Pediatr Cardiol 13:214-221, 1992

Pediatric Cardiology 9 Springer-Veflag New York Inc. J992

I n t e r r u p t e d A o r t i c A r c h in Infancy: A l O - Y e a r E x p e r i e n c e * Samuel Menahem, Anna U. Rahayoe, William J. Brawn, and Roger B.B. Mee Department of Cardiology and Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia

SUMMARY. Fifty infants with interrupted aortic arch (IAA), admitted between 1979 and 1988, were reviewed. They usually presented early in severe cardiac failure or shock. In the initial 5-year period, 17 of the 21 infants underwent diagnostic or confirmatory cardiac catheterization, in contrast with the latter 5 years when only eight of the subsequent 29 patients underwent catheterization. Since 1987, all patients underwent surgery after cross-sectional echocardiography. Fifteen infants had a type A IAA and 35 had type B. All had associated cardiac anomalies. Four infants were not operated on. In the initial 5-year period, of 17 infants who were surgically treated, four had a one-stage total repair, the remaining had a two-stage repair with initial reconstruction of the arch and pulmonary artery banding. There was an overall surgical mortality of 65%, reflecting the precarious state of many of these infants before surgery with a significant contribution from unrelieved subaortic stenosis. In the latter 5-year period, 29 underwent surgery, 22 had a one-stage total repair. There were three deaths, all in infants whose active treatment was withdrawn. The outcome of the survivors has generally been good, subsequent surgery being mainly related to the associated anomalies (e.g., recurrent subaortic stenosis, conduit replacement). Over this I0 year period the greater accuracy of noninvasive diagnoses, and perioperative intensive care, have led to an improvement in the preoperative state of these infants. Singlestage total repair is our procedure of choice. KEY WORDS: Interrupted aortic arch - - Infancy - - Surgery

Interruption of the aortic arch (IAA) occurs when there is discontinuity between the ascending and descending aorta, the latter being supplied by an aortic branch vessel or patent ductus arteriosus [2]. This rare lesion is generally part of a highly lethal complex that more commonly presents as a true emergency in the neonatal period [3]. Early noninvasive diagnosis [10, 18, 22] and intensive resuscitation [8] has increasingly allowed many of these infants to undergo surgery in a reasonable state. These advances have exerted pressure on surgeons to repair the interrupted arch with palliation of the intracardiac defects as a first step [1] or, more recently, to undertake a complete repair of the intracardiac anomalies at the same time [21].

* Presented in part to the Third World Congress of Paediatric Cardiology, Bangkok, November 1989. Address offprint requests to: Prof. Samuel Menahem, Department of Cardiology, Royal Children's Hospital, Flemington Road, Parkville, Victoria 3052 Australia.

Unrecognized and untreated, the median age at death is said to be 4-10 days [15], with a mortality as high as 80% in the first month of life [6, 28]. This paper reviews the experience with this condition over a 10-year period, highlighting changes in the perioperative care and surgical approach with a shift from a two-stage to a one-stage repair.

Patients and Methods The records of all infants with IAA admitted to the Royal Children's Hospital, Melbourne, between January 1979 and December 1988 were reviewed. Their clinical features, investigations, medical and surgical management, and the outcome of the survivors were collated. The patients were separated into two groups: those presenting in the initial 5-year period where the majority had a two-stage repair, and those in the second 5-year period where a one-stage repair was the norm. The local survivors were reviewed and their clinical, echocardiographic data collated, together with their cardiac catheterization findings when performed as clinically indicated. Information on interstate and overseas patients was obtained wherever possible.

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Table 1. Age at presentation

Table 3. Associated Noncardiac anomalies

Age at presentation

1 day to 10 months

Before 1 week Before 3 months Before 12 months

38 9 3

Weight at presentation

1.4-7.3 kg

Partial Di George syndrome Down syndrome Deletion of chromosome 4 Williams syndrome Yalipes Equinovarus Renal anomaly Cleft lip/palate Obstructive emphysema (?bronchomalacia)

12 3 1 1 2 2 1 1

Table 2. Associated cardiac anomalies Anomalies

Type A (n : 15)

Type B (n = 35)

Total (n = 50)

PDA VSD AP window Truncus arteriosus TGA ASD Anomalous RSCA SubAo stenosis DORV CAV septal defect Mitral atresia Tricuspid atresia Bilateral PDA Right arch Bicuspid Ao valve

15 13 5 1 1 1 1 1 -1 ---2 --

35 35 -5 1 7 11 12 2 -1 1 2 1 9

50 48 5 6 2 8 12 13 2 1 1 1 2 3 9

PDA, Patent ductus arteriosus; AP, aortopulmonary; TGA, transposition of the great arteries; ASD, atrial septal defect; RSCA, right subclavian artery; SubAo, subaortic; DORV, double-outlet right ventricle; CAV, complete atrioventricular; Ao, aortic.

Results

Clinical Features Fifty infants with IAA were identified: 15 (30%) had type A interruption (distal to the left subclavian artery) and 35 (70%) had type B (interruption between the left carotid and left subclavian) [2]. There were 23 males and 27 females. All patients presented early [20, 21, 26]. The majority before the age of 1 week, the weight being 1.4-7.3 kg (Table 1). Most presented in cardiac failure with tachypnea, with or without cyanosis, and unequal or poor pulses. Nearly 20% presented in a state of shock with acidosis and a low output state. All 50 patients were found to have associated intracardiac abnormalities [12, 20] as summarized in Table 2. Noncardiac abnormalities were noted in about 40% of patients, most of whom had multiple but generally not lethal abnormalities (Table 3). Twelve infants were considered to have possible thymic aplasia, or Di

George syndrome with impaired immune function with or without associated hypocalcemia [5, 16]. Improvement of the immune function occurred after a few weeks to a few months. Nevertheless, irradiated blood was initially used until the diagnosis was clarified. Four infants had chromosomal abnormalities which led to conservative management in two patients and withdrawal of treatment in one. A further infant with a poorly functioning single hypoplastic kidney did not proceed to the second stage intracardiac repair.

Investigations The chest x-ray at presentation usually revealed cardiomegaly with or without congestion and/or plethora [9]. The ECG was unremarkable. The introduction of high-resolution cross-sectional echocardiography significantly reduced the need for cardiac catheterization [22] (Figs. 1-6), which was then mainly done to clarify the associated anomalies and/or to determine the hemodynamics, particularly in those infants who presented late. The last 14 neonates proceeded to surgery based on the echocardiographic findings alone. There have been two potential errors when a bypoplastic arch and a localized coarctation were diagnosed instead of an interruption. These misdiagnoses did not adversely effect the immediate surgical management. Table 4 summarizes the investigations performed and Table 5 the frequency of cardiac catheterization. Two infants early in the first 5-year period died shortly after such catheterization prior to surgery.

Management Medical. The infants have increasingly been subjected to aggressive preoperative management. The traditional treatment of digoxin and diuretics was replaced by infusions of dopamine and prostaglandin E~, the latter to maintain duct patency, together with intermittent positive pressure ventilation [20,

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Fig. 3. Cross-sectional echocardiogram (suprasternal long axis) showing type A IAA. AAo, Ascending aorta; DAo, descending aorta; LA, left atrium; LCA, left carotid artery; LSCA, left subclavian artery; RPA, right pulmonary artery. Fig, 4. Cross-sectional echocardiogram (suprasternal long axis) showing type B IAA and illustrating the V sign made by the fight and left carotid artery. DAo, Descending aorta; LCA, left carotid artery; LSCA, left subclavian artery; RCA, right carotid artery; LCA, left carotid artery.

Fig. 1. Angiogram (left anterior oblique) showing type A IAA. AAo, Ascending aorta; LCA, left carotid artery; LSCA, left subclavian artery; LV, left ventricle; RV, right ventricle; VSD, ventricular septal defect.

Fig. 5. Cross-sectional echocardiogram (ductal cut) showing type B IAA. DAo, Descending aorta; LSCA, left subclavian artery; MPA, main pulmonary artery; PDA, patent ductus arteriosus; RPA, right pulmonary artery.

Fig. 2. Angiograms (left anterior oblique) showing type B IAA. A Ascending aortogram: AAo, ascending aorta; LCA, left carotid artery; RCA, right carotid artery; RSCA, right subclavian artery; VA, vetebral artery. B Descending aortogram: DAo, descending aorta; LSCA, left subclavian artery; PDA, patent ductus arte-

Fig. 6. Cross-sectional echocardiogram (parasternal long axis)

riosus.

showing posteriorly displaced infundibular septum resulting in considerable subaortic stenosis in an infant with a type B IAA. Ao, Aorta; InfS, infundibular septnm; LV, left ventricle; LVOT, left ventricular outflow tract; RV, right ventricle; V, aortic valve; VS, ventricutar septum.

Menahem et al.: Interrupted Aortic Arch in Infancy

217

Table 4. Investigations

Chest x-ray ECG 2D Echo

Cardiomegaly + / - Congestion + / - Plethora Unremarkable Done routinely since available Correct diagnosis "Incorrect" Interruption probable but uncertain Hypoplastic aortic arch Coarctation of aorta

(n = 42) 36

2D echo, cross-sectional echocardiography. In 1987 and 1988 all 14 proceeded to initial surgery on 2D echo alone.

Table 5, Cardiac catheterization IAA type A n

IAA type B

Cath Op n

1979-1983 7 7 t984-1988 8 2

7 8

Total IAA

Cath Op n

14 10 2t 6

10 21

C a t h OP

21 17 29 8

17" 29

IAA, Interrupted aortic arch; Cath, catheterization; Op, operation. Four, no surgery; two, Down--one with pulmonary vascular disease; two, dying after cath (1979).

21]. This intensive treatment enabled many of the infants to undergo surgery much improved and without appreciable acidosis. Four infants did not have surgery; two died after the initial cardiac catheterization, one infant had Down syndrome, and the fourth had Down syndrome but presented late with pulmonary vascular disease. Surgical. The remaining 46 infants underwent surgery, 38 before 1 week of age, this being carried out shortly after admission once the diagnosis was confirmed and the neonate reasonably stable. The interruption was repaired in 30 infants by a direct end-to-side or end-to-end anastomosis [20, 21]. Four with a type A IAA had a left subclavian aortoplasty. The remaining 12 had the insertion of a 8mm diameter polytetrafluorethylene conduit [20, 21] (Fig. 7). In the initial 5-year period (1979-1983), 13 had a staged repair. Initial banding of the main pulmonary artery and reconstitution of the arch, either by conduit or by direct anastomosis (Figs. 8 10) followed by later debanding and intracardiac repair. The remaining four (20%) had a one-stage procedure with reconstruction of the arch and intracardiac repair of the ventricular septal defect (VSD). In contrast, in the latter 5-year period (1984-1988), the situation was reversed and the majority (23) had a one-stage repair with only six pa-

Fig. 7. Angiogram of conduit repair of type A IAA. AAo, As-

cending aorta; DAo, descending aorta; LCA, left carotid artery; RCA, right carotid artery.

tients (21%) having a two-stage p r o c e d u r e - - t h e r e being a period of overlap between the two methods [20, 21]. In addition the insertion of a conduit to reconstitute the aortic arch dropped from nearly 60% during the first 5-year period to 8% (2 of 27) in the second 5-year period (Table 6). The surgery was performed through a midline sternotomy [25], the technique being described elsewhere [I9]. The overall mortality in the initial 5-year period was high, of the order of 65% (see Table 7), reflecting the poor state of many of the infants prior to surgery with a significant contribution from unrelieved subaortic stenosis. In the subsequent 5-year period, there were three deaths, all in infants in whom the treatment was withdrawn (Table 6). The mortality dropped substantially in the second period, despite a shift to a one-stage complete repair. Patients have required further surgery for repair of associated abnormalities, particularly recurrent subaortic stenosis [13] and replacement of conduit.

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TypeB IAA

.../

RSCA-~

/LCA

~~

LPA

LPA

DAO "~~ LCA/c r A REOTANASTOMOS,S

\__

/2"..

JI MPA| I

I~TANASTOMOSIS

L~G~4TE DI ~ D

STUMP..,.,,~~Z__~,~ L,GATE O00t_/

"

t,_ff

Fig. 8. Diagram of type A IAA and direct anastomosis to reconstitute the aortic arch. AAO, Ascending aorta; DAO, descending aorta; LCA, left carotid artery; LSCA, left subclavian artery; LPA, left pulmonary artery; MPA, main pulmonary artery; PDA, patent ductus arteriosus; RCA, right carotid artery; RPA, right pulmonary artery; RSCA, right subclavian artery.

Fig. 9. Diagram of type B IAA and direct anastomosis to reconstitute the aortic arch. AAO, Ascending aorta; DAO, descending aorta; IA, innominate artery; LCA, left carotid artery; LPA, left pulmonary artery; LSCA, left subclavian artery; MPA, main pulmonary artery; PDA, patent ductus arteriosus; RCA, right carotid artery; RPA, right pulmonary artery.

Table 6, Surgical management Stage

Type A

Type B

2-stage Conduit 1979-1983 1984-1988

+ 2 0

1-stage

3 4

+ 1 0

Total

2-stage 1 4

+ 7 2

l-stage 1 0

+ 0 0

2-stage 2 19

+ 9 2

1-stage 4 4

+ 1 0

3 23

Table 7. Cumulative surgical mortality Type A

1979-1983 1984-1988

Type B

Total

2-stage

t-stage

2-stage

1-stage

2-stage

1-stage

5(2) 4(0)

2(1) 4(0)

8(6)" 2(0)

2(2) 19(3)

13(8,6E,2L) 7(0)

4(3E) 22(3) b

E, Early deaths; L, late deaths. Treatment withdrawn: " included infant with single hypoplastic kidney; 0 chromosomal abnormality, small left ventricle; hydrocephalus; bronchopulmonary dysplasia, bronchomalacia.

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Table 8. Outcome Survivors Follow-up (6 months to 8 years) Postoperative catheter(s) No/slight gradient aortic arch Moderate gradient--aortic arch Conduit (20 mmHg) replaced Direct anast (30-50 mmHg) Angioplasty RV to PA conduit stenosis (truncus) Neoaortic and neopulmonary stenosis (TGA) Residual subaortic stenosis (3 after further surgery) Small residual VSD Clinical and echo--good result Pacemaker

Fig. 10. Angiogram (left anterior oblique) of direct anastomotic reconstitution of the aortic arch in type B interruption of the aorta. AAo, Ascending aorta; DAo, descending aorta; LCA, left carotid artery; RCA, right carotid artezy.

Outcome. The length of review varied between 6 months to 8 years, three patients being lost to follow-up. Of the 32 survivors, 11 on echocardiographic evidence showed no significant gradients across the reconstituted arch (Table 8). A further 18 infants have been catheterized to date, carried out 6 months to 8 years postoperatively, mainly to determine residual intracardiac or extracardiac defects and gradients across the anastomoses. Excluding the group of truncus arteriosus with interruption, and concentrating on the arch reconstruction, one patient has had a conduit removed after 4 years and direct anastomosis performed, having developed a 21-mm gradient across it at the time of further subaortic resection. Two infants have had a balloon angioplasty performed of a direct anastomosis, one with type A interruption who developed a 50 mmHg gradient and the other with a type B interruption with a 40 mmHg gradient with a residual gradient of 20 and 0 mmHg, respectively. A fourth infant appears stable with a 30 mmHg gradient across the direct anastomosis. Severe subaortic stenosis requiring surgical treatment occurred in 13 of the 50 patients and remains an on-going problem requiring a second resection in three infants, but with residual mild-to-moderate gradient, despite the surgery (see [131).

Discussion

IAA is a rare but serious congenital anomaly. Most infants, if untreated, die within the first month of life [26], the reported mean age at death being 10 days [28]. Advances in noninvasive diagnostic techniques and perioperative intensive care [20, 21]

32 29 19 14 4 1 3 2 2 1 7 11 10 2

Anast, Anastomosis; TGA, transposition of the great arteries; VSD, ventricular septat defect.

have shown better results. Our series confirmed type B interruption as being the most common; none of our patients had type C interruption (proximal to the left common carotid artery) [2]. Associated intracardiac anomalies were the rule. Apart from a patent ductus arteriosus nearly all patients had a VSD. There were two patients in type A interruption without a VSD, but had an aortopulmonary window. A further type A interruption with an aortopulmonary window had a small muscular VSD which subsequently closed. A bicuspid aortic valve was documented in nine patients, all in type B, although the number was probably an underestimate of the total incidence [27]. Subaortic stenosis requiring surgical treatment was a major cause of persistent gradients [7, 11], recurrent surgery, and mortality and is reported elsewhere [13] (Fig. 6). There were six patients with a persistent truncus arteriosus and interruption [4, t7], subsequent surgery being required to replace the conduit between the right ventricle and distal pulmonary arteries. An anomalous right subclavian artery, either arising from the descending aorta or from the right pulmonary artery, was also noted in our series [14, 27] emphasizing the need to feel the carotid pulses in all infants with poor peripheral pulses, to exclude the possiblity of critical aortic stenosis [24]. Associated noncardiac anomalies were not uncommon. The presence of a chromosomal abnormality had significant influence on the subsequent management of three infants, as did the presence of a hypoplastic single kidney with poor renal function. Di George syndrome was looked for and thought to be present in 12 infants [26, 27]. When such a diagnosis was considered, irradiated blood was used until such time as it could be excluded or, alternatively, the noted deficiencies had returned to normal, as occurred in all survivors.

220

Intensive perioperative management played an important role in the care of these infants improving their hemodynamic status prior to surgery, a finding emphasized by others [9, 20, 21, 29]. To some extent this improvement was aided by the decreasing need for cardiac catheterization before surgery and the improved diagnostic yield of noninvasive crosssectional echocardiography [10, 22] (Figs. 3-6) Digoxin and diuretics have been supplemented by ventilation, infusion of inotropes, and prostaglandin E1 [21], maintaining duct patency and improving perfusion to the lower half of the body and the kidneys. Once the infants became stable, or, in a few instances, when it was not possible to reverse the ongoing acidosis, surgery was undertaken forthwith before a drop in the pulmonary vascular resistance and the development of increasing heart failure. Over the 10-year period reviewed there has been a progressive shift to a total primary correction from a staged repair. Arch reconstruction and palliation of the intracardiac anomalies was initially thought more likely to produce a successful outcome in the sick neonate [11, 23]. However, greater experience and aggressive perioperative treatment have led, especially in the latter 5-year period, to total correction [23] with only three early deaths in infants when treatment was withdrawn. In addition there has been a progressive shift from the insertion of a conduit [23, 29] to a direct anastomosis for reconstituting the aortic arch [19], doing away with the need for subsequent replacement of the conduit [20, 21]. To date two such patients have had a successful balloon angioplasty of the stenosed anastomosis, while a further one remains stable with a 30 mmHg gradient. The results from this series have suggested that the intermediate outcome of the second 5-year period, with a shift to a total one-stage repair, have been good. Further surgery has mainly been confined to the problems arising from recurrent subaortic stenosis [13], and the replacement of the right ventricle to pulmonary artery conduit in those patients who had truncus arteriosus, as well as interruption. Our experience of the last 10 years has shown that the aggressive perioperative and surgical management of infants born with IAA can lead to good quality survivors whose long-term outlook will be dependent on the associated cardiac and noncardiac anomalies.

Conclusions

The diagnosis of IAA was considered in any neonate who presented early with poor pulses with or without circulatory collapse. Cross-sectional echocardiography had been helpful in that diagnosis and

Pediatric Cardiology Vol. 13, No. 4, 1992

subsequent postoperative follow-up of these patients. Cardiac catheterization appeared to be required only if the initial diagnosis remained unclear and/or to clarify the associated anomalies or postoperative status. Intensive perioperative treatment was required in most patients and included inotropic support, prostaglandin E1 infusion, and ventilation. Early surgery was undertaken with a shift to a one-stage total intracardiac repair and direct anastomosis of the interruption to provide aortic arch continuity. Irradiated blood was used initially until the suspected diagnosis of Di George syndrome was excluded. The outcome of these survivors has progressively improved, though long-term follow-up is still required. Cross-sectional echocardiography remains the mainstay of investigation, particularly in reviewing the subaortic area. Further cardiac catheterization, angioplasty, or subsequent surgery may be required both for the repaired interruption and, particularly, for problems arising from the associated anomalies.

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