How long do people live with hypoplastic left heart?

1. Report of the New England Regional Infant Cardiac Program. Pediatrics 1980;65:375–461.

2. Samánek M, Slavík Z, Zborilová B, Hrobonová V, Vorísková M, Skovránek J. Prevalence, treatment, and outcome of heart disease in live-born children: a prospective analysis of 91,823 live-born children. Pediatr Cardiol 1989;10:205–11.

   Article  PubMed  Google Scholar 

3. Lev M. Pathologic anatomy and interrelationship of hypoplasia of the aortic tract complexes. Lab Invest 1952;1:61–70.

   CAS  PubMed  Google Scholar 

4. Noonan JA, Nadas AS. The hypoplastic left heart syndrome; an analysis of 101 cases. Pediatr Clin North Am 1958;5:1029–56.

   Article  CAS  PubMed  Google Scholar 

5. Yabek SM, Mann JS. Prostaglandin E1 infusion in the hypoplastic left heart syndrome. Chest 1979;79:330–1.

   Article  Google Scholar 

6. Cayler GG, Smeloff EA, Miller GE Jr . Surgical palliation of hypoplastic left side of the heart. N Engl J Med 1970;282:780–3.

   Article  CAS  PubMed  Google Scholar 

7. Doty DB, Knott HW. Hypoplastic left heart syndrome. Experience with an operation to establish functionally normal circulation. J Thorac Cardiovasc Surg 1977;74:624–30.

   CAS  PubMed  Google Scholar 

8. 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 

9. Blalock A, Taussig H. The Surgical treatment of malformations of the heart in which there is pulmonary stenosis or pulmonary atresia. JAMA 1945;128:189–202.

   Article  Google Scholar 

10. Murphy AM, Cameron DE. The Blalock-Taussig-Thomas collaboration: a model for medical progress. JAMA 2008;300:328–30.

    Article  CAS  PubMed  Google Scholar 

11. Donnelly JP, Raffel DM, Shulkin BL, et al. Resting coronary flow and coronary flow reserve in human infants after repair or palliation of congenital heart defects as measured by positron emission tomography. J Thorac Cardiovasc Surg 1998;115:103–10.

    Article  CAS  PubMed  Google Scholar 

12. 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 

13. 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 

14. 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 

15. Mair R, Tulzer G, Sames E, et al. 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 

16. Frommelt PC, Gerstenberger E, Cnota JF, et al.; Pediatric Heart Network Investigators. Impact of initial shunt type on cardiac size and function in children with single right ventricle anomalies before the Fontan procedure: the single ventricle reconstruction extension trial. J Am Coll Cardiol 2014;64:2026–35.

    Article  PubMed  PubMed Central  Google Scholar 

17. Newburger JW, Sleeper LA, Frommelt PC, et al.; Pediatric Heart Network Investigators. Transplantation-free survival and interventions at 3 years in the single ventricle reconstruction trial. Circulation 2014;129:2013–20.

    Article  PubMed  PubMed Central  Google Scholar 

18. Wilder TJ, McCrindle BW, Phillips AB, et al. Survival and right ventricular performance for matched children after stage-1 Norwood: Modified Blalock-Taussig shunt versus right-ventricle-to-pulmonary-artery conduit. J Thorac Cardiovasc Surg 2015;150:1440–50, 1452.e1–8; discussion 1450–2.

    Article  PubMed  Google Scholar 

19. Ohye RG, Sleeper LA, Mahony L, et al.; 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 

20. Mahle WT, Cuadrado AR, Tam VK. 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 

21. Sano S, Ishino K, Kado H, et al. Outcome of right ventricle-to-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome: a multi-institutional study. Ann Thorac Surg 2004;78:1951–7; discussion 1957–8.

    Article  PubMed  Google Scholar 

22. Tabbutt S, Dominguez TE, Ravishankar C, et al. 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 

23. Sano S, Huang SC, Kasahara S, Yoshizumi K, Kotani Y, Ishino K. Risk factors for mortality after the Norwood procedure using right ventricle to pulmonary artery shunt. Ann Thorac Surg 2009;87:178–85; discussion 185–6.

    Article  PubMed  Google Scholar 

24. Oster ME, Ehrlich A, King E, et al. Association of interstage home monitoring with mortality, readmissions, and weight gain: a multicenter study from the National Pediatric Cardiology Quality Improvement Collaborative. Circulation 2015;132:502–8.

    Article  PubMed  Google Scholar 

25. Jaquiss RD. The single ventricle reconstruction trial: the gift that keeps on giving. J Thorac Cardiovasc Surg 2016;151:676–7.

    Article  PubMed  Google Scholar 

26. 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 

27. Bacha EA, Daves S, Hardin J, et al. Single-ventricle palliation for high-risk neonates: the emergence of an alternative hybrid stage I strategy. J Thorac Cardiovasc Surg 2006;131:163–171.e2.

    Article  PubMed  Google Scholar 

28. Murphy MO, Bellsham-Revell H, Morgan GJ, et al. Hybrid procedure for neonates with hypoplastic left heart syndrome at high-risk for norwood: midterm outcomes. Ann Thorac Surg 2015;100:2286–90; discussion 2291–2.

    Article  PubMed  Google Scholar 

29. Pizarro C, Derby CD, Baffa JM, Murdison KA, Radtke WA. Improving the outcome of high-risk neonates with hypoplastic left heart syndrome: hybrid procedure or conventional surgical palliation? Eur J Cardiothorac Surg 2008;33:613–8.

    Article  PubMed  Google Scholar 

30. Galantowicz M, Cheatham JP, Phillips A, et al. 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 

31. Stoica SC, Philips AB, Egan M, et al. The retrograde aortic arch in the hybrid approach to hypoplastic left heart syndrome. Ann Thorac Surg 2009;88:1939–46; discussion 1946–7.

    Article  PubMed  Google Scholar 

32. Davis CK, Pastuszko P, Lamberti J, Moore J, Hanley F, El Said H. The hybrid procedure for the borderline left ventricle. Cardiol Young 2011;21:26–30.

    Article  PubMed  Google Scholar 

33. Yerebakan C, Murray J, Valeske K, et al. Long-term results of biventricular repair after initial Giessen hybrid approach for hypoplastic left heart variants. J Thorac Cardiovasc Surg 2015;149:1112–20; discussion 1120–2.e2.

    Article  PubMed  Google Scholar 

34. Licht DJ, Shera DM, Clancy RR, et al. Brain maturation is delayed in infants with complex congenital heart defects. J Thorac Cardiovasc Surg 2009;137:529–36; discussion 536–7.

    Article  PubMed  PubMed Central  Google Scholar 

35. Mahle WT, Tavani F, Zimmerman RA, et al. An MRI study of neurological injury before and after congenital heart surgery. Circulation 2002;106(12 Suppl 1):I109–14.

    PubMed  Google Scholar 

36. Yerebakan C, Valeske K, Elmontaser H, et al. Hybrid therapy for hypoplastic left heart syndrome: Myth, alternative, or standard? J Thorac Cardiovasc Surg 2016;151:1112–21, 1123.e1–5.

    Article  PubMed  Google Scholar 

37. Ghanayem NS, Hoffman GM, Mussatto KA, et al. Home surveillance program prevents interstage mortality after the Norwood procedure. J Thorac Cardiovasc Surg 2003;126:1367–77.

    Article  CAS  PubMed  Google Scholar 

38. Ghanayem NS, Allen KR, Tabbutt S, et al.; Pediatric Heart Network Investigators. Interstage mortality after the Norwood procedure: results of the multicenter Single Ventricle Reconstruction trial. J Thorac Cardiovasc Surg 2012;144:896–906.

    Article  PubMed  PubMed Central  Google Scholar 

39. Furck AK, Uebing A, Hansen JH, et al. Outcome of the Norwood operation in patients with hypoplastic left heart syndrome: a 12-year single-center survey. J Thorac Cardiovasc Surg 2010;139:359–65.

    Article  PubMed  Google Scholar 

40. Ohye RG, Schonbeck JV, Eghtesady P, et al.; Pediatric Heart Network Investigators. Cause, timing, and location of death in the Single Ventricle Reconstruction trial. J Thorac Cardiovasc Surg 2012;144:907–14.

    Article  PubMed  PubMed Central  Google Scholar 

41. Cashen K, Gupta P, Lieh-Lai M, Mastropietro C. Infants with single ventricle physiology in the emergency department: are physicians prepared? J Pediatr 2011;159:273–7.e1.

    Article  PubMed  Google Scholar 

42. Rudd NA, Frommelt MA, Tweddell JS, et al. Improving interstage survival after Norwood operation: outcomes from 10 years of home monitoring. J Thorac Cardiovasc Surg 2014;148:1540–7.

    Article  PubMed  Google Scholar 

43. Petit CJ, Fraser CD, Mattamal R, Slesnick TC, Cephus CE, Ocampo EC. The impact of a dedicated single-ventricle home-monitoring program on interstage somatic growth, interstage attrition, and 1-year survival. J Thorac Cardiovasc Surg 2011;142:1358–66.

    Article  PubMed  Google Scholar 

44. Kelleher DK, Laussen P, Teixeira-Pinto A, Duggan C. Growth and correlates of nutritional status among infants with hypoplastic left heart syndrome (HLHS) after stage 1 Norwood procedure. Nutrition 2006;22:237–44.

    Article  CAS  PubMed  Google Scholar 

45. Mondini PG, Carlon CA, de Marchi R. Method of performing anastomosis between the superior vena cava and the right branch of the pulmonary artery; operative technic and experimental research. G Ital Chir 1950;6:775–91.

    CAS  PubMed  Google Scholar 

46. Glenn WW. Circulatory bypass of the right side of the heart. IV. Shunt between superior vena cava and distal right pulmonary artery; report of clinical application. N Engl J Med 1958;259:117–20.

    Article  CAS  PubMed  Google Scholar 

47. Bridges ND, Jonas RA, Mayer JE, Flanagan MF, Keane JF, Castaneda AR. Bidirectional cavopulmonary anastomosis as interim palliation for high-risk Fontan candidates. Early results. Circulation 1990;82(5 Suppl IV): 170–6.

    Google Scholar 

48. Douville EC, Sade RM, Fyfe DA. Hemi-Fontan operation in surgery for single ventricle: a preliminary report. Ann Thorac Surg 1991;51:893–9; discussion 900.

    Article  CAS  PubMed  Google Scholar 

49. Bove EL, Lloyd TR. Staged reconstruction for hypoplastic left heart syndrome. Contemporary results. Ann Surg 1996;224:387–94; discussion 394–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

50. Chang AC, Hanley FL, Wernovsky G, et al. Early bidirectional cavopulmonary shunt in young infants. Postoperative course and early results. Circulation 1993;88(5 Pt 2):II149–58.

    CAS  PubMed  Google Scholar 

51. Petrucci O, Khoury PR, Manning PB, Eghtesady P. Outcomes of the bidirectional Glenn procedure in patients less than 3 months of age. J Thorac Cardiovasc Surg 2010;139:562–8.

    Article  PubMed  Google Scholar 

52. Jaquiss RD, Ghanayem NS, Hoffman GM, et al. Early cavopulmonary anastomosis in very young infants after the Norwood procedure: impact on oxygenation, resource utilization, and mortality. J Thorac Cardiovasc Surg 2004;127:982–9.

    Article  PubMed  Google Scholar 

53. Mahle WT, Cohen MS, Spray TL, Rychik J. Atrioventricular valve regurgitation in patients with single ventricle: impact of the bidirectional cavopulmonary anastomosis. Ann Thorac Surg 2001;72:831–5.

    Article  CAS  PubMed  Google Scholar 

54. Jacobs ML, Rychik J, Rome JJ, et al. Early reduction of the volume work of the single ventricle: the hemi-Fontan operation. Ann Thorac Surg 1996;62:456–61; discussion 461–2.

    Article  CAS  PubMed  Google Scholar 

55. Fogel MA, Rychik J, Vetter J, Donofrio MT, Jacobs M. Effect of volume unloading surgery on coronary flow dynamics in patients with aortic atresia. J Thorac Cardiovasc Surg 1997;113:718–26; discussion 726–7.

    Article  CAS  PubMed  Google Scholar 

56. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax 1971;26:240–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

57. Stamm C, Friehs I, Mayer JE Jr, et al. Long-term results of the lateral tunnel Fontan operation. J Thorac Cardiovasc Surg 2001;121:28–41.

    Article  CAS  PubMed  Google Scholar 

58. Marcelletti C, Corno A, Giannico S, Marino B. Inferior vena cava-pulmonary artery extracardiac conduit. A new form of right heart bypass. J Thorac Cardiovasc Surg 1990;100:228–32.

    CAS  PubMed  Google Scholar 

59. Bridges ND, Mayer JE Jr, Lock JE, et al. Effect of baffle fenestration on outcome of the modified Fontan operation. Circulation 1992;86:1762–9.

    Article  CAS  PubMed  Google Scholar 

60. Lemler MS, Scott WA, Leonard SR, Stromberg D, Ramaciotti C. Fenestration improves clinical outcome of the fontan procedure: a prospective, randomized study. Circulation 2002;105:207–12.

    Article  PubMed  Google Scholar 

61. Hsu DT, Quaegebeur JM, Ing FF, Selber EJ, Lamour JM, Gersony WM. Outcome after the single-stage, nonfenestrated Fontan procedure. Circulation 1997;96(9 Suppl II): 335–40.

    Google Scholar 

62. Khairy P, Fernandes SM, Mayer JE Jr, et al. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation 2008;117:85–92.

    Article  PubMed  Google Scholar 

63. Pundi KN, Johnson JN, Dearani JA, et al. 40-year follow-up after the Fontan operation: long-term outcomes of 1,052 patients. J Am Coll Cardiol 2015;66:1700–10.

    Article  PubMed  Google Scholar 

64. Feinstein JA, Benson DW, Dubin AM, et al. Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol 2012;59(1 Suppl):S1–42.

    Article  PubMed  PubMed Central  Google Scholar 

65. Mastropietro CW, Clark JA, Grimaldi LM, Killinger JS, Richmond M. The patient with a single cardiac ventricle. Curr Pediatr Rev 2012;8:253–76.

    Article  Google Scholar 

66. Maxwell D, Allan L, Tynan MJ. Balloon dilatation of the aortic valve in the fetus: a report of two cases. Br Heart J 1991;65:256–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

67. Moon-Grady AJ, Morris SA, Belfort M, et al.; International Fetal Cardiac Intervention Registry. International fetal cardiac intervention registry: a worldwide collaborative description and preliminary outcomes. J Am Coll Cardiol 2015;66:388–99.

    Article  PubMed  Google Scholar 

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Hypoplastic left heart syndrome. At birth, children with hypoplastic left heart depend upon blood pumped by the right ventricle into the pulmonary artery (PA) to supply blood to the systemic circulation and end organs via a patent ductus arteriosus (DA). In many cases, coarctation of the aorta (CoAo) is also present.

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