Angiographically detectable Thebesian veins are a dynamic and reversible finding in the setting of congenital heart disease.

OBJECTIVE: 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.

METHODS: 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.

RESULTS: 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.

CONCLUSIONS: 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.