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Objective We sought to determine the prevalence of outcomes and associated patient and management factors for neonates with interrupted aortic arch. Methods From 1987 to 1997, a total of 472 neonates were enrolled prospectively from 33 institutions. Competing risks methodology was used to determine simultaneous risk and associated incremental risk factors for death, initial and subsequent left ventricular outflow tract procedures, and arch reinterventions. Results Overall survival was 59% at 16 years after study entry but improved with successive birth cohort. In general, risk factors for death in each of the competing risks analyses included lower birth weight, younger age at study entry, type B interrupted aortic arch, and major associated cardiac anomalies. Of 453 patients who had interrupted aortic arch repair, after 16 years 33% had died and 28% had undergone an arch reintervention. Reintervention was more likely for those who had truncus arteriosus repair, interrupted aortic arch repair by a method other than direct anastomosis with patch augmentation, and the use of polytetrafluoroethylene as either an interposition graft or a patch. From study entry, competing risks after 16 years showed that 28% had died and 34% had undergone an initial left ventricular outflow tract procedure. Initial left ventricular outflow tract procedure was more likely for those with single ventricle, type B interrupted aortic arch, bicuspid aortic valve, or anomalous right subclavian artery. Among those who had undergone an initial left ventricular outflow tract procedure, after 16 years 37% had died and 28% had undergone a second procedure. Conclusion Anatomic features affect mortality and initial left ventricular outflow tract procedures, whereas characteristics of the arch repair affect arch reintervention.

Berry syndrome, which consists of distal aortopulmonary septal defect (APSD), aortic origin of the right pulmonary artery, interrupted aortic arch, intact ventricular septum, and patent ductus arteriosus, is an extremely uncommon congenital cardiac malformation, with only 25 cases having been reported since the first report in 1982. Here we report the first case of Berry syndrome complicated by anomalous origin of the left coronary artery from the main pulmonary artery. The possibility of coronary anomaly should be seriously considered in all cases of Berry syndrome.

Between September 1, 1982, and March 1, 1992, 40 patients underwent anatomic repair of transposition of the great arteries, ventricular septal defect, and aortic arch obstruction. In group I, 26 patients (65%) underwent repair in a two-stage procedure, phases A and B. Phase A included repair of the aortic arch obstruction with (16 patients) or without (10 patients) pulmonary artery banding through a left thoracotomy (mean age 18.7 +/- 23.4 days). There were three deaths and three reoperations. Phase B included an arterial switch operation with closure of the ventricular septal defect (mean age 95.5 +/- 122 days). There were five early deaths and two late deaths. Eight patients required reoperation. Mean delay between phase A and phase B was 77.5 +/- 109 days. In group I, there were eight early and two late deaths, and 11 patients required reoperation. The mean stay in the intensive care unit was 24.7 +/- 20 days. Mean follow-up of 59.6 +/- 21.4 months was completed in all survivors. All but one were in New York Heart Association class I without medication. Actuarial survival rate and rate of freedom from reoperation at 5 years were 57.5% and 49.9%, respectively. In group II, 14 patients (35%) had a one-stage procedure through midsternotomy: an arterial switch operation with closure of the ventricular septal defect and repair of the aortic arch obstruction (mean age 10.2 +/- 5.5 days). There were two early deaths (14.2%) and one late death after reoperation for overlooked multiple ventricular septal defects. Two patients required reoperation. The mean stay in the intensive care unit was 11.7 +/- 2.5 days. Mean follow-up of 22.4 +/- 16.7 months was achieved in all survivors. They were all in New York Heart Association class I without medication. Actuarial survival rate and rate of freedom from reoperation at 3 years were 78.5% and 81.5%, respectively. The one-stage procedure allowed complete repair in neonates without the need for multiple operations. We believe that it may decrease early mortality rates (14.2% versus 30.7%), reduce the reoperation rate and cumulative stay in the intensive care unit (11.7 days versus 24.7 days, p = Not significant), and significantly decrease the overall rate of morbidity (p < 0.01). However, requirements for surgical intervention with a one-stage or a two-stage procedure must include accurate assessments of intracardiac and aortic arch anatomy.

A palliative operation is described for the treatment of infants with all types of aortic arch interruption without regard to anatomic variation. It consists of establishment of continuity between the main pulmonary artery and descending thoracic aorta with a prosthetic graft, pulmonary artery banding distal to the graft, and ligation of the closing ductus arteriosus. Infant mortality rate in the past has been high, rega-dless of the type of operation used. The method of treatment described in this report is one in which an initial palliative operation is employed in the newborn period with total correction planned at a later date, when the risk of morbidity and death will be less.

Objective: Our goal was to investigate factors for mortality and cerebral outcome in patients with aneurysm of the aortic arch. Methods: From 1993 to 1996, 148 patients with aortic arch aneurysm underwent operations involving deep hypothermic circulatory arrest with retrograde cerebral perfusion. Age was 63.9 ± 11.6 years (mean ± standard deviation) and 52 patients were older than 70 years. Twenty-eight had acute aortic dissection. Twelve had ruptured aneurysms. Fourteen had redo operations. Seventy had aortic dissection. The aneurysms were caused by atherosclerosis in 123 patients and by other causes in 25. Median sternotomy was used in 92 and left thoracotomy in 56. Twenty-eight patients underwent replacement of the ascending aorta to the proximal arch, 62 had total arch replacement, 38 had distal arch replacement, 12 had simultaneous replacement of the distal arch and the descending aorta or thoracoabdominal aorta, and 8 had patch repair. Results: Fifteen (10.1%) early deaths occurred. New stroke occurred in six (4.0%) patients and transient delirium in 37 (25.0%). The duration of deep hypothermic circulatory arrest plus retrograde cerebral perfusion was 49 ± 17 minutes, and it was more than 60 minutes in 36 patients. Patients awoke 7.5 ± 8.2 hours after the operation. Logistic regression analysis demonstrated that risk factors for mortality were ruptured aneurysm, chronic obstructive pulmonary disease, arterial cannulation in the ascending aorta, and stroke. Risks for stroke were ruptured aneurysm and replacement of the distal arch. Risks for delirium were age older than 70 years and atherosclerotic aneurysm. Duration of circulatory arrest plus cerebral perfusion did not correlate with length of time before the patient regained consciousness. No difference was found in mortality, stroke, and delirium between patients with and those without more than 60 minutes of circulatory arrest and cerebral perfusion. Conclusion: Prolonged (>60 minutes) deep hypothermic circulatory arrest with retrograde cerebral perfusion was not a risk factor for mortality and stroke in patients who underwent surgery for aneurysms of the aortic arch. However, the prevalence of transient delirium necessitates further investigations. (J Thorac Cardiovasc Surg 1998;115:129–38)