The Impact of the Left Ventricle on Right Ventricular Function and Clinical Outcomes in Infants with Single-Right Ventricle Anomalies up to 14 Months of Age.

BACKGROUND: Children with single-right ventricle anomalies such as hypoplastic left heart syndrome (HLHS) have left ventricles of variable size and function. The impact of the left ventricle on the performance of the right ventricle and on survival remains unclear. The aim of this study was to identify whether left ventricular (LV) size and function influence right ventricular (RV) function and clinical outcome after staged palliation for single-right ventricle anomalies. METHODS: In the Single Ventricle Reconstruction trial, echocardiography-derived measures of LV size and function were compared with measures of RV systolic and diastolic function, tricuspid regurgitation, and outcomes (death and/or heart transplantation) at baseline (preoperatively), early after Norwood palliation, before stage 2 palliation, and at 14 months of age. RESULTS: Of the 522 subjects who met the study inclusion criteria, 381 (73%) had measurable left ventricles. The HLHS subtype of aortic atresia/mitral atresia was significantly less likely to have a measurable left ventricle (41%) compared with the other HLHS subtypes: aortic stenosis/mitral stenosis (100%), aortic atresia/mitral stenosis (96%), and those without HLHS (83%). RV end-diastolic and end-systolic volumes were significantly larger, while diastolic indices suggested better diastolic properties in those subjects with no left ventricles compared with those with measurable left ventricles. However, RV ejection fraction was not different on the basis of LV size and function after staged palliation. Moreover, there was no difference in transplantation-free survival to Norwood discharge, through the interstage period, or at 14 months of age between those subjects who had measurable left ventricles compared with those who did not. CONCLUSIONS: LV size varies by anatomic subtype in infants with single-right ventricle anomalies. Although indices of RV size and diastolic function were influenced by the presence of a left ventricle, there was no difference in RV systolic function or transplantation-free survival on the basis of LV measures.

Prenatal detection of critical cardiac outflow tract anomalies remains suboptimal despite revised obstetrical imaging guidelines.

BACKGROUND: Fetal echocardiography can accurately diagnose critical congenital heart disease prenatally, but relies on referrals from abnormalities identified on routine obstetrical ultrasounds. Critical congenital heart disease that is frequently missed due to inadequate outflow tract imaging includes anomalies such as truncus arteriosus, double outlet right ventricle, transposition of the great arteries, tetralogy of Fallot, pulmonary stenosis, and aortic stenosis. OBJECTIVE: This study evaluated the prenatal detection rate of critical outflow tract anomalies in a single urban pediatric hospital before and after "AIUM Practice Guideline for the Performance of Obstetric Ultrasound Examinations," which incorporated outflow tract imaging. DESIGN: Infants with outflow tract anomalies who required cardiac catheterization and/or surgical procedure(s) in the first 3 months of life were retrospectively identified. This study evaluated two time periods; pre-guidelines from June 2010 to May 2013 and post-guidelines from January 2015 to June 2016. June 2013-December 2014 was excluded as a theoretical period necessary for obstetrical practices to implement the revised guidelines. RESULTS: Overall, prenatal diagnosis occurred in 55% of infants with critical outflow tract anomalies; of the three most common defects, prenatal diagnosis occurred in 53% of D-transposition of the great arteries, 63% of tetralogy of Fallot, and 80% of double outlet right ventricle patients. Pre-guidelines, prenatal diagnosis occurred in 52% (52 of 102) infants with critical outflow tract anomalies requiring early cardiac intervention. Post-guidelines, prenatal diagnosis occurred in 61% (33 of 54) infants, not significantly different than the prenatal detection rate pre-guidelines (P = .31). CONCLUSIONS: Despite revised obstetrical guidelines highlighting the importance of outflow tract imaging, referrals and prenatal diagnosis of these types of critical congenital heart disease remain low. Education of obstetrical sonographers and practitioners who perform fetal anatomic screening is vital to increase referrals and prenatal detection of critical outflow tract anomalies.

Impact of initial shunt type on cardiac size and function in children with single right ventricle anomalies before the Fontan procedure: the single ventricle reconstruction extension trial.

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).

White matter lesions in children and adolescents with migraine.

BACKGROUND: The etiology and clinical importance of white matter lesions in migraine remain poorly understood. To understand these issues more fully, we reviewed the brain magnetic resonance imaging scans of pediatric patients and assessed the relationships between white matter lesions, migraine type, patent foramen ovale, and right-to-left shunting. METHODS: The magnetic resonance imaging scans of a cohort of children (n = 89) and adolescents, ages 6 to 18 years, who participated in a study of migraine and patent foramen ovale were reviewed. All children in the cohort had undergone saline contrast transthoracic echocardiography and transcranial Doppler studies. RESULTS: White matter lesions were detected in 15 of the 89 patients (17%). White matter lesions were small (<5 mm) in the majority (10/15; 66%). We observed no relationship between the presence of white matter lesions and (1) migraine type (six patients with white matter lesions among 35 with migraine with aura [17%] vs. nine with white matter lesions among 54 without aura [17%]; P = 1.0); (2) patent foramen ovale (five with white matter lesions among 35 with patent foramen ovale [14%] vs. 10 with white matter lesions among 54 without patent foramen ovale [19%]; P = 0.77); or (3) shunt size (two large shunts in 15 with white matter lesions [13%] vs. nine large shunts among 72 without white matter lesions [13%]; P = 1.0). CONCLUSIONS: These results indicate that small white matter lesions are not infrequent in children and adolescents with migraine. However, no relationships between white matter lesions and migraine type, patent foramen ovale, or degree of right-to-left shunting were observed.

Doppler flow patterns in the right ventricle-to-pulmonary artery shunt and neo-aorta in infants with single right ventricle anomalies: impact on outcome after initial staged palliations.

BACKGROUND: A Pediatric Heart Network trial compared outcomes in infants with single right ventricle anomalies undergoing Norwood procedures randomized to modified Blalock-Taussig shunt (MBTS) or right ventricle-to-pulmonary artery shunt (RVPAS). Doppler patterns in the neo-aorta and RVPAS may characterize physiologic changes after staged palliations that affect outcomes and right ventricular (RV) function. METHODS: Neo-aortic cardiac index (CI), retrograde fraction (RF) in the descending aorta and RVPAS conduit, RVPAS/neo-aortic systolic ejection time ratio, and systolic/diastolic (S/D) ratio were measured early after Norwood, before stage II palliation, and at 14 months. These parameters were compared with transplantation-free survival, length of hospital stay, and RV functional indices. RESULTS: In 529 subjects (mean follow-up period, 3.0 ± 2.1 years), neo-aortic CI and descending aortic RF were significantly higher in the MBTS cohort after Norwood. The RVPAS RF averaged <25% at both interstage intervals. Higher pre-stage II descending aortic RF was correlated with lower RV ejection fraction (R = -0.24; P = .032) at 14 months for the MBTS cohort. Higher post-Norwood CI (5.6 vs 4.4 L/min/m(2), P = .04) and lower S/D ratio (1.40 vs 1.68, P = .01) were correlated with better interstage transplantation-free survival for the RVPAS cohort. No other Doppler flow patterns were correlated with outcomes. CONCLUSIONS: After the Norwood procedure, infants tolerated significant descending aortic RF (MBTS) and conduit RF (RVPAS), with little correlation with clinical outcomes or RV function. Neo-aortic CI, ejection time, and S/D ratios also had limited correlations with outcomes or RV function, but higher post-Norwood neo-aortic CI and lower S/D ratio were correlated with better interstage survival in those with RVPAS.

Myocardial strain and strain rate in Kawasaki disease.

AIMS: We sought to determine whether velocity vector imaging (VVI)-derived left ventricular (LV) myocardial deformation indices could detect subtle myocardial abnormalities in acute Kawasaki disease (KD). METHODS AND RESULTS: The study cohort of children with KD was divided by coronary artery dilation (CAD, Z-score >2.5) and/or uncomplicated vs. treatment-resistant (persistent/recrudescent fever) cases and compared with age-matched controls. Peak systolic LV myocardial strain (ε) and strain rate (SR) were obtained using VVI on pre-treatment echocardiograms. Comparisons were made between controls and (i) the entire KD group, (ii) KD group subdivided by CAD, and (iii) KD group subdivided by treatment resistance. The KD group consisted of 32 children (66% male, 24 ± 20 months). Of these, 17 had CAD and 14 had resistant KD. The control group consisted of 22 children (55% male, 20 ± 17 months). Routine echo indices of LV systolic function were normal for both groups. Compared with controls, KD patients had lower global longitudinal ε (-15.29 vs. -12.94, P = 0.04) and SR (-1.12 vs. -0.87, P = 0.003). On subgroup analysis compared with controls, KD patients with CAD (n = 17) had lower longitudinal ε (-15.29 vs. -11.87, P = 0.02) and SR (-1.12 vs. -0.86, P = 0.005). Subdivided by treatment resistance, compared with controls, those with resistant KD had lower longitudinal ε (-15.29 vs. -11.8, P = 0.01) and SR (-1.12 vs. -0.82, P = 0.003). CONCLUSION: Despite normal LV systolic function by routine echocardiographic measurements, KD patients have reduced longitudinal LV ε and SR, which may be more sensitive indicators of myocardial inflammation and may provide supportive criteria to avoid delayed diagnosis of KD.

Clinical outcomes and resource use for infants with hypoplastic left heart syndrome during bidirectional Glenn: summary from the Joint Council for Congenital Heart Disease National Pediatric Cardiology Quality Improvement Collaborative registry.

The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) registry captures information on interstage management of infants with hypoplastic left heart syndrome (HLHS). The purpose of this study was to identify interstage risk factors for increased resource use and adverse outcomes during bidirectional Glenn (BDG) hospitalization. All infants in the NPC-QIC registry (31 United States hospitals) undergoing BDG surgery were included (December 2009 to August 2010). Patient demographics, interstage variables, operative procedures, and complications were recorded. Days of hospitalization, ventilation, inotrope use, and complications were surrogates of resource use. Logistic regression analysis determined the associations between predictor variables and resource use. Of 162 infants, 105 (65 %) were males. At BDG, the median age was 155 days (range 78-128), mean weight-for-age z-score was -1.6 ± 1.1, mean length-for-age z-score was -1.5 ± 1.7, and mean preoperative oxygen saturation was 78 % ± 7 %. Caloric recommendations were met in 60 % of patients, and 85 % of patients participated in a home-surveillance program. Median days of intubation, inotrope use, and hospitalization were 1, 2, and 7, respectively. There were 4 post-BDG deaths and 55 complications. In multivariate analysis, lower weight-for-age z-score, female sex, and aortic atresia with mitral stenosis were associated with a higher risk of BDG complications. Meeting caloric recommendations before BDG was associated with fewer hospitalization days. Lower weight-for-age z-score was an independent and potentially modifiable risk factor for BDG complications. HLHS infants who met caloric recommendations before BDG had a lower duration of hospitalization at BDG. These data justify targeting nutrition in interstage strategies to improve outcomes and decrease costs for patients with HLHS.

Prenatally diagnosed coarctation: a more sinister disease?

Prenatal diagnosis of coarctation may improve survival, but little is known regarding its association with severity of left heart hypoplasia and surgical outcome. This study compared echocardiographic measurements of left heart structures, surgical strategies, and postoperative outcomes between neonates with a prenatal diagnosis of coarctation and those with a postnatal diagnosis. All the neonates who underwent coarctation repair during 2006-2010 were reviewed. The neonates with complex congenital heart disease or an unrestrictive ventricular septal defect requiring cardiopulmonary bypass (CPB) for closure were excluded from the study. Based on the time of diagnosis, the subjects were divided into the following three groups: group 1 (prenatal diagnosis), group 2 (diagnosis at 0-7 days), and group 3 (diagnosis at 8-28 days). The study population consisted of 46 neonates: 14 in group 1, 14 in group 2, and 18 in group 3. Compared with group 3, group 1 had a smaller left ventricular volume index, lower mitral and aortic valve diameter z-scores, and lower transverse arch z-scores. Compared with group 2, group 1 had smaller aortic valve z-scores but otherwise had similar measurements. At repair, 64 % of the neonates in group 1 required CPB versus 29 % in group 2 (p = 0.12) and 22 % in group 3 (p = 0.03). All the neonates underwent biventricular repair, with no surgical mortality. Group 1 had a hospital stay of 13.4 ± 10.8 versus 7.5 ± 4.2 days in group 2 (p = 0.06) and 7.3 ± 4.5 days in group 3 (p = 0.03). The neonates with prenatally diagnosed coarctation had smaller left heart structures than the neonates with coarctation diagnosed after the first week of age, were more likely to require extensive arch reconstruction under CPB, and had longer hospital stays.

Patent foramen ovale in children with migraine headaches.

OBJECTIVE: To determine the prevalence of patent foramen ovale (PFO) in children with migraine. STUDY DESIGN: Children aged 6.0 to 18.0 years with migraine headache were evaluated for PFO and right-to-left shunting with color-flow Doppler scanning, saline solution contrast transthoracic echocardiography, and contrast transcranial Doppler scanning. RESULTS: The population consisted of 109 children with migraine; 38 (35%) with aura and 71 (65%) without aura. The overall PFO prevalence was 35%, similar to the general population (35% vs 25%; P = .13). However, compared with the general population (25%), the PFO prevalence was significantly greater in subjects with aura (50%, P = .0004) but similar in those without aura (27%, P = .73). Atrial shunt size was not associated with the presence or absence of aura. CONCLUSION: Children with migraine with aura have a significantly higher prevalence of PFO compared with those without aura or the general population. These data suggest that PFO may contribute to the pathogenesis of migraine with aura in children and have implications for clinical decision making.

Does abciximab promote coronary artery remodeling in patients with Kawasaki disease?

Standard therapy, consisting of intravenous immunoglobulin and aspirin, reduces, but does not eliminate, coronary artery aneurysms (CAAs) in patients with Kawasaki disease. Large CAAs can persist or undergo varying degrees of regression. The treatment of large CAAs using abciximab has been associated with short-term regression; however, longer term data are unavailable. We sought to obtain longer term follow-up data regarding the changes in the diameters of large CAAs in patients receiving both abciximab and standard therapy and to compare these changes to those of a similar group receiving standard therapy alone. All patients with Kawasaki disease and large CAAs (diameter >5 mm or Z score >10) treated from 1986 to 2007 were identified and divided into 2 groups. The abciximab group received abciximab plus standard therapy and the no-abciximab group received standard therapy alone. The maximum diameters of the proximal right and left anterior descending CAAs were obtained from echocardiograms. The Z scores were calculated for 3 points: the acute/subacute phase (<8 weeks) and at 1 and 3 to 5 years of follow-up. The patients in the abciximab (n = 11) and no-abciximab (n = 7) groups were similar in age, interval to treatment, gender, and largest CAA Z score at diagnosis (19.6 +/- 6.2 vs 25.8 +/- 9.5, p = 0.11). The change in CAA Z score was similar between the 2 groups at 1 year (p = 0.99). At 3 to 5 years of follow-up, compared to baseline, the abciximab group had a greater decrease in the CAA Z score than did the no-abciximab group (-14.0 +/- 4.0 vs -8.2 +/- 5.9, p = 0.04). In conclusion, abciximab treatment might be associated with vascular remodeling in patients with large CAAs.