Article

Scimitar syndrome.

Chronic Respiratory Disease Center, National Research Institute of Tuberculosis and Lung Disease (NRITLD), Masih Daneshvari Hospital, Shaheed Beheshti University of Medical Sciences, Tehran, Iran.
Archives of Iranian medicine (Impact Factor: 1.22). 02/2009; 12(1):79-81.
Source: PubMed

ABSTRACT Scimitar syndrome or congenital pulmonary venolobar syndrome is a rare anomaly most commonly consisting of partial pulmonary venous drainage into the hepatic portion of the inferior vena cava, right lung hypoplasia, dextroposition of the heart, and anomalous systemic arterial supply from aorta or one of its branches to the right lung. We report a four-year-old girl with recurrent pneumonia and failure to thrive, who was diagnosed as having scimitar syndrome.

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    ABSTRACT: Scimitar syndrome (pulmonary venolobar syndrome) is a rare anomaly of venous return to the heart, most commonly consisting of partial or total anomalous pulmonary venous return from the right lung. This is the report of a case of a 29-year-old woman at 31 weeks of gestation of pregnancy who was previously diagnosed with scimitar syndrome. MR angiography and PET-CT results which were obtained before pregnancy demonstrated vascular malformation in the inferior part of the right lung. No specific treatment was planned throughout the pregnancy due to the absence of any symptoms. The patient's first physical examination was unremarkable except mild hypertension. In her follow-up, severe preeclampsia was developed and the patient had undergone a cesarean section of a live birth at 34 weeks and 2 days of gestation. This is the first case of scimitar syndrome with pregnancy in which the cardiac status of the patient deteriorated coincidentally due to the development of another manifestation such as severe preeclampsia besides the syndrome itself.
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    ABSTRACT: Anormal drainage of the pulmonary veins into the inferior vena cava is known as scimitar syndrome. Scimitar syndrome often presents during infancy and rarely during adulthood, and the adult patients are mostly asymptomatic. It is usually in association with dextrocardia, hypoplasia of right lung, and congenital heart defects. However, interruption of inferior vena cava with azygous continuation is rarely associated with this syndrome.
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    ABSTRACT: Scimitar syndrome and gastrointestinal bleeding from an aberrant right subclavian artery-esophageal fistula are each extremely rare. Although scimitar syndrome and aberrant right subclavian artery are typically asymptomatic in adults, fistulous connection between the aberrant artery and the esophagus is associated with a poor prognosis. Outcomes are contingent upon timely diagnosis and prompt surgical repair. Prolonged nasogastric and endotracheal intubation can lead to gastrointestinal bleeding in patients who have an aberrant right subclavian artery or other vascular ring. We recommend neither embolization nor the use of endovascular stents as anything other than a temporizing measure in the management of aberrant right subclavian artery injury. These methods can stop acute hemorrhage; however, sentinel bleeding will eventually occur and require definitive ligation.We report the case of a 57-year-old woman in whom an aberrant right subclavian artery-esophageal fistula developed after surgical correction of symptomatic scimitar syndrome. Massive gastrointestinal bleeding resulted from prolonged nasogastric and endotracheal intubation. To our knowledge, this is the first report of aberrant right subclavian artery and scimitar syndrome in the same patient, and the 4th report of a patient's surviving a fistula between the aberrant artery and the esophagus.
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