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J Thorac Cardiovasc Surg 1998;116:417-431
© 1998 Mosby, Inc.

Surgery for Congenital Heart Disease

INTERMEDIATE SURVIVAL IN NEONATES WITH AORTIC ATRESIA: AMULTI-INSTITUTIONAL STUDY

From the Division of Cardiothoracic Surgery, Department of Surgery,Deborah Heart and Lung Center, Browns Mills, NJ, The Department of Surgery,University of Alabama at Birmingham, The Division of Cardiac Surgery, Departmentof Surgery, Loma Linda University, Loma Linda, and the Congenital Heart SurgeonsSociety.

Presented at the Seventieth Scientific Sessions, American HeartAssociation, Orlando, Fla, Nov 11, 1997.

Received for publication May 14, 1998; revisions requested May 19,1998; revisions received May 26, 1998; accepted for publication May 26, 1998. Address for reprints: Marshall L. Jacobs, MD, Deborah Heart and LungCenter, 200 Trenton Rd, Browns Mills, NJ 08015

	Abstract

Top Abstract Introduction Patients and methods Results Discussion Appendix A Appendix B1. Aortic valve... Appendix B2. Aortic valve... Appendix B3. Aortic valve... Appendix B4. Aortic valve... References

 
Objective: Controversy persists withregard to the treatment of patients with aortic atresia. Staged reconstructiveoperations and primary transplantation have been advocated as treatmentstrategies, but in many instances no treatment is undertaken. Amulti-institutional study was undertaken for the purpose of characterizing thischallenging patient group, comparing the prevalence and outcomes of the varioustreatment strategies, and identifying potential predictors of success or failurewith each. Methods and results: A total of 323neonates with aortic atresia were entered into a 21-institution prospective,nonrandomized study between January 1, 1994, and January 1, 1997. Threeprotocols were used, nonexclusively in many institutions: (1) stagedreconstructive surgery with initial palliation by a Norwood procedure andeventual Fontan operation, (2) heart transplantation as initial definitivetherapy, and (3) nonsurgical management. Analysis was based on initial protocolassignment: staged reconstructive surgery in 253 patients, heart transplantationin 49 patients, and nonsurgical management in 21 patients. For all patientsinitially entered into the 2 surgical treatment protocols, survival at 1, 3, 12,24, and 36 months after entry was 67%, 59%, 52%, 51%,and 50%, respectively. A multivariable analysis found incremental riskfactors for death at any time after entry to be lower birth weight (P = .04), associated noncardiac anomaly (P = .007), and entry into the nonsurgical protocol (P < .0001) or the staged reconstructive surgeryprotocol (P = .03). Four institutions hadhigher survival statistics; 2 used a heart transplantation protocol and 2 used astaged reconstructive surgery protocol. For the 113 patients treated at these 4institutions, survival at 1, 3, 12, 24, and 36 months after entry was 77%,70%, 64%, 62%, and 61%, respectively. Survival amongthe 4 institutions was similar (P = 0.1).Conclusions: Among patients with aortic atresia,other features of cardiac structure including aortic size, degree of leftventricular hypoplasia, and degree of mitral hypoplasia or atresia are notpredictive of survival from 2 surgical protocols. The highest survival wasachieved with either treatment strategy at institutions strongly committed tothe use of one or the other surgical management protocol. (J Thorac CardiovascSurg 1998;116:417-31)

	Introduction

Top Abstract Introduction Patients and methods Results Discussion Appendix A Appendix B1. Aortic valve... Appendix B2. Aortic valve... Appendix B3. Aortic valve... Appendix B4. Aortic valve... References

 
Aortic valve atresia is the anatomic hallmark of the largest group ofneonates born with a cardiac malformation with single ventricle physiology. Ofall babies considered to have hypoplastic left heart syndrome, two thirds ormore have aortic valve atresia. ^1,2 In the absence of specifictherapy, physiologic constriction of the ductus arteriosus after birth leadsinvariably to death from systemic hypoperfusion, coronary insufficiency, orcongestive heart failure. Treatment strategies have evolved on the basis ofeither reconstructive surgery, along the lines developed by Norwood and others, ^3,4or cardiac replacement, as pioneered by Bailey and others. ^5,6There have been numerous reports of encouraging and improving outcomes with oneor the other treatment strategy from several institutions. ^7,9Nonetheless, the relative efficacy of these 2 treatment strategies remains amatter of speculation, and in many instances babies with aortic atresia areallowed to die of the predictable natural history of the disease withouttherapeutic intervention.

A study of outcomes in a large group of neonates with aortic atresiacould be of value to perinatologists, neonatologists, pediatric cardiologistsand surgeons, and others involved in the support and management of families withfetuses and neonates with aortic atresia. A multi-institutional study was begunto provide such information. Analysis is centered around the intention to treat(or not treat) with a protocol based on (1) staged reconstructive surgery(Norwood procedure followed ultimately by Fontan-type reconstruction), (2) hearttransplantation as initial definitive therapy, or (3) no surgical treatment.Among the goals of the study were to define the spectrum of cardiac morphologyassociated with aortic valve atresia, to define the spectrum of surgicalapproaches currently used and their respective outcomes, and to undertakeanalyses leading to inferences concerning the impact of patient characteristicsand treatment strategies on outcome. In this initial report of the CongenitalHeart Surgeons Society Multi-Institutional Study, analysis is strictly limitedto those patients with aortic valve atresia (a database has been simultaneouslygenerated for patients with aortic valve stenosis and associated left heartobstructive lesions), and consideration of outcomes is limited to determinationof intermediate-term survival.

	Patients and methods

Top Abstract Introduction Patients and methods Results Discussion Appendix A Appendix B1. Aortic valve... Appendix B2. Aortic valve... Appendix B3. Aortic valve... Appendix B4. Aortic valve... References

 
Patients
Between January 1, 1994, and January 1, 1997, 323 neonates (age <30 days) with aortic valve atresia were entered into a prospective study in 21institutions (Appendix A ). Two thirds of the patientswere aged 2 days or less at admission and 90% were aged 7 days or less.The diagnosis of aortic valve atresia was made in all instances by 2-dimensionalechocardiography.

Follow-up
The physician, family, or guardian of each patient not known to be deadwas contacted each year since the beginning of the study. The most recentfollow-up was conducted from January through March 1997. At that time, 165patients were known to be dead. During the 1997 follow-up, 149 of the 158patients not known to be dead were successfully traced. Seven additionalpatients have follow-up through 1996. Only 2 patients not known to be dead haveno follow-up since the time of hospital discharge. Median follow-up time forsurvivors is 20 months, range 1.1 to 38 months; mean follow-up time forsurvivors is 21 ± 9.6 months (standard deviation).

Morphology
Estimates of left ventricular size and of the structure and patency ofthe mitral valve were obtained from echocardiographic reports from theparticipating institutions. Information regarding associated cardiac anomaliesand size of the ascending aorta were obtained from echocardiographic andoperative reports.

Protocols
Each patient was entered into 1 of 3 protocols based on initial intentionto treat at the participating institution. Thus all patients listed for hearttransplantation were considered to have entered the heart transplantationprotocol. All patients managed with intent to perform an initial palliativereconstructive operation were considered to have entered the stagedreconstructive surgery protocol. Patients who entered into neither surgicaltreatment protocol were considered to have entered a protocol of nonsurgicalmanagement. Analysis was undertaken in relation to intention to treat (initialprotocol assignment). Inasmuch as there were a few instances of crossover (fromnonsurgical management to either of the treatment protocols or from one surgicaltreatment protocol to the other), analysis was subsequently undertaken withrespect to the event of either Norwood procedure or heart transplant operation.

Data collection and analyses
Copies of all hospital documents were sent to the Data and AnalysisCenter at the University of Alabama at Birmingham. The data were abstracted intocomputer files, and these and the copies of the hospital documents were retainedin confidential storage. Numerous tabulations, contingency tables, and lifetable analyses (Kaplan-Meier method) were made. Time-related freedom from deathor other outcome events, and the hazard function, were also computedparametrically. Numerous multivariable analyses were made in the time-relatedhazard function regression domain ¹⁰;other regression models were used when indicated. All continuous variables wereexamined as continuous variables (not polytomized). Interaction terms weresought in all multivariable analyses: in equations of this paper, these may bethought of as terms (variables) that are active or inactive only in certainspecified relations with other variables. Variables with a Pvalue < .1 were retained in the final equation. Variables used in themultivariable analyses and incremental risk factors for death at any time afterentry are listed in Appendix B.

To estimate the proportion of neonates entering each of the two surgicalprotocols that will achieve definitive repair, a Competing Risks of EventsAnalysis was performed. ¹¹The mutually exclusive definitive states after entry into the stagedreconstructive surgery protocol were as follows: Fontan operation, 2-ventriclerepair, crossover to heart transplantation, and death before definitive repair.Those for the heart transplantation protocol were heart transplantation,crossover to a Norwood operation, and death while awaiting transplantation.

	Results

Top Abstract Introduction Patients and methods Results Discussion Appendix A Appendix B1. Aortic valve... Appendix B2. Aortic valve... Appendix B3. Aortic valve... Appendix B4. Aortic valve... References

 
Morphology
Of the 323 patients with aortic valve atresia, 309 patients (96%)had severe left ventricular hypoplasia. Of these, 175 patients had mitralatresia, 117 had a patent but stenotic or hypoplastic mitral valve, and 17patients had unknown mitral valve morphology. Six patients (2%) had amoderate degree of left ventricular hypoplasia, and 8 patients (2.5%) hada normal-sized left ventricle. Of these 8 patients, all but 1 had anonrestrictive ventricular septal defect. In total, there were 13 patients (4%)with ventricular septal defects of various sizes. The spectrum of cardiacanomalies associated with aortic valve atresia is shown in Table I andassociated noncardiac anomalies in Table II.

Survival
For all 323 patients, survival at 1, 3, 12, 24, and 36 months after entrywas 63%, 55%, 50%, 48%, and 47%, respectively(Fig. 1, A).

View larger version (22K): [in this window] [in a new window]   Fig. 1.Non-risk-adjusted survival and hazard function for death for all 323 patients inthe study. A, Survival after entry (at timezero). Each circle represents an actual death,positioned at the time of death along the horizonal axis and actuarially alongthe vertical axis. The vertical bars depict ± 1 standard error. Thenumbers indicate the number of patientsremaining at risk at the time of the estimate. The solidline is the parametric estimate of survival, and thedashed lines enclose the 70% confidenceintervals. B, Hazard function for death.

View larger version (34K): [in this window] [in a new window]   Fig. 2.Schematic diagram of the treatment pathway and fate of the 323patients after initial protocol assignment. SVC-PA,Superior vena cava–pulmonary artery.

View larger version (26K): [in this window] [in a new window]   Fig. 3. Non-risk-adjusted survival forpatients treated at the 4 low-risk institutions.

View larger version (24K): [in this window] [in a new window]   Fig. 4.Non-risk-adjusted survival and hazard function for patients (n = 253) initially entered in the stagedreconstructive surgery protocol. A, Survivalafter entry (at time zero). B, Hazard functionfor death.

Transplantation protocol
The 49 patients entered into a heart transplantation protocol weredistributed among 6 different institutions, with 3 institutions accounting for45 (92%) of the patients. Time-related survival among the 49 patientsentered into a heart transplantation protocol is depicted in Fig. 6,A.

View larger version (31K): [in this window] [in a new window]   Fig. 5.Competing Risks Analysis for patients in the staged reconstructivesurgery protocol (n = 253). The number ofpatients (expressed as a percent of total) in each of 5 categories across thetime of follow-up is depicted. All patients begin alive, awaiting definitiverepair. Thereafter, patients migrate to death, the Fontan operation, hearttransplantation, and 2-ventricle repair according to the respective hazardfunctions operating on those remaining alive, awaiting definitive repair. Atevery point in time, the number of patients in all categories adds to 100%(so-called conservation of patients). Symbolsrepresent each event, positioned on the vertical axis by multiple decrementanalysis. The solid curves representmathematical estimates of the accumulative effect of the 4 hazard functionsoperating on the original group of patients.

View larger version (24K): [in this window] [in a new window]   Fig. 6.Non-risk-adjusted survival and hazard function for death for patients(n = 49) initially entered in the hearttransplant protocol. A, Survival after entry (attime zero). B, Hazard function for death.

That the altered natural history of patients receiving supportive care inanticipation of eventual transplantation is markedly different from the naturalhistory of the disease itself is illustrated in Fig. 8, depictingnon-risk-adjusted survival of patients awaiting transplantation and of those inthe nonsurgical treatment protocol.

View larger version (31K): [in this window] [in a new window]   Fig. 7.Competing Risks Analysis for patients (n =49) initially entered into the heart transplant protocol. The number of patients(expressed as a percent of total) in each of 4 categories across the time offollow-up is depicted. All patients begin alive, awaiting transplantation.Thereafter, patients migrate to heart transplantation, death awaitingtransplantation, and Norwood operations according to the respective hazardfunction. The depiction is otherwise as in Fig. 5.

View larger version (27K): [in this window] [in a new window]   Fig. 8.Non-risk-adjusted survival for patients awaiting transplantation inthe heart transplantation protocol, and for patients in the no surgicaltreatment protocol.

View larger version (35K): [in this window] [in a new window]   Fig. 9.Actuarial (life-table) survival of patients stratified according to the 3treatment protocols. Each symbol represents anactual death, positioned at the time of death along the horizontal axis andactuarially along the vertical axis. The vertical barsdepict ± 1 standard error. The numbersindicate the number of patients remaining at risk at the time of estimate. Thesolid lines are the parametric estimates ofsurvival, and the dashed lines enclose the 70%confidence intervals.

View larger version (36K): [in this window] [in a new window]   Fig.10.A, Non-risk-adjusted survival of patientsentered into treatment protocols at the 4 low-risk institutions. The 70%confidence intervals converge at a point approximately 1 week after entry andcontinue to overlap thereafter. B, Survival ofpatients initially entering either surgical treatment protocol at the 4 low-riskinstitutions is shown, together with results at the remaining institutions.Patients for whom the nonsurgical protocol was initially designated are excludedfrom this analysis.

	Discussion

Top Abstract Introduction Patients and methods Results Discussion Appendix A Appendix B1. Aortic valve... Appendix B2. Aortic valve... Appendix B3. Aortic valve... Appendix B4. Aortic valve... References

Study design did not enable us to ascertain whether there are anatomic,morphologic, or other patient characteristics which for this specific cardiacanomaly would mitigate for a higher likelihood of survival with one or the otherof the surgical treatment strategies. It has been speculated, for example, thatthis may be true with regard to either severe tricuspid valve incompetence orpulmonary venous obstruction resulting from intact atrial septum or anomalies ofpulmonary venous return. Although these last 2 were among the associated cardiacanomalies included in the multivariable analyses, their relatively lowprevalence together with the overall mortality of approximately 50% mayhave led to an underestimate of their potential importance.

The study is also limited by our current inability to identify andanalyze any late-phase events associated with either of the treatmentstrategies. Certainly a consideration of the late-phase events after creation ofthe Fontan-type circulation (eg, ventricular dysfunction, arrhythmias,protein-losing enteropathy) and those after transplantation (eg, late rejection,lymphoproliferative disease, graft atherosclerosis) is essential to the overallcomparison of surgical treatment strategies for aortic atresia. Additionally,the majority of patients enrolled in the reconstructive surgical protocol haveyet to undergo a definitive repair (Fontan operation), although to datemortality for the second- and third-stage procedures has been low. It should bepossible, after a longer period of follow-up of this patient cohort, to knowmore about the relative efficacy of treatment strategies.

Inferences as to therapy
For neonates with aortic atresia, supportive medical therapy (as appliedin anticipation of transplantation as initial and definitive therapy) results ina drastic alteration of the adverse natural history associated with the lesionitself. As such, it is possible that the application of the same principles ofsupportive therapy to all patients in anticipation of either transplantation orpalliative reconstructive surgery might favorably affect overall survival.Although the interval between entry and procedure was among those factorsanalyzed in the multivariable analyses and was not found to be predictive ofsurvival, 75% of the patients who underwent a Norwood operation did so by8 days of age, whereas the median age for heart transplantation was 1.9 months.The experience of the transplant centers is supportive of the inference that themajority of patients can be successfully stabilized and supported for a periodof time, obviating the necessity for early emergency surgery in most instances.It would be of interest to explore the hypothesis that an additional period ofmedical stabilization and supportive therapy might favorably affect the outcomeof initial palliative reconstructive surgery.

It was not uniformly the case that every high-volume center was among thelow-risk institutions. At the same time, the nearly exclusive use of one or theother of the 2 surgical treatment protocols at each of the 4 low-riskinstitutions suggests that a strong institutional commitment to a particulartherapeutic strategy is a principal ingredient of intermediate-term success. Atthe present time, survival of neonates with aortic atresia managed by either ofthe 2 surgical treatment protocols at a low-risk institution is slightly greaterthan 60% at 2 years. This is not importantly lower than the likelihood ofsurvival for patients with some of the other challenging congenital heartmalformations that require initial surgical interventions during the newbornperiod. ^17,18 This study was intentionallylimited to an examination of survival after initial neonatal management ofaortic atresia. Additional follow-up and further analyses of this relativelylarge patient group may further enhance our ability to optimize the medical andsurgical management of patients with this challenging cardiac malformation.

	Appendix A

Top Abstract Introduction Patients and methods Results Discussion Appendix A Appendix B1. Aortic valve... Appendix B2. Aortic valve... Appendix B3. Aortic valve... Appendix B4. Aortic valve... References

 
The 21 institutions, in randomly determined order, participating in this study of the Congenital Heart Surgeons Society.

Mott Children's Hospital, Ann Arbor, MI; The University of Alabama at Birmingham, Birmingham, AL; The Children's Hospital, Boston, MA; The Children's Hospital of Buffalo, Buffalo, NY; Children's Memorial Hospital, Chicago, IL; The University of Chicago, Chicago, IL; Children's Hospital Medical Center, Cincinnati, OH; The Children's Hospital, Denver, CO; The Children's Hospital of Michigan, Detroit, MI; Duke University Medical Center, Durham, NC; The Milton S. Hershey Medical Center, Hershey, PA; Loma Linda University Medical Center, Loma Linda, CA; The Children's Hospital of Los Angeles, Los Angeles, CA; Jackson Memorial Hospital, Miami, FL; Columbia-Presbyterian Medical Center, New York, NY; The Children's Memorial Hospital, Omaha, NB; The Children's Hospital of Philadelphia, Philadelphia, PA; University of Pittsburgh Children's Hospital, Pittsburgh, PA; The Mayo Clinic, Rochester, MN; The Hospital for Sick Children, Toronto, ON, Canada; Georgetown University Medical Center, Washington, DC.

	Appendix B1. Aortic valve atresia (CHSS; 1994-1997; n=323): Variables used in the multivariable analyses

Top Abstract Introduction Patients and methods Results Discussion Appendix A Appendix B1. Aortic valve... Appendix B2. Aortic valve... Appendix B3. Aortic valve... Appendix B4. Aortic valve... References

 

	Appendix B2. Aortic valve atresia (CHSS; 1994-1997; n=323): Incremental risk factors for death at any time after entry, entering patient-specific variables only

Top Abstract Introduction Patients and methods Results Discussion Appendix A Appendix B1. Aortic valve... Appendix B2. Aortic valve... Appendix B3. Aortic valve... Appendix B4. Aortic valve... References

	Appendix B3. Aortic valve atresia (CHSS; 1994-1997; n=323): Incremental risk factors for death at any time after entry, entering protocol factors only (above) and patient and protocol factors (below)

Top Abstract Introduction Patients and methods Results Discussion Appendix A Appendix B1. Aortic valve... Appendix B2. Aortic valve... Appendix B3. Aortic valve... Appendix B4. Aortic valve... References

	Appendix B4. Aortic valve atresia (CHSS; 1994-1997; n=323): Incremental risk factors for death at any time after entry, entering patient-related factors, protocol factors, and experience factors

Top Abstract Introduction Patients and methods Results Discussion Appendix A Appendix B1. Aortic valve... Appendix B2. Aortic valve... Appendix B3. Aortic valve... Appendix B4. Aortic valve... References

	References

Top Abstract Introduction Patients and methods Results Discussion Appendix A Appendix B1. Aortic valve... Appendix B2. Aortic valve... Appendix B3. Aortic valve... Appendix B4. Aortic valve... References

1. Murdison KA, Baffa JM, Farrell PE, Chang AC,Barber G, Norwood WI, et al. Hypoplastic left heart syndrome: outcome afterinitial reconstruction and before modified Fontan procedure. Circulation 1990;82:199-207.
   
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3. Norwood WI, Lang P, Hansen DD. Physiologicrepair of aortic atresia: hypoplastic left heart syndrome. N Engl J Med 1983;308:23-6.[Medline]
   
4. Doty DB, Knott HW. Hypoplastic left heartsyndrome: experience with an operation to establish functionally normalcirculation. J Thorac Cardiovasc Surg 1977;75:624-30.
   
5. Bailey LL, Nelsen-Cannarella SL, DoroshowRW, Jacobson JG, Mastin RD, Allard MW, et al. Cardiac allotransplantation innewborns as therapy for hypoplastic left heart syndrome. N Engl J Med 1986;315:949-51.[Medline]
   
6. Chiavarelli M, Gundry SR, Razzouk AJ, BaileyLL. Cardiac transplantation for infants with hypoplastic left heart syndrome.JAMA 1993;270:2944-7.[Abstract/Free Full Text]
   
7. Norwood WI, Jacobs ML, Murphy JD. Fontanprocedure for hypoplastic left heart syndrome. Ann Thorac Surg 1992;54:1025-30.[Abstract]
   
8. Iannettoni MD, Bove EL, Mosca RS, LupinettiFM, Dorostkar PC, Ludomirsky A, et al. Improving results with first-stagepalliation for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 1994;107:934-40.
   
9. Bailey LL, Gundry SR, Razzouk AJ, Wang N,Sciolaro CM, Chiavarelli M. Bless the babies: one hundred fifteen late survivorsof heart transplantation during the first year of life. J Thorac CardiovascSurg 1993;105:805-15.[Abstract]
   
10. Blackstone EH, Naftel DC, Turner ME. Thedecomposition of time-varying hazard into phases, each incorporating a separatestream of concomitant information. J Am Stat Assoc 1986;81:615-24.
    
11. David HA, Moeschberger ML. The theory ofcompeting risks. New York: Macmillian; 1978. p. 45-56.
    
12. Forbess JM, Cook N. Roth SJ, Serraf A,Mayer JE, Jonas RA. Ten-year institutional experience with palliative surgeryfor hypoplastic left heart syndrome. Circulation 1995;92:(Suppl):II262-6.
    
13. Pigott JD, Murphy JD, Barber G, Norwood WI.Palliative reconstructive surgery for hypoplastic left heart syndrome. AnnThorac Surg 1998;45:122-8.[Abstract]
    
14. Mosca RS, Bove EL, Crowley DC, Sandhu SK,Schork MA, Kulik TJ. Hemodynamic characteristics of neonates followingfirst stage palliation of hypoplastic left heart syndrome. Circulation1995;92:(Suppl)II267-71.
    
15. Kern JH, Hayes CJ, Michler RE, Gersony WM,Quaegebeur JM. Survival and risk factor analysis for the Norwood procedure forhypoplastic left heart syndrome. Am J Cardiol 1997;80:170-4.[Medline]
    
16. Bailey LL, Conception W, Shattuck H, HuangL. Method of heart transplantation for hypoplastic left heart syndrome. J ThoracCardiovasc Surg 1986;92:105.[Abstract]
    
17. Jonas RA, Quaegebeur JM, Kirklin JW,Blackstone EH, Daicoff G. Outcomes in patients with interrupted aortic arch andventricular septal defect. J Thorac Cardiovasc Surg 1994;107:1099-113.
    
18. Hanley FL, Sade RM, Blackstone EH, KirklinJW, FreedomRM, Nanda NC. Outcomes in neonatal pulmonary atresia with intactventricular septum. J Thorac Cardiovasc Surg 1993;105:406-27.[Abstract]
    

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