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Scimitar syndrome associated with absence of the right pulmonary artery and a persistent primitive hepatic venous plexus

Published online by Cambridge University Press:  22 April 2005

Gabriella Agnoletti
Affiliation:
Service de Cardiologie Pédiatrique, Necker Enfants Malades, Paris, France
Damien Bonnet
Affiliation:
Service de Cardiologie Pédiatrique, Necker Enfants Malades, Paris, France
Jacques De Blic
Affiliation:
Service de Pneumologie Pédiatrique, Necker Enfants Malades, Paris, France

Abstract

An 18-month-old boy, referred because of an infection of the airways, was found to have a right-sided heart, a hypoplastic right lung, absence of the right pulmonary artery, and persistence of the hepatic venous plexus. The benign association of this unusual variant of the scimitar syndrome and persistence of the hepatic venous plexus needs to be recognised in order to avoid extensive investigations and surgery involving the inferior caval vein.

Type
Brief Report
Copyright
© 2005 Cambridge University Press

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References

Neill CA, Ferencz C, Sabiston DC, Sheldon H. The familial occurrence of hypoplastic right lung with systemic arterial supply and venous drainage: “scimitar syndrome”. Johns Hopkins Med J 1960; 107: 121.Google Scholar
Gao YA, Burrows PE, Benson LN, Rabinovitch M, Freedom RM. Scimitar syndrome in infancy. J Am Coll Cardiol 1993; 22: 873882.Google Scholar
Dupuis C, Remy J, Remy-Jardin M, Rey C, Brevietre GM, Kerboub A. Scimitar syndrome with anatomical and functional absence of the right pulmonary artery. Apropos of 4 cases. Arch Pediatr 1995; 2: 347352.Google Scholar
MacDonald C, Mikhailian H, Yoo SJ, Freedom RM, Adatia I. Angiographic findings of persistent primitive hepatic venous plexus with underdevelopment of the infrahepatic inferior vena cava in pediatric patients. Am J Roentgenol 2000; 175: 13971401.Google Scholar
Madan N, Moore JW. Unusual angiographic appearance of scimitar syndrome associated with primitive hepatic venous plexus. Circulation 2002; 105: e78.Google Scholar