Interventions and Outcomes in Children With Hypoplastic Left Heart Syndrome Born in England and Wales Between 2000 and 2015 Based on the National Congenital Heart Disease Audit
2017; Lippincott Williams & Wilkins; Volume: 136; Issue: 18 Linguagem: Inglês
10.1161/circulationaha.117.028784
ISSN1524-4539
AutoresLibby Rogers, Christina Pagel, Ian D. Sullivan, Muhammad Mustafa, Victor Tsang, Martin Utley, Catherine Bull, Rodney Franklin, Kate Brown,
Tópico(s)Cardiovascular Conditions and Treatments
ResumoHomeCirculationVol. 136, No. 18Interventions and Outcomes in Children With Hypoplastic Left Heart Syndrome Born in England and Wales Between 2000 and 2015 Based on the National Congenital Heart Disease Audit Free AccessLetterPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessLetterPDF/EPUBInterventions and Outcomes in Children With Hypoplastic Left Heart Syndrome Born in England and Wales Between 2000 and 2015 Based on the National Congenital Heart Disease Audit Libby Rogers, MMath, Christina Pagel, PhD, Ian D. Sullivan, MD, FRCP, Muhammed Mustafa, MD, Victor Tsang, MS, FRCS, Martin Utley, PhD, Catherine Bull, MRCP, Rodney C. Franklin, MD, FRCP and Katherine L. Brown, MPH, MD Libby RogersLibby Rogers From Clinical Operational Research Unit, University College London, United Kingdom (L.R., C.P., M.U.); Cardiac, Critical Care and Respiratory Division, Great Ormond Street Hospital National Health Service Foundation Trust, London, United Kingdom (I.D.S., M.M., V.T., C.B., K.L.B.); and Paediatric Cardiology, Royal Brompton and Harefield National Health Service Foundation Trust , London, United Kingdom (R.C.F.). , Christina PagelChristina Pagel From Clinical Operational Research Unit, University College London, United Kingdom (L.R., C.P., M.U.); Cardiac, Critical Care and Respiratory Division, Great Ormond Street Hospital National Health Service Foundation Trust, London, United Kingdom (I.D.S., M.M., V.T., C.B., K.L.B.); and Paediatric Cardiology, Royal Brompton and Harefield National Health Service Foundation Trust , London, United Kingdom (R.C.F.). , Ian D. SullivanIan D. Sullivan From Clinical Operational Research Unit, University College London, United Kingdom (L.R., C.P., M.U.); Cardiac, Critical Care and Respiratory Division, Great Ormond Street Hospital National Health Service Foundation Trust, London, United Kingdom (I.D.S., M.M., V.T., C.B., K.L.B.); and Paediatric Cardiology, Royal Brompton and Harefield National Health Service Foundation Trust , London, United Kingdom (R.C.F.). , Muhammed MustafaMuhammed Mustafa From Clinical Operational Research Unit, University College London, United Kingdom (L.R., C.P., M.U.); Cardiac, Critical Care and Respiratory Division, Great Ormond Street Hospital National Health Service Foundation Trust, London, United Kingdom (I.D.S., M.M., V.T., C.B., K.L.B.); and Paediatric Cardiology, Royal Brompton and Harefield National Health Service Foundation Trust , London, United Kingdom (R.C.F.). , Victor TsangVictor Tsang From Clinical Operational Research Unit, University College London, United Kingdom (L.R., C.P., M.U.); Cardiac, Critical Care and Respiratory Division, Great Ormond Street Hospital National Health Service Foundation Trust, London, United Kingdom (I.D.S., M.M., V.T., C.B., K.L.B.); and Paediatric Cardiology, Royal Brompton and Harefield National Health Service Foundation Trust , London, United Kingdom (R.C.F.). , Martin UtleyMartin Utley From Clinical Operational Research Unit, University College London, United Kingdom (L.R., C.P., M.U.); Cardiac, Critical Care and Respiratory Division, Great Ormond Street Hospital National Health Service Foundation Trust, London, United Kingdom (I.D.S., M.M., V.T., C.B., K.L.B.); and Paediatric Cardiology, Royal Brompton and Harefield National Health Service Foundation Trust , London, United Kingdom (R.C.F.). , Catherine BullCatherine Bull From Clinical Operational Research Unit, University College London, United Kingdom (L.R., C.P., M.U.); Cardiac, Critical Care and Respiratory Division, Great Ormond Street Hospital National Health Service Foundation Trust, London, United Kingdom (I.D.S., M.M., V.T., C.B., K.L.B.); and Paediatric Cardiology, Royal Brompton and Harefield National Health Service Foundation Trust , London, United Kingdom (R.C.F.). , Rodney C. FranklinRodney C. Franklin From Clinical Operational Research Unit, University College London, United Kingdom (L.R., C.P., M.U.); Cardiac, Critical Care and Respiratory Division, Great Ormond Street Hospital National Health Service Foundation Trust, London, United Kingdom (I.D.S., M.M., V.T., C.B., K.L.B.); and Paediatric Cardiology, Royal Brompton and Harefield National Health Service Foundation Trust , London, United Kingdom (R.C.F.). and Katherine L. BrownKatherine L. Brown From Clinical Operational Research Unit, University College London, United Kingdom (L.R., C.P., M.U.); Cardiac, Critical Care and Respiratory Division, Great Ormond Street Hospital National Health Service Foundation Trust, London, United Kingdom (I.D.S., M.M., V.T., C.B., K.L.B.); and Paediatric Cardiology, Royal Brompton and Harefield National Health Service Foundation Trust , London, United Kingdom (R.C.F.). Originally published31 Oct 2017https://doi.org/10.1161/CIRCULATIONAHA.117.028784Circulation. 2017;136:1765–1767Among congenital heart diseases, none has undergone a more drastic change in diagnostic approach, management, and outcomes over the last 30 years than hypoplastic left heart syndrome (HLHS), with comfort care replaced as the predominant form of management by a palliative surgical treatment pathway.1 The outcomes of palliative procedures for HLHS have been well documented, but the longer-term picture is less clear.The UK National Congenital Heart Diseases Audit (NCHDA) captures every procedure undertaken for congenital heart disease and updated life status for patients resident in England and Wales from death certifications. From this data set, we identified patients born between 2000 and 2015 who fulfilled the criteria for HLHS: a small left ventricle, stenosis or atresia of the left-sided heart valves, normally aligned great arteries, and no common atrioventricular junction.2 All patients with HLHS require surgical palliation with a traditional Norwood or hybrid pathway or heart transplantation to survive. Hence, we were able to use procedure information in combination with diagnostic information to identify patients with HLHS within the NCHDA data set using the International Paediatric and Congenital Cardiac Codes. Because the NCHDA is a procedure-based data set, patients with HLHS who did not undergo any interventional treatment are not included. Because the hybrid procedure was introduced in the United Kingdom in 2006, we considered 2 eras for analyses dating up to and after 2006.The surgical and catheterization procedures undertaken were classified as follows:Pathway procedures consistent with HLHS treatment, including the Norwood (stage 1), Glenn (stage 2), and Fontan (stage 3) procedures of traditional surgical palliation; the hybrid procedure, the comprehensive stage 2; and transplantation procedures.Prepathway procedures either required for stabilization of very sick neonates with HLHS such as pulmonary artery banding and interventions for restrictive atrial septum or interventions to the aortic valve or arch in neonates with hypoplastic left ventricle when a failed biventricular approach was evident on the basis of subsequent events.Off-pathway procedures that may be required in patients with HLHS such as revision of arterial shunt or Sano, stenting or dilation of residual or recoarctation.3Although we report transplantation procedures within our description of pathways and outcomes for HLHS, transplantation did not represent an end point in our primary analysis of condition-based survival. Survival analysis was carried out with the Kaplan-Meier approach, with death representing failure. Data were analyzed with the Stata 13 statistical software package.The study was approved by the NCHDA Research Committee and by the National Health Service Healthcare Quality Improvement Partnership (study 14CONG03). Further ethics committee approval and patient consent were waived.Of 976 patients with HLHS, 9.6% had a prepathway intervention, which carried the highest in-hospital mortality of 34.0%; 89.5% embarked on a traditional surgical pathway of staged palliation; and 6.4% of smaller infants with more complex conditions embarked on a hybrid pathway.4The outcomes among patients who followed the different trajectories are shown in the Figure. For 20 of the patients lost to follow-up, survival was censored at the last known hospital discharge.Download figureDownload PowerPointFigure. Interventions and outcomes for 976 English and Welsh patients with hypoplastic left heart syndrome between 2000 and 2015. All patients who embarked on the traditional surgical treatment pathway (874, 89.5% of the cohort) are shown in purple; those who embarked on a hybrid pathway (62, 6.4% of the cohort), in orange. Patients who had a stage 1 procedure after their hybrid procedure (18, 29.0% of hybrids) are shown in blue from that point. The trajectories of patients who had prepathway procedures (94, 9.6% of the cohort) are shown in green to the point where they join the traditional or hybrid pathway. Heart transplantations are represented with a heart symbol.The in-hospital mortality for the primary procedure (Norwood or hybrid) was 22.9% (95% confidence interval [CI], 20.2–25.7), for stage 2 Glenn was 3.6% (95% CI, 2.2–5.4), and for stage 3 Fontan was 1.2% (95% CI, 0.3–3.1%). The interstage mortality between primary procedure and stage 2 (or comprehensive stage 2) was 13.0% (95% CI, 10.6–15.6) and between stage 2 (or comprehensive stage 2) and stage 3 was 7.3% (95% CI, 5.2–9.9). In a comparison of outcomes by era, the only statistically significant change in mortality at different stages was a reduction in in-hospital mortality after Norwood (stage 1) surgery.Kaplan-Meier survival inclusive of all patients was 60.7% (95% CI, 57.5–63.7) at 1 year and 56.3% (95% CI, 53.0–59.5) at 5 years. The log-rank test suggested better survival in patients undergoing a traditional surgical pathway (including all those undergoing prepathway procedures) in the later era (P=0.088). Because the hybrid procedure was introduced in 2006 (and noting the increased complexity among patients undergoing hybrid pathway), survival on the traditional pathway has been higher (P=0.050).There was at least 1 off-pathway procedure in 44.6% of patients. The rate for off-pathway cardiac surgery was 6 per 100 patient-years, and the rate for off-pathway interventional catheterization was 16 per 100 patient-years, with a significant increase between eras (P<0.001).The mandatory national audit of cardiac procedures and the independent tracking of survival were essential prerequisites for this population-based study, which captures the outcomes for all patients with HLHS who underwent at least 1 cardiac procedure between 2000 and 2015 in England and Wales. Early postoperative survival rates have improved over time and are now inadequate as indexes of quality or to inform clinicians and families about outcomes and events after a diagnosis of HLHS.Treatment pathways among patients with HLHS are highly variable and complex. The analysis of longer-term outcome based on diagnosis gives a more complete and useful picture than short-term outcomes based on procedure and may inform quality improvement initiatives. The outcome for HLHS treated in England and Wales compares well with international standards.3,5AcknowledgmentsThe authors thank the data managers and audit leads at the UK centers that contribute to the national audit data and the data management team at the National Institute for Cardiovascular Outcomes Research for its major contribution to the NCHDA and for providing the data for this study.Sources of FundingThis project was funded by the Great Ormond Street Children's Charity (v1248). Dr Utley was supported in part by the National Institute for Health Research Collaboration for Leadership in Applied Health Research and Care North Thames at Bart's Health National Health Service Trust. Drs Brown, Sullivan, Mustafa, and Tsang were supported by the National Institute for Health Research Biomedical Research Center at Great Ormond Street Hospital for Children National Health Service Foundation Trust and University College London.DisclosuresDrs Brown and Franklin sit on the steering committee of NCHDA. The other authors report no conflicts.FootnotesCirculation is available at http://circ.ahajournals.org.Correspondence to: Katherine L. Brown, MPH, MD, Charles West Division, Great Ormond Street Hospital for Children National Health Service Foundation Trust, Great Ormond St, London, UK WC1N 3JH. E-mail [email protected]References1. Feinstein JA, Benson DW, Dubin AM, Cohen MS, Maxey DM, Mahle WT, Pahl E, Villafañe 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. Hypoplastic left heart syndrome: current considerations and expectations.J Am Coll Cardiol. 2012; 59(suppl):S1–S42. doi: 10.1016/j.jacc.2011.09.022.CrossrefMedlineGoogle Scholar2. Tchervenkov CI, Jacobs JP, Weinberg PM, Aiello VD, Béland MJ, Colan SD, Elliott MJ, Franklin RC, Gaynor JW, Krogmann ON, Kurosawa H, Maruszewski B, Stellin G. The nomenclature, definition and classification of hypoplastic left heart syndrome.Cardiol Young. 2006; 16:339–368. doi: 10.1017/S1047951106000291.CrossrefMedlineGoogle Scholar3. Newburger JW, Sleeper LA, Frommelt PC, Pearson GD, Mahle WT, Chen S, Dunbar-Masterson C, Mital S, Williams IA, Ghanayem NS, Goldberg CS, Jacobs JP, Krawczeski CD, Lewis AB, Pasquali SK, Pizarro C, Gruber PJ, Atz AM, Khaikin S, Gaynor JW, Ohye RG; Pediatric Heart Network Investigators. Transplantation-free survival and interventions at 3 years in the single ventricle reconstruction trial.Circulation. 2014; 129:2013–2020. doi: 10.1161/CIRCULATIONAHA.113.006191.LinkGoogle Scholar4. Tabbutt S, Ghanayem N, Ravishankar C, Sleeper LA, Cooper DS, Frank DU, Lu M, Pizarro C, Frommelt P, Goldberg CS, Graham EM, Krawczeski CD, Lai WW, Lewis A, Kirsh JA, Mahony L, Ohye RG, Simsic J, Lodge AJ, Spurrier E, Stylianou M, Laussen P; Pediatric Heart Network Investigators. Risk factors for hospital morbidity and mortality after the Norwood procedure: a report from the Pediatric Heart Network Single Ventricle Reconstruction trial.J Thorac Cardiovasc Surg. 2012; 144:882–895. doi: 10.1016/j.jtcvs.2012.05.019.CrossrefMedlineGoogle Scholar5. Dean PN, McHugh KE, Conaway MR, Hillman DG, Gutgesell HP. Effects of race, ethnicity, and gender on surgical mortality in hypoplastic left heart syndrome.Pediatr Cardiol. 2013; 34:1829–1836. doi: 10.1007/s00246-013-0723-3.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Espuny Pujol F, Pagel C, Brown K, Doidge J, Feltbower R, Franklin R, Gonzalez-Izquierdo A, Gould D, Norman L, Stickley J, Taylor J and Crowe S (2022) Linkage of National Congenital Heart Disease Audit data to hospital, critical care and mortality national data sets to enable research focused on quality improvement, BMJ Open, 10.1136/bmjopen-2021-057343, 12:5, (e057343), Online publication date: 1-May-2022. Dipchand A, Honjo O, Alonso-Gonzalez R, McDonald M and Roche S (2022) Heart Transplant Indications, Considerations, and Outcomes in Fontan Patients: Age-Related Nuances, Transplant Listing, and Disease-Specific Indications, Canadian Journal of Cardiology, 10.1016/j.cjca.2022.02.019, Online publication date: 1-Feb-2022. Hadjicosta E, Franklin R, Seale A, Stumper O, Tsang V, Anderson D, Pagel C, Crowe S, Espuny Pujol F, Ridout D and Brown K (2021) Cohort study of intervened functionally univentricular heart in England and Wales (2000–2018), Heart, 10.1136/heartjnl-2021-319677, 108:13, (1046-1054), Online publication date: 1-Jul-2022. Tretter J and Jacobs J (2021) Global Leadership in Paediatric and Congenital Cardiac Care: "Coding our way to improved care: an interview with Rodney C. G. Franklin, MBBS, MD, FRCP, FRCPCH", Cardiology in the Young, 10.1017/S104795112000476X, 31:1, (11-19), Online publication date: 1-Jan-2021. Alphonso N, Angelini A, Barron D, Bellsham-Revell H, Blom N, Brown K, Davis D, Duncan D, Fedrigo M, Galletti L, Hehir D, Herberg U, Jacobs J, Januszewska K, Karl T, Malec E, Maruszewski B, Montgomerie J, Pizzaro C, Schranz D, Shillingford A and Simpson J (2020) Guidelines for the management of neonates and infants with hypoplastic left heart syndrome: The European Association for Cardio-Thoracic Surgery (EACTS) and the Association for European Paediatric and Congenital Cardiology (AEPC) Hypoplastic Left Heart Syndrome Guidelines Task Force, European Journal of Cardio-Thoracic Surgery, 10.1093/ejcts/ezaa188, 58:3, (416-499), Online publication date: 1-Sep-2020. Kang S, Jaeggi E, Ryan G and Chaturvedi R (2020) An Overview of Contemporary Outcomes in Fetal Cardiac Intervention: A Case for High-Volume Superspecialization?, Pediatric Cardiology, 10.1007/s00246-020-02294-2, 41:3, (479-485), Online publication date: 1-Mar-2020. Rogers L, Pagel C, Sullivan I, Mustafa M, Tsang V, Utley M, Bull C, Franklin R and Brown K (2018) Interventional treatments and risk factors in patients born with hypoplastic left heart syndrome in England and Wales from 2000 to 2015, Heart, 10.1136/heartjnl-2017-312448, 104:18, (1500-1507), Online publication date: 1-Sep-2018. Winters J (2018) When Parents Refuse: Resolving Entrenched Disagreements Between Parents and Clinicians in Situations of Uncertainty and Complexity, The American Journal of Bioethics, 10.1080/15265161.2018.1485758, 18:8, (20-31), Online publication date: 3-Aug-2018. Best K, Miller N, Draper E, Tucker D, Luyt K and Rankin J (2021) The Improved Prognosis of Hypoplastic Left Heart: A Population-Based Register Study of 343 Cases in England and Wales, Frontiers in Pediatrics, 10.3389/fped.2021.635776, 9 Lillitos P, Rittey L, Vass M, Ugwoke G, Akhtar M, Barwick S, Van Doorn C, Jaber O and Bentham J (2022) Performance of a single-ventricle home-monitoring programme: survival and predictors of adverse outcome, Cardiology in the Young, 10.1017/S1047951122001524, (1-8) October 31, 2017Vol 136, Issue 18 Advertisement Article InformationMetrics © 2017 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.117.028784PMID: 29084781 Originally publishedOctober 31, 2017 Keywordschildheart diseasestreatment outcomehypoplastic left heart syndromeUnited KingdomPDF download Advertisement SubjectsCardiovascular SurgeryCongenital Heart DiseaseMortality/SurvivalQuality and Outcomes
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