■Ebstein\的异常发生在三尖瓣存在根尖移位并带有间隔和后瓣小叶的束缚时。这种情况通常与其他先天性疾病有关,结构,或传导系统疾病,包括心内分流,瓣膜病变,心律失常,辅助传导通路,和一级房室传导阻滞。我们首次介绍了一例Ebstein异常患者,该患者患有二级MobitzII房室传导阻滞,并因其年龄小和长期高起搏百分比的可能性而成功接受传导系统起搏(CSP)治疗。
■我们介绍了一个42岁的女士患有复杂的先天性心脏病的病例,包括严重的肺动脉狭窄,Ebstein异常,和房间隔缺损(ASD)。她需要复杂的外科手术,包括三尖瓣(TV)修复和随后的置换,ASD闭合,和肺动脉瓣球囊成形术.她出现在我们医院,有症状的二级MobitzII房室传导阻滞(头晕,呼吸急促,和运动不耐受)和右束支传导阻滞(RBBB)在她的基线心电图上。她的超声心动图显示右心室(RV)和左心室(LV)扩张,左心室收缩功能正常较低。由于她的年龄很小,并且有可能长期进行高比例的RV起搏,在经过详细讨论和患者同意后,我们选择了CSP.HIS远端位置是我们中心首选的起搏策略。我们无法使用标准的MedtronicC315HIS导管穿过电视,所以我们不得不使用可偏转的C304导管.远端HIS束的定位和起搏通过MedtronicSelectsecure3830,69cm导线实现。HIS束起搏导致二级MobitzII房室传导阻滞和先前存在的RBBB的矫正。植入顺利,患者第二天出院,没有任何急性并发症。
■远端HIS起搏对于手术治疗的复杂Ebstein异常和心脏传导阻滞的患者是可行的。该方法可以以保持或改善LV功能的高概率来归一化QRS复合波。
UNASSIGNED: Ebstein\'s anomaly occurs when there is an apical displacement of the tricuspid valve with septal and posterior valve leaflets tethering. This condition often occurs in association with other congenital, structural, or conduction system diseases, including intracardiac shunts, valvular lesions, arrhythmias, accessory conduction pathways, and first-degree atrioventricular (AV) block. We present for the first time a case of a patient with Ebstein\'s anomaly who presented with second-degree Mobitz II AV block and was successfully treated with conduction system pacing (CSP) due to her young age and the likelihood of a long-term high percentage of pacing.
UNASSIGNED: We present a case of a 42-year-old lady with a background of complex congenital heart disease, including severe pulmonary stenosis, Ebstein anomaly, and atrial septal defect (ASD). She required complex surgical intervention, including tricuspid valve (TV) repair and subsequently replacement, ASD closure, and pulmonary balloon valvuloplasty. She presented to our hospital with symptomatic second-degree Mobitz II AV block (dizziness, shortness of breath, and exercise intolerance) and right bundle branch block (RBBB) on her baseline ECG. Her echocardiogram showed dilated right ventricle (RV) and left ventricle (LV) with low normal LV systolic function. Due to her young age and the likelihood of a long-term high percentage of RV pacing, we opted for CSP after a detailed discussion and patient consent. The distal HIS position is the preferred pacing strategy at our centre. We could not cross the TV with the standard Medtronic C315 HIS catheter, so we had to use the deflectable C304 HIS catheter. Mapping and pacing of the distal HIS bundle were achieved by Medtronic Selectsecure 3830, 69 cm lead. HIS bundle pacing led to the correction of both second-degree Mobitz II AV block and pre-existing RBBB. The implantation was uneventful, and the patient was discharged home the next day without any acute complications.
UNASSIGNED: Distal HIS pacing is feasible in patients with surgically treated complex Ebstein anomaly and heart block. This approach can normalize the QRS complex with a high probability of preserving or improving LV function.