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Journal of Radiological Review 2020 November-December;7(6):468-74

DOI: 10.23736/S2723-9284.20.00081-5


language: English, Italian

Scimitar syndrome (or Halasz Syndrome): description of a case originating in adulthood

Maria PETULLÀ , Laura COMITO, Valentina SETTINO, Lorenzo ZAPPIA, Domenico LAGANÀ

Unit of Radiology, Department of Experimental and Clinical Medicine, Mater Domini University Hospital, Catanzaro, Italy


Congenital anomalous pulmonary venous return is part of congenital heart disease and is characterized by the presence of all or some pulmonary veins that drain into the right atrium or its tributary veins. When at least one pulmonary vein drains into the left atrium, it is referred to as partial anomalous pulmonary venous return (RVPAP). The scimitar syndrome is part of this group of congenital heart pathologies. In affected individuals, the right pulmonary veins, partially or completely, drain into the inferior vena cava rather than into the left atrium. It has a rather low incidence, 2/100,000 births, and can be associated with other congenital heart anomalies, the most frequent is the inter-atrial defect. The name derives from the characteristic sign, the sign of scimitar, found on radiological examination of the chest. Chest CT angiography is certainly the most sensitive and specific examination for diagnosis and evaluation of some other associated anomalies. Clinical features depend on degree of left-to-right shunt. In sever forms, diagnosis is early and surgical treatment is timely. In some cases, diagnosis can be incidental, in completely asymptomatic subjects. In other cases, symptoms are mild and diagnosis occurs in adulthood. The clinical case that is described in this work falls into the latter condition. She is a 52-year-old woman with a history of recurrent bronchopneumonia episodes and an enlargement of the right heart sections on an echo-color-doppler examination of the heart.

KEY WORDS: Scimitar syndrome; Computed tomography angiography; Pulmonary veins

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