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.
Summary The Fontan operation is a widely used palliative procedure in patients with single ventricle anatomy that results in liver injury. As timely identification of liver fibrosis may result in ...management changes to Fontan patients, the aim of our study was to identify clinically meaningful semi-quantitative/quantitative pathologic parameters for biopsy assessment. We performed a retrospective review of 74 liver needle biopsies from Fontan patients. Fibrosis was assessed using quantitative % collagen deposition (%CD) by Sirius red image analysis, METAVIR, congestive hepatic fibrosis score (CHFS), sinusoidal fibrosis (SF) score, and sinusoidal dilation score. Contemporaneous laboratory, hemodynamic, and ultrasound data were collected. Centrilobular and peri-sinusoidal fibrosis was observed in all cases, with 39.2% high grade. Portal fibrosis was observed in 93.2%, with 36.2% high grade (METAVIR F3-F4). Cirrhosis was observed in 5.4%. %CD was increased over control tissue (p<0.001) and correlated with time from Fontan (r=0.3, p=0.009) and prothrombin time (PT) (r=0.25, p=0.034). Mildly elevated PT/INR was the only measure of liver function consistently associated with multiple high-grade fibrosis scores (METAVIR p=0.046, SF p=0.018). Abnormal liver echotexture on ultrasound was associated with high grade CHFS (p=0.03). Pathologic gradings and %CD correlated with each other (r=0.48-0.8, p<0.001). Hepatic fibrosis in Fontan patients in our study is universally present, appears to be time dependent, and correlates with few laboratory measurements of liver function. Careful assessment of needle liver biopsies lends a more meaningful measure of liver fibrosis in the Fontan patient than clinical and laboratory data, allowing for appropriate changes to patient management.
Background Progressive hepatic fibrosis is common after the Fontan operation, but little is known about its onset. We sought to determine whether hepatic abnormalities before the Fontan operation, ...and whether further abnormalities can be identified soon afterward. Methods Patients undergoing the Fontan operation at our institution were prospectively enrolled and underwent hepatic ultrasound with Doppler and serum testing immediately before and 3 to 6 months after the operation. Results Thirty patients were enrolled at a median age of 3.1 years (range, 2.2 to 8.1 years). Extracardiac Fontan was performed in 67%. Three patients (10%) had abnormal hepatic echotexture before the operation. At the post-Fontan study, mean liver length increased (9.9 versus 10.9 cm; p < 0.0001), and mean hepatic artery end-diastolic velocity decreased (18.8 versus 14.5 cm/s; p = 0.03). One patient showed new, abnormal hepatic echotexture after surgery. Mean aspartate aminotransferase (56.7 versus 60.7 U/L; p = 0.04), alanine transaminase (18.9 versus 33.9 U/L; p = 0.0002), and γ-glutamyl transferase (18.7 versus 46.1 U/L; p = 0.002) increased at the post-Fontan assessment compared with the preoperative values. By linear regression, hospital length of stay and duration with chest tube after Fontan operation were both significantly associated with an increase in γ-glutamyl transferase ( p < 0.001 for both) and alanine transaminase ( p = 0.008, p = 0.048). Conclusions In this cohort, hepatic abnormalities were observed in some patients before the Fontan operation and new abnormalities developed soon afterward. These findings suggest that liver insult may occur before or soon after the Fontan operation.
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.
Operative correction of tetralogy of Fallot frequently results in pulmonary insufficiency and chronic volume overload, which have been linked to increased risk for adverse outcomes. No consensus ...recommendations for the timing of pulmonary valve replacement (PVR) exist. The aim of this study was to examine the pattern of PVR in the United States from 2004 to 2012. The Pediatric Health Information Systems database was used to perform an observational study of children and adults ≥10 years of age with diagnoses of tetralogy of Fallot who underwent PVR at 35 centers in the United States from 2004 and 2012, to assess the rate of PVR and the age at which is performed. Mixed-effects multivariate regression was used to account for patient-level covariates and center-level covariance. Additional analyses assessed for trends in cost, hospital length of stay (LOS), intensive care unit LOS, and in-hospital mortality over the study period. In total, 799 subjects at 35 centers underwent PVR over the study period. The number of PVRs performed per year increased significantly over the study period. There was significant between-center heterogeneity in age at PVR (p <0.001). Age at PVR, intensive care unit LOS, hospital LOS, and cost did not change over the study period. In conclusion, PVR in patients with tetralogy of Fallot is being performed more frequently, without an accompanying change in the age at PVR or other measurable outcomes. There is significant variability in the age at which PVR is performed among centers across the United States. This highlights the need for additional research guiding the optimal timing of PVR.
The investigators recently validated a method of quantifying systemic–to–pulmonary arterial collateral flow using phase-contrast magnetic resonance imaging velocity mapping. Cross-sectional data ...suggest decreased collateral flow in patients with total cavopulmonary connections (TCPCs) compared with those with superior cavopulmonary connections (SCPCs). However, no studies have examined serial changes in collateral flow from SCPCs to TCPCs in the same patients. The aim of this study was to examine differences in collateral flow between patients with SCPCs and those with TCPCs. Collateral flow was quantified by 2 independent measures from 250 single-ventricle studies in 219 different patients (115 SCPC and 135 TCPC studies, 31 patients with both) and 18 controls, during routine studies using through-plane phase-contrast magnetic resonance imaging. Collateral flow was indexed to body surface area, aortic flow, and pulmonary venous flow. Regardless of indexing method, SCPC patients had significantly higher collateral flow than TCPC patients (1.64 ± 0.8 vs 1.03 ± 0.8 L/min/m2 , p <0.001). In 31 patients who underwent serial examinations, collateral flow as a fraction of aortic flow increased early after TCPC completion. In TCPC patients, indexed collateral flow demonstrated a significant negative correlation with time from TCPC. In conclusion, SCPC and TCPC patients demonstrate substantial collateral flow, with SCPC patients having higher collateral flow than TCPC patients overall. On the basis of the paired subset analysis, collateral flow does not decrease in the short term after TCPC completion and trends toward an increase. In the long term, however, collateral flow decreases over time after TCPC completion.
Objective To describe cumulative radiation exposure in a large single-center cohort of children with congenital heart disease (CHD) and identify risk factors for greater exposure. Study design A ...detailed medical radiation exposure history was collected retrospectively for patients aged <18 years who underwent surgery for CHD between January 1, 2001, and July 22, 2009. Cumulative per patient exposure was quantified as the effective dose in millisieverts (mSv) and annualized (mSv/year). Results A total of 4132 patients were subjected to 134 715 radiation examinations at a median follow-up of 4.3 years (range, 0-8.6 years). Exposure clustered around the time of surgery. The median exposure was 14 radiologic tests (the majority of which were plain film radiographs) at an effective dose of 0.96 mSv (the majority of which was from cardiac catheterization), although this distribution had a very wide range. Almost three-quarters (73.7%) were exposed to <3 mSv/year, and 5.3% were exposed to >20 mSv/year. Neonates, children with genetic syndromes, and children requiring surgery for cardiomyopathy, pulmonary valve, single ventricle, or tricuspid valve diseases were more likely to have higher exposure levels, and those requiring surgery for aortic arch anomalies or atrioventricular septal defects were more likely to have lower levels. Conclusion Children with CHD requiring surgery are exposed to numerous medical forms of ionizing radiation. Although the majority of patients receive <3 mSv/year, there are identifiable risk factors for higher exposure levels. This may have important health implications as these patients age.
Abstract Objectives The aim of this study was to define age-stratified, procedure-specific benchmark radiation dose levels during interventional catheterization for congenital heart disease. ...Background There is a paucity of published literature with regard to radiation dose levels during catheterization for congenital heart disease. Obtaining benchmark radiation data is essential for assessing the impact of quality improvement initiatives for radiation safety. Methods Data were obtained retrospectively from 7 laboratories participating in the Congenital Cardiac Catheterization Project on Outcomes collaborative. Total air kerma, dose area product, and total fluoroscopy time were obtained for the following procedures: 1) patent ductus arteriosus closure; 2) atrial septal defect closure; 3) pulmonary valvuloplasty; 4) aortic valvuloplasty; 5) treatment of coarctation of aorta; and 6) transcatheter pulmonary valve placement. Results Between January 2009 and July 2013, 2,713 cases were identified. Radiation dose benchmarks are presented including median, 75th percentile, and 95th percentile. Radiation doses varied widely between age groups and procedure types. Radiation exposure was lowest in patent ductus arteriosus closure and highest in transcatheter pulmonary valve placement. Total fluoroscopy time was a poor marker of radiation exposure and did not correlate well with total air kerma and dose area product. Conclusions This study presents age-stratified radiation dose values for 6 common congenital heart interventional catheterization procedures. Fluoroscopy time alone is not an adequate measure for monitoring radiation exposure. These values will be used as baseline for measuring the effectiveness of future quality improvement activities by the Congenital Cardiac Catheterization Project on Outcomes collaborative.
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