A 34-year-old woman presents to the emergency department with palpitations and a syncopal episode during exercise. Medical history is significant for Ehlers-Danlos syndrome type IV. Physical examination reveals a mid-to-late systolic click followed by a late systolic murmur best heard at the apex. Vital signs are stable. Echocardiography demonstrates posterior displacement of the mitral leaflets during systole with mild mitral regurgitation. Which of the following best explains her risk for sudden cardiac death in the setting of this cardiac abnormality?

  1. A)Increased arrhythmogenic substrate from myxomatous leaflet redundancy and papillary muscle stretchGABARITO
  2. B)Progressive aortic root dilation with risk of acute aortic dissection
  3. C)Severe acute mitral regurgitation leading to cardiogenic shock
  4. D)Development of infective endocarditis from abnormal valve anatomy
  5. E)Coronary artery ostial abnormalities causing exercise-induced myocardial ischemia

Explicação

Mitral valve prolapse (MVP) in Ehlers-Danlos syndrome type IV is characterized by myxomatous leaflet redundancy and elongated chordae tendineae. The abnormal leaflet geometry and papillary muscle stretch create an arrhythmogenic substrate that predisposes to v... Ver explicação completa e trilha adaptativa →

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