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Ann Thorac Surg 2001;72:1349-1353
© 2001 The Society of Thoracic Surgeons
a Division of Cardiovascular Surgery, Department of Surgery, The Hospital for Sick Children, University of Toronto School of Medicine, Toronto, Ontario, Canada
b Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto School of Medicine, Toronto, Ontario, Canada
Accepted for publication April 27, 2001.
Address reprint requests to Dr Williams, Division of Cardiovascular Surgery, The Hospital for Sick Children, 555 University Ave, Room 1525, Toronto, ON M5G 1X8, Canada
e-mail: tony.azakie{at}utoronto.ca
| Abstract |
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Methods. A modified Norwood operation was performed in 171 infants over a 10-year period. Sixty-eight percent of the infants were male, the median age at operation was 6 days (range 1 to 175 days), and the median weight was 3.3 kg (range 1.7 to 4.8 kg). The 10-year period was divided into three eras: era I; 1990 through 1993; era II; 1994 through 1997; and era III; 1998 into 2000. Outcomes and risk factors for mortality were sought.
Results. Hypoplastic left heart syndrome or a variant was the primary diagnosis in 118 infants (69%). The overall 5-year survival rate was 43%. Multivariate analysis revealed that only need of preoperative ventilatory support, earlier date of operation, and lower weight at operation were significant independent predictors of increased time-related mortality. Morphologic features such as a diagnosis other than hypoplastic left heart syndrome, ascending aortic size, and noncardiac anomalies were not significantly associated with an increased risk of death. The hospital survival rate for stage-one palliation in era III was 82%, significantly better than that in the preceding eras (p < 0.001). Attrition between stages one and two accounted for a 15% mortality rate among hospital survivors.
Conclusions. With increasing experience and improvements in perioperative care and surgical technique, good outcomes can be expected for the first-stage modified Norwood procedure. Greater monitoring of patients in the interstage period may reduce interval mortality and improve overall survival.
| Introduction |
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Over the past 10 years, our approach to the modified Norwood procedure has changed. Heart transplantation has been offered as a primary treatment modality for HLHS with increasing frequency, and preoperative and postoperative management, surgical technique, and perfusion strategies have evolved to promote earlier diagnosis, modified arch reconstruction, avoidance of circulatory arrest, and efforts to balance the circulations with aggressive afterload reduction. This study reviews our 10-year single-institution experience with the modified Norwood procedure to determine the factors associated with improved results of first-stage Norwood palliation in the current era and the midterm outcomes.
| Material and methods |
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From March 1990 to October 2000, 171 modified Norwood procedures were performed at The Hospital for Sick Children in Toronto. There were 117 male (68%) and 54 female patients (32%) with a median age at operation of 6 days (range, 1 to 175 days). Median weight at operation was 3.3 kg (1.7 to 4.8 kg) and the mean body surface area, 0.23 ± 0.06 m2. Hypoplastic left heart syndrome or its variants was the primary diagnosis in 118 infants (69%). In the other 53 patients (30%), the diagnosis was another malformation with single-ventricle physiology. The distribution of typical HLHS malformations included the following: aortic stenosis and mitral stenosis (n = 29%); aortic atresia and mitral atresia (n = 22%); aortic atresia and mitral stenosis (n = 19%); mitral atresia and aortic stenosis (n = 14%); aortic stenosis (n = 11%); and mitral stenosis (n = 5%). The nonHLHS diagnoses for which a modified Norwood procedure was performed were as follows: tricuspid atresia (n = 20), double-inlet left ventricle (n = 14); double-outlet right ventricle (n = 6); hypoplastic left heart complex with ventricular septal defect (n = 5); atrioventricular septal defect (n = 5); and miscellaneous lesions (n = 3). Systemic atrioventricular valve regurgitation (n = 162) was absent in 56 patients (35%), mild in 23 (14%), mild to moderate in 66 (41%), moderate in 9 (6%), and moderate to severe in 8 patients (5%). Ascending aortic diameter (n = 159) was 4 ± 2 mm.
Prior to the modified Norwood operation (n = 157), eight procedures had been performed: balloon atrial septostomy in 7 patients and repair of coarctation in 1 patient. Presurgical management included administration of prostaglandin in 92% (145 of 158 patients), use of inotropic agents in 30% (47 of 150 patients), and preoperative ventilatory support in 70% (108 of 154 patients).
Heart transplantation
The heart transplantation program at The Hospital for Sick Children was developed in 1990. During the study period, 20 newborns or fetuses have been placed on a waiting list for donor heart availability. Four neonates (20%) died awaiting transplantation, 1 neonate was stillborn. Since 1995, heart transplantation has been offered with increasing frequency to all families as a primary treatment of HLHS. During the study period, 14 neonates with HLHS or a variant had primary cardiac transplantation, and there was no operative mortality. Parental preference was the primary reason for selecting transplantation versus a reconstructive approach in 11 patients. For 9 patients, the cardiac care team advised in favor of transplantation for anatomic reasons (coronary fistulas in 2, ventricular dysfunction with persistent moderate to severe tricuspid regurgitation after resuscitation in 7). Since 1995, only 1 (6.2%) of 16 infants has died while awaiting a donor heart.
Operative technique
When circulatory arrest was used (n = 154 patients), cardiopulmonary bypass was established through main pulmonary artery and right atrial venous cannulation. The circulation was arrested at a mean temperature of 16°C ± 29°C for a mean duration of 45 ± 16 minutes. In the latest era, efforts to avoid circulatory arrest (n = 17) were accomplished by completing the arterial anastomosis of the modified Blalock-Taussig shunt and then cannulating the distal end for continuous perfusion of the innominate artery and its branches [11, 12].
Myocardial protection was achieved by antegrade cold blood cardioplegia. The mean cardiopulmonary bypass time was 94 ± 41 minutes. The mean duration of aortic cross-clamping was 51 ± 13 minutes.
Arch reconstruction was accomplished with a modified Norwood technique using a gusset of homograft material (n = 117 patients) or a modification as described by Ishino and colleagues [5] or by Fraser and Mee [13] (n = 54). In neonates having the former modification, an anterior arch homograft patch was used in 18%. The pulmonary artery confluence was patched in all instances.
The modified Blalock-Taussig shunt (n = 171) was constructed using Gore-Tex (W. L. Gore & Associates, Inc, Flagstaff, AZ) conduits with diameters ranging from 3.0 to 4.0 mm and a median length of 12 mm (range, 9 to 23 mm). A 3-mm shunt was used in 24 neonates, a 3.5-mm shunt in 117, and a 4-mm shunt in 30. In the current era (era III), delayed sternal closure was used in all patients.
Statistical analysis
Data are expressed as frequencies, means ± one standard deviation, and medians with ranges. Where data are missing, the number of available values is given. Time-related estimates of survival and intervention for neo-aortic obstruction (defined as a gradient > 20 mm Hg or discrete arch stenosis < 5 mm in diameter) were calculated using the Kaplan-Meier method. Risk factors for time-related survival and development of neo-aortic obstruction were evaluated by Cox proportional hazards modeling. The variables assessed as predictors of the time-related survival and intervention outcomes include date of operation, age, sex, weight, body surface area, diagnosis of HLHS, ascending aortic diameter, presence of aberrant right subclavian artery or interrupted arch, use of preoperative inotropic or ventilatory support, preoperative procedures, preoperative prostaglandin use, degree of systemic atrioventricular valve regurgitation, cardiopulmonary bypass time, aortic cross-clamp time, circulatory arrest time, avoidance of circulatory arrest, Blalock-Taussig shunt diameter and length, type of arch reconstruction, and use of phenoxybenzamine in the postoperative period. All analyses were performed with SAS statistical software Version 7 (SAS Institute, Inc, Cary, NC) using default settings. A p value of less than 0.05 was set as the level of significance.
| Results |
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Cox proportional hazards modeling was performed for each subgroup of infants within the three defined eras. Lower weight at the Norwood procedure was not an incremental risk factor for time-related mortality in era III (1998 into 2000).
Freedom from neoaortic obstruction was 99% (95% confidence interval, 97% to 100%) at 1 month, 88% (95% confidence interval, 82% to 94%) at 6 months, and 77% (95% confidence interval, 68% to 86%) at 1 year (Fig 4). In Cox proportional hazards modeling, the only significant independent factor associated with the development of neo-aortic obstruction was smaller diameter of the ascending aorta (hazard ratio of 1.49 per 1-mm decrease in diameter; p = 0.005). Sixty-three percent of infants in whom arch obstruction developed had an ascending aortic diameter of less than 3 mm. After controlling for this variable, no other variable, including arch reconstructive technique or presence of an aberrant right subclavian artery, was significantly associated with time-related neoaortic obstruction.
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Risk factors for mortality
Reported risk factors for mortality include earlier era of operation [6, 9], older age at operation [6, 9], lower weight at operation [4, 6], anatomic subtype [2, 3, 14], diagnosis of HLHS versus that of another single-ventricle physiology [15], prematurity [15], ascending aortic diameter [3], preoperative condition [3], associated noncardiac anomalies [4, 10], pulmonary venous obstruction or restrictive interatrial communication [10], and an aberrant right subclavian artery [5]. In our experience, low birth weight, preoperative condition characterized by use of mechanical ventilation, and earlier era of operation were independent risk factors for mortality after stage-one palliation and for overall death. Although low birth weight is a risk factor for stage-one mortality, it should not be considered a contraindication to reconstructive surgical intervention [16]. We have recently had favorable outcomes in neonates weighing as little as 1.5 kg, and by secondary Cox analysis, lower weight was not a risk factor for time-related mortality in era III. Furthermore, delaying repair in such infants would probably only result in increased morbidity.
The exact features of the "era effect" that have allowed for improved outcomes are difficult to quantify and are probably numerous. Although not directly evaluated in this study, the increased use of fetal echocardiography during prenatal ultrasound surveillance has led to earlier diagnosis, which may improve preoperative management and overall mortality. In neonates who are candidates for the modified Norwood procedure, aggressive early resuscitation with prostaglandins and avoidance of pulmonary overcirculation may obviate unnecessary endotracheal intubation, mechanical ventilation, and hemodynamic instability. Furthermore, the option of a primary transplant track has been offered to all families since 1995 and, recently has been encouraged (and used) for neonates who may represent a particularly "high-risk" group (eg, moderate to severe systemic atrioventricular valve regurgitation, presence of coronary sinusoids, poor ventricular function, extracardiac anomalies). Thus, improved outcomes for the modified Norwood procedure in era III may in part be due to the select population in whom it was performed.
Reconstructive technique
Our current operative approach to the first-stage reconstruction involves the avoidance of circulatory arrest using modified perfusion techniques and cannulation of the Blalock-Taussig shunt. A pH-stat strategy for acid-base management and modified ultrafiltration in the period after bypass are also used. The arch is reconstructed using the modification described by Ishino and coworkers [5]. All ductal tissue is resected, and primary autogenous tissuetissue anastomoses between the back walls of the descending aorta, the arch, and the main pulmonary artery are performed. If necessary, an anterior homograft patch is used to allow a tension-free, widely patent reconstruction. In the postoperative period, aggressive afterload reduction with phenoxybenzamine [17], myocardial contractile support, and maneuvers that minimize pulmonary overcirculation are combined to achieve a balanced circulation with adequate peripheral perfusion.
Midterm outcomes
After successful stage-one reconstruction, midterm survival ranges from 40% to 60% at 5 years and was 43% in our cohort, a result comparable to rates reported in other large series [4, 6, 10, 15]. Attrition between stage-one and stage-2 palliation has an important adverse effect on midterm survival and accounts for an additional mortality of 5% to 15% of patients. In our experience, 15% of hospital survivors died prior to second-stage reconstruction. Of the 15 infants who died prior to stage-two operation, 12 were greater than 2 months of age. Assuming that these patients would have been candidates for bidirectional cavopulmonary anastomosis and that the cause of death was related to the systemicpulmonary shunt, then earlier performance of the second-stage operation may have minimized interstage attrition. The causes of death in the majority of infants is not clear because of lack of autopsy data. However, death was apparently sudden in all patients and probably was related to an acute myocardial event, arrhythmia, shunt complications, or neo-aortic arch obstruction [18].
Neoaortic arch obstruction
Obstruction of the reconstructed arch [19] developed in 19 hospital survivors within the first year after repair and usually was identified and evaluated by echocardiography or cardiac catheterization prior to second-stage reconstruction. Percutaneous catheter-based balloon dilation [19] was initially successful in 88% of children but had to be repeated in 17% or required surgical augmentation in another 12%. Smaller size of the ascending aorta was a multivariate risk factor for the development of neo-aortic obstruction. Other factors including type of arch reconstruction, presence of an associated aberrant right subclavian artery, and interrupted arch were not significant.
Conclusions
Outcomes for the modified Norwood procedure are constantly improving. Prenatal diagnosis, preoperative stabilization, and improvements in surgical technique and perioperative care account for a substantial increase in survival after stage one palliation. Risk stratification for HLHS and appropriate triage to orthotopic heart transplantation also contribute to favorable results for the Norwood procedure. Aggressive efforts at monitoring between stage one and stage two and a move toward earlier performance of a bidirectional cavopulmonary anastomosis or a hemi-Fontan operation may limit early attrition. The favorable outcomes for second-stage and Fontan reconstruction [20] combined with optimizing stage-one results should have a favorable impact on perinatal counseling for HLHS or its variants.
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S. J. Roth, I. Adatia, G. D. Pearson, and and Members of the Cardiology Group Summary Proceedings From the Cardiology Group on Postoperative Cardiac Dysfunction Pediatrics, March 1, 2006; 117(Supplement_1): S40 - S46. [Abstract] [Full Text] [PDF] |
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C. N. Stasik, C. S. Goldberg, E. L. Bove, E. J. Devaney, and R. G. Ohye Current outcomes and risk factors for the Norwood procedure J. Thorac. Cardiovasc. Surg., February 1, 2006; 131(2): 412 - 417. [Abstract] [Full Text] [PDF] |
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M. Griselli, S. P. McGuirk, O. Stumper, A. J.B. Clarke, P. Miller, R. Dhillon, J. G.C. Wright, J. V. de Giovanni, D. J. Barron, and W. J. Brawn Influence of surgical strategies on outcome after the Norwood procedure J. Thorac. Cardiovasc. Surg., February 1, 2006; 131(2): 418 - 426. [Abstract] [Full Text] [PDF] |
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S. Tabbutt, T. E. Dominguez, C. Ravishankar, B. S. Marino, P. J. Gruber, G. Wernovsky, J. W. Gaynor, S. C. Nicolson, and T. L. Spray Outcomes After the Stage I Reconstruction Comparing the Right Ventricular to Pulmonary Artery Conduit With the Modified Blalock Taussig Shunt Ann. Thorac. Surg., November 1, 2005; 80(5): 1582 - 1591. [Abstract] [Full Text] [PDF] |
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S. Takabayashi, H. Shimpo, M. Kajimoto, K. Yokoyama, H. Kado, and Y. Mitani Stage I bilateral pulmonary artery banding maintains systemic flow by prostaglandin E1 infusion or a main pulmonary artery to the descending aorta shunt for hypoplastic left heart syndrome Interact CardioVasc Thorac Surg, August 1, 2005; 4(4): 352 - 355. [Abstract] [Full Text] [PDF] |
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C. L. Cua, R. R. Thiagarajan, R. Taeed, T. M. Hoffman, L. Lai, J. Hayes, P. C. Laussen, and T. F. Feltes Improved Interstage Mortality With the Modified Norwood Procedure: A Meta-Analysis Ann. Thorac. Surg., July 1, 2005; 80(1): 44 - 49. [Abstract] [Full Text] [PDF] |
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S. Takabayashi, H. Kado, Y. Shiokawa, K. Fukae, and T. Nakano Comparison of hemodynamics between Norwood procedure and systemic-to-pulmonary artery shunt for single right ventricle patients Eur J Cardiothorac Surg, June 1, 2005; 27(6): 968 - 974. [Abstract] [Full Text] [PDF] |
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P. A. Checchia, J. McCollegan, N. Daher, N. Kolovos, F. Levy, and B. Markovitz The effect of surgical case volume on outcome after the Norwood procedure J. Thorac. Cardiovasc. Surg., April 1, 2005; 129(4): 754 - 759. [Abstract] [Full Text] [PDF] |
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S. R. Meyer, P. M. Campbell, J. M. Rutledge, A. M. Halpin, L. E. Hawkins, J. R. T. Lakey, I. M. Rebeyka, and D. B. Ross Use of an allograft patch in repair of hypoplastic left heart syndrome may complicate future transplantation Eur J Cardiothorac Surg, April 1, 2005; 27(4): 554 - 560. [Abstract] [Full Text] [PDF] |
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U Theilen and L Shekerdemian The intensive care of infants with hypoplastic left heart syndrome Arch. Dis. Child. Fetal Neonatal Ed., March 1, 2005; 90(2): F97 - F102. [Abstract] [Full Text] [PDF] |
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Y. Tanoue, H. Kado, Y. Shiokawa, N. Fusazaki, and S. Ishikawa Midterm Ventricular Performance After Norwood Procedure With Right Ventricular-Pulmonary Artery Conduit Ann. Thorac. Surg., December 1, 2004; 78(6): 1965 - 1971. [Abstract] [Full Text] [PDF] |
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A. J. Lodge, J. Rychik, S. C. Nicolson, R. F. Ittenbach, T. L. Spray, and J. W. Gaynor Improving Outcomes in Functional Single Ventricle and Total Anomalous Pulmonary Venous Connection Ann. Thorac. Surg., November 1, 2004; 78(5): 1688 - 1695. [Abstract] [Full Text] [PDF] |
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N. C. De Oliveira, D. A. Ashburn, F. Khalid, H. M. Burkhart, I. T. Adatia, H. M. Holtby, W. G. Williams, and G. S. Van Arsdell Prevention of Early Sudden Circulatory Collapse After the Norwood Operation Circulation, September 14, 2004; 110(11_suppl_1): II-133 - II-138. [Abstract] [Full Text] [PDF] |
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S. M. Bradley, J. M. Simsic, T. C. McQuinn, D. M. Habib, G. S. Shirali, and A. M. Atz Hemodynamic status after the Norwood procedure: A comparison of right ventricle-to-pulmonary artery connection versus modified blalock-taussig shunt Ann. Thorac. Surg., September 1, 2004; 78(3): 933 - 941. [Abstract] [Full Text] [PDF] |
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R. G. Ohye, A. Ludomirsky, E. J. Devaney, and E. L. Bove Comparison of right ventricle to pulmonary artery conduit and modified Blalock-Taussig shunt hemodynamics after the Norwood operation Ann. Thorac. Surg., September 1, 2004; 78(3): 1090 - 1093. [Abstract] [Full Text] [PDF] |
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J. A. Connor, R. R. Arons, M. Figueroa, and K. M. Gebbie Clinical Outcomes and Secondary Diagnoses for Infants Born With Hypoplastic Left Heart Syndrome Pediatrics, August 1, 2004; 114(2): e160 - e165. [Abstract] [Full Text] [PDF] |
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A. P. Vlahos, J. E. Lock, D. B. McElhinney, and M. E. van der Velde Hypoplastic Left Heart Syndrome With Intact or Highly Restrictive Atrial Septum: Outcome After Neonatal Transcatheter Atrial Septostomy Circulation, May 18, 2004; 109(19): 2326 - 2330. [Abstract] [Full Text] [PDF] |
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A. Azakie, D. Martinez, A. Sapru, J. Fineman, D. Teitel, and T. R. Karl Impact of right ventricle to pulmonary artery conduit on outcome of the modified norwood procedure Ann. Thorac. Surg., May 1, 2004; 77(5): 1727 - 1733. [Abstract] [Full Text] [PDF] |
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R. D. B. Jaquiss, N. S. Ghanayem, G. M. Hoffman, R. T. Fedderly, J. R. Cava, K. A. Mussatto, and J. S. Tweddell Early cavopulmonary anastomosis in very young infants after the Norwood procedure: Impact on oxygenation, resource utilization, and mortality J. Thorac. Cardiovasc. Surg., April 1, 2004; 127(4): 982 - 989. [Abstract] [Full Text] [PDF] |
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R. Sittiwangkul, A. Azakie, G. S. Van Arsdell, W. G. Williams, and B. W. McCrindle Outcomes of tricuspid atresia in the Fontan era Ann. Thorac. Surg., March 1, 2004; 77(3): 889 - 894. [Abstract] [Full Text] [PDF] |
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T. Nakano, H. Kado, Y. Shiokawa, K. Fukae, Y. Nishimura, K. Miyamoto, Y. Tanoue, H. Tatewaki, and N. Fusazaki The low resistance strategy for the perioperative management of the Norwood procedure Ann. Thorac. Surg., March 1, 2004; 77(3): 908 - 912. [Abstract] [Full Text] [PDF] |
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P. A. Checchia, R. Larsen, R. Sehra, N. Daher, S. R. Gundry, A. J. Razzouk, and L. L. Bailey Effect of a selection and postoperative care protocol on survival of infants with hypoplastic left heart syndrome Ann. Thorac. Surg., February 1, 2004; 77(2): 477 - 483. [Abstract] [Full Text] [PDF] |
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N.S. Ghanayem, G.M. Hoffman, K.A. Mussatto, J.R. Cava, P.C. Frommelt, N.A. Rudd, M.M. Steltzer, S.M. Bevandic, S.J. Frisbee, R.D.B. Jaquiss, et al. Home surveillance program prevents interstage mortality after the Norwood procedure J. Thorac. Cardiovasc. Surg., November 1, 2003; 126(5): 1367 - 1375. [Abstract] [Full Text] [PDF] |
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C. Pizarro, E. Malec, K. O. Maher, K. Januszewska, S. S. Gidding, K. A. Murdison, J. M. Baffa, and W. I. Norwood Right Ventricle to Pulmonary Artery Conduit Improves Outcome After Stage I Norwood for Hypoplastic Left Heart Syndrome Circulation, September 9, 2003; 108(2011): II-155 - II-160. [Abstract] [Full Text] [PDF] |
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S. B. O'Blenes, S. L. Merklinger, A. Jegatheeswaran, A. Campbell, M. Rabinovitch, I. Rebeyka, and G. Van Arsdell Low Molecular Weight Heparin and Unfractionated Heparin Are Both Effective at Accelerating Pulmonary Vascular Maturation in Neonatal Rabbits Circulation, September 9, 2003; 108(2011): II-161 - II-166. [Abstract] [Full Text] [PDF] |
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C. Pizarro and W. I. Norwood Right ventricle to pulmonary artery conduit has a favorable impact on postoperative physiology after Stage I Norwood: preliminary results Eur J Cardiothorac Surg, June 1, 2003; 23(6): 991 - 995. [Abstract] [Full Text] [PDF] |
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D. A. Ashburn, B. W. McCrindle, C. I. Tchervenkov, M. L. Jacobs, G. K. Lofland, E. L. Bove, T. L. Spray, W. G. Williams, and E. H. Blackstone Outcomes after the Norwood operation in neonates with critical aortic stenosis or aortic valve atresia J. Thorac. Cardiovasc. Surg., May 1, 2003; 125(5): 1070 - 1082. [Abstract] [Full Text] [PDF] |
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E. Malec, K. Januszewska, J. Kolcz, and T. Mroczek Right ventricle-to-pulmonary artery shunt versus modified Blalock-Taussig shunt in the Norwood procedure for hypoplastic left heart syndrome - influence on early and late haemodynamic status Eur J Cardiothorac Surg, May 1, 2003; 23(5): 728 - 734. [Abstract] [Full Text] [PDF] |
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M. B. Mitchell, D. N. Campbell, M. M. Boucek, H. M. Sondheimer, K. C. Chan, D. D. Ivy, B. Pietra, and T. Mackenzie Mechanical limitation of pulmonary blood flow facilitates heart transplantation in older infants with hypoplastic left heart syndrome Eur J Cardiothorac Surg, May 1, 2003; 23(5): 735 - 742. [Abstract] [Full Text] [PDF] |
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J. S. Tweddell, G. M. Hoffman, K. A. Mussatto, R. T. Fedderly, S. Berger, R. D. B. Jaquiss, N. S. Ghanayem, S. J. Frisbee, and S. B. Litwin Improved Survival of Patients Undergoing Palliation of Hypoplastic Left Heart Syndrome: Lessons Learned From 115 Consecutive Patients Circulation, September 24, 2002; 106(12_suppl_1): I-82 - I-89. [Abstract] [Full Text] [PDF] |
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