ALCAPA: the role of myocardial viability studies in determining prognosis.
BACKGROUND
ALCAPA is optimally treated by coronary artery reimplantation early in neonatal life. Delayed diagnosis, however, is not infrequent, because symptoms often do not manifest until about 3 months of age, coinciding with the physiological nadir in pulmonary vascular resistance. With delayed diagnosis, there is potential for coronary steal and irreversible myocardial injury, which worsens outcome.


OBJECTIVE
To assess the utility of MRI in determining prognosis in children with surgically corrected ALCAPA.


METHODS
A retrospective chart review was performed in two children with ALCAPA who underwent coronary reimplantation and postoperative cardiac MRI. Both children subsequently underwent cardiac transplantation. The imaging findings and pathological findings at explant are presented.


RESULTS
In both children, there was severe, globally depressed left ventricular systolic function and abnormal delayed enhancement in a predominantly subendocardial distribution. Pathological examination of the cardiac explants showed extensive fibrotic tissue, which correlated with areas of abnormal delayed enhancement on MRI.


CONCLUSIONS
Severe reduction in systolic function and presence of delayed enhancement indicate extensive myocardial injury and pathologically correlate with irreversible fibrotic changes, which may help identify a subgroup of children who will not recover ventricular function and ultimately require heart transplantation.
Department of Diagnostic Imaging, Texas Children's Hospital, 6621 Fannin St., Houston, TX 77030, USA. lxbrowne@texaschildrenshospital.org
Lorna P
LP
Browne
Debra
D
Kearney
Michael D
MD
Taylor
Taylor
T
Chung
Timothy C
TC
Slesnick
Arni C
AC
Nutting
Rajesh
R
Krishnamurthy
D000015:Abnormalities, Multiple / Q000175:diagnosis*; D003330:Coronary Vessel Anomalies / Q000175:diagnosis*; D005260:Female; D006801:Humans; D007223:Infant; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods*; D008297:Male; D017682:Myocardial Stunning / Q000151:congenital* / Q000175:diagnosis*; D011379:Prognosis; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000473:pathology*