Repair of interrupted aortic arch: A ten-year experience

Abstract

Eighty-two consecutive patients with interrupted aortic arch were referred to our institution between 1985 and 1995. Three died before any attempt at operation and 79 underwent surgical repair. Median age at operation was 9 days (range 1 day to 6 years) and median weight was 3.0 kg (range 1.8 to 20 kg). All but one were in severe congestive heart failure and 31.5% had oliguria or anuria. Preoperative pH varied between 6.8 and 7.4 (median 7.3). Sixty-nine received prostaglandin E1 infusion and 54 received mechanical ventilation. Aggressive preoperative ressucitation was necessary in 43 cases. Preoperative transfontanellar echography (performed routinely) since 1987 revealed intracerebral bleeding in six patients. Type A interrupted aortic arch was present in 37 cases, 41 patients had type B, and one had type C. Interrupted aortic arch was associated with single ventricular septal defect in 35 cases, 24 patients had associated complex heart defects, and 30 had significant subaortic stenosis (six had both subaortic stenosis and complex association). Aortopulmonary window was found in four patients, truncus arteriosus was found in eight, and transposition of the great arteries was found in five, double-outlet right ventricle was found in one, single ventricle was found in three, multiple ventricular septal defects were found in two and superior-inferior ventricles were found in one. Sixty-four patients underwent single-stage repair and 15 underwent multistage repair. Aortic arch repair consisted of direct anastomosis in 59 cases, patch augmentation in eight, and conduit interposition in 12. Ten patients underwent associated pulmonary artery banding and 19 underwent concomitant repair of complex associated lesions. The subaortic stenosis was addressed by four surgical techniques: myotomy or myectomy in five patients; creation of a double-outlet left ventricle, aortopulmonary anastomosis, and conduit insertion between the right ventricle and pulmonary artery bifurcation in four; no direct attempt to relieve the subaortic stenosis in six; and left-sided ventricular septal defect patch in 15. Mean duration of deep hypothermic circulatory arrest, crossclamp time, and cardiopulmonary bypass time were 38.8 +/- 15.6 min, 60.5 +/- 24.7 min, and 143 +/- 40.1 min, respectively. Postoperative mortality rate was 18.9% (70% confidence limits 14% to 24.6%), and overall mortality rate was 31% (70% confidence limits 20.9% to 42.2%). The results have improved with time, with an overall operative mortality rate of 12% since 1990. Univariate statistical analysis revealed that early survival was influenced by preoperative renal function, detection of cerebral bleeding by transfontanellar echography, the number of cardioplegic injections, and the date of operation. Multivariate analysis revealed that preoperative renal function and the number of cardioplegic injections were independent risk factors for early mortality. Echocardiographic measurements of the left heart-aorta complex with preoperative Z values as low as-4 demonstrated rapid ...

The Fate of Children with Microdeletion 22q11.2 Syndrome and Congenital Heart Defect: Clinical Course and Cardiac Outcome

“…The mortality rate found for our patients was similar to that reported for surgical repair of interrupted aortic arch (31%) [27], but slightly higher than that described for pulmonary atresia and ventricular septal defect (15-22%) [7,19] and for truncus arteriosus (13-17%) [6,28]. It should be noted that studies reporting the outcome of selected conotruncal malformations did not differentiate between patients with and those without the microdeletion 22q11.2, and therefore are not completely comparable with our study [6,9,27]. In fact, other authors demonstrated that monosomy of a segment within chromosome band 22q11.2 may represent a significant risk for perioperative death [1,18].…”

Cardiac defects and results of cardiac surgery in 22q11.2 deletion syndrome

“…Salem et al [2000] reported that preoperative hypoplasia of the aortic valve annulus predicts the development of left ventricular outflow tract obstruction after arch repair, whereas size of the subaortic region does not. Serraf et al [1996] documented a postoperative catch-up growth of left ventricular outflow tract. On the other hand, Geva et al [1993] found an indexed cross-sectional area of the left ventricular outflow tract less than 0.7 mm/m 2 but not aortic valve dimension to predict left ventricular outflow tract obstruction.…”

Management strategies for interrupted aortic arch with associated anomalies

“…Most reports highlight type B morphology as the most common form (52-90%) [4,5,7,8]. A higher incidence of type A morphology (62.5%) could be found in patients with associated truncus arteriosus (TA) [4,9].…”