P5498Poor clinical outcome despite cardiac resynchronization therapy in Emery Dreifuss muscular dystrophy patients
P5498Poor clinical outcome despite cardiac resynchronization therapy in Emery Dreifuss muscular dystrophy patients
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P5498尽管对埃默里德莱福斯肌营养不良症患者进行心脏再同步治疗，但临床结果不佳
DOI：
10.1093/eurheartj/ehx493.p5498
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发表时间：
2017
影响因子：
39.3
通讯作者：
F. Anselme
中科院分区：
文献类型：
--
作者：
G. Viart;J. Burdeau;A. Savouré;B. G. Gardea;F. Bauer;C. Bizet;C. Alarcon;H. Eltchaninoff;F. Anselme
方法：所有连续接受CRT治疗的EDMD患者，尽管药物治疗最佳，但仍因有症状的心力衰竭伴左室射血分数受损而接受CRT治疗。植入物在局部麻醉下进行，由心脏小组进行个体化评估。结果：2002年至2013年，11例EDMD患者接受CRT治疗(男性4例，占36%；平均年龄52±5岁)：10/11例(91%)由起搏器(2/10)或植入式除颤器(8/10)升级，1例患者无心脏起搏器。CRT升级前平均起搏百分比为95%。植入前平均NYHA为2.6。大多数患者右室功能不全伴严重的三尖瓣返流(75%)。11例患者中有10例(91%)使用CRT除颤器。1例(9%)因口袋血肿需要再次介入治疗。中位随访时间为21个月。平均双室起搏率为98%。在植入除颤器的患者中，8/10(80%)的患者有适当的室性心律失常治疗，2/10(20%)的患者有不适当的治疗。中位左心室射血分数(LVEF)在最后一次随访时为37.5%，而CRT前为37%。末次随访时左室舒张末期内径(LVEDD)中位数为61.5 mm，CRT前为60 mm。11例患者中有6例(55%)在11个月的中位随访期后接受了心脏移植。结论：尽管CRT效果最佳，心脏移植在EDMD患者中往往是非常必要的，因为这是一种进展性疾病，会导致严重的双心衰竭。右室功能不全和严重的三尖瓣返流可能是这些患者接受CRT治疗后预后较差的部分原因。
Methods: All consecutive EDMD patients receiving CRT devices for symptomatic heart failure with impaired left ventricular ejection fraction despite optimal medical therapy were enrolled in the study. Implantations were performed under local anaesthesia, after individual assessment by a heart-team. Demographic, clinical, echocardiographic and device-interrogation data were collected during the follow-up period.Results: From 2002 to 2013, 11 EDMD patients had CRT (4 males (36%); mean age 52±5 years): 10/11 (91%) were upgrading procedures from a pacemaker (2/10) or implantable defibrillator (8/10), one patient had no prior device. Average pacing percentage was 95% before CRT upgrade. Average NYHA was 2.6 before implantation. Most patients had right ventricular dysfunction with severe tricuspid regurgitation (75%). 10/11 patients (91%) received CRT-Defibrillators. One patient (9%) required re-intervention for pocket hematoma. Median follow-up was 21 months. Average biventricular pacing rate was 98%. Among patients implanted with defibrillators, 8/10 (80%) had appropriate therapies for ventricular arrhythmias, 2/10 (20%) had inappropriate therapies. Median left ventricular ejection fraction (LVEF) was 37.5% at last follow-up vs 37% before CRT. Median left ventricular end diastolic diameter (LVEDD) was 61.5 mm at last follow-up vs 60 mm before CRT. Despite CRT, 6/11 patients (55%) had heart transplantation after a 11 months' median follow-up period.Conclusion: Despite optimal CRT, heart transplantation is often rapidly necessary in EDMD, as this is a progressive disease leading to severe biventricular failure. Right ventricular dysfunction and severe tricuspid regurgitation may partly explain these patients' poor prognosis despite CRT.