Heterotaxy
Heterotaxy
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异向性
DOI：
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发表时间：
2011
影响因子：
0.9
通讯作者：
J. Quintessenza
中科院分区：
文献类型：
--
作者：
J. Jacobs;S. Pasquali;D. Morales;M. Jacobs;C. Mavroudis;P. Chai;C. Tchervenkov;F. Lacour;Hal Walters;J. Quintessenza
根据国际儿科和先天性心脏病命名学会 (ISNPCHD) 的说法，“异位性与‘内脏异位’和‘异位综合征’同义。异位被定义为一种异常，即胸腹内器官在身体左右轴上表现出异常排列。按照惯例，异位不包括内脏器官沿预期正常或正常排列的患者。左右轴，也称为“孤立位置”，或内脏器官沿左右轴完全镜像排列的患者，也称为“内位逆位”。或内脏器官沿左右轴完全镜像排列的患者，也称为内脏倒置。本文的目的是回顾胸外科医生协会 (STS) 先天性心脏手术数据库中有关异位的数据。研究人员检查了STS先天性心脏病手术数据库中1998年1月1日至2009年12月31日（含）12年间的所有索引手术。该分析得出了总共 77 153 次索引操作的队列。其中，1505 例手术（1.95%）是针对异位患者进行的。在对异位患者进行的 1505 例索引手术中，1144 例为无脾患者，361 例为多脾患者。在每个 STS -EACTS 先天性心脏手术死亡率类别中，与未异位治疗的患者相比，异位治疗患者的出院死亡率较高（EACTS = 欧洲心胸外科协会）。除异位性单心室患者外，全身肺动脉分流术后的出院死亡率为 6.6%，而异位性单心室患者的出院死亡率为 10.8%。在除异位性单心室患者之外的所有单心室患者队列中，Fontan 后的出院死亡率为 1.8%，而异位性单心室患者的出院死亡率为 4.2%。 STS 先天性心脏病手术数据库是北美最大的先天性心脏病手术数据库。对 STS 先天性心脏手术数据库数据的审查可以对实践模式和结果进行独特的记录。从该分析中可以清楚地看出，异位性是一个具有挑战性的问题，大多数亚组的出院死亡率增加。
According to The International Society for Nomenclature of Pediatric and Congenital Heart Disease (ISNPCHD), “Heterotaxy is synonymous with ‘visceral heterotaxy’ and ‘heterotaxy syndrome’. Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. By convention, heterotaxy does not include patients with either the expected usual or normal arrangement of the internal organs along the left-right axis, also known as ‘situs solitus’, or patients with complete mirror-imaged arrangement of the internal organs along the left-right axis also known as `situs inversus’.” or patients with complete mirror-image arrangement of the internal organs along the left–right axis, also known as situs inversus. The purpose of this article is to review the data about heterotaxy in the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database. The investigators examined all index operations in the STS Congenital Heart Surgery Database over 12 years from January 1, 1998 to December 31, 2009, inclusive. This analysis resulted in a cohort of 77 153 total index operations. Of these, 1505 operations (1.95%) were performed in patients with heterotaxy. Of the 1505 index operations performed in patients with heterotaxy, 1144 were in patients with asplenia and 361 were in patients with polysplenia. In every STS -EACTS Congenital Heart Surgery Mortality Category, discharge mortality is higher in patients with heterotaxy compared with patients without heterotaxy (EACTS = European Association for Cardio-Thoracic Surgery). Discharge mortality after systemic to pulmonary artery shunt is 6.6% in a cohort of all single-ventricle patients except those with heterotaxy, whereas it is 10.8% in single-ventricle patients with heterotaxy. Discharge mortality after Fontan is 1.8% in a cohort of all single-ventricle patients except those with heterotaxy, whereas it is 4.2% in single-ventricle patients with heterotaxy. The STS Congenital Heart Surgery Database is largest congenital heart surgery database in North America. This review of data from the STS Congenital Heart Surgery Database allows for unique documentation of practice patterns and outcomes. From this analysis, it is clear that heterotaxy is a challenging problem with increased discharge mortality in most subgroups.