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.

Failure to impact prevalence of arterial ischemic stroke in pediatric cardiac patients over three decades.

INTRODUCTION: Over the past three decades, significant advances in treatment have improved the mortality of children with cardiac disease. The effect of these advances on the prevalence of arterial ischemic stroke (AIS) is unknown. We describe AIS in children with cardiac disease in the modern era. DESIGN: The prospectively enrolled Intermountain Pediatric Stroke Database (including Utah, Wyoming, Idaho, and Nevada) was queried for all patients less than 18 years old with new-onset AIS between January 1, 2003 and August 31, 2009. Medical records of patients with AIS and cardiac disease were reviewed for cardiac diagnosis, age at AIS, anticoagulant therapy, diuretics, hematocrit, bolus fluids, and ongoing morbidity. Data were analyzed using chi-square test and a mixed-effects Poisson regression growth curve model. RESULTS: AIS incidence in our catchment area was 0.01% (10.7/100,000; N = 97). The incidence of AIS in patients with cardiac disease was higher compared with AIS in the total population (incidence 0.13% [132/100,000], odds ratio [OR] 16.1, 95% confidence interval [CI; 9.7--25.9], P < 0.001). Of the 97 patients with AIS, 24 had cardiac disease (25%). The most common cardiac diagnosis was single ventricle (SV; 8/24, 33%). The incidence of AIS in patients with SV cardiac disease was higher compared with those with other cardiac diagnoses (incidence 1.38% [1380/100,000], OR 15.3, 95% CI [5.7--38.2], P < 0.001). Modeling the prevalence estimates reported since 1978, the prevalence of cardiac disease in AIS patients has remained unchanged across time (prevalence increase per each additional year, 0.5%, 95% CI [--2.1%, 3.1%], P = 0.71). CONCLUSION: Children with cardiac disease (particularly those with SV) have increased risk for AIS. The prevalence is unchanged from reports over previous decades. AIS occurred in SV patients despite compliance with current anticoagulation recommendations. Future efforts should focus on best practices to prevent AIS in cardiac patients.

Will a handheld ultrasound scanner be applicable for screening for heart abnormalities in newborns and children?

BACKGROUND: There is significant interest in opportunities to provide echocardiography services for detection of congenital heart disease with portable, or even handheld, devices in remote areas or third world countries where conventional ultrasound systems may not be available. We tested a handheld system (HHS) (SonoHeart, SonoSite Inc, Bothell, Wash) equipped with a broadband, 7- to 4-MHz, miniaturized, curved, linear-array transducer and implemented with an improved directional Doppler flow map. METHODS: All echocardiography scanning was performed in the neonatal nursery, pediatric intensive care department, or pediatric echocardiography laboratory of our institution. We reviewed limited echocardiography view sequences sequentially obtained by the same expert examiner (D.J.S.) in 50 infants and children (age: 1 day to 6 years), with preoperative or postoperative forms of congenital heart disease. Each patient was studied twice, once with a conventional full-feature system (FFS) and then a limited scan with the HHS using similar frequency transducers. The cardiologist (D.J.S.) and blinded research laboratory reviewers (X.L., G.K.M., R.A.R.) read the FFS and HHS image sequences for diagnosis and for grading the quality of the anatomic and flow feature images. The studies were performed and reviewed with the examiner and reviewers blinded to patient diagnosis. RESULTS: The major diagnoses (eg, patent ductus arteriosus, atrio-ventricular (AV) canal, peripheral pulmonary valve stenosis, aortic coarctation, atrial septal defect, ventricular septal defect, preoperative or postoperative tetralogy of Fallot, and mitral regurgitation) were made by both readers, who were unaware of each other's diagnosis results. Furthermore, the average composite HHS cardiac anatomic feature score on a scale of 0 (not visualized) to 3 (visualized precisely) from the parasternal long-axis and 4- or 5-chamber view for cardiac anatomy were 2.67 +/- 0.49 (SD) and 2.50 +/- 0.55, respectively, versus 2.73 +/- 0.45 and 2.55 +/- 0.54 for the FFS. The mean flow feature score, comprising all views, was 2.67 +/- 0.45 (HHS) versus 2.72 +/- 0.48 (FFS). The P values for all above comparisons were >.05. Image quality of the FFS anatomic structures were, thus, not statistically different from the HHS. Although the color cosmetic was different for the HHS directional (nonvelocity) map, only 9% of 150 total findings (including structural abnormalities and flow features, none of which were critical) were missed, whereas the other 91% regurgitant, shunt, stenosis flow features or heart structure were imaged adequately by the HHS in this population. CONCLUSIONS: Implementing high-frequency transducers and programs optimized for tissue and flow imaging on the HHS should provide images of sufficient quality for targeted echocardiography examinations to determine the presence, absence, or status of congenital heart disease in newborns and young children.

Validation of the myocardial performance index by echocardiography in mice: a noninvasive measure of left ventricular function.

BACKGROUND: The myocardial performance index (MPI) is a Doppler-based measure of left ventricular (LV) function. It is noninvasive, independent of LV shape, and does not require dimensional measurements. However, it has never been validated in mice. METHODS: A total of 29 anesthetized mice with LV pressure catheters underwent echocardiography (2-dimensional, M-mode, and Doppler) at baseline and during manipulations of beta-adrenergic tone, temperature, preload, and afterload. The maximum derivative of LV pressure with respect to time (dP/dt(max)) was compared with MPI, fractional shortening (FS), mean velocity of circumferential fiber shortening, and the FS/MPI ratio. RESULTS: MPI (baseline 0.44 +/- 0.07) correlated strongly with dP/dt(max) (R = -.779, P <.001), as did FS and mean velocity of circumferential fiber shortening. MPI differed significantly with contractility, preload, and afterload manipulation. FS/MPI showed the best correlation with dP/dt(max). CONCLUSIONS: MPI strongly correlates with dP/dt(max) over a range of hemodynamic conditions in mice. It can be used as a noninvasive index of LV function in this species.

Strain rate imaging: an in vitro "validation" study using a physiologic balloon model mimicking the left ventricle.

BACKGROUND: Strain rate imaging (SRI) can be implemented from digital ultrasound loops of tissue Doppler imaging (TDI) data and is performed as an autocorrelation solution of the distance between intramyocardial targets. As such, it should have better resolution along longer distances of wall segments that are imaged at the length of individual ultrasound scan lines. METHODS: We used a new left ventricular double-balloon phantom with a tissue-mimicking gel between the walls. Mounted in a water bath and connected to a pulsatile flow pump at four-stroke volume (30-50 ml/beat), the high frame rate, digital, multiple two-dimensional/tissue/TDI loops of balloon wall motion were recorded using a GE VingMed system FiVe (3.5 MHz phased array transducer), with the model scanned longitudinally from the apex. The strain rate (SR) values were measured at the apex and the lateral wall using an offline measurement program, and mean SR values for every 100 msec were calculated by averaging three determinations at each point. The excursions of the apex and lateral wall also were measured directly by high speed digital video imaging, and consecutive velocity profiles were calculated every 100 msec. A total of 40 data points for four-stroke volumes were analyzed. RESULTS: While our balloon model had enough gel targets between the walls to produce a good mimic of myocardial speckle with walls that thickened and thinned, samples immediately across the apex and apex SR values (Hz) varied substantially. In contrast, systematic signals could be obtained from lines imaged >15 degrees from the true apex and crossing a longer length of myocardium. At the lateral wall, there was a close correlation between the video velocities and SR values, as well as a close overlap of the phasic patterns. CONCLUSIONS: SRI produces more reliable data from wall segments parallel to scan lines.