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Hypoplastic Left Heart Syndrome: Natural and Surgical History

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Fetal and Hybrid Procedures in Congenital Heart Diseases

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Abstract

The spectrum of left heart hypoplasia has its extremes from a diminutive left ventricle and mitral/aortic atresia at one end to those classified as hypoplastic left heart complex [1–3] where pre- and postnatal interventions might achieve a biventricular circulation. This chapter is focused on the understanding of hypoplastic left heart syndrome (HLHS) as a lesion with the morphology and physiology of a severely underdeveloped left ventricle and outflow as the dominant findings.

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References

  1. Tchervenkov CI, Jacobs JP, Weinberg PM, Aiello VD, Béland MJ, Colan SD, Elliott MJ, Franklin RCG, Gaynor JW, Krogmann ON, Kurosawa H, Maruszewski B, Stellin G. The nomenclature, definition and classification of hypoplastic left heart syndrome. Cardiol Young. 2006;16:339–68.

    Article  PubMed  Google Scholar 

  2. Hickey EJ, Caldarone CA, McCrindle BW. Left ventricular hypoplasia. J Am Coll Cardiol. 2012;59:S43–54.

    Article  PubMed  Google Scholar 

  3. Kearney DL. The pathological spectrum of left-ventricular hypoplasia. Semin Cardiothorac Vasc Anesth. 2013;17:105–16.

    Article  PubMed  Google Scholar 

  4. Egbe A, Uppu S, Lee S, Stroustrup A, Ho D, Srivastava S. Temporal variation of birth prevalence of congenital heart disease in the United States. Congenit Heart Dis. 2015;10:43–50.

    Article  PubMed  Google Scholar 

  5. Yagel S, Weissman A, Rotstein Z, Manor M, Hegesh J, Anteby E, Lipitz S, Achiron R. Congenital heart defects: natural course and in utero development. Circulation. 1997;96:550–5.

    Article  CAS  PubMed  Google Scholar 

  6. Cole CR, Eghtesady P. The myocardial and coronary histopathology and pathogenesis of hypoplastic left heart syndrome. Cardiol Young. 2016;26(1):19–29.

    Google Scholar 

  7. Gardiner HM. Response of the fetal heart to changes in load: from hyperplasia to heart failure. Heart. 2005;91:871–3.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Taketazu M, Barrea C, Smallhorn JF, Wilson GJ, Hornberger LK. Intrauterine pulmonary venous flow and restrictive foramen ovale in fetal hypoplastic left heart syndrome. J Antimicrob Chemother. 2004;43:1902–7.

    Google Scholar 

  9. Vlahos AP, Lock JE, McElhinney DB, van der Velde ME. Hypoplastic left heart syndrome with intact or highly restrictive atrial septum: outcome after neonatal transcatheter atrial septostomy. Circulation. 2004;109:2326–30.

    Article  PubMed  Google Scholar 

  10. Hoque T, Richmond M, Vincent JA, Bacha E, Torres A. Current outcomes of hypoplastic left heart syndrome with restrictive atrial septum: a single-center experience. Pediatr Cardiol. 2013;34:1181–9.

    Article  PubMed  Google Scholar 

  11. Sharpe GL, Larsson KS. Studies on closure of the ductus arteriosus. X. In vivo effect of prostaglandin. Prostaglandins. 1975;9:703–19.

    Article  CAS  PubMed  Google Scholar 

  12. Graham TP, Atwood GF, Boucek RJ. Pharmacologic dilatation of the ductus arteriosus with prostaglandin E1 in infants with congenital heart disease. South Med J. 1978;71:1238–46.

    Article  PubMed  Google Scholar 

  13. Olley PM, Coceani F, Bodach E. E-type prostaglandins: a new emergency therapy for certain cyanotic congenital heart malformations. Circulation. 1976;53:728–31.

    Article  CAS  PubMed  Google Scholar 

  14. Heymann MA, Berman W, Rudolph AM, Whitman V. Dilatation of the ductus arteriosus by prostaglandin E1 in aortic arch abnormalities. Circulation. 1979;59:169–73.

    Article  CAS  PubMed  Google Scholar 

  15. Lang P, Freed MD, Rosenthal A, Castaneda AR, Nadas AS. The use of prostaglandin E1 in an infant with interruption of the aortic arch. J Pediatr. 1977;91:805–7.

    Article  CAS  PubMed  Google Scholar 

  16. Yabek SM, Mann JS. Prostaglandin E1 infusion in the hypoplastic left heart syndrome. Chest. 1979;76:330–1.

    Article  CAS  PubMed  Google Scholar 

  17. Norwood WI, Kirklin JK, Sanders SP. Hypoplastic left heart syndrome: experience with palliative surgery. Am J Cardiol. 1980;45:87–91.

    Article  CAS  PubMed  Google Scholar 

  18. Norwood WI, Lang P, Casteneda AR, Campbell DN. Experience with operations for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 1981;82:511–9.

    CAS  PubMed  Google Scholar 

  19. Norwood WI, Lang P, Hansen DD. Physiologic repair of aortic atresia-hypoplastic left heart syndrome. N Engl J Med. 1983;308:23–6.

    Article  CAS  PubMed  Google Scholar 

  20. Pigott JD, Murphy JD, Barber G, Norwood WI. Palliative reconstructive surgery for hypoplastic left heart syndrome. Ann Thorac Surg. 1988;45:122–8.

    Article  CAS  PubMed  Google Scholar 

  21. Norwood WI. Hypoplastic left heart syndrome. Ann Thorac Surg. 1991;52:688–95.

    Article  PubMed  Google Scholar 

  22. Sano S, Ishino K, Kawada M, Arai S, Kasahara S, Asai T, Masuda Z-I, Takeuchi M, Ohtsuki S-I. Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2003;126:504–9; discussion 509–10.

    Article  PubMed  Google Scholar 

  23. Sano S, Ishino K, Kawada M, Honjo O. Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2004;7:22–31.

    Article  PubMed  Google Scholar 

  24. Pizarro C, Malec E, Maher KO, Januszewska K, Gidding SS, Murdison KA, Baffa JM, Norwood WI. Right ventricle to pulmonary artery conduit improves outcome after stage I Norwood for hypoplastic left heart syndrome. Circulation. 2003;108 Suppl 1:II155–60. Lippincott Williams & Wilkins.

    PubMed  Google Scholar 

  25. Mair R, Tulzer G, Sames E, Gitter R, Lechner E, Steiner J, Hofer A, Geiselseder G, Gross C. Right ventricular to pulmonary artery conduit instead of modified Blalock-Taussig shunt improves postoperative hemodynamics in newborns after the norwood operation. J Thorac Cardiovasc Surg. 2003;126:1378–84.

    Article  PubMed  Google Scholar 

  26. Mahle WT, Cuadrado AR, Tam VKH. Early experience with a modified Norwood procedure using right ventricle to pulmonary artery conduit. Ann Thorac Surg. 2003;76:1084–8; discussion 1089.

    Article  PubMed  Google Scholar 

  27. Tabbutt S, Dominguez TE, Ravishankar C, Marino BS, Gruber PJ, Wernovsky G, Gaynor JW, Nicolson SC, Spray TL. Outcomes after the stage I reconstruction comparing the right ventricular to pulmonary artery conduit with the modified Blalock Taussig shunt. Ann Thorac Surg. 2005;80:1582–90; discussion 1590–1.

    Article  PubMed  Google Scholar 

  28. Azakie A, Martinez D, Sapru A, Fineman J, Teitel D, Karl TR. Impact of right ventricle to pulmonary artery conduit on outcome of the modified Norwood procedure. Ann Thorac Surg. 2004;77:1727–33.

    Article  PubMed  Google Scholar 

  29. 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 LL, Pizarro C, Kanter KR, Jaggers J, Jacobs JP, Krawczeski CD, Pike N, McCrindle BW, Virzi L, Gaynor JW. Pediatric Heart Network Investigators. Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med. 2010;362:1980–92.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  30. Kolcz J, Skladzien T, Kordon Z, Rudzinski A, Skalski J. Impact of right ventricle-pulmonary artery conduit placement on pulmonary artery development after the Norwood procedure in hypoplastic left heart syndrome. Eur J Cardiothorac Surg. 2012;42:218–23; discussion 223–4. Oxford University Press.

    Article  PubMed  Google Scholar 

  31. Frommelt PC, Sheridan DC, Mussatto KA, Hoffman GM, Ghanayem NS, Frommelt MA, Tweddell JS. Effect of shunt type on echocardiographic indices after initial palliations for hypoplastic left heart syndrome: Blalock-Taussig shunt versus right ventricle-pulmonary artery conduit. J Am Soc Echocardiogr. 2007;20:1364–73.

    Article  PubMed  Google Scholar 

  32. Ghanayem NS, Jaquiss RDB, Cava JR, Frommelt PC, Mussatto KA, Hoffman GM, Tweddell JS. Right ventricle–to–pulmonary artery conduit versus blalock-taussig shunt: a hemodynamic comparison. Ann Thorac Surg. 2006;82:1603–10.

    Article  PubMed  Google Scholar 

  33. Ohye RG, Ludomirsky A, Devaney EJ, Bove EL. Comparison of right ventricle to pulmonary artery conduit and modified Blalock-Taussig shunt hemodynamics after the Norwood operation. Ann Thorac Surg. 2004;78:1090–3.

    Article  PubMed  Google Scholar 

  34. Bartram U, Grünenfelder J, Van Praagh R. Causes of death after the modified Norwood procedure: a study of 122 postmortem cases. Ann Thorac Surg. 1997;64:1795–802.

    Article  CAS  PubMed  Google Scholar 

  35. Nathan M, Williamson AK, Mayer JE, Bacha EA, Juraszek AL. Mortality in hypoplastic left heart syndrome: Review of 216 autopsy cases of aortic atresia with attention to coronary artery disease. J Thorac Cardiovasc Surg. 2012;144:1301–6.

    Article  PubMed  Google Scholar 

  36. Sharma V, Deo SV, Huebner M, Dearani JA, Burkhart HM. In search of the ideal pulmonary blood source for the Norwood procedure: a meta-analysis and systematic review. Ann Thorac Surg. 2014;98:142–50.

    Article  PubMed  Google Scholar 

  37. Bautista-Hernandez V, Brown DW, Loyola H, Myers PO, Borisuk M, del Nido PJ, Baird CW. Mechanisms of tricuspid regurgitation in patients with hypoplastic left heart syndrome undergoing tricuspid valvuloplasty. – PubMed – NCBI. J Thorac Cardiovasc Surg. 2014;148:832–40.

    Article  PubMed  Google Scholar 

  38. Ruzmetov M, Welke KF, Geiss DM, Fortuna RS. Outcomes of tricuspid valve repair in children with hypoplastic left heart syndrome. J Card Surg. 2014;29:698–704.

    Article  PubMed  Google Scholar 

  39. Fraser CD, Mee RB. Modified Norwood procedure for hypoplastic left heart syndrome. Ann Thorac Surg. 1995;60:S546–9.

    Article  PubMed  Google Scholar 

  40. Mery CM, Guzmán-Pruneda FA, Carberry KE, Watrin CH, McChesney GR, Chan JG, Adachi I, Heinle JS, McKenzie ED, Fraser CD. Aortic arch advancement for aortic coarctation and hypoplastic aortic arch in neonates and infants. Ann Thorac Surg. 2014;98:625–33.

    Article  PubMed  Google Scholar 

  41. Gibbs JL, Wren C, Watterson KG, Hunter S, Hamilton JR. Stenting of the arterial duct combined with banding of the pulmonary arteries and atrial septectomy or septostomy: a new approach to palliation for the hypoplastic left heart syndrome. Br Heart J. 1993;69:551–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  42. Akintuerk H, Michel-Behnke I, Valeske K, Mueller M, Thul J, Bauer J, Hagel K-J, Kreuder J, Vogt P, Schranz D. Stenting of the arterial duct and banding of the pulmonary arteries: basis for combined Norwood stage I and II repair in hypoplastic left heart. Circulation. 2002;105:1099–103.

    Article  PubMed  Google Scholar 

  43. Akintürk H, Michel-Behnke I, Valeske K, Mueller M, Thul J, Bauer J, Hagel K-J, Schranz D. Hybrid transcatheter-surgical palliation: basis for univentricular or biventricular repair: the Giessen experience. Pediatr Cardiol. 2007;28:79–87.

    Article  PubMed  Google Scholar 

  44. Michel-Behnke I, Akintuerk H, Marquardt I, Mueller M, Thul J, Bauer J, Hagel KJ, Kreuder J, Vogt P, Schranz D. Stenting of the ductus arteriosus and banding of the pulmonary arteries: basis for various surgical strategies in newborns with multiple left heart obstructive lesions. Heart. 2003;89:645–50.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  45. Galantowicz M, Cheatham JP, Phillips A, Cua CL, Hoffman TM, Hill SL, Rodeman R. Hybrid approach for hypoplastic left heart syndrome: intermediate results after the learning curve. Ann Thorac Surg. 2008;85:2063–70; discussion 2070–1.

    Article  PubMed  Google Scholar 

  46. Galantowicz M, Cheatham JP. Lessons learned from the development of a New hybrid strategy for the management of hypoplastic left heart syndrome. Pediatr Cardiol. 2005;26:190–9.

    Article  Google Scholar 

  47. Caldarone CA, Benson LN, Holtby H, Van Arsdell GS. Main pulmonary artery to innominate artery shunt during hybrid palliation of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2005;130:e1–2.

    Article  PubMed  Google Scholar 

  48. Caldarone CA, Benson L, Holtby H, Li J, Redington AN, Van Arsdell GS. Initial experience with hybrid palliation for neonates with single-ventricle physiology. Ann Thorac Surg. 2007;84:1294–300.

    Article  PubMed  Google Scholar 

  49. Brescia AA, Jureidini S, Danon S, Armbrecht E, Fiore AC, Huddleston CB. Hybrid versus Norwood procedure for hypoplastic left heart syndrome: contemporary series from a single center. J Thorac Cardiovasc Surg. 2014;147:1777–82.

    Article  PubMed  Google Scholar 

  50. Bacha EA. Individualized approach in the management of patients with hypoplastic left heart syndrome (HLHS). Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2013;16:3–6.

    Article  PubMed  Google Scholar 

  51. Pizarro C, Davies RR, Woodford E, Radtke WA. Improving early outcomes following hybrid procedure for patients with single ventricle and systemic outflow obstruction: defining risk factors. Eur J Cardiothorac Surg. 2015;47:995–1001.

    Article  PubMed  Google Scholar 

  52. Malik S, Bird TM, Jaquiss RDB, Morrow WR, Robbins JM. Comparison of in-hospital and longer-term outcomes of hybrid and Norwood stage 1 palliation of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2015;150:474–80.e2.

    Article  PubMed  Google Scholar 

  53. Schranz D, Bauer A, Reich B, Steinbrenner B, Recla S, Schmidt D, Apitz C, Thul J, Valeske K, Bauer J, Müller M, Jux C, Michel-Behnke I, Akintürk H. Fifteen-year single center experience with the ‘Giessen Hybrid’ approach for hypoplastic left heart and variants: current strategies and outcomes. Pediatr Cardiol. 2015;36:365–73. Springer US.

    Article  PubMed  Google Scholar 

  54. Ghanayem NS, Hoffman GM, Mussatto KA, Cava JR, Frommelt PC, Rudd NA, Steltzer MM, Bevandic SM, Frisbee SS, Jaquiss RDB, Litwin SB, Tweddell JS. Home surveillance program prevents interstage mortality after the Norwood procedure. J Thorac Cardiovasc Surg. 2003;126:1367–77.

    Article  CAS  PubMed  Google Scholar 

  55. Ghanayem NS, Cava JR, Jaquiss RDB, Tweddell JS. Home monitoring of infants after stage one palliation for hypoplastic left heart syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2004;7:32–8.

    Article  PubMed  Google Scholar 

  56. Knirsch W, Bertholdt S, Stoffel G, Stiasny B, Weber R, Dave H, Prêtre R, von Rhein M, Kretschmar O. Clinical course and interstage monitoring after the Norwood and hybrid procedures for hypoplastic left heart syndrome. Pediatr Cardiol. 2014;35:851–6. Springer US.

    Article  PubMed  Google Scholar 

  57. Hansen JH, Furck AK, Petko C, Buchholz-Berdau R, Voges I, Scheewe J, Rickers C, Kramer H-H. Use of surveillance criteria reduces interstage mortality after the Norwood operation for hypoplastic left heart syndrome. Eur J Cardiothorac Surg. 2012;41:1013–8. Oxford University Press.

    Article  PubMed  Google Scholar 

  58. Dobrolet NC, Nieves JA, Welch EM, Khan D, Rossi AF, Burke RP, Zahn EM. New approach to interstage care for palliated high-risk patients with congenital heart disease. J Thorac Cardiovasc Surg. 2011;142:855–60.

    Article  PubMed  Google Scholar 

  59. Miller-Tate H, Stewart J, Allen R, Husain N, Rosen K, Cheatham JP, Galantowicz M, Cua CL. Interstage weight gain for patients with hypoplastic left heart syndrome undergoing the hybrid procedure. Congenit Heart Dis. 2013;8:228–33.

    Article  PubMed  Google Scholar 

  60. Emani SM, McElhinney DB, Tworetzky W, Myers PO, Schroeder B, Zurakowski D, Pigula FA, Marx GR, Lock JE, del Nido PJ. Staged left ventricular recruitment after single-ventricle palliation in patients with borderline left heart hypoplasia. J Am Coll Cardiol. 2012;60:1966–74.

    Article  PubMed  Google Scholar 

  61. Bailey LL. Role of cardiac replacement in the neonate. J Heart Transplant. 1985;4:506–9.

    CAS  PubMed  Google Scholar 

  62. Murtuza B, Dedieu N, Vazquez A, Fenton M, Burch M, Hsia T-Y, Tsang VT, Kostolny M. Results of orthotopic heart transplantation for failed palliation of hypoplastic left heart. Eur J Cardiothorac Surg. 2013;43:597–603. Oxford University Press.

    Article  PubMed  Google Scholar 

  63. Hosseinpour A-R, González-Calle A, Adsuar-Gómez A, Cuerpo G, Greco R, Borrego-Domínguez JM, Ordoñez A, Wallwork J. Surgical technique for heart transplantation: a strategy for congenital heart disease. Eur J Cardiothorac Surg. 2013;44:598–604. Oxford University Press.

    Article  PubMed  Google Scholar 

  64. Murtuza B, Elliott MJ. Changing attitudes to the management of hypoplastic left heart syndrome: a European perspective. Cardiol Young. 2011;21 Suppl 2:148–58.

    Article  PubMed  Google Scholar 

  65. Mavroudis C, Mavroudis CD, Farrell RM, Jacobs ML, Jacobs JP, Kodish ED. Informed consent, bioethical equipoise, and hypoplastic left heart syndrome. Cardiol Young. 2011;21 Suppl 2:133–40.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Pediatric Heart Center Vienna, Vienna, Austria

    Ina Michel-Behnke

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  1. Ina Michel-Behnke
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Correspondence to Ina Michel-Behnke .

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Editors and Affiliations

  1. IRCCS Policlinico San Donato, San Donato Milanese, Italy

    Gianfranco Butera

  2. Pediatric Cardiology, Nationwide Children's Hospital, Columbus, Ohio, USA

    John Cheatham

  3. Instituto Dante Pazzanese de Cardiologia and Hospital do Coração, São Paulo, SP, Brazil, Sao Paulo, Brazil

    Carlos AC Pedra

  4. Giessen Hospital, Giessen, Hessen, Germany

    Dietmar Schranz

  5. Linz University, Linz, Austria

    Gerald Tulzer

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Michel-Behnke, I. (2016). Hypoplastic Left Heart Syndrome: Natural and Surgical History. In: Butera, G., Cheatham, J., Pedra, C., Schranz, D., Tulzer, G. (eds) Fetal and Hybrid Procedures in Congenital Heart Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-40088-4_25

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  • DOI: https://doi.org/10.1007/978-3-319-40088-4_25

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