Outcome after prenatal diagnosis of tricuspid atresia: a multicenter experience.
BACKGROUND
The outcome of prenatally diagnosed tricuspid atresia (TA) is undefined. We sought to characterize clinical and echocardiographic features of fetal TA and to determine factors associated with mortality.


RESULTS
All fetuses with TA (n = 88) seen at 3 tertiary care institutions from 1990 to 2005 were reviewed. There were 58 liveborn infants (median gestational age 38 weeks, range 24-40 weeks), 4 in utero demises, 25 terminations of pregnancy, and 1 mother lost to follow-up. Obstruction was present at the pulmonary valve in 27 (45%), aortic valve in 6 (10%), and aortic arch in 15 (25%). Three neonates received compassionate care, 1 died with multiple extracardiac anomalies, 2 were lost to follow-up, and 52 liveborns were actively managed with Blalock-Taussig shunt (23), Norwood palliation (14), pulmonary artery band (10), bidirectional cavopulmonary connection (3), atrial septostomy (1), and right outflow stent (1). Of those actively managed, there were 7 (14%) of 52 who died. Kaplan-Meier estimates of survival were 91% at 1 month, 87% at 6 months, and 83% at 1 year with no subsequent deaths for 13 years. By multivariate analysis, 2 independent factors were associated with an increase in time-related mortality in the actively managed group: presence of chromosomal anomaly or syndrome (P = .005) and use of extracorporeal membrane oxygenation (P = .002).


CONCLUSIONS
This is the largest study describing TA in fetus. Compared with published observations of TA diagnosed postnatally, antenatal diagnosis of TA appears to have similar short-term survival in pregnancies surviving to birth.
Wald
Rachel M
RM
The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. rachel.wald@uhn.on.ca
Tham
Edythe B
EB
McCrindle
Brian W
BW
Goff
Donna A
DA
McAuliffe
Fionnuala M
FM
Golding
Fraser
F
Jaeggi
Edgar T
ET
Hornberger
Lisa K
LK
Tworetzky
Wayne
W
Nield
Lynne E
LE
D000015:Abnormalities, Multiple / Q000453:epidemiology; D000028:Abortion, Induced / Q000706:statistics & numerical data; D001900:Boston / Q000453:epidemiology; D002423:Cause of Death; D025063:Chromosome Disorders / Q000453:epidemiology; D015897:Comorbidity; D004452:Echocardiography; D015199:Extracorporeal Membrane Oxygenation / Q000706:statistics & numerical data; D005260:Female; D005315:Fetal Diseases / Q000175:diagnosis* / Q000453:epidemiology* / Q000235:genetics / Q000628:therapy; D005865:Gestational Age; D006801:Humans; D008297:Male; D009864:Ontario / Q000453:epidemiology; D010043:Outcome and Process Assessment, Health Care; D011247:Pregnancy; D011256:Pregnancy Outcome; D011296:Prenatal Diagnosis / Q000706:statistics & numerical data*; D012189:Retrospective Studies; D012495:San Francisco / Q000453:epidemiology; D016019:Survival Analysis; D018785:Tricuspid Atresia / Q000175:diagnosis* / Q000453:epidemiology* / Q000235:genetics / Q000628:therapy