Clinical characteristics, imaging findings, management, and outcomes of patients with scimitar syndrome at a tertiary referral healthcare center in Colombia

Ana M Aristizabal; Carlos A Guzmán-Serrano; Nancy Vanessa Mondol-Villamil; Lina Maria Bolaños-Vallejo; Valentina Mejia-Quiñones; Maria Alejandra Recio-Gómez; Enrique Carlos García-Pretelt; Mauricio Mejía-González; Walter Mosquera Alvarez; Jaiber Alberto Gutiérrez-Gil

doi:10.1007/s10554-024-03102-1

Clinical characteristics, imaging findings, management, and outcomes of patients with scimitar syndrome at a tertiary referral healthcare center in Colombia

Int J Cardiovasc Imaging. 2024 Apr 18. doi: 10.1007/s10554-024-03102-1. Online ahead of print.

Affiliations

¹ Facultad de Ciencias de la Salud, Facultad de Ciencias de la Salud, Universidad Icesi, Cali, Colombia.
² Departamento materno-infantil, Fundación Valle del Lili, Cardiología pediátrica, Cali, Colombia.
³ Centro de Investigaciones Clínicas, Fundación Valle del Lili, Av. Simón Bolívar - Carrera 98 # 18-49 Cali, Cali, Colombia. carlos.guzman.s@fvl.org.co.
⁴ Centro de Investigaciones Clínicas, Fundación Valle del Lili, Av. Simón Bolívar - Carrera 98 # 18-49 Cali, Cali, Colombia.
⁵ Departamento de Radiología e imágenes Diagnósticas, Fundación Valle del Lili, Cali, Colombia.

PMID: 38634941
DOI: 10.1007/s10554-024-03102-1

Abstract

Scimitar Syndrome is part of a complex spectrum of congenital cardiovascular anomalies related to anomalous pulmonary venous return. Depending on the extent of involvement, treatment can be either expectant or surgical. Prognosis and survival have been controversial, with some results supporting early surgical management. This research aims to disclose the outcomes and describe the management, clinical and imaging characteristics of patients diagnosed with Scimitar Syndrome treated in a tertiary referral healthcare center. Longitudinal descriptive observational study. The study included all patients diagnosed with scimitar syndrome in our institution between January/2011 and December/2022. A description of the sociodemographic and clinical characteristics, diagnostic tools used, treatment features, and patient outcomes is provided. Eleven patients were included, with a mean age at diagnosis of five years (CI 0-17), six of which were female (54.55%). Nine (81.82%) patients had evidence of a scimitar vein on the chest radiograph, six (54.55%) cardiac dextroposition, six (54.55%) pulmonary hypoplasia, five (45.45%) right pulmonary artery hypoplasia, and three (27.27%) had aortopulmonary collaterals. Four (36.36%) patients had horseshoe lungs, and four (36.36%) had bronchopulmonary sequestration. In the associations, two (18.18%) patients were found to have an atrial septal defect, three (27.27%) ventricular septal defect, and one (9%) had Tetralogy of Fallot. Pulmonary hypertension was demonstrated in two (18.18%) patients. Seven (63.64%) required surgical management to correct the scimitar vein, and two patients died due to unrelated complications. Scimitar syndrome presents diagnostic and treatment challenges, necessitating a multidisciplinary approach for timely care. Chest radiography and CT scans are primary diagnostic tools, with surgical intervention often warranted alongside other heart defects or significant hemodynamic repercussions. Medical management is effective for mild to moderate cases. Long-term patient outcomes remain uncertain due to study limitations, but improved life expectancy is anticipated with ongoing care.

Keywords: Chest radiography; Computed tomography; Echocardiography; Heart disease; Magnetic resonance; Scimitar syndrome.