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Review

Current evidence for pharmacologic therapy following stage 1 palliation for single ventricle congenital heart disease

, &
Pages 627-636 | Received 10 Dec 2021, Accepted 15 Jul 2022, Published online: 20 Jul 2022

References

  • Ohye RG, Schranz D, D’Udekem Y. Current therapy for hypoplastic left heart syndrome and related single ventricle lesions. Circulation. 2016;134(17):1265–1279.
  • Schilling C, Dalziel K, Nunn R, et al. The Fontan epidemic: population projections from the Australia and New Zealand Fontan registry. Int J Cardiol. 2016;219:14–19.
  • Hirsch JC, Goldberg C, Bove EL, et al. Fontan operation in the current era: a 15-year single institution experience. Ann Surg. 2008;248(3):402–410.
  • Ghanayem NS, Allen KR, Tabbutt S, et al. Interstage mortality after the Norwood procedure: results of the multicenter Single Ventricle Reconstruction trial. J Thorac Cardiovasc Surg. 2012;144(4):896–906.
  • Gewillig M. The Fontan circulation. Heart. 2005;91(6):839–846.
  • Cross RR, Harahsheh AS, McCarter R, et al. Collaborative (NPC-QIC NPCQI. Identified mortality risk factors associated with presentation, initial hospitalisation, and interstage period for the Norwood operation in a multi-centre registry: a report from the National Pediatric Cardiology-Quality Improvement Collaborative. Cardiol Young. 2014;24(2):253–262.
  • Simsic JM, Bradley SM, Stroud MR, et al. Risk factors for interstage death after the Norwood procedure. Pediatr Cardiol. 2005;26(4):400–403.
  • Hehir DA, Dominguez TE, Ballweg JA, et al. Risk factors for interstage death after stage 1 reconstruction of hypoplastic left heart syndrome and variants. J Thorac Cardiovasc Surg. 2008;136(1):94–99. e3.
  • Rudd NA, Ghanayem NS, Hill GD, et al. Interstage home monitoring for infants with single ventricle heart disease: education and management: a scientific statement from the American Heart Association. J Am Heart Assoc. 2020;9(16):e014548.
  • Schidlow DN, Anderson JB, Klitzner TS, et al. Variation in interstage outpatient care after the Norwood procedure: a report from the joint council on congenital heart disease national quality improvement collaborative. Congenit Heart Dis. 2011;6(2):98–107.
  • Kaltman JR, Andropoulos DB, Checchia PA, et al. Report of the pediatric heart network and national heart, lung, and blood institute working group on the perioperative management of congenital heart disease. Circulation. 2010;121(25):2766–2772.
  • Anderson JB, Brown DW, Lihn S, et al. Power of a learning network in congenital heart disease. World J Pediatr Congenit Heart Surg. 2019;10(1):66–71.
  • National Pediatric Cardiology Quality Improvement Collaborative. http://npcqic.org. Accessed 2021 Dec 9.
  • Anderson JB, Brown DW, Horsley M, et al. Reduction in interstage growth failure in infants with hypoplastic left heart syndrome and impact on stage 2 outcomes: a report from the national pediatric cardiology quality improvement collaborative. J Am Coll Cardiol. 2017;69(11):584.
  • Tweddell JS, Hoffman GM, Fedderly RT, et al. Phenoxybenzamine improves systemic oxygen delivery after the Norwood procedure. Ann Thorac Surg. 1999;67(1):161–167.
  • Guzzetta NA. Phenoxybenzamine in the treatment of hypoplastic left heart syndrome: a core review. Anesth Analg. 2007;105(2):312–315.
  • Mills KI, Kaza AK, Walsh BK, et al. Phosphodiesterase inhibitor‐based vasodilation improves oxygen delivery and clinical outcomes following stage 1 palliation. J Am Heart Assoc. 2016;5(11):e003554.
  • 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(3):738–745.
  • Hoffman GM, Niebler RA, Scott JP, et al. Interventions associated with treatment of low cardiac output after stage 1 Norwood palliation. Ann Thorac Surg. 2021;111(5):1620–1627.
  • Brown NJ, Vaughan DE. Angiotensin-converting enzyme inhibitors. Circulation. 1998;97(14):1411–1420.
  • Mori Y, Nakazawa M, Tomimatsu H, et al. Long-term effect of angiotensin-converting enzyme inhibitor in volume overloaded heart during growth: a controlled pilot study. J Am Coll Cardiol. 2000;36(1):270–275.
  • Calabrò R, Pisacane C, Pacileo G, et al. Hemodynamic effects of a single oral dose of enalapril among children with asymptomatic chronic mitral regurgitation. Am Heart J. 1999;138(5):955–961.
  • Shaw NJ, Wilson N, Dickinson DF. Captopril in heart failure secondary to a left to right shunt. Arch Dis Child. 1988;63(4):360–363.
  • Balaguru D, Artman M, Auslender M. Management of heart failure in children. Curr Probl Pediatr. 2000;30(1):5–30.
  • Group CTS. Effects of enalapril on mortality in severe congestive heart failure. N Engl J Med. 1987;316(23):1429–1435.
  • Garg R, Yusuf S; for the Collaborative Group on ACE Inhibitor Trials. Overview of randomized trials of angiotensin-converting enzyme inhibitors on mortality and morbidity in patients with heart failure. Jama. 1995;273(18):1450–1456.
  • Hsu DT, Zak V, Mahony L, et al., Enalapril in infants with single ventricle: results of a multicenter randomized trial. Circulation. 122(4): 333–340. 2010.
  • Yimgang DP, Sorkin JD, Evans CF, et al. Angiotensin converting enzyme inhibitors and interstage failure in infants with hypoplastic left heart syndrome. Congenit Heart Dis. 2018;13(4):533–540.
  • Zak V, Hsu DT, Pemberton VL, et al. Translating clinical trials into clinical practice: a survey assessing the potential impact of the pediatric heart network infant single ventricle trial. Cardiol Young. 2017;27(7):1265–1270.
  • Hansen JE, Brown DW, Hanke SP, et al., Angiotensin 2010; converting enzyme inhibitor prescription for patients with single ventricle physiology enrolled in the NPC 2010;QIC registry. J Am Heart Assoc. 9(10): e014823. 2020.
  • Mathur K, Hsu DT, Lamour JM, et al. Safety of enalapril in infants: data from the pediatric heart network infant single ventricle trial. J Pediatr. 2020;227:218–223.
  • Mital S, Chung WK, Colan SD, et al. Pediatric Heart Network Investigators. Renin-angiotensin-aldosterone genotype influences ventricular remodeling in infants with single ventricle. Circulation. 2011;123(21):2353–2362.
  • Jain S, Vaidyanathan B. Digoxin in management of heart failure in children: should it be continued or relegated to the history books? Ann Pediatr Cardiol. 2009;2(2):149.
  • Uretsky BF, Young JB, Shahidi FE, et al. Randomized study assessing the effect of digoxin withdrawal in patients with mild to moderate chronic congestive heart failure: results of the PROVED trial. J Am Coll Cardiol. 1993;22(4):955–962.
  • Group DI. The effect of digoxin on mortality and morbidity in patients with heart failure. N Engl J Med. 1997;336(8):525–533.
  • Ziff OJ, Lane DA, and Samra M, et al. Safety and efficacy of digoxin: systematic review and meta-analysis of observational and controlled trial data. BMJ. 2015;351:h4451.
  • Rathore SS, Curtis JP, Wang Y, et al. Association of serum digoxin concentration and outcomes in patients with heart failure. Jama. 2003;289(7):871–878.
  • Kimball TR, Daniels SR, Meyer RA, et al. Effect of digoxin on contractility and symptoms in infants with a large ventricular septal defect. Am J Cardiol. 1991;68(13):1377–1382.
  • Berman JW, Yabek SM, Dillon T, et al. Effects of digoxin in infants with a congested circulatory state due to a ventricular septal defect. N Engl J Med. 1983;308(7):363–366.
  • Seguchi M, Nakazawa M, Momma K. Further evidence suggesting a limited role of digitalis in infants with circulatory congestion secondary to large ventricular septal defect. Am J Cardiol. 1999;83(9):1408–1411.
  • O’Byrne ML, Song L, Huang J, et al. Trends in discharge prescription of digoxin after Norwood operation: an analysis of data from the Pediatric Health Information System (PHIS) database. Pediatr Cardiol. 2021;42(4):793–803.
  • Brown DW, Mangeot C, Anderson JB, et al. Digoxin use is associated with reduced interstage mortality in patients with no history of arrhythmia after stage I palliation for single ventricle heart disease. J Am Heart Assoc. 2016;5(1):e002376.
  • Oster ME, Kelleman M, McCracken C, et al. Association of digoxin with interstage mortality: results from the pediatric heart network single ventricle reconstruction trial public use dataset. J Am Heart Assoc. 2016;5(1):e002566.
  • Truong DT, Menon SC, Lambert LM, et al., Digoxin use in infants with single ventricle physiology: secondary analysis of the pediatric heart network infant single ventricle trial public use dataset. Pediatr Cardiol. 39(6): 1200–1209. 2018.
  • National Pediatric Cardiology Quality Improvement Collaborative. Phase II: measure Definitions. NPCQIC Reporting - Measure Definitions, Cincinnati Children’s Hospital Medical Center, 2018, reports.npcqic.org/reports/phase2/measure_definitions.xhtml.
  • Simsic JM, Cuadrado A, Kirshbom PM, et al. Novel management strategy for severe cyanosis after Sano modification of the Norwood procedure. J Thorac Cardiovasc Surg. 2005;129(6):1450–1451.
  • Kumar KR, Flair A, Thompson EJ, et al. Association between digoxin use and cardiac function in infants with single-ventricle congenital heart disease during the interstage period. Pediatr Crit Care Med. 2022Apr11; 23(6)453–463. published online ahead of print.
  • Bristow MR. Mechanism of action of beta-blocking agents in heart failure. Am J Cardiol. 1997;80(11A):26L–40L.
  • Shaddy RE, Boucek MM, Hsu DT, et al., Carvedilol for children and adolescents with heart failure: a randomized controlled trial. JAMA. 298(10): 1171–1179. 2007.
  • Miyamoto SD, Stauffer BL, Polk J, et al. Gene expression and β-adrenergic signaling are altered in hypoplastic left heart syndrome. J Heart Lung Transplant. 2014;33(8):785–793.
  • Szwarc E, Rodrigues MH, Pedra SF, et al. Use of beta-blockers in the post-operative period of Sano modification of the Norwood procedure with persistent cyanosis. Arq Bras Cardiol. 2007;88(6):e182–e184.
  • Mienert T, Esmaeili A, Steinbrenner B, et al. Cardiovascular drug therapy during interstage after hybrid approach: a single-center experience in 51 newborns with hypoplastic left heart. Pediatr Drugs. 2021;23(2):195–202.
  • Oster ME, Chen S, Dagincourt N, et al. Development and impact of arrhythmias after the Norwood procedure: a report from the Pediatric Heart Network. J Thorac Cardiovasc Surg. 2017;153(3):638–645. e2.
  • Moffett BS, Mattamal R, Ocampo EC, et al. Impact of pharmacotherapy on interstage outcomes in single ventricle infants. Congenit Heart Dis. 2011;6(4):286–293.
  • Ghelani SJ, Spurney CF, Martin GR, et al. Impact of pharmacotherapy on interstage mortality and weight gain in children with single ventricle. Congenit Heart Dis. 2013;8(3):219–227.
  • Agarwal A, Firdouse M, Brar N, et al. Incidence and management of thrombotic and thromboembolic complications following the Norwood procedure: a systematic review. Clin Appl Thromb. 2017;23(8):911–921.
  • White MH, Kelleman M, Sidonio RF, et al. Incidence and timing of thrombosis after the Norwood procedure in the single‐ventricle reconstruction trial. J Am Heart Assoc. 2020;9(24):e015882.
  • Manlhiot C, Brandão LR, Kwok J, et al. Thrombotic Complications and Thromboprophylaxis Across All Three Stages of Single Ventricle Heart Palliation. J Pediatr. https://www.jpeds.com/article/S0022-3476(12)00260-0/fulltext. Accessed 2021 Nov 15.
  • Silvey M, Hall M, Bilynsky E, et al. Increasing rates of thrombosis in children with congenital heart disease undergoing cardiac surgery. Thromb Res. 2018;162:15–21.
  • Gedicke M, Morgan G, Parry A, et al. Risk factors for acute shunt blockage in children after modified Blalock-Taussig shunt operations. Heart Vessels. 2010;25(5):405–409.
  • Do N, Hill KD, Wallace AS, et al. Shunt failure—risk factors and outcomes: an analysis of the society of thoracic surgeons congenital heart surgery database. Ann Thorac Surg. 2018;105(3):857–864.
  • Li JS, Yow E, Berezny KY, et al. Clinical outcomes of palliative surgery including a systemic-to-pulmonary artery shunt in infants with cyanotic congenital heart disease: does aspirin make a difference? Circulation. 2007;116(3):293–297.
  • Giglia TM, Massicotte MP, Tweddell JS, et al. Prevention and treatment of thrombosis in pediatric and congenital heart disease: a scientific statement from the American Heart Association. Circulation. 2013;128(24):2622–2703.
  • Ramachandran P, King E, Nebbia A, et al. Variability of antithrombotics use in patients with hypoplastic left heart syndrome and its variants following first-and second-stage palliation surgery: a national report using the National Pediatric Cardiology Quality Improvement Collaborative registry. Cardiol Young. 2017;27(4):731–738.
  • Truong DT, Johnson JT, Bailly DK, et al. Platelet inhibition in shunted infants on aspirin at short and midterm follow-up. Pediatr Cardiol. 2017;38(2):401–409.
  • Mir A, Frank S, Journeycake J, et al. Aspirin resistance in single-ventricle physiology: aspirin prophylaxis is not adequate to inhibit platelets in the immediate postoperative period. Ann Thorac Surg. 2015;99(6):2158–2164.
  • Emani S, Zurakowski D, Mulone M, et al. Platelet testing to guide aspirin dose adjustment in pediatric patients after cardiac surgery. J Thorac Cardiovasc Surg. 2017;154(5):1723–1730.
  • Saini A, Joshi AD, Cowan KM, et al. High acetylsalicylic acid dosing in infants after modified Blalock–Taussig shunt. Cardiol Young. 2019;29(3):389–397.
  • Wessel DL, Berger F, Li JS, et al. Clopidogrel in infants with systemic-to-pulmonary-artery shunts. N Engl J Med. 2013;368(25):2377–2384.
  • Young G, Lensing AW, Monagle P, et al. Rivaroxaban for treatment of pediatric venous thromboembolism. An Einstein‐Jr phase 3 dose‐exposure‐response evaluation. J Thromb Haemost. 2020;18(7):1672–1685.
  • Heo JH, Rascati KL, Lopez KN, et al. Increased fracture risk with furosemide use in children with congenital heart disease. J Pediatr. 2018;199:92–98.e10.
  • Saarela T, Lanning P, Koivisto M, et al. Nephrocalcinosis in full-term infants receiving furosemide treatment for congestive heart failure: a study of the incidence and 2-year follow up. Eur J Pediatr. 1999;158(8):668–672.
  • Alon US, Scagliotti D, Garola RE. Nephrocalcinosis and nephrolithiasis in infants with congestive heart failure treated with furosemide. J Pediatr. 1994;125(1):149–151.
  • Robertson CM, Alton GY, Bork KT, et al. Bilateral sensory permanent hearing loss after palliative hypoplastic left heart syndrome operation. Ann Thorac Surg. 2012 Apr;93(4):1248–1253.
  • Robertson CMT, Bork KT, Tawfik G. Avoiding furosemide ototoxicity associated with single-ventricle repair in young infants. Pediatr Crit Care Med. 2019;20(4):350–356.
  • Zheng H, Li L, Liang H, et al. Normalization of N-terminal pro-B-type natriuretic peptide after cardiac surgery among children with tetralogy of Fallot. Am J Cardiol. 2018;122(12):2125–2130.
  • Goldberg DJ, French B, McBride MG, et al. Impact of oral sildenafil on exercise performance in children and young adults after the Fontan operation: a randomized, double-blind, placebo-controlled, crossover trial. Circulation. 2011;123(11):1185–1193.
  • Garcia AM, Nakano SJ, Karimpour-Fard A, et al. Phosphodiesterase-5 is elevated in failing single ventricle myocardium and affects cardiomyocyte remodeling in vitro. Circ Heart Fail. 2018 Sep;11(9):e004571.
  • Jeremiasen I, Tran-Lundmark K, Idris N, et al. Pulmonary vasodilator therapy in children with single ventricle physiology: effects on saturation and pulmonary arterial pressure. Pediatr Cardiol. 2020 Dec;41(8):1651–1659.
  • Handler SS, Ogawa MT, Hopper RK, et al. Subcutaneous treprostinil in pediatric patients with failing single-ventricle physiology. J Heart Lung Transplant. 2017. DOI:10.1016/j.healun.2017.09.008
  • van der Pol RJ, Smits MJ, van Wijk MP, et al. Efficacy of proton-pump inhibitors in children with gastroesophageal reflux disease: a systematic review. Pediatrics. 2011;127(5):925–935.
  • Orenstein SR, Hassall E, Furmaga-Jablonska W, et al. Multicenter, double-blind, randomized, placebo-controlled trial assessing the efficacy and safety of proton pump inhibitor lansoprazole in infants with symptoms of gastroesophageal reflux disease. J Pediatr. 2009;154(4):514–520. e4.
  • Wang Y-H, Wintzell V, Ludvigsson JF, et al. Association between proton pump inhibitor use and risk of fracture in children. JAMA Pediatr. 2020;174(6):543–551.
  • Wang YH, Wintzell V, Ludvigsson JF, et al. Association between proton pump inhibitor use and risk of asthma in children. JAMA Pediatr. 2021;175(4):394–403.
  • van der Pol R, Langendam M, Benninga M, et al. Efficacy and safety of histamine-2 receptor antagonists. JAMA Pediatr. 2014;168(10):947–954.
  • Tighe M, Afzal NA, Bevan A, et al. Pharmacological treatment of children with gastro‐oesophageal reflux. Cochrane Database Syst Rev. 2014 11;2016(11). DOI:10.1002/14651858.CD008550.pub2
  • Ohye RG, Schonbeck JV, Eghtesady P, et al. Cause, timing, and location of death in the Single Ventricle Reconstruction trial. J Thorac Cardiovasc Surg. 2012;144(4):907–914.
  • Ahmed H, Anderson JB, Bates KE, et al. Development of a validated risk score for interstage death or transplant after stage I palliation for single-ventricle heart disease. J Thorac Cardiovasc Surg. 2020;160(4):1021–1030.

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