Abstract
The objective of this study was to assess outcomes of hypoplastic left heart syndrome (HLHS) patients weighing ≤2.5 kg throughout staged palliation. We performed a single-center retrospective review. Abstracted data included gestational age, birth weight, presence of noncardiac anomalies, and survival through Fontan. Fifty-two patients met inclusion criteria, with a median birth weight of 2.14 kg and gestational age of 36 weeks. Five patients received comfort care only. Of 47 patients who underwent initial surgical palliation, 51% survived to initial hospital discharge. Birth weight and gestational age (GA) were similar between survivors and nonsurvivors. Compared with survivors, risk factors for death prior to initial hospital discharge were as follows: small for GA (P = 0.005), noncardiac anomalies (P = 0.04), need for post-perative extracorporeal membrane oxygenation (P = 0.0004), and conversion from initial palliation to Sano shunt (n = 5, no survivors). Operative survival following Stage 2 palliation was 91% (21/23) and 94% after Fontan (17/18). Overall survival for palliated patients from birth through Fontan was 36%. Low-birth-weight neonates with HLHS have poor overall survival through the Fontan operation, with highest mortality following Stage 1 palliation. Being small for GA and the presence of noncardiac anomalies are important preoperative risk factors for early mortality.
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Ades AM, Dominguez TE, Nicolson SC, Gaynor JW, Spray TL, Wernovsky G, Tabbutt S (2010) Morbidity and mortality after surgery for congenital cardiac disease in the infant born with low weight. Cardiol Young 20:8–17
Archer JM, Yeager SB, Kenny MJ, Soll RF, Horbar JD (2011) Distribution of and mortality from serious congenital heart disease in very low birth weight infants. Pediatrics 127:293–299
Atz AM, Travison TG, Williams IA, Pearson GD, Laussen PC, Mahle WT, Cook AL, Kirsh JA, Sklansky M, Khaikin S, Goldberg C, Frommelt M, Krawczeski C, Puchalski MD, Jacobs JP, Baffa JM, Rychik J, Ohye RG (2010) Prenatal diagnosis and risk factors for preoperative death in neonates with single right ventricle and systemic outflow obstruction: screening data from the Pediatric Heart Network Single Ventricle Reconstruction Trial(*). J Thorac Cardiovasc Surg 140:1245–1250
Azakie T, Merklinger SL, McCrindle BW, Van Arsdell GS, Lee KJ, Benson LN, Coles JG, Williams WG (2001) Evolving strategies and improving outcomes of the modified Norwood procedure: a 10-year single-institution experience. Ann Thorac Surg 72:1349–1353
Bove EL, Lloyd TR (1996) Staged reconstruction for hypoplastic left heart syndrome. Contemporary results. Ann Surg 224:387–394 discussion 394–395
Curzon CL, Milford-Beland S, Li JS, O’Brien SM, Jacobs JP, Jacobs ML, Welke KF, Lodge AJ, Peterson ED, Jaggers J (2008) Cardiac surgery in infants with low birth weight is associated with increased mortality: analysis of the Society of Thoracic Surgeons Congenital Heart Database. J Thorac Cardiovasc Surg 13:546–551
Fenton TR (2003) A new growth chart for preterm babies: Babson and Benda’s chart updated with recent data and a new format. BMC Pediatr 3:13
Forbess JM, Cook N, Roth SJ, Serraf A, Mayer JE Jr, Jonas RA (1995) Ten-year institutional experience with palliative surgery for hypoplastic left heart syndrome. Risk factors related to stage I mortality. Circulation 92:II262–II266
Galantowicz M, Cheatham JP, Phillips A, Cua CL, Hoffman TM, Hill SL, Rodeman R (2008) Hybrid approach for hypoplastic left heart syndrome: intermediate results after the learning curve. Ann Thorac Surg 85:2063–2070 discussion 2070–2071
Gaynor JW, Mahle WT, Cohen MI, Ittenbach RF, DeCampli WM, Steven JM, Nicolson SC, Spray TL (2002) Risk factors for mortality after the Norwood procedure. Eur J Cardiothorac Surg 22:82–89
Hirsch JC, Gurney JG, Donohue JE, Gebremariam A, Bove EL, Ohye RG (2008) Hospital mortality for Norwood and arterial switch operations as a function of institutional volume. Pediatr Cardiol 29:713–717
Mahle WT, Spray TL, Wernovsky G, Gaynor JW, Clark BJ III (2000) Survival after reconstructive surgery for hypoplastic left heart syndrome: a 15-year experience from a single institution. Circulation 102:III136–III141
McGuirk SP, Griselli M, Stumper OF, Rumball EM, Miller P, Dhillon R, de Giovanni JV, Wright JG, Barron DJ, Brawn WJ (2006) Staged surgical management of hypoplastic left heart syndrome: a single institution 12 year experience. Heart 92:364–370
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 (2010) Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med 362(21):1980–1992
Owens GE, Gomez-Fifer C, Gelehrter S, Owens ST (2009) Outcomes for patients with unbalanced atrioventricular septal defects. Pediatr Cardiol 30:431–435
Pizarro C, Davis DA, Galantowicz ME, Munro H, Gidding SS, Norwood WI (2002) Stage I palliation for hypoplastic left heart syndrome in low birth weight neonates: Can we justify it? Eur J Cardiothorac Surg 21:716–720
Pizarro C, Derby CD, Baffa JM, Murdison KA, Radtke WA (2008) Improving the outcome of high-risk neonates with hypoplastic left heart syndrome: Hybrid procedure or conventional surgical palliation? Eur J Cardiothorac Surg 33:613–618
Sano S, Huang SC, Kasahara S, Yoshizumi K, Kotani Y, Ishino K (2009) Risk factors for mortality after the Norwood procedure using right ventricle to pulmonary artery shunt. Ann Thorac Surg 87:178–185 discussion 185:186
Stasik CN, Gelehrter S, Goldberg CS, Bove EL, Devaney EJ, Ohye RG (2006) Current outcomes and risk factors for the Norwood procedure. J Thorac Cardiovasc Surg 131:412–417
Weinstein S, Gaynor JW, Bridges ND, Wernovsky G, Montenegro LM, Godinez RI, Spray TL (1999) Early survival of infants weighing 2.5 kilograms or less undergoing first-stage reconstruction for hypoplastic left heart syndrome. Circulation 100:II167–III170
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Gelehrter, S., Fifer, C.G., Armstrong, A. et al. Outcomes of Hypoplastic Left Heart Syndrome in Low-Birth-Weight Patients. Pediatr Cardiol 32, 1175–1181 (2011). https://doi.org/10.1007/s00246-011-0053-2
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DOI: https://doi.org/10.1007/s00246-011-0053-2