Isolated left ventricular arrhythmogenic cardiomyopathy: A case report
Raimundo Barbosa-Barros1, Andrés Ricardo Pérez-Riera2, Luiz Carlos de Abreu2
1Coronary Center of the Hospital de Messejana Dr. Carlos Alberto Studart Gomes, Fortaleza, CE, Brazil.
Journal of Electrocardiology
|October 16, 2016
Summary
Arrhythmogenic cardiomyopathy (AC) can affect the left ventricle (LV) in advanced stages, often presenting with ECG changes like T-wave inversion. This case highlights isolated LV AC with specific electrocardiogram findings and ventricular tachycardia.
Area of Science:
- Cardiology
- Genetics
- Molecular Biology
Background:
- Arrhythmogenic cardiomyopathy (AC) is a desmosome-related disease.
- Intercalated disc dysfunction and altered signaling pathways (Wnt/β-catenin, Hippo) contribute to AC.
- Left ventricular (LV) involvement signifies advanced AC, typically marked by T-wave inversion in lateral leads on ECG.
Observation:
- A case of isolated LV AC was observed.
- The patient presented with fragmented QRS (fQRS) and T-wave inversion (TWI) in lateral leads.
- Ventricular tachycardia originated from the inferobasal LV wall.
Findings:
- The case demonstrates an evolution of desmosomopathy into isolated LV AC.
- Phenotypic variability in AC is linked to intercalated disc mutations and abnormal trafficking.
- Specific ECG markers (fQRS, TWI) and VT origin indicate LV involvement.
Implications:
- Understanding LV involvement in AC is crucial for diagnosis and management.
- Intercalated disc abnormalities and signaling pathways are key targets for future research.
- This case underscores the importance of ECG interpretation in identifying advanced AC and LV disease.
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