Tannous Paul, Ghelani Sunil J, Marshall Audrey C, Porras Diego
Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts, USA.
Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA.
Congenit Heart Dis. 2017 Jul;12(4):467-474. doi: 10.1111/chd.12457. Epub 2017 Mar 10.
Angiographically detectable Thebesian veins (ThVs) are a rare finding sometimes associated with coronary steal and myocardial ischemia in adults, but there are limited data regarding prominent ThVs in the setting of complex congenital heart disease (CHD). This study represents the largest series to date describing the presence and temporal changes of angiographically detectable ThVs in children with CHD.
This is a single center case series describing the clinical characteristics and coronary anatomy in children with CHD and angiographicall detectable ThVs. After identification of the index case, additional patients were identified in a prospective manner during the course of routine clinical care. We developed a qualitative scale to grade ThV burden, with changes tracked over time in the subset of patients who underwent serial cardiac catheterizations.
A total of 10 patients are included in this report. There was a predominance of female gender (8 of 10 patients), right-dominant single ventricle physiology (7 of 10 patients), and heterotaxy syndrome (6 of 10 patients). The degree and location of epicardial coronary artery tortuosity was qualitatively related to ThV burden. The subset of patients who underwent serial cardiac catheterizations demonstrate that ThV dilation can progress or regress over time.
Angiographically detectable Thebesian veins are a rare finding in patients with congenital heart disease and may represent global changes in the coronary circulation. This is the first report, adult or pediatric, to demonstrate that ThV dilation is a dynamic process capable of both progression and regression. The physiologic impact of these findings remains to be elucidated.
血管造影可检测到的Thebesian静脉（ThV）是一种罕见的发现，在成人中有时与冠状动脉窃血和心肌缺血有关，但关于复杂先天性心脏病（CHD）背景下明显的ThV的数据有限。本研究是迄今为止描述CHD患儿血管造影可检测到的ThV的存在及其时间变化的最大系列研究。
这是一个单中心病例系列，描述了患有CHD且血管造影可检测到ThV的儿童的临床特征和冠状动脉解剖结构。在确定索引病例后，在常规临床护理过程中以前瞻性方式确定了其他患者。我们制定了一个定性量表来对ThV负荷进行分级，并在接受系列心脏导管检查的患者子集中跟踪其随时间的变化。
本报告共纳入10例患者。女性占优势（10例患者中的8例），右优势型单心室生理状态（10例患者中的7例），以及内脏异位综合征（10例患者中的6例）。心外膜冠状动脉迂曲的程度和位置与ThV负荷在质量上相关。接受系列心脏导管检查的患者子集表明，ThV扩张可随时间进展或消退。
血管造影可检测到的Thebesian静脉在先天性心脏病患者中是一种罕见的发现，可能代表冠状动脉循环的整体变化。这是第一份，无论是成人还是儿科的报告，证明ThV扩张是一个能够进展和消退的动态过程。这些发现的生理影响仍有待阐明。
