Repair of interrupted aortic arch in infancy

Twenty-one patients undergoing repair of interrupted aortic arch between December of 1979 and January of 1987 were reviewed to determine the cause(s) of late morbidity and mortality. Ten underwent staged repair, and 11 had complete repair including all coexisting defects at the initial operation. Sixty-two percent are alive and clinically well 6 months to 6 years after the initial operation. Among the five patients who died late postoperatively, four had severe left ventricular outflow tract obstruction. Two other patients have had surgical relief of severe subaortic stenosis. In addition, significant recurrent or residual coarctation was found in four patients; it was relieved by balloon angioplasty in two patients, and two had surgical repair. None of the most recent seven patients, however, have had a residual ascending-descending aortic gradient. Careful follow-up for the detection of previously masked or newly developed left ventricular outflow tract obstruction is imperative and may be lifesaving.