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Authors:Dr. Nandam Ajitha,Dr. Chunawalla Yusuf,Dr. Morawala Abdul.

Tetralogy of Fallot is the most common form of congenital heart disease found in children. It is the most common cyanotic heart defect and the most common cause of blue baby syndrome. The anatomic defects comprising Tetralogy of Fallot lead to the systemic circulation of desaturated blood resulting in symptoms of cyanosis, clubbing, polycythemia and hypoxia . This case report of a 7yr old child discusses the oral findings and dental management of a child with Tetralogy of Fallot. It also stresses on the medical status of the patient prior to and following dental procedures.

Cardiovascular diseases comprise of widespread group of conditions that have significant morbidity and mortality. 1Tetralogy of Fallot was first described by Niels Stenson in 1671 and later refined by Etienne –Louis Fallot in 1888 in his description of  l’anatomicpathologique de la maladie bleu but the term Tetralogy of Fallot which is a   tetrad of 1) ventricular septal defect 2) overriding aorta 3) right ventricular outflow obstruction 4) right ventricular hypertrophy is attributed to Canadian Maude Abbott in 1924 .2

The etiology can be either   due to environmental, monogenetic or polygenetic factors. Tetralogy of Fallot arises from a single genetic defect involving the TBX1 gene, which encodes for a transcription factor integral to the outflow of cardiac outflow tracts.3This isolated genetic anomaly creates the various developmental derangement associated with Tetralogy of Fallot. 1/5th of the patients associated with this disease show microdeletions of chromosome 22q 11.2.4Factors associated with high incidence include family history, maternal age older than 40 yrs, maternal alcohol use, poor prenatal nutrition, mothers with phenylketonuria, fetal alcohol syndrome  and diabetes.1
About 3.5% of all infants have Tetralogy of Fallot with  males and females being equally affected .5Tetralogy of Fallot is associated with Down syndrome and Di George syndrome.4

A 7 yr old girl was referred to the Department of Pedodontics, MA Rangoonwala College of Dental Sciences,Pune with a chief complaint of decayed teeth .  The patient had a known history of Tetralogy of Fallot and had undergone surgery 3 years ago. Family history was non contributory. General physical examination of the patient revealed a well-nourished child with normal growth and development. Patient showed clinodactaly on her left index finger. Intraoral examination revealed teeth with multiple carious lesions. Deep carious lesions were present in51,52, 55,62,65,75,85. Grossly decayed  teeth in 61.  Root piece in 54 and proximal carious lesions in 64, 71,72,81,82.
Patient was asked to get a complete blood examination which showed slightly lower count of hemoglobin.  The CBC,PT and PTT were normal. Examination of chest revealed good bilateral aeration without wheezing.
On correlation with intraoral radiographs it was decided that the patient needed oral prophylaxis, restorations in 55,64, 84  , pulpectomy  followed by stainless steel crown in 65, pit and fissure sealants in 16,26,36 and 46 and extractions  of  51,52,61,62,54,71,72,81,82,74,75,85 under local anesthesia  with antibiotic coverage of amoxicillin clavulanate 600 mg ,1 hr before the procedure . Following the procedures the patient was recalled after 1 month, 3 months and  6 months for general checkup.

The dental patient with Tetralogy of fallot requires modifications in the treatment plan. A primary concern in treating the child is minimizing the stress during dental procedure. This is important to avoid any cyanotic episode. 4
Children with Tetralogy of Fallot display squatting as a compensatory mechanism to the right and left shunting of blood.6The hypoxic ‘tet spells’ that lead to the act of squatting can be potentially lethal resulting from spasming of musculature around the right ventricular tract .7 Patients display cyanosis, dyspnoea and clubbing. Postures to relieve dyspnoea include sitting with knees drawn close to the chest, lying down and crossing the legs while sitting or standing. 4 Gingival hypertrophy along with fissured tongue may be present.1 Tetralogy of Fallot patients have increased tendency of developing iron deficiency anemia. 6 In these patients heart is enlarged and boot shaped. 1Children show delayed eruption and there is a higher incidence of caries and periodontal activity.1
Tetralogy of Fallot leads to fatal complication such as bacterial endocarditis. It is caused by bacterial infection in heart valve/endocardium. Transient bacteremia due to Streptococcus sanguis and Streptococcus mitis causes endocarditis.8

American Heart Association provides antibiotic prophylaxis for bacterial endocarditis: 9,10
Infective endocarditis prophylaxis for dental procedures should only be used in patients with underlying cardiac conditions associated with the highest risk for adverse outcomes such as prosthetic valves or prior Infective endocarditis.
Endocarditis prophylaxis is recommended in :9

High risk category

  1. Prosthetic cardiac valves
  2. Previous bacterial endocarditis
  3. Complex cyanotic congenital heart disease
  4. Surgically constructed systemic pulmonary shunts or conduits.

Modifications to an “ideal” treatment plan must be made to accommodate the existing medical condition of the patient. Extractions are preferred over any pulp therapy. Adequate oral hygiene is to be maintained to reduce the risk of infection.
Space maintainer was not advised for this patient to prevent the risk of bacteremia and as there is possibility of food accumulation around the bands. Poor oral hygiene can accelerate the risk of infective endocarditis in cardiac patients.
Children with Tetralogy of Fallot have increased risk of caries due to certain medications which contain high concentration of sugars. Preventive dental measures such as routine dental checkup,  fluoridation, dietary modification to minimize the risk of dental caries and periodontal disease must be advocated.11

Standard prophylaxis Amoxicillin 50 mg per kg weight
Taken 1 hr before a planned dental procedure
Patients who are unable to take oral medication Ampicillin 50 mg per kg weight
Given into the vein or large muscle within 30 min before a planned dental procedure
Patients who are allergic to penicillin Clindamycin 20 mg per kg body weight
Taken 1 hr before a planned dental procedure
Pateints who are allergic to penicillin Cephalexin or cephadroxil 50 mg per kg weight
Taken 1 hr before a planned dental procedure
Patients who are allergic to penicillin Azithromycin or clarithromycin 15 mg per kg body weight
Taken 1 hr before a planned dental procedure
Pateints who are unable to take penicillin and unable to take medications by mouth clindamycin 20 mg per  kg body weight
Given into the vein or large muscle within 30 min before a planned dental procedure
Pateints who are unable to take penicillin and unable to take medications by mouth cefazolin 25 mg per kg body weight
Given into the vein or large muscle within 30 min before a planned dental procedure

Precautions to be taken for patients with Tetralogy of Fallot during dental procedures:1,8

 1) Complete history of the child along with the consent of a pediatrician is necessary.
2) Treatment should be performed in a stress free environment.
3) Premedication with anti-anxiety drugs is can be advocated to calm the patient.
4) If the child is uncooperative treatment can be performed under conscious sedation or general anesthesia.
5) Prior to dental procedure, patients mouth should be rinsed with 0.2% chlorhexidine to reduce the bacterial  load.
6) During hypercyanotic attack, administer oxygen 0.2mg/kg body weight placing the patient in knee-chest position.
7) Treatment should be of short durations
8) Children should avoid vigorous brushing to prevent the risk of bacteremia.

CONCLUSION: Children with cardiac disorders must be provided with effective dental treatment, as a simple dental problem may compromise a child’s medical management. Hence knowledge of cardiac conditions and their management is essential for providing efficacious dental delivery. Good communication amongst the patient, dentist and the physicians are essential in achieving this goal.


  1. Prashant Babaji  TETRALOGY OF FALLOT: a case report and dental considerations  Rev Clín Pesq Odontol. 2009 set/dez;5(3):289-292.
  2. Pinsky WW, Arciniegas E. Tetra logy of Fallot. Pediatr Clin North Am 37:179-92, 1990.
  3.  Lilly, Pathophysiology  of heart disease : A collaborative project of medical  students and faculty ,4th ed, Lippincott  Williams and Wilkins,2007.
  4. Evan Spivack, Tetralogy of Fallot: an overview, case report, and discussion of dental implications Spec Care Dentist21(5):172-175, 2001
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  7. Bernstein B, Kliegman RM, Arvin AM. The transitional circulation. In: Berstein B. Nelson textbook of paediatrics. 15th ed. Philadelphia: WB Saunders; 1995. p. 1283- 1315.
  8. Show L, Welbury RR. Cardiovascular disorders. In: Welbury R. Paediatric dentistry. New York. Oxford medical publications; 2000. p. 354-8.
  9. Guideline on Antibiotic Prophylaxis for Dental  Patients at Risk for Infection, AAPD 2014
  10. Dajani AS, Taubert KA, Wilson W, et al.Prevention of bacterial endocarditis. Recommendations by the American Heart Association. J Am Med Assoc 277: 1794-801, 1997.