Repair of persistent truncus arteriosus with interrupted aortic arch.

OBJECTIVE: The aim of our study was to analyse experience with repair of truncus arteriosus with interrupted aortic arch. METHODS: Between 1993 and 2004, eight consecutive patients underwent repair of truncus arteriosus with interrupted aortic arch. The median age was 6.5 days (range 1-85 days) and median weight was 3.2 kg (range 2.6-4.8 kg). Five patients had type A and 3 patients had type B aortic arch interruption. The repair was performed in deep hypothermia with circulatory arrest in 4 patients and isolated selective low-flow perfusion of the head and the heart in the last 4 patients. The repair consisted in aortic arch reconstruction by direct anastomosis between descending and ascending aorta, closure of ventricular septal defect and reconstruction of the right ventricular to pulmonary artery continuity using a valved conduit. RESULTS: One (12.5%) patient died from sepsis and hepato-renal failure 18 days after surgery. Seven (87.5%) patients were followed up for 2.0-11.7 years (median 2.6 years). No patient died after the discharge from hospital. In 4 patients 1-3 reinterventions were required 0.6-10.0 years after repair. Reoperations were performed for conduit obstruction in 2 patients, aortic regurgitation in 2 patients, right pulmonary artery stenosis in 2 patients and airway obstruction in 1 patient. In 2 patients concommitant aortic valve and conduit replacement was required. Balloon angioplasty for aortic arch obstruction was necessary in 1 patient, and for bilateral pulmonary branch stenosis in 1 patient. Five (28.6%) surviving patients are in NYHA class I and 2 (71.4%) patients are in NYHA class II. CONCLUSIONS: Primary repair of persistent truncus arteriosus with interrupted aortic arch can be done with low mortality and good mid-term results. Aortic arch reconstruction in isolated low-flow perfusion of the head and the heart influences favourably the postoperative recovery. The main postoperative problems are associated with conduit obstruction and aortic insufficiency.     

Surgical treatment of persistent fifth aortic arch associated with interrupted aortic arch

This study describes two cases of the rare congenital anomaly, persistent fifth aortic arch. Both cases involve boys (1 at 9 years of age and another at 7 months of age). To detect persistent fifth aortic arch with interrupted aortic arch, the following methods were used: echocardiogram, angiocardiography, and magnetic resonance imaging. In both cases the blood pressure between the upper and lower limbs differed. To relieve the obstruction of blood flow, each case was surgically repaired using patching or conduit interposition. Postoperative courses were uneventful. Two-year to 5-year follow-up examinations were positive showing that the anastomosis was unobstructed and the velocity of blood flow to the descending aorta was normal.     

Successful surgical repair of truncus arteriosus with interrupted aortic arch in infancy by an anterior approach

A 98-day-old infant was successfully operated on for truncus arteriosus (type I) with interrupted aortic arch (type B) using a one-stage anterior approach. The interrupted aortic arch was reconstructed by direct anastomosis between the ascending and descending aorta. The ventricular septal defect was closed, and a 12-mm porcine valved conduit was placed on the right side of the ascending aorta to establish continuity between the right ventricle and pulmonary artery. Surgical procedures for the repair of this malformation are the focus of discussion.     

Truncus arteriosus with interrupted aortic arch: successful repair using modified cardiopulmonary bypass and surgical techniques

A neonate with truncus arteriosus (type 1) and interrupted aortic arch (type A) associated with an excessively large gap between interrupted aortic segments underwent successful reconstruction of the aortic arch with a partial subclavian flap aortoplasty plus bovine jugular patch (Contegra 200, Medtronic, Inc, Minneapolis, MN) using continuous selective low-flow cardiopulmonary bypass without circulatory arrest. The ventricular septal defect was closed with a Dacron (IMPRA, Inc, Tempe, AZ) patch, and a Contegra prosthesis was used to re-establish right ventricle to pulmonary artery continuity. At an 18-month follow-up, the patient was free of symptoms with normal development. Echocardiography revealed a widely patent aortic arch and an unobstructed right ventricle to pulmonary artery connection.     

Reverse subclavian flap and aorto-pulmonary window technique for repair of interrupted aortic arch and truncus arteriosus

We report a surgical strategy for repairing an interrupted aortic arch (IAA) with truncus arteriosus (TA) by using a reverse subclavian flap and an aorto-pulmonary (A-P) window technique for preserving the pulmonary artery architecture. A 10-day-old neonate with type B IAA and type I TA with echocardiographic evidence of a significant distance between the ascending and descending aorta underwent surgical repair at the Bristol Royal Hospital for Children. The superior part of the arch was reconstructed using a reverse subclavian flap and the undersurface with a pulmonary homograft patch. The ascending aorta was separated from the pulmonary arteries using a Gore-Tex patch (A-P window type of repair) without disconnecting the branch pulmonary arteries, in order to preserve their architecture. The continuity between the right ventricle and the pulmonary artery bifurcation was established using a 12 mm Contegra conduit. The postoperative course was uneventful, and the neonate was discharged after 12 days. At follow-up, the patient remains well, gaining weight, with no echocardiographic evidences of obstruction. Reverse subclavian flap with homograft patch combined with and 'A-P window' technique for preservation of the pulmonary artery architecture is a useful and effective surgical strategy for neonates presenting with IAA associated with TA.     

Truncus arteriosus with coarctation of persistent fifth aortic arch

Truncus arteriosus is a rare form of congenital heart defect. Among cases, 10% to 20% are associated with arch anomalies, such as interruption, coarctation, or patent ductus arteriosus. We treated a rare case of combined truncus with complex arch anomaly. The patient was a 2-month-old girl weighing 3.8 kg. Echocardiogram and computed tomogram showed interrupted aortic arch, truncus arteriosus, persistent fifth aortic arch, and its coarctation. One-stage total correction was performed under cardiopulmonary bypass without circulatory arrest. The operation consisted of a Rastelli procedure with a 12-mm valved conduit, coarctoplasty, and intraventricular baffling. The patient recovered well without complications.     

Repair of the truncal valve and associated interrupted arch in neonates with truncus arteriosus

OBJECTIVE: Truncal valve regurgitation and interrupted aortic arch have frequently been identified as risk factors in the repair of truncus arteriosus. We wished to examine these factors in the current era including the impact of truncal valve repair. METHODS: Between January 1992 and August 1998, 50 patients underwent surgical repair of truncus arteriosus. Their ages ranged from 2 days to 6 months (median, 2 weeks). Nine patients had associated interrupted aortic arch. Of the 14 patients (28%) in whom truncal valve regurgitation was diagnosed preoperatively, 5 had mild regurgitation, 5 had moderate regurgitation, and 4 had severe regurgitation. Five underwent truncal valve repair and 1 underwent homograft replacement of the truncal valve with coronary reimplantation. RESULTS: The actuarial survival was 96% at 30 days, 1 year, and 3 years. There were no deaths in patients with associated interrupted aortic arch. The 2 deaths in the series occurred in patients with truncal valve regurgitation, neither of whom underwent repair. Postoperative transthoracic echocardiography in patients who underwent valve repair showed minimal residual valvular regurgitation. None of the patients has required reoperation because of truncal valve problems or aortic arch stenosis at a median follow-up of 23 months (range, 1-60 months). Conduit replacement has been done in 17 patients (34%) after a mean duration of 2 years. The freedom from reoperation for those who had an aortic homograft was 4 years and for those who had a pulmonary homograft was 3 years. CONCLUSION: Despite the magnitude of the operation, excellent results can be achieved in complex forms of truncus arteriosus. In the current era interrupted aortic arch is no longer a risk factor for repair of truncus. Aggressive application of truncal valvuloplasty methods should neutralize the traditional risk factor of truncal valve regurgitation.     

Associated factors and trends in outcomes of interrupted aortic arch

Background

Interrupted aortic arch (IAA) continues to be associated with important mortality, both before and immediately after repair, with ongoing morbidity during follow-up. We sought to determine trends in presentation, management, outcomes and associated factors.

Methods

We reviewed all consecutive patients (n = 119) presenting from 1975 to 1999, and data were collected regarding demographics, anatomy, management and outcomes.

Results

Significant trends over time for patients born in three consecutive periods (1975 to 1984, 1985 to 1993, and 1994 to 1999) demonstrated a smaller proportion of patients with presentation with circulatory collapse (65%, 51%, and 25%, respectively), greater use of prostaglandins (72%, 90%, 100%), fewer deaths without IAA repair (49%, 15%, 13%) and greater use of one-stage repair (68%, 75%, 100%). Independent risk factors for death without IAA repair (p < 0.001) included absence of ventricular septal defect, and the presence of noncardiac anomaly, complex cardiac anomaly, episode of acidosis and earlier birth cohort. Overall survival after repair was 50% at age 1 month, 35% at 1 year, and 34% at 5 years. Early and constant-hazard phases were noted, with incremental risk factors for early phase mortality being cyanosis at presentation, presence of truncus arteriosus or aortic stenosis, an episode of circulatory collapse before repair, earlier date of repair, and lower weight at repair. Greatest survival occurred in those patients with uncomplicated IAA who had repair since 1993 (5 year survival, 83%). Freedom from reintervention for arch obstruction was 60% at 5 years.

Conclusions

While improving, outcomes of IAA remain of concern, especially in patients with associated lesions.     

Truncus arteriosus with interrupted aortic arch: successful correction in a neonate

Truncus arteriosus (type II) with interrupted aortic arch (type B) was successfully repaired at 11 days of age using anterior translocation of the pulmonary arteries, resection of the ductus arteriosus, and direct anastomosis between the descending aorta and truncus. This technique permitted wide reconstruction of the aortic arch and minimized the number of suture lines. It also positioned the right ventricle-pulmonary artery conduit anteriorly, which may simplify its subsequent replacement.     

Spontaneous closure of ductus arteriosus in interrupted aortic arch with ventricular septal defect

A 2-month-old boy diagnosed with interrupted aortic arch type B was treated with a two-stage procedure. His ductus arteriosus had closed spontaneously. Collaterals via both vertebral arteries developed. A 15-mm stenotic segment existed between the left subclavian artery and the descending aorta. The direct anastomosis between the common carotid artery and the descending aorta was performed as a first palliation at the age of 3 months. The left subclavian artery was reconstructed by end-to-side anastomosis to the descending aorta. The postoperative course was uneventful. The closure of ventricular septal defect and pulmonary artery debanding were performed as a second operation 4 months after the first palliation. The patient is alive and well 7 months after the second operation.     

Truncus arteriosus with interrupted aortic arch: report of a successful surgical repair

The association of truncus arteriosus with interrupted aortic arch represents a formidable surgical challenge. Two successful repairs have been reported, but none for the past ten years. This report presents a third successful repair using a technique that allows the widely patent ductus arteriosus to maintain continuity between the truncus (with pulmonary arteries detached) and the descending aorta. Right ventricle-pulmonary artery continuity is established in the usual way with a porcine-valved conduit. While long-term potential difficulties with this approach are recognized, it appears to give satisfactory initial palliation and to be an acceptable method of treatment for this combination of defects. The embryology and the anatomy of the lesion are briefly discussed.     

One-stage correction of interrupted aortic arch combined with heart-lung transplantation

A technique for correction of interrupted aortic arch during heart-lung transplantation is described. This operation was performed in a 14-year-old patient with situs solitus, absent right atrioventricular connection, large atrial septal defect, ventricular septal defect, transposition of the great arteries, type B interrupted aortic arch, and severe pulmonary hypertension. The postoperative course was uneventful. Clinical, radiologic, and nuclear magnetic resonance investigation have shown evidence of good correction of the aortic arch anomaly. We concluded that donor tissue available during heart-lung transplantation is useful in correcting extracardiac anomalies.     

Aortic atresia and type B interrupted aortic arch: diagnosis by physiologic cerebral monitoring

Physiologic cerebral monitoring has become an important part of our cardiovascular surgical unit. We recently encountered an unusual variant of aortic atresia that was first suggested by physiologic cerebral monitoring and required modification of our operative technique. We describe and discuss the anatomy, its translation into cerebral monitor findings, and how we modified our operative technique.