Interestingly, the repolarization abnormalities were changing and became more prominent in the left lateral precordial leads during follow-up (figure 1). The conduction abnormalities worsened, as a complete RBBB associated with a left fascicular anterior block associated with permanent long AV block appeared four years later. Non-sustained VT (NSVT) persisted despite anti-arrhythmic therapy and an ablation was attempted that revealed five different VTs coming from both ventricles including one of epicardial origin. An ICD was then implanted. The patient secondarily developed persistent atrial fibrillation that needed cardioversion.
Figure 1 : Top : Electrocardiograms of the proband between 2007 and 2011. At the time of diagnosis (2007), T-wave inversion was present in all precordial leads but later was only present in left lateral leads. Please notice the three different morphologies of ventricular ectopies : A with RBBB pattern and right axis suggestive of lateral LV origin, B : narrow QRS with normal axis and V1-V2 transition suggestive of septal origin, C: LBBB with inferior axis suggestive of RVOT origin; D : same morphology as A but with wider QRS suggestive of epicardial origin. * : note the worsening of conduction disease with apparition of complete RBBB with left axis and long AV block. Bottom : clinical VT with LBBB and inferior axis suggesting VT from RVOT.
The familial history revealed that the mother suddenly died aged 80 (figure 2). The maternal uncle was implanted with a pace-maker but died suddenly aged 80. Two maternal aunts displayed heart failure and suddenly died aged 60. The maternal grandfather suddenly died aged 51. The sister experienced several episodes of syncope from age 50 and was implanted with a pace-maker aged 67 due to high-degree AV block. She further developed paroxysmal atrial fibrillation and ventricular arrhythmias with NSVT. The echocardiography was normal. The 28 yo daughter had a normal echocardiography but displayed atrial ectopies and NSVT.
Figure 2 : Pedigree of the family. The arrow indicates the proband. Squares indicate male and circles female. Affected individuals are shown with solid symbols, unaffected with white symbols and possibly affected in grey. ICD: implantable cardiac defibrillator; HF: heart failure; VT: ventricular tachycardia; NSVT: non-sustained VT; AF: atrial fibrillation; AEc: atrial ectopies; PM: pace-maker; SD: sudden death
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