Focus on echocardiographic and Doppler analysis of coronary artery abnormal origin from the pulmonary trunk with mild myocardial dysfunction.
BACKGROUND
Late presentation of abnormal origin of coronary artery from the pulmonary artery (ACAPA) is uncommon compared with early presentation, which usually induces extended myocardial necrosis and severe heart failure. The late presentation is characterized by abundant development of intercoronary collaterals resulting in mild and rare symptoms, but nevertheless can cause sudden cardiac death. Our objective was to describe presentation, cardiovascular imaging methods for diagnosis and outcomes of patients with late presentation of ACAP.


METHODS
The study is a retrospective review of a single-center database to identify all patients diagnosed with ACAPA beyond the first year of life.


RESULTS
From 1976 to 2011, 10 patients were identified with ACAPA at the age of 1.1-64 years: 6 with left coronary artery from the pulmonary artery (ALCAPA) and 4 with right coronary artery from the pulmonary artery (ARCAPA). Echocardiography and Doppler imaging evidenced: (1) direct signs: the abnormal coronary ostium arising from the pulmonary trunk with retrograde coronary artery flow and (2) indirect signs: abundant intercoronary septal collaterals with anterograde flow (ARCAPA) or retrograde flow (ALCAPA) and dilatation of the controlateral normally originated coronary artery. Nine patients underwent surgical implantation of the ACAPA into the ascending aorta. After 7.9 years mean follow-up, all were asymptomatic except one who required a second surgery.


CONCLUSIONS
Noninvasive cardiovascular imaging, namely transthoracic echocardiography and Doppler specific parameters, can reach diagnosis of late presentation of ACAPA. Direct aortic implantation is a reliable and effective to establish dual coronary artery circulation and prevent risks due to myocardial ischemia.
Department of Congenital Heart Disease, Louis Pradel Hospital, Hospices civils de Lyon, Lyon, France. pycourand@hotmail.com
Pierre-Yves
PY
Courand
André
A
Bozio
Jean
J
Ninet
Roland
R
Henaine
Magali
M
Veyrier
Mohamed
M
Bakloul
Loic
L
Boussel
Sylvie
S
Di Filippo
D000293:Adolescent; D000328:Adult; D003330:Coronary Vessel Anomalies / Q000150:complications* / Q000000981:diagnostic imaging*; D015150:Echocardiography, Doppler / Q000379:methods*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging*; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D018487:Ventricular Dysfunction, Left / Q000150:complications* / Q000000981:diagnostic imaging*; D055815:Young Adult
ALCAPA; ARCAPA; anomalous coronary artery; surgery