Arrhythmogenic right ventricular cardiomyopathy without arrhythmias?

    Authors

    Keywords

    arrhythmogenic right ventricular cardiomyopathy, arrhythmias, sudden cardiac death

    DOI

    https://doi.org/10.15836/ccar2019.257

    Full Text

    Introduction : Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare cardiovascular disease that predisposes to ventricular arrhythmias potentially leading to sudden cardiac death (SCD). In 1994 and 2010, an International Task Force document proposed guidelines for the standardized diagnosis of ARVC based on electrocardiographic (ECG), arrhythmic, morphological, histopathologic, and clinical-genetic factors. ( 1 - 3 ) Our case report shows how important is to diagnose this disease, because the patients with ARVC should undergo lifelong clinical follow-up to periodically evaluate new onset or worsening of symptoms, progression of morphological and/or functional ventricular abnormalities, and ventricular arrhythmias in order to reassess the risk of SCD. Case report : 53-year-old man with history of arterial hypertension and hyperlipidemia was treated at cardiac dispensary because of bad control of blood pressure. He was asymptomatic and his family history for SCD was negative. ECG demonstrated normal sinus rhythm with complete right bundle branch block and transthoracic echocardiography (TTE) was recommended. The left ventricle had normal dimension with normal ejection fraction (EF), right ventricle was enlarged (PLAX 34mm) with bulging of apical segment RV and thickness of trabecular muscles ( Figure 1 ). Cardiac MRI presented imaging criteria for ARVC with reduced EF RV 35%. More detailed analysis of ECG showed epsilon wave in lead V2 ( Figure 2 ). The 72-hours Holter electrocardiogram monitoring did not show any cardiac arrhythmias, same as during the exercise test. The patient was referred to University Clinic to make additional cardiological examinations. The ventricular late potential test was positive. Programmed stimulation of the left ventricle was made - without causing arrhythmia. The diagnosis of ARVC was confirmed – one major criteria on TTE and on cardiac MRI with present epsilon wave on ECG. The risk stratification was made, the patient had low risk and the therapy with beta blocker was prescribed with limitation of physical activity and regular follow-up. Genetic testing is planned. Enlargement of the right ventricle with bulging of the apical segment. Epsilon wave visible in lead V2. Conclusion : ARVC without arrhythmias is rare and easily misdiagnosed. The presentation can be non-specific, as was in our case, making the diagnosis of this condition challenging.

    Cardiologia Croatica
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    Arrhythmogenic right ventricular cardiomyopathy without arrhythmias?

    Extended Abstract
    Issue9-10
    Published
    Pages257-258
    PDF via DOIhttps://doi.org/10.15836/ccar2019.257
    arrhythmogenic right ventricular cardiomyopathy
    arrhythmias
    sudden cardiac death

    Authors

    Vlasta Soukup Podravec*ORCIDCroatia
    Ivana Petrović JurenORCIDCroatia
    Sandra PršaORCIDCroatia
    Andreja Čleković-KovačićORCIDCroatia
    Kristina Milevoj KrižićORCIDCroatia
    Renata Ivanac JankovićORCIDCroatia

    Full Text

    Introduction : Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare cardiovascular disease that predisposes to ventricular arrhythmias potentially leading to sudden cardiac death (SCD). In 1994 and 2010, an International Task Force document proposed guidelines for the standardized diagnosis of ARVC based on electrocardiographic (ECG), arrhythmic, morphological, histopathologic, and clinical-genetic factors. ( 1 - 3 ) Our case report shows how important is to diagnose this disease, because the patients with ARVC should undergo lifelong clinical follow-up to periodically evaluate new onset or worsening of symptoms, progression of morphological and/or functional ventricular abnormalities, and ventricular arrhythmias in order to reassess the risk of SCD. Case report : 53-year-old man with history of arterial hypertension and hyperlipidemia was treated at cardiac dispensary because of bad control of blood pressure. He was asymptomatic and his family history for SCD was negative. ECG demonstrated normal sinus rhythm with complete right bundle branch block and transthoracic echocardiography (TTE) was recommended. The left ventricle had normal dimension with normal ejection fraction (EF), right ventricle was enlarged (PLAX 34mm) with bulging of apical segment RV and thickness of trabecular muscles ( Figure 1 ). Cardiac MRI presented imaging criteria for ARVC with reduced EF RV 35%. More detailed analysis of ECG showed epsilon wave in lead V2 ( Figure 2 ). The 72-hours Holter electrocardiogram monitoring did not show any cardiac arrhythmias, same as during the exercise test. The patient was referred to University Clinic to make additional cardiological examinations. The ventricular late potential test was positive. Programmed stimulation of the left ventricle was made - without causing arrhythmia. The diagnosis of ARVC was confirmed – one major criteria on TTE and on cardiac MRI with present epsilon wave on ECG. The risk stratification was made, the patient had low risk and the therapy with beta blocker was prescribed with limitation of physical activity and regular follow-up. Genetic testing is planned. Enlargement of the right ventricle with bulging of the apical segment. Epsilon wave visible in lead V2. Conclusion : ARVC without arrhythmias is rare and easily misdiagnosed. The presentation can be non-specific, as was in our case, making the diagnosis of this condition challenging.