Abstract
Infants with hypoplastic left heart syndrome (HLHS) are at risk for interstage morbidity and mortality, especially between the first and second surgical stages after the Norwood and hybrid procedures. This study compared the morbidity and mortality of patients treated by either the Norwood or the hybrid procedure for HLHS between the first and second stages who were undergoing interstage monitoring. Between October 2008 and December 2011, 26 infants (14 boys) with HLHS (n = 16) and other univentricular heart malformations with aortic arch anomaly (n = 10) were scheduled for interstage monitoring after Norwood I (n = 12) and hybrid (n = 14) procedures. Three infants (11.5 %) died after first-stage palliation (one hybrid patient and two Norwood patients), and three infants (11.5 %) died after second-stage palliation (two hybrid patients and one Norwood patient) (p = 0.83), all after early second-stage surgery (<90 days). The Norwood I and hybrid procedures did not differ in terms of overall mortality (23 %) (three hybrid and three Norwood patients; p = 1.00). Seven infants (26.9 %) could not be discharged from the hospital due to hemodynamic instability and were referred for early second-stage surgery (<90 days). After the first stage, the invasive reevaluation rate before discharge was high (53.8 %), with cardiac catheterizations for 8 of 14 patients after the hybrid procedure and for 6 of 12 patients after the Norwood procedure (p = 0.69). A total of 11 reinterventions were performed (eight by catheter and three by surgery). Of the eight catheter reinterventions, five were performed for hybrid patients (p = 0.22). For 14 infants, 89 days (range 10–177 days) of interstage monitoring were scheduled. One infant (3.9 %) died during the interstage monitoring. The findings showed a breach of the physiologic criteria for interstage monitoring in seven infants (50 %) after 10 days (range 4–68 days) (five hybrid and two Norwood patients), leading to rehospitalization and catheterization for six patients (four hybrid and two Norwood patients), requiring interventions for two patients (patent arterial duct stent dilation, and atrial septal defect stenting, all for hybrid patients). Overall, three of the seven patients with red flag events of interstage monitoring were candidates for early second-stage surgery. In conclusion, morbidity among infants treated for HLHS remains high, either before or after hospital discharge, emphasizing the need of interstage monitoring programs. Despite retrograde aortic flow in infants with HLHS after the hybrid procedure, the mortality rate was comparable between the two groups. Mortality occurs after early second-stage surgery (<90 days).
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Akintuerk H, Michel-Behnke I, Valeske K, Mueller M, Thul J, Bauer J, Hagel KJ, Kreuder J, Vogt P, Schranz D (2002) Stenting of the arterial duct and banding of the pulmonary arteries: basis for combined Norwood stage 1 and 2 repair in hypoplastic left heart. Circulation 105:1099–1103
Ashburn DA, McCrindle BW, Tchervenkov CI, Jacobs ML, Lofland GK, Bove EL, Spray TL, Williams WG, Blackstone EH (2003) Outcomes after the Norwood operation in neonates with critical aortic stenosis or aortic valve atresia. J Thorac Cardiovasc Surg 125:1070–1082
Baba K, Kotani Y, Chetan D, Chaturvedi RR, Lee KJ, Benson LN, Grosse-Wortmann L, Van Arsdell GS, Caldarone CA, Honjo O (2012) Hybrid versus Norwood strategies for single-ventricle palliation. Circulation 126:S123–S131
Barron DJ, Kilby MD, Davies B, Wright JG, Jones TJ, Brawn WJ (2009) Hypoplastic left heart syndrome. Lancet 374:551–564
Brown DW, Gauvreau K, Moran AM, Jenkins KJ, Perry SB, del Nido PJ, Colan SD (2003) Clinical outcomes and utility of cardiac catheterization prior to superior cavopulmonary anastomosis. J Thorac Cardiovasc Surg 126:272–281
Cross R, Steury R, Randall A, Fuska M, Sable C (2012) Single-ventricle palliation for high-risk neonates: examining the feasibility of an automated home monitoring system after stage I palliation. Future Cardiol 8:227–235
Dahnert I, Riede FT, Razek V, Weidenbach M, Rastan A, Walther T, Kostelka M (2007) Catheter interventional treatment of Sano shunt obstruction in patients following modified Norwood palliation for hypoplastic left heart syndrome. Clin Res Cardiol 96:719–722
Dobrolet NC, Nieves JA, Welch EM, Khan D, Rossi AF, Burke RP, Zahn EM (2011) New approach to interstage care for palliated high-risk patients with congenital heart disease. J Thorac Cardiovasc Surg 142:855–860
Eicken A, Schreiber C (2006) Stenting of a stenosed Sano shunt after palliation in hypoplastic left heart syndrome. Ann Thorac Surg 82:1168–1169 author reply 1169
Feinstein JA, Benson DW, Dubin AM, 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 (2012) Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol 59:S1–S42
Galantowicz M, Cheatham JP (2005) Lessons learned from the development of a new hybrid strategy for the management of hypoplastic left heart syndrome. Pediatr Cardiol 26:190–199
Ghanayem NS, Hoffman GM, Mussatto KA, Cava JR, Frommelt PC, Rudd NA, Steltzer MM, Bevandic SM, Frisbee SS, Jaquiss RD, Litwin SB, Tweddell JS (2003) Home surveillance program prevents interstage mortality after the Norwood procedure. J Thorac Cardiovasc Surg 126:1367–1377
Ghanayem NS, Cava JR, Jaquiss RD, Tweddell JS (2004) Home monitoring of infants after stage one palliation for hypoplastic left heart syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 7:32–38
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 (2012) Interstage mortality after the Norwood procedure: results of the Multicenter Single-Ventricle Reconstruction Trial. J Thorac Cardiovasc Surg 144:896–906
Hansen JH, Furck AK, Petko C, Buchholz-Berdau R, Voges I, Scheewe J, Rickers C, Kramer HH (2012) Use of surveillance criteria reduces interstage mortality after the Norwood operation for hypoplastic left heart syndrome. Eur J Cardiothorac Surg 41:1013–1018
Hartman DM, Medoff-Cooper B (2012) Transition to home after neonatal surgery for congenital heart disease. MCN Am J Matern Child Nurs 37:95–100
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 102:136–141
McCrossan B, Morgan G, Grant B, Sands AJ, Craig BG, Doherty NN, Agus AM, Crealey GE, Casey FA (2012) A randomised trial of a remote home support programme for infants with major congenital heart disease. Heart 98:1523–1528
Miller-Tate H, Stewart J, Allen R, Husain N, Rosen K, Cheatham JP, Galantowicz M, Cua CL (2012) Interstage weight gain for patients with hypoplastic left heart syndrome undergoing the hybrid procedure. Congenit Heart Dis 8(3):228–233
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 (2010) Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med 362:1980–1992
Petit CJ, Fraser CD, Mattamal R, Slesnick TC, Cephus CE, Ocampo EC (2011) The impact of a dedicated single-ventricle home-monitoring program on interstage somatic growth, interstage attrition, and 1-year survival. J Thorac Cardiovasc Surg 142:1358–1366
Photiadis J, Sinzobahamvya N, Hraska V, Asfour B (2012) Does bilateral pulmonary banding in comparison to Norwood procedure improve outcome in neonates with hypoplastic left heart syndrome beyond second-stage palliation? A review of the current literature. Thorac Cardiovasc Surg 60:181–188
Sarajuuri A, Lonnqvist T, Schmitt F, Almqvist F, Jokinen E (2012) Patients with univentricular heart in early childhood: parenting stress and child behaviour. Acta Paediatr 101:252–257
Wernovsky G, Kuijpers M, Van Rossem MC, Marino BS, Ravishankar C, Dominguez T, Godinez RI, Dodds KM, Ittenbach RF, Nicolson SC, Bird GL, Gaynor JW, Spray TL, Tabbutt S (2007) Postoperative course in the cardiac intensive care unit following the first stage of Norwood reconstruction. Cardiol Young 17:652–665
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Knirsch, W., Bertholdt, S., Stoffel, G. et al. Clinical Course and Interstage Monitoring After the Norwood and Hybrid Procedures for Hypoplastic Left Heart Syndrome . Pediatr Cardiol 35, 851–856 (2014). https://doi.org/10.1007/s00246-014-0865-y
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DOI: https://doi.org/10.1007/s00246-014-0865-y