Management of Fallot's Uncorrected Tetralogy in Adulthood: A Narrative Review

Overview

Journal Cureus

Specialty Biomedical Engineering

Date 2024 Sep 17

PMID 39286683

Authors

Anne Elizabeth Kaiser

Muhammad Ammar Husnain

Laraib Fakhare Alam

Siva Kumar Murugan

Rajanikant Kumar

Affiliations

Soon will be listed here.

Abstract

The majority of cyanotic congenital cardiac defects are caused by the tetralogy of Fallot. Some symptoms include a biventricular connection of the aortic root, right ventricular hypertrophy, blockage of the right ventricular outflow tract, and a ventricular septal defect. Our understanding of tetralogy of Fallot (TOF) has significantly advanced since it was first described in 1888, and early diagnosis has led to improved surgical management and increased life expectancy. Adults with unrepaired and repaired TOF present with a range of late complications, including heart failure, the need for re-interventions, and late arrhythmias. Right ventricular (RV) failure, often caused by chronic pulmonary regurgitation, is a significant cause of heart failure in patients with TOF. Current treatment options are limited, and mainstay surgical procedures such as pulmonary-valve replacement (PVR), trans-annular repair (TAR), or infundibular widening repair have not shown a significant reduction in preventing right ventricular (RV) failure or death. Here, we explain the mechanisms of RV failure in ToF, chronic pulmonary regurgitation, heart failure, and secondary polycythemia. HF management in untreated adults is discussed. The progression of the disease, as well as complications, are also discussed. The treatment plan and the need to investigate the best management approach for this unsolved problem are included. This review aims to fill the knowledge gaps and supply valuable information regarding mechanisms of RV failure, chronic pulmonary regurgitation, and secondary polycythemia. To summarize, a new combat strategy must be found to battle RVF, and a more profound vision of these mechanisms is required. If it is not corrected, it will be one of the future research lines that will contribute to designing more efficacious treatment techniques for adults with TOF.

References

Wald R, Valente A, Marelli A . Heart failure in adult congenital heart disease: Emerging concepts with a focus on tetralogy of Fallot. Trends Cardiovasc Med. 2015; 25(5):422-32. DOI: 10.1016/j.tcm.2014.11.011. View

van der Linde D, Konings E, Slager M, Witsenburg M, Helbing W, Takkenberg J . Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J Am Coll Cardiol. 2011; 58(21):2241-7. DOI: 10.1016/j.jacc.2011.08.025. View

Dorobantu D, Stoicescu C, Tulloh R, Stoica S . Surgical Repair of Tetralogy of Fallot With Absent Pulmonary Valve: Favorable Long-Term Results. Semin Thorac Cardiovasc Surg. 2019; 31(4):847-849. DOI: 10.1053/j.semtcvs.2019.05.022. View

Restivo A, Anderson R, Carletti R, Di Gioia C . Correlating the morphological features of tetralogy of Fallot and the Eisenmenger malformation. Cardiol Young. 2016; 27(1):161-172. DOI: 10.1017/S1047951116000287. View

Rodenbarger A, Thorsson T, Stiver C, Jantzen D, Chevenon M, Yu S . Third trimester predictors of interventional timing and accuracy of fetal anticipatory guidance in tetralogy of Fallot: A multi-center study. Prenat Diagn. 2020; 40(7):870-877. DOI: 10.1002/pd.5697. View

Semeraro O, Scott B, Vermeersch P . Surgical correction of tetralogy of Fallot in a seventy-five year old patient. Int J Cardiol. 2007; 128(3):e98-100. DOI: 10.1016/j.ijcard.2007.05.081. View

Mouws E, de Groot N, van de Woestijne P, de Jong P, Helbing W, van Beynum I . Tetralogy of Fallot in the Current Era. Semin Thorac Cardiovasc Surg. 2018; 31(3):496-504. DOI: 10.1053/j.semtcvs.2018.10.015. View

Becker A, Connor M, Anderson R . Tetralogy of Fallot: a morphometric and geometric study. Am J Cardiol. 1975; 35(3):402-12. DOI: 10.1016/0002-9149(75)90034-x. View

Stout K, Broberg C, Book W, Cecchin F, Chen J, Dimopoulos K . Chronic Heart Failure in Congenital Heart Disease: A Scientific Statement From the American Heart Association. Circulation. 2016; 133(8):770-801. DOI: 10.1161/CIR.0000000000000352. View

10.

Wood P . The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J. 1958; 2(5099):755-62. PMC: 2026272. DOI: 10.1136/bmj.2.5099.755. View

11.

van den Bosch E, van Genuchten W, Luijnenburg S, Duppen N, Kamphuis V, Roos-Hesselink J . Associations between blood biomarkers, cardiac function and adverse outcome in a young tetralogy of Fallot cohort. Int J Cardiol. 2022; 361:31-37. DOI: 10.1016/j.ijcard.2022.04.065. View

12.

Gandhi P, Gaggin H, Redfield M, Chen H, Stevens S, Anstrom K . Insulin-Like Growth Factor-Binding Protein-7 as a Biomarker of Diastolic Dysfunction and Functional Capacity in Heart Failure With Preserved Ejection Fraction: Results From the RELAX Trial. JACC Heart Fail. 2016; 4(11):860-869. PMC: 5500914. DOI: 10.1016/j.jchf.2016.08.002. View

13.

Miller J, Stephens E, Goldstone A, Glatz A, Kane L, Van Arsdell G . The American Association for Thoracic Surgery (AATS) 2022 Expert Consensus Document: Management of infants and neonates with tetralogy of Fallot. J Thorac Cardiovasc Surg. 2022; 165(1):221-250. DOI: 10.1016/j.jtcvs.2022.07.025. View

14.

Bokma J, de Wilde K, Vliegen H, van Dijk A, van Melle J, Meijboom F . Value of Cardiovascular Magnetic Resonance Imaging in Noninvasive Risk Stratification in Tetralogy of Fallot. JAMA Cardiol. 2017; 2(6):678-683. PMC: 5540000. DOI: 10.1001/jamacardio.2016.5818. View

15.

Niwa K . Metabolic syndrome and coronary artery disease in adults with congenital heart disease. Cardiovasc Diagn Ther. 2021; 11(2):563-576. PMC: 8102264. DOI: 10.21037/cdt-20-781. View

16.

Kervancioglu M, Tokel K, Varan B, Yildirim S . Frequency, origins and courses of anomalous coronary arteries in 607 Turkish children with tetralogy of Fallot. Cardiol J. 2011; 18(5):546-51. DOI: 10.5603/cj.2011.0011. View

17.

Vehmeijer J, Koyak Z, Bokma J, Budts W, Harris L, Mulder B . Sudden cardiac death in adults with congenital heart disease: does QRS-complex fragmentation discriminate in structurally abnormal hearts?. Europace. 2017; 20(FI1):f122-f128. DOI: 10.1093/europace/eux044. View

18.

Healy F, Hanna B, Zinman R . Pulmonary complications of congenital heart disease. Paediatr Respir Rev. 2012; 13(1):10-5. DOI: 10.1016/j.prrv.2011.01.007. View

19.

Gaeta S, Ward C, Krasuski R . Extra-cardiac manifestations of adult congenital heart disease. Trends Cardiovasc Med. 2016; 26(7):627-36. DOI: 10.1016/j.tcm.2016.04.004. View

20.

Alipour Symakani R, van Genuchten W, Zandbergen L, Henry S, Taverne Y, Merkus D . The right ventricle in tetralogy of Fallot: adaptation to sequential loading. Front Pediatr. 2023; 11:1098248. PMC: 10061113. DOI: 10.3389/fped.2023.1098248. View