| A 56-year-old male
presented with dyspnea
on mild exertion
(New York Heart
Association-Class III),
which had remained
stable over previous
few years. His medical
history was otherwise
unremarkable. On
admission, physical
examination revealed
a holosystolic III/VI
murmur over the apex
and an audible third
heart sound on auscultation. The patient also had bibasilar
fine crackles and pedal edema. Echocardiography
showed biatrial enlargement, severe mitral regurgitation
with an eccentric jet, and severe pulmonary
artery hypertension with an estimated left ventricular
ejection fraction (LVEF) of 50% (Video 1*). Additionally,
a continuous flow (arrows pointing to the fistulous
dilation) was noted in the coronary sinus indicating a
pattern compatible with an arteriovenous (AV) fistula
in the short-axis view of the left ventricle (Figure A,
Video 2 and 3*). The mitral valve annulus seemed to
be intact despite the tortuous course of AV malformation.
Three-dimensional, volume rendered reconstruction
of CT angiographic images were obtained, and
showed the course of the left circumflex artery connecting
to the coronary sinus over the course of aneurysmal
dilatation of the left circumflex artery and tortuous
clusters of AV malformation (Figure B and C).
Coronary AV malformation was further confirmed by
conventional coronary angiography (Figure D, Video
4*). No underlying coronary artery disease was found
in coronary angiographic studies. The patient underwent
surgical ligation of the fistula and Alfieri edgeto-
edge mitral valve repair, following which there was |