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Tetralogy of Fallot (TF) is the most common cyanotic congenital heart disease. As the name suggests, it consists of four changes in the heart, namely, the existence of a defect or communication in the ventricular septum, the root of the aorta overlapping the ventricular or right-handed septal defect, stenosis of the pulmonary artery, and right ventricular hypertrophy. Therefore, there is a degree of obstruction of blood flow that compromises the transport of oxygen in the body. The objective of this study was to conduct a review of the qualitative narrative literature, in which scientific articles on TF and its relationship with dentistry, published in the last ten years, in the PubMed, SciELO and Google Scholar databases were searched. The prevalence of TF is 3 per 10,000 births, which represents 10% of all congenital heart diseases, being more frequent in males, and the etiology is multifactorial, but little known. The main clinical signs are hypoxia and cyanosis. In the stomatognathic system, tooth development and bone growth, as well as soft tissues, are affected. The main manifestations are agenesis, enamel hypoplasia, and delayed tooth eruption and cyanotic mucous membranes. A correct, complete and immediate diagnosis is essential for uncomplicated treatment and a good prognosis. The tests necessary to detect the disease are the electrocardiogram and the fetal echocardiogram in the antepartum and puerperium, and it is possible to observe the ventricular septal defect and the associated repercussions. The treatment consists of a very early surgical intervention. Children with TF should start dental consultations in the first years of life, and it is necessary to adopt adjustments by the dentist in the care of these patients, always with the objective of avoiding more invasive procedures. The dentist plays an important role in the multidisciplinary team involved with TF, in order to promote and prevent oral health, controlling the occurrence of related oral problems and their aggravation with systemic involvement.
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