Hostname: page-component-848d4c4894-5nwft Total loading time: 0 Render date: 2024-06-03T11:28:29.537Z Has data issue: false hasContentIssue false
  • English
  • Français

Performance of a single-ventricle home-monitoring programme: survival and predictors of adverse outcome

Published online by Cambridge University Press:  16 May 2022

Peter J. Lillitos Open the ORCID record for Peter J. Lillitos [Opens in a new window] ,
Leila Rittey ,
Madeleine Vass ,
Gerald Ugwoke ,
Muhammad Akhtar ,
Shuba Barwick ,
Carin Van Doorn ,
Osama Jaber  and
James R. Bentham
Peter J. Lillitos
Affiliation:
Department of Congenital Heart Disease, Leeds Teaching Hospitals NHS Trust, Leeds, UK
Leila Rittey
Affiliation:
Department of Congenital Heart Disease, Leeds Teaching Hospitals NHS Trust, Leeds, UK
Madeleine Vass
Affiliation:
Department of Congenital Heart Disease, Leeds Teaching Hospitals NHS Trust, Leeds, UK
Gerald Ugwoke
Affiliation:
Department of Congenital Heart Disease, Leeds Teaching Hospitals NHS Trust, Leeds, UK
Muhammad Akhtar
Affiliation:
Department of Congenital Heart Disease, Leeds Teaching Hospitals NHS Trust, Leeds, UK
Shuba Barwick
Affiliation:
Department of Congenital Heart Disease, Leeds Teaching Hospitals NHS Trust, Leeds, UK
Carin Van Doorn
Affiliation:
Department of Congenital Heart Disease, Leeds Teaching Hospitals NHS Trust, Leeds, UK
Osama Jaber
Affiliation:
Department of Congenital Heart Disease, Leeds Teaching Hospitals NHS Trust, Leeds, UK
James R. Bentham*
Affiliation:
Department of Congenital Heart Disease, Leeds Teaching Hospitals NHS Trust, Leeds, UK
*
Address for correspondence: Dr J.R. Bentham, Department of Congenital Heart Disease, E Floor Jubilee Wing, Leeds General Infirmary, Great George Street, Leeds LS1 3EX, UK. Tel: +44 (0)113 243 2799. Email: Jamie.bentham@nhs.net
Get access Rights & Permissions [Opens in a new window]

Abstract

Introduction and background:

Mortality between stages 1 and 2 single-ventricle palliation is significant. Home-monitoring programmes are suggested to reduce mortality. Outcomes and risk factors for adverse outcomes for European programmes have not been published.

Aims:

To evaluate the performance of a home-monitoring programme at a medium-sized United Kingdom centre with regards survival and compare performance with other home-monitoring programmes in the literature.

Methods:

All fetal and postnatal diagnosis of a single ventricle were investigated with in-depth analysis of those undergoing stage 1 palliation and entered the home-monitoring programme between 2016 and 2020. The primary outcome was survival. Secondary outcomes included multiple parameters as potential predictors of death or adverse outcome.

Results:

Of 217 fetal single-ventricle diagnoses during the period 2016–2020, 50.2% progressed to live birth, 35.4% to stage 1 and 29.5% to stage 2. Seventy-four patients (including 10 with postnatal diagnosis) entered the home-monitoring programme with six deaths making home-monitoring programme mortality 8.1%. Risk factors for death were the hybrid procedure as the only primary procedure (OR 33.0, p < 0.01), impaired cardiac function (OR 10.3, p < 0.025), Asian ethnicity (OR 9.3, p < 0.025), lower mean birth-weight (2.69 kg versus 3.31 kg, p < 0.01), and lower mean weight centiles during interstage follow-up (mean centiles of 3.1 versus 10.8, p < 0.01)

Conclusion:

Survival in the home-monitoring programme is comparable with other home-monitoring programmes in the literature. Hybrid procedure, cardiac dysfunction, sub-optimal weight gain, and Asian ethnicity were significant risk factors for death. Home-monitoring programmes should continue to raise awareness of these factors and seek solutions to mitigate adverse events. Future work to generalise home-monitoring programme and single-ventricle fetus to stage 2 outcomes in the United Kingdom will require multi-centre collaboration.

Keywords

Single ventriclesurvivalhome monitoringfetal cardiology
Type
Original Article
Information
Cardiology in the Young , Volume 33 , Issue 5 , May 2023 , pp. 710 - 717
Check for updates
Copyright
© The Author(s), 2022. Published by Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Fontan, F, Mounicot, FB, Baudet, E, et al. [“Correction” of tricuspid atresia. 2 cases “corrected” using a new surgical technic]. Ann chir thorac cardio-vasc 1971; 10: 39–47, [published Online First: 1971/01/01].Google ScholarPubMed
Norwood, WI, Lang, P, Hansen, DD. Physiologic repair of aortic atresia-hypoplastic left heart syndrome. N Engl J Med 1983; 308: 23–26. DOI 10.1056/NEJM198301063080106.CrossRefGoogle ScholarPubMed
Gibbs, JL, Wren, C, Watterson, KG, et al. 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. Brit Heart J 1993; 69: 551–555. DOI 10.1136/hrt.69.6.551.CrossRefGoogle ScholarPubMed
Bove, EL, Lloyd, TR. Staged reconstruction for hypoplastic left heart syndrome. contemporary results. Ann Surg 1996; 224: 387–394. DOI 10.1097/00000658-199609000-00015.CrossRefGoogle ScholarPubMed
Castellanos, DA, Herrington, C, Adler, S, et al. Home monitoring program reduces mortality in high-risk sociodemographic single-ventricle patients. Pediatr Cardiol 2016; 37: 1575–1580. DOI 10.1007/s00246-016-1472-x.CrossRefGoogle Scholar
Dobrolet, NC, Nieves, JA, Welch, EM, et al. New approach to interstage care for palliated high-risk patients with congenital heart disease. J Thorac Cardiovasc Surg 2011; 142: 855–860. DOI 10.1016/j.jtcvs.2011.01.054.CrossRefGoogle ScholarPubMed
Forbess, JM, Cook, N, Roth, SJ, et al. Ten-year institutional experience with palliative surgery for hypoplastic left heart syndrome. risk factors related to stage I mortality. Circulation 1995; 92. DOI 10.1161/01.cir.92.9.262.CrossRefGoogle ScholarPubMed
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: 896–906. DOI 10.1016/j.jtcvs.2012.05.020.CrossRefGoogle ScholarPubMed
Mahle, WT, Spray, TL, Wernovsky, G, et al. Survival after reconstructive surgery for hypoplastic left heart syndrome: a 15-year experience from a single institution. Circulation 2000; 102: III136–III141. DOI 10.1161/01.cir.102.suppl_3.iii-136.CrossRefGoogle ScholarPubMed
Petit, CJ, Fraser, CD, Mattamal, R, et al. 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: 1358–1366. DOI 10.1016/j.jtcvs.2011.04.043.CrossRefGoogle Scholar
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: II262. DOI 10.1161/CIRCULATIONAHA.105.001438.CrossRefGoogle ScholarPubMed
Siehr, SL, Norris, JK, Bushnell, JA, et al. Home monitoring program reduces interstage mortality after the modified Norwood procedure. J Thorac Cardiovasc Surg 2014; 147: 718–723 e1. DOI 10.1016/j.jtcvs.2013.04.006.CrossRefGoogle ScholarPubMed
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: I82–I89.CrossRefGoogle ScholarPubMed
Ghanayem, NS, Hoffman, GM, Mussatto, KA, et al. Home surveillance program prevents interstage mortality after the Norwood procedure. J Thorac Cardiovasc Surg 2003; 126: 1367–1375. DOI 10.1016/s0022-5223(03)00071-0.CrossRefGoogle ScholarPubMed
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: 502–508. DOI 10.1161/CIRCULATIONAHA.114.014107.CrossRefGoogle ScholarPubMed
Gardner, MM, Mercer-Rosa, L, Faerber, J, et al. Association of a home monitoring program with interstage and stage 2 outcomes. J Am Heart Assoc 2019; 8: e010783. DOI 10.1161/JAHA.118.010783.CrossRefGoogle ScholarPubMed
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: 1540–1547. DOI 10.1016/j.jtcvs.2014.02.038.CrossRefGoogle ScholarPubMed
https://www.rcpch.ac.uk/resources/growth-charts [20/02/2021].Google Scholar
NICE U.K. Faltering Growth: Recognition and Management of Faltering Growth in Children, 2017.Google Scholar
Rogers, L, Pagel, C, Sullivan, ID, et al. Interventions and outcomes in children with hypoplastic left heart syndrome born in England and Wales Bbetween 2000 and 2015 based on the National Congenital Heart Disease Audit. Circulation 2017; 136: 1765–1767. DOI 10.1161/CIRCULATIONAHA.117.028784.CrossRefGoogle Scholar
Ohye, RG, Sleeper, LA, Mahony, L, et al. Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med 2010; 362: 1980–1992. DOI 10.1056/NEJMoa0912461.CrossRefGoogle ScholarPubMed
Michielon, G, DiSalvo, G, Fraisse, A, et al. In-hospital interstage improves interstage survival after the Norwood stage 1 operation. Eur J Cardiothorac Surg 2020; 57: 1113–1121. DOI 10.1093/ejcts/ezaa074.CrossRefGoogle ScholarPubMed
Best, KE, Vieira, R, Glinianaia, SV, et al. Socio-economic inequalities in mortality in children with congenital heart disease: a systematic review and meta-analysis. Paediatr Perinat Epidemiol 2019; 33: 291–309. DOI 10.1111/ppe.12564.CrossRefGoogle ScholarPubMed
Roland, D, Oliver, A, Edwards, ED, et al. Use of paediatric early warning systems in Great Britain: has there been a change of practice in the last 7 years? Arch Dis Child 2014; 99: 26–29. DOI 10.1136/archdischild-2012-302783.CrossRefGoogle ScholarPubMed
Pearson, GA, Ward-Platt, M, Harnden, A, et al. Why children die: avoidable factors associated with child deaths. Arch Dis Child 2011; 96: 927–931. DOI 10.1136/adc.2009.177071.CrossRefGoogle ScholarPubMed
Lillitos, PJ, Maconochie, IK. Paediatric early warning systems (PEWS and trigger systems) for the hospitalised child: time to focus on the evidence. Arch Dis Child 2017; 102: 479–480. DOI 10.1136/archdischild-2016-312136.CrossRefGoogle ScholarPubMed
Gaskin, KL, Wray, J, Barron, DJ. Acceptability of a parental early warning tool for parents of infants with complex congenital heart disease: a qualitative feasibility study. Arch Dis Child 2018; 103: 880–886. DOI 10.1136/archdischild-2017-313227.CrossRefGoogle ScholarPubMed
Liu, MY, Zielonka, B, Snarr, BS, et al. Longitudinal assessment of outcome from prenatal diagnosis through fontan operation for over 500 fetuses with single ventricle-type congenital heart disease: the Philadelphia fetus-to-fontan cohort study. J Am Heart Assoc 2018; 7: e009145. DOI 10.1161/JAHA.118.009145.CrossRefGoogle ScholarPubMed
Supplementary material: File

Lillitos et al. supplementary material

Lillitos et al. supplementary material

Download Lillitos et al. supplementary material(File)
File 179.7 KB