Objective The study objective was to identify factors associated with death and cardiac transplantation in infants undergoing the Norwood procedure and to determine differences in associations that ...might favor the modified Blalock–Taussig shunt or a right ventricle-to-pulmonary artery shunt. Methods We used competing risks methodology to analyze death without transplantation, cardiac transplantation, and survival without transplantation. Parametric time-to-event modeling and bootstrapping were used to identify independent predictors. Results Data from 549 subjects (follow-up, 2.7 ± 0.9 years) were analyzed. Mortality risk was characterized by early and constant phases; transplant was characterized by only a constant phase. Early phase factors associated with death included lower socioeconomic status ( P = .01), obstructed pulmonary venous return ( P < .001), smaller ascending aorta ( P = .02), and anatomic subtype. Constant phase factors associated with death included genetic syndrome ( P < .001) and lower gestational age ( P < .001). The right ventricle-to-pulmonary artery shunt demonstrated better survival in the 51% of subjects who were full term with aortic atresia ( P < .001). The modified Blalock–Taussig shunt was better among the 4% of subjects who were preterm with a patent aortic valve ( P = .003). Lower pre-Norwood right ventricular fractional area change, pre-Norwood surgery, and anatomy other than hypoplastic left heart syndrome were independently associated with transplantation (all P < .03), but shunt type was not ( P = .43). Conclusions Independent risk factors for intermediate-term mortality include lower socioeconomic status, anatomy, genetic syndrome, and lower gestational age. Term infants with aortic atresia benefited from a right ventricle-to-pulmonary artery shunt, and preterm infants with a patent aortic valve benefited from a modified Blalock–Taussig shunt. Right ventricular function and anatomy, but not shunt type, were associated with transplantation.
Objectives To describe congenital heart disease death rates in infants born between 34 and 40 weeks, estimate the relationship between gestational age and congenital heart disease infant death rates, ...and compare congenital heart disease death rates across 1- and 2-week intervals in gestational age. Study design The 2000 to 2003 national linked birth/infant death cohort datasets were obtained. Congenital heart disease deaths were identified by using International Statistical Classification of Diseases, 10th Revision codes. Proportional death rates were calculated by using congenital heart disease deaths and all live births. The relationship between congenital heart disease death rates and gestational age was determined. Death rates were compared across intervals. Results A total of 14.9 million records were analyzed. Congenital heart disease deaths occurred in 4736 infants (0.04%) born between 34 and 40 weeks. There was a significant, negative linear relationship between congenital heart disease death rate and gestational age (R2 = 0.97). Comparisons across 1-week intervals varied ( P = .02-.23). All 2-week intervals were statistically significant ( P < .01). Conclusions Congenital heart disease death rates decrease as gestational age approaches 40 weeks. These results should be considered before elective delivery for the sole indication of prenatally diagnosed congenital heart disease.
Objective To assess the variability in asymmetric growth and its association with neurodevelopment in infants with single ventricle (SV). Study design We analyzed weight-for-age z -score minus head ...circumference-for-age z -score (HCAZ), relative head growth (cm/kg), along with individual growth variables in subjects prospectively enrolled in the Infant Single Ventricle Trial. Associations between growth indices and scores on the Psychomotor Developmental Index (PDI) and Mental Developmental Index (MDI) of the Bayley Scales of Infant Development-II (BSID-II) at 14 months were assessed. Results Of the 230 subjects enrolled in the Infant Single Ventricle trial, complete growth data and BSID-II scores were available in 168 (73%). Across the cohort, indices of asymmetric growth varied widely at enrollment and before superior cavopulmonary connection (SCPC) surgery. BSID-II scores were not associated with these asymmetry indices. In bivariate analyses, greater pre-SCPC HCAZ correlated with higher MDI ( r = 0.21; P = .006) and PDI ( r = 0.38; P < .001) and a greater HCAZ increase from enrollment to pre-SCPC with higher PDI ( r = 0.15; P = .049). In multivariable modeling, pre-SCPC HCAZ was an independent predictor of PDI ( P = .03), but not MDI. Conclusion In infants with SV, growth asymmetry was not associated with neurodevelopment at 14 months, but pre-SCPC HCAZ was associated with PDI. Asymmetric growth, important in other high-risk infants, is not a brain-sparing adaptation in infants with SV. Trial registration Clinicaltrials.gov : NCT00113087.
Staged surgical palliation has revolutionized the care of patients with hypoplastic left heart syndrome (HLHS), although the outcomes of survival and cost at a national level remain unclear. This ...study sought to evaluate (1) trends in HLHS surgical outcomes including in-hospital mortality, length of stay (LOS), and cost, and (2) patient and hospital risk factors associated with these outcomes. Hospitalizations for patients with HLHS, including stage I, II, and III palliations, were analyzed using the Kids' Inpatient Database from 2000 through 2009. Trends in mortality, LOS, and cost were analyzed and chi-squared tests were used to test association between categorical variables. Patient and hospital characteristics associated with death were analyzed using logistic regression and associations with LOS were analyzed using ordinary least squared regression. There were 16,923 hospital admissions in patients with HLHS of which 5,672 (34%) included surgical intervention. Total (3,201–5,102) and surgery-specific admissions (1,165–1,618) increased from 2000 to 2009. Mortality decreased 14% per year in stage III palliations (odds ratio OR 0.86; 95% confidence interval CI: 0.79–0.94) and 6% per year for stage I palliations (OR 0.94; 95% CI 0.90–0.99) but not for stage II palliations (OR 1.01; 95% CI; 0.89–1.14). Length of stay increased for stage I and II palliations; however, per-patient hospital cost decreased in 2009. In conclusion, recent decrease in per patient cost for staged surgical palliation for HLHS has correlated temporally with improved mortality.
Indications for intervention in early-stage (Quintero I and II) twin-twin transfusion syndrome (TTTS) are not standardized. Fetal echocardiography can be used to guide the management of early-stage ...patients. The aim of this study was to identify early cardiovascular findings that may precede progression to overt recipient twin (RT) cardiomyopathy in early-stage TTTS.
This was a retrospective review of pregnancies evaluated from 2004 to 2010. Subjects were included when initial evaluation identified Quintero I or II TTTS without evidence of "overt" RT cardiomyopathy, defined on the basis of atrioventricular valve regurgitation, ventricular hypertrophy, and abnormal Doppler myocardial performance indices. Patients elected management with observation or amnioreduction. Pregnancies were grouped by whether the RT developed overt cardiomyopathy. Initial values, including myocardial performance index, diastolic filling time corrected for heart rate (Doppler inflow duration/cardiac cycle length), pulsatility indices of the ductus venosus, umbilical artery, and middle cerebral artery, and cerebroplacental ratio (middle cerebral artery PI/umbilical artery PI), were compared.
Of 174 pregnancies evaluated with early-stage TTTS, 45 (26%) did not show evidence of RT cardiomyopathy. Follow-up echocardiography identified cardiomyopathy in 20 of 45 RTs (44%). Those RTs with subsequent cardiomyopathy had shorter diastolic filling times corrected for heart rate, higher ductus venosus PIs, lower middle cerebral artery PIs, and lower cerebroplacental ratios on initial echocardiography.
Diastolic dysfunction and cerebroplacental redistribution precede findings of overt cardiomyopathy in RTs with early-stage TTTS. Assessment of these parameters may allow earlier identification of RTs with cardiac disease and help guide management. Prospective studies are needed to assess the role of echocardiography in patient selection for the treatment of early-stage TTTS.
Superior cavopulmonary anastomosis timing and outcomes in infants with single ventricle Cnota, James F., MD; Allen, Kerstin R., MA; Colan, Steven, MD ...
Journal of thoracic and cardiovascular surgery/The Journal of thoracic and cardiovascular surgery/The journal of thoracic and cardiovascular surgery,
05/2013, Letnik:
145, Številka:
5
Journal Article
Recenzirano
Odprti dostop
Objectives We sought to identify factors associated with the timing and surgical outcomes of the superior cavopulmonary anastomosis. Methods The Pediatric Heart Network's Infant Single Ventricle ...trial database identified participants who underwent superior cavopulmonary anastomosis. Factors potentially associated with age at superior cavopulmonary anastomosis, length of stay and death by 14 months of age were evaluated. Factors included subject demographics, cardiac anatomy, measures from neonatal hospitalization and pre–superior cavopulmonary anastomosis visit, adverse events, echocardiographic variables, intraoperative variables, superior cavopulmonary anastomosis type, and number of concurrent cardiac surgical procedures. Age at superior cavopulmonary anastomosis was analyzed using Cox proportional hazards regression. Natural log length of stay was analyzed by multiple linear regression. Results Superior cavopulmonary anastomosis was performed in 193 subjects at 5.2 months of age (interquartile range, 4.2, 6.2) and weight of 5.9 kg (interquartile range, 5.3, 6.6). The median length of stay was 7 days (interquartile range, 6, 10). There were 3 deaths and 1 transplant during the superior cavopulmonary anastomosis hospitalization, and 3 deaths and 3 transplants between discharge and 14 months of age. Age at superior cavopulmonary anastomosis was associated with center and interstage adverse events. A longer length of stay was associated with younger age and greater case complexity. Superior cavopulmonary anastomosis type, valve regurgitation, ventricular ejection fraction, and ventricular end-diastolic pressure were not independently associated with age at superior cavopulmonary anastomosis or the length of stay. Conclusions Greater case complexity and more frequent interstage adverse events are associated with an earlier age at superior cavopulmonary anastomosis. Significant variation in age at superior cavopulmonary anastomosis among centers, independent of subject factors, highlights a lack of consensus regarding the optimal timing. Factors associated with length of stay could offer insights for improving presuperior cavopulmonary anastomosis care and surgical outcome.
Infants with single ventricular physiology have volume and pressure overload that adversely affect ventricular mechanics. The impact of superior cavopulmonary anastomosis (SCPA) on single left ...ventricles versus single right ventricles is not known.
As part of the Pediatric Heart Network placebo-controlled trial of enalapril in infants with single ventricular physiology, echocardiograms were obtained before SCPA and at 14 months and analyzed in a core laboratory. Retrospective analysis of the following measurements included single ventricular end-diastolic volume (EDV), end-systolic volume (ESV), mass, mass-to-volume ratio (mass/volume), and ejection fraction. Qualitative assessment of atrioventricular valve regurgitation and assessment of diastolic function were also performed.
A total of 156 participants underwent echocardiography at both time points. Before SCPA, mean ESV and mass Z scores were elevated (3.4 ± 3.7 and 4.2 ± 2.9, respectively) as were mean EDV and mass/volume Z scores (2.1 ± 2.5 and 2.0 ± 2.9, respectively). EDV, ESV, and mass decreased after SCPA, but mass/volume and the degree of atrioventricular valve regurgitation did not change. Subjects with morphologic left ventricles demonstrated greater reductions in ventricular volumes and mass than those with right ventricles (mean change in Z score: left ventricular LV EDV, -1.9 ± 2.1; right ventricular EDV, -0.7 ± 2.5; LV ESV, -2.3 ± 2.9; right ventricular ESV, -0.9 ± 4.6; LV mass, -2.5 ± 2.8; right ventricular mass, -1.3 ± 2.6; P ≤ .03 for all). Approximately one third of patients whose diastolic function could be assessed had abnormalities at each time point.
Decreases in ventricular size and mass occur in patients with single ventricle after SCPA, and the effect is greater in those with LV morphology. The remodeling process resulted in commensurate changes in ventricular mass and volume such that the mass/volume did not change significantly in response to the volume-unloading surgery.
Abstract Background In children with single right ventricular (RV) anomalies, changes in RV size and function may be influenced by shunt type chosen at the time of the Norwood procedure. Objectives ...The study sought to identify shunt-related differences in echocardiographic findings at 14 months and ≤6 months pre-Fontan in survivors of the Norwood procedure. Methods We compared 2-dimensional and Doppler echocardiographic indices of RV size and function, neo-aortic and tricuspid valve annulus dimensions and function, and aortic size and patency at 14.1 ± 1.2 months and 33.6 ± 9.6 months in subjects randomized to a Norwood procedure using either the modified Blalock-Taussig shunt (MBTS) or right ventricle to pulmonary artery shunt (RVPAS). Results Acceptable echocardiograms were available at both time points in 240 subjects (114 MBTS, 126 RVPAS). At 14 months, all indices were similar between shunt groups. From the 14-month to pre-Fontan echocardiogram, the MBTS group had stable indexed RV volumes and ejection fraction, while the RVPAS group had increased RV end-systolic volume (p = 0.004) and decreased right ventricular ejection fraction (RVEF) (p = 0.004). From 14 months to pre-Fontan, the treatment groups were similar with respect to decline in indexed neo-aortic valve area, >mild neo-aortic valve regurgitation (<5% at each time), indexed tricuspid valve area, and ≥moderate tricuspid valve regurgitation (<20% at each time). Conclusions Initial Norwood shunt type influences pre-Fontan RV remodeling during the second and third years of life in survivors with single RV anomalies, with greater RVEF deterioration after RVPAS. Encouragingly, other indices of RV function remain stable before Fontan regardless of shunt type. (Comparison of Two Types of Shunts in Infants with Single Ventricle Defect Undergoing Staged Reconstruction—Pediatric Heart Network; NCT00115934 )
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