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Abstract：
BACKGROUND: Arrhythmogenic right ventricular (RV) cardiomyopathy is a rare and currently underrecognized cardiomyopathy characterized by the replacement of RV myocardium by fibrofatty tissue. It may be asymptomatic or symptomatic (palpitations or syncope) and may induce sudden cardiac death, especially during exercise. To prevent adverse events such as sudden cardiac death and heart failure, early diagnosis and treatment of arrhythmogenic RV cardiomyopathy (ARVC) are crucial. We report a patient with ARVC characterized by recurrent syncope during exercise who was successfully treated with combined endocardial and epicardial catheter ablation.
METHODS: A 43-year-old man was referred for an episode of syncope during exercise. Previously, the patient experienced two episodes of syncope without a firm etiological diagnosis. An electrocardiogram obtained at admission indicated ventricular tachycardia originating from the inferior wall of the right ventricle. The ventricular tachycardia was terminated with intravenous propafenone. A repeat electrocardiogram showed a regular sinus rhythm with negative T waves and a delayed S-wave upstroke from leads V1 to V4. Cardiac magnetic resonance imaging showed RV free wall thinning, regional RV akinesia, RV dilatation and fibrofatty infiltration (RV ejection fraction of 38%). An electrophysiological study showed multiple inducible ventricular tachycardia as of a focal mechanism from the right ventricle. Endocardial and epicardial voltage mapping demonstrated scar tissue in the anterior wall, free wall and posterior wall of the right ventricle. Late potentials were also recorded. The patient was diagnosed with ARVC and treated with combined endocardial and epicardial catheter ablation with a very satisfactory follow-up result.
CONCLUSIONS: Clinicians should be aware of ARVC, and further workup, including imaging with multiple modalities, should be pursued. The combination of epicardial and endocardial catheter ablation can lead to a good outcome.
METHODS: A 43-year-old man was referred for an episode of syncope during exercise. Previously, the patient experienced two episodes of syncope without a firm etiological diagnosis. An electrocardiogram obtained at admission indicated ventricular tachycardia originating from the inferior wall of the right ventricle. The ventricular tachycardia was terminated with intravenous propafenone. A repeat electrocardiogram showed a regular sinus rhythm with negative T waves and a delayed S-wave upstroke from leads V1 to V4. Cardiac magnetic resonance imaging showed RV free wall thinning, regional RV akinesia, RV dilatation and fibrofatty infiltration (RV ejection fraction of 38%). An electrophysiological study showed multiple inducible ventricular tachycardia as of a focal mechanism from the right ventricle. Endocardial and epicardial voltage mapping demonstrated scar tissue in the anterior wall, free wall and posterior wall of the right ventricle. Late potentials were also recorded. The patient was diagnosed with ARVC and treated with combined endocardial and epicardial catheter ablation with a very satisfactory follow-up result.
CONCLUSIONS: Clinicians should be aware of ARVC, and further workup, including imaging with multiple modalities, should be pursued. The combination of epicardial and endocardial catheter ablation can lead to a good outcome.
摘要：
背景：致心律失常性右心室（RV）心肌病是一种罕见且目前未被认可的心肌病，其特征是纤维脂肪组织替代RV心肌。它可能是无症状或有症状（心悸或晕厥），并可能导致心源性猝死。特别是在锻炼的时候。为了预防心脏猝死和心力衰竭等不良事件，心律失常性RV心肌病(ARVC)的早期诊断和治疗至关重要。我们报告了一名ARVC患者，其特征是在运动期间复发性晕厥，该患者已成功采用心内膜和心外膜导管消融联合治疗。
方法：一名43岁男子在运动期间因晕厥发作而被转诊。以前,患者经历了两次晕厥发作，但没有明确的病因诊断。入院时获得的心电图表明室性心动过速源于右心室下壁。通过静脉注射普罗帕酮终止室性心动过速。重复的心电图显示有规律的窦性心律，具有负T波和从V1到V4的延迟S波上行。心脏磁共振成像显示RV游离壁变薄，区域RV运动不能，RV扩张和纤维脂肪浸润（RV射血分数为38％）。电生理研究显示，作为右心室的局灶性机制，多次诱导性室性心动过速。心内膜和心外膜电压标测显示前壁有瘢痕组织，右心室的游离壁和后壁。还记录了晚期电位。患者被诊断为ARVC，并接受心内膜和心外膜导管联合消融治疗，随访结果非常满意。
结论：临床医生应该注意ARVC，和进一步的工作，包括多种模式的成像，应该追求。心外膜和心内膜导管消融的组合可以导致良好的结果。
方法：一名43岁男子在运动期间因晕厥发作而被转诊。以前,患者经历了两次晕厥发作，但没有明确的病因诊断。入院时获得的心电图表明室性心动过速源于右心室下壁。通过静脉注射普罗帕酮终止室性心动过速。重复的心电图显示有规律的窦性心律，具有负T波和从V1到V4的延迟S波上行。心脏磁共振成像显示RV游离壁变薄，区域RV运动不能，RV扩张和纤维脂肪浸润（RV射血分数为38％）。电生理研究显示，作为右心室的局灶性机制，多次诱导性室性心动过速。心内膜和心外膜电压标测显示前壁有瘢痕组织，右心室的游离壁和后壁。还记录了晚期电位。患者被诊断为ARVC，并接受心内膜和心外膜导管联合消融治疗，随访结果非常满意。
结论：临床医生应该注意ARVC，和进一步的工作，包括多种模式的成像，应该追求。心外膜和心内膜导管消融的组合可以导致良好的结果。
