Skip to main content

Advertisement

SpringerLink
Log in

Clinical Outcomes and Risk Factors for In-Hospital Mortality in Neonates with Hypoplastic Left Heart Syndrome

  • Original Article
  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

The objective of this study was to identify patient and hospitalization characteristics associated with in-hospital mortality in infants with hypoplastic left heart syndrome (HLHS). We conducted a retrospective analysis of a large administrative database, the National Inpatient Sample dataset of the Healthcare Cost and Utilization Project for the years 2002–2016. Neonates with HLHS were identified by ICD-9 and ICD-10 codes. Hospital and patient factors associated with inpatient mortality were analyzed. Overall, 18,867 neonates met the criteria of inclusion; a total of 3813 patients died during the hospitalization (20.2%). In-hospital mortality decreased over the years of the study (27.0% in 2002 vs. 18.3% in 2016). Extracorporeal membrane oxygenation utilization was 8.1%. Univariate and multivariate logistic regression analyses were used to identify risk factors for in-hospital mortality in infants with hypoplastic left heart syndrome. Independent non-modifiable risk factors for mortality were birth weight < 2500 g (Adjusted odds ratio (aOR) 2.16 [1.74–2.69]), gestational age < 37 weeks (aOR 1.73 [1.42–2.10]), chromosomal abnormalities (aOR 3.07 [2.60–3.64]) and renal anomalies (aOR 1.34 [1.10–1.61]). Independent modifiable risk factors for mortality were being transferred-in from another hospital (aOR 1.15 [1.03–1.29]), use of extracorporeal membrane oxygenation (aOR 12.74 [10.91–14.88]). Receiving care in a teaching hospital is a modifiable variable, and it decreased the odds of mortality (aOR 0. 78 [0.64–0.95]). In conclusion, chromosomal anomalies, Extra Corporeal Membrane Oxygenation, gestational age < 37 weeks or birth weight < 2500 g were associated with increased odds of mortality. Modifiable variables as receiving care at birth center and in a hospital designated as a teaching hospital decreased the odds of mortality.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

Abbreviations

HLHS:

Hypoplastic left heart disease

ECMO:

Extra Corporeal Membrane Oxygenation

BAS:

Balloon atrial septostomy

G-tube:

Gastrostomy tube

NIS:

National Inpatient Sample

HCUP:

Healthcare Cost and Utilization Project

AHRQ:

Agency for Healthcare Research and Quality

ICD-9&10:

International Classification of Diseases 9 and 10

LOS:

Length of stay

OR:

Odds ratio

CI:

Confidence interval

IQR:

Inter Quartile Range

References

  1. Hoffman JIE, Kaplan S (2002) The incidence of congenital heart disease. J Am Coll Cardiol 39:1890–1900

    Article  Google Scholar 

  2. Jacobs JP, Mayer JE, Mavroudis C et al (2017) The Society of Thoracic Surgeons congenital heart surgery database: 2017 update on outcomes and quality. Ann Thorac Surg 103(3):699–709

    Article  Google Scholar 

  3. Feinstein JA, Benson DW, Dubin AM et al (2012) Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol 59(suppl 1):S1–S42

    Article  Google Scholar 

  4. Oster ME, Lee KA, Honein MA, Riehle-Colarusso T, Shin M, Correa A (2013) Temporal trends in survival among infants with critical congenital heart defects. Pediatrics 131(5):e1502–e1508

    Article  Google Scholar 

  5. Noonan JA, Nadas AS (1958) The hypoplastic left heart syndrome; an analysis of 101 cases. Pediatr Clin North Am 5:1029–1056

    Article  CAS  Google Scholar 

  6. Gutgesell HP, Massaro TA (1995) Management of hypoplastic left heart syndrome in a consortium of university hospitals. Am J Cardiol. 76(11):809–811

    Article  CAS  Google Scholar 

  7. Jacobs ML, Blackstone EH, Bailey LL (1998) Intermediate survival in neonates with aortic atresia: a multi-institutional study: The Congenital Heart Surgeons Society. J Thorac Cardiovasc Surg 116(3):417–431

    Article  CAS  Google Scholar 

  8. Agency for Healthcare Research and Quality. Overview of the National (Nationwide) Inpatient Sample (NIS). Available at https://www.hcupus.ahrq.gov/nisoverview.jsp Accessed 20 Sep 2019.

  9. Siffel C, Riehle-Colarusso T, Oster ME, Correa A (2015) Survival of children with hypoplastic left heart syndrome. Pediatrics 136(4):e864–e870

    Article  Google Scholar 

  10. Zakaria D, Tang X, Bhakta R, ElHassan NO, Prodhan P (2018) Chromosomal abnormalities affect the surgical outcome in infants with hypoplastic left heart syndrome: a large cohort analysis. Pediatr Cardiol 39(1):11–18

    Article  Google Scholar 

  11. Czosek RJ, Anderson JB, Heaton PC et al (2013) Staged palliation of hypoplastic left heart syndrome : trends in mortality, cost, and length of stay using a national database from 2000 through 2009. Am J Cardiol 111:1792–1799

    Article  Google Scholar 

  12. Jacobs JP, Mayer JE, Pasquali SK et al (2019) The society of thoracic surgeons congenital heart surgery database: 2019 update on outcomes and quality. Ann Thorac Surg 107(3):691–704

    Article  Google Scholar 

  13. Tweddell JS, Hoffman GM (2002) Postoperative management in patients with complex congenital heart disease. Pediatr Card Surg Annu 5(1):187–205

    Google Scholar 

  14. Hoffman GM, Ghanayem NS, Scott JP, Tweddell JS, Mitchell ME, Mussatto KA (2017) Postoperative cerebral and somatic near-infrared spectroscopy saturations and outcome in hypoplastic left heart syndrome. Ann Thorac Surg 103(5):1527–1535

    Article  Google Scholar 

  15. Hoffman TM, Wemovsky G, Atz AM et al (2003) Efficacy and safety of milrinone in preventing low cardiac output syndrome in infants and children after corrective surgery for congenital heartdisease. Circulation 107(7):996–1002

    Article  CAS  Google Scholar 

  16. Gaies MG, Gurney JG, Yen AH et al (2010) Vasoactive-inotropic score as a predictor of morbidity and mortality in infants after cardiopulmonary bypass. Pediatr Crit Care Med 11(2):234–238

    Article  Google Scholar 

  17. Cooper DS, Costello JM, Bronicki RA et al (2008) Current challenges in cardiac intensive care: optimal strategies for mechanical ventilation and timing of extubation. Cardiol Young 18(S3):72–83

    Article  Google Scholar 

  18. Schatorjé E, Van Der Flier M, Seppänen M et al (2016) Primary immunodeficiency associated with chromosomal aberration - An ESID survey. Orphanet J Rare Dis 11:110

    Article  Google Scholar 

  19. Shamszad P, Gospin TA, Hong BJ, Mckenzie ED, Petit CJ (2010) Impact of preoperative risk factors on outcomes after Norwood palliation for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 147(3):897–901

    Article  Google Scholar 

  20. Erickson LC, Wise PH, Cook EF, Beiser A, Newburger JW (2004) The impact of managed care insurance on use of lower-mortality hospitals by children undergoing cardiac surgery in California. Pediatrics 105(6):1271–1278

    Article  Google Scholar 

  21. Dean PN, McHugh KE, Conaway MR, Hillman DG, Gutgesell HP (2013) Effects of race, ethnicity, and gender on surgical mortality in hypoplastic left heart syndrome. Pediatr Cardiol 34(8):1829–1836

    Article  Google Scholar 

  22. Peyvandi S, Baer RJ, Moon-Grady AJ et al (2018) Socioeconomic mediators of racial and ethnic disparities in congenital heart disease outcomes: a population-based study in California. J Am Heart Assoc 7(20):1–12

    Article  Google Scholar 

  23. McInturff WD, Neely S (1997) What Americans say about the nation’s medical schools and teaching hospitals. Acad Med 72(2):132–133

    Article  CAS  Google Scholar 

  24. Jenkins KJ, Newburger JW, Lock JE et al (1995) In-hospital mortality for surgical repair of congenital heart defects: pre- liminary observations of variation by hospital caseload. Pediatrics 95:323–330

    CAS  PubMed  Google Scholar 

  25. Sollano JA, Gelijns AC, Moskowitz AJ et al (1999) Volume-outcome relationships in cardiovascular operations: New York State, 1990–1995. J Thorac Cardiovasc Surg 117(419–428):428–430

    Google Scholar 

  26. Bazzani LG, Marcin JP (2007) Case volume and mortality in pediatric cardiac surgery patients in California, 1998–2003. Circulation 115:2652–2659

    Article  Google Scholar 

  27. Pasquali SK, Li JS, Burstein DS et al (2012) Association of center volume with mortality and complications in pediatric heart surgery. Pediatrics 129:e370–e376

    Article  Google Scholar 

  28. Welke KF, O'Brien SM, Peterson ED et al (2009) The complex relationship between pediatric cardiac surgical case volumes and mortality rates in a national clinical database. J Thorac Cardiovasc Surg 137:1133–1140

    Article  Google Scholar 

  29. Yeager SB, Duff J, Greco KM et al (2006) Pretransport and posttransport characteristics and outcomes of neonates who were admitted to a cardiac intensive care unit. Pediatrics 118:1070–1077

    Article  Google Scholar 

  30. Hellström-Westas L, Hanséus K, Jögi P et al (2001) Long-distance transports of newborn infants with congenital heart disease. Pediatr Cardiol 22:380–384

    Article  Google Scholar 

  31. Shi S, Zhao Z, Liu X et al (2008) Perioperative risk factors for prolonged mechanical ventilation following cardiac surgery in neonates and young infants. Chest 134:768–774

    Article  Google Scholar 

  32. Chrisant MRK, Naftel DC, Drummond-Webb J et al (2005) Fate of infants with hypoplastic left heart syndrome listed for cardiac transplantation: a multicenter study. J Hear Lung Transplant 24:576–582

    Article  CAS  Google Scholar 

Download references

Funding

No funding was secured for this study.

Author information

Authors and Affiliations

  1. Department of Pediatric Critical Care, Cleveland Clinic Children’s, 9500 Euclid Ave. M14, Cleveland, OH, 44195, USA

    Mohammed Hamzah & Hemant Agarwal

  2. Department of Pediatrics, Michigan State University/Sparrow Health System, Lansing, MI, USA

    Hasan F. Othman

  3. Department of Neonatology, Cleveland Clinic Children’s, Cleveland, OH, USA

    Esraa Elsamny & Hany Aly

Authors
  1. Mohammed Hamzah
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Hasan F. Othman
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Esraa Elsamny
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Hemant Agarwal
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Hany Aly
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Mohammed Hamzah.

Ethics declarations

Conflict of interest

The authors declared that they have no conflict of interest.

Ethical Approval

This article does not contain any studies with human participants performed by any of the authors. An institutional board review was not required for this project as the use of data from approved public data sets is not considered human subject research.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Hamzah, M., Othman, H.F., Elsamny, E. et al. Clinical Outcomes and Risk Factors for In-Hospital Mortality in Neonates with Hypoplastic Left Heart Syndrome. Pediatr Cardiol 41, 781–788 (2020). https://doi.org/10.1007/s00246-020-02312-3

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-020-02312-3

Keywords

Search

Navigation