Unusual Cause of Bidirectional Ventricular Rhythm

Area of Science:

• Cardiology
• Genetics
• Pathology

Background:

• Bidirectional ventricular tachycardia (BDVT) is a rare ventricular arrhythmia.
• Commonly associated with digitalis toxicity or channelopathies, rarely with other conditions like aconite toxicity, myocarditis, infarction, or sarcoidosis.

Observation:

• A patient presented with BDVT, recurrent syncope, and histological findings of myocardial disarray and interstitial fibrosis.
• Echocardiography results were normal, ruling out significant structural heart abnormalities visible on ultrasound.

Findings:

• Genetic analysis revealed variants in the TTN, KCNH2, and GATA4 genes.
• These genetic variants are implicated as potential contributors to the observed BDVT and associated cardiac pathology.

Implications:

• This case expands the known genetic underpinnings of BDVT.
• Highlights the importance of genetic testing in unexplained arrhythmias with specific histological findings.
• Suggests a potential link between TTN, KCNH2, and GATA4 variants and the development of BDVT and myocardial changes.