• S Huth, D Jäger, and J Barth.
• Medizinische Klinik, Berufsgenossenschaftliche Kliniken Bergmannstrost, Halle/S.
• Internist (Berl). 2007 May 1;48(5):532-4, 536.

AbstractAn 18-year-old, asymptomatic male underwent a routine chest x-ray examination which showed a pathological result. It revealed a characteristic crescent-like shadow in the right lower lung field, resembling a muslim sword (scimitar), responsible for the name of the underlying disease. Scimitar syndrome is a rare congenital malformation with a wide clinical spectrum ranging from asymptomatic to severe disturbances manifesting in childhood. The typical findings are hypoplasia of the right lung with marked mediastinal shift to the right and dextrocardia, reduced right pulmonary artery perfusion with maintained bronchial connection, anomalous arterial supply to the right lower lobe from the aorta and abnormal draining of the enlarged right pulmonary vein into the pulmonary circulation, producing the characteristic radiographic "scimitar sign". The development of right ventricular failure due to long-standing right ventricular overload and recurrent respiratory infections depend on the severity of anatomical abnormalities and functional disorders, sometimes requiring surgical intervention. Nowadays, sophisticated imaging techniques allow precise and rapid diagnosis of these complex abnormalities and their functional implications. The findings for our patient differed from the classic constellation, with a regular emptying of the enlarged right pulmonary vein into the left atrium, leading to a decreased burden on the central haemodynamics. In our very rare case of a scimitar syndrome variant, the functional disorders were negligible.

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