RESUMEN
The surgical management of d-transposition of great arteries (d-TGAs) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) is ever evolving and still remains a challenge because of wide anatomic variability, age of presentation, surgical options available, and their variable long-term results in different series. We describe a patient with d-TGA, VSD, and LVOTO who presented to us at 13 years of age and underwent an arterial switch operation along with neoaortic valve replacement with a mechanical prosthesis. The postoperative course was uneventful, and at hospital discharge, the echocardiogram was satisfactory. We present the pros and cons of this hitherto undescribed treatment option.
Asunto(s)
Anomalías Múltiples , Válvula Aórtica/cirugía , Operación de Switch Arterial/métodos , Defectos del Tabique Interventricular/cirugía , Implantación de Prótesis de Válvulas Cardíacas/métodos , Transposición de los Grandes Vasos/cirugía , Obstrucción del Flujo Ventricular Externo/cirugía , Adolescente , Válvula Aórtica/anomalías , Válvula Aórtica/diagnóstico por imagen , Ecocardiografía , Femenino , Defectos del Tabique Interventricular/diagnóstico , Humanos , Transposición de los Grandes Vasos/diagnóstico , Obstrucción del Flujo Ventricular Externo/diagnósticoRESUMEN
Holt-Oram syndrome is a rare inherited disorder involving the hands, arms, and the heart. The defects involve carpal bones of the wrist and the thumb and the associated cardiac anomalies like atrial or ventricular septal defects. Congenital cardiac and upper-limb malformations frequently occur together and are classified as heart-hand syndromes. The most common amongst the heart-hand disorders is the Holt-Oram syndrome, which is characterized by septal defects of the heart and preaxial radial ray abnormalities. Its incidence is one in 100,000 live births. Approximately three out of four patients have some cardiac abnormality with common associations being either an atrial septal defect or ventricular septal defect. Herein, we report a rare sporadic case of Holt-Oram syndrome with atrial septal defect with symptoms of heart failure in a forty-five-year-old lady who underwent emergency cardiac surgery for the symptoms.