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Novel Approaches to the Failing Congenital Heart

  • Congenital Heart Disease (RA Krasuski and G Fleming, Section Editors)
  • Published:
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Abstract

Purpose of Review

Heart failure is the leading cause of morbidity and mortality in adults with congenital heart disease and is characterized by a variety of underlying mechanisms. Here, we aim to elaborate on the medical and technological advancements in the management of heart failure in adult patients with congenital heart disease and highlight the use of imaging modalities to guide therapy.

Recent Findings

There have been several advances over the past decade with angiotensin receptor neprilysin and sodium-glucose cotransporter-2 inhibitors, atrioventricular valve clips, transcatheter pulmonary valves, catheter ablation, and cardiac resynchronization therapy, as well as the introduction of lymphatic interventions. Expanded use of echocardiography, cardiac magnetic resonance imaging, and cardiac computed tomography has guided many of these therapies.

Summary

Significant innovations in the management of heart failure in adults with congenital heart disease have evolved with advancements in imaging modalities playing a critical role in guiding treatment therapies.

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Abbreviations

ACC:

American College of Cardiology

ACE:

Angiotensin converting enzyme inhibitor

ACHD:

Adults with congenital heart disease

AHA:

American Heart Association

AP:

Anatomic and physiological

ARB:

Angiotensin II receptor blocker

ARNi:

Angiotensin Receptor Neprilysin Inhibitor

AV:

Atrioventricular

CMRI:

Cardiac magnetic resonance imaging

CRT:

Cardiac resynchronization therapy

CT:

Computed tomography

DCMRL:

Dynamic contrast enhanced magnetic resonance lymphangiogram

EDVi:

End diastolic volume indexed

GDMT:

Guideline directed medical therapy

HFSA:

Heart Failure Society of America

NT-proBNP:

N-terminal pro brain natriuretic peptides

NYHA:

New York Heart Association

SGLT2i:

Sodium-glucose cotransporter-2 inhibitors

SVC:

Superior vena cava

TEER:

Transcatheter edge-to-edge repair

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Gilboa SM, Devine OJ, Kucik JE, Oster ME, Riehle-Colarusso T, Nembhard WN, et al. Congenital heart defects in the United States: estimating the magnitude of the affected population in 2010. Circulation. 2016;134(2):101–9. https://doi.org/10.1161/CIRCULATIONAHA.115.019307.

    Article  PubMed  PubMed Central  Google Scholar 

  2. Ross HJ, Law Y, Book WM, Broberg CS, Burchill L, Cecchin F, et al. American Heart Association Adults With Congenital Heart Disease Committee of the Council on Clinical Cardiology and Council on Cardiovascular Disease in the Young, the Council on Cardiovascular Radiology and Intervention, and the Council on Functional Genomics and Translational Biology. Transplantation and mechanical circulatory support in congenital heart disease: a scientific statement from the American Heart Association. Circulation. 2016;133(8):802–20. https://doi.org/10.1161/CIR.0000000000000353.

  3. Stout KK, Broberg CS, Book WM, Cecchin F, Chen JM, Dimopoulos K, et al. American Heart Association Council on Clinical Cardiology, Council on Functional Genomics and Translational Biology, and Council on Cardiovascular Radiology and Imaging. Chronic heart failure in congenital heart disease: a scientific statement from the American Heart Association. Circulation. 2016;133(8):770–801. https://doi.org/10.1161/CIR.0000000000000352.

  4. Heidenreich PA, Bozkurt B, Aguilar D, Allen LA, Byun JJ, Colvin MM, et al. 2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure: Executive Summary: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2022;145(18):e876–94. https://doi.org/10.1161/CIR.0000000000001062.

    Article  PubMed  Google Scholar 

  5. Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019;139(14):e698–800. https://doi.org/10.1161/CIR.0000000000000603.

    Article  PubMed  Google Scholar 

  6. Givertz MM, DeFilippis EM, Landzberg MJ, Pinney SP, Woods RK, Valente AM. Advanced heart failure therapies for adults with congenital heart disease: JACC state-of-the-art review. J Am Coll Cardiol. 2019;74(18):2295–312. https://doi.org/10.1016/j.jacc.2019.09.004.

    Article  PubMed  Google Scholar 

  7. Crossland DS, Van De Bruaene A, Silversides CK, Hickey EJ, Roche SL. Heart failure in adult congenital heart disease: from advanced therapies to end-of-life care. Can J Cardiol. 2019;35(12):1723–39. https://doi.org/10.1016/j.cjca.2019.07.626.

    Article  PubMed  Google Scholar 

  8. Nagata Y, Wu VC, Kado Y, Otani K, Lin FC, Otsuji Y, et al. Prognostic value of right ventricular ejection fraction assessed by transthoracic 3D echocardiography. Circ Cardiovasc Imaging. 2017;10(2):e005384. https://doi.org/10.1161/CIRCIMAGING.116.005384.

  9. Geva T. Repaired tetralogy of Fallot: the roles of cardiovascular magnetic resonance in evaluating pathophysiology and for pulmonary valve replacement decision support. J Cardiovasc Magn Reson. 2011;13(1):9. https://doi.org/10.1186/1532-429X-13-9.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Lewis MJ, Van Dissel A, Kochav J, DiLorenzo MP, Ginns J, Zemer-Wassercug N, et al. Cardiac MRI predictors of adverse outcomes in adults with a systemic right ventricle. ESC Heart Fail. 2022;9(2):834–41. https://doi.org/10.1002/ehf2.13745.

    Article  PubMed  PubMed Central  Google Scholar 

  11. Meyer SL, St Clair N, Powell AJ, Geva T, Rathod RH. Integrated clinical and magnetic resonance imaging assessments late after Fontan operation. J Am Coll Cardiol. 2021;77(20):2480–9. https://doi.org/10.1016/j.jacc.2021.03.312.

    Article  PubMed  Google Scholar 

  12. Fogel MA, Anwar S, Broberg C, Browne L, Chung T, Johnson T, et al. Society for Cardiovascular Magnetic Resonance/European Society of Cardiovascular Imaging/American Society of Echocardiography/Society for Pediatric Radiology/North American Society for Cardiovascular Imaging Guidelines for the use of cardiovascular magnetic resonance in pediatric congenital and acquired heart disease: endorsed by The American Heart Association. J Cardiovasc Magn Reson. 2022;24(1):37. https://doi.org/10.1186/s12968-022-00843-7.

  13. •• Egbe AC, Miranda WR, Pellikka PA, DeSimone CV, Connolly HM. Prevalence and prognostic implications of left ventricular systolic dysfunction in adults with congenital heart disease. J Am Coll Cardiol. 2022;79(14):1356–65. https://doi.org/10.1016/j.jacc.2022.01.040. This article demonstrates how guideline directed medical therapy used in adults with congenital heart disease with systemic left ventricles does lead to improvement in ventricular function.

  14. van der Bom T, 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. https://doi.org/10.1161/CIRCULATIONAHA.112.135392.

    Article  CAS  PubMed  Google Scholar 

  15. van Dissel AC, Winter MM, van der Bom T, Vliegen HW, van Dijk APJ, Pieper PG, et al. Long-term clinical outcomes of valsartan in patients with a systemic right ventricle: follow-up of a multicenter randomized controlled trial. Int J Cardiol. 2019;278:84–7. https://doi.org/10.1016/j.ijcard.2018.11.027.

    Article  PubMed  Google Scholar 

  16. Tutarel O, Meyer GP, Bertram H, Wessel A, Schieffer B, Westhoff-Bleck M. Safety and efficiency of chronic ACE inhibition in symptomatic heart failure patients with a systemic right ventricle. Int J Cardiol. 2012;154(1):14–6. https://doi.org/10.1016/j.ijcard.2010.08.068.

    Article  PubMed  Google Scholar 

  17. Lester SJ, McElhinney DB, Viloria E, Reddy GP, Ryan E, Tworetzky W, et al. Effects of losartan in patients with a systemically functioning morphologic right ventricle after atrial repair of transposition of the great arteries. Am J Cardiol. 2001;88(11):1314–6. https://doi.org/10.1016/s0002-9149(01)02098-7.

    Article  CAS  PubMed  Google Scholar 

  18. 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. https://doi.org/10.1016/j.ijcard.2008.04.056.

    Article  PubMed  Google Scholar 

  19. Dore A, Houde C, Chan KL, Ducharme A, Khairy P, Juneau M, et al. Angiotensin receptor blockade and exercise capacity in adults with systemic right ventricles: a multicenter, randomized, placebo-controlled clinical trial. Circulation. 2005;112(16):2411–6. https://doi.org/10.1161/CIRCULATIONAHA.105.543470.

    Article  CAS  PubMed  Google Scholar 

  20. Hechter SJ, Fredriksen PM, Liu P, Veldtman G, Merchant N, Freeman M, et al. Angiotensin-converting enzyme inhibitors in adults after the Mustard procedure. Am J Cardiol. 2001;87(5):660–3, A11. https://doi.org/10.1016/s0002-9149(00)01452-1.

  21. Ladouceur M, Segura de la Cal T, Gaye B, Valentin E, Ly R, Iserin L, et al. Effect of medical treatment on heart failure incidence in patients with a systemic right ventricle. Heart. 2021;107(17):1384–9. https://doi.org/10.1136/heartjnl-2020-318787.

  22. Doughan AR, McConnell ME, Book WM. Effect of beta blockers (carvedilol or metoprolol XL) in patients with transposition of great arteries and dysfunction of the systemic right ventricle. Am J Cardiol. 2007;99(5):704–6. https://doi.org/10.1016/j.amjcard.2006.10.025.

    Article  CAS  PubMed  Google Scholar 

  23. Giardini A, Lovato L, Donti A, Formigari R, Gargiulo G, Picchio FM, et al. A pilot study on the effects of carvedilol on right ventricular remodelling and exercise tolerance in patients with systemic right ventricle. Int J Cardiol. 2007;114(2):241–6. https://doi.org/10.1016/j.ijcard.2006.01.048.

    Article  PubMed  Google Scholar 

  24. Bouallal R, Godart F, Francart C, Richard A, Foucher-Hossein C, Lions C. Interest of β-blockers in patients with right ventricular systemic dysfunction. Cardiol Young. 2010;20(6):615–9. https://doi.org/10.1017/S1047951110000764.

    Article  PubMed  Google Scholar 

  25. Josephson CB, Howlett JG, Jackson SD, Finley J, Kells CM. A case series of systemic right ventricular dysfunction post atrial switch for simple D-transposition of the great arteries: the impact of beta-blockade. Can J Cardiol. 2006;22(9):769–72. https://doi.org/10.1016/s0828-282x(06)70293-8.

    Article  PubMed  PubMed Central  Google Scholar 

  26. Dos L, Pujadas S, Estruch M, Mas A, Ferreira-González I, Pijuan A, et al. Eplerenone in systemic right ventricle: double blind randomized clinical trial. The evedes study. Int J Cardiol. 2013;168(6):5167–73. https://doi.org/10.1016/j.ijcard.2013.07.163.

    Article  PubMed  Google Scholar 

  27. McMurray JJ, Packer M, Desai AS, Gong J, Lefkowitz MP, Rizkala AR, et al. PARADIGM-HF Investigators and Committees. Angiotensin-neprilysin inhibition versus enalapril in heart failure. N Engl J Med. 2014;371(11):993–1004. https://doi.org/10.1056/NEJMoa1409077.

  28. Nederend M, Kiès P, Regeer MV, Vliegen HW, Mertens BJ, Robbers-Visser D, et al. Tolerability and beneficial effects of sacubitril/valsartan on systemic right ventricular failure. Heart. 2023;11:heartjnl-2022–322332. https://doi.org/10.1136/heartjnl-2022-322332.

  29. Fusco F, Scognamiglio G, Merola A, Iannuzzi A, Palma M, Grimaldi N, et al. Safety and efficacy of sacubitril/valsartan in patients with a failing systemic right ventricle: a prospective single-center study. Circ Heart Fail. 2023;16(2):e009848. https://doi.org/10.1161/CIRCHEARTFAILURE.122.009848.

  30. Appadurai V, Nicolae M, Aboulhosn J, Lluri G. The protocol and rationale for the efficacy aNd ToleRability of sacUbitril-valSarTan in adult congenital heart disease patients with heart failure (ENTRUST ACHD HF) registry. Int J Cardiol Congenit Heart Dis. 2021;3:100096. https://doi.org/10.1016/j.ijcchd.2021.100096.

  31. Appadurai V, Thoreau J, Malpas T, Nicolae M. Sacubitril/valsartan in adult congenital heart disease patients with chronic heart failure - a single centre case series and call for an international registry. Heart Lung Circ. 2020;29(1):137–41. https://doi.org/10.1016/j.hlc.2018.12.003.

    Article  PubMed  Google Scholar 

  32. Lluri G, Lin J, Reardon L, Miner P, Whalen K, Aboulhosn J. Early experience with sacubitril/valsartan in adult patients with congenital heart disease. World J Pediatr Congenit Heart Surg. 2019;10(3):292–5. https://doi.org/10.1177/2150135119825599.

    Article  PubMed  Google Scholar 

  33. Maurer SJ, Pujol Salvador C, Schiele S, Hager A, Ewert P, Tutarel O. Sacubitril/valsartan for heart failure in adults with complex congenital heart disease. Int J Cardiol. 2020;300:137–40. https://doi.org/10.1016/j.ijcard.2019.06.031.

    Article  PubMed  Google Scholar 

  34. Zandstra TE, Nederend M, Jongbloed MRM, Kiès P, Vliegen HW, Bouma BJ, et al. Sacubitril/valsartan in the treatment of systemic right ventricular failure. Heart. 2021;107(21):1725–30. https://doi.org/10.1136/heartjnl-2020-318074.

    Article  CAS  PubMed  Google Scholar 

  35. Yan L, Loh JK, Tan JL. Sacubitril/valsartan for heart failure in patients with complex adult congenital heart disease – experience from a tertiary centre in Singapore. Int J Cardiol Congenit Heart Dis. 2021;6:100268. https://doi.org/10.1016/j.ijcchd.2021.100268.

  36. Andi K, Abozied O, Miranda WR, Anderson JH, Connolly HM, Jain CC, et al. Clinical benefits of angiotensin receptor-neprilysin inhibitor in adults with congenital heart disease. Int J Cardiol. 2023;387:131152. https://doi.org/10.1016/j.ijcard.2023.131152.

  37. Striepe K, Jumar A, Ott C, Karg MV, Schneider MP, Kannenkeril D, et al. Effects of the selective sodium-glucose cotransporter 2 inhibitor empagliflozin on vascular function and central hemodynamics in patients with type 2 diabetes mellitus. Circulation. 2017;136(12):1167–9. https://doi.org/10.1161/CIRCULATIONAHA.117.029529.

    Article  CAS  PubMed  Google Scholar 

  38. McMurray JJV, Solomon SD, Inzucchi SE, Køber L, Kosiborod MN, Martinez FA, et al. DAPA-HF trial committees and investigators. dapagliflozin in patients with heart failure and reduced ejection fraction. N Engl J Med. 2019;381(21):1995–2008. https://doi.org/10.1056/NEJMoa1911303.

  39. Muneuchi J, Sugitani Y, Kobayashi M, Ezaki H, Yamada H, Watanabe M. Feasibility and safety of sodium glucose cotransporter-2 inhibitors in adults with heart failure after the Fontan procedure. Case Rep Cardiol. 2022;2022:5243594. https://doi.org/10.1155/2022/5243594.

    Article  PubMed  PubMed Central  Google Scholar 

  40. Saef J, Sundaravel S, Ortega-Legaspi J, Vaikunth S. Safety and treatment experience with sodium/glucose cotransporter-2 inhibitors in adult patients with congenital heart disease. J Card Fail. 2023;29(6):974–5. https://doi.org/10.1016/j.cardfail.2023.03.011.

    Article  PubMed  Google Scholar 

  41. Neijenhuis RML, Nederend M, Jongbloed MRM, Kiès P, Rotmans JI, Vliegen HW, et al. The potential of sodium-glucose cotransporter 2 inhibitors for the treatment of systemic right ventricular failure in adults with congenital heart disease. Front Cardiovasc Med. 2023;10:1093201. https://doi.org/10.3389/fcvm.2023.1093201.

    Article  PubMed  PubMed Central  Google Scholar 

  42. •• Hansen JH, Duong P, Jivanji SGM, Jones M, Kabir S, Butera G, et al. Transcatheter correction of superior sinus venosus atrial septal defects as an alternative to surgical treatment. J Am Coll Cardiol. 2020;75(11):1266–78. https://doi.org/10.1016/j.jacc.2019.12.070. This article details the experience of the Evelina Children’s Hospital with transcatheter correction of superior sinus venosus defects.

  43. Haeffele CL, Lui GK, Peng L, Chan F, Sharma RP. First described mitral clip in an adult extracardiac Fontan patient: a case report. Eur Heart J Case Rep. 2022;7(1):ytac479. https://doi.org/10.1093/ehjcr/ytac479.

  44. Blusztein D, Moore P, Qasim A, Mantri N, Mahadevan VS. Transcatheter edge-to-edge repair of systemic tricuspid valve in extracardiac Fontan circulation: first in human. JACC Case Rep. 2022;4(4):221–5. https://doi.org/10.1016/j.jaccas.2021.12.024.

    Article  PubMed  PubMed Central  Google Scholar 

  45. Luedike P, Riebisch M, Weymann A, Ruhparwar A, Rassaf T, Mahabadi AA. Feasibility of a novel transcatheter valve repair system to treat tricuspid regurgitation in ccTGA. JACC Case Rep. 2021;3(6):893–6. https://doi.org/10.1016/j.jaccas.2021.04.024.

    Article  PubMed  PubMed Central  Google Scholar 

  46. Alshawabkeh L, Mahmud E, Reeves R. Percutaneous mitral valve repair in adults with congenital heart disease: report of the first case-series. Catheter Cardiovasc Interv. 2021;97(3):542–8. https://doi.org/10.1002/ccd.29238.

    Article  PubMed  Google Scholar 

  47. Iriart X, Guérin P, Jalal Z, Thambo JB. Edge to edge repair using a MitraClip for severe tricuspid valve regurgitation after a Mustard operation. Catheter Cardiovasc Interv. 2021;98(1):E108–14. https://doi.org/10.1002/ccd.29681.

    Article  PubMed  Google Scholar 

  48. Lewis A, Hakemi E, Lopez D, Cubeddu RJ. MitraClip repair of right-sided atrioventricular valve in a patient with congenitally corrected transposition of the great arteries: a case report. Eur Heart J Case Rep. 2021;6(1):ytab479. https://doi.org/10.1093/ehjcr/ytab479.

  49. Gaydos SS, Capps CD, Judd RN, Rhodes JF, Steinberg DH, Katz MR, et al. Hemodynamic impact of MitraClip procedure for systemic tricuspid regurgitation in congenitally corrected transposition of great arteries: a case report. Cardiovasc Revasc Med. 2021;28S:114–7. https://doi.org/10.1016/j.carrev.2020.08.034.

    Article  PubMed  Google Scholar 

  50. Tan W, Calfon Press M, Lluri G, Aboulhosn J. Percutaneous edge-to-edge repair for common atrioventricular valve regurgitation in a patient with heterotaxy syndrome, single ventricle physiology, and unbalanced atrioventricular septal defect. Catheter Cardiovasc Interv. 2020;96(2):384–8. https://doi.org/10.1002/ccd.28782.

    Article  PubMed  Google Scholar 

  51. Russo MJ, Garg A, Okoh A, Chaudhary A, Hakeem A, Lee LY, et al. MitraClip implantation in a patient with post-surgical repair of primum atrial septal defect and residual mitral cleft. JACC Case Rep. 2020;2(12):2027–9. https://doi.org/10.1016/j.jaccas.2020.07.055.

    Article  PubMed  PubMed Central  Google Scholar 

  52. Picard F, Tadros VX, Asgar AW. From tricuspid to double orifice Morphology: Percutaneous tricuspid regurgitation repair with the MitraClip device in congenitally corrected-transposition of great arteries. Catheter Cardiovasc Interv. 2017;90(3):432–6. https://doi.org/10.1002/ccd.26834.

    Article  PubMed  Google Scholar 

  53. van Melle JP, Schurer R, Willemsen M, Hoendermis ES, van den Heuvel AF. Percutaneous tricuspid valve repair using MitraClip® for the treatment of severe tricuspid valve regurgitation in a patient with congenitally corrected transposition of the great arteries. Neth Heart J. 2016;24(11):696–7. https://doi.org/10.1007/s12471-016-0866-y.

    Article  PubMed  PubMed Central  Google Scholar 

  54. Franzen O, von Samson P, Dodge-Khatami A, Geffert G, Baldus S. Percutaneous edge-to-edge repair of tricuspid regurgitation in congenitally corrected transposition of the great arteries. Congenit Heart Dis. 2011;6(1):57–9. https://doi.org/10.1111/j.1747-0803.2010.00428.x.

    Article  PubMed  Google Scholar 

  55. Khairy P, Van Hare GF, Balaji S, Berul CI, Cecchin F, Cohen MI, et al. PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in Adult Congenital Heart Disease: developed in partnership between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American College of Cardiology (ACC), the American Heart Association (AHA), the European Heart Rhythm Association (EHRA), the Canadian Heart Rhythm Society (CHRS), and the International Society for Adult Congenital Heart Disease (ISACHD). Heart Rhythm. 2014;11(10):e102–65. https://doi.org/10.1016/j.hrthm.2014.05.009.

  56. Venkatesh P, Evans AT, Maw AM, Pashun RA, Patel A, Kim L, et al. Predictors of late mortality in D-transposition of the great arteries after atrial switch repair: systematic review and meta-analysis. J Am Heart Assoc. 2019;8(21):e012932. https://doi.org/10.1161/JAHA.119.012932.

  57. Kamp AN, Nair K, Fish FA, Khairy P. Catheter ablation of atrial arrhythmias in patients post-Fontan. Can J Cardiol. 2022;38(7):1036–47. https://doi.org/10.1016/j.cjca.2022.02.023.

    Article  PubMed  Google Scholar 

  58. •• Moore JP, Marelli A, Burchill LJ, Chubb H, Roche SL, Cedars AM, et al. Management of heart failure with arrhythmia in adults with congenital heart disease: JACC state-of-the-art review. J Am Coll Cardiol. 2022;80(23):2224–38. https://doi.org/10.1016/j.jacc.2022.09.038. This review details the management of arrhythmia and heart failure in adults with congenital heart disease and comments on the role of anti-arrhythmic medications, catheter and surgical interventions, resynchronization, sudden cardiac death and transplantation.

  59. Fagan TE, Truong UT, Jone PN, Bracken J, Quaife R, Hazeem AA, et al. Multimodality 3-dimensional image integration for congenital cardiac catheterization. Methodist Debakey Cardiovasc J. 2014;10(2):68–76. https://doi.org/10.14797/mdcj-10-2-68.

  60. Soto-Iglesias D, Penela D, Jáuregui B, Acosta J, Fernández-Armenta J, Linhart M, et al. Cardiac magnetic resonance-guided ventricular tachycardia substrate ablation. JACC Clin Electrophysiol. 2020;6(4):436–47. https://doi.org/10.1016/j.jacep.2019.11.004.

    Article  PubMed  Google Scholar 

  61. Bhagirath P, van der Graaf M, Karim R, Rhode K, Piorkowski C, Razavi R, et al. Interventional cardiac magnetic resonance imaging in electrophysiology: advances toward clinical translation. Circ Arrhythm Electrophysiol. 2015;8(1):203–11. https://doi.org/10.1161/CIRCEP.114.002371.

    Article  PubMed  Google Scholar 

  62. Koyak Z, de Groot JR, Krimly A, Mackay TM, Bouma BJ, Silversides CK, et al. Cardiac resynchronization therapy in adults with congenital heart disease. Europace. 2018;20(2):315–22. https://doi.org/10.1093/europace/euw386.

    Article  PubMed  Google Scholar 

  63. Chubb H, Rosenthal DN, Almond CS, Ceresnak SR, Motonaga KS, Arunamata AA, et al. Impact of cardiac resynchronization therapy on heart transplant-free survival in pediatric and congenital heart disease patients. Circ Arrhythm Electrophysiol. 2020;13(4):e007925. https://doi.org/10.1161/CIRCEP.119.007925.

  64. Kharbanda RK, Moore JP, Lloyd MS, Galotti R, Bogers AJJC, Taverne YJHJ, et al. Cardiac resynchronization therapy for adult patients with a failing systemic right ventricle: a multicenter study. J Am Heart Assoc. 2022;11(22):e025121. https://doi.org/10.1161/JAHA.121.025121.

  65. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971;26(3):240–8. https://doi.org/10.1136/thx.26.3.240.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  66. Rychik J, Atz AM, Celermajer DS, Deal BJ, Gatzoulis MA, Gewillig MH, et al. American Heart Association Council on Cardiovascular Disease in the Young and Council on Cardiovascular and Stroke Nursing. Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association. Circulation. 2019;140(6):e234–84. https://doi.org/10.1161/CIR.0000000000000696.

  67. Mossad EB, Motta P, Vener DF. Anesthetic considerations for adults undergoing fontan conversion surgery. Anesthesiol Clin. 2013;31(2):405–19. https://doi.org/10.1016/j.anclin.2012.12.005.

    Article  PubMed  Google Scholar 

  68. • Book WM, Gerardin J, Saraf A, Marie Valente A, Rodriguez F 3rd. Clinical phenotypes of fontan failure: implications for management. Congenit Heart Dis. 2016;11(4):296–308. https://doi.org/10.1111/chd.12368. This articles proposes a manner to categorize 4 different types of Fontan failure- those with reduced ejection fraction, preserved ejection fraction, normal hemodynamics, and lymphatic dysfunction.

  69. Dori Y, Mazurek J, Birati E, Smith C. Ascites in animals with right heart failure: correlation with lymphatic dysfunction. J Am Heart Assoc. 2023;12(7):e026984. https://doi.org/10.1161/JAHA.122.026984.

  70. •• Dori Y, Keller MS, Rome JJ, Gillespie MJ, Glatz AC, Dodds K, et al. Percutaneous lymphatic embolization of abnormal pulmonary lymphatic flow as treatment of plastic bronchitis in patients with congenital heart disease. Circulation. 2016;133(12):1160–70. https://doi.org/10.1161/CIRCULATIONAHA.115.019710. This article highlights the revolutionary treatment of plastic bronchitis via lymphatic intervention.

  71. Rychik J, Dodds KM, Goldberg D, Glatz AC, Fogel M, Rossano J, et al. Protein losing enteropathy after fontan operation: glimpses of clarity through the lifting fog. World J Pediatr Congenit Heart Surg. 2020;11(1):92–6. https://doi.org/10.1177/2150135119890555.

    Article  PubMed  Google Scholar 

  72. Biko DM, DeWitt AG, Pinto EM, Morrison RE, Johnstone JA, Griffis H, et al. MRI evaluation of lymphatic abnormalities in the neck and thorax after fontan surgery: relationship with outcome. Radiology. 2019;291(3):774–80. https://doi.org/10.1148/radiol.2019180877.

    Article  PubMed  Google Scholar 

  73. Smith CL, Hoffman TM, Dori Y, Rome JJ. Decompression of the thoracic duct: a novel transcatheter approach. Catheter Cardiovasc Interv. 2020;95(2):E56–61. https://doi.org/10.1002/ccd.28446.

    Article  PubMed  Google Scholar 

  74. Gartenberg AJ, Gillespie MJ, Glatz AC. Transcatheter approaches to pulmonary valve replacement in congenital heart disease: revolutionizing the management of RVOT dysfunction? Semin Thorac Cardiovasc Surg. 2023 Summer;35(2):333–8. https://doi.org/10.1053/j.semtcvs.2022.02.009.

  75. Vaikunth S, Sundaravel S, Saef J, Ortega-Legaspi J. Novel therapeutic strategies in heart failure in adult congenital heart disease: of medicines and devices. Curr Heart Fail Rep. 2023. https://doi.org/10.1007/s11897-023-00621-1.

    Article  PubMed  Google Scholar 

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Acknowledgements

This work was supported by the Research Creative Services at the Children’s Hospital of Philadelphia Research Institute.

Funding

Sumeet Vaikunth has received grant funding from Abbott to support the Adult Congenital Heart Disease Fellowship program at the University of Pennsylvania.

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Authors and Affiliations

  1. Philadelphia Adult Congenital Heart Center, Penn Medicine & Children’s Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA, USA

    Bruke A. Tedla, Yuli Y. Kim & Sumeet Vaikunth

  2. Perelman Center for Advanced Medicine, 11th Floor, South Pavilion, 3400 Civic Center Boulevard, Philadelphia, PA, 19104-5127, USA

    Sumeet Vaikunth

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  1. Bruke A. Tedla
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  2. Yuli Y. Kim
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Correspondence to Sumeet Vaikunth.

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Tedla, B.A., Kim, Y.Y. & Vaikunth, S. Novel Approaches to the Failing Congenital Heart. Curr Cardiol Rep 25, 1633–1647 (2023). https://doi.org/10.1007/s11886-023-01979-3

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