Ventricular Fibrillation in a Patient with Marfan Syndrome without Severe Valvular Disease or Ventricular Dysfunction.
Mai Ishiwata, Kazutaka Ueda, Shunsuke Inoue et al.
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Case in brief
A 22-year-old man with Marfan syndrome experienced ventricular fibrillation (VF) despite having no severe valvular disease or significant ventricular dysfunction. Following resuscitation, he exhibited mild QT prolongation and slightly elevated B-type natriuretic peptide levels, but echocardiography showed preserved ejection fraction and only mild valvular regurgitation. Diagnosed with idiopathic VF, he received a subcutaneous implantable cardioverter-defibrillator.
Diagnostic / clinical pearl
Ventricular fibrillation can occur in Marfan syndrome patients without severe valvular disease or ventricular dysfunction, highlighting the need for vigilant monitoring of arrhythmia risk.
Why it matters
This case emphasizes the complexity of arrhythmia risk in Marfan syndrome, suggesting that subtle cardiac abnormalities may contribute to life-threatening events.