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      Síndrome de la cimitarra en resonancia magnética cardiovascular Translated title: Scimitar syndrome in a cardiovascular magnetic resonance scan

      case-report

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          Abstract

          Resumen El síndrome de la cimitarra es una anomalía parcial del retorno venoso pulmonar de características complejas. Está asociado a múltiples malformaciones cardiovasculares y pulmonares, y representa un diagnóstico diferencial importante en los pacientes con dilatación inexplicada de las cavidades derechas. Se expone el caso de una mujer en la cuarta década de la vida, con un cuadro clínico de dolor torácico, episodios sincopales y deterioro de su clase funcional, que por sus hallazgos en la ecocardiografía fue llevada a resonancia magnética cardíaca que mostró anomalía parcial del retorno venoso pulmonar infracardiaca, hallazgo no visualizado en la ecocardiografía, y confirmó el diagnóstico específico de síndrome de la cimitarra.

          Translated abstract

          Abstract Scimitar syndrome is a partial anomaly, of complex characteristics, of pulmonary venous return. It is associated with multiple cardiovascular and lung malformations, and requires a significant differential diagnosis in patients with an unexplained dilation of the right cavities. The case is presented of a woman, in the fourth decade of life, with a clinical picture of chest pain, syncopal episodes, and a deterioration of her functional class. Due to her findings in the echocardiograph, a cardiac magnetic resonance scan was performed that showed an infra-cardiac partial anomalous pulmonary venous return, a finding that was not observed in the echocardiograph, and confirmed the specific diagnosis of scimitar syndrome.

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          Most cited references8

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          Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study.

          Scimitar syndrome is a rare congenital heart disease. To evaluate the surgical results, we embarked on the European Congenital Heart Surgeons Association (ECHSA) multicentric study. From January 1997 to December 2007, we collected data on 68 patients who underwent surgery for scimitar syndrome. Primary outcomes included hospital mortality and the efficacy of repair at follow-up. Median age at surgery was 1.4 years (interquartile range, 0.46 to 7.92 years). Forty-four patients (64%) presented with symptoms. Surgical repair included intraatrial baffle in 38 patients (56%; group 1) and reimplantation of the scimitar vein onto the left atrium in 21 patients (31%; group 2). Eight patients underwent right pneumectomy, and 1 had a right lower lobe lobectomy (group 3). Four patients died in hospital (5.9%; 1 patient in group 1, 2.6%; 3 patients in group 3, 33%). Median follow-up time was 4.5 years. There were 2 late deaths (3.1%) resulting from severe pulmonary arterial hypertension. Freedom from scimitar drainage stenosis at 13 years was 83.8% in group 1 and 85.8% in group 2. Four patients in group 1 were reoperated, and 3 patients (2 in group 1 [6%] and 1 in group 2 [4.8%]) required balloon dilation/stenting for scimitar drainage stenosis. The surgical treatment of this rare syndrome is safe and effective. The majority of patients were asymptomatic at the follow-up control. There were a relatively high incidence of residual scimitar drainage stenosis that is similar between the 2 reported corrective surgical techniques used.
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            Magnetic resonance imaging of pediatric lung parenchyma, airways, vasculature, ventilation, and perfusion: state of the art.

            Magnetic resonance (MR) imaging is a noninvasive imaging modality, particularly attractive for pediatric patients given its lack of ionizing radiation. Despite many advantages, the physical properties of the lung (inherent low signal-to-noise ratio, magnetic susceptibility differences at lung-air interfaces, and respiratory and cardiac motion) have posed technical challenges that have limited the use of MR imaging in the evaluation of thoracic disease in the past. However, recent advances in MR imaging techniques have overcome many of these challenges. This article discusses these advances in MR imaging techniques and their potential role in the evaluation of thoracic disorders in pediatric patients.
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              Anomalous pulmonary venous connections.

              Developmental lung anomalies are classified into 3 main categories: bronchopulmonary (lung bud) anomalies, vascular anomalies, and combined lung and vascular anomalies. These anomalies are uncommon, and patients are at times asymptomatic; hence, identifying a developmental lung anomaly in the adult can be a challenge. Pulmonary vascular anomalies include interruption or absence of the main pulmonary artery, anomalous origin of the left pulmonary artery from the right pulmonary artery, anomalous pulmonary venous drainage (partial or complete), and pulmonary arteriovenous malformations. Systemic vascular anomalies comprise persistent left superior vena cava, anomalies of azygos and hemiazygos systems, and anomalies of the thoracic aorta and its major branches. In this article, we present embryology, classification, epidemiology, clinical presentation, and imaging features of anomalous pulmonary venous connections, with special emphasis on multidetector computed tomography and magnetic resonance imaging. These state-of-art imaging techniques have facilitated accurate and prompt diagnosis of these anomalies.
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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Role: ND
                Journal
                rcca
                Revista Colombiana de Cardiología
                Rev. Colomb. Cardiol.
                Sociedad Colombiana de Cardiologia. Oficina de Publicaciones (Bogota, Cundinamarca, Colombia )
                0120-5633
                February 2018
                : 25
                : 1
                : 81
                Affiliations
                [1] Medellín orgnameInstituto de alta tecnología médica (IATM) Colombia
                [2] Medellín Antioquía orgnameUniversidad CES Colombia
                Article
                S0120-56332018000100081
                10.1016/j.rccar.2017.02.005
                a09aaae1-1e4f-4737-9e5d-9d9c1b3e0a1d

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

                History
                : 18 November 2016
                : 20 February 2017
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 8, Pages: 1
                Product

                SciELO Colombia


                Hipertensión arterial pulmonar,Venas,Resonancia magnética cardiovascular,Congenital heart disease,Pulmonary arterial hypertension,Cardiopatía congénita,Veins,Cardiovascular magnetic resonance

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