Use of total circulatory arrest to support vital organs during heart surgery in infancy is generally associated with greater functional deficits than is use of low-flow cardiopulmonary bypass, although both strategies are associated with increased risk of neurodevelopmental vulnerabilities.
Cerebellar hemorrhagic injury in preterm infants is associated with a high prevalence of long-term pervasive neurodevelopment disabilities and may play an important and underrecognized role in the cognitive, learning, and behavioral dysfunction known to affect survivors.
We found that the effect of duration of total circulatory arrest on later neurodevelopmental outcomes is nonlinear, with little influence at shorter durations and with steadily worsening outcomes after longer durations of circulatory arrest. Because the effects of duration of circulatory arrest may vary according to diagnosis, age at surgery, and other bypass and perioperative variables, this study cannot ascertain a universally "safe" duration of total circulatory arrest.
Background-Patients with a single ventricle have multiple risk factors for central nervous system injury, both before and after the Fontan procedure. Methods and Results-A geographically selected cohort was invited to undergo standardized testing, including age-appropriate measures of intelligence quotient (IQ) and achievement tests. Historical information was obtained by chart review and patient questionnaires. Of the 222 eligible patients, 133 (59.9%) participated. Median age at testing was 11.1 years (range, 3.7 to 41.0 years), 6.0 years (range, 1.6 to 19.6 years) after surgery. Mean full-scale IQ was 95.7Ϯ17.4 (PϽ0.006 versus normal); 10 patients (7.8%) had full-scale IQ scores Ͻ70 (Pϭ0.001). After adjustment for socioeconomic status, lower IQ was associated with the use of circulatory arrest before the Fontan operation (Pϭ0.002), the anatomic diagnoses of hypoplastic left heart syndrome (PϽ0.001) and "other complex" (Pϭ0.05), and prior placement of a pulmonary artery band (Pϭ0.04). Mean composite achievement score was 91.6Ϯ15.4 (PϽ0.001 versus normal); 14 patients (10.8%) scored Ͻ70 (PϽ0.001). After adjustment for socioeconomic status, independent risk factors for low achievement scores included the diagnoses of hypoplastic left heart syndrome (Pϭ0.004) and "other complex" (Pϭ0.003) or prior use of circulatory arrest (Pϭ0.03), as well as a reoperation with cardiopulmonary bypass within 30 days of the Fontan (Pϭ0.01).
Conclusions-Most
We have previously reported concordant changes in cerebral intravascular oxygenation measured by near infrared spectroscopy (NIRS) and mean arterial blood pressure (MAP) in premature infants. We hypothesized that the cerebral oxygenation changes are caused by MAP-induced alterations in cerebral blood flow (CBF) and studied these parameters in neonatal piglets (n = 6). Changes in cerebral intravascular oxygenation were measured by NIRS from the hemoglobin difference (HbD) signal (oxyhemoglobin-deoxyhemoglobin). CBF was measured by the radioactive microsphere technique. The cerebral circulation was also monitored by Doppler determinations of CBF velocity (time average mean velocity) in the anterior cerebral artery. Hypotension to <50% of baseline MAP was achieved by a ligature around the ascending aorta. Arterial oxygenation was maintained constant by mechanical ventilation. As observed in our studies of premature infants, cerebral HbD and MAP showed concordant changes. Hypotension was accompanied by significant decreases both in CBF (42.8 +/- 12.5% of baseline p < 0.01) and HbD (-65.0 +/- 22.0 micromol/L x dpf, p < 0.01). HbD was significantly correlated with MAP (p < 0.05) and time average mean velocity (p = 0.01). Importantly, decreases in cerebral total hemoglobin (HbT), a measure of cerebral blood volume, did not correlate significantly with decreases in MAP. We conclude that 1) decreases in cerebral intravascular oxygenation, as assessed by NIRS, observed with decreases in MAP reflect a decline in CBF, and hence oxygen delivery, 2) the HbD signal is more sensitive to changes in CBF than the HbT signal, and 3) NIRS recordings may have clinical utility in detecting cerebral ischemia.
Neurodevelopmental evaluation preoperatively and postoperatively in CHD patients should be standard practice, not only to identify those with impairments who would benefit from intervention services but also to identify risk factors and strategies to optimize outcome. Fetal management and intervention strategies for specific defects may ultimately play a role in improving in-utero hemodynamics and increasing cerebral oxygen delivery to enhance brain development.
Introduction
Placental insufficiency remains a common cause of perinatal mortality and neurodevelopmental morbidity. Congenital heart disease (CHD) in the fetus and its relationship to placental function is unknown. This study explores placental health and its relationship to neonatal outcomes by comparing placental volumes in healthy pregnancies and pregnancies complicated by CHD using in vivo three-dimensional MRI studies.
Methods
In a prospective observational study, pregnant women greater than 18 weeks gestation with normal pregnancies or pregnancies complicated by CHD were recruited and underwent fetal MR imaging. The placenta was manually outlined and the volume was calculated in cm3. Brain volume was also calculated and clinical data were also collected. Relationships, including interactive effects, between placental and fetal growth, including brain growth, were evaluated using longitudinal multiple linear regression analysis.
Results
135 women underwent fetal MRI between 18 and 39 weeks gestation (mean 31.6 ± 4.4). Placental volume increased exponentially with gestational age (p=0.041). Placental volume was positively associated with birth weight (p <0.001) and increased more steeply with birth weight in CHD-affected fetuses (p=0.046). Total brain and cerebral volumes were smaller in the CHD group (p<0.001), but brainstem volume (p<0.001) was larger. Placental volumes were not associated with brain volumes.
Discussion
Impaired placental growth in CHD is associated with gestational age and birth weight at delivery. Abnormalities in placental development may contribute to the significant morbidity in this high-risk population. Assessment of placental volume by MRI allows for in vivo assessments of placental development.
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