A Successful Correction of a Truncus Arteriosus in an 8-Year-Old Girl with Functional Pulmonary Stenosis by a Particular Opening of the Truncal Valve

Abstract

<i>Background: </i>Truncus arteriosus is a rare congenital heart malformation requiring repair in the neonatal period. Without correction, the majority of patients die within the first year of life. However, this case shows that some patients remain operable even at an advanced age. <i>Case presentation: </i>We report the case of an 8-year-old girl with type 2 truncus arteriosus with pulmonary vascular protection by a particular opening of the truncal valve partially obstructing the origin of the pulmonary artery in systole and causing a reduction in pulmonary flow. The initial evaluation based on the clinical presentation, the electrocardiogram and the echocardiography showed a significant elevation of the pressures of the right ventricle with a functional stenosis at the origin of the pulmonary artery. Hemodynamic exploration demonstrated that the pulmonary arterial hypertension is reversible, thereby the patient was successfully operated. <i>Conclusion: </i>The objective of this observation is to demonstrate that truncus arteriosus referred late does not eliminate the possibility of complete surgical repair, which underlines the importance of an exhaustive evaluation of these patients based on several arguments.