Ann Thorac Surg 2008;86:1049-1051. doi:10.1016/j.athoracsur.2007.05.001
© 2008 The Society of Thoracic Surgeons
Key References
Hypoplastic Left Heart Syndrome
Marshall L. Jacobs, MD*
Section of Cardiothoracic Surgery, St. Christopher's Hospital for Children, Philadelphia, Pennsylvania
* Address correspondence to Dr Jacobs, Section of Cardiothoracic Surgery, St. Christopher's Hospital for Children, Erie Ave at Front St, Philadelphia, PA 19134-1095 (Email: marshall.jacobs{at}tenethealth.com).
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Selected References Through 1996
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- 1 Penkoske PA. Hypoplastic left heart syndrome. Ann Thorac Surg 1997;63(1):285-6.
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Anatomy, Embryology, and Physiology
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- 2 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.
- 3 Minich LL, Tani LY, Hawkins JA, Shaddy RE. Possibility of postnatal left ventricular growth in selected infants with non-apex-forming left ventricles. Am Heart J 1997;133:570-4.
- 4 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.
- 5 Mahle WT, Rychik J, Weinberg PM, Cohen MS. Growth characteristics of the aortic arch after the Norwood operation. J Am Coll Cardiol 1998;32:1951-4.
- 6 Rychik J, Rome JJ, Collins MH, DeCampli WM, Spray TL. The hypoplastic left heart syndrome with intact atrial septum; atrial morphology, pulmonary vascular histopathology and outcome. J Am Coll Cardiol 1999;34:554-60.
- 7 Tchervenkov CI, Jacobs ML, Tahta SA. Congenital Heart Surgery Nomenclature and Database Project: hypoplastic left heart syndrome. Ann Thorac Surg 2000;69(Suppl 4):S170-9.
- 8 Migliavacca F, Pennati G, Dubini G, et al. Modeling of the Norwood circulation: effects of shunt size, vascular resistance and heart rate. Am J Physiol Heart Circ Physiol 2001;280:H2076-86.
- 9 Makikallio K, McElhinney DB, Levine JC, et al. Fetal aortic valve stenosis and the evolution of hypoplastic left heart syndrome: patient selection for fetal intervention. Circulation 2006;113:1401-5.
- 10 Cardis BM, Fyfe DA, Mahle WT. Elastic properties of the reconstructed aorta in hypoplastic left heart syndrome. Ann Thorac Surg 2006;81:988-91.
- 11 Tchervenkov CI, Jacobs JP, Weinberg PM, et al. The nomenclature, definition and classification of hypoplastic left heart syndrome. Cardiol Young 2006;16:339-68.
- 12 Griselli M, McGuirk SP, Ofoe V, et al. Fate of pulmonary arteries following Norwood procedure. Eur J Cardiothorac Surg 2006;30:930-5.
- 13 Shillingford AJ, Ittenbach RF, Marino BS, et al. Aortic morphometry and microcephaly in hypoplastic left heart syndrome. Cardiol Young 2007;5:1-7.
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Surgical Techniques and Innovations
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- 14 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.
- 15 Reyes A 2nd, Bove EL, Mosca RS, Kulik TJ, Ludomirsky A. Tricuspid valve repair in children with hypoplastic left heart syndrome during staged surgical reconstruction. Circulation 1997:96(Suppl 9):II-341–3; discussion II-344–5.
- 16 Tchervenkov CI, Tahta SA, Jutras LC, Beland MJ. Biventricular repair in neonates with hypoplastic left heart complex. Ann Thorac Surg 1998;66:1350-7.
- 17 Vricella LA, Razzouk AJ, del Rio M, Gundry SR, Bailey LL. Heart transplantation for hypoplastic left heart syndrome: modified technique for reducing circulatory arrest time. J Heart Lung Transplant 1998;17:1167-71.
- 18 Serraf A, Piot JD, Bonnet N, et al. Biventricular repair approach in ducto-dependent neonates with hypoplastic but morphologically normal left ventricle. J Am Coll Cardiol 1999;33:827-34.
- 19 Pigula FA, Siewers RD, Nemoto EM. Regional perfusion of the brain during neonatal aortic reconstruction. J Thorac Cardiovasc Surg 1999;117:1023-4.
- 20 Ishino K, Stumper O, DeGiovanni JJ. The modified Norwood procedure for hypoplastic left heart syndrome: early to intermediate results of 120 patients with particular reference to aortic arch repair. J Thorac Cardiovasc Surg 1999;117:920-30.
- 21 Imoto Y, Kado H, Shiokawa Y, Fukae K, Yasui H. Norwood procedure without circulatory arrest. Ann Thorac Surg 1999;68:559-61.
- 22 Douglas WI, Goldberg CS, Mosca RS, Law IH, Bove EL. Hemi-Fontan procedure for hypoplastic left heart syndrome: outcome and suitability for Fontan. Ann Thorac Surg 1999;68:1361-7; discussion 1368.
- 23 Kishimoto H, Kawahira Y, Kawata H, Miura T, Iwai S, Mori T. The modified Norwood palliation on a beating heart. J Thorac Cardiovasc Surg 1999;118:1130-2.
- 24 Shiraishi I, Yamagishi M, Oka T, Kawakita A, Hamaoka K. Intraoperative balloon angioplasty for aortic coarctation after Norwood operation. Ann Thorac Surg 2000;70:289-91.
- 25 Jacobs ML. Aortic reconstruction in hypoplastic left heart syndrome: a reappraisal. J Thorac Cardiovasc Surg 2000;120:872-4.
- 26 Dandolu BR, Jacobs ML. Recent advances in reconstructive surgical management of hypoplastic left heart syndrome. Adv Card Surg 2001;13:21-60.
- 27 Pizarro C, Davis DA, Healy RM, Kerins PJ, Norwood WI. Is there a role for extracorporeal life support after stage I Norwood? Eur J Cardiothorac Surg 2001;19:294-301.
- 28 Pigula FA, Gandhi SK, Siewers RD, Davis PJ, Webber SA, Nemoto EM Regional low-flow perfusion provides somatic circulatory support during neonatal aortic arch surgery. Ann Thorac Surg 2001;72:401-6; discussion 406-7.
- 29 Imoto Y, Kado H, Shiokawa Y, Minami K, Yasui H. Experience with the Norwood procedure without circulatory arrest. J Thorac Cardiovasc Surg 2001;122:879-82.
- 30 Ishizaka T, Ohye RG, Suzuki T, Devaney EJ, Bove EL. Bilateral pulmonary artery banding for resuscitation in hypoplastic left heart syndrome. Ann Thorac Surg 2003;75:277-9.
- 31 Pizarro C, Norwood WI. Pulmonary artery banding before Norwood procedure. Ann Thorac Surg 2003;75:1008-10.
- 32 Sano S, Ishino K, Kawada M, et al. 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.
- 33 Pizarro C, Malec E, Maher KO, et al. Right ventricle to pulmonary artery conduit improves outcome after stage I Norwood for hypoplastic left heart syndrome. Circulation 2003;108(Suppl 1):II155-60.
- 34 Sano S, Ishino K, Kawada M, Yoshizumi K, Takeuchi M, Ohtsuki S. Experience over five years using a shunt placed between the right ventricle and the pulmonary arteries during initial reconstruction of hypoplasia of the left heart. Cardiol Young 2004;3(Suppl 14):90-5.
- 35 Ohye RG, Gomez CA, Goldberg CS, Graves HL, Devaney EJ, Bove EL. Tricuspid valve repair in hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 2004;127:465-72.
- 36 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.
- 37 Meyer SR, Campbell PM, Rutledge JM, et al. Use of an allograft patch in repair of hypoplastic left heart syndrome may complicate future transplantation. Eur J Cardiothorac Surg. 2005;27:554-60.
- 38 Galantowicz M, Cheatham JP. Lesions learned from the development of a new hybrid strategy for the management of hypoplastic left heart syndrome. Pediatr Cardiol 2005;26:190-9.
- 39 Cardis BM, Fyfe DA, Ketchum D, Mahle WT. Echocardiographic features and complications of the modified Norwood operation using the right ventricle to pulmonary artery conduit. J Am Soc Echocardiogr 2005;18:660-5.
- 40 Januszewska K, Kolcz J, Mroczek T, Procelewska M, Malec E. Right ventricle-to-pulmonary artery shunt and modified Blalock-Taussig shunt in preparation to hemi-Fontan procedure in children with hypoplastic left heart syndrome. Eur J Cardiothorac Surg 2005;27:956-61.
- 41 Dent CL, Spaeth JP, Jones BV, et al. Brain magnetic resonance imaging abnormalities after the Norwood procedure using regional cerebral perfusion. J Thorac Cardiovasc Surg 2005;130:1523-30.
- 42 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;131:163-71.e2.
- 43 Reinhartz O, Reddy VM, Petrossian E, et al. Homograft valved right ventricle to pulmonary artery conduit as a modification of the Norwood procedure. Circulation 2006;114(Suppl 1):I594-9.
- 44 Wilkins-Haug LE, Tworetzky W, Benson CB, Marshall AC, Jennings RW, Lock JE. Factors affecting technical success of fetal aortic valve dilation. Ultrasound Obstet Gynecol 2006;28:47-52.
- 45 Ghanayem NJ, Jaquiss RD, Cava JR, et al. Right ventricle-to-pulmonary artery conduit versus Blalock-Taussig shunt: a hemodynamic comparison. Ann Thorac Surg 2006;82:1603-9; discussion 1609-10.
- 46 Visconti KJ, Rimmer D, Gauvreau K, et al. Regional low-flow perfusion versus circulatory arrest in neonates: one-year neurodevelopment outcome. Ann Thorac Surg 2006;82:2207-11; discussion 2211-3.
- 47 Jacobs JP, Quintessenza JA, Chai PJ, et al. Rescue cardiac transplantation for failing staged palliation in patients with hypoplastic left heart syndrome. Cardiol Young 2006;16:556-62.
- 48 Graham EM, Atz AM, Bradley SM, et al. Does a ventriculotomy have deleterious effects following palliation in the Norwood procedure using a shunt placed from the right ventricle to the pulmonary arteries? Cardiol Young 2007;17:145-50.
- 49 Ohye RG, Devaney EJ, Hirsch JC, Bove EL. The modified Blalock-Taussig shunt versus the right ventricle-to-pulmonary artery conduit for the Norwood procedure. Pediatr Cardiol 2007;28:122-5.
- 50 Goldberg CS, Bove EL, Devaney EJ, 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 2007;133:880-7.
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Perioperative Management, Monitoring, and Investigations
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- 51 Riordan CJ, Randsbaek F, Storey JH, Montgomery WD, Santamore WP, Austin EH 3rd. Inotropes in the hypoplastic left ventricle syndrome: effects in an animal model. Ann Thorac Surg 1996;62(1):83-90.
- 52 Riordan CJ, Locher JP Jr, Santamore WP, Villafane J, Austin EH 3rd. Monitoring systemic venous oxygen saturations in the hypoplastic left heart syndrome. Ann Thorac Surg 1997;63(3):835-7.
- 53 Barnea O, Santamore WP, Rossi A, Salloum E, Chien S, Austin EH. Estimation of oxygen delivery in newborns with a univentricular circulation. Circulation 1998;98:1407-13.
- 54 Chessa M, Dindar A, Vettukattil JJ, et al. Balloon angioplasty in infants with aortic obstruction after the modified stage I Norwood procedure. Am Heart J 2000;140:227-31.
- 55 Hoffman GM, Ghanayem NS, Kampine JM, et al. Venous saturation and the anaerobic threshold in neonates after the Norwood procedure for hypoplastic left heart syndrome. Ann Thorac Surg 2000;70:1515-20; discussion 1521.
- 56 Charpie JR, Dekeon MK, Goldberg CS, Mosca RS, Bove EL, Kulik TJ. Postoperative hemodynamics after Norwood palliation for hypoplastic left heart syndrome. Am J Cardiol 2001;87:198-202.
- 57 Tabbutt S, Ramamoorthy C, Montenegro LM, et al. Impact of inspired gas mixtures on preoperative infants with hypoplastic left heart syndrome during controlled ventilation. Circulation 2001;104(12 Suppl 1):I159-64.
- 58 Bradley WM, Simsic JM, Atz AM. Hemodynamic effects of inspired carbon dioxide after the Norwood procedure. Ann Thorac Surg 2001;72:2088-93; discussion 2093-4.
- 59 Maher KO, Pizarro C, Gidding SS, et al. Hemodynamic profile after the Norwood procedure with right ventricle to pulmonary artery conduit. Circulation 2003;108:782-4.
- 60 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.
- 61 Ungerleider RM, Shen I, Yeh T, et al. Routine mechanical ventricular assist following the Norwood procedure-improved neurologic outcome and excellent hospital survival. Ann Thorac Surg 2004;77:18-22.
- 62 Wright GE, Crowley DC, Charpie JR, Ohye RG, Bove EL, Kulik TJ. High systemic vascular resistance and sudden cardiovascular collapse in recovering Norwood patients. Ann Thorac Surg. 2004;77:48-52.
- 63 Bradley SM, Atz AM, Simsic JM. Redefining the impact of oxygen and hyperventilation after the Norwood procedure. J Thorac Cardiovasc Surg 2004;127:473-80.
- 64 Hoffman GM, Tweddell JS, Ghanayem NS, et al. Alteration of the critical arteriovenous oxygen saturation relationship by sustained afterload reduction after the Norwood procedure. J Thorac Cardiovasc Surg 2004;127:738-45.
- 65 Zeltser I, Menteer J, Gaynor JW, et al. Impact of re-coarctation following the Norwood operation on survival in the balloon angioplasty era. J Am Coll Cardiol 2005;45:1844-8.
- 66 Ghanayem NS, Tweddell JS, Hoffman GM, Mussatto K, Jaquiss RD. Optimal timing of the second stage of palliation for hypoplastic left heart syndrome facilitated through home monitoring and the results of early cavopulmonary anastomosis. Cardiol Young 2006;16(Suppl 1):61-6.
- 67 Gossett JG, Rocchini AP, Lloyd TR, Graziano NJ. Catheter-based decompression of the left atrium in patients with hypoplastic left heart syndrome and restrictive atrial septum is safe and effective. Catheter Cardiovasc Interv 2006;67:619-24.
- 68 Ravishankar C, Dominguez TE, Kreutzer J, et al. Extracorporeal membrane oxygenation after stage I reconstruction for hypoplastic left heart syndrome. Pediatr Crit Care Med 2006;7:319-23.
- 69 Li J, Zhang G, McCrindle BW, Holtby H, et al. Profiles of hemodynamics and oxygen transport derived by using continuous measured oxygen consumption after the Norwood procedure. J Thorac Cardiovasc Surg 2007;133:441-8.
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Outcome Studies
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- 70 Forbess JM, Cook N, Serraf A, Burke RP, Mayer JE Jr, Jonas RA. An institutional experience with second- and third-stage palliative procedures for hypoplastic left heart syndrome: the impact of the bidirectional cavopulmonary shunt. J Am Coll Cardiol 1997;29:665-70.
- 71 Reddy VM, McElhinney DB, Moore P, Haas GS, Hanley FL. Outcomes after bidirectional cavopulmonary shunt in infants less than 6 months old. J Am Coll Cardiol 1997;29:1365-70.
- 72 Joshi VM, Carey A, Simpson P, Paridon SM. Exercise performance following repair of hypoplastic left heart syndrome: A comparison with other types of Fontan patients. Pediatr Cardiol 1997;18:357-60.
- 73 Johnston JK, Chinnock RE, Zuppan CW, Razzouk AJ, Gundry SR, Bailey LL. Limitations to survival for infants with hypoplastic left heart syndrome before and after transplant: the Loma Linda experience. J Transpl Coord 1997;7:180-4; quiz 185-6.
- 74 Bartram U, Grunenfelder 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.
- 75 Kern JH, Hinton VJ, Nereo NE, Hayes CJ, Gersony WM. Early developmental outcome after the Norwood procedure for hypoplastic left heart syndrome. Pediatrics 1998;102:1148-52.
- 76 Kuhn MA, Larsen RL, Mulla NF, Johnston JK, Chinnock RE, Bailey LL. Outcome of infants with hypoplastic left heart syndrome who undergo atrial septostomy before heart transplantation. Am J Cardiol 2000;85:124-7; A9.
- 77 Mahle WT, Clancy RR, Moss EM, Gerdes M, Jobes DR, Wernovsky G. Neurodevelopmental outcome and lifestyle assessment in school-aged and adolescent children with hypoplastic left heart syndrome. Pediatrics 2000;105:1082-9.
- 78 Mosca RS, Kulik TJ, Goldberg CS, et al. Early results of the Fontan procedure in one hundred consecutive patients with hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 2000;119:1110-8.
- 79 Jenkins PC, Flanagan MF, Jenkins KJ, et al. Survival analysis and risk factors for mortality in transplantation and staged surgery for hypoplastic left heart syndrome. J Am Coll Cardiol 2000;36:1178-85.
- 80 Mahle WT, Spray TL, Wernovsky G, Gaynor JW, Clark BJ 3rd. Survival after reconstructive surgery for hypoplastic left heart syndrome: a 15-year experience from a single institution. Circulation 2000;102(19 Suppl 3):III136-41.
- 81 Goldberg CS, Schwartz EM, Brunberg JA, et al. Neurodevelopmental outcome of patients after the Fontan operation: a comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions. J Pediatr 2000;137:646-52.
- 82 Mahle WT, Clancy RR, McGaurn SP, Goin JE, Clark BJ. Impact of prenatal diagnosis on survival and early neurologic morbidity in neonates with the hypoplastic left heart syndrome. Pediatrics 2001;107:1277-82.
- 83 Clancy RR, McGaurn SA, Goin JE, et al. Allopurinol neurocardiac protection trial in infants undergoing heart surgery using deep hypothermic circulatory arrest. Pediatrics 2001;108:61-70.
- 84 Gaynor JW, Bridges ND, Cohen MI, et al. Predictors of outcome after the Fontan operation: is hypoplastic left heart syndrome still a risk factor? J Thorac Cardiovasc Surg 2002;123:237-45.
- 85 Tweddell JS, Hoffman GM, Mussatto KA, et al. Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: lessons learned from 115 consecutive patients. Circulation 2002;106(12 Suppl 1):I82-9.
- 86 Ikle L, Hale K, Fashaw L, Boucek M, Rosenberg AA. Developmental outcome of patients with hypoplastic left heart syndrome treated with heart transplantation. J Pediatr 2003;142:20-5.
- 87 Raja R, Johnston JK, Fitts JA, Bailey LL, Chinnock RE, Ashwal S. Post-transplant seizures in infants with hypoplastic left heart syndrome. Pediatr Neurol 2003;28:370-8.
- 88 Cohen MS, Marino BS, McElhinney DB, et al. Neo-aortic root dilation and valve regurgitation up to 21 years after staged reconstruction for hypoplastic left heart syndrome. J Am Coll Cardiol 2003;42:533-40.
- 89 Chrisant MR, Naftel DC, Drummond-Webb J, et al. Pediatric Heart Transplant Study Group. Fate of infants with hypoplastic left heart syndrome listed for cardiac transplantation: a multicenter study. J Heart Lung Transplant 2005;24:576-82.
- 90 Simsic JM, Bradley SM, Stroud MR, Atz AM. Risk factors for interstage death after the Norwood procedure. Pediatr Cardiol 2005;26:400-3.
- 91 Hoffman GM, Mussatto KA, Brosig CL, et al. Systemic venous oxygen saturation after the Norwood procedure and childhood neurodevelopmental outcome. J Thorac Cardiovasc Surg 2005;130:1094-100.
- 92 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.
- 93 Mahle WT, Visconti KJ, Freier MC, et al. Relationship of surgical approach to neurodevelopmental outcomes in hypoplastic left heart syndrome. Pediatrics 2006;117:e90-7.
- 94 McGuirk SP, Griselli M, Stumper OF, et al. Staged surgical management of hypoplastic left heart syndrome: a single institution 12 year experience. Heart 2006;92:364-70.
- 95 McGuirk SP, Stickley J, Griselli M, et al. Risk assessment and early outcome following the Norwood procedure for hypoplastic left heart syndrome. Eur J Cardiothorac Surg 2006;29:675-81.
- 96 Dent CL, Spaeth JP, Jones BV, et al. Brain magnetic resonance imaging abnormalities after the Norwood procedure using regional cerebral perfusion. J Thorac Cardiovasc Surg 2006;131:190-7 Retraction in: J Thorac Cardiovasc Surg 2006;131:1226.
- 97 Artrip JH, Campbell DN, Ivy DD, et al. Birth weight and complexity are significant factors for the management of hypoplastic left heart syndrome. Ann Thorac Surg 2006;82:1252-7; discussion 1258-9.
Top
Selected References Through 1996
Anatomy, Embryology, and...