Neonates with hypoplastic left heart syndrome and their fate after staged palliation
Keywords:
Hypoplastic left heart syndrome, HLHS, Norwood procedure, Glenn procedure, Fontan procedure
Abstract
Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect in which the left side of the heart is severely underdeveloped. Etiology of this condition remains unknown. After the labour, three-stage cardio-surgical treatment is necessary, however all of the procedures run a significant risk of complications.
Depending on the general condition of the newborn, the first operation – Norwood procedure – may be carried out in different modifications. Complications are most common after this stage of palliation, and may include morbidities of both cardiogenic and non-cardiogenic etiology. First procedure is also characterized by the highest mortality rate. It is followed by Glenn procedure, usually performed on patients at the age of 3 to 6 months. Finally, HLHS patients undergo the Fontan operation, which is a method used commonly in cardiosurgical management of many single-ventricle lesions. A heart transplantation is an alternative method of treatment.
Development of cardiac surgery significantly increased survival rate of children born with congenital heart defects, hypoplastic left heart syndrome among them. Nowadays, a high percentage of those patients may have hope to reach adulthood. Nevertheless, the surgical treatment still bears a great risk of complications and continually is considered to be mere palliation, as HLHS remains a lethal condition.
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References
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18. Pundi KN, Johnson JN, Dearani JA, Pundi KN, Li Z, Hinck CA, Dahl SH, Cannon BC, O’Leary PW, Driscoll DJ, Cetta F; 40-Year Follow-Up After the Fontan Operation, Long-Term Outcomes of 1,052 Patients; Journal of the American College of Cardiology; 2015 Oct 13;66(15):1700-10. doi: 10.1016/j.jacc.2015.07.065. [PubMed]
2. Feinstein JA, Benson DW, Dubin MA, Cohen MS, Maxey DM, Mahle WT, Pahl E, Villafane J, Bhatt AB, Peng LF, Johnson BA, Marsden AL, Daniels CJ, Rudd NA, Caldarone CA, Mussatto KA, Morales DL, Ivy DD, Gaynor JW, Tweddell JS, Deal BJ, Furck AK, Rosenthal GL, Ohye RG, Ghanayem NS, Cheatham JP, Tworetzky W, Martin GR; Hypoplastic Left Heart Syndrome, Current Considerations and Expectations; Journal of the American College of Cardiology; 2012 Jan 3;59(1 Suppl):S1-42. doi: 10.1016/j.jacc.2011.09.022. [PubMed]
3. Murtuza B, Dedieu N, Vazquez A, Fenton M, Burch M, Hsia TY, Tsang VT, Kostolny M; Results of orthotopic heart transplantation for failed palliation of hypoplastic left heart; European Journal of Cardio-Thoracic Surgery; 2013 Mar;43(3):597-603. doi: 10.1093/ejcts/ezs326 [PubMed]
4. Kanter KR, Mahle WT, Vincent RN, Berg AM, Kogon BE, Kirshbom PM; Heart Transplantation in Children With a Fontan Procedure; The Society of Thoracic Surgeons Published by Elsevier Inc; 2011 Mar;91(3); 823-830. doi: 10.1016/j.athoracsur.2010.11.031 [PubMed]
5. Chrisant MR, Naftel DC, Drummond-Webb J, Chinnock R, Canter CE, Boucek MM et al.; Fate of infants with hypoplastic left heart syndrome listed for cardiac transplantation: a multicenter study; J Heart Lung Transplant; 2005 May;24(5):576-82. doi: 10.1016/j.healun.2004.01.019 [PubMed]
6. Tabbutt S, Ghanayem N, Ravishankar C, Sleeper LA, Cooper DS, Frank DU, Lu M, Pizarro C, Frommelt P, Goldberg CS, Graham EM, Krawczeski CD, Lai WW, Lewis A, Kirsh JA, Mahony L, Ohye RG, Simsic J, Lodge AJ, Spurrier E, Stylianou M, Laussen P; Risk factors for hospital morbidity and mortality after the Norwood procedure: A report from the Pediatric Heart Network Single Ventricle Reconstruction trial; The Journal of Thoracic and Cardiovascular Surgery; 2012 Oct; 144(4): 882–895. doi: 10.1016/j.jtcvs.2012.05.019.
7. Ghanayem NS, Hoffman GM, Mussatto KA, Frommelt MA, Cava JR, Mitchell ME, Tweddell JS; Perioperative monitoring in high-risk infants after stage 1 palliation of univentricular congenital heart disease; The Journal of Thoracic and Cardiovascular Surgery; 2010 Oct;140(4):857-63. doi: 10.1016/j.jtcvs.2010.05.002.
8. Tweddell JS, Sleeper LA, Ohye RG, Williams IA, Mahony L, Pizarro C, Pemberton VL, Frommelt PC, Bradley SM, Cnota JF, Hirsch J, Kirshbom PM, Li JS, Pike N, Puchalski M, Ravishankar C, Jacobs JP, Laussen PC, McCrindle BW; Intermediate-term mortality and cardiac transplantation in infants with single-ventricle lesions: Risk factors and their interaction with shunt type; The Journal of Thoracic and Cardiovascular Surgery; 2012 Jul; 144(1): 152–159. doi: 10.1016/j.jtcvs.2012.01.016.
9. Ohye RG, Sleeper LA, Mahony L, Newburger JW, Pearson GD, Lu M, Goldberg CS, Tabbutt S, Frommelt PC, Ghanayem NS, Laussen PC, Rhodes JF, Lewis AB, Mital S, Ravishankar C, Williams IA, Dunbar-Masterson C, Atz AM, Colan S, Minich L, Pizarro C, Kanter KR, Jaggers J, Jacobs JP, Krawczeski CD, Pike N, McCrindle BW, Virzi L, Gaynor JW; Comparison of Shunt Types in the Norwood Procedure for Single-Ventricle Lesions; The New England Journal of Medicine; 2010 May 27; 362(21): 1980–1992. doi: 10.1056/NEJMoa0912461.
10. Johnson JN, Jaggers J, Li S, O’Brien SM, Li JS, Jacobs JP, Jacobs ML, Welke KF, Peterson ED, Pasquali SK; Center variation and outcomes associated with delayed sterna closure after stage 1 palliation for hypoplastic left heart syndrome; The Journal of Thoracic and Cardiovascular Surgery; 2010 May;139(5):1205-10. doi: 10.1016/j.jtcvs.2009.11.029.
11. Honjo O, Caldarone CA; Hybrid Palliation for Neonates With Hypoplastic Left Heart Syndrome: Current Strategies and Outcomes; The Korean Society of Cardiology; 2010 Mar; 40(3): 103–111. doi: 10.4070/kcj.2010.40.3.103. [PubMed]
12. Knirsch W, Liamlahi R, Hug MI, Hoop R, von Rhein M, Pretre R, Kretschmar O, Latal B; Mortality and neurodevelopmental outcome at 1 year of age comparing hybrid and Norwood procedures; European Journal of Cardio-Thoracic Surgery; 2012 Jul;42(1):33-9. doi: 10.1093/ejcts/ezr286 [PubMed]
13. Furck AK, Uebing A, Hansen JH, Scheewe J, Jung O, Fischer G, Rickers C, Holland-Letz T, Kramer HH; Outcome of the Norwood operation in patients with hypoplastic left heart syndrome: A 12-year single-center survey; The Journal of Thoracic and Cardiovascular Surgery; 2010 Feb;139(2):359-65. doi: 10.1016/j.jtcvs.2009.07.063.
14. Ghanayem NS, Allen KR, Tabbutt S, Atz AM, Clabby ML, Cooper DS, Eghtesady P, Frommelt PC, Gruber PJ, Hill KD, Kaltman JR, Laussen PC, Lewis AB, Lurito KJ, Minich LL, Ohye RG, Schonbeck JV, Schwartz SM, Singh RK, Goldberg CS; Interstage mortality after the Norwood procedure: Results of the multicenter Single Ventricle Reconstruction trial; The Journal of Thoracic and Cardiovascular Surgery; 2012 Oct;144(4):896-906. doi: 10.1016/j.jtcvs.2012.05.020.
15. Friedland-Little JM, Aiyagari R, Yu S, Donohue JE, Hirsch-Romano JC; Survival Through Staged Palliation: Fate of Infants Supported by Extracorporeal Membrane Oxygenation After the Norwood Operation; The Society of Thoracic Surgeons Published by Elsevier Inc; 2014 Feb;97(2):659-65. doi: 10.1016/j.athoracsur.2013.10.066.
16. d’Udekem Y, Iyengar AJ, Galati JC, Forsdick V, Weintraub RG, Wheaton GR, Bullock A, Justo RN, Grigg LE, Sholler GF, Hope S, Radford DJ, Gentles TL, Celermajer DS, Winlaw DS; Redefining Expectations of Long-Term Survival After the Fontan Procedure, Twenty-Five Years of Follow-Up From the Entire Population of Australia and New Zealand; American Heart Association; 2014 Sep 9;130(11 Suppl 1):S32-8. doi: 10.1161/CIRCULATIONAHA.113.007764. [PubMed]
17. Rogers LS, Glatz AC, Ravishankar C, Spray TL, Nicolson SC, Rychik J, Rush CH, Gaynor JW, Goldberg DJ; 18 Years of the Fontan Operation at a Single Institution, Results From 771 Consecutive Patients; Journal of the American College of Cardiology; 2012; 012 Sep 11;60(11):1018-25. doi: 10.1016/j.jacc.2012.05.010
18. Pundi KN, Johnson JN, Dearani JA, Pundi KN, Li Z, Hinck CA, Dahl SH, Cannon BC, O’Leary PW, Driscoll DJ, Cetta F; 40-Year Follow-Up After the Fontan Operation, Long-Term Outcomes of 1,052 Patients; Journal of the American College of Cardiology; 2015 Oct 13;66(15):1700-10. doi: 10.1016/j.jacc.2015.07.065. [PubMed]
CITATION
DOI: 10.17511/ijpr.2017.i05.02
Published: 2017-05-31
How to Cite
Dr. Baran K., Dr. Gilewska J., Dr. Gałuszka M., Dr. Surmiak P., & Dr. Baumert M. (2017). Neonates with hypoplastic left heart syndrome and their fate after staged palliation. Pediatric Review: International Journal of Pediatric Research, 4(5), 295-302. https://doi.org/10.17511/ijpr.2017.i05.02
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Review Article
Copyright (c) 2017 Author (s). Published by Siddharth Health Research and Social Welfare Society
This work is licensed under a Creative Commons Attribution 4.0 International License.
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