Kuhn A, Kottkamp H, Thiele H, Schuler G, Hindricks G
Herzzentrum, Klinik für Innere Medizin/Kardiologie, Universität Leipzig.
Dtsch Med Wochenschr. 2000 Jun 2;125(22):692-7. doi: 10.1055/s-2007-1024438.
While cycling a 38-year-old man suddenly experienced palpitations associated with marked weakness. 90 min later his general practitioner, having diagnosed a ventricular tachycardia (VT) with a rate of 218/min, terminated it by a drug injection.
Electrocardiography (ECG), echocardiography and biventricular cardiac catheterization with right ventricular contrast injection failed to provide any evidence of structural abnormality. However, ergometry and EPS with programmed ventricular stimulation induced VT of identical morphology (left bundle branch bloc [LBBB] with right axis deviation [RAD]).
Idiopathic right-ventricular outflow tract tachycardia (IRVT) having been diagnosed, the patient was put on a maintenance dose of 50 mg/d atenolol. After 6 months without symptoms he again experienced several attacks of tachycardia. Resting ECG merely revealed an epsilon potential and negative T waves in V1-V3. Right ventricular contrast injection revealed inferolateral dyskinesia. EPS demonstrated both the known VT and a second, morphologically different one (LBBB with LAD). These findings indicated arrhythmogenic right-ventricular cardiomyopathy (ARCV). A cardioverter/defibrillator was implanted (ICD) and over the subsequent 8 months he had six episodes of VT which were quickly terminated by the ICD.
At first presentation of right-ventricular outflow tract tachycardia it is often not possible to differentiate between IRVT and arrhythmogenic RV cardiomyopathy. The two being significantly different in prognosis and treatment, follow-up monitoring is essential to establish the definitive diagnosis.
一名38岁男性在骑自行车时突然感到心悸，并伴有明显乏力。90分钟后，他的全科医生诊断为室性心动过速（VT），心率为218次/分钟，通过药物注射终止了发作。
心电图（ECG）、超声心动图以及右心室造影的双心室心脏导管检查均未发现任何结构异常的证据。然而，运动试验和程序心室刺激的电生理检查（EPS）诱发了形态相同的室性心动过速（左束支传导阻滞[LBBB]伴右轴偏移[RAD]）。
诊断为特发性右心室流出道心动过速（IRVT）后，患者开始服用50毫克/天的阿替洛尔维持剂量。6个月无症状后，他再次经历了几次心动过速发作。静息心电图仅显示V1-V3导联有ε波和T波倒置。右心室造影显示下外侧运动障碍。电生理检查显示既有已知的室性心动过速，又有另一种形态不同的室性心动过速（左束支传导阻滞伴左轴偏移）。这些发现提示致心律失常性右心室心肌病（ARCV）。植入了心脏复律除颤器（ICD），在随后的8个月里，他发生了6次室性心动过速发作，均被ICD迅速终止。
初次出现右心室流出道心动过速时，往往无法区分特发性右心室流出道心动过速和致心律失常性右心室心肌病。两者在预后和治疗上有显著差异，随访监测对于明确诊断至关重要。
