Affordable Access

Publisher Website

When what appears to be mitral stenosis is not: Diagnostic and therapeutic implications

Authors
Publisher
Elsevier España
Identifiers
DOI: 10.1016/j.repce.2014.03.004
Keywords
  • Supravalvular Ring
  • Supravalvular Mitral Ring
  • Mitral Stenosis
  • Congenital Mitral Stenosis
  • Supravalvular Mitral Stenosis
  • Anel Supramitral
  • Anel Mitral Supravalvular
  • Estenose Mitral
  • Estenose Mitral Congénita
  • Estenose Mitral Supravalvular
Disciplines
  • Medicine

Abstract

Abstract The authors report the case of a 53-year-old man, with a long-standing history of mild mitral stenosis, admitted for worsening fatigue. Transthoracic echocardiography (limited by poor image quality) showed mitral annular calcification, leaflets that were difficult to visualize and an estimated mitral valve area of 1.8 cm2 by the pressure half-time method. However, elevated mean transmitral and right ventricle/right atrium gradients were identified (39 and 117 mmHg, respectively). This puzzling discrepancy in the echocardiographic findings prompted investigation by transesophageal echocardiography, which revealed an echogenic structure adjacent to the mitral annulus, causing severe obstruction (effective orifice area 0.7 cm2). The suspicion of supravalvular mitral ring was confirmed during surgery. Following ring resection and mitral valve replacement there was significant improvement in the patient's clinical condition and normalization of the left atrium/left ventricle gradient. Supravalvular mitral ring is an unusual cause of congenital mitral stenosis, characterized by an abnormal ridge of connective tissue on the atrial side of the mitral valve, which often obstructs mitral valve inflow. Few cases have been reported, most of them in children with concomitant congenital abnormalities. Diagnosis of a supravalvular mitral ring is challenging, since it is very difficult to visualize in most diagnostic tests. It was the combination of clinical and various echocardiographic findings that led us to suspect this very rare condition, enabling appropriate treatment, with excellent long-term results.

There are no comments yet on this publication. Be the first to share your thoughts.

Statistics

Seen <100 times
0 Comments

More articles like this

When what appears to be mitral stenosis is not: Di...

on Revista Portuguesa de Cardiolo...

[Mitral valve prolapse--diagnostic and therapeutic...

on Polski merkuriusz lekarski : o... August 2000

[Mitral stenosis and active diastole; diagnostic p...

on Rassegna clinico-scientifica May 1951

Invited letter concerning: what is the best therap...

on Journal of Thoracic and Cardio... September 1993
More articles like this..