Twenty-year outcome of anomalous origin of left coronary artery from pulmonary artery: management of mitral regurgitation.
BACKGROUND
This study is a single-center experience with surgical repair of anomalous origin of left coronary artery from pulmonary artery (ALCAPA) with focus on the management of associated mitral regurgitation (MR).


METHODS
We performed a retrospective analysis of cases presenting to a quaternary referral center between November 1990 and October 2011.


RESULTS
In all, 25 patients (18 female) presented with a diagnosis of ALCAPA at a median age of 5 months (range, 1.5 to 102). Twenty-one patients (84%) had moderate to severe impairment of left ventricular function with median fractional shortening of 14% (range, 2% to 33%), and 19 patients (76%) had moderate to severe MR. Surgery was performed with direct coronary reimplantation in 16 patients (64%) and intrapulmonary tunnel (Takeuchi repair) in 9 (36%). Four patients had mitral valve repair at time of surgery, all for structural anomalies. Functional MR with a structurally normal mitral valve was not repaired. The median duration of postoperative follow-up was 93 months (range, 9 to 240). There were no early or late deaths, and no patient required mechanical support. Four patients (16%) required surgical or catheter reintervention. At last follow-up, 24 of 25 patients were asymptomatic; the left ventricular function was normal in 22 patients. Moderate MR was present in 4 patients. There was significant improvement in left ventricular function and MR (p < 0.01) during follow-up.


CONCLUSIONS
Surgical repair of ALCAPA has good long-term results with low mortality and reintervention rates. The majority of MR is functional and will improve with reperfusion, but structural mitral valve abnormalities should be repaired at the time of surgery.
Heart Unit, Birmingham Children's Hospital, National Health Service Foundation Trust, Birmingham, United Kingdom.
Vikram
V
Kudumula
Heart Unit, Birmingham Children's Hospital, National Health Service Foundation Trust, Birmingham, United Kingdom. Electronic address: chetan.mehta@bch.nhs.uk.
Chetan
C
Mehta
Heart Unit, Birmingham Children's Hospital, National Health Service Foundation Trust, Birmingham, United Kingdom.
Oliver
O
Stumper
Heart Unit, Birmingham Children's Hospital, National Health Service Foundation Trust, Birmingham, United Kingdom.
Tarak
T
Desai
Heart Unit, Birmingham Children's Hospital, National Health Service Foundation Trust, Birmingham, United Kingdom.
Ashish
A
Chikermane
Heart Unit, Birmingham Children's Hospital, National Health Service Foundation Trust, Birmingham, United Kingdom.
Paul
P
Miller
Heart Unit, Birmingham Children's Hospital, National Health Service Foundation Trust, Birmingham, United Kingdom.
Rami
R
Dhillon
Heart Unit, Birmingham Children's Hospital, National Health Service Foundation Trust, Birmingham, United Kingdom.
Timothy J
TJ
Jones
Heart Unit, Birmingham Children's Hospital, National Health Service Foundation Trust, Birmingham, United Kingdom.
Joseph
J
De Giovanni
Heart Unit, Birmingham Children's Hospital, National Health Service Foundation Trust, Birmingham, United Kingdom.
William J
WJ
Brawn
Heart Unit, Birmingham Children's Hospital, National Health Service Foundation Trust, Birmingham, United Kingdom.
David J
DJ
Barron
D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D003330:Coronary Vessel Anomalies / Q000150:complications* / Q000175:diagnosis / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008944:Mitral Valve Insufficiency / Q000150:complications* / Q000175:diagnosis / Q000601:surgery*; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000601:surgery*; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome