Hostname: page-component-78c5997874-m6dg7 Total loading time: 0 Render date: 2024-11-10T07:57:12.463Z Has data issue: false hasContentIssue false
  • English
  • Français

Severe pulmonary hypertension secondary to a parachute-like mitral valve, with the left superior caval vein draining into the coronary sinus, in a girl with Turner's syndrome

Published online by Cambridge University Press:  24 May 2005

Marc van Heerde ,
Jaroslav Hruda  and
Mark G. Hazekamp
Marc van Heerde
Affiliation:
Department of Paediatrics, VU University Medical Center, Amsterdam, The Netherlands
Jaroslav Hruda
Affiliation:
Department of Paediatrics, VU University Medical Center, Amsterdam, The Netherlands
Mark G. Hazekamp
Affiliation:
Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, The Netherlands
Get access Rights & Permissions [Opens in a new window]

Abstract

A 17-year-old girl with Turner's syndrome underwent two cardiac operations due to severe mitral stenosis with pulmonary hypertension, caused by a parachute-like mitral valve. The anomaly was associated with persistence of the left superior caval vein, which drained to the coronary sinus, and non-compaction of the left ventricular myocardium. The association of these lesions is rare in patients with Turner's syndrome.

Keywords

Mitral valveXO syndromeleft superior vena cava
Type
Brief Report
Information
Cardiology in the Young , Volume 13 , Issue 4 , August 2003 , pp. 364 - 366
Copyright
© 2003 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

Mazzanti I, Cacciati E. Congenital heart disease in patients with Turner's syndrome. Italian Study Group for Turner Syndrome (ISGTS). J Pediat 1998; 133: 688–692.Google Scholar
Sybert VP. Cardiovascular malformations and complications in Turner syndrome. Pediatrics 1998; 101: E11.Google Scholar
Bastianon V, Pasquino AM, Giglioni E, et al. Mitral valve prolapse in Turner syndrome. Eur J Pediatr 1989; 6: 533–534.Google Scholar
Gotzsche CO, Krag-Olsen B, Nielsen J, Sorenson KE, Kristensen BO. Prevalence of cardiovascular malformations and association with karyotypes in Turner's syndrome. Arch Dis Child 1994; 71: 433–436.Google Scholar
Hilton T, Castello R, Ohar J, Kong B, Kern M, Labovitz A. Persistent left superior vena cava in Turner's syndrome: a transesophageal echocardiographic study. Am Heart J 1992; 123: 234–236.Google Scholar
Douchin S, Rossignol AM, Klein SK, Siche JP, Baguet JP, Bost M. Heart malformations and vascular complications associated with Turner's syndrome. Prospective study of 26 patients. Arch Mal Coeur Vaiss 2000; 93: 565–570.Google Scholar
Cochrane AD, Marath A, Mee FBB. Can a dilated coronary sinus produce left ventricular inflow obstruction? An unrecognized entity. Ann Thorac Surg 1994; 58: 1114–1116.Google Scholar
Agnoletti G, Annecchino F, Preda L, Borghi A. Persistence of the left superior caval vein: can it potentiate obstructive lesions of the left ventricle? Cardiol Young 1999; 9: 285–290.Google Scholar
Macedo AJ, Oosthoek PW, Wenink ACG, Bartelings MM. Does persistent left superior vena cava to coronary sinus influence the growth of the left heart in the fetus? Cardiol Young 1996; 6 (Suppl 1): S43.Google Scholar
Oosthoek PW, Wenink ACG, Wisse LJ, Gittenberger-de Groot AC. Development of the papillary muscles of the mitral valve: morphogenetic background of parachute-like asymmetric mitral valves and other mitral valve anomalies. J Thorac Cardiovasc Surg 1998; 116: 36–46.Google Scholar