Case Report

Dental Management of a Patient with Pulmonary Atresia and Ventricular Septal Defect

Review of the literature and a case report.


Pulmonary atresia with ventricular septal defect (PA/VSD) is one of the congenital heart diseases that results in cyanosis, susceptibility to bacterial endocarditis, and increased risk of complications during general anesthesia. Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common inherited genetic disorder affecting the red blood cells. We aimed to elaborate the potential dental management for patients with this serious condition. This report presents the single-visit dental treatment of a three-year-old female with PA/VSD, G6PD deficiency and rampant caries. The complexity of dental treatments, high incidence of dental caries, lack of cooperation, and the systemic condition limit treatment options to providing service under general anesthesia and hospitalization. Careful monitoring of oxygen saturation during general anesthesia and antibiotic prophylaxis are essential due to the invasive nature of dental treatments. It appears that single-visit dental management under general anesthesia minimizes the risk of treatment of patients at high risk of bacterial endocarditis.

Garrocho-Rangel A E-GA, Rosales-Bérber MÁ, Flores-Velázquez J, Pozos-Guillén A. Dental management of pediatric patients affected by pulmonary atresia with ventricular septal defect: A scoping review. Med Oral Patol Oral Cir Bucal. 2017 Jul;22(4):e458.

Freedom RM, Benson LN, Smallhorn JF. Pulmonary atresia and ventricular septal defect, in Neonatal heart disease. London, Springer, 1992:229-56.

Gatzoulis MA, Swan L, Therrien J, Pantely G. Pulmonary atresia with ventricular septal defect, in adult congenital heart disease: A practical guide. Blackwell Publishing Ltd., 2005:132-138.

Pourmoghaddas Z, Meskin M, Sabri M, Tehrani MH, Najafi T. Dental caries and gingival evaluation in children with congenital heart disease. Int J Prev Med. 2018 Jun,9(1):52.

Cantekin K CI, Torun Y. Comprehensive dental evaluation of children with congenital or acquired heart disease. Cardiol Young. 2013 Oct;23(5):705-10.

Pimentel EL, AzevedoV, Castro RD, Reis LC, Lorenzo AD. Caries experience in young children with congenital heart disease in a developing country. Braz Oral Res. 2013 Mar-Apr;27(2):103-8.

White MC, Peyton J. Anaesthetic management of children with congenital heart disease for non-cardiac surgery. Continuing Education in Anaesthesia, Critical Care & Pain. 2011 Dec 12(1):17-22.

Elyassi CA, Rowshan M. Perioperative management of the glucose-6-phosphate dehydrogenase deficient patient: a review of literature. Anesth Prog. 2009 Autumn;56(3):86-91.

Monteiro WM, Val FF, Siqueira AM, Franca GP, Sampaio VS, Melo GC, et al. G6PD deficiency in Latin America: systematic review on prevalence and variants. Mem Inst Oswaldo Cruz. 2014 Aug;109(5):553-68.

Hernández-Pérez D, Téllez Girón C, Ruiz-Rodríguez S, Garrocho-Rangel A, Pozos-Guillén A. Dental considerations in children with glucose-6-phosphate dehydrogenase deficiency (Favism): A review of the literature and case report. Case Rep Dent. 2015 Sep(9):459-506.

Kumar RA, Venkatesh B, Karumaran CS, Rajasekaran MS, Shankar P. Protocol for dental management in a patient with glucose-6-phosphate dehydrogenase deficiency. J Clin Diagn Res. 2017 Oct;11(10): ZD09-11.

Altikat S ÇM, Buyukokuroglu ME. In vitro effects of some anesthetic drugs on enzymatic activity of human red blood cell glucose 6-phosphate dehydrogenase. Pol J Pharmacol. 2002 Jan-Feb;54(1):67-72.

Stecksén-Blicks C, Rydberg A, Nyman L, Asplund S, Svanberg C. Dental caries experience in children with congenital heart disease: a case-control study. Int J Paediatr Dent. 2004 Mar;14(2):94-100.

Ajami B, Abolfathi G, Mahmoudi E,

Mohammadzadeh Z. Evaluation of salivary Streptococcus mutans and dental caries in children with heart diseases. J Dent Res Dent Clin Dent Prospects 2015 Jun;9(2): 105-8.

Ayala C, Aguayo L. Oral and systemic manifestations, and dental management of a pediatric patient with Tetralogy of Fallot. A case report. J Oral Res. 2016 Mar;5(2):87-91.

Pertiwi AS, Sasmita I, Nonong YH. Oral and dental management in children with tetralogy of fallot. Dental Journal (Majalah Kedokteran Gigi). 2007 Mar;40(1):42-5.

Hasan MS, Chan L. Dexmedetomidine and ketamine sedation for dental extraction in children with cyanotic heart disease. J Oral Maxillofac Surg. 2014 Oct;72(10):1920. e1-4.

Moosazadeh M, Amiresmaili M, Aliramezany M. Prevalence of G6PD deficiency in Iran, a meta-analysis. Acta Med Iranica. 2014;52(4):256-64.

Greenwood M. Meechan J. General medicine and surgery for dental practitioners. Part 2—metabolic disorders. Br Dent J. 2010 May;208(9):389–92.

IssueVol 18 (Continuously Published Article-Based) QRcode
SectionCase Report
Dentistry; Anesthesia General; Glucosephosphate Dehydrogenase Deficiency; Heart Defects Congenital

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How to Cite
Ansari G, Mansouri M, Eftekhar L. Dental Management of a Patient with Pulmonary Atresia and Ventricular Septal Defect. Front Dent. 2021;18.