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Early Survival of Infants Weighing 2.5 Kilograms or Less Undergoing First-Stage Reconstruction for Hypoplastic Left Heart Syndrome Samuel Weinstein, MD; J. William Gaynor, MD; Nancy D. Bridges, MD; Gil Wernovsky, MD; Lisa M. Montenegro, MD; Rodolfo I. Godinez, MD, PhD; Thomas L. Spray, MD Downloaded from http://circ.ahajournals.org/ by guest on June 14, 2017 Background—Results of staged palliation for hypoplastic left heart syndrome (HLHS) have improved in recent years; however, certain risk factors have been associated with decreased survival rates. Methods and Results—We retrospectively reviewed the medical records of 67 patients weighing #2.5 kg undergoing the first stage of reconstructive surgery at our institution between January 1, 1990, and December 31, 1997. HLHS was present in 45 patients, complex double-outlet right ventricle in 10, unbalanced AV canal in 5, tricuspid atresia with transposition of the great vessels in 4, and other diagnoses in 3. Mean age at surgery was 10.1610.7 days (median, 8 days), and mean weight was 2.260.3 kg (median, 2.2 kg). Fourteen patients weighed #2.0 kg, and 2 patients weighed #1.5 kg. Early mortality (death within 30 days or before hospital discharge) was 51% (34 of 67). No patient, procedural, or time-related variables correlated with increased mortality. However, there was a trend toward increased mortality with increased cardiopulmonary bypass time (P50.076) and decreased preoperative ventricular performance (P50.139). Conclusions—These findings suggest that low weight alone in a patient with HLHS or an anatomic variant should not be considered a contraindication to staged reconstructive surgery. (Circulation. 1999;100[suppl II]:II-167–II-170.) Key Words: heart defects, congenital n surgery n risk factors E arly survival after the first stage of palliative surgical reconstruction for hypoplastic left heart syndrome (HLHS) has steadily improved since Norwood et al1 and Doty et al2 first described the operation in 1979. In a recent multi-institutional review from the Congenital Heart Surgeons Society (CHSS), 1-month survival for stage 1 palliation for aortic atresia was 64%, with better results achieved at 2 “low-risk” institutions.3 Bove and colleagues4 from the University of Michigan recently reported hospital survival rate of 76% after stage 1 palliation. Results from our own institution are similar, with an overall early survival rate of 73% in .171 cases between January 1, 1995, and December 31, 1998. Early survival for HLHS is significantly less than for other cardiac defects requiring neonatal repair. Anatomic variation, size of the ascending aorta, age at surgery, tricuspid valve function, ventricular function, low birth weight, associated noncardiac anomalies, and other variables have been suggested as possible predictors of mortality for patients undergoing staged reconstruction for HLHS.5–11 Identification of such risk factors would ideally lead to increased survival, either as a result of preoperative management strategies to reduce these “high-risk” characteristics or by leading to referral for cardiac transplantation rather than staged recon- struction to the Fontan operation for a specific subset of patients. There have been only a few recent reports on the outcome of low-weight (,2.5 or 2.0 kg) infants undergoing cardiac surgery.12–15 The overall early survival rate for infants undergoing corrective or palliative surgery for congenital heart defects in these studies was acceptable (80% to 87%) but still lower than for babies of normal weight. These studies included few patients with HLHS. Because of the potential additive risks of low weight and complicated neonatal palliative cardiac surgery, we evaluated the results of first-stage reconstruction in infants weighing #2.5 kg to describe this population and to attempt to identify factors predictive of early mortality. Methods The cardiopulmonary bypass (CPB) records of cardiac surgical cases performed at the Children’s Hospital of Philadelphia between January 1990 and December 1997 were reviewed to identify babies weighing #2.5 kg at the time of stage 1 reconstruction for HLHS or an anatomic variant (see Table 1). Among the 69 patients identified, 67 had complete records and are included in this study. Patient data were compiled by review of clinical records, preoperative echocardiographic reports, perfusion records, operative notes, and autopsy reports. From the Divisions of Pediatric Cardiothoracic Surgery and Pediatric Cardiology, Cardiac Center, Children’s Hospital of Philadelphia, Philadelphia, Penn. Correspondence to Thomas L. Spray, MD, Children’s Hospital of Philadelphia, Division of Cardiothoracic Surgery, 34th & Civic Center Blvd, Suite 8527, Philadelphia, PA 19104-4399. E-mail [email protected] © 1999 American Heart Association, Inc. Circulation is available at http://www.circulationaha.org II-167 II-168 Circulation November 9, 1999 TABLE 1. Characteristics Examined as Potential Predictors of Mortality in Low-Birth-Weight Infants Undergoing Stage 1 Palliation Analyzed as Either Dichotomous or Continuous Variables TABLE 2. Characteristics of 67 Low-Birth-Weight Infants Undergoing Stage 1 Palliation and Probability Values P Sex, F/M Dichotomous variables 32/35 Anatomic diagnosis, n Anatomic subtype HLHS Aortic atresia and/or mitral atresia or mitral stenosis Aortic stenosis and/or mitral atresia or mitral stenosis HLHS variants Complex double-outlet right ventricle Unbalanced AV canal Transposition with tricuspid atresia Critical aortic stenosis Transposition with small left ventricle Echocardiographic assessment of right ventricular performance Downloaded from http://circ.ahajournals.org/ by guest on June 14, 2017 Normal to mildly depressed Moderately to severely depressed Echocardiographic assessment of AV valve function Normal or mildly regurgitant Moderately or severely regurgitant Continuous variables Pulmonary venous drainage HLHS 45 HLHS variants 22 Complex double-outlet right ventricle Unbalanced AV canal 0.7 10 5 Transposition with tricuspid atresia 2 Critical aortic stenosis 2 Transposition with small left ventricle 1 Ventricular performance, n Normal or mildly depressed Moderately or severely depressed 64 0.139 3 AV valve function Normal or mildly regurgitant Moderately or severely regurgitant 63 0.95 4 Pulmonary veins Unobstructed 55 Mildly or moderately obstructed 9 Severely obstructed or intact atrial septum 3 0.289 Gestational age, wk Unobstructed Mild or moderately obstructed Severely obstructed or intact atrial septum Birth weight Mean 3662 Median 36 Range 29–40 0.5 Age at surgery, d Weight at surgery Mean Gestational age at birth Age at time of surgery CPB time 10611 Median 8 Range 1–46 0.5 Shunt size, n Circulatory arrest time Year of surgery Stage 1 reconstruction at our institution includes aortic arch augmentation with cryopreserved homograft, creation of an aortopulmonary shunt, and an atrial septectomy when necessary to ensure adequate interatrial mixing. Early mortality was defined as death within 30 days or before hospital discharge. Prematurity was defined as birth at a gestational age of ,37 weeks. Appropriateness of weight for gestational age was assessed with the Growth Record for Infants (Abbott Laboratories). Time on CPB was calculated as circulatory arrest time plus time on CPB. Characteristics examined as potential predictors of mortality are shown in Table 1. Operations were performed by 4 surgeons, and because the variable “operating surgeon” was essentially collinear with “year of operation,” only the latter variable was analyzed. Logistic regression or x2 was used to analyze the dichotomous variables. The remaining characteristics were analyzed as continuous variables. Results of univariate analysis did not support proceeding to multivariate analysis. All statistical tests were carried out by use of Stata 5.0 software. 3.0 mm 15 3.5 mm 28 4.0 mm 24 CPB time, min Mean 100–19 Range 68–172 Characteristics and anatomic diagnoses of the 67 patients who met study criteria are listed in Table 2. Premature birth occurred in 39 of 67 patients (58%). Weight was appropriate for gestational age in 44 patients (79%), low for gestational 0.5 Circulatory arrest time, min Mean 61611 Range 32–84 0.466 Weight at surgery, kg Mean 2.260.3 Range 1.4–2.5 0.368 Birth weight, kg Mean 2.260.4 Range 1.39–2.6 Year of surgery 1990–1991 1992–1993 Results 0.5 1994–1995 1996–1997 0.285 0.537 Weinstein et al TABLE 3. Causes of Hospital Mortality n Cardiac 23 Noncardiac 11 Abdominal Necrotizing enterocolitis 3 Perforated intestine 2 Pulmonary Aspiration pneumonia 1 Pneumonia 3 Meningitis 2 Downloaded from http://circ.ahajournals.org/ by guest on June 14, 2017 age in 11 (16%), and high for gestational age in only 2. Fourteen patients (21%) weighed #2 kg at the time of surgery. Even in patients in whom surgery was performed after the median age, there was no appreciable weight gain or loss before surgery. Specific genetic syndromes were identified in 5 patients, including 2 infants with Turner’s syndrome and 1 each with Rubinstein-Taybi syndrome, 22Q deletion, and CHARGE syndrome. Early mortality was 51% (34 of 67). Causes of hospital mortality are listed in Table 3. One patient died after discharge but before 30 days of life. Two thirds of the deaths were secondary to cardiac causes, defined as a chart description of cardiopulmonary arrest, arrhythmia, or pulmonary overcirculation preceding death. Infection and sepsis were a factor in all noncardiac deaths. Necrotizing enterocolitis was present before surgery in 6 patients, and 3 survived to hospital discharge. In addition, 2 patients died of perforated viscus (1 colon and 1 appendiceal) in the postoperative period. Pulmonary infections were the primary cause of death in 4 patients. Evidence of meningitis at autopsy was present in 2 patients. The median time of death from cardiac causes was 1 day after surgery (range, 0 to 19 days); for noncardiac causes, it was 17 days (range, 4 to 160 days). In univariate analysis, no patient, procedural, or time-related variable was predictive of mortality in this cohort. However, there was a trend toward higher mortality with longer CPB times (P50.07). In addition, all 3 patients with severely decreased ventricular function and all 3 patients with severely obstructed pulmonary venous drainage died. Three of the 5 infants with genetic syndromes, 2 with Turner’s syndrome, and 1 with Rubinstein-Taybi syndrome died. Discussion Although improvements in surgical technique and perioperative care have led to increased survival after surgical reconstruction for HLHS, operative mortality after the first stage is still high (9% to 47%).4,6,10 Many investigators have attempted to identify factors predictive of death after the Norwood procedure. Identification of high-risk characteristics would aid in the management of these patients and may help in deciding more precisely which patients should be listed for transplantation and which should undergo staged reconstruction.16 Prematurity and low weight in patients with HLHS have been found to be risk factors for higher mortality in some reports. However, contemporary reviews of low- Low-Weight Infants and Stage 1 Reconstruction II-169 weight neonates with a wide variety of lesions requiring CPB for repair have suggested that low weight alone is not a contraindication to early repair.12–14 In the present study, survival was 47% for low-weight infants undergoing stage 1 reconstruction. Overall survival for stage 1 reconstruction at our institution is 74%. Within the cohort of low-weight infants, weight at the time of stage 1 reconstruction for HLHS had no effect on survival. In addition, no statistically significant increase or decrease in weight was evident in patients in whom surgery was delayed. Only a few studies have focused on the influence of weight on survival after CPB.12,14,17,18 Pawade and associates14 from the Royal Children’s Hospital in Melbourne analyzed risk factors for mortality in infants weighing ,2.5 kg undergoing CPB and compared them with a similar group undergoing CPB with weights .2.5 kg. There were 8 univentricular repairs and 52 biventricular repairs. HLHS was present in only 4 of the low-weight infants. There were 946 patients in the control group. Early mortality in the low-weight group was 16.5% compared with 7.5% for the overall population (P,0.006). Predictors of mortality were preoperative metabolic acidosis, univentricular repair, and longer CPB time. Of the 4 patients with HLHS, 2 were early survivors.14 Rossi and colleagues13 recently reviewed the outcomes of cardiac operations in 30 infants weighing #2 kg at the Mount Sinai Hospital in New York. Hospital survival was 83%, with no difference in mortality rates based on age, weight, or type of procedure. HLHS was present in 4 patients; 3 of these were early survivors. Bove and Lloyd3 reviewed 158 patients undergoing staged reconstruction from 1990 through 1995 and identified several risk factors for early death, including age .1 month, pulmonary venous obstruction, significant noncardiac congenital anomalies, gestational age ,35 weeks, and birth weight ,2.5 kg. Hospital survival for the 31 patients with these high-risk characteristics was 42%, which was significantly lower than the 86% survival rate seen in the remaining 127 patients (P50.0001).3 Forbess and colleagues17 reviewed 212 consecutive patients undergoing stage 1 reconstruction at the Children’s Hospital of Boston between 1983 and 1993. Operative mortality in this group was 46.2%. Patients weighing ,3 kg were at higher risk for hospital death, and weight ,3 kg was an independent risk factor for mortality in the multivariate analysis. The authors suggested that low-weight patients might be best served by referral for transplantation.17 However, in the CHSS study, low birth weight was found to be an incremental risk factor for death in both staged reconstruction and heart transplantation protocols.3 Overall experience of transplantation in low-weight infants is limited. In the largest series reported of infants transplanted for HLHS at Loma Linda (142 patients of 176 listed), the average weight was 3.760.8 kg (range, 2.0 to 6.4 kg; median weight, 3.6 kg).19 Traditional experience with cardiac surgery in small infants has suggested that weight is indeed a risk factor for early intervention.12–14,18 The reasons for the increased mortality are probably multifactorial. The neonatal heart is less compliant that the mature heart and is thought to be less capable of handling a volume load.20,21 The immature pulmonary II-170 Circulation November 9, 1999 Downloaded from http://circ.ahajournals.org/ by guest on June 14, 2017 vascular bed can make the postoperative course unpredictable, and underdeveloped renal and hepatic functions impair fluid balance.12–15,22 Patients born prematurely may have hyaline membrane disease and are at risk for necrotizing enterocolitis.13,22 In addition, the germinal matrix of the premature infant is more susceptible to hemorrhage and neurological complications from heparinization and CPB.18 Newborns of small size undergoing the Norwood procedure present the additional technical challenges of cannulation, cerebral protection, aortic arch reconstruction for extremely small vessels, and selection of an appropriate shunt size to provide adequate but not excessive pulmonary blood flow while allowing for sufficient growth. While this may be the largest reported series of small infants undergoing stage 1 reconstructive surgery, the statistical power to define significant predictors of mortality is limited. For example, the influence of severely diminished ventricular performance, obstructed pulmonary venous drainage, and prolonged CPB times may be underestimated because of the small number of patients in the series with those characteristics. As in previous reports examining the influence of weight on cardiac surgery, weight differences within this group had no effect on mortality.12–14 Because of the entry criteria of the study, we did not identify patients who might have been born at ,2.5 kg but grew preoperatively to weigh more than this at the time of surgery or patients who were not referred for surgery because of low birth weight. Yet in this review, patients in whom surgery was delayed did not experience a statistical increase or decrease in weight before the date of surgery, similar to previous reports.13,14 This finding suggests that a delay in surgery to allow weight gain is not justified and exposes the patient to prolonged risks of pulmonary overcirculation, long-term mechanical ventilation, and other ICU-related complications.13,14 The study design also does not reveal how many infants at various postgestational ages might have died before surgery. Within the limitations of this study, however, age at operation did not influence survival. Previous studies have not compared ageweight appropriateness and outcome in cardiac surgery, but in this study, being born small for gestational age did not predict a higher mortality. Although low-birth-weight infants with congenital cardiac defects, including HLHS, are likely to be at a higher risk for postoperative death and complications than their normal-size counterparts, current experience does not suggest that waiting to obtain a minimum weight before surgery improves outcome. Therefore, a delay in surgical intervention in the hope of somatic growth is not warranted. Survival for low-weight infants undergoing stage 1 reconstruction in general is decreased compared with larger babies having the same operation at the same institution; however, the absolute survival rates are acceptable by national standards. Weight alone should not be considered a contraindication to staged surgical reconstruction in infants with HLHS. Acknowledgment This work was supported in part by the Daniel M. Tabas Endowed Chair in Cardiothoracic Surgery. References 1. Norwood WI, Kirklin JK, Sanders SP. Hypoplastic left heart syndrome: experience with palliative surgery. Am J Cardiol. 1980;45:87–91. 2. Doty DB, Marvin WJ, Schieken RM, Lauer RM. Hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 1980;80:148 –152. 3. Jacobs ML, Blackstone EH, Bailey LL. Intermediate survival in neonates with aortic atresia: a multi-institutional study. J Thorac Cardiovasc Surg. 1998;116:417– 431. 4. Bove EL, Lloyd TR. Staged reconstruction for hypoplastic left heart syndrome. Ann Surg. 1996;224:387–395. 5. Jacobs ML, Rychik J, Murphy JD, Nicolson SC, Steven JM, Norwood WI. Results of Norwood’s operation for lesions other than hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 1995;110:1557–1562. 6. Jonas RA, Hansen DD, Cook N, Wessel D. Anatomic subtype and survival after reconstructive operation for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 1994;107:1121–1128. 7. Helton JG, Aglira BA, Chin AJ, Murphy JD, Pigott JD, Norwood WI. Analysis of potential or physiologic determinants of outcome of palliative surgery for hypoplastic left heart syndrome. Circulation. 1986;74(suppl I):I-70 –I-76. 8. Starnes VA, Griffin ML, Pitlick PT, Bernstein D, Baum D, Ivens K, Shumway NE. Current approach to hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 1992;104:189 –195. 9. Barber G, Chin AJ, Murphy JD, Pigott JD, Norwood WI. Hypoplastic left heart syndrome: lack of correlation between preoperative demographic and laboratory findings and survival following palliative surgery. Pediatr Cardiol. 1989;10:129 –134. 10. Murdison KA, Baffa JM, Farrell PE, Chang AC, Barber G, Norwood WI, Murphy JD. Hypoplastic left heart syndrome: outcome after initial reconstruction and before modified Fontan procedure. Circulation. 1990;82(suppl IV):IV-199 –IV-207. 11. Barber G, Helton JG, Aglira BA, Chin AJ, Murphy JD, Pigott JD, Norwood WI. The significance of tricuspid regurgitation in hypoplastic left-heart syndrome. Am Heart J. 19888;116:1563–1567. 12. Chang AC, Hanley FL, Lock JE, Castaneda AR, Wessel DL. Management and outcome of low birth weight neonates with congenital heart disease. J Pediatr. 1994;124:461– 466. 13. Rossi AF, Seiden HS, Sadeghi AM, Nguyen KH, Quintana CS, Gross RP, Griepp RB. The outcome of cardiac operations in infants weighing two kilograms or less. J Thorac Cardiovasc Surg. 1998;116: 28 –35. 14. Pawade A, Waterson K, Laussen P, Karl TR, Mee RBB. Cardiopulmonary bypass in neonates weighing less than 2.5 kg: analysis of the risk factors for early and late mortality. J Card Surg. 1993;8:1– 8. 15. Borowski A, Schickendantz S, Mennicken U, Korb H. Open heart interventions in premature low and very low birth weight neonates: risk profile and ethical considerations. J Thorac Cardiovasc Surg. 1997;45;238 –241. 16. Kern JH, Hayes CJ, Michler RE, Gersony WM, Quaegebeur JM. Survival and risk factor analysis for the Norwood procedure for hypoplastic left heart syndrome. Am J Cardiol. 1997;80:170 –174. 17. Forbess JM, Cook N, Roth SJ, Serraf A, Mayer JE, Jonas RA. Ten-year institutional experience with palliative surgery for hypoplastic left heart syndrome. Circulation. 1995;92(suppl II):II262–II-266. 18. Kirklin JK, Blackstone EH, Kirklin JW, McKay R, Pacifico AD, Bargeron LM. Intracardiac surgery in infants under age 3 months: incremental risk factors for hospital mortality. Am J Cardiol. 1981; 48:500 –506. 19. Razzouk AJ, Chinnock RE, Gundry SR, Johnston JK, Larsen RL, Baum MF, Mulla NF, Bailey LL. Transplantation as a primary treatment for hypoplastic left heart syndrome: intermediate-term results. Ann Thorac Surg. 1996;62:1– 8. 20. Flanagan MF, Fugii AM, Colan SD, Lock JE. Inhibitory effects on myocardial perfusion in pressure overload hypertrophy in immature lambs. Pediatr Res. 1988;23:218. Abstract. 21. Friedman WF. The intrinsic physiologic properties of the developing heart. Prog Cardiovasc Dis. 1972;15:87–111. 22. Bove EL, Behrendt DM. Open heart surgery in the first week of life. Ann Thorac Surg. 1980;29:130 –134. Early Survival of Infants Weighing 2.5 Kilograms or Less Undergoing First-Stage Reconstruction for Hypoplastic Left Heart Syndrome Samuel Weinstein, J. William Gaynor, Nancy D. Bridges, Gil Wernovsky, Lisa M. Montenegro, Rodolfo I. Godinez and Thomas L. Spray Downloaded from http://circ.ahajournals.org/ by guest on June 14, 2017 Circulation. 1999;100:II-167-Ii-170 doi: 10.1161/01.CIR.100.suppl_2.II-167 Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © 1999 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539 The online version of this article, along with updated information and services, is located on the World Wide Web at: http://circ.ahajournals.org/content/100/suppl_2/II-167 Permissions: Requests for permissions to reproduce figures, tables, or portions of articles originally published in Circulation can be obtained via RightsLink, a service of the Copyright Clearance Center, not the Editorial Office. Once the online version of the published article for which permission is being requested is located, click Request Permissions in the middle column of the Web page under Services. Further information about this process is available in the Permissions and Rights Question and Answer document. Reprints: Information about reprints can be found online at: http://www.lww.com/reprints Subscriptions: Information about subscribing to Circulation is online at: http://circ.ahajournals.org//subscriptions/