June 1976 TheJournalofPEDIATRICS

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Interrupted aortic arch in infancy Data from 30 infants with interrupted aortic arch in the New England Regional Infant Cardiac Program, 1968 to 1974, were reviewed. All patients had major associated cardiac abnormalities: approximately one third had ventricular septal defect with patent ductus arteriosus, one third had complex ventricular septal defect with left ventricular outflow obstruction, and one third had complex intracardiac lesions incompatible with survival. Twenty-one of the patients had either palliative or reconstructive surgery, with a hospital mortality rate of 76%. Nine patients did not have surgery," eight of them died at a median age o f four days. One-stage primary repair of interrupted aortic arch including the associated cardiac defects, using deep hypothermia and circulatory arrest, is proposed as the current method o f treatment in such infants.

Ruth L. Collins-Nakai, M.D.,* Macdonald Dick, M.D., Lucy Parisi-Buckley, M.D., Donald C. Fyler, M.D., and Aldo R. Castaneda, M.D., Boston, Mass.

INTERRUPTION of the aortic arch may be defined as discontinuity of the aortic arch in which either an aortic branch vessel or a patent ductus arteriosus supplies the descending aorta. This uncommon lesion was first described by Steidele in 17781 and was later classified into three types by Celoria and Patton3 The majority of patients with interrupted aortic arch have associated Cardiovascular anomalies which significantly influence prognosis and surgical management. 1-z9 This report reviews six years of experience (1968 to 1974) with interrupted aortic arch in infants in New England. From the Departments of Cardiology and Cardiovascular Surgery, Children's Hospital Medical Center, and the Departments o f Pediatrics and Surgery, Harvard Medical School. Supported in part by Grants H L 05855-06 and H L 09476-09from the National Institutes of Health and Project No. 260, Maternal and Child Health Service, Health Service Administration of the United States Public Health Service, Department of Health, Education, and Welfare. Presented in part at the Twenty-fourth Annual Scientific Session of the American College of Cardiology, February 10-13, 1975, Houston, Texas. *Reprint address: The Children's Hospital Medical Center, Department of Cardiology, 300 LongwoodAve., Boston, Mass. 02115.

MATERIALS AND M E T H O D S From 1968 to 1974, thirty infants with interrupted aortic arch were identified in the New England Regional Infant Cardiac Program (NERICP). TM Data regarding sex, anatomic classification, associated cardiovascular anomalies, age at surgery, type of surgery, and survival were collected. Three types of interrupted aortic arch ~ were identified: Type A, interruption distal to the left subclavian artery; Type B, interruption between the subclavian and left common carotid arteries; and Type C, interruption distal to the innominate artery, or to the right common carotid artery if the right subclavian artery is aberrant (Fig. 1). The data obtained from the NERICP were then compared to selected reviews in the literature? 6, 10, 14, 19, ~9 Twenty-one (70%) of these patients had surgery; nine patients (30%) did not have operations. Twelve of the 21 infants were less than 30 days and an additional six less than 3 months of age at the time of operation. All 21 surgical patients had either spontaneous (1) or operative (20) closure of the patent duCtus arteriosus. Nine infants had palliative operations: three had banding of the pulmonary artery, and six had interposition of a tubular synthetic conduit between the pulmooary artery proximal to a pulmonary artery band and the distal aorta. TM

Vol. 88, No. 6, pp. 959-962

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Collins-Nakai et aL

The Journal ofPediatncs June 1976

RCCLCC

RCCLCC

A

8

Reported N ~ 260

44%

51%

m~PaCp

30%

43%

N=27 N/K = 3 (10%)

RCC R ~

LCC

LS

C 5%

l~

Fig. 1. Three types of interrupted aortic arch, A, B, and C, and their frequencies in previous collected series and in NERICP series, 1968-1974. RPA = Right pulmonary artery; RS = right subclavian; RCC = right common carotid; LCC = left common carotid; LS = left subclavian; PDA = patent ductus arteriosus; LPA = left pulmonary artery; MPA = main pulmonary artery; As = ascending aorta. (Redrawn from Celoria GC, and Patton RB: Am Heart J 58:407, 1959.) Reconstructive operations were performed in 12 patients and encompassed: (1) primary anastomosis of the proximal and distal aortic segments (1 patien0; (2) anastomosis of the distal end of an aortic branch vessel (left common carotid, right subclavian, or left subclavian artery, or ductus arteriosus) to the descending aorta (5 patients); (3) interposition of a tubular prothesis between ascending and descending aorta (6 patients). RESULTS Interrupted aortic arch accounted for 1.3% of critical congenital heart disease seen in New England, as compiled by the NERICP from 1968 to 1974, and represented a frequency of 0.003/1,000 births. Ten infants (30%) had a Type A lesion, 13 had Type B (437~), and five had Type C (17%); three were not classified (10%) (Fig. 1). Distribution by sex was essentially equal (14 females). All 30 patient s had associated intracardiac abnormalities (Table I). Other serious noncardiac congenital abnormalities were present in 12 different patients (40%), most of whom had multipl e but not lethal anomalies. All patients presented with respiratory distress and mild to severe cyanosis, with or without decreased peripheral pulses. Twenty-seve n of 30 patients had signs or symptoms of congestive heart failure; the three remaining infants had low cardiac output states. Eight of nine infants who did not have surgery died at the median age of four days (range 1 to 13 days). The single surviving unoperated patient is alive and ambulatory at 5 1/2 years of age, with d-transposition of the great arteries, ventricular septal defect, and advanced pulmonary vascular obstructive disease. Five of the 21 patient s operated on for interrupted aortic arch survived initial surgery, three after palliative,

and two after reconstructive procedures. Two of the survivors, however, died at later operations. Palliative procedures were performed in nine patients, three of whom survived: one of three infants who had pulmonary artery banding alone, and two of six patients who ~had a conduit interposed between the main pulmonary artery (proximal to a pulmonary artery band) and the descending aorta. The child who survived pulmonary artery banding as the sole procedure had a ventricular septal defect and double aortic arch with interruption of one arch and coarctation of the uninterrupted arch; a complete repair is planned for this child. 1~ One of the two survivors after interpositio n of a pulmonary artery to descending aorta conduit is still alive at 6 years of age, but has a ventricular septal defect and severe pulmonary vascular obstructive disease. The second survivor died at 3 3/12 years of age after an attempt at complete correction (closure of ventricular septal defect, removal of pulmonary artery band with patch arterioplasty of the pulmonary artery, removal of the pulmonary artery to descending aorta conduit, and interposition of a new 10 mm dacron graft between ascending and descending aorta). She died from cerebral complications several weeks after the second operation. Postmortem examination revealed a good technical repair. Re,constructive procedures were successful in 2 of 12 patients, both of whom had 10 mm (diameter) synthetic tubular conduits interposed between proximal and distal aortic segments. One child is alive and ambulatory at 5 1/2 years of age. The other infant who had survived the arch reconstructive procedure died at a later aortic valvulotomy. In summary, 21 patients with interrupted aortic arch had surgers, either palliative or reconstructive; five patients survived the initial surgery, and three patients

Volume 88 Number 6

Interrupted aortic arch

Table I. Interrupted aortic arch: Associated cardiac anomalies (N = 30)

Anomalies

Total

Patent ductus arteriosus* Other cardiac communications Interventricular communications: Ventricular septal defect Ventricular septal defect with transposition of the great arteries Ventricular septal defect with underdeveloped left ventricle Single ventriclet Other communications Aortico-pulmonary window Atrial septal defect (only) Left-sided obstructive lesions Suba0rtic stenosis Valvar aortic stenosis Hypoplastic ascending aorta Double aortic arch with coarctation Other abnormalities Aberrant right subclavian artery Aberrant left subclavian artery

28 30 26 15 3 4 4 4 3 1 9 5 1 2 1 6 4

*One patient not known. tCommon A-V valve 1 "1 Mitral atresia 3 J~ Functional single ventricle.

Table lI. Interrupted aortic arch (N = 30): Associated noncardiac defects (N = 12) (40%)

Defects CNS abnormalities Gastrointestinal abnormalities Renal abnormalities Skeletal abnormalities DiGeorge syndrome Cleft lip and palate Trisomy 21

I

No. 6 5 4 4 3 1 1

have survived three to six years. Although the three survivors are ambulatory and at home, pulmonary vascular obstructive disease, congestive heart failure, recurrent pulmonary infections, a n d growth failure continue to present problems. DISCUSSION Interrupted aortic arch is an uncommon cardiac anomaly. In an earlier report (autopsy series) of interrupted aortic arch from this institution, this malformation represented 1.4% of autopsied infants with congenital heart disease, 4 which coincides with the NERICP incidence of 1.3% of critical congenital heart disease (defined as those infants who require cardiac catheterization or

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cardiac surgery or die of heart disease in the first year of life). Classification by site of the interruption in other collected series revealed the following distribution: Type A 44%, Type B 51%, Type C 5%. In contrast, our distribution was 30%, 43%, and 17%, respectively. This discrepancy, particularly in Type C lesions, may be explained in part by the preponderance of infants and greater mortality rate of Type C lesions in our series. This becomes especially apparent when one considers that reports which cite a lower incidence of Type C lesions include a larger number of patients older than one year of age, thus excluding patients who do not survive beyond infancy. Interrupted aortic arch in our series was always associated with other defects, most commonly patent ductus arteriosus and ventricular septal defect. Approximately one third of the infants had, as their associated cardiac defects, only a ventricular septal defect and a patent ductus arteriosus and, therefore, are potentially salvageable and could be expected to benefit from aggressive surgical management. The age of death in infants with medical management alone was, on the average, less than a week (10 days, 6 five days, 4 and this report, four days), and included in our group eight of the nine unoperated patients. The cause of death was usually cardiac failure. Thus surgery was the only alternative available to these infants. Eight patients reported in the literature were operated within the first month of life and two others within the first three months?, 6. 6-11.14, 16Three infants survived onestage aortic arch reconstruction and intracardiac repair? 6-12We have one survivor, now age 5 1/2 years, who underwent total repair at 2 I/2 years of age, with good hemodynamic results (no residual ventricular septal defect, no ascending aorta-descending aorta gradient). The coexistence of complex cardiac anomalies with interrupted arch most likely contributed to the high infant mortality rate? DIAGNOSTIC AND SURGICAL CONSIDERATIONS Early and precise diagnosis, which must include both the site of aortic arch interruption and associated cardiovascular abnormalities, is imperative if the potentially more salvageable group of patients (with ventricular septal defect and patent ductus arteriosus) is to be selected for early surgical repair. The presence of left-sided obstructive lesions, common atrioventricular valves, or single ventricle militates against successful surgical repair.2, The type of surgical procedure itself must be considered

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carefully before attempting any operation on infants with an interrupted aortic arch. Palliation bears a high mortahty rate and implies later reoperation with its attendant morbidity and mortality. Ideally, one-stage reconstructive surgery, including the establishment of aortic continuity by direct anastomosis or by interposition of a synthetic conduit between ascending and descending aorta with simultaneous repair of the intracardiac lesions, should be carried out. Several reports 1~ have appeared which cite successful primary reconstruction of the interrupted aortic arch in infants including repair o f the coexisting intracardiac lesions. D e e p hypothermia and circulatory arrest m a y facilitate this procedure in small infants. REFERENCES

1. Steidele RJ: Sammig. Verschiedener in der chirug, 'prakt, Lehrschule Gemachten Beobb 2:114, 1777-78. 2. Celoria GC, and Patton RB: Congenital absence of the aortic arch, Am Heart J 58:407, 1959. 3. Rochette M, Stanley P, Ethier MF, and Davignon A: Complete interruption of the aortic arch in infancy, Can Med Assoc J 98:131, 1968. 4. Van Praagh R, Bernhard WF, Rosenthal A, Parisi LF, and Fyler DC: Interrupted aortic arch: surgical treatment, Am J Cardiol 27:200, 1971. 5. Moiler JH, and Edwards JE! Interruption of aortic arch. Anatomic patterns and associated cardiac malformations, Am J Roentgenol 95:3557, 1965. 6. Murphy DA, Collins G, and Dobell ARC: Surgical correction of Type A congenital aortic arch interruption, Ann Thorac Surg 11:593, 1971. 7. Losman JG, Joffe HS, Beck W, and Barnard C: Successful total repair of interrupted aortic arch associated with ventricular septal defect and large patent ductus arteriosus, Am J Cardiol 33:566, 1974. 8. Tyson KET, Harris LC, and Nghiem QX: Repair of aortic arch interruption in the neonate, Surgery 67:1006, 1970.

The Journal of Pediatrics June 1976

9. Norton JB, Ullyet DJ, Rudolph AM, and Edmunds LN: Aortic arch atresia with transposition of the great vessels: physiologic considerations and surgical management, Surgery 6"7:1011, 1970. 10. Baratt-Boyes BG, Nicholls TT, Brandt PWT, and Neutze JM: Aortic arch interruption associated with patent ductus arteriosus, ventricular septal defect, and total anomalous pulmonary venous connection, J Thorac Cardiovasc Surg 63:367, 1972. 11. Murphy DA, Lemure GG, Tessler I, and Dunn GL: Correction of Type B aortic arch interruption with ventricular and atrial septal defects in a three-day-old infant, J Thorac Cardiovasc Surg 65:882, 1973. 12. Trusler GA, and Izukava T: Interrupted aortic arch and ventricular septal defect: direct repair through a median sternotomy incision in a 13-day-old infant, J Thorac Cardiovasc Surg 69:126, 1975. 13. Litwin SB, Van Praagh R, and Bernhard WF: A palliative operation for certain infants with aortic arch interruption, Ann Thorac Surg 14:369, 1972. 14. Tawes RL, Panagopoulos P, Aberdeen E, Waterston D J, and Bonham-Carter RE: Aortic arch atresia and interruption of the aortic arch: experience in 11 cases of operation, J Thorac Cardiovasc Surg 58:492, 1969. 15. Brenner JI, Berman MA, Talner NS, and Stansel HC: Double aortic arch system producing obstruction to left ventricular outflow, Am J Cardiol 34:855, 1974. 16. Singh MP, Bentall HH, and Oakley CM: Successful total correction of congenital interruption of the aortic arch and ventricular septal defect, Thorax 25:615, 1970. 17. Asano K, Washio M, and Griswalt T: Surgical treatment of aortic arch interruption, Jap J Thorac Surg 21:562, 1968. 18. Fyler DC, Parisi LF, and Berman MA: Regionalization of infant cardiac care in New England, Cardiovasc Clin 4:3, 1972. 19. Jones EL, Plauth WH, and Hatcher CR Jr: A palliative operation for all types of aortic arch interruption in the neonate, J Thorac Cardiovasc Surg 69:579, 1975. 20. Feldman M, Ruiz J, Price EC, and Cooley DA: Interrupted aortic arch: surgical correction, Cardiovasc Dis Bull Texas Heart Inst 165:1974.

Interrupted aortic arch in infancy.

Data from 30 infants with interrupted aortic arch in the New England Regional Infant Cardiac Program, 1968 to 1974, were reviewed. All patients had ma...
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