Jw. Gaynor et al., Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection, J THOR SURG, 117(3), 1999, pp. 506-514
Citations number
12
Categorie Soggetti
Cardiovascular & Respiratory Systems","Cardiovascular & Hematology Research
Background and methods: Between January 1, 1984, and December 1, 1997, 73 i
nfants with functional single ventricle and total anomalous pulmonary venou
s connection were admitted to our institution. A retrospective review was u
ndertaken to determine factors influencing survival. Results: Heterotaxy sy
ndrome was present in 52 patients and hypoplastic left heart syndrome in 14
, Obstructed total anomalous pulmonary venous connection was present in 21
patients. The pulmonary venous connection was supracardiac in 32 patients,
cardiac in 21. patients, infracardiac in 13, and mixed in 7, Twelve patient
s died before the operation. The remaining 61 patients underwent surgery at
a median age of 5 days (range 1 day-2.5 years). Overall survival was 45% a
t 6 months of age, 37% at 1 year, and 19% at 5 years. Survival for patients
undergoing surgery was 54% at 6 months of age, 44% at 1 year, and 23% at 5
years. By univariate analysis with the Cox proportional hazards model, you
nger age at the time of the initial operation and repair of total anomalous
pulmonary venous connection were predictors of mortality Lung tissue from
14 patients was available for histologic examination. The pulmonary veins w
ere dilated and wall thickness was increased. Increased muscularization of
the arteries was seen in 11 patients. Conclusions: The long-term prognosis
for children undergoing staged reconstructive operations for single ventric
le and total anomalous pulmonary venous connection is poor. Early mortality
is high and late death is a continuing risk. Development of the pulmonary
vasculature, especially the pulmonary veins, is abnormal, even in children
without clinical evidence of pulmonary venous obstruction.