Skip to main content
View expanded cover

Heart Failure in Adult Congenital Heart Disease pp 251–263Cite as

Future Research

  • 524 Accesses

Part of the Congenital Heart Disease in Adolescents and Adults book series (CHDAA)

Abstract

As heart failure is becoming ever more common in congenital heart disease (CHD), there is increasing need for studies to support clinical practice. Indeed, most practice in CHD is based on soft evidence from cohort studies and expert consensus, resulting in significant variation. This chapter provides an overview of the areas of need and potential future research in congenital heart disease and heart failure.

Keywords

  • Research
  • Risk stratification
  • Therapy
  • Prevention
  • Genetics

This is a preview of subscription content, access via your institution.

Chapter
USD   29.95
Price excludes VAT (USA)
  • DOI: 10.1007/978-3-319-77803-7_15
  • Chapter length: 13 pages
  • Instant PDF download
  • Readable on all devices
  • Own it forever
  • Exclusive offer for individuals only
  • Tax calculation will be finalised during checkout
eBook
USD   79.99
Price excludes VAT (USA)
  • ISBN: 978-3-319-77803-7
  • Instant PDF download
  • Readable on all devices
  • Own it forever
  • Exclusive offer for individuals only
  • Tax calculation will be finalised during checkout
Softcover Book
USD   99.99
Price excludes VAT (USA)
Hardcover Book
USD   139.99
Price excludes VAT (USA)
Learn about institutional subscriptions

Abbreviations

ACE:

Angiotensin-converting enzyme

CHD:

Congenital heart disease

PAH:

Pulmonary arterial hypertension

PAH-CHD:

Pulmonary arterial hypertension related to congenital heart disease

SNPs:

Single nucleotide polymorphisms

TCPC:

Total cavopulmonary connection

References

  1. Ponikowski P, Voors AA, Anker SD, Bueno H, Cleland JGF, Coats AJS, et al. 2016 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure. The Task Force for the diagnosis and treatment of acute and chronic heart failure of the European Society of Cardiology (ESC) Developed with the special contribution of the Heart Failure Association (HFA) of the ESC. Eur Heart J. 2016;37(27):2129–200.

    CrossRef  PubMed  Google Scholar 

  2. Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease. J Am Coll Cardiol. 2008;52(23):e143–263.

    CrossRef  PubMed  Google Scholar 

  3. Baumgartner H, Bonhoeffer P, De Groot NMS, de Haan F, Deanfield JE, Galie N, et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J. 2010;31(23):2915–57.

    CrossRef  PubMed  Google Scholar 

  4. van der BT, Winter MM, Bouma BJ, Groenink M, Vliegen HW, Pieper PG, et al. Effect of valsartan on systemic right ventricular function a double-blind, randomized, placebo-controlled pilot trial. Circulation. 2013;127(3):322–30.

    CrossRef  CAS  Google Scholar 

  5. Robinson B, Heise CT, Moore JW, Anella J, Sokoloski M, Eshaghpour E. Afterload reduction therapy in patients following intraatrial baffle operation for transposition of the great arteries. Pediatr Cardiol. 2002;23(6):618–23.

    CrossRef  CAS  PubMed  Google Scholar 

  6. Therrien J, Provost Y, Harrison J, Connelly M, Kaemmerer H, Webb GD. Effect of angiotensin receptor blockade on systemic right ventricular function and size: a small, randomized, placebo-controlled study. Int J Cardiol. 2008;129(2):187–92.

    CrossRef  PubMed  Google Scholar 

  7. Dimopoulos K. Trials and tribulations in adult congenital heart disease. Int J Cardiol. 2008;129(2):160–2.

    CrossRef  PubMed  Google Scholar 

  8. Gurvitz M, Burns KM, Brindis R, Broberg CS, Daniels CJ, Fuller SMPN, et al. Emerging research directions in adult congenital heart disease: a report from a National Heart, Lung, and Blood Institute/Adult Congenital Heart Association Working Group. J Am Coll Cardiol. 2016;67(16):1956–64.

    CrossRef  PubMed  PubMed Central  Google Scholar 

  9. Dimopoulos K, Alonso-Gonzalez R, D’Alto M. Heart failure, exercise intolerance, and physical training. In: Diagnosis and management of adult congenital heart disease [Internet]. 3rd ed. 2017 [cited 2017 Mar 27]. https://www.elsevier.com/books/diagnosis-and-management-of-adult-congenital-heart-disease/unknown/978-0-7020-6929-1.

  10. Stout KK, Broberg CS, Book WM, Cecchin F, Chen JM, Dimopoulos K, et al. Chronic heart failure in congenital heart disease: a scientific statement from the American Heart Association. Circulation. 2016;133(8):770–801.

    PubMed  Google Scholar 

  11. Tutarel O, Kempny A, Alonso-Gonzalez R, Jabbour R, Li W, Uebing A, et al. Congenital heart disease beyond the age of 60: emergence of a new population with high resource utilization, high morbidity, and high mortality. Eur Heart J. 2014;35(11):725–32.

    CrossRef  PubMed  Google Scholar 

  12. Gatzoulis MA, Clark AL, Cullen S, Newman CG, Redington AN. Right ventricular diastolic function 15 to 35 years after repair of tetralogy of Fallot. Restrictive physiology predicts superior exercise performance. Circulation. 1995;91(6):1775–81.

    CrossRef  CAS  PubMed  Google Scholar 

  13. Diller G-P, Dimopoulos K, Okonko D, Uebing A, Broberg CS, Babu-Narayan S, et al. Heart rate response during exercise predicts survival in adults with congenital heart disease. J Am Coll Cardiol. 2006;48(6):1250–6.

    CrossRef  PubMed  Google Scholar 

  14. Alonso-Gonzalez R, Borgia F, Diller G-P, Inuzuka R, Kempny A, Martinez-Naharro A, et al. Abnormal lung function in adults with congenital heart disease: prevalence, relation to cardiac anatomy, and association with survival. Circulation. 2013;127(8):882–90.

    CrossRef  PubMed  Google Scholar 

  15. Dimopoulos K, Diller G-P, Giannakoulas G, Petraco R, Chamaidi A, Karaoli E, et al. Anemia in adults with congenital heart disease relates to adverse outcome. J Am Coll Cardiol. 2009;54(22):2093–100.

    CrossRef  PubMed  Google Scholar 

  16. Dimopoulos K, Wort SJ, Gatzoulis MA. Pulmonary hypertension related to congenital heart disease: a call for action. Eur Heart J. 2014;35(11):691–700.

    CrossRef  PubMed  Google Scholar 

  17. Ohye RG, Sleeper LA, Mahony L, Newburger JW, Pearson GD, Lu M, et al. Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med. 2010;362(21):1980–92.

    CrossRef  CAS  PubMed  PubMed Central  Google Scholar 

  18. Kempny A, Hjortshøj CS, Gu H, Li W, Opotowsky AR, Landzberg MJ, et al. Predictors of death in contemporary adult patients with Eisenmenger syndrome: a multicenter study. Circulation. 2017;135(15):1432–40.

    CrossRef  PubMed  Google Scholar 

  19. Khairy P, Harris L, Landzberg MJ, Viswanathan S, Barlow A, Gatzoulis MA, et al. Implantable cardioverter-defibrillators in tetralogy of Fallot. Circulation. 2008;117(3):363–70.

    CrossRef  PubMed  Google Scholar 

  20. Giannakoulas G, Dimopoulos K, Bolger AP, Tay EL, Inuzuka R, Bedard E, et al. Usefulness of natriuretic peptide levels to predict mortality in adults with congenital heart disease. Am J Cardiol. 2010;105(6):869–73.

    CrossRef  CAS  PubMed  Google Scholar 

  21. Sharma R, Bolger AP, Li W, Davlouros PA, Volk H-D, Poole-Wilson PA, et al. Elevated circulating levels of inflammatory cytokines and bacterial endotoxin in adults with congenital heart disease. Am J Cardiol. 2003;92(2):188–93.

    CrossRef  CAS  PubMed  Google Scholar 

  22. Bolger AP, Sharma R, Li W, Leenarts M, Kalra PR, Kemp M, et al. Neurohormonal activation and the chronic heart failure syndrome in adults with congenital heart disease. Circulation. 2002;106(1):92–9.

    CrossRef  CAS  PubMed  Google Scholar 

  23. McMurray JJV, Packer M, Desai AS, Gong J, Lefkowitz MP, Rizkala AR, et al. Angiotensin-neprilysin inhibition versus enalapril in heart failure. N Engl J Med. 2014;371(11):993–1004.

    CrossRef  CAS  PubMed  Google Scholar 

  24. Swedberg K, Komajda M, Böhm M, Borer JS, Ford I, Dubost-Brama A, et al. Ivabradine and outcomes in chronic heart failure (SHIFT): a randomised placebo-controlled study. Lancet. 2010;376(9744):875–85.

    CrossRef  CAS  PubMed  Google Scholar 

  25. NOTE registry|ISACHD [Internet]. [cited 2017 Dec 3]. http://www.isachd.org/content/note-registry.

  26. The British Association for Cardiovascular Prevention and Rehabilitation. The BACPR standards and core components for cardiovascular disease prevention and rehabilitation [Internet]. 2017 [cited 2016 Sep 23]. http://www.bacpr.com/pages/page_box_contents.asp?pageid=791.

  27. Dua JS, Cooper AR, Fox KR, Graham Stuart A. Exercise training in adults with congenital heart disease: feasibility and benefits. Int J Cardiol. 2010;138(2):196–205.

    CrossRef  PubMed  Google Scholar 

  28. Duppen N, Kapusta L, de Rijke YB, Snoeren M, Kuipers IM, Koopman LP, et al. The effect of exercise training on cardiac remodelling in children and young adults with corrected tetralogy of Fallot or Fontan circulation: a randomized controlled trial. Int J Cardiol. 2015;179:97–104.

    CrossRef  CAS  PubMed  Google Scholar 

  29. Becker-Grünig T, Klose H, Ehlken N, Lichtblau M, Nagel C, Fischer C, et al. Efficacy of exercise training in pulmonary arterial hypertension associated with congenital heart disease. Int J Cardiol. 2013;168(1):375–81.

    CrossRef  PubMed  Google Scholar 

  30. van der Bom T, Winter MM, Knaake JL, Cervi E, de Vries LSC, Balducci A, et al. Long-term benefits of exercise training in patients with a systemic right ventricle. Int J Cardiol. 2015;179:105–11.

    CrossRef  PubMed  Google Scholar 

  31. Piepoli MF, Hoes AW, Agewall S, Albus C, Brotons C, Catapano AL, et al. 2016 European Guidelines on cardiovascular disease prevention in clinical practice. Eur Heart J. 2016;37(29):2315–81.

    CrossRef  PubMed  PubMed Central  Google Scholar 

  32. Lewis M, Rosenbaum M. The miracle baby grows up: hypoplastic left heart syndrome in the adult. Curr Cardiol Rep. 2017;19(8):74.

    CrossRef  PubMed  Google Scholar 

  33. Galiè N, Beghetti M, Gatzoulis MA, Granton J, Berger RMF, Lauer A, et al. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation. 2006;114(1):48–54.

    CrossRef  CAS  PubMed  Google Scholar 

  34. Galie N, Landzberg M, Beghetti M, Berger RM, Efficace M, Gesang S, et al. Evaluation of macitentan in patients with Eisenmenger syndrome: results from the randomised controlled MAESTRO study. Eur Heart J [Internet]. 2017 Aug 1 [cited 2017 Dec 3];38(suppl_1). https://academic.oup.com/eurheartj/article/38/suppl_1/ehx493.P5462/4086822.

  35. Balzer DT, Kort HW, Day RW, Corneli HM, Kovalchin JP, Cannon BC, et al. Inhaled nitric oxide as a preoperative test (INOP test I): the INOP test study group. Circulation. 2002;106(12 Suppl 1):I76–81.

    PubMed  Google Scholar 

  36. Frescura C, Thiene G, Giulia Gagliardi M, Mazzucco A, Pellegrino PA, Daliento L, et al. Is lung biopsy useful for surgical decision making in congenital heart disease? Eur J Cardiothorac Surg. 1991;5(3):118–22. discussion 122–3

    CrossRef  CAS  PubMed  Google Scholar 

  37. Wagenvoort CA. Morphological substrate for the reversibility and irreversibility of pulmonary hypertension. Eur Heart J. 1988;9(Suppl J):7–12.

    CrossRef  PubMed  Google Scholar 

  38. Novick WM, Sandoval N, Lazorhysynets VV, Castillo V, Baskevitch A, Mo X, et al. Flap valve double patch closure of ventricular septal defects in children with increased pulmonary vascular resistance. Ann Thorac Surg. 2005;79(1):21–8.

    CrossRef  PubMed  Google Scholar 

  39. Dimopoulos K, Peset A, Gatzoulis MA. Evaluating operability in adults with congenital heart disease and the role of pretreatment with targeted pulmonary arterial hypertension therapy. Int J Cardiol. 2008;129(2):163–71.

    CrossRef  PubMed  Google Scholar 

  40. Perera AH, Rudarakanchana N, Hamady M, Kashef E, Mireskandari M, Uebing A, et al. New-generation stent grafts for endovascular management of thoracic pseudoaneurysms after aortic coarctation repair. J Vasc Surg. 2014;60(2):330–6.

    CrossRef  PubMed  Google Scholar 

  41. Benza R, Correa-jaque P, Franco V, Doyle M, White JA, Ginn G, et al. Abstract 19282: safety and utility of the CardioMEMs device in pulmonary arterial hypertension. Circulation. 2016;134(Suppl 1):A19282.

    Google Scholar 

  42. Janoušek J, Gebauer RA, Abdul-Khaliq H, Turner M, Kornyei L, Grollmuß O, et al. Cardiac resynchronisation therapy in paediatric and congenital heart disease: differential effects in various anatomical and functional substrates. Heart. 2009;95(14):1165–71.

    CrossRef  PubMed  Google Scholar 

  43. Janousek J, Tomek V, Chaloupecký VA, Reich O, Gebauer RA, Kautzner J, et al. Cardiac resynchronization therapy: a novel adjunct to the treatment and prevention of systemic right ventricular failure. J Am Coll Cardiol. 2004;44(9):1927–31.

    CrossRef  PubMed  Google Scholar 

  44. Authors/Task Force Members, Priori SG, Blomström-Lundqvist C, Mazzanti A, Blom N, Borggrefe M, et al. ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: The Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC) Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC). Eur Heart J. 2015;36(41):2793–867.

    CrossRef  Google Scholar 

  45. Lutembacher R. De la sténose mitrale avec communication interauriculaire. Arch Mal Coeur. 1916;9:237–60.

    Google Scholar 

  46. Hasenfuß G, Hayward C, Burkhoff D, Silvestry FE, McKenzie S, Gustafsson F, et al. A transcatheter intracardiac shunt device for heart failure with preserved ejection fraction (REDUCE LAP-HF): a multicentre, open-label, single-arm, phase 1 trial. Lancet Lond Engl. 2016;387(10025):1298–304.

    CrossRef  Google Scholar 

  47. Kaye DM, Hasenfuß G, Neuzil P, Post MC, Doughty R, Trochu J-N, et al. One-year outcomes after transcatheter insertion of an interatrial shunt device for the management of heart failure with preserved ejection fraction. Circ Heart Fail. 2016;9(12):pii: e003662.

    CrossRef  Google Scholar 

  48. Trigo MD, Bergeron S, Bernier M, Amat-Santos IJ, Puri R, Campelo-Parada F, et al. Unidirectional left-to-right interatrial shunting for treatment of patients with heart failure with reduced ejection fraction: a safety and proof-of-principle cohort study. Lancet. 2016;387(10025):1290–7.

    CrossRef  PubMed  Google Scholar 

  49. Abraham WT, Zile MR, Weaver FA, Butter C, Ducharme A, Halbach M, et al. Baroreflex activation therapy for the treatment of heart failure with a reduced ejection fraction. JACC Heart Fail. 2015;3(6):487–96.

    CrossRef  PubMed  Google Scholar 

  50. Barostim therapy for heart failure—full text view. ClinicalTrials.gov [Internet]. [cited 2017 Dec 3]. https://clinicaltrials.gov/ct2/show/NCT02627196.

  51. Costanzo MR, Ponikowski P, Javaheri S, Augostini R, Goldberg L, Holcomb R, et al. Transvenous neurostimulation for central sleep apnoea: a randomised controlled trial. Lancet Lond Engl. 2016;388(10048):974–82.

    CrossRef  Google Scholar 

  52. Jagielski D, Ponikowski P, Augostini R, Kolodziej A, Khayat R, Abraham WT. Transvenous stimulation of the phrenic nerve for the treatment of central sleep apnoea: 12 months’ experience with the remedē® System. Eur J Heart Fail. 2016;18(11):1386–93.

    CrossRef  PubMed  Google Scholar 

  53. Weber B, Dijkman PE, Scherman J, Sanders B, Emmert MY, Grünenfelder J, et al. Off-the-shelf human decellularized tissue-engineered heart valves in a non-human primate model. Biomaterials. 2013;34(30):7269–80.

    CrossRef  CAS  PubMed  Google Scholar 

  54. Duan B, Kapetanovic E, Hockaday LA, Butcher JT. Three-dimensional printed trileaflet valve conduits using biological hydrogels and human valve interstitial cells. Acta Biomater. 2014;10(5):1836–46.

    CrossRef  CAS  PubMed  Google Scholar 

  55. Truskey GA. Advancing cardiovascular tissue engineering. F1000Research [Internet]. 2016 May 31 [cited 2017 Nov 27];5. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4890312/.

  56. Ross HJ, Law Y, Book WM, Broberg CS, Burchill L, Cecchin F, et al. Transplantation and mechanical circulatory support in congenital heart disease a scientific statement from the American Heart Association. Circulation. 2016;133(8):802–20.

    PubMed  Google Scholar 

  57. Fahed AC, Gelb BD, Seidman JG, Seidman CE. Genetics of congenital heart disease: the glass half empty. Circ Res. 2013;112(4):707–20.

    CrossRef  CAS  PubMed  Google Scholar 

  58. Elliott P, Andersson B, Arbustini E, Bilinska Z, Cecchi F, Charron P, et al. Classification of the cardiomyopathies: a position statement from the European Society Of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J. 2008 Jan;29(2):270–6.

    CrossRef  PubMed  Google Scholar 

  59. Yang J, Xu W-W, Hu S-J. Heart failure: advanced development in genetics and epigenetics. Biomed Res Int. 2015;2015:352734.

    PubMed  PubMed Central  Google Scholar 

  60. Preuss C, Andelfinger G. Genetics of heart failure in congenital heart disease. Can J Cardiol. 2013;29(7):803–10.

    CrossRef  PubMed  Google Scholar 

  61. Postma AV, van Engelen K, van de Meerakker J, Rahman T, Probst S, Baars MJH, et al. Mutations in the sarcomere gene MYH7 in Ebstein anomaly. Circ Cardiovasc Genet. 2011 Feb;4(1):43–50.

    CrossRef  CAS  PubMed  Google Scholar 

  62. Lopes LR, Elliott PM. Genetics of heart failure. Biochim Biophys Acta. 2013;1832(12):2451–61.

    CrossRef  CAS  PubMed  Google Scholar 

  63. Kirwan JR, de Wit M, Frank L, Haywood KL, Salek S, Brace-McDonnell S, et al. Emerging guidelines for patient engagement in research. Value Health. 2017;20(3):481–6.

    CrossRef  PubMed  Google Scholar 

  64. Nelson EC, Eftimovska E, Lind C, Hager A, Wasson JH, Lindblad S. Patient reported outcome measures in practice. BMJ. 2015;350:g7818.

    CrossRef  PubMed  Google Scholar 

  65. Moons P, Kovacs AH, Luyckx K, Thomet C, Budts W, Enomoto J, et al. Patient-reported outcomes in adults with congenital heart disease: inter-country variation, standard of living and healthcare system factors. Int J Cardiol. 2017;251:34–41.

    CrossRef  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital and Imperial College London, London, UK

    Konstantinos Dimopoulos

  2. Centre for Bioinformatics and System Biology, Imperial College London, London, UK

    Alessia David

Authors
  1. Konstantinos Dimopoulos
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Alessia David
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Royal Brompton Hospital, London, United Kingdom

    Lorna Swan

  2. ATTIKON University Hospital, Athens University, Athens, Greece

    Alexandra A. Frogoudaki

Rights and permissions

Reprints and Permissions

Copyright information

© 2018 Springer International Publishing AG, part of Springer Nature

About this chapter

Verify currency and authenticity via CrossMark

Cite this chapter

Dimopoulos, K., David, A. (2018). Future Research. In: Swan, L., Frogoudaki, A. (eds) Heart Failure in Adult Congenital Heart Disease. Congenital Heart Disease in Adolescents and Adults. Springer, Cham. https://doi.org/10.1007/978-3-319-77803-7_15

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-77803-7_15

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-77802-0

  • Online ISBN: 978-3-319-77803-7

  • eBook Packages: MedicineMedicine (R0)