This ECG Does Not Fit

Figures

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  Visual Summary. Progressive Left Ventricular Hypertrophy With an Atypical Electrocardiogram in a 70-Year-Old Patient With Several Comorbidities A 70-year-old man with hypertension (HTN), diabetes mellitus (DM), chronic kidney disease (CKD), prior percutaneous coronary intervention (PCI) on the left anterior descending artery (LAD), with progressive left ventricular hypertrophy (LVH) during follow-up (FU). Electrocardiogram (ECG) showed an atypical pattern with right bundle branch block (RBBB). Cardiac magnetic resonance (CMR) demonstrated reduced native T1 values. Genetic testing identified the p.N215S (p.Asn215Ser) mutation, confirming late-onset Anderson-Fabry disease (AFD).

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  Figure 1. Electrocardiogram Performed Right After the First Hospitalization of Heart Failure Electrocardiogram showing demonstrated atrial fibrillation, normal QTc interval, QRS axis of 31°, complete right bundle branch block with a QRS duration of 136 ms, pathologic Q waves in the inferior leads, and nonspecific T-wave abnormalities.

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  Figure 2. Electrocardiogram Evolution During a 14-Year Span Before AFD Diagnosis Electrocardiogram (A) performed 14 years before diagnosis, showing PR interval at the lower limit of normal, signs of left ventricular hypertrophy, and T-wave inversion in the lateral leads. Electrocardiogram (B) performed 10 years before Anderson-Fabry disease (AFD) diagnosis, with prolongation of PR interval and relative reduction of repolarization abnormalities. Electrocardiogram (C) shows development of right bundle branch block at electrocardiogram performed few months before heart failure decompensation and AFD diagnosis.

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  Figure 3. Cardiac Magnetic Resonance Findings Left: 4-chamber view from the cine sequences left ventricular hypertrophy with hypertrophy of the papillary muscles, atrial enlargement, and mild pericardial effusion. At T1 mapping, panel on the right, evidence of reduced T1 values (898 ms, normal value 970 ± 70 ms, at the mid-septal level). Although reduced, T1 values were just slightly below the lower limit of normal, possibly due to pseudo-normalization due to fibrosis and chronic kidney disease.