Cor triatriatum: presentation, diagnosis and long-term surgical results.
BACKGROUND
Cor triatriatum sinister is a rare, surgically correctable, congenital cardiac anomaly. Fewer than 250 cases have been reported. It can occur as an isolated defect (classic) or in association with other congenital cardiac anomalies (atypical).


METHODS
A retrospective review of 28 consecutive patients diagnosed at the Royal Children's Hospital in Melbourne, Australia during a 22-year span from 1981 to 2003.


RESULTS
There were 13 men (46%) and 15 women (54%). Fifteen patients were less than 1 year of age (neonates, [n = 7], 25%; infants, [n = 8], 29%). 17 patients (61%) had a communication between the right atrium and either the proximal or distal chamber. Fifteen patients (54%) had atypical cor triatriatum. Median age at presentation was 6 months (range, 0.6 to 240). Twenty-four patients (86%) had presented by 5 years of age. Five patients (18%) underwent emergency surgery. Median age at operation was 7 months (range, 1 to 243). Twenty-seven patients (96%) underwent preoperative transthoracic echocardiography. Nine patients (32%), all with atypical cor triatriatum, required cardiac catheterization for diagnosis. Twenty-seven patients (96%) were correctly diagnosed before treatment. Twenty-seven patients (96%) were treated surgically. The defect was approached through the right atrium in 26 patients (93%). There was 1 early death and 1 patient died 10 years after repair. Follow-up was 86% complete. At a median follow-up of 98 months (range, 0.2 to 284), all patients including those with atypical cor triatriatum were in New York Heart Association's function class 1. Post-repair survival was 96% and 88% at 5 and 15 years, respectively.


CONCLUSIONS
Surgery offers good early and long-term results for both classic and atypical cor triatriatum.
Alphonso
Nelson
N
Victorian Pediatric Cardiac Surgery Unit, Royal Children's Hospital, Melbourne, Australia. nelson.a@bigfoot.com
Nørgaard
Martin A
MA
Newcomb
Andrew
A
d'Udekem
Yves
Y
Brizard
Christian P
CP
Cochrane
Andrew
A
D002675:Child, Preschool; D015897:Comorbidity; D003310:Cor Triatriatum / Q000145:classification / Q000175:diagnosis / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011184:Postoperative Period; D012189:Retrospective Studies; D016019:Survival Analysis