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Related Experiment Videos

[Supravalvular aortic stenosis and coronary aneurysm].

J L Martínez Sande1, A Amaro Cendón, E Blanco Labrador

  • 1Sección de Cardiología, Complexo Hospitalario Cristal-Piñor, Orense.

Revista Espanola De Cardiologia
|October 1, 1992
PubMed
Summary

A young woman with Streptococcus mitis endocarditis experienced sudden cardiac arrest due to complex aortic valve disease. This case highlights the critical association between supravalvular aortic stenosis and coronary artery anomalies.

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Area of Science:

  • Cardiology
  • Cardiovascular Surgery
  • Medical Genetics

Background:

  • Endocarditis caused by Streptococcus mitis can lead to severe valvular complications.
  • Supravalvular aortic stenosis (SVAS) is a rare congenital heart defect.
  • Coronary artery anomalies are increasingly recognized in association with bicuspid aortic valves and other aortic pathologies.

Observation:

  • A 32-year-old woman presented with endocarditis and a significant systolic murmur.
  • Doppler echocardiography revealed a 150 mmHg systolic gradient across the aortic valve.
  • Aortography demonstrated multiple membranous supravalvular aortic stenosis, aneurysmal dilatation of the left main and circumflex coronary arteries, a bicuspid aortic valve, and mild aortic insufficiency.

Findings:

  • The patient suffered sudden cardiac arrest and died before surgical intervention.

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  • The complex interplay between SVAS, coronary artery anomalies, and bicuspid aortic valve was implicated.
  • Despite the lack of autopsy, the clinical presentation strongly suggested a syndromic association.

    • Implications:

    • This case underscores the importance of thorough cardiovascular evaluation in patients with endocarditis, especially when associated with murmurs and suspected valvular disease.
    • Early recognition and management of associated coronary artery anomalies are crucial in patients with SVAS to prevent sudden cardiac death.
    • Further research into the genetic and developmental mechanisms underlying SVAS and associated anomalies may improve diagnostic and therapeutic strategies.