A Rare Associated Anomaly in Tetralogy of Fallot: Scimitar Syndrome in an Infant

Abstract Pulmonary venous connections may be infrequently abnormal in patients with tetralogy of Fallot (TOF). A special subgroup of partial anomalous pulmonary venous return,"scimitar cyndrome", and its coexistence with TOF is less frequently reported. It may proceed unnoticed, as cyanosis already predominates in the clinical picture. This uncommon association must be kept in mind for patients with TOF who have an accessory flow in the inferior vena cava, especially when all pulmonary venous return to the left atrium is not clearly seen.

Successful repair of infantile scimitar syndrome with contralateral pulmonary vein stenosis in an infant

Abstract Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.

Síndrome de Cimitarra: revisión a partir de un caso clínico / Scimitar syndrome: a review based on a clinical case

Resumen Así como otros síndromes congénitos considerados raros por su baja incidencia, el diagnóstico del síndrome de la Cimitarra se basa en la presencia de un drenaje venoso anómalo de las venas pulmonares derechas que en muchas ocasiones se asocia con la presencia de otras malformaciones pulmonares y cardíacas. La gran mayoría pasan desapercibido puesto que los síntomas pueden manifestarse hasta en edades adultas y puede pasarse por alto. A propósito de un caso clínico, presentamos una revisión de este tema, que es importante tomar en cuenta para darles a estos pacientes un seguimiento y tratamiento adecuados.

Abstract As other congenital syndromes are considered rare based on their low incidence, the diagnosis of scimitar síndrome is based on the presence of an abnormal right venous pulmonary drainage, accompanied in many cases with other pulmonary and cardiac malformations. The vast majority of them has been undiagnosed, because the symptoms present later in adulthood. With regard to a clinical case, we present a review of this topic, which is important to take into account to give this patients a follow-up and adequate treatment.

Mal-positioning of dialysis catheter in anomalous left superior pulmonary vein in a patient with acute type a dissection, a case report

Abstract The partial anomalous pulmonary vein drainage is a rare congenital defect. The pulmonary vein drains in to a systemic vein instead of draining in to the left atrium. In this rare birth defect, the right sided pulmonary vein involvement is more prevalent than the left sided pulmonary veins. We present a case where the anomalous left superior pulmonary vein was diagnosed when a renal dialysis catheter (size = 12F x 16cm) was mal-positioned in to the Anomalous left superior pulmonary vein, demonstrating confusing blood results. We describe how a systematic multidisciplinary approach and use of advanced imaging techniques can recognise and deal with this rare clinical dilemma.

Síndrome de cimitarra, valoración con ecocardiografía transesofágica intraoperatoria. Informe de caso / Scimitar syndrome; intraoperative transesophageal echocardiography assessment. Case report

9-year-old patient diagnosed with scimitar syndrome through transthoracic echocardiogram and cardiac catheterization. Surgical correction is performed by anastomosing the collector of the pulmonary right vein to AI through the interatrial septum, tunneling it with a pericardic patch. The study findings and echocardiographic images of such surgical repair and the spectrum of pulsed Doppler differentiating between right and left pulmonary veins will be shown below. The connection through the septum was evidenced, simulating an interatrial communication. A difference was found among the measured points S, D, A and between the left and right pulmonary veins, without this resulting in a stenosis during the repair.

Paciente de 9 años de edad el cual es diagnosticado con síndrome de cimitarra a través de ecografía transtorácica y cateterismo cardiaco. Se realiza cirugía correctora la cual se anastomosa el colector de vena pulmonar derecha hacia AI a través del septum interauricular, tunelizándolo con parche pericárdico. A continuación se muestran los hallazgos e imágenes ecocardiográficas de tal reparación y el espectro doppler pulsado diferenciado entre las venas pulmonares derechas e izquierdas. Se pudo evidenciar la conexión a través del septum, simulando una comunicación interauricular. Se halló una diferencia entre los puntos medidos S, D, A entre las venas pulmonares izquierdas y derechas, sin que esto significara una estenosis en la reparación.

Signo de la cimitarra / Scimitar sign

The scimitar sign refers to the image resembling a short, curved Turkish sword called scimitar, created by an anomalous pulmonary vein. It can be seen in chest radiography, computed tomography and magnetic resonance. It forms part of the scimitar syndrome, characterized by partial or total anomalous pulmonary venous drainage, associated with hypoplasia of the right lung and dextrocardia. Depending on the magnitude of the right-to-left shunt, it may present in children with pulmonary hypertension and right cardiac failure, or as asymptomatic imaging findings in adults.

El signo de la cimitarra es la imagen creada por una vena pulmonar anómala similar a una espada turca, corta y curva llamada cimitarra. Se puede ver en radiografía de tórax, tomografía computada y resonancia magnética. Forma parte del síndrome de la cimitarra, caracterizado por un drenaje pulmonar anómalo, parcial o total, asociado a hipoplasia del pulmón derecho y dextrocardia. Dependiendo de la magnitud del shunt de derecha a izquierda producido, se puede presentar en edades infantiles con hipertensión pulmonar y falla cardiaca derecha, o en adultos, de manera asintomática, como hallazgo imagenológico.

Partial anomalous pulmonary venous return [scimitar syndrome]: multislice computer tomography

New cardiovascular imaging techniques provide high resolution and better anatomical assessment of cardiovascular malformations. We present a three dimensional reconstruction of scimitar syndrome using multislice computer tomography. The scimitar syndrome, also called venolobar syndrome and hypogentic lung syndrome, is a rare congenital cardiovascular anomaly involving the right lung, in its complete form, the syndrome consists of ipsilateral anomalous pulmonary drainage of part or all of the right lung into the inferior vena cava, hypoplasia of the right lung, dextrorotation of the heart, hypoplasia of the right pulmonary artery and anomalous systemic arterial supply to the lower lobe of the right lung from the subdiaphargmatic aorta or its main branches

Report of two cases of scimitar syndrome: the classical one with subaortic membrane and the scimitar variant

Scimitar syndrome is characterized by an anomalous pulmonary vein draining into the inferior vena Cava, visible roentgenographically as a crescentic shadow of vascular density along the right border of the cardiac silhouette and dextroposition of the heart. We are reporting two cases of Scimitar sydrome; one being classical associated with subaortic membrane and the other a Scimitar variant. The clinical and anatomical spectrum for each case is described