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Morphological and Functional Assessment of the Right Ventricle Using 3D Echocardiography
Published on: October 28, 2020
Clinical profile of arrhythmogenic right ventricular cardiomyopathy in Chinese patients
1Division of Cardiology, Department of Medicine and Therapeutics, Prince of Wales Hospital, Chinese University of Hong Kong, Hong Kong, China.
Insights
Arrhythmogenic right ventricular cardiomyopathy (ARVC) in Chinese patients presents with palpitations and dizziness. Magnetic resonance imaging (MRI) is the preferred diagnostic tool, with low familial incidence of sudden death observed.
Area of Science:
- Cardiology
- Genetics
- Medical Imaging
Background:
- Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic heart muscle disease.
- It primarily affects the right ventricle, leading to arrhythmias and sudden cardiac death.
- Understanding the clinical profile in diverse populations is crucial for diagnosis and management.
Purpose of the Study:
- To delineate the clinical characteristics of Chinese patients diagnosed with arrhythmogenic right ventricular cardiomyopathy (ARVC).
- To evaluate the diagnostic utility of various imaging modalities, particularly MRI, in this cohort.
- To assess the incidence of familial sudden cardiac death and left ventricular involvement in Chinese ARVC patients.
Main Methods:
- Retrospective analysis of clinical data from Chinese patients meeting established ARVC diagnostic criteria.
- Inclusion of data from electrocardiography (ECG), echocardiography, cardiac catheterization, MRI, and electrophysiology studies (EPS).
- Assessment of presenting symptoms, family history, and therapeutic interventions.
Main Results:
- Eleven Chinese patients (7 male, mean age 42.6 years) were diagnosed with ARVC.
- Common symptoms included palpitations (73%) and dizziness (46%); 54% presented with ventricular tachycardia.
- MRI revealed RV abnormalities in 90%, while familial sudden death incidence was low (18.1%).
Conclusions:
- Familial incidence of premature sudden death and left ventricular involvement are uncommon in Chinese ARVC patients.
- Magnetic resonance imaging (MRI) is highly effective for diagnosing ARVC in this population.
- Palpitations and dizziness are frequent presenting symptoms, with ventricular tachycardia being a common arrhythmia.
Objective:
To study the clinical profile of Chinese patients with arrhythmogenic right ventricular cardiomyopathy (ARVC).
Patients:
Chinese patients who fulfilled the diagnostic criteria of ARVC proposed by the Task Force of the European Society of Cardiology and of the scientific council on cardiomyopathy of the International Society and Federation of Cardiology were recruited for analysis.
Methods:
Clinical data of patients with ARVC including age, sex, family history, presenting symptoms, electrocardiograph (ECG), echocardiography, cardiac catheterization, magnetic resonance imaging (MRI), electrophysiology study (EPS) and therapeutic intervention were analyzed.
Results:
Eleven patients (seven males) were diagnosed with ARVC. Mean age at clinical presentation was 42.6+/-14.8 years. Two patients (18.1%) had positive family history of ARVC or premature sudden cardiac death. The commonest presenting symptoms were palpitation (73%) and dizziness (46%). Spontaneous ventricular tachycardia (VT) was the presenting arrhythmia in 54% and 1 (9%) with ventricular fibrillation and cardiac arrest. Seven patients (64%) had the ECG abnormality as defined by the Task Force. Echocardiography showed right ventricular (RV) dilatation in five patients (46%) and all patients had normal left ventricular function. Nine patients (90%) had RV wall thinning or fibrofatty replacement on MRI examination. Inducible monomorphic VT was detected in four out of nine patients at EPS. All eight patients had normal coronary arteries and left ventriculogram but RV dilatation and global hypokinesia was seen in three patients. Implantable cardioverter defibrillators were implanted in five patients and two of them had shocks delivered during the follow-up period.
Conclusion:
In this study, familial incidence of premature sudden death in patients with ARVC appears to be low and left ventricular involvement in affected individuals is uncommon. MRI is still the best investigation for ARVC.
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