Long-term outcome of patients with right atrial isomerism after common atrioventricular valve plasty.
OBJECTIVE
To review long-term outcomes of patients with right atrial (RA) isomerism who underwent common atrioventricular valve (CAVV) plasty.


METHODS
We retrospectively analysed 59 patients with RA isomerism operated on between January 2004 and April 2016. We divided patients into those with CAVV plasty (CAVV plasty (+), 29 patients) and without CAVV plasty (CAVV plasty (-), 30), and we compared the outcome between the groups. We further divided patients into those with CAVV plasty before bidirectional cavopulmonary shunt (BCPS) operation (group before BCPS, 13 patients) or CAVV plasty with or after BCPS (group with or after BCPS, 16), and we compared the outcome between these groups. We reviewed the outcomes of 7 neonatal patients who underwent CAVV plasty.


RESULTS
Kaplan-Meier estimated survival rates at 10 years were 70 ± 10% and 69 ± 9% in the CAVV plasty (+) and CAVV plasty (-) groups, respectively ( P  = 0.45). Kaplan-Meier estimated survival rates at 10 years were 47 ± 17% and 85 ± 10% in the group before BCPS and group with or after BCPS, respectively ( P  = 0.01). Among 7 neonates in the group before BCPS, 4 are alive; Kaplan-Meier estimated survival rates at 1 year and 5 years were 60 ± 20% and 30 ± 24%, respectively.


CONCLUSIONS
Patients who underwent CAVV plasty with or after BCPS had good outcomes; the outcome of patients with CAVV plasty was the same as that of those without CAVV plasty. Treatment for patients who require CAVV plasty before BCPS, especially neonates, is challenging.
Kenta
K
Imai
Masaya
M
Murata
Yujiro
Y
Ide
Mikio
M
Sugano
Hiroki
H
Ito
Kazuyoshi
K
Kanno
Motonori
M
Ishido
Ryohei
R
Fukuba
Kisaburo
K
Sakamoto
D058366:Blalock-Taussig Procedure; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure; D006349:Heart Valve Diseases / Q000601:surgery; D006351:Heart Valves / Q000601:surgery*; D059446:Heterotaxy Syndrome / Q000453:epidemiology / Q000401:mortality* / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome
Common atrioventricular valve plasty ; Neonate; Right atrial isomerism 