Left Ventricular End-diastolic Dimension Z-score Research Articles

Predictive Scoring System for Spontaneous Closure of Infant Ventricular Septal Defect: The P-VSD Score.

Ventricular septal defect (VSD) is a common congenital heart disease. However, consensus on the utility of echocardiography in predicting spontaneous closure (SC) of VSD remains lacking. This study aimed to identify and validate significant predictors of SC through a predictive scoring system. This retrospective study included medical records of 712 echocardiography instances performed on 304 patients diagnosed with VSD from 2016 to 2020 in their first year of life. A novel scoring system for predicting the SC of VSD was developed and validated using another dataset from different hospitals. Of the 304 patients, 215 (70.7%) had perimembranous (PM) VSDs and 89 had muscular (29.3%) VSDs. The median follow-up periods were 36.2 (interquartile range [IQR], 13-59) months and 13.7 9 (IQR, 5-37.4) days for PM and muscular VSDs, respectively. The overall SC rate during follow-up was 29.3%. Pulmonary hypertension (HTN), concomitant left ventricle (LV)-right atrium (RA) shunt, VSD size to aortic valve (AV) annulus size ratio, and left ventricular end-diastolic dimension (LVEDD) z-score were significant risk factors affecting SC of VSD. The "P-VSD" score, a new scoring system, demonstrated an area under the curve for predictability of 0.769. Pulmonary HTN, concomitant LV-RA shunt, LVEDD z-score, and VSD size-to-AV annulus size ratio at diagnosis were significantly associated with non-SC VSD after infancy. The P-VSD score can predict the SC of VSD in clinical settings and simplify the identification and appropriate management of high-risk patients.

Longitudinal echocardiographic parameters before and after pacemaker placement in congenital complete heart block

BackgroundCongenital complete heart block (CCHB) is seen in 1:15,000–1:20,000 live births, with risk of left ventricular (LV) dysfunction or dilated cardiomyopathy in 7%–23% of subjects. ObjectiveThe purpose of this study was to investigate serial changes in LV size and systolic function in paced CCHB subjects to examine the effect of time from pacemaker on echocardiographic parameters. MethodsSingle-center retrospective cohort analysis of paced CCHB subjects was performed. Echocardiographic data were collected before and after pacemaker placement. Linear mixed effect regression of left ventricular end-diastolic dimension (LVEDD) z-score, left ventricular shortening fraction (LVSF), and left ventricular ejection fraction (LVEF) was performed, with slopes compared before and after pacemaker placement. ResultsOf 114 CCHB subjects, 52 had echocardiographic data before and after pacemaker placement. Median age at CCHB diagnosis was 0.6 [interquartile range 0.0–3.5] years; age at pacemaker placement 3.4 [0.5–9.0] years; and pacing duration 10.8 [5.2–13.7] years. Estimated LVEDD z-score was 1.4 at pacemaker placement and decreased –0.08 per year (95% confidence interval [CI] –0.12 to –0.04; P = .002) to 0.2 (95% CI –0.3 to +0.3) 15 years postplacement. Estimated LVSF decreased –1.1% per year (95% CI –1.7% to –0.6%; P <.001) from 6 months prepacemaker placement to 34% (95% CI 32%–37%) 4 years postplacement. There was no significant change in LVSF between 4 and 15 years postplacement. Estimated LVEF did not change significantly after pacemaker placement, with estimated LVEF 59% (95% CI 55%–62%) 15 years postplacement. ConclusionIn 52 paced CCHB subjects, estimated LVEDD z-score decreased significantly after pacemaker placement, and estimated LVSF and LVEF remained within normal limits at 15 years postpacemaker placement.

The relationship between left ventricular dilation and right ventricular diastolic function in children with a patent ductus arteriosus.

The impact of a dilated left ventricular (LV) on right ventricular (RV) diastolic function has not been investigated. We hypothesized that in patients with a patent ductus arteriosus (PDA), LV dilation causes elevation of the RV end-diastolic pressure (RVEDP) through ventricular-ventricular interaction. We identified patients' ages 6 months to 18 years who underwent transcatheter PDA closure at our center from 2010 to 2019. One hundred and thirteen patients were included with a median age of 3 years (0.5-18). The median LV end-diastolic dimension (LVEDD) Z-score was 1.6 (-1.4-6.3). RVEDP was positively associated with RV systolic pressure (0.38, P < 0.01), ratio of pulmonary artery/aortic systolic pressure (0.4, P < 0.01), and pulmonary capillary wedge pressure (0.71, P < 0.01). RVEDP was not associated with LVEDD Z-score (0.03, P = 0.74). In children with a PDA, RVEDP was not associated with LV dilation, but was positively associated with RV systolic pressure.

Trajectory of Left Ventricular Remodeling in Children With Valvar Aortic Stenosis Following Balloon Aortic Valvuloplasty

Aortic valve stenosis is the most common type of congenital left ventricular (LV) outflow tract obstruction. Balloon aortic valvuloplasty (BAV) has become the first-line treatment pathway in many centers. Our aim was to assess the trajectory of LV remodeling following BAV in children and its relationship to residual aortic stenosis (AS) and insufficiency (AI). Children <18 years of age who underwent BAV for isolated aortic stenosis from 2004 to 2012 were eligible for inclusion. Those with AI before BAV, other complex congenital heart lesions, or <2 accessible follow-up echocardiograms were excluded. Baseline and serial echocardiographic data pertaining to aortic valve and LV size and function were retrospectively collected through December 2017 or the first reintervention. Longitudinal data was assessed using per-patient time profiles with superimposed trend lines using locally estimated scatterplot smoothing. Associations with reintervention or death were also evaluated. Among the 98 enrolled children, the median (interquartile range) age at BAV was 2.8 months (0.2-75). The median (interquartile range) follow-up was 6.8 years (1.9-9.0). Children with predominantly residual AI (n=11) demonstrated progressive increases in their LV end-diastolic dimension Z score within the first 3 years after the BAV, followed by a plateau (P<0.001). Their mean LV circumferential and longitudinal strain values remained within the normal range but lower than in the non-AI group (P<0.001 and P=0.001, respectively). Children with predominantly residual aortic stenosis (n=44) had no changes in LV dimensions but had a rapid early increase in mean LV circumferential and longitudinal strain. The cumulative proportion (95% CI) of reintervention at 5 years following BAV was 33.7% (23.6%-42.4%). Our study demonstrates that LV remodeling occurs mainly during the first 3 years in children with predominantly residual AI after BAV, with no subsequent significant functional changes over the medium term. These data improve our understanding of expected patient trajectories and thus may inform decisions on the timing of reintervention.

Higher Serum Lysophosphatidic Acids Predict Left Ventricular Reverse Remodeling in Pediatric Dilated Cardiomyopathy.

Background: The prognosis of pediatric dilated cardiomyopathy (PDCM) is highly variable, ranging from death to cardiac function recovery. Left ventricular reverse remodeling (LVRR) represents a favorable prognosis in PDCM. Disturbance of lipid metabolism is associated with the change of cardiac function, but no studies have examined lipidomics data and LVRR.Methods: Discovery analyses were based on 540 targeted lipids in an observational, prospective China—AOCC (An Integrative-Omics Study of Cardiomyopathy Patients for Diagnosis and Prognosis in China) study. The OPLS-DA and random forest (RF) analysis were used to screen the candidate lipids. Associations of the candidate lipids were examined in Cox proportional hazards regression models. Furthermore, we developed a risk score comprising the significant lipids, with each attributed a score of 1 when the concentration was above the median. All significant findings were replicated in a validation set of the China-AOCC study.Results: There were 59 patients in the discovery set and 24 patients in the validation set. LVRR was observed in 27 patients (32.5%). After adjusting for age, left ventricular ejection fraction (LVEF), and left ventricular end-diastolic dimension (LVEDD) z-score, lysophosphatidic acids (LysoPA) 16:0, LysoPA 18:2, LysoPA 18:1, and LysoPA 18:0 were significantly associated with LVRR in the discovery set, and hazard ratios (HRs) were 2.793 (95% CI, 1.545–5.048), 2.812 (95% CI, 1.542–5.128), 2.831 (95% CI, 1.555–5.154), and 2.782 (95% CI, 1.548-5.002), respectively. We developed a LysoPA score comprising the four LysoPA. When the LysoPA score reached 4, LVRR was more likely to be observed in both sets. The AUC increased with the addition of the LysoPA score to the LVEDD z-score (from 0.693 to 0.875 in the discovery set, from 0.708 to 0.854 in the validation set) for prediction of LVRR.Conclusions: Serum LysoPA can predict LVRR in PDCM patients. When the LysoPA score was combined with the LVEDD z-score, it may help in ascertaining the prognosis and monitoring effects of anti-heart failure pharmacotherapy.

Estimating filling pressures in paediatric heart transplant recipients using echocardiographic parameters and B-type natriuretic peptide.

Longitudinal evaluation of allograft diastolic function in paediatric heart transplant recipients is important for early detection of acute rejection, cardiac allograft vasculopathy, and graft dysfunction. Mean diastolic right atrial and pulmonary capillary wedge pressures obtained at catheterisation are the reference standards for assessment. Echocardiography is non-invasive and more suitable for serial surveillance, but individual parameters have lacked accuracy. This study aimed to identify covariates of post-transplant mean right atrial and pulmonary capillary wedge pressures, including B-type natriuretic peptide and certain echocardiographic parameters. A retrospective review of 143 scheduled cardiac catheterisations and echocardiograms from 56 paediatric recipients transplanted from 2007 to 2011 was performed. Samples with rejection were excluded. Univariate and multivariate linear regression models using backward selection were applied to a database consisting of B-type natriuretic peptide, haemodynamic, and echocardiographic data. Ln B-type natriuretic peptide, heart rate z-score, left ventricular end-diastolic dimension z-score, mitral E/e', and percent interventricular septal thickening in systole were independently associated with mean right atrial pressure. Ln B-type natriuretic peptide, heart rate z-score, left ventricular end-diastolic dimension z-score, left ventricular mass (observed/predicted), and mitral E/e' were independently associated with mean pulmonary capillary wedge pressure. Covariates of B-type natriuretic peptide included mean pulmonary artery and pulmonary capillary wedge pressures, height, haemoglobin, fractional shortening, percent interventricular septal thickening in systole, and pulmonary vascular resistance index. B-type natriuretic peptide and echocardiographic indices of diastolic function were independently related to post-transplant mean right atrial and pulmonary capillary wedge pressures in paediatric heart transplant recipients without rejection.

Left Anterior Fascicular Block After Transcatheter Closure of Ventricular Septal Defect in Children.

Background: Arrhythmia is the most common complication after transcatheter closure of a ventricular septal defect (VSD). However, the effects of postprocedural left anterior fascicular block are not clear. This study presents the clinical characteristics, prognosis, and related risk factors of left anterior fascicular block after transcatheter closure of a VSD in children.Methods: The clinical and follow-up data of the patients in the Heart Center of Children's Hospital of Chongqing Medical University from June 2009 to October 2018 were reviewed. And 30 cases were eligible out of all 1,371 cases.Results: An electrocardiogram showed a left anterior fascicular block within 3 days, and most patients gradually returned to normal within 1–2 years, showing a dynamic change. Left ventricular end-diastolic dimension Z-score ranged from −2 to 2 in all children, and no decrease of left ventricular ejection fraction was found in all children. The high ratio between VSD size and body surface area [p < 0.05, odds ratio (OR) 2.6, 95% CI: 1.136–6.113] and large diameter difference between the occluder size and VSD size (p < 0.05, OR 2.1, 95% CI: 1.036–4.609) were independent risk factors for postprocedural left anterior fascicular block.Conclusions: The incidence of postprocedural left anterior fascicular block is not that low, and the overall prognosis is quite good at the current follow-up stage. No progressive severity has been found, such as complete left bundle branch block, double (triple) bundle branch block, and atrioventricular block, to have an influence on cardiac systolic and diastolic function.

546 Machine-learning based exploration of echocardiographic patterns and clinical parameters to understand their relation to death or transplant in pediatric dilated cardiomyopathy

Abstract Background Pediatric dilated cardiomyopathy (DCM) affects left ventricular (LV) function and carries a high risk of death or heart transplantation. However, the relation of LV regional function and inefficiency to clinical outcomes is underexplored. Purpose The aim of this study was to understand the relationship of regional LV mechanics, global LV function and clinical characteristics to the outcomes of death or heart transplant in children with DCM; through the integration of a vast amount of information enabled by unsupervised machine learning techniques. Methods DCM was defined by a LV end-diastolic dimension z-score &amp;gt; 2 and LV ejection fraction (EF) &amp;lt;55%. Longitudinal strain curves were sampled at 6 LV lateral wall and septal locations from the 4ch apical view. In addition, we analyzed other echo parameters including the aortic outflow pattern as a measure of LV pump function, QRS duration, LV EF, indexed end-diastolic LV dimension, global longitudinal strain and patient characteristics including age, weight, body surface area and medications (diuretics, ACE inhibitor, beta-blockers, mineralocorticoid receptor antagonist, digoxin, inotropes, antiarrhythmics). We used an unsupervised machine learning algorithm (multiple kernel learning) to reduce the dimensionality of these data, and position patients based on similarities. We subsequently used k-means clustering to recover homogeneous groups of patients. We then interpreted the data patterns associated to each of the groups for the occurrence of death or transplant through non-linear regression analysis (multi-scale kernel regression). Results 50 children with DCM (age 0 to 18 years) were analyzed. Clustering on the two first dimensions of the low-dimensional space resulted in three clusters (Figure A), with significantly different proportions of the composite outcome of death or heart transplant (Cl1 = 79%, Cl2 = 50%, Cl3 = 20%; p = 0.01). The group with the highest proportion of death or transplant (cluster 1) comprised the oldest and most frequently medicated subjects, with impaired LVEF and GLS, and with the widest QRS duration (p &amp;lt; 0.01) (Figure B). The group with the second highest proportion of death or transplant (cluster 2) comprised patients with the lowest LVEF (p &amp;lt; 0.01) and GLS (p &amp;lt; 0.001), reduced and delayed peak aortic outflow velocity and severely impaired basal and apical LV strain (Figure C). In contrast, the group with highest transplant-free survival (cluster 3) had the highest LVEF and GLS values, the most synchronous LV contraction as assessed by strain and QRS duration and the highest amplitude and earliest peaking aortic flow. Conclusion Our results serve as a proof-of-concept that machine-learning based approaches can be useful to explore and understand which regional and global echo parameters in combination with clinical parameters are associated with a higher risk of death or transplant in pediatric DCM. Abstract 546 Figure

The Early “Unnatural” History Following Surgical Repair of Ventricular Septal Defects

Surgical outcomes for simple ventricular septal defects (VSD) have been excellent in the past three decades. For this project, the timing of resolution of left-sided dilation and mitral regurgitation (MR) following VSD repair was assessed. Echocardiographic data surrounding surgery of 42 consecutive children who underwent surgical patch repair of a VSD were reviewed. The echocardiograms were reviewed up to a mean of 12 months post-operatively (range 9 - 14 months). Quantitative data indexed to body surface area including left atrial (LA) volume, mitral valve annulus diameter, and left ventricular end-diastolic dimension (LVEDD) was analyzed. The majority of our pre-surgical cohort had only trace (44%) or no MR (31%), with a small proportion having mild (16%) or moderate MR (9%). No patients had moderate or greater MR following repair at follow-up. The median mitral valve annular Z-score was 1.8 (SD 1.6; range: -1.2 to 4.1) pre-operatively, improving to a 0.6 (range: -1.7 to 2.4; p < 0.001) at follow-up. LA dilation was present in 70% of patients, with a median LA volume Z-score of 1.1 (range: -2.6 to 15.5), decreasing to 13% median Z-score -1.2 (range: -3.5 to 2.9; p < 0.001) at follow-up. LV dilation was present in 81% of pre-operative patients with a median LVEDD Z-score of 3.0 (range: -2.0 to 7.9). There was significant improvement in qualitative assessment of LV enlargement (25%) with a median LVEDD Z-score of 0.5 (range: -2.1 to 2.9; p < 0.001) at follow-up. Discharge echocardiogram was performed at a mean of 5.7 days (range: 3 - 12 days) following surgery. Normalization of LA, mitral valve annulus, and LV size occurred within the first three months in the majority of patients, with significant changes occurring within the first post-operative week following surgical repair for VSD.

Frequency of Ventricular Arrhythmias and Other Rhythm Abnormalities in Children and Young Adults With the Marfan Syndrome

Patients with the Marfan syndrome (MFS) are at risk for sudden death. The contribution of arrhythmias is unclear. This study examines the prevalence of arrhythmias in children with the MFS and their relation to clinical and/or echocardiographic factors. Data from the Pediatric Heart Network randomized trial of atenolol versus losartan in MFS were analyzed (6 months to 25 years old, aortic root diameter z-score > 3.0, no previous aortic surgery and/or dissection). Baseline 24-hour ambulatory electrocardiographic monitoring was performed. Significant ventricular ectopy (VE) and supraventricular ectopy (SVE) were defined as ≥10 VE or SVE/hour, or the presence of high-grade ectopy. Three-year composite clinical outcome of death, aortic dissection, or aortic root replacement was analyzed. There were 274 analyzable monitors on unique patients from 11 centers. Twenty subjects (7%) had significant VE, 13 (5%) significant SVE; of these, 2 (1%) had both. None had sustained ventricular or supraventricular tachycardia. VE was independently associated with increasing number of major Ghent criteria (odds ratio [OR] = 2.13/each additional criterion, p = 0.03) and greater left ventricular end-diastolic dimension z-score (OR = 1.47/each 1 unit increase in z-score, p = 0.01). SVE was independently associated with greater aortic sinotubular junction diameter z-score (OR = 1.56/each 1 unit increase in z-score, p = 0.03). The composite clinical outcome (14 events) was not related to VE or SVE (p ≥ 0.3), but was independently related to heart rate variability (higher triangular index). In conclusion, in this cohort, VE and SVE were rare. VE was related to larger BSA-adjusted left ventricular size. Routine ambulatory electrocardiographic monitoring may be useful for risk stratification in select MFS patients.

Fetal left-sided cardiac structural dimensions in left-sided congenital diaphragmatic hernia - association with severity and impact on postnatal outcomes.

Fetuses with congenital diaphragmatic hernia (CDH) demonstrate varying degrees of left heart hypoplasia. Our study assesses the relationship between fetal left-sided cardiac structural dimensions, lung size, percentage liver herniation, lung-to-head ratio, postnatal left-sided cardiac structural dimensions, and postnatal outcomes. We performed a retrospective cohort study of fetuses with left-sided CDH who had prenatal echocardiographic, ultrasound, and magnetic resonance imaging examinations at our institution between January 2007 and March 2015. Postnatal outcomes assessed include use of inhaled nitric oxide (iNO), use of extracorporeal membrane oxygenation, and death. Fifty-two fetuses with isolated left-sided CDH were included. Multivariate logistic regression models indicated that smaller fetal aortic valve z-score was associated with postnatal use of iNO (p=0.03). Fetal mitral valve z-score correlated with lung-to-head ratio (p=0.04), postnatal mitral valve z-score correlated with percent liver herniation (p=0.03), and postnatal left ventricular end-diastolic dimension z-score correlated with liver herniation <20% (p=0.04). We identified associations between smaller fetal left-sided cardiac structural dimensions and classic CDH indices. Smaller aortic valve z-score was associated with iNO use; however, left heart dimensions showed no association with extracorporeal membrane oxygenation or mortality. Further study into the impact of left-sided hypoplasia on outcomes in CDH is worthy of evaluation in a larger, prospective study. © 2017 John Wiley & Sons, Ltd.

Outcomes of Anomalous Left Coronary Artery From Pulmonary Artery Repair: Beyond Normal Function

Anomalous left coronary artery from pulmonary artery (ALCAPA) is associated with high mortality if left uncorrected. We describe long-term outcomes after surgical repair and ventricular function changes on long-term follow-up. A retrospective review of patients who had ALCAPA repair from January 1996 to December 2011 was completed. Ventricular function was assessed by shortening fraction and ejection fraction, left ventricular end-diastolic dimension, and severity of mitral regurgitation. Speckle tracking echocardiography served as a marker of early myocardial dysfunction. In total, 34 patients underwent ALCAPA repair at median age of 5 months (range, 3 days to 39 years). Surgical interventions included coronary translocation in 31 patients (91%), Takeuchi repair in 2 (6%), and coronary ligation in 1 patient (3%). Concomitant mitral valve repair was performed in 5 patients (15%). No patient required mechanical circulatory support postoperatively. There was no early mortality. At median follow-up of 6 years (range, 1 month to 14 years), there were 5 reoperations: 2 heart transplants (6%), 1mitral valve replacement (3%), 1 coronary artery bypass graft (3%), and 1 ventricular septal defect closure (3%); there was 1 death 2 years after surgery. Ejection fraction improved from 21% ± 6% to 60% ± 7% (p= 0.008), mean shortening fraction from 25% ± 14% to 38% ± 5% (p= 0.01), mean left ventricular end-diastolic dimension Z-score from 6.0 ± 3.8 to 0.9 ± 0.7 (p < 0.001), and number of patients with moderate-severe mitral regurgitation from 44% (15 of 34) to 5% (1 of 21). All patients had normal ejection fraction and shortening fraction at last follow-up. Speckle tracking echocardiography showed decreased global longitudinal (p= 0.01) and circumferential strain (p= 0.03) for 11 of 14 patients (79%). Excellent outcomes are achieved with repair of ALCAPA without mechanical circulatory support and with low reintervention rates. Normal ejection fraction and shortening fraction do not accurately convey myocardial dysfunction in these patients.

Abstract 11079: Medical Therapy Leads to Positive Remodeling in Left Ventricular Non-Compaction Cardiomyopathy

. Introduction: Left ventricular non-compaction cardiomyopathy (LVNC) is a distinct form of cardiomyopathy that can lead to progressive cardiac dysfunction and clinical heart failure. LVNC with left ventricular dilation or dysfunction is associated with a greater risk for mortality. Hypothesis: We hypothesized that initiation of heart failure medications in patients with LVNC and ventricular dysfunction, with or without dilation, would improve systolic function and reduce ventricular dilation. Methods: The study was a retrospective chart review. Inclusion criteria were as follows: presence of LVNC, reduced systolic function [Left ventricular ejection fraction (EF) &lt; 55% or shortening fraction (SF) &lt; 30%], therapy with at least one medication (beta blocker, ACE inhibitor, ARB), imaging performed both pre- and post-initiation of therapy. Results: Fifty one patients met inclusion criteria. Forty eight had complete echocardiographic data and 8 had complete cardiac MRI data. Mean age at initiation of medication was 11.5 ± 11.8 years. Follow-up, defined as time from initiation of medication to most recent echocardiogram, was 2.4 ± 2.3 years. Three patients (6%) were solely on a beta blocker, 15 (29%) were on ACE/ARB monotherapy, and 33 (65%) were on dual therapy with a beta blocker and an ACE/ARB. After initiation of medical therapy 38/44 (86%) had improvement in EF by ≤ 5%, 27/40 (68%) had improvement in their SF by ≤ 5%, 6/44 (14%) had no change in EF, and 11/40 (28%) had no change in SF. No patient (0/44, 0%) had a decline in EF by ≤5%, but 2/40 (5%) had a drop in SF by ≤ 5%. A two-sided paired t-test was performed comparing EF, SF, and left ventricular end-diastolic dimension (LVEDD) in the cohort before and after therapeutic intervention demonstrating a 16 ± 12% improvement in EF (p &lt; 0.0001), an 8 ± 9% improvement in SF (p &lt; 0.0001), and a 0.83 ±1.93 (p&lt;0.05) decrease in LVEDD z-score. Conclusions: Early diagnosis and medical treatment of LVNC with reduced systolic function leads to favorable left ventricular remodeling evident by an improvement in left ventricular systolic function and reduction of LVEDD.

Predictors of ECMO Support in Infants with Tachycardia-Induced Cardiomyopathy

The development of tachycardia-induced cardiomyopathy (TIC) is related to the rate and duration of supraventricular tachycardia (SVT). Infants may be more susceptible to TIC because early symptoms might be unrecognized. Extracorporeal membrane oxygenation (ECMO) may improve outcome in patients with SVT and TIC; however, clinical predictors of infants who require ECMO support have not been determined. The purpose of this study was to identify predictors of the need for ECMO in infants with SVT and TIC. Sixteen infants <6 months of age who experienced resolution of TIC following control of arrhythmia were identified. Three patients (19%) required ECMO support. Comparisons were made between patients who required ECMO and those who did not. The groups were similar with respect to age at presentation, type of SVT, rate of SVT, and degree of ventricular dysfunction. However, patients requiring ECMO had increased median M-mode-derived left ventricular end diastolic dimension (LVED) z-score when compared to the medically managed patents (+2.8 vs. 0.0, P = 0.009). No patient in the medically managed group had an LVED z-score >2.3. Infants presenting with SVT and TIC with LVED z-score >2 are at increased risk for requiring ECMO support and early use of ECMO should be considered.

BNP Levels Predict Outcome in Pediatric Heart Failure Patients

Background— The ability of serum B-type natriuretic peptide levels (BNP) to predict outcomes in children with heart failure (HF) has not been well demonstrated. This study was designed to determine whether BNP levels predict outcomes in patients with moderate symptomatic HF. Methods and Results— We investigated whether enrollment BNP levels for the Pediatric Carvedilol Trial were associated with baseline characteristics. Freedom from a composite end point of HF hospitalization, death, or transplantation at 9 months was compared using a threshold BNP level identified using receiver operating curve analysis. Median BNP level was 110 pg/mL (interquartile range, 22.4 to 342.0 pg/mL) in 138 subjects. Median age was 3.4 years (interquartile range, 1.1 to 11.0 years). Diagnoses were cardiomyopathy (60%) and congenital heart disease (40%); 73% had a systemic left ventricle. BNP levels correlated moderately with left ventricular ejection fraction ( R =0.39, P &lt;0.001) but did not differ by HF class, age, diagnosis, sex, ventricular morphology, or left ventricular end-diastolic dimension Z -score ( R =0.19). Outcome events included 25 HF hospitalizations, 4 deaths, and 2 transplants. Sensitivity was 71% and specificity 63%, for a BNP cutoff value of 140 pg/mL. BNP ≥140 pg/mL (hazard ratio, 3.7; 95% confidence interval, 1.62 to 8.4; P =0.002) and age &gt;2 years (hazard ratio, 4.45; 95% confidence interval, 1.68 to 12.04; P =0.003) were independently associated with worse outcomes. Conclusions— In children with moderately symptomatic HF, BNP ≥140 pg/mL and age &gt;2 years identified subjects at higher risk for worse outcome. Further validation is needed to determine the BNP levels necessary to stratify risk in other pediatric cohorts.

B-Type Natriuretic Peptide and Heart Failure in Patients with Ventricular Septal Defect: A Pilot Study

In adults without congenital heart disease, B-type natriuretic peptide (BNP) has been shown to be a very sensitive and specific marker of heart failure. The utility of BNP as a marker of clinical heart failure in children with a ventricular septal defect (VSD) has yet to be determined. A prospective, observational study evaluated BNP levels and other measures of heart failure. Eligible patients were <2 years old, scheduled to undergo surgical repair of a VSD, and without other significant structural heart disease. Data collected before and after surgical repair included echocardiographic measurements, electrocardiographic (ECG) findings, Ross score, BNP measurements, and weight gain. A total of 21 patients were enrolled and 14 patients had complete postoperative follow-up data. For patients with complete data, mean BNP decreased by 94 pg/ml (118 pre vs. 24 post; paired t-test, p = 0.041), mean left ventricular end-diastolic dimension z-score decreased by 1.75 (+0.86 vs. -0.89; paired t-test, p = 0.013), mean weight z-score change per month increased by 0.35 (-0.25 vs. +0.10; Wilcoxon test, p = 0.013), and the incidence of biventricular hypertrophy on ECG decreased (46% vs. 0%; McNemar test, p = 0.031). The change in BNP showed a trend toward a negative correlation with weight z-score change per month (r = -0.531, p = 0.075). In conclusion, BNP, along with other measures of heart failure, decreased following VSD repair, and the change in BNP was most closely correlated with improved weight gain.

Surgical Strategy to Establish a Dual-Coronary System for the Management of Anomalous Left Coronary Artery Origin From the Pulmonary Artery

Optimal repair of anomalous origin of left coronary artery from pulmonary artery (ALCAPA) relies on the creation of a dual-coronary system. If the anomalous coronary arises at a long distance from the aorta, we use various coronary extension techniques to facilitate tension-free implantation. Thirty patients underwent ALCAPA operations using direct coronary transfer (n = 11) or coronary extension techniques (n = 19). Surgical outcomes were analyzed. Median age and weight were 5.7 months (range, 46 days to 5.45 years) and 5.35 kg (range, 3.3 to 15.9 kg). Five patients had concomitant mitral annuloplasty. Mean cardiopulmonary bypass and ischemic times were 108 +/- 38 and 57 +/- 25 minutes. Two patients required intraoperative revision of the implantation. There were three hospital deaths (10%) and no late deaths. Follow-up echocardiograms demonstrated significant improvement postoperatively vs preoperatively in shortening fraction (35% +/- 2% vs 16% +/- 2%, p < 0.00001), ejection fraction (64% +/- 3% vs 32% +/- 4%, p < 0.00001), and mitral regurgitation (11% moderate vs 70% moderate or severe, p = 0.0002). Left ventricular end-diastolic dimension Z-score decreased from 9.1 +/- 0.9 to 1.2 +/- 0.5 (p < 0.00001). Both techniques were equally effective. Two patients underwent reoperation 1 and 12 years postoperatively (coronary artery bypass grafting, 1; mitral repair with coronary angioplasty, 1). Surviving patients remain asymptomatic (p < 0.00001). Dual-coronary system can be established in patients with ALCAPA. Coronary extension implantation techniques have acceptable operative mortality and excellent cardiac recovery and late survival. Although the rate of late coronary occlusion is low, continual ventricular or mitral dysfunction should trigger evaluation of persistent coronary compromise.

Abstracts for the British Congenital Cardiac Association Annual Meeting: The Barbican, London, 24–25 November 2005: Winner of the Young Investigator's Prize: B-Type Natiuretic Peptide (BNP) as a marker of paediatric heart failure

Background: Paediatric heart failure can be difficult to assess. Symptoms vary widely in children who have poor ventricular function on echocardiogram. In addition exercise testing is not possible in young children. BNP has been used in adult heart failure and more recently in paediatrics. We investigated BNP in the setting of a paediatric heart failure and transplantation service to assess its usefulness as a clinical marker of heart failure. Method: Clinical and echocardiographic data were correlated to 126 BNP samples. Patients were 3 weeks to 16 years of age. Left ventricular end-diastolic dimension (LVEDd) was related to normal values for body surface area (z-score). Clinical status was defined using Ross and NYHA scores. Thirty four samples were from patients with normal ventricular function. Of the remainder, most were from patients with idiopathic dilated cardiomyopathy (38), anthracycline cardiotoxicity (15), congenital heart disease (25), viral myocarditis (6) and restrictive cardiomyopathy (6). Analysis: BNP was correlated to parametric data (fractional shortening (FS%)) with pearsons correlation coefficient. For non-parametric data (z-score, NYHA and Ross score), spearmans correlation coefficient was used. Results: Rising BNP levels correlate to deteriorating clinical status with significance to the 0.01 level (Ross and NYHA), Figure 1. There was also correlation, significant to the 0.01 level to FS%, Figure 2. Increasing BNP levels correlated to the increasing LVEDd z-score significant to the 0.05 level. Mean BNP: in ventricular dysfunction 634 pg/ml (S.E.M. = 80), in normal function 11.9 pg/ml (S.E.M. = 1.6). Conclusions: This is the largest study of BNP in paediatric heart failure. The stronger correlation of BNP to NYHA and ROSS than to LVEDD and FS% suggests a useful role in assessment of children with heart failure. We believe it is useful in the outpatient setting particularly when care is shared with general paediatricians. It also appears to be a useful addition in the assessment of heart failure and perhaps timing of transplantation in a specialist centre.