Artículo de actualización para formación continuada: Tetralogía de Fallot

The tetralogy of Fallot is the most frequent cyanotic congenital heart disease in the general population with a general incidence of 0.1/1000 live births. The morphologic diagnostic axis is the left anterior displacement of the infundibular ventricular septum - towards the right ventricle way out- during the embryogenic period that causes aortic override, ventricular septal defect, subpulmonary stenosis and right ventricular hypertrophy. Without surgical intervention, survival is 66% in 1 year and only 10% to 15% in > 20 years. Clinical presentation is variable and depends on the grade of pulmonary stenosis; when it is already significant in the neonatal period or in infants < 3 to 6 months, hypoxemic crisis that may require urgent medical or surgical intervention may appear. Complete surgical correction of the malformation offers good survival outcomes during decades, although with the first techniques -trans-anular patch and closure of the interventricular defect- there appear long term problems that may generate an additional mobimortality risk. Severe pulmonary insufficiency, presence of right ventricular dilation and development of potentially fatal arrhythmias are problems that become important and must be recognized in order to assess an early re-intervention and to repair residual defects inductors of arrhythmia. The new surgical techniques try to preserve as much as possible ventricular-pulmonary joint and the valve itself, if anatomy allows it. That will without doubt benefit the patient because the residual problems described have lesser clinical significance.