Hereditary transthyretin amyloidosis (ATTRv) is a progressive, life-threatening disease caused by mutations in the TTR gene. It is characterized by marked clinical heterogeneity, and diagnosis is often delayed. Here, we report the tortuous diagnostic process in a patient with late-onset ATTRv carrying a c.349G > T (p.Ala117Ser) mutation in the TTR gene. A 59-year-old man initially presented with distal numbness and weakness in both lower limbs, followed by hoarseness and cough. Over two years, he consulted multiple specialists for these predominant symptoms, but no definitive diagnosis was made. Electrocardiography revealed low limb lead voltages. Echocardiography showed increased echogenicity in the left ventricular posterolateral wall with wall thickness at the upper normal limit. With a strong clinical suspicion of transthyretin amyloidosis, 99mTc-pyrophosphate (PYP) scintigraphy demonstrated cardiac amyloid deposition, and the diagnosis was ultimately confirmed by genetic testing, which identified a heterozygous Ala117Ser mutation in the TTR gene. This case illustrates that in middle-aged and older patients with progressive peripheral neuropathy of unclear cause, atypical symptoms such as hoarseness and cough may serve as important clues to ATTRv. Prompt integration of cardiac imaging and genetic screening can help avoid diagnostic delay.

Despite being rare within paediatric patients, emerging evidence shows that low QRS voltage may be a risk factor for increased mortality even in formerly healthy patients. The prognostic value of low-voltage electrocardiography in a paediatric population has yet to be defined. Here, we present a case of an adolescent experiencing sudden cardiac arrest after Parvo B19-associated myocarditis. A striking finding was a marked low-voltage electrocardiography throughout the following years.

Systemic amyloidosis, which may involve the heart as an infiltrative cardiomyopathy, is frequently underdiagnosed, particularly in low-resource settings where access to advanced imaging and laboratory investigations for amyloid subtyping is limited. We report a case of a 57-year-old man from Ethiopia who presented with progressive symptoms of heart failure with preserved ejection fraction (HFpEF). Electrocardiography demonstrated low-voltage QRS complexes with poor R-wave progression, while transthoracic echocardiography revealed concentric left ventricular hypertrophy with severe diastolic dysfunction. The marked discordance between the electrocardiographic and echocardiographic findings raised suspicion of infiltrative cardiomyopathy, specifically systemic amyloidosis with probable cardiac involvement. Advanced diagnostic modalities, including serum protein electrophoresis and free light-chain assay, were unavailable because of financial constraints; however, an abdominal fat pad biopsy with Congo red staining confirmed amyloid deposition. Critically, amyloid subtyping could not be performed. The patient was managed with optimized diuretic therapy, resulting in symptomatic improvement. This case highlights the diagnostic approach integrating basic clinical findings, electrocardiography, echocardiography, and minimally invasive biopsy to confirm systemic amyloidosis with probable cardiac involvement in resource-limited settings. Amyloid subtyping remains essential for disease-specific therapy. Early recognition and careful diagnostic interpretation are critical for guiding appropriate management and improving outcomes.

The traditional electrocardiographic diagnosis of left ventricular hypertrophy (LVH) rests on the Voltage Paradigm, which assumes a linear relationship between QRS amplitude and left ventricular mass. High-resolution cardiac imaging has exposed fundamental anomalies in this framework: most patients with anatomic LVH have normal QRS amplitudes, while many with high amplitudes have normal mass. These discrepancies arise from non-spatial determinants-conduction velocity, fibrosis, cell-to-cell coupling and thoracic conductivity-that the linear model cannot accommodate. We propose replacing the term 'ECG-LVH' with Left Ventricular Electrical Remodeling (LVER), a framework endorsed by the International Society of Electrocardiology and the International Society for Holter and Noninvasive Electrocardiology encompassing four phenotypes: high QRS amplitude, conduction delay, low QRS amplitudes and QRS fragmentation. Each phenotype reflects a specific electrophysiological substrate carrying prognostic information independent of mass. LVER reframes the clinical task as electrical risk phenotyping.

A 70-year-old man with a history of prior ablation for atrial fibrillation presented with atrial tachycardia (AT). Electrophysiological studies revealed roof-dependent and localized-reentrant ATs. During each AT, widespread breakthrough activation was observed just anterior to the low-voltage area on the anterior wall, suggesting that Bachmann's bundle served as an epicardial bypass within the tachycardia circuits. This case highlights the potential role of Bachmann's bundle in the development of ATs in the remodeled left atrium.

BackgroundAmyloid deposition is a key pathologic hallmark of Alzheimer's disease (AD). Since cardiac amyloidosis also involves abnormal amyloid accumulation, shared proteinopathy mechanisms may underlie both conditions.ObjectiveWe investigated whether brain amyloidosis on PET imaging is associated with electrocardiogram (EKG) findings of cardiac involvement by AD pathology.MethodsWe included 191 participants (mean age 66 ± 13.0 years) in our analysis, who underwent amyloid PET imaging and EKG within one year. EKG abnormalities (including low QRS voltage, poor R-wave progression (PRWP), bundle branch block, and sinus bradycardia) were assessed for associations with brain amyloid deposition, quantified in Centiloids, and cognitive function. A subset (n = 164) also underwent [18]F-MK6240 tau PET, and the relationship between these EKG abnormalities and tau deposition was also assessed.ResultsPRWP was associated with greater brain amyloid deposition (coefficient = 45 ± 12.15, p < 0.001) and worse cognition on the Clinical Dementia Rating scale (coefficient = 0.22 ± 0.098, p = 0.027). There was a trend for higher likelihood of cortical tau deposition with PRWP (odds ratio = 3.66, p = 0.05). Sinus bradycardia was associated with higher likelihood of cortical tau deposition (odds ratio = 2.81, p = 0.009). Other EKG abnormalities were not significantly associated with brain amyloid/tau deposition or cognition.ConclusionsPRWP and sinus bradycardia were found to be associated with AD pathology in the brain. These findings warrant further investigation into how cardiac electrophysiologic abnormalities may reflect a possible link between brain and cardiac pathologies or AD-associated cardiac dysfunction.