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Heart failure with preserved ejection fraction: blame the veins…
Claudia Jorge1*, Nick Hiltrop1, andWalter Desmet1,2
1Department of Cardiovascular Medicine, Leuven University Hospital, Herestraat 49, 3000 Leuven, Belgium; and 2Department of Cardiovascular Sciences, KU Leuven, Leuven University Hospital, Herestraat 49, 3000 Leuven, Belgium
*Corresponding author. Tel: 00351913251313, Email: firstname.lastname@example.org
A 59-year-old female with heart failure with preserved ejection fraction (HFpEF), NYHA class III, with eccentric left ventricle (LV) hypertrophy and anterior inverted T waves on the ECG (Panel A), underwent a coronary angiography that excluded coronary artery disease. However, an unusual venous circulation pattern was incidentally observed: persistent embryonic coronary arterial fistulas (CAF) from the left and right coronaries to both ventricular cavities (see Supplementary material online, Videos S2 and S4). This corresponds to an unusually severe form of Thebesian veins. This exuberant venous drainage displayed a dense intramyocardial sinusoidal network, extensive enough to produce LV endocardial blush (Panels B–C, arrowheads) and a late ventriculography pattern (Panel D), by only coronary contrast injection. No contrast was seen in venous time frames over the coronary sinus (Panel D.1), that on coronary CT scan appeared hypoplasic as well as the cardiac veins. Remarkably, the CAF were so highly developed that they could be visualized throughout the LV myocardium in the CT scan (Panel E, arrowheads). Furthermore, tortuous coronaries, fistulae-flow-mediated, with long intramyocardial coronary paths (Panel F) were also displayed. Ischemic heart failure by coronary steal phenomenon was excluded by dobutamine-stress cardiac magnetic resonance imaging.
Exuberant Thebesian veins having origin on both coronary system and draining to both ventricles, is an extremelly rare entity. Although the majority of patients are asymptomatic, dyspnea and fatigability, can be the presenting symptoms. Coronary arterial fistulas have repeatedly been reported as a cause of abnormalities in myocardial microcirculation, inducing reactive myocardial hypertrophy, leading to diastolic dysfunction and HFpEF, as in this patient. Other causes of LV hypertrophy were excluded.
Figure 1 ECG: sinus rythm with anterior inverted T waves (A). Coronary angiography: Multiple coronary arteries fistulas (arrowheads) supplied by the right and the left coronary arteries, defining the left ventricle endocardial border (Panels B–C). Ventriculography by coronary contrast injection (D), without visualization of the coronary sinus in a venous time frame (D.1). Coronary CT scan: Multiple intramyocardial coronary arteries fistulas draining directly to the left ventricle cavity (E) and a long tortuous intramyocardial diagonal path (F).
Supplementary material is available at European Heart Journal online.
See figure legend on page 376.
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