Single-stage repair of acute type A aortic dissection associated with aortic coarctation, perimembranous ventricular septal defect, and bicuspid aortic valve

A 17-year-old man with coarctation of the thoracic aorta, bicuspid aortic valve, perimembranous ventricular septal defect, and acute type A dissection of the ascending aorta underwent emergency repair for dissection by means of single-stage replacement of not only the aortic valve and ascending aorta, but also simultaneous repair of the coarctation by graft interposition. These combined conditions comprised some difficulties that included decisions on the optimal timing and sequence of surgical repair, technique, and circulatory support.     

Bilateral minithoractomy repair of aortic coarctation with ventricular septal defect and persistent ductus arteriosus

A 6-year-old female child with aortic coarctation, a ventricular septal defect, and a persistent ductus arteriosus underwent surgical repair by bilateral minithoracotomies. At 6-month follow-up, the patient had recovered well, with increased exercise tolerance. Transthoracic echocardiography revealed an intact ventricular septum and no significant gradient across the aortic repair.     

Outcome following single-stage repair of coarctation with ventricular septal defect.

OBJECTIVE: A recent multi-institutional study suggested improved survival (97%) after staged repair of coarctation with ventricular septal defect (VSD) compared with single-stage repair. The current study was undertaken to determine outcome and need for reintervention following single-stage repair of coarctation and VSD at our institution. METHODS: Retrospective review of patients undergoing single-stage repair of coarctation with VSD between October 1, 1994 and August 15, 1999. RESULTS: Single-stage repair of coarctation with VSD was performed in 25 infants (12 males, 13 females) at a median age of 12 days (range 1-87 days) and median weight of 3.3 kg (range 1.3-4.4 kg). The VSD was conoventricular in ten patients, malalignment type with posterior deviation of the infundibular septum in ten, muscular in four and conal septal hypoplasia in one. Arch hypoplasia was present in all patients and bicuspid aortic valve in 13. At least moderate subaortic narrowing was present in six patients (secondary to prolapse of tricuspid valve tissue across the VSD in four). Repairs were performed via a median sternotomy with a mean circulatory arrest time of 38+/-12 min. Overall patient survival was 96% with one operative death and no late deaths at a median follow-up of 16 months (range 1-50 months). Reinterventions included balloon dilatation of recurrent coarctation (five), closure of residual VSD (one) and Ross-Konno procedure (two). Actuarial freedom from reintervention for the hospital survivors was 81% (95% confidence limit (CL) 61%, 92%) at 6 months, 71% (95% CL 47%, 87%) at 1 year and 59% (95% CL 31%, 82%) at 2 years following surgery. CONCLUSION: Single-stage repair of coarctation with VSD can be performed with low operative mortality and good midterm survival equivalent to reported results for staged repair.     

Outcome of single-stage repair of coarctation with ventricular septal defect

Abstract Background: The optimal surgical strategy for coarctation of the aorta (CoA) with ventricular septal defect (VSD) is controversial. The aim of this study is to evaluate the clinical outcome of a single‐stage repair of CoA with VSD. Methods: We reviewed 72 patients who underwent single‐stage repair for CoA with VSD between January 1995 and December 2007. There were 43 males and 29 females. The median age of the patients was 28 days (range = 3 to 188) and median weight was 3.7 kg (range = 2.16 to 5.6). Deep hypothermic circulatory arrest was used in 22 patients and selective antegrade cerebral perfusion was performed in 43 patients. Results: There were no operative deaths and one late death at a median follow‐up of 60 months (range = 16 to 158). Postoperative complications were left main bronchus compression requiring aortopexy in one patient and recoarctation requiring balloon dilatation in one patient. Subaortic stenosis occurred in two patients and surgical repair was performed. Conclusions: One‐stage simultaneous repair can be performed with a low risk at an early age. (J Card Surg 2011;26:420‐424)     

Single-stage repair of complete atrioventricular septal defect and coarctation of the aorta in neonate

Controversy over patients with complete atrioventricular septal defect (CAVSD) and coarctation of the aorta (CoA) continues: whether they should be treated with a primary total correction or a staged approach utilizing initial pulmonary artery banding during neonatal period. A 17-day-old neonate with Down’s syndrome underwent definitive repair of CAVSD and CoA. With the postoperative course uneventful, he was discharged from the hospital on 19th postoperative day. Single-stage repair of CAVSD and CoA appears to offer a good prognosis provided that valve morphology is amenable to repair.     

Single-stage off-pump repair of coarctation of the aorta and ventricular septal defects in children

Open in a separate window OBJECTIVESThe appropriate approach for surgical repair of coarctation of the aorta with a ventricular septal defect (VSD) remains controversial. This study evaluated the outcomes of primary repair of VSDs with periventricular device closure without cardiopulmonary bypass through a left thoracotomy in patients without arch hypoplasia.METHODSWe selected 21 patients aged <1 year, including 7 neonates, who underwent repair of coarctation of the aorta with periventricular device closure of a VSD.RESULTSThe median occluder size was 6 (range, 5–8) mm. The median mechanical ventilation time was 14 (range, 2–68) h, and the median duration of hospital stay was 11 (range, 7–16) days. No reoperations were required to correct VSD shunting, and the median residual shunt size was 1 (range, 1–2) mm. The median follow-up period was 13 (range, 4–31) months. No late deaths were reported, and no haemodynamically significant pressure gradient at the anastomotic site was observed. The median distal aortic arch z-score was 0.39 (range, −0.1–to 0.9). Only 1 patient had a permanent pacemaker implanted towards the end of the follow-up period.CONCLUSIONSPeriventricular device closure can be used safely for closure of VSD in children with coarctation of the aorta without a hypoplastic aortic arch, even in neonates, to reduce the risk of prolonged cardiopulmonary bypass. This hybrid approach can be performed with a low incidence of rhythm disturbances and residual shunting. However, a meticulous assessment of the VSD anatomy is essential to avoid any unfavourable events.     

Single-stage repair of complete atrioventricular septal defect

We report our experience with 31 consecutive children who underwent single-stage repair of complete atrioventricular septal defect between 1984 and December 1991. Ages ranged from 2 months to 2.5 years, mean 11 months. 18 were classified Rastelli type A, 13 type C. 22 patients had Down's syndrome, 12 were Rastelli type C. 1 patch was used in Rastelli type A cases and 2 patches in type C patients, without incision of the atrioventricular valve tissue. In all but 1 case the left superior and inferior valve leaflets were approximated. 5 patients died postoperatively resulting in an overall mortality of 16.1%. In all survivors, good clinical results and sinus rhythm were seen, although all show some degree of mitral incompetence.     

Late results after repair of aortic insufficiency associated with ventricular septal defect.

The late results of 70 patients aged 1.96 to 35.9 (mean 10.1) years who had repair of ventricular septal defect and aortic insufficiency from 1968 to 1988 were reviewed. The ventricular septal defect was subcristal in 50 and subpulmonary in 20 patients. Two thirds were situated immediately below some part of the right coronary leaflet with prolapse of that leaflet. Most of the remainder were below the right commissure or the anterior part of the noncoronary leaflet with prolapse of one or both adjacent leaflets. Associated structural defects, usually including some fusion at a commissure, were present in 18 of the 70 patients and occurred more often with a ventricular septal defect in or below the commissure between the right and noncoronary leaflets (p less than 0.001). Follow-up ranged from 1.9 to 19.6 (mean 9.8) years. There were no early deaths or cases of atrioventricular block, but there were two late deaths. Patient survival rate was 96% at 10 years. Freedom from valvuloplasty failure and freedom from reoperation were 76% and 85%, respectively, at 10 years. The major predictor for failure by multivariate analysis was the presence of an associated structural defect (p less than 0.01). Age at repair and position of the ventricular septal defect were not significant risk factors. We conclude that aortic valvuloplasty produces good palliation in most children. The few failures occurred early and chiefly in patients with associated structural valve defects that occurred more frequently in children who had a ventricular septal defect in the right commissure, where both the right and noncoronary leaflets may be affected.     

Primary definitive repair of interrupted aortic arch with ventricular septal defect

The optimal surgical management (primary or staged repair) of interrupted aortic arch (IAA) with ventricular septal defect (VSD) remains to be determined. A consecutive series of 14 neonates, aged 3-18 days (mean: 10 +/- 6 days) underwent primary complete repair. Mean weight was 3.3 +/- 0.4 kg. Eleven patients had IAA type B, 2 had type A and 1 had type C. Six infants had the Di George syndrome. Preoperative management (mean: 5 +/- 4 days) included prostaglandin E1 (14/14), intubation and ventilation (13/14), and inotropic support (11/14). Surgery was performed under deep hypothermia and circulatory arrest and involved resection of all ductal tissue, direct end-to-side aortic arch anastomosis and patch closure of the VSD. There were 2 early deaths (14%, 70% CL: 5%-31%): low cardiac output (1), residual VSD (1). Four patients (33%, 70% CL: 13%-52%) underwent reoperation for recurrent aortic obstruction (3 patients, 1 death) or left ventricular outflow tract obstruction (LVOTO) (1 patient). The results improved with time: no death and no recurrent aortic obstruction in the last 8 patients. At last follow-up (11 patient, mean follow-up = 24 +/- 9 months), all patients were free of cardiac symptoms; none had persistent aortic obstruction; 4 had LVOTO (gradient greater than 20 mm Hg) and 1 (with the Di George syndrome) had severe mental disorders. Primary complete repair provides satisfactory results in most infants born with IAA and VSD. An adequate direct aortic arch anastomosis should entail a low risk of recurrent obstruction. LVOTO develops in many cases and may require further surgery.     

Single-stage repair of aortic coarctation with concomitant bicuspid aortic valve and ascending aortic aneurysm

The optimal surgical management of patients presenting with an aortic coarctation together with other cardiovascular disorders is unclear. In this study, we report the case of an adult male with an aortic coarctation associated with a bicuspid aortic valve and an ascending aortic aneurysm. The patient underwent single-stage repair involving the Bentall technique and total arch replacement combined with stented elephant trunk implantation, which was performed through median sternotomy. We consider this procedure to be a suitable alternative for treating these complex cases.     

Management of infants with coarctation and ventricular septal defect

The management of patients with aortic coarctation and ventricular septal defect (VSD) remains controversial. A 2-stage repair uses staged coarctation repair +/- pulmonary artery banding followed by VSD closure with 2 separate operations. This has the advantage of a straightforward coarctation repair (except in the case of proximal arch hypoplasia) and a simpler VSD closure at a later date. A subset of patients will have spontaneous VSD closure that obviates the need for subsequent operation. Disadvantages include a period of palliation between operations and the complications of a pulmonary band. A single-stage approach involves simultaneous coarctation repair and VSD closure on cardiopulmonary bypass with circulatory arrest or regional perfusion during coarctation repair. This has the advantages of complete repair in infancy without palliation and the ability to deal with proximal arch hypoplasia. Disadvantages include a technically more challenging operation and the need for circulatory arrest or regional cerebral perfusion. Some series have suggested a higher risk for recoarctation. An alternative method involves coarctation repair without cardiopulmonary bypass through a thoracotomy followed by VSD closure during the same operation (1 stage, 2 incisions). This affords excellent clinical results with complete repair in infancy. One can avoid prolonged periods of aortic cross clamping, cardiopulmonary bypass, and circulatory arrest/regional perfusion. Compared with the other strategies, there are decreased total intensive care unit and hospital stays.     

Biventricular repair for aortic atresia or hypoplasia and ventricular septal defect.

OBJECTIVE: Aortic valve atresia or hypoplasia can present with a ventricular septal defect and a normal mitral valve and left ventricle. These patients may be suitable for biventricular repair, although the optimal initial management strategy remains unknown. METHODS: From January 1991 through March 1999, 20 patients with aortic atresia or hypoplasia and ventricular septal defect underwent operation with the intent to achieve biventricular repair. Aortic atresia was present in 7 patients, and aortic valve hypoplasia was present in 13 patients. Among those patients with aortic hypoplasia, Z-scores of the aortic valve anulus ranged from -8.8 to -2.7. Associated anomalies included interrupted aortic arch (n = 12 patients), coarctation (n = 6 patients), aortopulmonary window (n = 1 patient), and heterotaxia (n = 1 patient). Nine patients were staged with an initial Norwood procedure followed by biventricular repair in 8 patients. One patient awaits biventricular repair after a Norwood procedure. The conditions of 11 patients were corrected with a single procedure. RESULTS: Among the 9 patients who underwent staged repair, there were no deaths after the Norwood procedure and 1 death after biventricular repair. For the 11 patients who underwent a primary biventricular repair, there was 1 early death and 2 late deaths from noncardiac causes. Follow-up ranged from 1 to 85 months (mean, 28 months). Actuarial survival for the entire group was 78% +/- 10% at 5 years and was not significantly different between staged repair (89%) and primary biventricular repair (73%). CONCLUSIONS: Both primary and staged biventricular repair for patients with aortic atresia or hypoplasia and ventricular septal defect may be performed with good late survival. Refinements in technique of conduit insertion and arch reconstruction have resulted in primary biventricular repair becoming our preferred approach.     

Primary repair for aortic arch obstruction associated with ventricular septal defect

Background

The aim of the present study was to evaluate the current outcome and reoperation rate after applying a one-stage correction strategy for interrupted aortic arch (IAA) with ventricular septal defect (VSD) and also for aortic coarctation and hypoplastic aortic arch (CoA-HyAA) with VSD beginning September 1999.

Methods

Twenty-four consecutive patients with IAA (n = 12) or CoA-HyAA (n = 12) with VSD underwent early one-stage correction. Patients' mean age was 12 days (range, 2 to 188); mean weight was 3.6 kg (range, 2.1 to 7.3), 6 patients were less than 2.5 kg. Three IAA were type A, 5 type B1, 3 type B2, and 1 type C. Associated anomalies included a large VSD in all, left ventricular outlet tract obstruction in 5, transposition of the great arteries, aortopulmonary window, persistent truncus arteriosus, and double-outlet right ventricle in 1 patient. Selective brain perfusion through innominate artery and selective coronary perfusion through aortic root during aortic arch reconstruction was used in all patients. Mean follow-up was 2.2 ± 0.9 years.

Results

There was no early, no late mortality, and no postoperative neurologic complications. Mean crossclamp duration was 72 ± 32 minutes, lowest temperature 22.8 ± 4°C and selective brain and coronary perfusion duration was 34 ± 13 minutes. Eighteen patients required delayed sternal closure at 1.7 days postoperatively. New perioperative management reduced the overall morbidity. Four patients after IAA plus VSD repair developed aortic arch restenosis and were successfully treated by balloon dilatation. One patient with d-TGA underwent right ventricular outflow tract reconstruction of right ventricular outlet tract obstruction 7 months after the initial repair. Pressure gradients across the anastomosis at most recent follow up were less than 10 mm Hg. All patients are asymptomatic and are developing normally.

Conclusions

One-stage complete correction is feasible in newborns with aortic arch obstruction with VSD. Complex cardiac anatomy presents no additional risk for the procedure. The early one-stage correction yields excellent surgical results and good functional outcome.     

Arterial switch operation with ventricular septal defect repair and aortic arch reconstruction

The arterial switch operation for transposition of the great arteries or double outlet right ventricle with ventricular septal defect (VSD) and aortic arch obstruction is a challenging procedure. One-stage neonatal repair is preferred; however, palliation may be indicated in the newborn who presents with Swiss cheese septum or the patient with extracardiac complications such as necrotizing enterocolitis or subarachnoid hemorrhage. The aortic arch repair is performed with continuous cerebral perfusion and includes patch enlargement of the transverse aortic arch and ascending aorta to control for the important diameter mismatch between the aorta and the pulmonary root. The VSD is no longer closed through the pulmonary valve. Instead, the VSD is approached through the aortic valve after harvesting of the coronary buttons and/or through the tricuspid valve and, if necessary, through an infundibulotomy. The coronary artery transfer is often complex because of the presence of unusual coronary artery patterns and requires precise technique. Subaortic obstruction is often present and requires correction. The right ventricle can be mildly hypoplastic but is not a contraindication to repair unless there is important tricuspid stenosis.     

Staged repair of interrupted aortic arch with ventricular septal defect compared with primary repair in infancy

Interrupted aortic arch is a poor prognosis cardiac anomaly with nearly 100 percent mortality if not recognized and treated early. The associated intracardiac lesions often lead to death if only the arch defect is repaired. Several recent reports have described patients with interrupted aortic arch who were treated as infants by primary repair of the arch defect with simultaneous repair of the intracardiac lesions. The improved survival data from these series have been attributed to the simultaneous repair of both lesions. We report herein on nine patients with both interrupted aortic arch and ventricular septal defect seen at Children's Hospital and Medical Center in Seattle from 1979 to 1987. Three patients had partial expression of DiGeorge's syndrome. All patients underwent primary repair of the interrupted aortic arch with concomitant pulmonary artery banding during infancy (mean age 18 days, range 2 days to 4 months). Operative mortality was 11 percent (1 of 9 patients). Eight patients had eventual repair of the ventricular septal defect (mean age 18 months, range 6 to 29 months) with one death occurring at 5 months postoperatively (12 percent mortality). The overall mortality of these nine patients was 22 percent. Staged repair of interrupted aortic arch with associated ventricular septal defect can be performed with results comparable to simultaneous primary repair in infancy. The improved survival from either approach is more likely to be attributable to improved perioperative stabilization, particularly the use of prostaglandin E.