Abstract Objective Existing studies of patients palliated with the Fontan operation are limited by heterogeneous patient populations and incomplete follow-up. This study aimed to describe long-term ...post-Fontan survival in a modern patient cohort. Methods All 773 patients who underwent a first Fontan operation at our institution between 1992 and 2009 were reviewed. The primary outcome was the composite endpoint of Fontan takedown, heart transplantation, or death before 2013. Results Follow-up rate was 99.2%. Survival with intact Fontan circulation was 94% at 1 year (95% confidence interval 95% CI, 92%-95%), 90% at 10 years (95% CI, 88%-92%), 85% at 15 years (95% CI, 82%-88%), and 74% at 20 years (95% CI, 67%-80%). Distinct risk factors were identified for early (≤1 year) and late composite outcomes. Independent risk factors for early outcome included prolonged pleural drainage (hazard ratio HR, 4.4; P < .001), intensive care unit stay >1 week (HR, 2.4; P < .001), Fontan before 1997 (HR, 3.3; P < .001), preoperative atrioventricular valve regurgitation (HR, 2.0; P < .001), and longer crossclamp time (HR, 1.3 per 10 minutes; P < .001). Late outcome was predicted by atrioventricular valve regurgitation prior to Fontan (HR, 2.0; P ≤ .001), and post-Fontan ICU stay >1 week (HR, 2.4; P < .001). Conclusions Long-term mortality after Fontan operation remains substantial. Risk factors for death or loss of Fontan circulation differ between the early and late postoperative periods. Long-term survival has not improved appreciably over the last decade, suggesting that alternatives to the Fontan are warranted.
Abstract Although survival after the Fontan operation has improved, little is known about the burden of major medical morbidities associated with the modern total cavopulmonary connection (TCPC). A ...total of 773 consecutive patients who underwent a first Fontan operation at our institution between 1992 and 2009 were retrospectively reviewed. All subjects underwent TCPC (53% lateral tunnel, 47% extracardiac conduit). Median length of follow-up was 5.3 years (IQR 1.4-11.2), and 30% had follow-up >10 years. Freedom from a composite medical morbidity outcome (protein-losing enteropathy, plastic bronchitis, serious thromboembolic event, or tachyarrhythmia) was 47% at 20 years (95% CI 38-55%). Independent risk factors for morbidity included pre-Fontan atrioventricular valve regurgitation (HR 1.7, 95% CI 1.2-2.4; p=0.001), pleural drainage > 14 days (HR 1.5, 95% CI 1.01-2.2, p=0.04), and longer cross-clamp time (HR 1.2 per 10 minutes, 95% CI 1.06-1.3; p=0.004) at the time of TCPC. Surgical era, Fontan type, and ventricular morphology were not associated with the composite outcome. Presence of Fontan-associated morbidity was associated with a 36-fold increase in the risk of subsequent Fontan takedown, heart transplantation, or death (95% CI 17– 76, p<0.001). For patients without any component of the composite outcome, freedom from Fontan failure was 98% at 20 years (95% CI 96-99%). Medical morbidities after TCPC are common and significantly reduce the longevity of the Fontan circulation. However, for those patients who remain free from the composite morbidity outcome, 20-year survival with intact Fontan circulation is encouraging.
Abstract Objectives Despite improved survival in children with hypoplastic left heart syndrome (HLHS), significant concern persists regarding their neurodevelopmental (ND) outcomes. Previous studies ...have identified patient factors, such as prematurity and genetic syndromes, to be associated with worse ND outcomes. However, no consistent relationships have been identified among modifiable management factors, including cardiopulmonary bypass strategies, and ND outcomes after cardiac surgery in infancy. Studies in immature animals, including primates, have demonstrated neurodegeneration and apoptosis in the brain after certain levels and extended durations of anesthetic exposure. Retrospective human studies have also suggested relationships between adverse ND effects and anesthetic exposure. Methods Cumulative minimum alveolar concentration hours (MAC-hrs) of exposure to volatile anesthetic agents (VAA) (desflurane, halothane, isoflurane, and sevoflurane) were collected from an anesthetic database and medical record review for 96 patients with HLHS or variants. ND testing was performed between ages 4 and 5 years, including full-scale IQ, verbal IQ, performance IQ, and processing speed. Four generalized linear modes were hypothesized a priori and tested using a Gaussian (normal) distribution with an identity link. Results Cumulative VAA exposure ranged from 0 to 35.3 MAC-hrs (median 7.5 hours). Using specified covariates identified previously as significant predictors of ND outcomes, statistically significant relationships were identified between total MAC-hrs exposure and worse full-scale IQ and verbal IQ scores ( P 's < .05) alone and after adjusting for relevant covariates. Conclusions Increased cumulative MAC-hrs exposure to VAA is associated with worse ND outcomes in certain domains in children with HLHS and variants.
Objective To report practice and outcomes in infants undergoing elective repair of tetralogy of Fallot. Methods A review of a retrospective cohort of elective complete repair of infants age 6 months ...or younger from 1995 to 2009 was performed. Patients were excluded because of previous interventions, hypercyanotic episodes, intensive care admissions, additional major cardiac defects, or if they were not discharged after birth. Length of stay, mortality, and complications were recorded. Association was determined using logistic or linear regression models and univariate testing determined the multivariate model. Results There were 277 patients included. The hospital mortality rate was zero. A total of 87.4% of patients were discharged home within 7 days of repair, and 21.6% of patients were discharged on or before the third postoperative day. The postoperative course was uncomplicated in 245 patients (88.4%). Longer support time was associated independently with increased odds of complications ( P < .001). Longer support time, younger age, chromosomal abnormality, and presence of a complication were associated independently with a longer hospital stay (all P < .001). Patients younger than 3 months (n = 110) had a longer median hospital stay (4 vs 3 days; P < .001) and longer support times (77.3 ± 35.1 min vs 66.4 ± 34 min; P < .01). Conclusions Elective tetralogy of Fallot repair was performed at 6 months or younger with low morbidity, no hospital mortality, and an 11.6% complication rate. Longer support times, lower weight, chromosomal abnormalities, and complications were associated with a significantly increased duration of hospital stay.
Background Cerebral hypoxia-ischemia remains a complication in children with congenital heart disease. Near-infrared spectroscopy can be utilized at the bedside to detect cerebral hypoxia-ischemia. ...This study aimed to calibrate and validate an advanced technology near-infrared cerebral oximeter for use in children with congenital heart disease. Methods After institutional review board approval and parental consent, 100 children less than 12 years and less than 40 kg were enrolled. Phase I (calibration) measured arterial and jugular venous saturation (SaO2 , SjO2 ) by co-oximetry simultaneously with device signals to calibrate an algorithm to determine regional cerebral saturation against a weighted average cerebral saturation (0.7 SjO2 + 0.3 SaO2 ). Phase II (validation) evaluated regional cerebral saturation from the algorithm against the weighted average cerebral saturation by correlation, bias, precision, and ARoot Mean Square assessed by linear regression and Bland-Altman analysis. Results Of 100 patients, 86 were evaluable consisting of 7 neonates, 44 infants, and 35 children of whom 55% were female, 79% Caucasian, and 41% with cyanotic disease. The SaO2 and regional cerebral saturation ranged from 34% to 100% and 34% to 91%, respectively. There were no significant differences in subject characteristics between phases. For the entire cohort, ARMS , bias, precision, and correlation coefficient were 5.4%, 0.5%, 5.39%, and 0.88, respectively. Age, skin color, and hematocrit did not affect these values. Conclusions This cerebral oximeter accurately measures the absolute value of cerebral saturation in children over a wide range of oxygenation and subject characteristics, offering advantages in assessment of cerebral hypoxia-ischemia in congenital heart disease.
Objective Small head circumferences and white matter injury in the form of periventricular leukomalacia have been observed in populations of infants with severe forms of congenital heart defects. ...This study tests the hypothesis that congenital heart defects delay in utero structural brain development. Methods Full-term infants with hypoplastic left heart syndrome or transposition of the great arteries were prospectively evaluated with preoperative brain magnetic resonance imaging. Patients with independent risk factors for abnormal brain development (shock, end-organ injury, or intrauterine growth retardation) were excluded. Outcome measures included head circumferences and the total maturation score on magnetic resonance imaging. Total maturation score is a previously validated semiquantitative anatomic scoring system used to assess whole brain maturity. The total maturation score evaluates 4 parameters of maturity: (1) myelination, (2) cortical infolding, (3) involution of glial cell migration bands, and (4) presence of germinal matrix tissue. Results The study cohort included 29 neonates with hypoplastic left heart syndrome and 13 neonates with transposition of the great arteries at a mean gestational age of 38.9 ± 1.1 weeks. Mean head circumference was 1 standard deviation below normal. The mean total maturation score for the cohort was 10.15 ± 0.94, significantly lower than reported normative data in infants without congenital heart defects, corresponding to a delay of 1 month in structural brain development. Conclusion Before surgery, term infants with hypoplastic left heart syndrome and transposition of the great arteries have brains that are smaller and structurally less mature than expected. This delay in brain development may foster susceptibility to periventricular leukomalacia in the preoperative, intraoperative, and postoperative periods.
Abstract Objectives The American Clinical Neurophysiology Society recommends continuous electroencephalographic monitoring after neonatal cardiac surgery because seizures are common, often ...subclinical, and associated with worse neurocognitive outcomes. We performed a quality improvement project to monitor for postoperative seizures in neonates with congenital heart disease after surgery with cardiopulmonary bypass. Methods We implemented routine continuous electroencephalographic monitoring and reviewed the results for an 18-month period. Clinical data were collected by chart review, and continuous electroencephalographic tracings were interpreted using standardized American Clinical Neurophysiology Society terminology. Electrographic seizures were classified as electroencephalogram-only or electroclinical seizures. Multiple logistic regression was used to assess associations between seizures and potential clinical and electroencephalogram predictors. Results A total of 161 of 172 eligible neonates (94%) underwent continuous electroencephalographic monitoring. Electrographic seizures occurred in 13 neonates (8%) beginning at a median of 20 hours after return to the intensive care unit after surgery. Neonates with all types of congenital heart disease had seizures. Seizures were electroencephalogram only in 11 neonates (85%). Status epilepticus occurred in 8 neonates (62%). In separate multivariate models, delayed sternal closure or longer deep hypothermic circulatory arrest duration was associated with an increased risk for seizures. Mortality was higher among neonates with than without seizures (38% vs 3%, P < .001). Conclusions Continuous electroencephalographic monitoring identified seizures in 8% of neonates after cardiac surgery with cardiopulmonary bypass. The majority of seizures had no clinical correlate and would not have been otherwise identified. Seizure occurrence is a marker of greater illness severity and increased mortality. Further study is needed to determine whether seizure identification and management lead to improved outcomes.
Objectives Blood pressure control is important after repair of coarctation of the aorta. We report the first prospective multi-institutional trial addressing the safety and efficacy of esmolol after ...repair of coarctation of the aorta in infants and children. Methods The primary objective of this phase IIIb, multicenter, double-blind, randomized, dose-ranging trial was the efficacy of esmolol to control hypertension. Candidates included subjects younger than 6 years and weighing 2.5 kg or more who underwent surgical intervention for coarctation of the aorta and required therapy for systemic hypertension. One hundred sixteen subjects received esmolol: 36 received a low dose (125 μg/kg), 43 received a medium dose (250 μg/kg), and 37 received a high dose (500 μg/kg). The primary outcomes were the change in systolic blood pressure and the need for additional antihypertensive rescue medication 5 minutes after the initiation of esmolol. Results All dose groups showed a significant decrease from baseline in systolic blood pressure (−9.6 ± 16.3 mm Hg, P < .001). There were no differences in systolic blood pressure response at 5 minutes between dose groups (high, medium, or low) or age groups. The need for rescue medication at 5 minutes was not different between dose groups. All dose groups showed similar incidences of adverse events. There were no serious adverse events. Discussion Esmolol can be administered safely to patients younger than 6 years after repair of coarctation of the aorta. In the dose range of 125 to 500 μg/kg, esmolol significantly decreased systolic blood pressure.
Objective Risk factors for poor outcome with congenital complete heart block include prematurity, low birth weight, hydrops, low ventricular rates, and congenital heart disease. In this group, ...medical therapy is often ineffective, pacing is technically challenging, and mortality exceeds 80%. The purpose of this study is to assess outcomes of patients with congenital complete heart block who were paced in the first 24 hours after birth owing to the presence of known risk factors. Methods We performed a retrospective review of patients with congenital complete heart block paced in the first 24 hours after birth at our institution between November 1, 1995, and July 31, 2007. Results Thirteen patients were identified, 4 of whom had heterotaxy syndrome. Eleven patients had temporary epicardial pacing wires placed; 2 received permanent pacemakers as the initial mode of pacing. There were 7 deaths (54% mortality) at a mean age of 19.9 ± 19 days. Among 7 patients with structural heart disease, there was 1 survivor. Among 6 patients with structurally normal hearts, there were 5 survivors ( P = .025). Patients with temporary wires who survived to permanent pacemaker implantation (6/11) used their temporary leads for 33.8 ± 18.3 days. Conclusions In the severely affected fetus with congenital complete heart block and significant structural heart disease, outcomes remain poor; however, neonates with congenital complete heart block and structurally normal hearts who are monitored antenatally and delivered in a planned fashion at an institution capable of early pacing can have favorable outcomes. The use of temporary pacing wires is an option in the management of these patients.
Objective Hypoxic-ischemic white mater brain injury commonly occurs in neonates with hypoplastic left heart syndrome (HLHS). Approximately one half of HLHS survivors will exhibit neurobehavioral ...symptoms believed to be associated with this injury, although the exact timing of the injury is unknown. Methods Neonates with HLHS were recruited for pre- and postoperative monitoring of cerebral oxygen saturation, cerebral oxygen extraction fraction, and cerebral blood flow using 2 noninvasive optical-based techniques: diffuse optical spectroscopy and diffuse correlation spectroscopy. Anatomic magnetic resonance imaging was performed before and approximately 1 week after surgery to quantify the extent and timing of the acquired white matter injury. The risk factors for developing new or worsened white matter injury were assessed using uni- and multivariate logistic regression. Results A total of 37 neonates with HLHS were studied. On univariate analysis, neonates who developed a large volume of new, or worsened, postoperative white matter injury had a significantly longer time to surgery ( P = .0003). In a multivariate model, a longer time between birth and surgery, delayed sternal closure, and greater preoperative cerebral blood flow were predictors of postoperative white matter injury. Additionally, a longer time to surgery and greater preoperative cerebral blood flow on the morning of surgery correlated with lower cerebral oxygen saturation ( P = .03 and P = .05, respectively) and greater oxygen extraction fraction ( P = .05 for both). Conclusions A longer time to surgery was associated with new postoperative white matter injury in otherwise healthy neonates with HLHS. The results suggest that earlier Norwood palliation might decrease the likelihood of acquiring postoperative white matter injury.
PDF
