Quadruple therapy for heart failure with reduced ejection fraction of the systemic right ventricle and tricuspid regurgitation in a child

Keywords: transposition of the great arteries, Mustard’s operation, atrial switch technique, chronic heart failure, congenital heart disease

Abstract

Transposition of the Great Arteries (d-TMA) is a congenital heart defect with unrelated lung and systemic circulation. More than 90% of infants die without surgical treatment in the first year of life. Anatomical correction is the “gold standard”, however, atrial switch technique (Mustard or Senning procedure) can be used in selected patients. The long-term result of the atrial switch procedure is characterized by the development of heart failure with a decrease of systemic right ventricular function, tricuspid valve insufficiency, cardiac arrhythmias, etc. These complications may occur in childhood, but medication and its features remain unclear. This paper presents a case of successful quadruple therapy in chronic heart failure with reduced ejection fraction of the systemic right ventricle in a child after Mustard procedure, followed by successful tricuspid replacement.

Author Biographies

I.A. Vishnyakova, Bakoulev National Medical Research Center for Cardiovascular Surgery

Resident Physician

A.V. Minaev, Bakoulev National Medical Research Center for Cardiovascular Surgery

Dr. Med. Sci., Senior Researcher

I.A. Kovalev, Veltischev Research and Clinical Institute for Pediatrics and Pediatric Surgery of the N.I. Pirogov Russian National Research Medical University

Dr. Med. Sci., Professor

T.Yu. Danilov, Bakoulev National Medical Research Center for Cardiovascular Surgery

Dr. Med. Sci., Chief Researcher

References

Khairy P. Sudden cardiac death in adults with transposition of the great arteries and systemic right ventricles: preventing (night) MAREs. Eur. Heart J. 2022; 43 (28): 2695–2697. DOI: 10.1093/eurheartj/ehac228

Sabbah B.N., Arabi T.Z., Shafqat A., Abdul Rab S., Razak A., Albert-Biotons D.C. Heart failure in systemic right ventricle: mechanisms and therapeutic options. Front Cardiovasc. Med. 2023; 9: 1064196. DOI: 10.3389/fcvm.2022.1064196

Голухова Е.З., Ким А.И., Завалихина Т.В., Нефедова И.Е., Черногривов А.Е., Авакова С.А. Анализ оказания медицинской помощи детям с врожденными пороками сердца в Российской Федерации и предпосылки к созданию регистра в современную эру цифровых медицинских информационных систем. Креативная кардиология. 2023; 17 (3): 315–321. DOI: 10.24022/1997-3187-2023-17-3-315-321

Bevilacqua F., Pasqualin G., Ferrero P., Micheletti A., Negura D.G., D’Aiello A.F. et al. Overview of long-term outcome in adults with systemic right ventricle and transposition of the great arteries: a review. Diagnostics (Basel). 2023; 13 (13): 2205. DOI: 10.3390/diagnostics13132205

Mee R.B.B. Severe right ventricular failure after Mustard or Senning operation. Two-stage repair: pulmonary artery banding and switch. J. Thorac. Cardiovasc. Surg. 1986; 92 (3): 385–390.

Минаев А.В. Особенности течения корригированных пороков конотрункуса сердца с бивентрикулярной гемодинамикой у взрослых пациентов. Сердечно-сосудистые заболевания. Бюллетень НЦССХ им. А.Н. Бакулева РАМН. 2019; 20 (4): 324–333. DOI: 10.24022/1810-0694-2019-20-4-324-333

Duncan B.W., Poirier N.C., Mee R.B., Drummond-Webb J.J., Qureshi A., Mesia C.I. et al. Selective timing for the arterial switch operation. Ann. Thorac. Surg. 2004; 77: 1691–1696.

Jaggers J.J., Cameron D.E., Herlong J.R., Ungerleider R.M. Congenital Heart Surgery Nomenclature and Database Project: transposition of the great arteries. Ann. Thorac. Surg. 2000; 69 (4 Suppl): 205–235. DOI: 10.1016/s0003-4975(99)01282-5

George E.S., Christian B., Pipina B., Juan V.C., Eduardo C., Luca D.C. et al. Clinical guidelines for the management of patients with transposition of the great arteries with intact ventricular septum. Eur. J. Cardio-Thorac. Surg. 2017; 51 (1): 1–32. DOI: 10.1093/ejcts/ezw360

Parker N.M., Zuhdi M., Kouatli A., Baslaim G. Late presenters with dextro-transposition of great arteries and intact ventricular septum: to train or not to train the left ventricle for arterial switch operation? Congenit. Heart Dis. 2009; 4 (6): 424–432. DOI: 10.1111/j.1747-0803.2009.00352.x

Lacour-Gayet F., Piot D., Zoghbi J., Serraf A., Gruber P., Mace L. et al. Surgical management and indication of left ventricular retraining in arterial switch for transposition of the great arteries with intact ventricular septum. Eur. J. Cardiothorac. Surg. 2001; 20: 824–829.

Акилов Х.А., Пирназаров Ж.Т., Алимов А.Б. Оптимизация диагностического протокола при врожденных пороках сердца, сопровождающихся неотложными состояниями, на основе анализа госпитальной летальности. Детские болезни сердца и сосудов. 2024; 21 (1): 30–40. DOI: 10.24022/1810-0686-2024-21-1-30-40

Апханова Т.В., Барышникова И.Ю., Бокерия Л.А. Транспозиция магистральных артерий. Клинические рекомендации 2024. Министерство здравоохранения РФ. М.; 2024.

Jonas R.A., Giglia T.M., Sanders S.P., Wernovsky G., Nadal-Ginard B., Mayer J.J. et al. Rapid, two-stage arterial switch for transposition of the great arteries and intact ventricular septum beyond the neonatal period. Circulation. 1989; 80: 203–208.

Yacoub M.H., Radley-Smith R., Maclaurin R. Two stage operation for anatomical correction of transposition of the great arteries with intact ventricular septum. Lancet. 1977; 1: 1275–1278.

Nakazawa M., Oyama K., Imai Y., Nojima K., Aotsuka H., Satomi G. et al. Criteria for two-staged arterial switch operation for simple transposition of great arteries. Circulation. 1988; 78: 124–131.

Борисков М.В., Ткаченко И.А. Эволюция хирургического лечения простой транспозиции магистральных артерий. Детские болезни сердца и сосудов. 2022; 19 (1): 5–12. DOI: 10.24022/1810-0686-2022-19-1-5-12

Барышникова И.Ю., Шарипов Р.А., Калашников С.В., Нефедова И.Е., Беришвили Д.О., Бокерия Л.А. Функциональное состояние миокарда желудочков сердца у новорожденных с простой транспозицией магистральных артерий до и после операции артериального переключения. Детские болезни сердца и сосудов. 2022; 19 (3): 206–212. DOI: 10.24022/1810-0686-2022-19-3-206-212

Ansari R.M., Yarmohammadi H., Gareb B., Voors A.A., van Melle J.P. Long-term outcome of patients with transposition of the great arteries and a systemic right ventricle: a systematic review and meta-analysis. Int. J. Cardiol. 2023; 389: 131159. DOI: 10.1016/j.ijcard.2023.131159

Koolbergen D.R., Ahmed Y., Bouma B.J., Scherptong R.W., Bruggemans E.F., Vliegen H.W. et al. Follow-up after tricuspid valve surgery in adult patients with systemic right ventricles. Eur. J. Cardiothorac. Surg. 2016; 50 (3): 456–463. DOI: 10.1093/ejcts/ezw059

Minaev A.V., Podzolkov V.P., Danilov T.Y., Chiaureli M.R., Bupe M.M., Dontsova V.I. Improved surgical outcomes in adults with congenital heart disease and decompensated heart failure: the role of perioperative medical optimization. Am. J. Case Rep. 2023; 24: e939230. DOI: 10.12659/ajcr.939230

Данилов Т.Ю., Минаев А.В., Малинкин И.А., Землянская И.В. Протезирование обоих атриовентрикулярных клапанов у взрослой пациентки с корригированной транспозицией магистральных сосудов и декомпенсированной хронической сердечной недостаточностью со сниженной фракцией выброса. Грудная и сердечно-сосудистая хирургия. 2023; 65 (1): 101–105. DOI: 10.24022/0236-2791-2023-65-1-101-105

Loss K.L., Shaddy R.E., Kantor P.F. Recent and upcoming drug therapies for pediatric heart failure. Front. Pediatr. 2021; 9: 681224. DOI: 10.3389/fped.2021.681224

Fusco F., Scognamiglio G., Merola A., Iannuzzi A., Palma M., Grimaldi N., Sarubbi B. 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): e00984. DOI: 10.1161/circheartfailure.122.009848

Zandstra T.E., Nederend M., Jongbloed M.R., Kiès P., Vliegen H.W., Bouma B.J. et al. Sacubitril/valsartan in the treatment of systemic right ventricular failure. Heart. 2021; 107: 1725–1730.

Egorova A.D., Nederend M., Tops L.F., Vliegen H.W., Jongbloed M.R.M., Kiès P. The first experience with sodium-glucose cotransporter 2 inhibitor for the treatment of systemic right ventricular failure. ESC Heart Fail. 2022; 9 (3): 2007–2012. DOI: 10.1002/ehf2.13871

Neijenhuis R.M.L., Nederend M., Jongbloed M.R.M. 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. DOI: 10.3389/fcvm.2023.1093201

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): 974–975. DOI: 10.1016/j.cardfail.2023.03.011

Neijenhuis R.M.L., MacDonald S.T., Zemrak F., Mertens B.J.A., Dinsdale A., Hunter A. et al. Effect of sodium-glucose cotransporter 2 inhibitors in adults with congenital heart disease. J. Am. Coll. Cardiol. 2024; 83 (15): 1403–1414. DOI: 10.1016/j.jacc.2024.02.017

Ndiaye J.F., Nekka F., Craig M. Understanding the mechanisms and treatment of heart failure: quantitative systems pharmacology models with a focus on SGLT2 inhibitors and sex-specific differences. Pharmaceutics. 2023; 15 (3): 1002. DOI: 10.3390/pharmaceutics15031002

Newland D.M., Law Y.M., Albers E.L., Friedland-Little J.M., Ahmed H., Kemna M.S., Hong B.J. Early clinical experience with dapagliflozin in children with heart failure. Pediatr. Cardiol. 2023; 44 (1): 146–152. DOI: 10.1007/s00246-022-02983-0

Abdul R.K., Doughan M.E., Wendy M.B. 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): 704–706. DOI: 10.1016/j.amjcard.2006.10.025

Galves R., Da Costa A., Pierrard R., Bayard G., Guichard J.B., Isaaz K. Impact of β-blocker therapy on right ventricular function in heart failure patients with reduced ejection fraction. A prospective evaluation. Echocardiography. 2020; 37 (9): 1392–1398. DOI: 10.1111/echo.14813

Jhund P.S., Talebi A., Henderson A.D., Claggett B.L., Vaduganathan M., Desai A.S. et al. Mineralocorticoid receptor antagonists in heart failure: an individual patient level meta-analysis. Lancet. 2024; 404 (10458): 1119–1131.

Elsaeidy A.S., Abuelazm M., Ghaly R., Soliman Y., Amin A.M., El-Gohary M. et al. The Efficacy and Safety of Levosimendan in Patients with Advanced Heart Failure: An Updated Meta-Analysis of Randomized Controlled Trials. Am. J. Cardiovasc. Drugs. 2024; DOI: 10.1007/s40256-024-00675-z

Published
2025-07-01
Section
Клинические случаи