Hostname: page-component-586b7cd67f-2plfb Total loading time: 0 Render date: 2024-12-02T21:56:36.254Z Has data issue: false hasContentIssue false
  • English
  • Français

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 ,
Damien Bonnet  and
Jacques De Blic
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
Get access Rights & Permissions [Opens in a new window]

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.

Keywords

Anomalous pulmonary venous connectionanomalous systemic venous connectioninferior caval vein
Type
Brief Report
Information
Cardiology in the Young , Volume 15 , Issue 2 , March 2005 , pp. 216 - 218
Copyright
© 2005 Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

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: 1–21.Google Scholar
Gao YA, Burrows PE, Benson LN, Rabinovitch M, Freedom RM. Scimitar syndrome in infancy. J Am Coll Cardiol 1993; 22: 873–882.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: 347–352.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: 1397–1401.Google Scholar
Madan N, Moore JW. Unusual angiographic appearance of scimitar syndrome associated with primitive hepatic venous plexus. Circulation 2002; 105: e78.Google Scholar