PEDIATRICS Vol. 107 No. 6 June 2001, pp. 1277-1282
Impact of Prenatal Diagnosis on Survival and Early Neurologic Morbidity in Neonates With the Hypoplastic Left Heart Syndrome
Received Jun 1, 2000; accepted Sep 27, 2000.
, §, 
,,
From the Divisions of * Cardiology and Background. Prenatal echocardiography
can identify the fetus that has complex congenital heart disease and
may improve early management and surgical outcome. Prenatal diagnosis
may be particularly beneficial to patients who have hypoplastic left
heart syndrome (HLHS) and who are at risk for hypoxic-ischemic insult
at presentation.
Objectives. We sought to determine whether prenatal
diagnosis reduces neurologic morbidity and operative mortality in
patients who undergo palliative surgery for the HLHS.
Methods. Data from all patients who had HLHS, except for
those with lethal genetic anomalies, and who were admitted to our
institution between July 1992 and September 1997 were analyzed to
assess the impact of prenatal diagnosis on preoperative management,
neurologic morbidity, and surgical mortality. The primary outcome
measures were hospital mortality and the incidence of adverse
neurologic events (seizure or coma).
Results. There were 216 patients who had HLHS and were
referred for surgical palliation, 79 (36.6%) of whom had been
diagnosed prenatally. All patients who had been diagnosed prenatally
were delivered in an advanced nursery and were started on prostaglandin
E1 on the first day of life. Patients whose HLHS was
diagnosed postnatally were begun on prostaglandin
E1 later in life (median = day 2 [range = 1-28 days]). There were 4 preoperative deaths and 53 operative or postoperative deaths. Overall hospital mortality was
26.4% and did not differ between patients whose HLHS had been
diagnosed prenatally and those whose HLHS had been diagnosed
postnatally. With the use of multivariable analysis, prenatal diagnosis
was associated with fewer adverse perioperative neurologic events in
the patients whose HLHS had been diagnosed prenatally than in those
whose HLHS had been diagnosed postnatally (odds ratio = 0.46).
Conclusions. These data suggest that prenatal diagnosis
has a favorable impact on treatment of patients who have HLHS and are
undergoing staged palliation and reduces early neurologic morbidity.
Prenatal diagnosis was not associated with reduced hospital mortality. It is possible that prenatal diagnosis may improve long-term neurologic outcome.
Neurology at the
Children's Hospital of Philadelphia and the Departments of
§ Pediatrics and Neurology at the University of Pennsylvania School
of Medicine, Philadelphia, Pennsylvania; and ¶ DataMedix Corporation,
Media, Pennsylvania.
CiteULike 
Connotea 
Del.icio.us 
Digg 
Facebook 
Reddit 
Technorati 
Twitter What's this?
This article has been cited by other articles:
|
|
 |
|
  W. T. Mahle, J. W. Newburger, G. P. Matherne, F. C. Smith, T. R. Hoke, R. Koppel, S. S. Gidding, R. H. Beekman III, S. D. Grosse, and on behalf of the American Heart Association Congen Role of Pulse Oximetry in Examining Newborns for Congenital Heart Disease: A Scientific Statement From the American Heart Association and American Academy of Pediatrics Circulation, August 4, 2009; 120(5): 447 - 458. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 W. T. Mahle, J. W. Newburger, G. P. Matherne, F. C. Smith, T. R. Hoke, R. Koppel, S. S. Gidding, R. H. Beekman III, S. D. Grosse, and on behalf of the American Heart Association Congen Role of Pulse Oximetry in Examining Newborns for Congenital Heart Disease: A Scientific Statement from the AHA and AAP Pediatrics, August 1, 2009; 124(2): 823 - 836. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. S. Sklansky, D. P. Berman, J. D. Pruetz, and R.-K. R. Chang Prenatal Screening for Major Congenital Heart Disease: Superiority of Outflow Tracts Over the 4-Chamber View J. Ultrasound Med., July 1, 2009; 28(7): 889 - 899. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R.-K. R. Chang, M. Gurvitz, and S. Rodriguez Missed Diagnosis of Critical Congenital Heart Disease Arch Pediatr Adolesc Med, October 1, 2008; 162(10): 969 - 974. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. A. Hehir, T. E. Dominguez, J. A. Ballweg, C. Ravishankar, B. S. Marino, G. L. Bird, S. C. Nicolson, T. L. Spray, J. W. Gaynor, and S. Tabbutt Risk factors for interstage death after stage 1 reconstruction of hypoplastic left heart syndrome and variants J. Thorac. Cardiovasc. Surg., July 1, 2008; 136(1): 94 - 99. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. A. Glatz, S. Tabbutt, J. W. Gaynor, J. J. Rome, L. Montenegro, T. L. Spray, and J. Rychik Hypoplastic Left Heart Syndrome With Atrial Level Restriction in the Era of Prenatal Diagnosis Ann. Thorac. Surg., November 1, 2007; 84(5): 1633 - 1638. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. Espinoza, R. Romero, J. P. Kusanovic, F. Gotsch, O. Erez, W. Lee, L. F. Goncalves, M. L. Schoen, and S. S. Hassan The Role of the Sagittal View of the Ductal Arch in Identification of Fetuses With Conotruncal Anomalies Using 4-Dimensional Ultrasonography J. Ultrasound Med., September 1, 2007; 26(9): 1181 - 1188. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. R. Rempel and M. J. Harrison Safeguarding Precarious Survival: Parenting Children Who Have Life-Threatening Heart Disease Qual Health Res, July 1, 2007; 17(6): 824 - 837. [Abstract] [PDF]
|
|
|
|
 |
|
 A. Tzifa, C. Barker, S. M Tibby, and J. M Simpson Prenatal diagnosis of pulmonary atresia: impact on clinical presentation and early outcome Arch. Dis. Child. Fetal Neonatal Ed., May 1, 2007; 92(3): F199 - F203. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. S. Goldberg, E. L. Bove, E. J. Devaney, E. Mollen, E. Schwartz, S. Tindall, C. Nowak, J. Charpie, M. B. Brown, T. J. Kulik, et al. A randomized clinical trial of regional cerebral perfusion versus deep hypothermic circulatory arrest: Outcomes for infants with functional single ventricle J. Thorac. Cardiovasc. Surg., April 1, 2007; 133(4): 880 - 887. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. S. Sklansky Prenatal Screening for Congenital Heart Disease: A Moving Proposal J. Ultrasound Med., January 1, 2007; 26(1): 1 - 3. [Full Text] [PDF]
|
|
|
|
|
|
M. S. Cohen, A. H. Schultz, Z.-Y. Tian, D. D. Donaghue, P. M. Weinberg, J. W. Gaynor, and J. Rychik Heterotaxy Syndrome with Functional Single Ventricle: Does Prenatal Diagnosis Improve Survival? Ann. Thorac. Surg., November 1, 2006; 82(5): 1629 - 1636. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. R. Liske, C. S. Greeley, D. J. Law, J. D. Reich, W. R. Morrow, H. S. Baldwin, T. P. Graham, A. W. Strauss, A. L. Kavanaugh-McHugh, and W. F. Walsh Report of the Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Disease Pediatrics, October 1, 2006; 118(4): e1250 - e1256. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K L Brown, D A Ridout, A Hoskote, L Verhulst, M Ricci, and C Bull Delayed diagnosis of congenital heart disease worsens preoperative condition and outcome of surgery in neonates Heart, September 1, 2006; 92(9): 1298 - 1302. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. Espinoza, J. P. Kusanovic, L. F. Goncalves, J. K. Nien, S. Hassan, W. Lee, and R. Romero A Novel Algorithm for Comprehensive Fetal Echocardiography Using 4-Dimensional Ultrasonography and Tomographic Imaging. J. Ultrasound Med., August 1, 2006; 25(8): 947 - 956. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
T.-Y. Hsia and P. J. Gruber Factors Influencing Neurologic Outcome After Neonatal Cardiopulmonary Bypass: What We Can and Cannot Control Ann. Thorac. Surg., June 1, 2006; 81(6): S2381 - S2388. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
N. Sinzobahamvya, J. Photiadis, D. Kumpikaite, C. Fink, H. C. Blaschczok, A. M. Brecher, and B. Asfour Comprehensive aristotle score: implications for the norwood procedure. Ann. Thorac. Surg., May 1, 2006; 81(5): 1794 - 1800. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
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]
|
|
|
|
|
|
W. T. Mahle, K. J. Visconti, M. C. Freier, S. M. Kanne, W. G. Hamilton, A. M. Sharkey, R. E. Chinnock, K. J. Jenkins, P. K. Isquith, T. G. Burns, et al. Relationship of Surgical Approach to Neurodevelopmental Outcomes in Hypoplastic Left Heart Syndrome Pediatrics, January 1, 2006; 117(1): e90 - e97. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Michelfelder, C. Gomez, W. Border, W. Gottliebson, and C. Franklin Predictive Value of Fetal Pulmonary Venous Flow Patterns in Identifying the Need for Atrial Septoplasty in the Newborn With Hypoplastic Left Ventricle Circulation, November 8, 2005; 112(19): 2974 - 2979. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. H. Kaplan, A. M. Ades, and J. Rychik Effect of Prenatal Diagnosis on Outcome in Patients With Congenital Heart Disease NeoReviews, July 1, 2005; 6(7): e326 - e331. [Full Text] [PDF]
|
|
|
|
|
|
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]
|
|
|
|
|
|
B. Khoshnood, C. De Vigan, V. Vodovar, J. Goujard, A. Lhomme, D. Bonnet, and F. Goffinet Trends in Prenatal Diagnosis, Pregnancy Termination, and Perinatal Mortality of Newborns With Congenital Heart Disease in France, 1983-2000: A Population-Based Evaluation Pediatrics, January 1, 2005; 115(1): 95 - 101. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
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]
|
|
|
|
|
|
J. M. Bartlett, D. Wypij, D. C. Bellinger, L. A. Rappaport, L. J. Heffner, R. A. Jonas, and J. W. Newburger Effect of Prenatal Diagnosis on Outcomes in D-Transposition of the Great Arteries Pediatrics, April 1, 2004; 113(4): e335 - e340. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. S. Sklansky, G. R. DeVore, and P. C. Wong Real-time 3-Dimensional Fetal Echocardiography With an Instantaneous Volume-Rendered Display: Early Description and Pictorial Essay J. Ultrasound Med., February 1, 2004; 23(2): 283 - 289. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. W. Gaynor, W. T. Mahle, M. I. Cohen, R. F. Ittenbach, W. M. DeCampli, J. M. Steven, S. C. Nicolson, and T. L. Spray Risk factors for mortality after the Norwood procedure Eur. J. Cardiothorac. Surg., July 1, 2002; 22(1): 82 - 89. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
I D Sullivan Prenatal diagnosis of structural heart disease: does it make a difference to survival? Arch. Dis. Child. Fetal Neonatal Ed., July 1, 2002; 87(1): F19 - 20. [Full Text] [PDF]
|
|
|
|
|
|
I D Sullivan Prenatal diagnosis of structural heart disease: does it make a difference to survival? Heart, May 1, 2002; 87(5): 405 - 406. [Full Text] [PDF]
|
|
