- Email: [email protected]
Single-stage repair of aortic arch obstruction and associated intracardiac defects in the neonate
Single-Stage Repair of Aortic Arch Obstruction and Associated Intracardiac Defects in the Neonate Satinder
K. Sandhu, MB,BS, Robert H. Beekman, MD, Ralph S. Mosca, MD, and Edward L. Bove, MD
The effectiveness of a single-stage anterior approach for the repair of aortic arch obstruction and associoted intracardiac defects has not been well evaluated. We therefore reviewed our experience with 60 neonates (median age 8 days, range 1 to 28) who underwent a single-stage repair by way of a median sternotomy at our institution between 1986 and 1994. Nineteen (32%) had coorctation with ventricular septal defect, 18 (30%) had interrupted aortic arch with ventricular septal defect, and 23 (38%) had coarctation or interrupted aortic arch with complex intracardiac anatomy. The arch obstruction was repaired using resection and primary anastomosis in 54 patients, synthetic patch aortoplasty in 3, subclavian flap aortoplosty in 2, and an interposition gortex graft placement in 1. Total circulatory arrest time was 48 f 3 minutes (mean
f SEM). There were 7 early postoperative deaths (11.7%; 70% confidence limit 8% to 16.6%). The 53 survivors were followed for a mean of 23 months (range 1 to 78), for a total of 1,219 patient-months. Recurrent arch obstruction 220 mm Hg has occurred in 2 of 53 tients (3.8%; 70% confidence limit 1.9% to 7.5%); Ii th underwent successful balloon ongiopbs . There were 2 late deatfts, 1 of which was noncar Jriac. We conclude that repair of aortic arch obstruction and intracardiac defects by a single-stage approach through median stemotomy con be accomplished with low mortali in infancy, even with associated complex intraco r!Jiac anatomy. Recurrent coarctation is relative uncommon and can be successfully managed with baYloon angioplasty. (Am J Cordiol 1995;75:37&373)
he effectivenessof a single-stage anterior approach for the repair of aortic arch obstruction and associT ated intracardiac defects in neonateshas not been well
surgical repair, and early and late outcome including evidence of recurrent arch obstruction or residual intracardiac defects.Neonateswith a univentricular heart undergoing surgical palliation, and those undergoing isolated arch repair were excluded from the study. Operative techniques: All procedureswere done using a median stemotomy approach with a single period of deep hypothermia and circulatory arrest. The total circulatory arrest time was 48 f 3 minutes (mean f SEM). The bypass circuit was pruned with blood to achieve an equilibrated hcmatocrit of 15% to 20% during cardiopulmonary bypass. Ionized calcium levels were lowcred to 10.8 mg/dl by the addition of titrated blood, and were not corrected until 220 minutes after the crossclamp was removed. Cannulation for bypasswas accomplished by placing 1 arterial cannula into the ascending aorta and a second cannula from a bifurcated aortic line into the proximal main pulmonary trunk after occluding the branch pulmonary arteries. Venous return was achieved by a single right atria1 cannula. Cooling commenced immediately after the onset of cardiopulmonary bypass,with initial prime temperaturebetween28°C and 30°C. A single dose of blood cardioplegia (20 ml/kg) was administered to all patients at the onset of circulatory arrest. Complete mobilization of the ascendingaorta, transverse arch, arch vessels, and upper descending thoracic aorta was performed during the cooling period. Division of at least 1 or 2 intercostal arteries was usually required for adequate mobiliznion. The arch obstruction was repaired before the intracardiac portion of the procedure and was accomplished using resection and primary anastomosisin 54 patients, synthetic patch aortoplasty in 3, subclavian flap aortoplasty in 2, and an interposition gortex graft in 1.When the arch repair was performed by primary anastomosis.the descending aor-
evaluated. Debate continues regarding the advantagesof complete repair versus initial palliation, as well the method of coarctation repair itself.‘-5 Recent reports have advocatedthe useof resection and end-to-end anastomosis for repair of aortic coarctation, extending the anastomosisinto the transversearch when there is associated arch hypoplasia in infants.6,7Because the effectivenessof this approach has not been defined when performed from an anterior approach, we reviewed the results in all neonates who underwent a single-stage repair through an anterior approach of aortic arch obstruction and associated intracardiac defects at the University of Michigan Medical Center from January 1986 through February 1994.
METHODS The medical records of all 60 neonates (aged 128 days) with aortic arch obstruction and associatedintracardiac defects who underwent a single-stage repair through a median stemotomy approach betweenJanuary 1986 and February 1994 were retrospectively reviewed. Charts were reviewed for detailed diagnosis of the aortic arch anatomy and intracardiac defects, the nature of From the Department of Pediatrics, Division of Pediatric Cardiology, and the Department of Sur cry Section of Thoracic Surgery, Unir Ann Arbor, Michigan. Manuversity of Michigan School o PMedicrne, script received June 27, 1994; revised manuscript received and accepted October 17, 1994. Address for reprints: Satinder K. Sandhu, MD, Division of Pediatric Cardioloav. Oreaon Health Sciences Universitv 3 18 1 S. W. Sam Jackson ?ad;k Roa>, Portland, Oregon 9720 l-3098
370
THE AMERICAN
JOURNAL
OF CARDIOLOGY@
VOL. 7.5
FEB. 1.5, 1995
ta was spatulated and sutured to the TABLE I Early and Late Mortality opened undersurface of the transverse Weight at Age at arch with continuous absorbablesuture Early Late Operation (kg) Operation (d) material, most often extending the Number Mortality Mortality (mean f SEM) Group (median) anastomosisproximal to the left carotid Coarc. with VSD 19 1 (5%) 0 1.4-4.7 l-29 artery. Cardiopulmonary bypass was (3.1 * 0.2) (1 11 resumed and rewarming was begun IAA with VSD 18 2 (11.1%) 1 1.3-4 2-l 5 after completing the intracardiac repair (3.0 f 0.2) id Coorc./lAA/ 23 4 (17.4%) 1 2.1-4 2-24 or during the completion of the right complex (3.1 % 0.1) PI ventricular outflow tract reconstrucintracardiac tion. Hemofiltration was used during defects the final stagesof rewarming and carCoarc. = coorctation; I44 = interrupted aortic arch; VSD = ventricular septal defect. diopulmonary bypass was discontinued. with a hematocrit 235%. hdividual circumstances: Ventricular septal defect TABLE II Associated Anomalies closure was accomplished transatrially in most patients, Number with the exception of those with subpulmonic defects of (whose approach was through the main pulmonary Patients artery) and, occasionally, those in whom a ventriculotoDoubleoutlet right ventricle coarctation/ 5 my was necessaryfor placement of a conduit or outflow interrupted aortic arch Truncus arteriosus/interrupted aortic arch 5 patch. In patients with interrupted aortic arch or coarcMultiple left-sided lesions/coarctation 3 tation with isolated ventricular septal defect, subaortic D-transposition of the great arteries/coarctation 3 resection was performed in those with posterior deviaAtrioventricular septal defect/coarctation 3 Cor triatriatum/coarctation 2 tion of the infundibular septum who were predicted to septal 1 have a high likelihood of significant subaortic stenosis t-transposition of the great arteries/ventricular defect/pulmonary valve stenosis/coarctation after repair, aspreviously reported.*In patients with trunAnomalous right pulmonary vein/coronary artery from 1 cus arteriosus and interrupted aortic arch, the ascending main pulmonary artery/bicuspid aortic valve/coarctation aorta was augmented from the pulmonary artery exciSurvivors: There were 7 early postoperative deaths sion site to the anastomosis,with the descending aorta (operative mortality 11.7%;70% CL 8% to 16.6%).Four at the base of the left carotid artery. Follow-up data: Patients were evaluated on an out- of the 7 patients who died early had arch obstruction patient basis every 6 to 12 months after operation. The with complex intracardiac anatomy, 2 had interrupted pressuregradient acrossthe repair site was evaluatedby aortic arch with ventricular septal defect, and 1 had blood pressure measurementsin the upper and lower coarctation and ventricular septal defect with Noonan’s extremities by standard noninvasive techniques. All syndrome and endocrine abnormalities. The 53 survivors were followed for 1 to 78 months patients underwent complete color flow Doppler echocardiographic examination during the follow-up period. (mean23 If:3), for a total follow-up 1,219patient-months. Cardiac catheterization was performed when indicated There were 2 late deaths, 1 of which was noncardiac in origin. The tirst late death occurred in a preterm infant for hemodynamic evaluation or balloon angioplasty. with interrupted aortic arch, ventricular septal defect, Statistical methods: Data are presented as mean f SEM. A p value co.05 was required as evidence of a subaortic stenosis, DiGeorge syndrome, and microsignificant effect. The actuarial survival rates were mea- cephaly. He underwent repair at 15 days of age and was sured using the Kaplan-Meier survival function esti- dischargedhome with a residual arch gradient of 18 mm mates: The 70% confidence limit (CL) for the mean is Hg and mild subaortic stenosis. This patient died 4 months later due to aspirationpneumonia and sepsis.The reported where appropriate. secondlate death occurred in a child with Shone’s complex with coarctation and ventricular septal defect who RESULTS The study group consisted of 60 neonates between underwent repair at 4 days of age, without resection of the age of 1 and 28 days (mean 9.9 + 0.9, median 8). the infundibular septum. This patient developed severe These children ranged in weight from 1.3 to 4.69 kg subaortic stenosisand required a left ventricular apex to (mean 3 + 0.1). Nineteen (32%) had coarctation with descending aorta conduit 3 months later. Aortic valve ventricular septal defect, 18 (30%) had interrupted aor- replacementwith aortic root enlargement was later pertic arch with ventricular septal defect, and 23 (38%) had formed at 4 years of age, but the patient died from vencoarctation or interrupted aortic arch with complex intra- tricular fibrillation 2 days after the operation9 The Kaplan-Meier technique was used to evaluate cardiac anatomy (Table I). Interrupted aortic arch type A was present in 11patients, and type B in 14.Fourteen overall patient survival. The actuarial survival rate for patients had posterior deviation of the infundibular sep- all patients at 1,3,48,52, and 78 months was 8X%,86%, tum. Other associated defects are listed in Table II. 86%, 72%, and 72%, respectively. There was no statisExtracardiac defects included DiGeorge syndrome in 5 tically significant difference in survival among the 3 subpatients, Noonan’s syndrome in 1, XY chromosome in groups (p = 0.3801) using the actuarial technique, ala female child, and mixed karyotype 46 XY, 45X in though the early and late mortality rates listed in Table I suggesta greater mortality in the complex group. another child. CONGENITAL
HEART DISEASE/ARCH
REPAIR IN NEONATES
371
Early mortality was significantly increasedin patients weighing <3 kg. Sixteen patients weighed <3 kg, and there were 5 deaths in this group. In contrast, there were only 4 early deathsamong the 44 children who weighed >3 kg (p = 0.03, by chi-square analysis). Recurrent arch obslruction: Recurrent arch obstruction, defined as resting systolic pressure gradient 220 mm Hg, occurred in 2 of the 53 hospital survivors (3.8%; 70% CL 1.9% to 7.5%). Both patients had coarctation with ventricular septal defect and underwent repair by resection and end-to-end anastomosisat age 9 and 23 days, respectively. At the time of discharge from the hospital after repair, the gradients were 0 and 18 mm Hg, respectively. At follow-up of 2.5 and 5 months after operation, gradients of 30 and 70 mm Hg, respectively, were measured. These 2 patienls underwent successful balloon angioplasty, resulting in a decreasein residual gradient to 0 and 8 mm Hg, respectively. Both patients continued to have evidence of recoarctation 7 and 17months, repectively, after balloon angioplasty. In addition to these 2 patients, 9 other patients had a residual systolic gradient 120 mm Hg (range 10 to 18, mean 11.6+ 0.9) and continue to be hemodynamically stable at follow-up. Forty-two patients (79%) had no residual arch gradient. Left ventricular ouffbw obstruction: Left ventricular outflow tract obstruction was responsible for 1 early and 3 late interventions. One patient with double-outlet right ventricle, posterior deviation of the infundibular septum, and a hypoplastic left ventricle did not have subaortic resection and did poorly. This patient underwent reoperation for resection of the left ventricular outflow tract obstruction, takedown of the atrial and ventricular patches, and pulmonary artery band placement, but did not survive. In retrospect,the left ventricle was too small for a biventricular repair. Two patients developed significant subaortic stenosis which required late surgical intervention. In 1, subaortic stenosis was not present at initial operation. This patient required aortic valvotomy and resection of subaortic stenosis 3 years later. The second patient had valvar and subvalvar (tunnel type) aortic stenosis and required a left ventricular apex to descending aorta conduit 3 months after surgery and an aortic valve replacement with enlargement of the left venbicular outflow tract (Konno procedure) at age 4 years. He died 48 hours after operation from ventricular fibrillation.9 Another patient with valvar aortic stenosis underwent successful aortic balloon valvuloplasty 13 months after operation and is doing well. Three of 7 patients who had resection of the posteriorly deviated infundibular septum at the initial repair died after operation (2 early, and the other 4 months later from aspiration pneumonia). The remaining 4 infants had no residual subaortic gradients at follow-up 10to 24 months (median 18) after operation. Additional sequelae:One infant with truncus arteriosus and interrupted aortic arch developed left mainstem bronchial compressionat 3.5 months of age and required placement of an aortic interposition graft for relief of symptoms. Six patients have trivial ventricular level shunts that are hemodynamically insignificant. Two patients with truncus arteriosus have 25 and 36 mm Hg gradients, respectively,acrossthe right ventricular to pul-
This experience indicates that a single-stageanterior approach for repair of aortic arch obstruction and intracardiac defects can be accomplished with low mortality in the neonatedespite the complexity of these anomalies and the poor preoperativecondition of many of thesechildren. The overall mortality in this cohort was 15%, with an early mortality of 11.6%.In neonateswith an associated complex intracardiac defect, mortality was 17.4%; in neonateswith an interrupted aortic arch and ventricular scptal defect it was 11.1%;and in neonateswith coarctation and ventricular septal defect, it was only 5%. The mortality rate in the group with complex intracardiac anatomy is relatively high but compares favorably with the mortality reported by other groups utilizing a staged repair.6,7,‘(b’4Vouche et al6 reported their experience using a stagedapproachin patients with complex intracardiac defects: early mortality 36%, late mortality 44%. Van Heum et al’ reported 35 patients with coarctation and complex intracardiac defectswho underwent stagedrepair, with 11of 35 early deaths(31%), and 6 late deathsat the time of definitive repair (overall mortality 48%). It was their impression that even small residual coarctation gradients after operation (40 mm Hg) resulted in increasedmortality when associatedwith concurrent pulmonary artery banding. They concluded that biventricular outflow obstruction resulted in increased mortality, and that a single-stagerepair should be undertaken when appropriate. Previous reports of the single-stage approach in the repair have been cncourging. Karl et al’” reported 55 patients with arch obstruction, of which 45 underwent bivcntricular single-stage repair with an operative mortality of 9% (CL 5% to 15%).Turley et al” reported their results in 29 patients with interrupted aortic arch with ventricular septal defect using a single-stage approach and concluded that single-stagerepair could be achieved with lower mortality than palliation. In their series the operative mortality rate was 20%. Thus, survival after single-stage repair of these complex lesions compares favorably with that of the staged approach, suggesting that single-stage repair may be the preferable surgical strategy. Recoarctation (defined as a gradient 220 mm Ilg) occurred in only 2 of the 53 survivors in our series, and was successfully treated with balloon angioplasty. Both infants with recoarctation had a ventricular septal defect and a narrow transverse arch and underwent operation early in this series without extension of the distal aorta into the hypoplastic segment of transverse aorta. Routine use of an extended primary anastomosishas virtually eliminated residual coarctation in more recent patients. No child with interrupted aortic arch or coarctation with complex intracardiac anatomy has developed recurrent arch obstruction. Previous reports have described postoperative recoarctation in 9% to 25% of infants and in 15% to 25% of neonates.15-I7Van Heum et al’ compared actuarial freedom from recoarctation in 151children with subclavian flap, end-to-end anastomo-
372
FEB. 15, 1995
THE AMERICAN JOURNAL OF CARDIOLOGY@’
VOL. 7.5
monary artery conduits. No patient has developed an aneurysm of the aortic arch, paraplegia, or heart block. DISCUSSION
sis, or extended and radical end-to-end anastomosisrepairs. It was their impression that prognosis was best in patients with extended and radical end-to-end anastomosis.7In our series,the incidence of recoarctation was also low (3.7%), but the follow-up period was only 23 + 3 months; longer term data are needed to assessthe true incidence of recoarctation in these patients. A single-stage approach to the neonate with aortic arch obstruction and intracardiac defects has several advantagesover a stagedapproach.First, it provides the child with early and complete physiologic repair. Second, it sparesmost children the need for a second operation. Finally, the anterior approach provides improved surgical exposure, which may explain the low incidence of recurrent arch obstruction comparedwith other series. However, a single-stage approach may also have disadvantages. It requires more complex surgery during the neonatal period, including cardiopulmonary bypass and hypothermic arrest. In this series, the total period of hypothermic arrest was 48 minutes, and none of our patients had evidence of central nervous system injury.
1. &mini L, Gargiulo G, Albanese SB, Santorelli MC, Picchio FM, Pierangeli A. Aortic coarctation with hypoplastic arch in neonates: a spectrum of anatomic lesions requiring different surgical options. Ann Thorac Surg 1993;56:288-294. 2. Hesslein PS, McNamx~a DG, Morriss MJH, Hallman CL, Cooley DA. Comparison of resection versus patch aortoplasty for repair of coarctation in infants and children. Circulation 1981;64:16&168. 3. Be&man RH, Rocchini AP, Behrendt DM, Bove EL, Dick M, Crowley DC, Snider RA, Rosenthal A. Long-term outcome after repair of coarctation in infancy: subclavian angioplasty does not reduce the need for reoperation. J Am Coil Car-
dial 1986;8:140&1411. 4. Irwin ED, Braunlin EA, Faker JE. Staged repair of interrupted aotic arch and ventricular septal defect in infancy. Ann Thorac Surg 1991;52:632-639. 5. Hammon JW, Graham TP, Boucek RJ, Bender HW. Operative repair of coarctation of the aorta in infancy: results with and without ventricular septal defect. Am J Car&l 1985;55:1555-1559. 6. Vouche PR, Trinquet F, Lecompte Y, Vemant F, Roux PM, Tauati G, Pome G, Leca F, Neveux .N. Aortic coarctation with hypoplastic aortic arch-results of extended end-to-end aortic arch anastomosis. J Thorac Cardiovasc Surg 1988;96: 557-563. 7. van Heum LWE, Wang CM: Spiegelhalter DJ, Sorensen K, de Lewd MR, Stark J, Elliot MJ. Surgical treatment of aortic coarctation in infants younger than three months: 1985.1990. J Thorac Cardiovasc Surg 1994;107:74-86. 8. Bove EL, Minich L, Prijdan AK, Lupinetti FL, Snider RS, Dick M, Be&man RH. The management of severe subaortic stenosis, ventricular septal defect, and aottic arch obshuction in the neonate. J Thor-m Cardiovasc Surg 1993; 105:2X9-296. 9. Scott WA, Rocchini AP, Bow EL, Behrendt DM, Beckman RH, Dick M, Serwer G, Snider R, Rosenthal A. Repair of interrupted aortic arch in infancy. J Thorat Cardiovasc Surg 1988;96:56&568. 10. DietJ CA, Torres AR, Favaloro RG, Fessler CL, Gmnkemeier GL. Risk of recoarctation in neonates and infants after repair with patch aortoplasty, sub&viz& flap, and the combined resection-flap procedure. J Thorac Cardiovasc Surg 1992; 103:724-732. 11. Turley K, Yee ES, Ebert PA. The total repair of interrupted aortic arch complex in infants: the anterior approach. Circ&fion 1984;7O(suppl I)%lM-20. 12. Planche C, Serraf A, Comas JV, Lacour-Gayet F, Bmniaux .I, Touchot A, Kirklin JW. Anatomic repair of transposition of great arteries with ventricular septal defect and aortic arch obstmction. J ThorF Cardiovasc Surg 1993;105:925-933. 13. Karl TR, &no S, Brawn W, Mee RBB. Repair of hypoplastic or interrupted aortic arch via sternotomy. J Thorac Cardiovasc Surg 1992;104:688-695. 14. Sell JE, Jonas RA, Mayer JE, Blackstone EH, Kirklin JW, Castaneda AR. The results of a surgical program for interrupted aortic arch. J Thorac Cardiovasc Surg 1988;96:864-877. 15. Kirklin JW, Barratt-Boyes BG. Coarctation of the aorta and aortic arch intaoption. In: Kirklin JW, Barr&t-Boyes BG, eds. Cardiac Surgery. 1st ed. New York: Churchill-Livingstone, 1988:1035-1080. 16. Ziemer G, Jonas RA, Perry SB, Freed MD, &tan& AR. Surgery for coarctation of the aorta in the neonate. Circularion 1986;74(suppl 1):1-25-I-31. 17. Korfer R, Meyer H, Kleikamp G, Bircks W. Early and late results after resection and end-to-end anastomosis of coaxtation of the thoracic aorta in early infancy. J Thorac Cardiovasc Sur-g 1986;89:6164i22.
CONGENITAL
HEART DISEASE/ARCH REPAIRIN NEONATES
373
K. Sandhu, MB,BS, Robert H. Beekman, MD, Ralph S. Mosca, MD, and Edward L. Bove, MD
The effectiveness of a single-stage anterior approach for the repair of aortic arch obstruction and associoted intracardiac defects has not been well evaluated. We therefore reviewed our experience with 60 neonates (median age 8 days, range 1 to 28) who underwent a single-stage repair by way of a median sternotomy at our institution between 1986 and 1994. Nineteen (32%) had coorctation with ventricular septal defect, 18 (30%) had interrupted aortic arch with ventricular septal defect, and 23 (38%) had coarctation or interrupted aortic arch with complex intracardiac anatomy. The arch obstruction was repaired using resection and primary anastomosis in 54 patients, synthetic patch aortoplasty in 3, subclavian flap aortoplosty in 2, and an interposition gortex graft placement in 1. Total circulatory arrest time was 48 f 3 minutes (mean
f SEM). There were 7 early postoperative deaths (11.7%; 70% confidence limit 8% to 16.6%). The 53 survivors were followed for a mean of 23 months (range 1 to 78), for a total of 1,219 patient-months. Recurrent arch obstruction 220 mm Hg has occurred in 2 of 53 tients (3.8%; 70% confidence limit 1.9% to 7.5%); Ii th underwent successful balloon ongiopbs . There were 2 late deatfts, 1 of which was noncar Jriac. We conclude that repair of aortic arch obstruction and intracardiac defects by a single-stage approach through median stemotomy con be accomplished with low mortali in infancy, even with associated complex intraco r!Jiac anatomy. Recurrent coarctation is relative uncommon and can be successfully managed with baYloon angioplasty. (Am J Cordiol 1995;75:37&373)
he effectivenessof a single-stage anterior approach for the repair of aortic arch obstruction and associT ated intracardiac defects in neonateshas not been well
surgical repair, and early and late outcome including evidence of recurrent arch obstruction or residual intracardiac defects.Neonateswith a univentricular heart undergoing surgical palliation, and those undergoing isolated arch repair were excluded from the study. Operative techniques: All procedureswere done using a median stemotomy approach with a single period of deep hypothermia and circulatory arrest. The total circulatory arrest time was 48 f 3 minutes (mean f SEM). The bypass circuit was pruned with blood to achieve an equilibrated hcmatocrit of 15% to 20% during cardiopulmonary bypass. Ionized calcium levels were lowcred to 10.8 mg/dl by the addition of titrated blood, and were not corrected until 220 minutes after the crossclamp was removed. Cannulation for bypasswas accomplished by placing 1 arterial cannula into the ascending aorta and a second cannula from a bifurcated aortic line into the proximal main pulmonary trunk after occluding the branch pulmonary arteries. Venous return was achieved by a single right atria1 cannula. Cooling commenced immediately after the onset of cardiopulmonary bypass,with initial prime temperaturebetween28°C and 30°C. A single dose of blood cardioplegia (20 ml/kg) was administered to all patients at the onset of circulatory arrest. Complete mobilization of the ascendingaorta, transverse arch, arch vessels, and upper descending thoracic aorta was performed during the cooling period. Division of at least 1 or 2 intercostal arteries was usually required for adequate mobiliznion. The arch obstruction was repaired before the intracardiac portion of the procedure and was accomplished using resection and primary anastomosisin 54 patients, synthetic patch aortoplasty in 3, subclavian flap aortoplasty in 2, and an interposition gortex graft in 1.When the arch repair was performed by primary anastomosis.the descending aor-
evaluated. Debate continues regarding the advantagesof complete repair versus initial palliation, as well the method of coarctation repair itself.‘-5 Recent reports have advocatedthe useof resection and end-to-end anastomosis for repair of aortic coarctation, extending the anastomosisinto the transversearch when there is associated arch hypoplasia in infants.6,7Because the effectivenessof this approach has not been defined when performed from an anterior approach, we reviewed the results in all neonates who underwent a single-stage repair through an anterior approach of aortic arch obstruction and associated intracardiac defects at the University of Michigan Medical Center from January 1986 through February 1994.
METHODS The medical records of all 60 neonates (aged 128 days) with aortic arch obstruction and associatedintracardiac defects who underwent a single-stage repair through a median stemotomy approach betweenJanuary 1986 and February 1994 were retrospectively reviewed. Charts were reviewed for detailed diagnosis of the aortic arch anatomy and intracardiac defects, the nature of From the Department of Pediatrics, Division of Pediatric Cardiology, and the Department of Sur cry Section of Thoracic Surgery, Unir Ann Arbor, Michigan. Manuversity of Michigan School o PMedicrne, script received June 27, 1994; revised manuscript received and accepted October 17, 1994. Address for reprints: Satinder K. Sandhu, MD, Division of Pediatric Cardioloav. Oreaon Health Sciences Universitv 3 18 1 S. W. Sam Jackson ?ad;k Roa>, Portland, Oregon 9720 l-3098
370
THE AMERICAN
JOURNAL
OF CARDIOLOGY@
VOL. 7.5
FEB. 1.5, 1995
ta was spatulated and sutured to the TABLE I Early and Late Mortality opened undersurface of the transverse Weight at Age at arch with continuous absorbablesuture Early Late Operation (kg) Operation (d) material, most often extending the Number Mortality Mortality (mean f SEM) Group (median) anastomosisproximal to the left carotid Coarc. with VSD 19 1 (5%) 0 1.4-4.7 l-29 artery. Cardiopulmonary bypass was (3.1 * 0.2) (1 11 resumed and rewarming was begun IAA with VSD 18 2 (11.1%) 1 1.3-4 2-l 5 after completing the intracardiac repair (3.0 f 0.2) id Coorc./lAA/ 23 4 (17.4%) 1 2.1-4 2-24 or during the completion of the right complex (3.1 % 0.1) PI ventricular outflow tract reconstrucintracardiac tion. Hemofiltration was used during defects the final stagesof rewarming and carCoarc. = coorctation; I44 = interrupted aortic arch; VSD = ventricular septal defect. diopulmonary bypass was discontinued. with a hematocrit 235%. hdividual circumstances: Ventricular septal defect TABLE II Associated Anomalies closure was accomplished transatrially in most patients, Number with the exception of those with subpulmonic defects of (whose approach was through the main pulmonary Patients artery) and, occasionally, those in whom a ventriculotoDoubleoutlet right ventricle coarctation/ 5 my was necessaryfor placement of a conduit or outflow interrupted aortic arch Truncus arteriosus/interrupted aortic arch 5 patch. In patients with interrupted aortic arch or coarcMultiple left-sided lesions/coarctation 3 tation with isolated ventricular septal defect, subaortic D-transposition of the great arteries/coarctation 3 resection was performed in those with posterior deviaAtrioventricular septal defect/coarctation 3 Cor triatriatum/coarctation 2 tion of the infundibular septum who were predicted to septal 1 have a high likelihood of significant subaortic stenosis t-transposition of the great arteries/ventricular defect/pulmonary valve stenosis/coarctation after repair, aspreviously reported.*In patients with trunAnomalous right pulmonary vein/coronary artery from 1 cus arteriosus and interrupted aortic arch, the ascending main pulmonary artery/bicuspid aortic valve/coarctation aorta was augmented from the pulmonary artery exciSurvivors: There were 7 early postoperative deaths sion site to the anastomosis,with the descending aorta (operative mortality 11.7%;70% CL 8% to 16.6%).Four at the base of the left carotid artery. Follow-up data: Patients were evaluated on an out- of the 7 patients who died early had arch obstruction patient basis every 6 to 12 months after operation. The with complex intracardiac anatomy, 2 had interrupted pressuregradient acrossthe repair site was evaluatedby aortic arch with ventricular septal defect, and 1 had blood pressure measurementsin the upper and lower coarctation and ventricular septal defect with Noonan’s extremities by standard noninvasive techniques. All syndrome and endocrine abnormalities. The 53 survivors were followed for 1 to 78 months patients underwent complete color flow Doppler echocardiographic examination during the follow-up period. (mean23 If:3), for a total follow-up 1,219patient-months. Cardiac catheterization was performed when indicated There were 2 late deaths, 1 of which was noncardiac in origin. The tirst late death occurred in a preterm infant for hemodynamic evaluation or balloon angioplasty. with interrupted aortic arch, ventricular septal defect, Statistical methods: Data are presented as mean f SEM. A p value co.05 was required as evidence of a subaortic stenosis, DiGeorge syndrome, and microsignificant effect. The actuarial survival rates were mea- cephaly. He underwent repair at 15 days of age and was sured using the Kaplan-Meier survival function esti- dischargedhome with a residual arch gradient of 18 mm mates: The 70% confidence limit (CL) for the mean is Hg and mild subaortic stenosis. This patient died 4 months later due to aspirationpneumonia and sepsis.The reported where appropriate. secondlate death occurred in a child with Shone’s complex with coarctation and ventricular septal defect who RESULTS The study group consisted of 60 neonates between underwent repair at 4 days of age, without resection of the age of 1 and 28 days (mean 9.9 + 0.9, median 8). the infundibular septum. This patient developed severe These children ranged in weight from 1.3 to 4.69 kg subaortic stenosisand required a left ventricular apex to (mean 3 + 0.1). Nineteen (32%) had coarctation with descending aorta conduit 3 months later. Aortic valve ventricular septal defect, 18 (30%) had interrupted aor- replacementwith aortic root enlargement was later pertic arch with ventricular septal defect, and 23 (38%) had formed at 4 years of age, but the patient died from vencoarctation or interrupted aortic arch with complex intra- tricular fibrillation 2 days after the operation9 The Kaplan-Meier technique was used to evaluate cardiac anatomy (Table I). Interrupted aortic arch type A was present in 11patients, and type B in 14.Fourteen overall patient survival. The actuarial survival rate for patients had posterior deviation of the infundibular sep- all patients at 1,3,48,52, and 78 months was 8X%,86%, tum. Other associated defects are listed in Table II. 86%, 72%, and 72%, respectively. There was no statisExtracardiac defects included DiGeorge syndrome in 5 tically significant difference in survival among the 3 subpatients, Noonan’s syndrome in 1, XY chromosome in groups (p = 0.3801) using the actuarial technique, ala female child, and mixed karyotype 46 XY, 45X in though the early and late mortality rates listed in Table I suggesta greater mortality in the complex group. another child. CONGENITAL
HEART DISEASE/ARCH
REPAIR IN NEONATES
371
Early mortality was significantly increasedin patients weighing <3 kg. Sixteen patients weighed <3 kg, and there were 5 deaths in this group. In contrast, there were only 4 early deathsamong the 44 children who weighed >3 kg (p = 0.03, by chi-square analysis). Recurrent arch obslruction: Recurrent arch obstruction, defined as resting systolic pressure gradient 220 mm Hg, occurred in 2 of the 53 hospital survivors (3.8%; 70% CL 1.9% to 7.5%). Both patients had coarctation with ventricular septal defect and underwent repair by resection and end-to-end anastomosisat age 9 and 23 days, respectively. At the time of discharge from the hospital after repair, the gradients were 0 and 18 mm Hg, respectively. At follow-up of 2.5 and 5 months after operation, gradients of 30 and 70 mm Hg, respectively, were measured. These 2 patienls underwent successful balloon angioplasty, resulting in a decreasein residual gradient to 0 and 8 mm Hg, respectively. Both patients continued to have evidence of recoarctation 7 and 17months, repectively, after balloon angioplasty. In addition to these 2 patients, 9 other patients had a residual systolic gradient 120 mm Hg (range 10 to 18, mean 11.6+ 0.9) and continue to be hemodynamically stable at follow-up. Forty-two patients (79%) had no residual arch gradient. Left ventricular ouffbw obstruction: Left ventricular outflow tract obstruction was responsible for 1 early and 3 late interventions. One patient with double-outlet right ventricle, posterior deviation of the infundibular septum, and a hypoplastic left ventricle did not have subaortic resection and did poorly. This patient underwent reoperation for resection of the left ventricular outflow tract obstruction, takedown of the atrial and ventricular patches, and pulmonary artery band placement, but did not survive. In retrospect,the left ventricle was too small for a biventricular repair. Two patients developed significant subaortic stenosis which required late surgical intervention. In 1, subaortic stenosis was not present at initial operation. This patient required aortic valvotomy and resection of subaortic stenosis 3 years later. The second patient had valvar and subvalvar (tunnel type) aortic stenosis and required a left ventricular apex to descending aorta conduit 3 months after surgery and an aortic valve replacement with enlargement of the left venbicular outflow tract (Konno procedure) at age 4 years. He died 48 hours after operation from ventricular fibrillation.9 Another patient with valvar aortic stenosis underwent successful aortic balloon valvuloplasty 13 months after operation and is doing well. Three of 7 patients who had resection of the posteriorly deviated infundibular septum at the initial repair died after operation (2 early, and the other 4 months later from aspiration pneumonia). The remaining 4 infants had no residual subaortic gradients at follow-up 10to 24 months (median 18) after operation. Additional sequelae:One infant with truncus arteriosus and interrupted aortic arch developed left mainstem bronchial compressionat 3.5 months of age and required placement of an aortic interposition graft for relief of symptoms. Six patients have trivial ventricular level shunts that are hemodynamically insignificant. Two patients with truncus arteriosus have 25 and 36 mm Hg gradients, respectively,acrossthe right ventricular to pul-
This experience indicates that a single-stageanterior approach for repair of aortic arch obstruction and intracardiac defects can be accomplished with low mortality in the neonatedespite the complexity of these anomalies and the poor preoperativecondition of many of thesechildren. The overall mortality in this cohort was 15%, with an early mortality of 11.6%.In neonateswith an associated complex intracardiac defect, mortality was 17.4%; in neonateswith an interrupted aortic arch and ventricular scptal defect it was 11.1%;and in neonateswith coarctation and ventricular septal defect, it was only 5%. The mortality rate in the group with complex intracardiac anatomy is relatively high but compares favorably with the mortality reported by other groups utilizing a staged repair.6,7,‘(b’4Vouche et al6 reported their experience using a stagedapproachin patients with complex intracardiac defects: early mortality 36%, late mortality 44%. Van Heum et al’ reported 35 patients with coarctation and complex intracardiac defectswho underwent stagedrepair, with 11of 35 early deaths(31%), and 6 late deathsat the time of definitive repair (overall mortality 48%). It was their impression that even small residual coarctation gradients after operation (40 mm Hg) resulted in increasedmortality when associatedwith concurrent pulmonary artery banding. They concluded that biventricular outflow obstruction resulted in increased mortality, and that a single-stagerepair should be undertaken when appropriate. Previous reports of the single-stage approach in the repair have been cncourging. Karl et al’” reported 55 patients with arch obstruction, of which 45 underwent bivcntricular single-stage repair with an operative mortality of 9% (CL 5% to 15%).Turley et al” reported their results in 29 patients with interrupted aortic arch with ventricular septal defect using a single-stage approach and concluded that single-stagerepair could be achieved with lower mortality than palliation. In their series the operative mortality rate was 20%. Thus, survival after single-stage repair of these complex lesions compares favorably with that of the staged approach, suggesting that single-stage repair may be the preferable surgical strategy. Recoarctation (defined as a gradient 220 mm Ilg) occurred in only 2 of the 53 survivors in our series, and was successfully treated with balloon angioplasty. Both infants with recoarctation had a ventricular septal defect and a narrow transverse arch and underwent operation early in this series without extension of the distal aorta into the hypoplastic segment of transverse aorta. Routine use of an extended primary anastomosishas virtually eliminated residual coarctation in more recent patients. No child with interrupted aortic arch or coarctation with complex intracardiac anatomy has developed recurrent arch obstruction. Previous reports have described postoperative recoarctation in 9% to 25% of infants and in 15% to 25% of neonates.15-I7Van Heum et al’ compared actuarial freedom from recoarctation in 151children with subclavian flap, end-to-end anastomo-
372
FEB. 15, 1995
THE AMERICAN JOURNAL OF CARDIOLOGY@’
VOL. 7.5
monary artery conduits. No patient has developed an aneurysm of the aortic arch, paraplegia, or heart block. DISCUSSION
sis, or extended and radical end-to-end anastomosisrepairs. It was their impression that prognosis was best in patients with extended and radical end-to-end anastomosis.7In our series,the incidence of recoarctation was also low (3.7%), but the follow-up period was only 23 + 3 months; longer term data are needed to assessthe true incidence of recoarctation in these patients. A single-stage approach to the neonate with aortic arch obstruction and intracardiac defects has several advantagesover a stagedapproach.First, it provides the child with early and complete physiologic repair. Second, it sparesmost children the need for a second operation. Finally, the anterior approach provides improved surgical exposure, which may explain the low incidence of recurrent arch obstruction comparedwith other series. However, a single-stage approach may also have disadvantages. It requires more complex surgery during the neonatal period, including cardiopulmonary bypass and hypothermic arrest. In this series, the total period of hypothermic arrest was 48 minutes, and none of our patients had evidence of central nervous system injury.
1. &mini L, Gargiulo G, Albanese SB, Santorelli MC, Picchio FM, Pierangeli A. Aortic coarctation with hypoplastic arch in neonates: a spectrum of anatomic lesions requiring different surgical options. Ann Thorac Surg 1993;56:288-294. 2. Hesslein PS, McNamx~a DG, Morriss MJH, Hallman CL, Cooley DA. Comparison of resection versus patch aortoplasty for repair of coarctation in infants and children. Circulation 1981;64:16&168. 3. Be&man RH, Rocchini AP, Behrendt DM, Bove EL, Dick M, Crowley DC, Snider RA, Rosenthal A. Long-term outcome after repair of coarctation in infancy: subclavian angioplasty does not reduce the need for reoperation. J Am Coil Car-
dial 1986;8:140&1411. 4. Irwin ED, Braunlin EA, Faker JE. Staged repair of interrupted aotic arch and ventricular septal defect in infancy. Ann Thorac Surg 1991;52:632-639. 5. Hammon JW, Graham TP, Boucek RJ, Bender HW. Operative repair of coarctation of the aorta in infancy: results with and without ventricular septal defect. Am J Car&l 1985;55:1555-1559. 6. Vouche PR, Trinquet F, Lecompte Y, Vemant F, Roux PM, Tauati G, Pome G, Leca F, Neveux .N. Aortic coarctation with hypoplastic aortic arch-results of extended end-to-end aortic arch anastomosis. J Thorac Cardiovasc Surg 1988;96: 557-563. 7. van Heum LWE, Wang CM: Spiegelhalter DJ, Sorensen K, de Lewd MR, Stark J, Elliot MJ. Surgical treatment of aortic coarctation in infants younger than three months: 1985.1990. J Thorac Cardiovasc Surg 1994;107:74-86. 8. Bove EL, Minich L, Prijdan AK, Lupinetti FL, Snider RS, Dick M, Be&man RH. The management of severe subaortic stenosis, ventricular septal defect, and aottic arch obshuction in the neonate. J Thor-m Cardiovasc Surg 1993; 105:2X9-296. 9. Scott WA, Rocchini AP, Bow EL, Behrendt DM, Beckman RH, Dick M, Serwer G, Snider R, Rosenthal A. Repair of interrupted aortic arch in infancy. J Thorat Cardiovasc Surg 1988;96:56&568. 10. DietJ CA, Torres AR, Favaloro RG, Fessler CL, Gmnkemeier GL. Risk of recoarctation in neonates and infants after repair with patch aortoplasty, sub&viz& flap, and the combined resection-flap procedure. J Thorac Cardiovasc Surg 1992; 103:724-732. 11. Turley K, Yee ES, Ebert PA. The total repair of interrupted aortic arch complex in infants: the anterior approach. Circ&fion 1984;7O(suppl I)%lM-20. 12. Planche C, Serraf A, Comas JV, Lacour-Gayet F, Bmniaux .I, Touchot A, Kirklin JW. Anatomic repair of transposition of great arteries with ventricular septal defect and aortic arch obstmction. J ThorF Cardiovasc Surg 1993;105:925-933. 13. Karl TR, &no S, Brawn W, Mee RBB. Repair of hypoplastic or interrupted aortic arch via sternotomy. J Thorac Cardiovasc Surg 1992;104:688-695. 14. Sell JE, Jonas RA, Mayer JE, Blackstone EH, Kirklin JW, Castaneda AR. The results of a surgical program for interrupted aortic arch. J Thorac Cardiovasc Surg 1988;96:864-877. 15. Kirklin JW, Barratt-Boyes BG. Coarctation of the aorta and aortic arch intaoption. In: Kirklin JW, Barr&t-Boyes BG, eds. Cardiac Surgery. 1st ed. New York: Churchill-Livingstone, 1988:1035-1080. 16. Ziemer G, Jonas RA, Perry SB, Freed MD, &tan& AR. Surgery for coarctation of the aorta in the neonate. Circularion 1986;74(suppl 1):1-25-I-31. 17. Korfer R, Meyer H, Kleikamp G, Bircks W. Early and late results after resection and end-to-end anastomosis of coaxtation of the thoracic aorta in early infancy. J Thorac Cardiovasc Sur-g 1986;89:6164i22.
CONGENITAL
HEART DISEASE/ARCH REPAIRIN NEONATES
373