Transcatheter Pulmonary Valve Replacement in an Extra-Large RVOT Using a Custom-Made Pulmonary Valve

Abstract

Background: Transcatheter pulmonary valve replacement (TPVR) offers a less invasive alternative to surgical pulmonary valve replacement in patients with severe pulmonary regurgitation after tetralogy of Fallot repair. However, a complex right ventricular outflow tract (RVOT) anatomy can prevent the use of conventional TPVR devices. Case Summary: We report a patient with tetralogy of Fallot and a hypoplastic left pulmonary artery who had undergone transannular patch repair and developed severe pulmonary regurgitation. Imaging revealed an excessively dilated, aneurysmal RVOT, precluding the use of available TPVR systems. Discussion: A customized self-expandable endovascular graft system was created by incorporating a tailored pulmonary valve with fenestration for the left pulmonary artery. Preprocedural planning used multimodality imaging and three-dimensional printing for simulation. This is to our knowledge the first report of a patient-specific, custom-made TPVR system successfully used in a patient with a large, complex RVOT. Take-Home Messages: Careful assessments of RVOT morphology, branch pulmonary artery anatomy, and coronary relationships are essential for planning. This approach expands treatment options for anatomies unsuitable for standard TPVR devices.