We describe a patient in whom this anomaly was diagnosed during cardiac
catheterization for evaluation of progressive shortness of breath and
mild to moderate mitral regurgitation founded on a transthoracic
echocardiogram.A 58-year-old hypertensive female was evaluated for
progressive shortness of breath. Her physical examination showed a
regular heart rate and rhythm, with normal heart sounds with a
holosystolic systolic murmur on the 4th intercostal midclavicular line
on auscultation. Electrocardiography showed normal sinus rhythm and
criteria and possible left ventricular hypertrophy. Stress
Echocardiogram showed with no evidence of ischemia preserved left
ventricular function with an ejection fraction of 55-60%, with mild to
moderate mitral regurgitation. Patient underwent a coronary
catheterization for persistent shortness of breath which indicated no
evidence of obstructive coronary disease, normal left ventricular
function with mild to moderate mitral insufficiency. Angiography of the
right coronary artery showed a very large and tortuous artery that
collateralizes into the left main system and supplies the entire left
coronary artery system. The left main coronary origin was from the
pulmonary artery. CTA confirmed the same findings. The recommendations
between cardiology and cardiothoracic surgery were to maximize medical
therapy and follow up due to lack of myocardial ischemia or severe
valvular disease. Currently there are no guidelines or gold standard
management for patients with adult form of ALCAPA that present without
ischemic burden or for patients with the disease that present
asymptomatic with significant coronary collateralization.
Effect of graded narrowing of left main coronary artery on left ventricular function
