Objectives
Our primary objective was to test the effects of first postoperative hematocrit on early shunt occlusion for children undergoing systemic to pulmonary artery shunt placement. Because any intervention to reduce shunt occlusion is only beneficial if it either reduces mortality or is, at least, mortality neutral, we also tested the effects of first postoperative hematocrit on in-hospital mortality.
Methods
We conducted a retrospective study on all neonates who underwent primary systemic to pulmonary artery shunt placement, with or without a Norwood / Damus-Kaye-Stansel procedure, at our institution between January 2010 and July 2015. Univariable regression was used to test the effects of first postoperative hematocrit on early shunt occlusion and 30-day mortality, clustering standard errors by surgeon. In secondary analyses, we also tested the associations between red blood cell transfusion volumes in the first 24 postoperative hours on first postoperative hematocrit, shunt occlusion, and mortality.
Results
Eighty infants met inclusion criteria. Median initial postoperative hematocrit was 41.7% (IQR 37.9–46.0). Six (7.5%) died. Four (5.0%) died within the first 30 days. Five infants (6.3%) experienced early shunt occlusion. No children with early shunt occlusion died. In univariable models, for every five additional percentage points of hematocrit, an infant’s odds of early shunt occlusion more than doubled (OR 2.70, p=0.009). The odds of all-cause 30-day mortality remained unchanged.
Conclusions
Higher postoperative hematocrit levels are associated with early shunt occlusions in infants undergoing primary systemic to pulmonary artery shunt placement. Randomized controlled trials are needed to validate these findings and to determine ideal postoperative hematocrit targets for this population.
Survival is significantly compromised in infants born with congenital diaphragmatic hernia and major cardiac anomalies. Mortality is highest when congenital diaphragmatic hernia occurs in association with d-transposition of the great arteries. We present three infants with congenital diaphragmatic hernia associated with d-transposition of the great arteries from a single institution. All three infants survived to discharge after surgical repair/palliation of both the diaphragmatic hernia and heart defect and are doing well at last follow-up. The clinical course and management of these three patients are described.
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