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Palpitations in a middle-aged male… Searching a needle in a haystack

Published:January 10, 2022DOI:https://doi.org/10.1016/j.ejim.2022.01.005

      Keywords

      1. Case description

      A gentleman in his 50s presented with episodes of intermittent palpitations which were self-limiting. His symptoms were not related to exertion and had short duration with sudden onset and offset. He had no prior comorbidities and denied history of syncope. Cardiovascular system examination was unremarkable. Baseline electrocardiogram is shown in Fig. 1A. The electrocardiogram during an episode of palpitations requiring urgent hospitalization is shown in Fig. 1B. He was treated with intravenous amiodarone infusion and subsequently switched to oral anti arrhythmic medications.
      Fig 1
      Fig. 1Baseline ECG and ECG during palpitations.
      A. It shows a sinus rhythm with a broad QRS and T-wave inversions in leads V1-V4. There are low-amplitude small deflections between the end of QRS complexes and the onset of the T waves in leads V1-V4.
      B.
      It shows a ventricular tachycardia with left bundle branch block morphology, superior axis and a negative precordial concordance.
      What is the diagnosis suggested by these two electrocardiograms?

      2. Discussion

      2.1 Diagnosis: Arrhythmogenic right ventricular cardiomyopathy

      Electrocardiogram in Fig. 1A shows a sinus rhythm with a heart rate of 62 beats per minute. There is slight QRS widening (130 milliseconds) and T-wave inversion in precordial leads. On closer inspection, there are low-amplitude small deflections between the end of QRS complexes and the onset of the T waves in leads V1-V4. These deflections, also eponymously known as ‘Epsilon waves’, are pathognomonic of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) [
      • Hoffmayer K.S.
      • Scheinman M.M.
      Electrocardiographic patterns of ventricular arrhythmias in arrhythmogenic right ventricular dysplasia/cardiomyopathy.
      ]. Additional features suggesting the diagnosis are localized QRS widening and T-inversions in leads V1-V4. Subsequent two-dimension transthoracic echocardiogram confirmed the diagnosis.
      The electrocardiogram shown in Fig. 1B depicts a wide complex tachycardia with a ventricular rate of 170 beats/min, with left bundle branch block morphology and superior axis. The precordial leads show a negative concordant pattern. These findings may suggest a ventricular tachycardia originating from inferior aspect of right ventricle.
      The underlying pathophysiologic substrate in ARVC is replacement of right ventricular myocardial tissue with fibrous and fatty tissue which facilitates the development of re-entrant ventricular arrhythmias. The characteristic low-amplitude potential Epsilon waves are the depolarization abnormalities in precordial leads and inverted T waves are the repolarization abnormalities. Epsilon waves are present in only a minority of patients but, if seen, are pathognomonic for ARVC [
      • Marcus F.I.
      • McKenna W.J.
      • Sherrill D.
      Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force Criteria.
      ]. Considering the malignant potential of arrhythmias associated with ARVC, early diagnosis and prompt risk stratification and family screening is important.

      Declaration of Competing Interest

      None.

      Acknowledgements

      None.

      References

        • Hoffmayer K.S.
        • Scheinman M.M.
        Electrocardiographic patterns of ventricular arrhythmias in arrhythmogenic right ventricular dysplasia/cardiomyopathy.
        Front Physiol. 2012; 3: 23https://doi.org/10.3389/fphys.2012.00023
        • Marcus F.I.
        • McKenna W.J.
        • Sherrill D.
        Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force Criteria.
        Eur Heart J. 2010; 31 (Apr): 806-814https://doi.org/10.1093/eurheartj/ehq025