Arrhythmic mitral valve prolapse in Kohlschütter–Tönz syndrome

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  Figure 1. ECG and imaging features of mitral valve prolapse in 41-year-old patient with Kohlschütter–Tönz syndrome. Baseline 12-lead electrocardiogram (A) shows sinus rhythm with T-wave inversion in the inferior leads (II, III, aVF) and isolated premature ventricular contraction with right bundle branch block pattern and superior axis morphology. Transthoracic echocardiography showed mitral valve prolapse with inferolateral mitral annular disjunction (red arrow) and mild mitral regurgitation (B–C). Tissue Doppler imaging of the lateral mitral annulus showing a Pickelhaube sign (S′ velocity 25 cm/s) (D). A 24-h 12-lead Holter monitoring revealed a non-sustained monomorphic ventricular tachycardia with right bundle branch block and superior axis morphology.