the onset of irreversible changes of the pulmonary vasculature.