Repair of interrupted aortic arch in infancy

J

THoRAc CARDIOVASC SURG

1988;96:564-8

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 weD 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 bad surgical repair. None of the most recent seven patients, however, have had a residual ascendingdescending aortic gradient Careful foDow-up for the detection of preliously masked or newly developed left ventricular outflow tract obstruction is imperative and may be lifesaling.

William A. Scott, MD, Albert P. Rocchini, MD, Edward L. Bove, MD, Ann Arbor, Mich., Douglas M. Behrendt, MD,a Iowa City, Iowa, Robert H. Beekman, MD, Macdonald Dick II, MD, Gerald Serwer, MD, Rebecca Snider, MD, and Amnon Rosenthal, MD, Ann Arbor, Mich.

Interruption of the aortic arch and its repair have long been associated with high mortality. '-J Although the use of prostaglandin therapy to maintain ductal patency during evaluation and initial treatment has dramatically improved operative survival, reports of late complications and death are common.v' One proposed cause of late morbidity and mortality is left ventricular outflow tract obstruction. The presence of residual or recurrent arch obstruction in addition to aortic stenosis may be influenced by the method of initial repair. In an attempt to determine the cause(s) of late postoperative death, we have reviewed our last 7 year's experience with repair of interrupted aortic arch in infancy with emphasis on late postoperative outcome and possible causes of late postoperative deaths. Patients and methods From September of 1979 through January of 1987, 21 patients underwent repair of interrupted aortic arch at the University of Michigan Medical Center. Patients with severe

From the Department of Pediatrics Division of Pediatric Cardiology. and the Department of Thoracic Surgery. C. S. Mott Children's Hospital, University of Michigan Medical Center, Ann Arbor. Mich.. and the Department of Thoracic Surgery.' University of Iowa, Iowa City, Iowa. Received for publication Sept. 7, 1987. Accepted for publication Feb. 20, 1988. Address for reprints: William A. Scott. C. S. Mott Children's Hospital, University of Michigan. 1500 E. Medical Center Dr., Ann Arbor, MI 48109.

564

coarctation or hypoplasia of an aortic arch segment without interruption were excluded from this review. According to the classification of Celoria and Patton: eight of the patients had type A interrupted aortic arch, 12 had type S, and one had type C. All patients had a ventricular septal defect and patent ductus arteriosus. The ventricular septal defect was multiple in two patients (one with two mid muscular defects and the second with an outlet and mid muscular defect), perimembranous with malalignment into the left ventricular outflow tract in five, and infundibular with or without extension to the peri membranous region in the remaining 14. Major associated cardiac malformations were present in six, including truncus arteriosus (two), transposition of the great arteries (two), tricuspid atresia (one), and double-outlet right ventricle with subpulmonic ventricular septal defect (one) (Table I). At the time of presentation all patients had some degree of acidosis as a result of limited systemic perfusion through a restrictive ductus arteriosus and cardiomegaly with increased pulmonary blood flow on chest x-ray film. All patients received prostaglandin E, to maintain ductal patency. The median age at initial operation was 2 days with a range of I to 30 days. The surgical management consisted of staged repair in 10 patients and primary reconstruction including all coexisting defects in the other II. Staged repair. Among the 10 patients who initially underwent palliative procedures, nine had muscular or multiple ventricular septal defects and/or other complex malformations. Only two patients had an isolated type A interrupted aortic arch with an infundibular septal defect. The median age at operation was 7 days, ranging from I to 30 days. Weight at initial operation was 2.96 ± 0.68 kg (mean ± standard deviation). After ligation of the ductus, the interrupted arch segment was managed by polytetrafluoroethylene* tube graft "Gore-Tcx. registered trade name of W. L. Gore & Associates, Inc.. Elkton. Md.

Volume 96 Number 4 October 1988

Interrupted aortic arch in infancy

565

Table I. Presentation and management of patients with interrupted aortic arch Patient

Weight (kg)

Staged 1 2 3 4

3.7 2.2 3.3 3.2

5 6 7 8 9 10

3.1 2.1 3.1 2.8 4.1 2.6

7 1 22 30 2 I

B A B A A B

12

3.5 1.9

2 1

B B

13 14

2.1 2.3

2 1

B B

15 16

3 2

18 19 20

2.8 2.3 2.9 3.6 4.1 3.1

4 2 13

B B B A A B

21

3.4

3

A

Age at op. (days)

1 7 2 13

Type

B C A A

Associated malformations

Taussig-Bing DiGeorge Subaortic stenosis, TGA, polysplenia, multiple VSD Aortic stenosis Truncus TGA, subaortic stenosis Multiple VSD Tricuspid atresia. DiGeorge

Primary II

17

Ao, Aorta: E-S. end-to-side; great arteries.

I

LV.

Bicuspid aortic valve, DiGeorge DiGeorge Aortic stenosis, DiGeorge Truncus Aorticstenosis DiGeorge Aortic stenosis

left ventricle: PAB. pulmonary artery band:

Operation I

Status

8 nun PTFE + PAB 10 nun PTFE + PAB E-S + PAB 8 mm PTFE + PAB

Mustard + VSD VSD PAB removal only LV-Ao conduit

Dead Alive Alive Dead

6 mm PTFE + PAB PTFE patch + PAB E-S + PAB E-S + PAB 8 mm PTFE. PAB 6 mm PTFE + PAB

Total repair Arterial switch + VSD VSD + arch revision VSD

Dead Dead Alive Alive Alive Dead

E-S + VSD E-S + VSD

Alive Dead

E-S + VSD E-S + VSD

Dead Dead

E-S + total repair E-S + VSD E-S + VSD E-S + VSD E-S + VSD E-S + VSD E-S + VSD

PTFE. polytetrafluorocthylcnc: VSD.

(6 to 10 mm diameter) in six patients and by end-to-side anastomosis of the proximal and distal aortic segments in the remaining four patients. The pulmonary artery was banded in all patients in this group. In all patients, the approach was a left thoracotomy and the repair performed during aortic cross-clamping. There were two hospital deaths in the immediate postoperative period. All eight of the remaining patients underwent complete repair 2 weeks to 48 months after the initial palliation. In each, the pulmonary artery band was removed. Ventricular septal defect repair was performed in all except one patient with an isolated defect that underwent spontaneous closure. In addition, one patient each underwent a Mustard repair, arterial switch, and apical-aortic conduit placement. Primary repair. Eleven patients underwent complete repair of associated cardiac malformations in addition to primary reconstruction of the aortic arch. An isolated single infundibular or membranous ventricular septal defect was present in 10 patients, and the eleventh had truncus arteriosus. The age at the time of operation was I to 13 days, with a median of 2 days. Weight at initial operation was 2.91 ± 0.62 kg. The arch interruption was managed by direct end-to-side anastomosis in each patient, and the ventricular septal defect was closed transatrially, In the patient with truncus arteriosus, a valveless

Operation 2

Coarctation revision Konno VSD reoperation Coarctation; 3rd op.-Konno

Alive Alive Alive Alive Alive Alive Alive

ventricular septal defect: TGA. transposition ofthe

conduit was used to establish continuity between the right ventricle and pulmonary artery. All operations were done through a median sternotomy with the use of deep hypothermia and circulatory arrest for the arch reconstruction. Bypass was then generally resumed for the remainder of the repair.

Results Of the 21 patients in this series, 13 (62%) are alive and clinically well 6 months to 6 years after either primary or staged operations. Among the 10 patients who underwent staged repair, there were three early deaths. One patient with an associated ventricular septal defect in addition to valvular and subvalvular aortic stenosis died when the aorta was irreparably lacerated during cannulation for cardiopulmonary bypass, and the second died at the time of complete repair for truncus arteriosus 9 days after the initial procedure. The third patient had tricuspid atresia and persistent congestive failure after pulmonary artery banding. This patient died after a postoperative catheterization that demonstrated severe subaortic stenosis. There were two late

The Journal of Thoracic and Cardiovascular Surgery

566 Scott et a/.

Table II. Follow-up cardiac catheterization data

Patient

Month postop.

LV (mmHg)

5 6

(RV) 88/11

3

5

150/18

7

(RV) 130/12

8 9 10

19 43 3 6 1

11

12

169/110

15

2.5 8.5 14 3 3 21

180/11 101/14 175/79 116/10 185/12 200/19

94/16

Ascending Ao (mmHg)

Descending Ao (mmHg)

Arch repair

87/48 82/42

88/48 90/50

PTFE PTFE

69 mm Hg across LVOT (PA band) 25 mm Hg across LVOT (after PA

150/78 146/70 120/82 108/59 130/68 110/62 80/41

84/67 121/78 120/82 108/58 120/66 102/59 80/45

E-S E-S E-S E-S E-S PTFE PTFE

47 mm Hg across RVOT (PA band)

145/49 125/66 185/50 105/55 80/35 92/46 180/65 110/54

86/52 122/62 120/75 107/54 85/50 92/41 50/40 110/50

E-S E-S E-S E-S E-S E-S E-S E-S

Comment

band takedown)

16 17 20

108/10 140/156/12 120/12

After balloon angioplasty

71 mm Hg across RVOT Died awaiting operation; low cardiac output After balloon angioplasty Residual coarctation surgically repaired 40 mm Hg across RV-PA conduit Tunnel sub-AS, subsequently sip Konno Reoperation for VSD Residual coarctation surgically repaired Tunnel sub-AS, subsequently sip Konno

AS. Aortic stenosis: E-S. end-to-side: LV. left ventricle: PA. pulmonary artery: PTFE. polytetrafluoroethylene: RV. right ventricle: sip. status post; OT. outflow tract; VSD. ventricular septal defect.

deaths. The first occurred 2 months after pulmonary artery banding, at the time of placement of a left ventricular-aortic conduit to relieve severe subaortic obstruction. The second occurred in a patient with Taussig-Bing anomaly who died suddenly of presumed sepsis 2Yz years after Mustard repair and ventricular septal defect closure. Among the 11 patients undergoing complete repair, there were two operative deaths. One patient was believed to have inadequate relief of the interruption and died during attempted pericardial augmentation of the direct anastomosis. The second had a hypoplastic ascending aorta and remained in a low output state postoperatively. There was one late death 4 months after complete repair in a patient who had sepsis, severe subaortic stenosis, and a residual coarctation. Eleven patients underwent postoperative cardiac catheterization 1 to 43 months postoperatively (Table II). Six of these had catheterization evidence of a pressure gradient between the ascending and descending thoracic aorta. Three of these patients had significant recurrent or residual arch obstruction, defined as a peak systolic gradient >20 mm Hg. One additional patient who did not undergo catheterization had clinical evidence of a significant ascending-descending aortic gradient. In two of the patients (patients 3 and 11), the gradient was relieved by balloon angioplasty (Table II, Fig. 1). One other patient (15) required reoperation 2

months after initial repair; late postoperative death occurred in the fourth patient (14). Among the seven patients who underwent end-to-side anastomosis since November of 1985, none have had significant residual or recurrent coarctation. In addition to coarctation, other forms of left ventricular outflow tract obstruction were frequently encountered. Of the 21 patients, 12 had evidence of left ventricular outflow tract obstruction. Eight of these obstructions were discovered by catheterization and four at postmortem examination. The obstruction was unsuspected in two patients. In seven of the patients, the left ventricular outflow obstruction was severe (three with a gradient >60 mm Hg at catheterization and four in whom the obstruction was found at postmortem examination). Severe left ventricular outflow tract obstruction correlated with postoperative mortality (p = 0.08). Discussion

The goals of surgical therapy for infants with interrupted aortic arch (i.e., reestablishment of continuity between the segments of the aorta and correction of the other intracardiac defects) are apparent. However, the exact surgical treatment remains controversial. Interrupted aortic arch is a complex lesion involving multiple sites of aortic interruption (types A, B, and C) and varied types of intracardiac anatomy. Our more recent experience indicates that one-stage complete repair of all

Volume 96 Number 4 October 1988

cardiac defects should be performed whenever feasible. Three patients with complex lesions in whom primary repair could have been done were treated with staged repair; all of these represent early experience, and none are long-term survivors. In the primary repair group, the single patient with complex anomalies (15) is doing well 2 years postoperatively. Only in patients with anatomy not suitable for correction in infancy (i.e., univentricular heart) is a staged approach used, with the initial operation consisting of arch reconstruction and pulmonary artery banding. Preferably, the ascending and descending aorta are directly anastomosed end to side; alternatively a 10 to 12 mrn polytetrafluoroethylene graft is used. This approach has evolved with experience, and overall we have had a 76% early and 62% late survival rate in our 21 patients with interrupted aortic arch regardless of intracardiac anatomy. These results compare favorably with other recent reports." Despite the fact that our early survival rate has been good, the occurrence of five late postoperative deaths is of concern, In reviewing these patients, four had severe left ventricular outflow tract obstruction. It was unsuspected in two of these patients (6 and 14); another died while awaiting corrective surgery (10), and one died at the time of attempted apical-aortic conduit insertion (4). Significant left ventricular outflow tract obstruction also developed in two of the surviving patients. Both successfully underwent a Konno procedure. The anatomic basis of the obstruction in these seven patients varied. Posterior deviation of the infundibular septum was the cause in four of the patients (5, 10, 16, and 20), subaortic obstruction was associated with polysplenia syndrome in one patient (4), and in the remaining two patients truncal and aortic valve stenosis was present (6 and 12). In addition to these seven patients, four others had hemodynamic evidence of some degree of subaortic or aortic valve obstruction. Thus 12 of 21 patients (57%) in our series had some degree of left ventricular outflow tract obstruction. Others have reported this association with interrupted aortic arch. Van Praagh and colleagues I reported that eight of 10 autopsy cases had subaortic stenosis. Norwood and associates' reported that in seven of 24 long-term survivors of interrupted aortic arch some degree of subaortic stenosis developed, and five of those seven required surgical management of the stenosis. Braunlin, Lock, and Foker' reported that five of seven patients had some degree of left ventricular outflow obstruction, and in one of these patients the subaortic obstruction was the cause of late postoperative death, In addition to the development of valvular and subvalvular aortic obstruction, we observed a large

Interrupted aortic arch in infancy

567

Fig. 1. Result of balloon angioplasty (A, before; B, after) in patient with recurrent postoperative coarctation. lc, Left common carotid; Is, left subclavian; rc, right carotid; rs, right subclavian; arrow, site of coarctation.

incidence of residual thoracic aortic stenosis early in this series. In six of our 13 long-term survivors of interrupted aortic arch repair, evidence of a pressure gradient between the ascending and descending thoracic aorta developed. Among these six patients, residual gradients were severe in four (61 to 70 mrn Hg). Reoperation was required in one of these patients; in a second, the gradient was associated with severe left ventricular outflow tract obstruction and resulted in late postoperative death. Recently, two patients were successfully treated with balloon angioplasty (Table II, Fig. 1). Our most recent experience (November 1985 to present, n = 7) has been with direct end-to-side anastomoses, and none of these seven patients have had recurrent stenosis of the descending aorta. Others have reported

The Journal of Thoracic and Cardiovascular

5 6 8 Scott et ai.

that a large tube graft proves a reliable means of establishing unobstructed continuity between the ascending and descending aorta.t' Although the tube interposition graft provides a reliable means of establishing continuity between the ascending and descending aorta over the short term, it is likely that all of these grafts will necessitate revision as the child grows. Therefore, we believe better long-term results may be achieved with the direct anastomosis. REFERENCES I. Van Praagh R, Bernhard WF, Rosenthal A, Parisi LF, Fyler DC. Interrupted aortic arch: surgical treatment. Am J Cardiol 1971;27:200-11. 2. Heymann MA, Berman W Jr, Rudolph AM, Whitman V. Dilatation of the ductus arteriosus by prostaglandin E, in aortic arch abnormalities. Circulation 1979;59: 16973.

Surgery

3. Zahka KG, Roland JM. Cutilletta AF. Gardner T J. Donahoo JS, Kidd L. Management of aortic arch interruption with prostaglandin E, infusion and microporous expanded polytetrafluoroethylene grafts. Am J Cardiel 1980;46:1001-5. 4. Norwood WI, Lang P, Castaneda AR. Hougen T J. Reparative operations for interrupted aortic arch with ventricular septal defect. J THOR\C C\RDIOVASC SLRG 1983;86:832-7. 5. Braunlin EA, Lock JE. Foker .fE. Repair of type B interruption of the aortic arch . J TflOR.\C C"RDIOVASC SLRG 1983;86:920-5. 6. Celoria GC, Patton RB. Congenital absence of the aortic arch. Am Heart J 1959;58:407-13. 7. Hammon JW, Merrill WHo Prager RL Graham TP. Bender HW. Repair of interrupted aortic arch and associated malformations in infancy: indications for complete or partial repair. Ann Thorac Surg 1986:42: 17-21.

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