Simplified method for total correction of interrupted aortic arch with ventricular septal defect in infancy

J THORAC CARDIOVASC SURG 78:744-749, 1979

Simplified method for total correction of interrupted aortic arch with ventricular septal defect in infancy Type A interrupted aortic arch with a ventricular septal defect (VSD) in a 3-month-old infant was successfully corrected with the aid of profound hypothermia, limited cardiopulmonary bypass, and total circulatory arrest. The aortic arch was reconstructed by side-to-side anastomosis of the ascending aorta and the main pulmonary artery and then creation of a tube from the anastomotic orifice to the patent ductus arteriosus (PDA) by using a superabundant flap of the anterior wall of the main pulmonary artery. The pulmonary arteriotomy and VSD then were closed. The operative field can be approached easily through a median sternotomy with minimum dissection. Cardiac catheterization and angiocardiography 2 months later demonstrated a satisfactory reconstruction of both the aortic arch and the pulmonary artery.

Ryusuke Muraoka, M.D., Dr.Med.Sci., Michio Yokota, M.D., Dr.Med.Sci., Minoru Aoshima, M.D., Shinichi Nomoto, M.D., Masataka Osaragi, M.D., Inshin Kyoku, M.D., Hiroyuki Nakano, M.D., Ken Veda, M.D., and Akihiro Saito, M.D.,

Shizuoka, Japan

Interrupted aortic arch is an uncommon malformation frequently associated with intracardiac anomalies, of which ventricular septal defect (VSD) is the commonest. 1 As the majority of patients with this condition die in early infancy, 2-4 surgical intervention must be directed to this age group. Successful palliative operations have been reported by several authors. ;-9 However, the risk of operation remains high and postoperative improvement is not always satisfactory. Iffeasible, one-stage total correction would seem to be preferable. Five successful primary total corrections in infancy have been reported in the literature,10-14 but the procedures employed were complicated. This report describes a simple method to reconstruct the new aortic arch, which consists of side-to-side anastomosis of the ascending aorta and the main pulmonary artery and separation of the systemic and the pulmonary circulations with a flap of the anterior wall of the main pulmonary artery. From the Department of Cardiovascular Surgery and Cardiology, Shizuoka Children's Hospital, Shizuoka, Japan. Address for reprints: Ryusuke Muraoka, M.D., Dr.Med.Sci., Department of Cardiovascular Surgery, Shizuoka Children's Hospital, 860 Urushiyama, Shizuoka City 420, Japan. Received for publication March 7, 1979. Accepted for publication April 18, 1979.

744

Case report A 3-month-old female infant was admitted to Shizuoka Children's Hospital for tachypnea with a cardiac murmur and a history of poor feeding. The baby had been born of a term, normal delivery with a birth weight of 3,140 gm, and the mother's antenatal history had been uneventful. On admission, she was an undernourished, tachypneic infant with pale skin but without distinct differential cyanosis. Moist and dry rales throughout both lung fields and moderate hepatomegaly were present. The femoral pulses were easily palpable. Along the left sternal border, a Grade 2/6 systolic murmur with a blowing quality and long duration was audible. On chest roentgenogram, patchy shadows were visible in the right upper and middle lung fields and the pulmonary vasculature was increased. The cardiothoracic ratio was 0.57. The electrocardiogram revealed biventricular hypertrophy with a mean electric QRS axis of + 100 degrees. Marked dilatation of the right ventricle and moderate dilatation of the right atrium with an essentially normal-sized left atrium and ventricle were detected on echocardiograms. The diagnosis of congestive heart failure with respiratory infection was made, and aggressive medical management with antibiotics, digoxin, and dopamine was begun. There was no improvement after 48 hours of vigorous therapy. Cardiac catheterization, performed 2 days after admission with the aid of endotracheal intubation and artificial ventilation, revealed marked pulmonary hypertension and a large left-to-right shunt at the ventricular level (Table I). Right ventricular cineangiograms showed an enlarged pulmonary artery, and the descending aorta was seen to fill from this injection through a large ductus. An aortogram via the right carotid artery showed com-

0022-5223/79/110744+06$00.60/0 © 1979 The C. V. Mosby Co.

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745

Table I. Cardiac catheterization data O2 sat. (%)

Site

Main pulmonary artery Right ventricle Right atrium Superiorvena cava Inferiorvena cava Left atrium Left ventricle Descending aorta

88/18 94/4 (13)* 12 (mean)

II (mean) 12 (mean) 92/4 (13)* 92/58

12.8 13.2 7.5 7.3 5.8 12.6 12.1 12.8

85.5 86.5 80.0

*End-diastolic pressures

Table II. Anesthesia for profound hypothermia Drug

Premedication: Scopolamine Maintenance: Althesin* Fentanyl

I

Dosage

0.02 mg 2.3 mI 100 gm drip infusion

(during cooling) Chlorpromazine Nitrous oxide gas Pancuronium bromide CO2 gas

4 mg 50% I. 7 mg divided in 6 doses 2%-3% in volumebelow 31° C

*Althesin (Glaxo) contains 9 mg/ml of alphaxalone and 3 mg/ml of alphadolone acetate. plete interruption of the aortic arch distal to the left subclavian artery (Fig. I). After cardiac catheterization, artificial ventilation could not be discontinued for 6 days because of dyspnea despite roentgenologic improvement and decrease of bronchopulmonary secretion. After being weaned from the respirator, she continued to be dyspneic, with the high Pco, levels ranging from 52.7 to 80.0 mm Hg. While dopamine, digoxin, and diuretics were continued, marked hepatomegaly and peripheral edema developed. Three days after weaning from the respirator, she was reintubated and artificially ventilated because of a dyspneic attack that seemed to be caused by severe congestive heart failure. An emergency operation was carried out on Oct. 19, 1978. Her age at operation was 3 months, 12 days and body weight was 3,940 gm. The operation was performed with the aid of profound hypothermia, which was essentially the same method as previously described by Hikasa." Mori , 16 Muraoka," and colleagues. Anesthesia was maintained with a mixture of intravenous Althesin, fentanyl, and chlorpromazine (Table II), with pancuronium bromide as a muscle relaxant. Carbon dioxide gas, 2% to 3% of volume, was mixed with inhaled gases below 31° C of core temperature. Surface cooling to a rectal temperature of 22.0° C was achieved with a cooling blanket and ice bags. A median sternotomy incision was performed at a rectal temperature of 31.4° C during surface cooling after removal of ice bags from the anterior part of the chest. The heart was in situs solitus with normally related great arteries. The ascending aorta, 7.5 mm in diameter, ran

Fig. 1. Preoperative aortogram (anteroposterior view) shows type A interrupted aortic arch. directly cephalad to divide into the right brachiocephalic truncus, the left common carotid artery, and the left subclavian artery. The main pulmonary artery was very large, 17.5 mm in diameter, and the right and left pulmonary arteries were 7 and 6 mm in diameter, respectively. After dissection of these vessels, heparin, 2 mg/kg , was given at a rectal temperature of 25.0° C. A single venous cannula was inserted into the right atrium through the right atrial appendage, and two arterial cannulas were inserted into both the ascending aorta and the main pulmonary artery. These cannulas were connected to the arterial line of the pump oxygenator by means of a Y connector. The heart-lung machine was primed with 750 ml of fresh acid-citrate-dextrose blood (added heparin and calcium gluconate), 300 ml of Saviosol* (3% dextran in lactated Ringer's solution), 150,000 units of Trasylol.t 10 ml of 8% sodium bicarbonate, and antibiotics; this solution was cooled to 15.5° C. The ascending aorta, the main pulmonary artery, the right and left pulmonary arteries, and the superior and inferior venae cavae were taped. Core cooling was commenced at the rectal temperature of 22.0° C for 12 minutes and reduced the temperature to 15.2° C. During bypass the right and left pulmonary arteries were occluded with tourniquets to prevent perfusion of the lungs. Circulation was stopped and the ascending aorta then was cross-clamped distal to the aortic cannula. Through this cannula 10 ml of Young's solution was injected into the aortic root to protect the myocardium. The arterial cannulas were removed and the superior and inferior venae cavae were snugged. Both the ascending aorta and distal portion of the main pulmonary artery were incised vertically, and a side-to-side *Green Cross Company, Osaka, Japan. tBayer, A. G., Leverkusen, West Germany.

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(A)

(D)

(C)

Fig. 2. Method of repair for interrupted aortic arch in the present case. A. Both the ascending aorta and distal portion of the main pulmonary artery were incised vertically and a side-to-side anastomosis was performed. B. The anterior wall of the main pulmonary artery was incised obliquely from the proximal portion of the anastomosis to the midpoint between the ductus and the left pulmonary artery. C. The edge of the flap of the main pulmonary artery was sutured to the inside wall of the pulmonary artery to form a tube from the anastomosis to the ductus and the descending aorta. D. The edge of the incised main pulmonary artery was sutured to the anterior aspect of newly built tube which connected the ascending aorta to the descending aorta. Ao. Aorta. PA. Pulmonary artery. PDA. Patent ductus arteriosus. RPA. Right pulmonary artery. LPA. Left pulmonary artery. anastomosis was performed with continuous Prolene suture (Fig. 2. A). The anterior aspect of the main pulmonary artery was incised obliquely from the proximal portion of the anastomosis just formed to the midpoint between the ductus and the left pulmonary artery (Fig. 2, B). The edge of the flap of the main pulmonary artery was sutured to the posterior intimal surface of the pulmonary artery to form a tube extending from the aorta-pulmonary anastomosis to the ductus and the descending aorta (Fig. 2, C). The leftward edge of the incised main pulmonary artery was sutured to the anterior aspect of the newly built tube, which connected the ascending aorta to the descending aorta (Fig. 2,D). The infracristal YSD, 9 mm in diameter, was closed through a right ventriculotomy with a Dacron patch by means of interrupted mattress sutures. The aortic cannula was replaced and, after air was evacuated, bypass was recommenced and the infant was rewarmed to a nasopharyngeal temperature of 34.7° C. During bypass the right ventriculotomy was closed. Bypass was discontinued after 26 minutes and the cannulas were removed. The period of total circulatory arrest was 67 minutes. The procedure is summarized in Table III. Postoperatively, the patient required mechanical ventilation for 6 days and dopamine and/or isoproterenol for 8 days

for respiratory and circulatory support. Subsequent recovery was uneventful. and she was discharged to her home on Dec. 28, 1978, in excellent condition without any medication. Cardiac catheterization and angiocardiography at discharge demonstrated no evidence of significant stenosis of the new aortic arch or of the pulmonary artery (Figs. 3 and 4).

Discussion Interrupted aortic arch is a rare congenital anomaly, probably constituting less than I % of all congenital heart lesions." In this condition, the ascending and descending portions of the aorta are not continuous, and in almost all cases blood flow to the descending aorta occurs through a patent ductus arteriosus (PDA). The pattern of origin of the aortic vessels has been classified by Celoria and Patton 18 into three types: The interruption occurs distal to the left subclavian artery in type A, distal to the left common carotid artery in type B, and distal to the innominate or right common carotid artery in type C. The incidence of types A, B, and C was

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Fig. 4. Postoperative angiocardiogram (anteroposteriorview) shows no significant stenosis at any site of the pulmonary artery.

Table III. Operative procedure Rectal temperatures (OC)

Procedure

Fig. 3. Postoperative aortogram (lateral view) shows the aortic arch repair with a slight narrowing at the ductus where no gradient was noted in the pressure study. 42%,53%, and 4%, respectively, of the cases reviewed by Van Praagh and his associates." Interrupted aortic arch is almost always associated with PDA and VSD, but other defects such as complete transposition of the great arteries, truncus arteriosus, and atrial septal defect can coexist with this condition. 1 This anomaly is associated with a very short life. The reported mean age at death is only 10 days ," and 80% of the patients die in the first month of life. 3 Only 10% survive the first year. 4 Since both interrupted aortic arch and associated cardiovascular anomalies are responsible for the fatal outcome in early infancy, surgical intervention at this time is imperative and should include repair of interrupted aortic arch and correction or palliation of associated anomalies. Several authors have reported successful reconstruction of interrupted aortic arch combined with pulmonary artery banding in infancy. 5-9 However, clinical improvement was not always satisfactory and, in addition, further operations will be required for these infants. If feasible, one-stage total correction in infancy would seem to be preferable. The first successful total correction of interrupted aortic arch (type A) with

Median sternotomy incision Heparin administration (2 mg/kg) Insertion of arterial and venous cannulas Core cooling Circulatory arrest Aortic arch reconstruction VSD closure Core rewarming

31.4

25.0 25.0-22.5 12

67

22.0-17.0 17.0-15.1

31 16

26

15.1-33.2

Legend: VSD. Ventricular septal defect.

PDA, VSD, and total anomalous pulmonary venous connection was reported by Barratt-Boyes and his associates'" in 1972. Their patient was an 8-day-old boy in whom the interrupted aortic arch was reconstructed with a Dacron graft, for which a separate left thoracotomy and median sternotomy were used. Murphy and his associates!' reported the second successful total correction of this anomaly (type B) associated with VSD and atrial septal defect in a 3-day-old female infant in 1973. This case was also the first successful correction of a type B anomaly, and aortic continuity was restored with homograft vein from the infant's father. A median sternotomy with extension into the third left intercostal space was used. The third case was reported by Trusler and Izukawa" in 1975. A 13-day-old female infant with interrupted aortic arch (type B) and VSD was treated by resection of the PDA and direct side-to-end anastomosis of the ascending and descending portions

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Table IV. Reported cases (including our case) of successful one-stage correction of interrupted aortic arch in infancy Author Barratt-Boyes et al. 1()

1972

8 days

A

Murphy et al. 11

1973

3 days

B

Trusler et al. 12

1975

13 days

B

Monro et al. 13

1977

6 mo

B

Bailey et all.

1978

2 days

B

3 mo

A

Muraoka et al.

Associated anomalies

Method of aortic arch reconstruction

PDA, VSD, andTAPVC PDA, VSD, and ASD

Dacron graft (ascenting to descending aorta) and ligation of PDA Homograft vein (ascending to descending aorta) and division of PDA

PDA, VSD, and ASD PDA and VSD PDA, VSD, and ASD PDA and VSD

Direct side-to-end anastomosis of ascending to descending aorta and resection of PDA Side-to-end anastomosis of ascending aorta to PDA-PA segment Direct side-to-end anastomosis of ascending to descending aorta and ligation of PDA Side-to-side anastomosis of ascending aorta to MPA and tube formation using wall of MPA

Approach Left thoracotomy and median sternotomy Median sternotomy with extension to left third intercostal space Median sternotomy Median sternotomy Bilateral transverse thoracotomy Median sternotomy

Legend: PDA, Patentductus arteriosus. VSD, Ventricular septal defect. TAPVC, Total anomalous pulmonary venous connection. ASD, Atrial septal defect. PA,

Pulmonary artery. MPA, Main pulmonary artery.

of the aorta after mobilization of the descending aorta. These procedures were carried out through a median sternotomy incision. The fourth successful total correction of the anomaly, reported by Monro and his associates 13 in 1977, was performed in a 6-month-old female infant with type B interrupted aortic arch associated with PDA and VSD. In this case, a flap of the pulmonary artery, together with the PDA, was dissected free and sutured so as to form a tube which then was anastomosed to the ascending aorta through a median sternotomy incision. This method of reconstruction of the new aortic arch was first proposed by Kawashima and his associates 19 in 1975. The fifth successful correction in infancy was reported by Bailey and his associates 14 in 1978, Their patient was a 2-day-old girl who had type B anomaly with PDA, VSD, and an atrial septal defect. The anomaly was repaired by direct end-to-side anastomosis of the descending and ascending portions of the aorta after ligation of the PDA and mobilization of 75% to 80% of the descending intrathoracic aorta through a bilateral transverse thoracotomy, Types of interrupted aortic arch, associated anomalies, and the methods employed in the previously reported five cases and our own are summarized in Table IV. In reconstructing a new aortic arch, synthetic graft'? or venous allograft!' is not a problem at the time of operation. However, later intervention will probably be necessary because of the relative stenosis produced by the conduit as the child grows, For total correction without any need for later operation, the best choice is to use an inherent vessel with its assumed potential for

growth. Direct anastomosis of the ascending and descending aorta is appropriate in this respect, but extensive dissection and mobilization of the descending aorta are necessary to achieve a tension-free anastornosis.P: 14 Both the operative time and the amount of intraoperative and postoperative hemorrhage are increased. Use of a large PDA as the new aortic arch seems reasonable for this highly lethal anomaly'? despite uncertainty about the long-term status of the ductus tissue. The first successful case of this type of aortic arch reconstruction in interrupted aortic arch was reported by Gomes and Mcfloon'" in 1971. In a 2-year-old boy with truncus arteriosus, the continuity between the right ventricle and the pulmonary arteries was established by an aortic homograft with the ductus left intact as a substitute aortic arch, Usually, the main pulmonary artery in interrupted aortic arch is large enough to allow separation of the systemic and pulmonary circulations by using the superabundant part of the pulmonary artery itself. Our method described here is extremely simple, without the need of dissecting the PDA or transecting the PDA- pulmonary arterial flap from the main pulmonary artery. Thus the time required for reconstruction of the new aortic arch and plastic repair of the main pulmonary artery was much shorter than for the other methods reported previously. The time for this procedure in the present case was only 31 minutes. Several approaches were used for the primary repair of the defects, such as combined left thoracotomy and median sternotomy, 10 median sternotomy with extension into the left thoracic cavity, II bilateral transverse thoracotomy," and median sternotomy alone.": 13 In

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Interrupted aortic arch with VSD

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our previous experience, compression of the lung during the aortic arch reconstruction through a left thoracotomy led to alveolar bleeding which resulted in excessive and uncontrollable bleeding following generalized heparinization. Aortic arch reconstruction as well as intracardiac repair could be manipulated easily through a median sternotomy in the method just described, In all of the previously reported five cases and in our own, surface-induced profound hypothermia combined with limited cardiopulmonary bypass and total circulatory arrest was used, as developed by Hikasa and coworkers." This approach is the method of choice. The technique of aortic arch reconstruction and separation and establishment of the pulmonary circulation described here is the simplest one available for early total correction in any type of interrupted aortic arch with intracardiac defects. It will also be applicable for repair of the coarctation complex with a sufficiently large PDA in early infancy. REFERENCES

2

3

4 5

6

7

8

Moller JH, Edwards JE: Interruption of the aortic arch. Anatomic patterns and associated cardiac malformations. Am J Roentgenol Radium Ther Nucl Med 95:557-572, 1965 Van Praagh R, Bernard WF, Rosenthal A, Parisi LF, Fyler DC: Interrupted aortic arch. Surgical treatment. Am J Cardiol 27:200-211, 1971 Rochette M, Stanley P, Ethier MF, Davignon A: Complete interruption of the aortic arch in infancy. Can Med Assoc J 98:131-139, 1968 Robert WC, Morrow AG, Braunwald E: Complete interruption of the aortic arch. Circulation 26:39-59, 1962 Norton 18, Ullyot DJ, Steward ET, Rudolph AM, Edmunds LH: Aortic arch atresia with transposition of the great vessels. Physiological considerations and surgical management. Surgery 67: 1011-1016, 1970 Tyson KRT, Harris LC, Nghiem QX: Repair of aortic arch interruption in the neonate. Surgery 67: 1006-10 10, 1970 Fishman NH, Bronstein MH, Berman W Jr, Roe BB, Edmunds LH Jr, Robinson SJ, Rudolph AM: Surgical management of severe aortic coarctation and interrupted aortic arch in neonates. J THORAC CARDIOVASC SURG 71:35-47, 1976 Ventemiglia R, OgIietti J, Wukasch DC, Hallman GL, Cooley DA: Interruption of the aortic arch. Surgical con-

9

10

II

12

13

14

15

16

17

18 19

20

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siderations. J THORAC CARDIOVASC SURG 72:235-242, 1976 Higgins CB, French JW, Silverman JF, Wexler L: Interruption of the aortic arch. Preoperative and postoperative clinical. hemodynamic and angiographic features. Am J Cardiol 39:563-571, 1977 Barratt-Boyes BG, Nicholls TT, Brandt PWT, Neutze JM: Aortic arch interruption associated with patent ductus arteriosus, ventricular septal defect, and total anomalous pulmonary venous connection. J THORAC CARDIOVASC SURG 63:367-373, 1972 Murphy DA, Lemire GG, Tessler I, 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 65:882-886, 1973 Trusler GA, Izukawa 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 69:126-131, 1975 Monro JL, Brawn W, Conway N: Correction of type B interrupted aortic arch with ventricular septal defect in infancy. J THORAC CARDIOVASC SURG 74:618-623, 1977 Bailey LL, Jacobson JG, Vyhmeister E, Petry E: Interrupted aortic arch complex. Successful total correction in the neonate. Ann Thorac Surg 25:66-70, 1978 Hikasa Y, Shirotani H, Satomura K, Muraoka R, Abe K, Tsushimi K, Yokota Y, Miki S, Kawai J, Mori A, Okamoto Y, Koie H, Ban T, Kanzaki Y, Yokota M: Open heart surgery in infants with an aid of hypothermic anesthesia. Arch Jpn Chir 36:495-508, 1967 Mori A, Muraoka R, Yokota Y, Okamoto Y, Ando F, Fukumasu H, Oku H, Ikeda M, Shirotani H, Hikasa Y: Deep hypothermia combined with cardiopulmonary bypass for cardiac surgery in neonates and infants. J THORAC CARDIOVASC SURG 64:422-429, 1972 Muraoka R, Hikasa Y, Shirotani H, Mori A, Okamoto Y, Koie H, Abe K, Yokota M, Shirai 0, Konishi Y: Openheart surgery in infants under two years of age using deep hypothermia with surface cooling and partial cardiopulmonary bypass. J Cardiovasc Surg 15:231-241, 1974 Celoria GC, Patton RB: Congenital absence of the aortic arch. Am Heart J 58:407-413, 1959 Kawashima Y, Oyama C, Mori T, Manabe H: Interruption of the aortic arch associated wih patent ductus arteriosus and ventricular septal defect. Proposal of a new surgical technique for total correction. J Cardiovasc Surg 16:426-431, 1975 Gomes MM, McGoon DC: Truncus arteriosus with interruption of the aortic arch. Report of a case successfully repaired. Mayo Clin Proc 46:40-43, 1971