Tetralogy of Fallot was classically described as the combination of pulmonary stenosis, right ventricle hypertrophy, perimembranous ventricular septal defect and an aortic root that overrides the ventricular septal defect. Full surgical repair, which is usually indicated in toddlers, comprises closure of the ventricular septal defect and alleviation of the pulmonary stenosis. Because there is a wide anatomical spectrum, the cause and severity of the pulmonary stenosis are variable. However, when the pulmonary valve is stenotic and not well-developed, it has to be removed, and one has to accept severe pulmonary regurgitation as a sequela. In the ensuing years, when signs of damage to the right ventricle are proven, pulmonary valve replacement is indicated. We present the case of a 16-year-old patient with tetralogy of Fallot that was corrected during the first year of life. Follow-up demonstrated progressive exercise intolerance and moderate-to-severe right ventricle dilatation. Thus, surgical pulmonary valve replacement was indicated. The surgical approach was through a full median redo sternotomy. Central cardiopulmonary bypass was established with bicaval drainage. The operation was performed under assisted non-clamped circulatory support. The calcified previous pericardial patch was removed, and a 25-mm bioprosthetic valve was implanted in the pulmonary annulus. A new pericardial heterologous patch was used for the transannular pulmonary plasty.
Keywords: Absent pulmonary valve syndrome; Reoperation; Right ventricular failure; Tetralogy of Fallot.
© The Author 2023. Published by MMCTS on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.