Outcome predictors and implications for management of scimitar syndrome

Transcatheter intervention in a child with scimitar syndrome

“…Recently, multiple reports have shown transcatheter intervention, including embolisation of the APC and closure of the cardiac defects, may improve symptoms, decrease pulmonary arterial pressure,11,12 and postpone or even eliminate the need for surgical correction 7,8,13. Instead of a surgical approach, patients with scimitar syndrome who have significant left-to-right shunt due to APC and other cardiac defects are largely suitable for transcatheter intervention.…”

Signo de la Cimitarra

“…Se presenta más frecuentemente en mujeres; de patogenia poco clara (1,4) . Existen dos presentaciones clínicas: infantil, caracterizada por taquipnea, neumonías a repetición y falla cardiaca durante los primeros meses de vida, de mal pronóstico (1,8) , y adulta, donde la mayoría de los pacientes son asintomáticos, la patología es de curso benigno y el diagnóstico usualmente es un hallazgo imagenológico (3,4,8,11) . Lo anterior determinado por la presencia de un shunt de izquierda a derecha que, dependiendo de su magnitud, llevará al desarrollo de hipertensión pulmonar y falla cardiaca derecha (2,3) .…”

Scimitar Syndrome Repair in Adults: Intermediate-Term Results Using an Extracardiac Conduit

“…Vida and associates [10]) found that the incidence of scimitar vein stenosis was lower for patients who underwent an intracardiac baffle repair compared with patients who had direct reimplantation of the vein into the left atrium (18% vs 50%). Other investigators found no difference in the incidence of postoperative scimitar vein stenosis, regardless of the surgical technique used, but they did find a higher incidence in patients operated on in infancy [14]. However, promising results have been published by Brown and colleagues [5].…”

“…Thrombosis of the anastomosis could lead to catastrophic postoperative complications [9] (lung infarction, hemoptysis, pneumonectomy), although balloon dilation or stenting for scimitar-vein stenosis has been described [8,10]. Postoperative scimitar vein stenosis that requires reoperation or intervention in the cardiac catheterization laboratory is an important complication of scimitar syndrome repair, whatever the surgical technique used [9,10,14,17].…”

“…Patients presenting during their first year of life have a higher incidence of symptoms, extracardiac anomalies, aortopulmonary collateral vessels, coexisting congenital heart disease (eg, ventricular septal defect, pulmonary vein stenosis, or left-sided obstructive lesions), and pulmonary hypertension [10,14,15]. The infant group has a worse prognosis [13][14][15] compared with in whom the diagnosis is made later. The other form of presentation is the adolescent or adult form: patients are usually only mildly symptomatic with exertional dyspnea and recurrent respiratory infections [16].…”