, for the International Cardiac Collaborative on Neurodevelopment (ICCON) Investigators abstract BACKGROUND: Neurodevelopmental disability is the most common complication for survivors of surgery for congenital heart disease (CHD). METHODS:We analyzed individual participant data from studies of children evaluated with the Bayley Scales of Infant Development, second edition, after cardiac surgery between 1996 and 2009. The primary outcome was Psychomotor Development Index (PDI), and the secondary outcome was Mental Development Index (MDI).RESULTS: Among 1770 subjects from 22 institutions, assessed at age 14.5 6 3.7 months, PDIs and MDIs (77.6 6 18.8 and 88.2 6 16.7, respectively) were lower than normative means (each P , .001). Later calendar year of birth was associated with an increased proportion of high-risk infants (complexity of CHD and prevalence of genetic/extracardiac anomalies). After adjustment for center and type of CHD, later year of birth was not significantly associated with better PDI or MDI. Risk factors for lower PDI were lower birth weight, white race, and presence of a genetic/extracardiac anomaly (all P # .01). After adjustment for these factors, PDIs improved over time (0.39 points/year, 95% confidence interval 0.01 to 0.78; P = .045). Risk factors for lower MDI were lower birth weight, male gender, less maternal education, and presence of a genetic/extracardiac anomaly (all P , .001). After adjustment for these factors, MDIs improved over time (0.38 points/year, 95% confidence interval 0.05 to 0.71; P = .02).CONCLUSIONS: Early neurodevelopmental outcomes for survivors of cardiac surgery in infancy have improved modestly over time, but only after adjustment for innate patient risk factors. As more high-risk CHD infants undergo cardiac surgery and survive, a growing population will require significant societal resources.

Adult-Pediatric-Congenital-Heart-Disease Dysfunction Study (LADS) GroupHepatic dysfunction is a recognized complication after Fontan palliation of congenital heart disease. We sought to quantitatively measure hepatic stiffness and vascular Doppler indices using ultrasound (US) and shear wave elastography (SWE) in a Fontan cohort. Subjects were prospectively recruited for echocardiography and real-time hepatic duplex US with SWE for hepatic stiffness (kPa). Doppler peak velocities, velocity time integral, resistive, pulsatility, acceleration indices (RI, PI, AI), and flow volume were measured in celiac artery, superior mesenteric artery, and main portal vein (MPV). A subset underwent cardiac catheterizations with liver biopsy. Correlations were explored between SWE, duplex, hemodynamic, and histopathologic data. In all, 106 subjects were studied including 41 patients with Fontan physiology (age 13.8 6 6 years, weight 45.4 6 23 kg) and 65 controls (age 15.0 6 8.4 years, weight 47.9 6 22 kg). Patients with Fontan physiology had significantly higher hepatic stiffness (15.6 versus 5.5 kPa, P < 0.0001), higher celiac RI (0.78 versus 0.73, P 5 0.04) superior mesenteric artery RI (0.89 versus 0.84, P 5 0.005), and celiac PI (1.87 versus 1.6, P 5 0.034); while MPV flow volume (287 versus 420 mL/ min in controls, P 5 0.007) and SMA AI (829 versus 1100, P 5 0.002) were lower. Significant correlation was seen for stiffness with ventricular end-diastolic pressure (P 5 0.001) and pulmonary artery wedge pressure (P 5 0.009). Greater stiffness correlated with greater degrees of histopathologic fibrosis. No significant change was seen in stiffness or other duplex indices with age, gender, time since Fontan, or ventricular morphology. Conclusion: Elevated hepatic afterload in Fontan, manifested by high ventricular end-diastolic pressures and pulmonary arterial wedge pressures, is associated with remarkably increased hepatic stiffness, abnormal vascular flow patterns, and fibrotic histologic changes. The MPV is dilated and carries decreased flow volume, while the celiac and superior mesenteric arterial RI is increased. SWE is feasible in this population and shows promise as a means for predicting disease severity on liver biopsy. (HEPATOLOGY 2014;59:251-260) U niventricular congenital heart disease (CHD) comprises 5% of all CHD admissions in United States.1 The Fontan procedure has been an effective strategy for the management of univentricular CHD over the past four decades, commonly performed as the final step of a staged surgical palliation.2 Large numbers of patients with this physiology are surviving into adulthood, and the presence of hepatic abnormalities in these patients is increasingly recognized. [3][4][5][6] The pathologic changes in the liver

Aerodigestive issues may persist after vascular ring division despite initial improvement. Accurate preoperative anatomic imaging is imperative to surgical planning.

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Operative factors may be less important than innate patient and preoperative factors and postoperative events in predicting early neurodevelopmental outcomes after cardiac operations in infants. Neurodevelopmental outcomes improved over calendar time when adjusted for patient and medical variables.