RESUMO
We report a case of a 25-year-old male with a heterotaxy-like constellation of congenital heart defects consisting of complete atrioventricular septal defect, transposition of the great arteries, subpulmonary stenosis, L-looped ventricles, hypoplastic right ventricle, and a distant aorta arising from the right ventricle. This case demonstrates how 3D printing and interactive 3D visualization may facilitate a unique surgical repair.
Assuntos
Defeitos dos Septos Cardíacos , Síndrome de Heterotaxia , Transposição dos Grandes Vasos , Masculino , Humanos , Adulto , Transposição dos Grandes Vasos/cirurgia , Ventrículos do Coração/cirurgia , Impressão TridimensionalRESUMO
We report a case of a 38-year-old female with an FLNA variant who underwent valve-sparing aortic root replacement. FLNA encodes Filamin A, an actin-binding protein. Our patient had aortic root dilation due to this variant. Aortic root repair was conducted using the David procedure, with modifications to account for tissue fragility associated with this genetic condition. This case demonstrates the value of patient-specific genetic information for the timing of surgery and operative course planning.
