遗传性心律失常新指南发布
2013-05-16 10:51:47   来源： 丁香园   作者：  评论：0 点击：

New Guidance for Inherited Arrhythmias
遗传性心律失常新指南发布

DENVER -- A redefinition of Brugada syndrome diagnosis based on ECG alone, and a new look at short QT syndrome and repolarization are featured in new consensus guidelines on inherited arrhythmias.
DENVER指的是在重新达成共识的指南上关于遗传性心律失常方面仅根据心电图诊断的布鲁加达综合征和重新看待QT期缩短综合征和特征性复极化的重新定义。

The recommendations also took broader aim, calling for evaluation of all patients with such conditions and their first-degree relatives in a dedicated clinic with appropriately trained staff.
这些建议同样有更广的目标，需要有经过适当训练的工作人员的专门诊所和患有这种病的病人以及他们的一级亲属去评价。

These kind of referral centers are more common in Europe but "in the States there's been a delay implementing them," noted writing group co-chair Silvia Priori, MD, PhD, of the University of Pavia, Italy. She spoke at a press briefing here at the Heart Rhythm Society (HRS) meeting where the document was released.
意大利的帕维亚大学博士兼写作组联主席Silvia Priori指出：“这种转诊中心在欧洲更常见，但在美国给他们有一种推迟的现象”。silvia priori 主席在心律协会发布文件的新闻简报会上说道。

"Ideally every electrophysiology center should have at least one person that has an expertise," she told reporters. "We hope that this document will really activate the process of organization of inherited arrhythmia clinics."
silvia priori 主席对记者说道：“比较理想的是在每一个电生理中心至少有一位电生理专家。我们希望这份文件能够促进遗传性心律失常诊所的组织过程。”


The HRS was joined by the Asia Pacific Heart Rhythm Society and the European Society of Cardiology's heart rhythm branch, the European Heart Rhythm Association, in putting out the guidelines.
这个心律协会是由亚太心律协会和欧洲心脏协会的分支心律协会组成的，欧洲心律协会正提出心律失常新指南的建议。

The American College of Cardiology, American Heart Association, and other organizations collaborated, but have not yet endorsed the recommendations.
美国心脏学院，美国心脏协会和其他的一些组织合作，但是还没有签署这项建议。

The guidelines covered several inherited arrhythmias that haven't made it into prior consensus documents, including early repolarization, short QT syndrome, and progressive cardiac conduction disease, another writing group co-chair Arthur Wilde, MD, PhD, of the University of Amsterdam, noted at the briefing.
另外一位阿姆斯特丹大学的博士兼写作组联主席Arthur Wilde在新闻简报会上指出：这些关于一些遗传性心率疾病的指南还没有写入之前已经达到共识的文件的当中，包括提前复极化，QT期缩短综合征和渐进式心脏传导的疾病。

The more common Brugada and long QT syndromes, though, are where the guidelines may make their biggest impact, he suggested.
Arthur Wilde博士认为尽管这些指南可能会对在布鲁加达综合征和QT期增长综合征越常见的地方有很大的影响。

"Brugada syndrome used to be defined as a particular ECG abnormality in combination with clinical issues," he said. "We changed that to make it a pure electrocardiographic diagnosis."
Arthur Wilde主席说：“布鲁加达综合征一般定义为与临床症状结合起来的一种特殊的心电图反常性，我们把它改为纯粹心电图诊断的一些症状。”

These guidelines, the first since 2006, seemed like a good opportunity to eliminate some of the confusion caused by requiring a clinical event for diagnosis in Brugada but not long QT syndrome, Wilde explained.
Wilde博士解释说：自从2006年，这些指南第一次看起来是个去除一些疑惑的好机会。这些疑惑是在需要一些临床事件去辅助诊断的布鲁加达而不是QT期增长综合征。

For long QT syndrome, the big change was with regard to sports participation.
对于患有QT期增长综合征的病人来说，对生活改变较大的是参加一些体育运动。

Swimming and diving seems to be a trigger in type 1 disease and should be avoided for those patients, Wilde pointed out, but the other two main types have a different clinical pattern.

Wilde博士指出游泳和潜水看起来是1型疾病的激发剂，这些病人应该避免这类运动，但是其它两大类型的疾病有不同的临床表现类型。

"Sport activity in general is something we struggle with a lot," he told MedPage Today. "Up until now, everybody always discouraged everybody with long QT to do competitive sport.

Wilde博士告诉MedPage Today：“总的来说体育运动与疾病的关系是我们一直努力研究的，直到现在，我们并不鼓励患有QT期增长综合征的病人去做一些有挑战性的运动。”


The data that pop up now do not really support it. There have not been any events at all in patients with long QT syndrome that continue to be in sport participation."
根据目前展现的数据并不支持这一观点。并不是所有的事故都发生在继续参加体育运动QT期增长综合征病人的身上。

The guidelines suggest that nontype 1 patients considered at low risk due to borderline QT prolongation, no history of cardiac symptoms, and no family history of multiple sudden cardiac deaths should be allowed to participate after a full evaluation and with proper precautions taken.
这些指南建议因为QT期边界的增长，无心脏病症状史，无多个突发性心脏病死亡的家庭史而降低死亡风险的非1型疾病患者在完整评估和采取适当预防措施之后允许参加体育运动。

But that evaluation should be done by a clinical expert in inherited arrhythmias, not just any pre-participation sports physician, and this received a class I recommendation.
但是这些评估应该是在遗传性心律失常的临床专家下完成的，并且评估获得I 类建议，而不是一些先前参加体育运动的医师来评估。
European guidelines and the Bethesda Conference recommendations leave open essentially no possibility of sports for long QT syndrome patients, so these guidelines are a real departure, Silva noted.
Silva博士指出：欧洲指南和贝塞斯达会议建议为QT期增长综合征患者开放一些基本上没有可能的运动,这些指南才是真正的开始。

"We hope with this document to slowly, carefully open patients with this condition to do safe sport," she said. "We wanted to lift the ban [so] that a physician, feeling that a specific patient would have a low risk in the proper environment, could [clear a patient for participation] without being against every single document written."
Silva博士说：“我们希望这份文件能够渐渐地，小心地为在这种情况下的患者开放一些安全的运动，我们想解除这项禁令这样不会当医生感觉到某个特殊的病人在某种坏境下有低风险，阻止这位病人去参加运动而违反每一个书面文件。”

The group acknowledged that their recommendations were all based on expert opinion as the only available evidence is from registries without any randomized or blinded studies.
这些组织承认他们的建议都是根据专家的意见作为唯一可信的证据，这些证据来源于事实的登记，而不是一些随机的盲目的研究。

The guidelines are slated for publication in HeartRhythm, EP Europace, and the Journal of Arrhythmias this fall.

今年秋天这些指南将会在心率期刊，EP Europace和心率失常期刊上发表。