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From Palpitations to Prevention: Timely Recognition of Biventricular ACM Preventing Sudden Cardiac Death
Jasraj Singh1, Fadi W Adel1, Horng H Chen1
1Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA, mayo.edu.
Insights
Arrhythmogenic cardiomyopathy (ACM) can cause sudden cardiac death (SCD) in young adults. Early recognition of biventricular ACM, even with subtle signs, is crucial for timely intervention and preventing fatal outcomes.
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Sudden cardiac death (SCD) is a significant concern, particularly in young individuals.
- Arrhythmogenic cardiomyopathy (ACM) is a rare but critical cause of SCD, characterized by high morbidity and mortality.
- Biventricular (BiV) ACM involves both ventricles and presents unique diagnostic challenges.
Purpose of the Study:
- To present a classic case of BiV ACM in a young male patient.
- To highlight the diagnostic criteria for BiV ACM according to the 2024 European Task Force Guidelines.
- To emphasize the importance of early recognition and intervention in managing BiV ACM.
Main Methods:
- Case report of a man in his 30s presenting with palpitations and syncope.
- Diagnostic confirmation using the 2024 European Task Force Criteria for ACM.
- Cardiac imaging including Cardiac Magnetic Resonance (CMR) and transthoracic echocardiography.
- Electrocardiogram (ECG) analysis for T wave inversions and bundle branch blocks.
Main Results:
- The patient met major criteria for BiV ACM: T wave inversions (V1-V3), RV systolic dysfunction (RVEF 34%), and RV enlargement (indexed RV EDV 131 mL/m²).
- Minor criteria included reduced LV global longitudinal strain (-17%) and T wave inversions in left precordial leads (V4-V6).
- Nonsustained ventricular tachycardia was detected, indicative of arrhythmogenic substrate.
Conclusions:
- This case underscores the importance of recognizing subtle signs of BiV ACM.
- Timely diagnosis and intervention, including ICD placement and antiarrhythmic medication, led to a favorable outcome.
- Effective management of BiV ACM can prevent sudden cardiac death and improve patient prognosis.
Abstract:
Sudden cardiac death (SCD) is a prevalent and significant health concern which may be preceded by palpitations and presyncope in a young patient. Of the arrhythmogenic causes of SCD, arrhythmogenic cardiomyopathy (ACM) is rare but important with a high morbidity and mortality. Here, we present a classic case of biventricular (BiV) ACM in a man in his 30s who presented with progressive palpitations and syncope found to have nonsustained ventricular tachycardia and suspicious cardiac imaging findings. Diagnosis was confirmed according to the 2024 European Task Force Criteria: T wave inversions in V1-V3 without a right bundle branch block (major criterion); regional right ventricular (RV) systolic dyskinesis with both reduced RV systolic function by CMR (RVEF 34% and normal 42%-66%); and enlarged RV by indexed RV EDV by CMR (131 mL/m2) (major criterion). LV global longitudinal strain was reduced on transthoracic echocardiography at -17% (normal more negative than -18%) (minor criterion) and T wave inversions in left precordial leads (V4-V6) (in the absence of complete LBBB) (minor criterion). With timely intervention, including ICD placement and sotalol initiation, the patient made a good recovery. This case serves as a critical reminder that recognizing the subtle yet telling signs of BiV-ACM can mean the difference between life and sudden cardiac death.
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