In Ross patients who require reintervention on the PV homograft, both tPVR and sPVR provide low procedural mortality and comparable midterm outcome with no significant difference in mortality or PV reintervention. However, IE is more common following tPVR. A larger randomized study is needed to determine the role of each procedure in patient management.
Hyperimmunoglobulin E syndrome (HIES) is a primary immunodeficiency disorder with multisystem abnormalities including the vascular system. We report a child with autosomal recessive (AR)-HIES secondary to dedicator of cytokinesis 8 (DOCK8) deficiency who developed critical aortic aneurysm involving the ascending aorta and aortic arch with narrowing of descending aorta that was successfully managed surgically. This report highlights the underrecognized and serious complication of DOCK8 deficiency that could contribute to significant morbidity and mortality in such patients.
Percutaneous pulmonary valve implantation is increasingly adopted as an alternative procedure to surgery in dysfunctional homograft, and in patients with "native" or wide right ventricle outflow tract dysfunction. Pre-stenting is mandatory in this category of patients for many reasons, one of which is to create an adequate landing zone for the bioprosthesis. Here we report on a tricky situation that occurred during pre-stenting, and we describe how we successfully overcame it.
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