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Lutembacher Syndrome With Unroofed Left Superior Vena Cava: A Diagnostic Dilemma

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Abstract

Lutembacher syndrome involving the association of congenital atrial septal defect (ASD), usually of the ostium secundum variety, and mitral valve disease is a well-known entity. Its association with a coronary sinus, ASD, and a persistent left superior vena cava (LSVC) draining into the left atrium (LA) (Raghib syndrome) is rarely described in the literature. This association in a 15-year-old boy erroneously deemed to be inoperable before referral to the authors’ hospital due to cyanosis in the presence of atrial septal defect (ASD) and mitral stenosis is described in this report. Evaluation by echocardiography followed by cine angiography confirmed the cause of cyanosis to be drainage of the LSVC into the LA together with an ASD and rheumatic mitral stenosis, a combination of Raghib and Lutembacher syndromes. The boy underwent successful surgical correction. The authors believe this is the second such case to be reported in the English literature and the first of its kind to be managed by surgical intervention.

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Author information

Correspondence to Neeraj Awasthy.

Electronic supplementary material

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Movie clip 1 Apical 4c view showing restricted opening of the mitral valve suggestive of mitral stenosis. Also note the prominence of the RA and right ventricle due to the associated coronary sinus type of ASD (not seen in this view). (AVI 1,556 kb)

Movie clip 2 Two-dimensional contrast echocardiography with an apical 4c view and injection of agitated saline in the left antecubital vein showing filling of the LA and left ventricle, with a few bubbles going immediately into the RA. This was followed by subsequent filling of the RA and right ventricle. (AVI 1,485 kb)

Movie clip 1 Apical 4c view showing restricted opening of the mitral valve suggestive of mitral stenosis. Also note the prominence of the RA and right ventricle due to the associated coronary sinus type of ASD (not seen in this view). (AVI 1,556 kb)

Movie clip 2 Two-dimensional contrast echocardiography with an apical 4c view and injection of agitated saline in the left antecubital vein showing filling of the LA and left ventricle, with a few bubbles going immediately into the RA. This was followed by subsequent filling of the RA and right ventricle. (AVI 1,485 kb)

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Awasthy, N., Ambatkar, P., Radhakrishnan, S. et al. Lutembacher Syndrome With Unroofed Left Superior Vena Cava: A Diagnostic Dilemma. Pediatr Cardiol 34, 1985–1988 (2013) doi:10.1007/s00246-012-0524-0

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Keywords

  • Coronary sinus type of atrial septal defect
  • Lutembacher syndrome
  • Mitral valve disease
  • Raghib syndrome
  • Unroofed left superior vena cava