Tetralogy of Fallot Associated with Amelogenesis Imperfecta: a Case Report

Authors

  • Maria P.N.C. Manna Department of Operative Dentistry, School of Dentistry of São Paulo, University of São Paulo, São Paulo, São Paulo, Brazil. Author https://orcid.org/0000-0002-9315-1507
  • Mirelle A. M. Tashima Special Care Dentistry center-CAPE/FOUSP, São Paulo, Brazil. Author https://orcid.org/0009-0008-2312-1207
  • Matheus C. Costa Department of Operative Dentistry, Endodontics and Dental Materials, School of Dentistry of Bauru, University of São Paulo, Bauru, São Paulo, Brazil. Author https://orcid.org/0000-0001-7689-2226
  • Marina Gallottini Department of Stomatology and CAPE/FOUSP, Dental School, University of São Paulo, São Paulo, São Paulo, Brazil. Author https://orcid.org/0000-0001-6071-5110
  • Fabiana Martins Department of Stomatology and CAPE/FOUSP, Dental School, University of São Paulo, São Paulo, São Paulo, Brazil. Postgraduate program in Dentistry, School of Dentistry, University of Santo Amaro, São Paulo - SP, Brazil. Author https://orcid.org/0000-0002-4352-7959

DOI:

https://doi.org/10.15517/2kga2y88

Keywords:

Tetralogy of Fallot; Amelogenesis imperfecta; Infective endocarditis; Dental prophylaxis; Composite resin.

Abstract

Tetralogy of Fallot (TOF) is the prevalent cyanotic congenital cardiac anomaly, accounting for 80% of all cases. Despite the incidence rate, the etiopathogenesis of TOF remains unknown. Amelogenesis imperfecta (AI) is a hereditary condition that impacts enamel development, resulting in morphological and functional tooth modifications. This report aims to present the case of a young patient with TOF and AI who received direct restorative treatment, emphasizing the medical and dental factors involved in enhancing oral health and dental esthetics. The patient has a history of cardiac procedures, including the implantation of a metallic pulmonary valve, along with moderate intellectual disability and a prior seizure episode. The intraoral examination indicated extensive gingivitis, inadequate oral hygiene, hypomineralized enamel with structural deterioration, and numerous plaque retention niches, resulting in pain during mastication and oral hygiene practices. Due to the high risk of infective endocarditis, infected foci were eliminated, and antibiotic prophylaxis was administered according to the American Heart Association guidelines. Restorative therapy was conducted utilizing direct composite resin veneers to restore dental aesthetics and functionality. Post-treatment, the patient had enhanced self-esteem, maintained dental hygiene, and achieved remission of gingivitis. This case demonstrates the significance of comprehensive dental care for patients with complex congenital heart conditions and enamel developmental disorders, stressing the necessity for preventive measures, effective periodontal management, and advanced restorative techniques to reduce the risks of systemic infections.

Downloads

Download data is not yet available.

References

Su Z., Zou Z., Hay S.I., Liu Y., Li S., Chen H., Naghavi M., Zimmerman M.S., Martin G.R., Wilner L.B., Sable C.A., Murray C.J.L., Kassebaum N.J., Patton G.C., Zhang H. Global, regional, and national time trends in mortality for congenital heart disease, 1990-2019: An age-period-cohort analysis for the Global Burden of Disease 2019 study. EClinicalMedicine. 2022 Jan 11;43:101249. doi: 10.1016/j.eclinm.2021.101249. PMID: 35059612; PMCID: PMC8760503.

Suzuki N., Svensson K., Eriksson U.J. High glucose concentration inhibits migration of rat cranial neural crest cells in vitro. Diabetologia. 1996 Apr; 39 (4): 401-11. doi: 10.1007/BF00400671. PMID: 8777989.

Bailliard F., Anderson R.H. Tetralogy of Fallot. Orphanet J Rare Dis. 2009 Jan 13; 4: 2. doi: 10.1186/1750-1172-4-2. PMID: 19144126; PMCID: PMC2651859.

Postoev V.A., Talykova L.V., Vaktskjold A. Epidemiology of Cardiovascular Malformations among Newborns in Monchegorsk (North-West Russia): a Register-Based Study. J Public Health Res. 2014 Aug 4; 3 (2): 270. doi: 10.4081/jphr.2014.270. PMID: 25343136; PMCID: PMC4207024.

Park G., Thayer J.F. From the heart to the mind: cardiac vagal tone modulates top-down and bottom-up visual perception and attention to emotional stimuli. Front Psychol. 2014 May 1; 5: 278. doi: 10.3389/fpsyg.2014.00278. PMID: 24817853; PMCID: PMC4013470.

Althali N.J., Hentges K.E. Genetic insights into non-syndromic Tetralogy of Fallot. Front Physiol. 2022 Oct 6; 13: 1012665. doi: 10.3389/fphys.2022.1012665. PMID: 36277185; PMCID: PMC9582763.

Roma M., Hegde P., Durga Nandhini M., Hegde S. Management guidelines for amelogenesis imperfecta: a case report and review of the literature. J Med Case Rep. 2021 Feb 9; 15 (1): 67. doi: 10.1186/s13256-020-02586-4. PMID: 33557885; PMCID: PMC7871387.

Toupenay S., Fournier B.P., Manière M.C., Ifi-Naulin C., Berdal A., de La Dure-Molla M. Amelogenesis imperfecta: therapeutic strategy from primary to permanent dentition across case reports. BMC Oral Health. 2018 Jun 15; 18 (1): 108. doi: 10.1186/s12903-018-0554-y. PMID: 29907114; PMCID: PMC6003150.

Witkop C.J. Jr. Amelogenesis imperfecta, dentinogenesis imperfecta and dentin dysplasia revisited: problems in classification. J Oral Pathol. 1988 Nov; 17 (9-10): 547-53. doi: 10.1111/j.1600-0714.1988.tb01332.x. PMID: 3150442.

Hoffman J.I., Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002 Jun 19; 39 (12):1890-900. doi: 10.1016/s0735-1097(02)01886-7. PMID: 12084585.

Eitoku T., Nishii N., Morita H., Kasahara S. A rare case of tetralogy of Fallot with catecholaminergic polymorphic ventricular tachycardia. HeartRhythm Case Rep. 2022 Dec 10; 9 (3):152-155. doi: 10.1016/j.hrcr.2022.12.005. PMID: 36970376; PMCID: PMC10030305.

Peck D., Tretter J., Possner M., Yutzey K., Zafar F., Morales D., Alsaied T. Timing of Repair in Tetralogy of Fallot: Effects on Outcomes and Myocardial Health. Cardiol Rev. 2021 Mar-Apr 01; 29 (2): 62-67. doi: 10.1097/CRD.0000000000000293. PMID: 31934899.

Hashem A., Kelly A., O'Connell B., O'Sullivan M. Impact of moderate and severe hypodontia and amelogenesis imperfecta on quality of life and self-esteem of adult patients. J Dent. 2013 Aug; 41 (8): 689-94. doi: 10.1016/j.jdent.2013.06.004. Epub 2013 Jun 15. PMID: 23778130.

Tasioula V., Balmer R., Parsons J. Dental health and treatment in a group of children with congenital heart disease. Pediatr Dent. 2008 Jul-Aug; 30 (4): 323-8. PMID: 18767512.

Assunção C.M., Falleiros T., Gugisch R.C., Fraiz F.C., Losso E.M. Tetralogia de Fallot e sua repercussão na saúde bucal. Rev Paul Pediatr 2008; 26 (1): 93-96. doi:10.1590/S0103-05822008000100016.

Van Amerongen W.E., Kreulen C.M. Cheese molars: A pilot study of the etiology of hypocalcifications in first permanent molars. J Dent Child 1995; 62: 266-9.

Jalevik B., Noren J.G. Enamel hypomineralization of permanent first molars: A morphological study and survey of possible aetiological factors. Int J Paediatr Dent 2000; 10: 278-89.

Barbosa M., Prada-López I., Álvarez M., Amaral B., de los Angeles C.D., Tomás I. Post-tooth extraction bacteraemia: a randomized clinical trial on the efficacy of chlorhexidine prophylaxis. PLoS One. 2015 May 8; 10 (5): e0124249. doi: 10.1371/journal.pone.0124249. PMID: 25955349; PMCID: PMC4425363.

Jayaraman J. A Child with Tetralogy of Fallot Presents for Extraction of Mandibular Premolars. Dent Clin North Am. 2023 Jul; 67 (3): 415-417. doi: 10.1016/j.cden.2023.02.008. Epub 2023 Apr 18. PMID: 37244704.

Wilson W., Taubert K.A., Gewitz M., Lockhart P.B., Baddour L.M. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. 2007; 116 (15): 1736-1754 (23)

Tubiana S., Blotière P.O., Hoen B., Lesclous P., Millot S., Rudant J., Weill A., Coste J., Alla F., Duval X. Dental procedures, antibiotic prophylaxis, and endocarditis among people with prosthetic heart valves: nationwide population based cohort and a case crossover study. BMJ. 2017 Sep 7; 358: j3776. doi: 10.1136/bmj.j3776. PMID: 28882817; PMCID: PMC5588045.

Published

2025-11-06