Correlations Between Echocardiographic Systolic and Diastolic Function with Cardiac Catheterization in Biventricular Congenital Heart Patients

Newer echocardiographic techniques may allow for more accurate assessment of left ventricular (LV) function. Adult studies have correlated these echocardiographic measurements with invasive data, but minimal data exist in the pediatric congenital heart population. Purpose of this study was to evaluate which echocardiographic measurements correlated best with LV systolic and diastolic catheterization parameters. Patients with two-ventricle physiology who underwent simultaneous echocardiogram and cardiac catheterization were included. Images were obtained in the four-chamber view. LV systolic echocardiographic data included ejection fraction, displacement, tissue Doppler imaging (TDI) s′ wave, global longitudinal strain, and strain rate (SR) s′ wave. Diastolic echocardiographic data included mitral E and A waves, TDI e′ and a′ waves, and SRe′ and SRa′ waves. E/TDI e′, TDI e′/TDI a′, E/SRe′, and SRe′/SRa′ ratios were also calculated. Catheterization dP/dt was used as a marker for systolic function, and LV end-diastolic pressure (EDP) was used as a marker for diastolic function. Correlations of the echocardiographic and catheterization values were performed using Pearson correlation. Twenty-nine patients were included (14 females, 15 males). Median age at catheterization was 3.4 years (0.04–17.4 years). dP/dt was 1258 ± 353 mmHg/s, and LVEDP was 10.8 ± 2.4 mmHg. There were no significant correlations between catheterization dP/dt and systolic echocardiographic parameters. LVEDP correlated significantly with SRe′ (r = ?0.4, p = 0.03), SRa′ (r = ?0.4, p = 0.03), and E/SRe′ (r = 0.5, p = 0.004). In pediatric congenital heart patients, catheterization dP/dt did not correlate with echocardiographic measurements of LV systolic function. Further studies are needed to determine which echocardiographic parameter best describes LV systolic function in this population. Strain rate analysis significantly correlated with LVEDP. Strain rate analysis should be considered as an alternative method to estimate LVEDP in this patient population.     

Predictors of Long-Term Outcome in Children with Hypertrophic Cardiomyopathy

To date limited data are available to predict the progression to end-stage heart failure (HF) with subsequent death (non-SCD), need for heart transplantation, or sudden cardiac death (SCD) in children with hypertrophic cardiomyopathy (HCM). We aimed to determine predictors of long-term outcome in children with HCM. A total of 112 children (median 14.1, IQR 7.8–16.6 years) were followed up for the median of 6.5 years for the development of morbidity and mortality, including arrhythmic and HF-related secondary end points. HF end point included HF-related death or heart transplant, and arrhythmic end point included resuscitated cardiac arrest, appropriate ICD discharge, or SCD. Overall, 23 (21 %) patients reached the pre-defined composite primary end point. At 10-year follow-up, the event-free survival rate was 76 %. Thirteen patients (12 %) reached the secondary arrhythmic end point, and 10 patients (9 %) reached the secondary HF end point. In multivariate model, prior cardiac arrest (r = 0.658), QTc dispersion (r = 0.262), and NSVT (r = 0.217) were independent predictors of the arrhythmic secondary end point, while HF (r = 0.440), LV posterior wall thickness (r = 0.258), LA size (r = 0.389), and decreased early transmitral flow velocity (r = 0.202) were all independent predictors of the secondary HF end point. There are differences in the risk factors for SCD and for HF-related death in childhood HCM. Only prior cardiac arrest, QTc dispersion, and NSVT predicted arrhythmic outcome in patients aged <18 years. LA size, LV posterior wall thickness, and decreased early transmitral flow velocity were strong independent predictors of HF-related events.     

Reliability of Left Ventricular Hypertrophy by ECG Criteria in Children with Syncope: Do the Criteria Need to be Revised?

In the outpatient setting, children who present with syncope routinely undergo electrocardiograms (ECG). Because of concerns for hypertrophic cardiomyopathy, children with syncope meeting ECG criteria for left ventricular hypertrophy (LVH) will frequently undergo an echocardiogram. Our objectives were to determine whether Davignon criteria for ECG waves overestimate LVH in children presenting with syncope, and to study the usefulness of echocardiography in these children. We hypothesize that the Davignon criteria presently used for interpretation of ECGs overestimate LVH, resulting in unnecessary echocardiography in this clinical setting. The clinical database of The Children’s Hospital of Philadelphia was evaluated from 2002 to 2012 to identify children between 9 and 16 years of age, who presented with non-exercise-induced, isolated syncope. From this group of patients, only those with clear-cut evidence of LVH (by Davignon criteria), who also underwent an echocardiogram, were selected. A total of 136 children with syncope were identified as having LVH by Davignon ECG criteria. None of these patients manifested any evidence of hypertrophic cardiomyopathy, with normal ventricular septum (average Z-score ?0.68 ± 0.84), LV posterior wall (average Z-score ?0.66 ± 1.18) and LV mass (average Z-score 0.52 ± 1.29). No significant correlation was found between summed RV6 plus SV1 and LV mass. Correlations between additional ECG parameters and measures of LVH by echocardiography were similarly poor. In children presenting with syncope and LVH by ECG, there was no evidence of true LVH by echocardiography. We propose that the Davignon ECG criteria for interpreting LVH in children overestimate the degree of hypertrophy in these children and the yield of echocardiography is extremely low.     

Plasma adrenomedullin level in children with obesity: relationship to left ventricular function

Background

Obese children are at increased risk for abnormal cardiac structure and function. Little is known about adrenomedullin (AM), a cytokine produced in various organs and tissues, as a biomarker of cardiac hypertrophy in obese children. This study aimed to assess the plasma AM levels in a cohort of obese children and its relationship to left ventricular (LV) functions.

Methods

The study included 60 obese children and 60 non-obese children matched for age and gender as control group. Blood pressure, serum lipid profile, fasting glucose, insulin and plasma AM and the homeostatic model assessment of insulin resistance (HOMA-IR) were measured. Cardiac dimensions and LV functions were assessed using conventional echocardiography.

Results

Compared to control subjects, obese children had higher blood pressure (P = 0.01), insulin (P = 0.001), HOMA-IR (P = 0.001), and AM (P = 0.001). Moreover, obese children had higher LV mass index (LVMI) (P = 0.001), indicating LV hypertrophy; prolonged isovolumic relaxation times (P = 0.01), prolonged mitral deceleration time (DcT) (P = 0.01) and reduced ratio of mitral E-to-mitral A-wave peak velocity (P = 0.01), indicating LV diastolic dysfunction. Laboratory abnormalities were only present in children with LV hypertrophy. In multivariate analysis in obese children with LV hypertrophy, AM levels were positively correlated with LVMI [odds ratio (OR) 1.14, 95% confidence interval (Cl) 1.08–1.13, P = 0.0001] and mitral DcT (OR 2.25, 95% CI 1.15–2.05, P = 0.01) in the presence of higher blood pressure and HOMA-IR. A cut-off value of AM at 52 pg/mL could differentiate obese children with and without left ventricular hypertrophy at a sensitivity of 94.32% and specificity of 92.45%.

Conclusions

Plasma AM levels may be elevated in obese children particularly those with LV hypertrophy and is correlated with higher blood pressure and insulin resistance. Measurement of plasma AM levels in obese children may help to identify those at high risk of developing LV hypertrophy and dysfunction.

     

Comparison of Immune Profiles in Fetal Hearts with Idiopathic Dilated Cardiomyopathy,Maternal Autoimmune-Associated Dilated Cardiomyopathy and the Normal Fetus

The etiology of idiopathic dilated cardiomyopathy (iDCM) remains unknown. Immune therapies have improved outcome in fetuses with DCM born to mothers with autoimmune disease (aDCM). The purpose of this retrospective study was to compare the myocardial B and T cell profiles in fetuses and neonates with idiopathic DCM (iDCM) versus autoimmune-mediated DCM (aDCM) and to describe the normal cell maturation within the human fetal myocardium. Of 60 fetal autopsy cases identified from institutional databases, 10 had aDCM (18–38 weeks), 12 iDCM (19–37 weeks) and 38 had normal hearts (11–40 weeks). Paraffin-embedded myocardium sections were stained for all lymphocyte (CD45), B cells (CD20, CD79a), T cells (CD3, CD4, CD7, CD8) and monocyte (CD68) surface markers. Two independent, blinded cell counts were performed. Normal hearts expressed all B and T cell markers in a bimodal fashion, with peaks at 22 and 37 weeks of gestation. The aDCM cohort was most distinct from normal hearts, with less overall T cell markers [EST ?9.1 (2.6) cells/mm², p = 0.001], CD4 [EST ?2.0 (0.6), p = 0.001], CD3 [EST ?3.9 (1.0), p < 0.001], CD7 [EST ?3.0 (1.1), p = 0.01] overall B cell markers [EST ?4.9 (1.8), p = 0.01] and CD79a counts [EST ?2.3 (0.9), p = 0.01]. The iDCM group had less overall B cell markers [EST ?4.0 (1.8), p = 0.03] and CD79a [EST ?1.7 (0.9), p = 0.05], but no difference in T cell markers. Autoimmune-mediated DCM fetuses have less B and T cell markers, whereas iDCM fetuses have less B cell markers compared with normal fetal hearts. The fetal immune system may play a role in the normal development of the heart and evolution of dilated cardiomyopathy.     

Decline of Systolic and Diastolic 2D Strain Rate During Follow-Up of HLHS Patients After Fontan Palliation

Accurate assessment of ventricular function is particularly important in children with hypoplastic left heart syndrome (HLHS) after completion of the total cavopulmonary connection (TCPC). For this purpose, two-dimensional speckle tracking (2DST) is a promising technique as it does not depend on the angle of insonation or the geometry of the ventricle. The objective of this study was to assess changes in systolic and diastolic right ventricular (RV) function within a 5-year follow-up period of HLHS patients after TCPC using conventional and 2DST echocardiography. RV fractional area change (RVFAC), tricuspid annular plane systolic excursion (TAPSE), E/A, E/e′ and 2DST parameters [global longitudinal peak systolic strain (GS) and strain rate (GSRs), global strain rate in early (GSRe) and late (GSRa) diastole] of 40 HLHS patients were compared at 1.6 and at 5.1 years after TCPC. RVFAC, E/A, E/e′ and GS did not change, whereas TAPSE (13.7 ± 3.2 vs. 10.5 ± 2.4 mm/m², p < 0.001), GSRs (?1.56 ± 0.28 vs. ?1.35 ± 0.31 1/s, p < 0.001), GSRe (2.22 ± 0.49 vs. 1.96 ± 0.44 1/s, p = 0.004) and GSRa (1.19 ± 0.39 vs. 0.92 ± 0.39 1/s, p < 0.001) decreased significantly. Systolic and diastolic RV function parameters of HLHS patients decreased from 1.6 to 5.1 years after TCPC in our patients. Changes in global strain rate parameters may be signaling early RV dysfunction that is not detectable by traditional echocardiography. Further study is needed to verify this and to determine whether these changes are clinically relevant.     

Citation classics in pediatrics: a bibliometric analysis

Background

Citation analysis provides insights into the history and developmental trajectory of scientific fields. Our objective was to perform an analysis of citation classics in the journals of pediatric specialty and to examine their characteristics.

Methods

Initially, all the journals listed under the category of pediatrics (n = 120) were identified using Journal Citation Reports. Web of science database was then searched (1950–2016) to select the top-100 cited articles in the above identified pediatric journals. The top-100 cited article were categorized according the study design, sub-specialty, country, institutional affiliation, and language.

Results

The top-100 articles were published in 18 different journals, with Pediatrics having the highest numbers (n = 40), followed by The Journal of Pediatrics (n = 17). The majority (n = 62) of classics were published after 1990. The most cited article had citation count of 3516 and the least cited had a citation count of 593. The USA (n = 71) was the most commonly represented country, and 60 institutions contributed to 100 articles. Fifteen authors contributed to more than one classic as first or second author. Observational study (n = 55) was the commonest study design across all decades, followed by reviews (n = 12), scale development studies (n = 11), and guidelines (n = 11). Among the pediatric sub-specialties, growth and development articles were highly cited (n = 24), followed by pediatric psychiatry and behavior (n = 21), endocrinology (n = 15), and neonatology (n = 12).

Conclusions

The top-100 cited articles in pediatrics identify the impactful authors, journals, institutes, and countries. Observational study design was predominant—implying that inclusion among citation classics is not related to soundness of study design.

     

Assessment of cardiac function in infants with transposition of the great arteries after surgery: comparison of two methods

Background

Assessment of cardiac function is crucial in pediatric patients undergoing cardiovascular surgery, monitoring cardiac output and changing hemodynamic conditions during surgery accordingly is important to improve post-surgical outcome. We aimed to measure cardiac index (CI) and maximal rate of the increase of left ventricular pressure dp/dt(max) with the pressure recording analytic method (PRAM, MostCare^®) and compared it with transthoracic echocardiographic cardiac index estimation in infants with transposition of the great arteries (TGA) undergoing surgical correction.

Methods

We enrolled 74 infants with TGA consecutively into this study. CI and dp/dt(max) were measured with PRAM and echocardiography at 0, 4, 8, 12, 24 and 48 h postoperatively. Blood brain natriuretic peptide (BNP) and blood lactate (Lac) were measured at baseline and after operation.

Results

The median age at surgery was 13 days (range 1–25 days) with an average weight of 3.24 kg (range 2.31–4.17 kg). CI estimated by PRAM was 1.11 ± 0.12 L/min/m² (range 0.69–1.36) and by Doppler echocardiography was 1.13 ± 0.13 L/min/m² (range 0.76–1.40). dp/dt(max) estimated by PRAM was 1.31 ± 0.03 mmHg/s (range 1.23–1.43) and by Doppler echocardiography was 1.31 ± 0.04 L/min/m² (range 1.25–1.47). CI (r = 0.817, P < 0.001) and dp/dt(max) (r?=?0.794, P?<?0.001) measured by two methods were highly correlated with a linear relation. Blood BNP and lactate increased to the highest level at 8–12 h post-operatively.

Conclusions

In the early post-operative period, PRAM provides reliable estimates of cardiac index and dp/dt(max) value compared with echocardiographic measurements. PRAM through mostcare^® is a reliable continuous monitoring method for peri-operative management in children with congenital heart disease.

     

The Use of Speckle Tracking Echocardiography for Early Detection of Myocardial Dysfunction in Patients with Duchenne Muscular Dystrophy

Cardiac complications are the leading cause of death in patients with Duchenne muscular dystrophy (DMD). Two-dimensional echocardiography is the current standard for monitoring of LV systolic function in these patients, but it might not detect early systolic dysfunction. The current study examined the use of speckle tracking echocardiography (STE) to detect early signs of cardiac dysfunction in DMD patients. A retrospective review of charts and offline strain analysis of transthoracic echocardiographic studies of DMD patients at our institution from April 2014 to January 2015 were performed and compared to age-matched healthy male subjects. Nineteen DMD patients (age range 12.6 ± 3.1 years) with normal ejection fraction and shortening fraction were compared with sixteen controls. The global circumferential strain was lower in DMD patients compared with controls (?14.7 ± 4.7 vs. ?23.1 ± 2.9 %, respectively, p value: 0.001). Circumferential strain measured at basal, mid-ventricular and apical parasternal short-axis views was lower in DMD patients compared with controls. Segmental circumferential strain was lower in DMD patients in most segments compared with controls. The global longitudinal strain was lower in DMD patients compared with controls (?13.6 ± 5 vs. ?18.8 ± 3 %, respectively, p value: 0.001). Segmental longitudinal strain measured in various segments was lower in DMD patients compared with controls. DMD patients can have occult cardiovascular dysfunction as shown by reduction in circumferential and longitudinal strain measurements with STE despite normal standard echocardiographic parameters. The clinical significance of early detection of cardiac dysfunction in these patients warrants further studies.     

Impact of Interatrial Communication on Left Ventricle Performance in Patients with Significant Post-tricuspid Shunt

Infants with post-tricuspid valve shunts (PTS) may benefit from interatrial communication (IAC). The effect of IAC on left ventricular (LV) performance in these patients was studied. IAC was documented prospectively in 55 patients with PTS. Clinical status, echocardiographic dimensions of LV, mitral inflow Doppler, tissue Doppler velocities and time intervals were measured. Creatinine kinase (CK), CKMB, troponin-I and NT pro-brain natriuretic peptide (NT pro-BNP) were measured. Patients were divided into four groups: (A) PTS but no IAC (n = 32); (B) PTS and IAC (n = 23); (C) VSD but no IAC (n = 16); and (D) VSD and IAC (n = 19). Group A had more frequent mitral regurgitation (p = 0.041), larger mitral annulus (1.80 vs. 1.30 cm, p < 0.0001) and larger LV systolic and diastolic dimensions (2.01 vs. 1.40 and 3.28 vs. 2.35 cm, p < 0.001) than group B. The E-wave deceleration time tended to be longer in group A (121.0 vs. 106.8 ms, p = 0.06). By tissue Doppler, group A had E′- and S-waves significantly taller (15.51 vs. 13.14 and 7.69 vs. 6.72 cm, p = 0.04 and p = 0.005, respectively) than group B. Also, NT pro-BNP was significantly higher in group A (1116.15 vs. 458.73 pg/ml, p = 0.028). Group C had significant larger mitral z-score values (1.2 vs. 0.01, p < 0.001), larger LV diameter z-score (p = 0.001) and higher NT pro-BNP level (1477.37 vs. 451.66 pg/ml, p = 0.001) than group D. There was no significant difference in the clinical status between the groups. In children with PTS, the presence of IAC could be beneficial. Their echocardiographic parameters and biomarker show better systolic and diastolic LV performance.     

The Impact of Aortic Valve Replacement on Left Ventricular Remodeling in Children

There are scant data in pediatrics on the optimal timing for aortic valve repair (AVR). This study assesses the midterm response to AVR and possible predictors of poor outcome. From 2001 to 2006, 41 patients had greater than 3-month follow-up after AVR for aortic insufficiency, aortic stenosis, or both. Pre-, peri-, and post-operative data were collected, including demographics and clinical symptoms. Two reviewers measured echocardiographic parameters from the pre-operative and latest follow-up echocardiograms. Ventricular dimensions were indexed to body surface area (z-score). Median age at AVR was 13 years with 83 % having a Ross operation. The average left ventricular end-diastolic dimension pre-op, z-score of +1.3, significantly decreased at last follow-up to a mean z-score of ?0.1 (p < 0.001). Similarly the indexed LV mass decreased from +3.9 to +0.5 (p < 0.001). There was no significant correlation between the presence of pre-op symptoms and the presence of post-op LV dilatation, hypertrophy, or dysfunction. In the subset of patients (7/41) with persistent LV dysfunction at last follow-up, there was a significant correlation with pre-op LV dilatation as assessed by both LVEDD (p = 0.02) and LVESD (p = 0.05). Children demonstrate significant reverse remodeling after AVR. Pre-op LV dilatation may predict patients with persistent LV dysfunction post-AVR. Symptoms are less useful in children, suggesting the need for more objective data for functional assessment.     

Early Ventricular Dysfunction After Anthracycline Chemotherapy in Children

The aim of this study was to determine the effect of induction dose of anthracycline chemotherapy on the biventricular function among children with acute hematological malignancies (AHM) using tissue Doppler imaging (TDI) and 2D speckle tracking echocardiography (2D-STE). Thirty pediatric patients with AHM and a mean age of 9.24 ± 4.14 years performed conventional echocardiography, TDI and 2D-STE. After induction chemotherapy, the RV showed mainly a diastolic alteration in its function manifested in significant reduction in the tricuspid TDI-derived E′ and E′/A′ ratio compared with the baseline (20.40 ± 3.81 vs. 17.47 ± 3.87 cm/s, p = 0.001, 1.29 ± 0.27 vs. 1.03 ± 0.37, p < 0.01, respectively), while the TDI-derived RV MPI and isovolumetric relaxation time of RV were significantly increased (0.32 ± 0.06 vs. 0.36 ± 0.08, p < 0.01, 24.73 ± 8.62 vs. 28.47 ± 11.51 ms, p < 0.05, respectively). The LV showed post-chemotherapy mainly an alteration in its longitudinal systolic function in the form of a reduction in MAPSE (13.61 ± 2.00 vs. 11.95 ± 1.75 mm; p < 0.001), TDI-derived systolic velocity of lateral mitral annulus (10.98 ± 2.34 vs. 10.03 ± 1.83 cm/s, p < 0.05), 2D-STE-derived global longitudinal strain (?21.58 ± 2.54 vs. ?19.18 ± 3.59 %, p = 0.001) and 2D-STE-derived global longitudinal strain rate (?1.76 ± 0.22 vs. 1.55 ± 0.29 1/s, p < 0.05), with preservation of LV diastolic function when compared to baseline. TDI and 2D-STE could be used for early detection of anthracycline-induced cardiotoxicity in the pediatric age group. Early after induction chemotherapy, the RV develops mainly diastolic dysfunction, while the LV showed a relative longitudinal systolic impairment.     

Predictors of Early Extubation After Pediatric Cardiac Surgery: A Single-Center Prospective Observational Study

This prospective, observational, single-center study aimed to determine the perioperative predictors of early extubation (<24 h after cardiac surgery) in a cohort of children undergoing cardiac surgery. Children aged between 1 month and 18 years who were consecutively admitted to pediatric intensive care unit after cardiac surgery for congenital heart disease between January 2012 and June 2014. Ninety-nine patients were qualified for inclusion during the study period. The median duration of mechanical ventilation was 20 h (range 1–480), and 64 patients were extubated within 24 h. Four of them failed the initial attempt at extubation, and the success rate of early extubation was 60.6 %. Older patient age (p = .009), greater body weight (p = .009), absence of preoperative pulmonary hypertension (p = .044), lower RACHS-1 category (OR, 3.8; 95 % CI 1.35–10.7; p < .05), shorter cardiopulmonary bypass (p = .008) and cross-clamp (p = .022) times, lower PRISM III-24 (p < .05) and PELOD (p < .05) scores, lower inotropic score (p < .05) and vasoactive-inotropic score (p < .05), and lower number of organ failures (OR, 2.26; 95 % CI 1.30–3.92; p < .05) were associated with early extubation. Our study establishes that early extubation can be accomplished within the first 24 h after surgery in low- to medium-risk pediatric cardiac surgery patients, especially in older ones undergoing low-complexity procedures. A large prospective multiple institution trial is necessary to identify the predictors and benefits of early extubation and to facilitate defined guidelines for early extubation.     

Serial evaluation of myocardial function using the myocardial performance index in Kawasaki disease

Background

Kawasaki disease (KD) is known as systemic vasculitis, and more than half of the patients with KD have myocarditis, which can induce ventricular dysfunction. In this study, we evaluate left ventricular (LV) dysfunction in patients with KD based on the myocardial performance index (MPI) using pulse Doppler (PD) and tissue Doppler imaging (TDI), from the acute to convalescent phases.

Methods

We retrospectively studied 89 children diagnosed with KD from January 2010 to August 2012. We assessed the presence of coronary artery lesions (CALs) and the LV ejection fraction, PD-MPI, and TDI-MPI at diagnosis, and 2, 14, and 56 days after intravenous immunoglobulin (IVIG) treatment. We enrolled 70 healthy children as a control group.

Results

The ejection fraction in patients with KD at diagnosis (67.3 ± 0.9%) was lower than that in the control group (69.8 ± 0.8%, P = 0.035), and the LV TDI-MPIs for patients with KD at diagnosis (0.49 ± 0.01) and 2 days after IVIG treatment (0.48 ± 0.01) were higher than those in the control group (0.45 ± 0.01, P = 0.002, P = 0.033, respectively). No significant differences were found in the LV dysfunction between the patients with complete and incomplete KD. Septal TDI-MPIs in patients with KD with CAL at diagnosis (0.52 ± 0.02) were higher than those in patients with KD without CAL (0.47 ± 0.01, P = 0.019).

Conclusions

Transient LV dysfunction occurred in patients with complete and incomplete KD in the acute stage. In patients with KD with CAL at diagnosis, the LV dysfunction was more prominent. The PD-MPI and TDI-MPI are useful parameters for assessing LV function in patients with KD.

     

Outcomes in Patients with Persistent Ventricular Dysfunction After Stage I Palliation for Hypoplastic Left Heart Syndrome

We sought to describe the clinical course for patients with hypoplastic left heart syndrome and persistent ventricular dysfunction and identify risk factors for death or transplantation before stage II palliation. 138 children undergoing stage I palliation from 2004 to 2011 were reviewed. Twenty-two (16 %) patients (seven Hybrid, 15 Norwood) with two consecutive echocardiograms reporting at least moderate dysfunction were included and compared to case-matched controls. Eleven of the 22 patients with dysfunction (50 %) underwent stage II, seven (32 %) were transplanted, and four (18 %) died prior to stage II. Of the patients who survived to hospital discharge (n = 17) following stage 1, 14 (82 %) required readmission for heart failure (HF) compared to only two (10 %) for controls (p < 0.001). Among patients with ventricular dysfunction, there was an increased use of ACE inhibitors or beta-blockers (82 vs. 25 %; p = 0.001), inotropes (71 vs. 15 %; p = 0.001), ventilation (58 vs. 10 %; p = 0.001), and ECMO (29 vs. 0 %; p = 0.014) for HF management post-discharge when compared to controls. There was a lower heart transplant-free survival at 7 months in patients with dysfunction compared to controls (50.6 vs. 90.9 %; p = 0.040). ECMO support (p = 0.001) and duration of inotropic support (p = 0.04) were significantly associated with death or transplantation before stage II palliation. Patients with ventricular dysfunction received more HF management and related admissions. Longer inotropic support should prompt discussion regarding alternative treatment strategies given its association with death or transplant.     

Relationship of Right Ventricular Size and Function with Respiratory Status in Duchenne Muscular Dystrophy

The relationship between pulmonary function and right ventricle (RV) in Duchenne muscular dystrophy (DMD) has not been evaluated. Using cardiac magnetic resonance (CMR), we describe the relationship of RV size and function with spirometry in a DMD cohort. Fifty-seven boys undergoing CMR and pulmonary function testing within 1 month at a single center (2013–2015) were enrolled. Comparisons of RV ejection fraction (RVEF) and end-diastolic volume index (RVEDVI) were made across categories of percent forced vital capacity (FVC%), and relationships were assessed. Mean age was 15.5 ± 3.5 years. Spirometry and CMR were performed within 3.9 ± 4.1 days. Median FVC% was 92.0 % (67.5–116.5 %). Twenty-three (40 %) patients had abnormal FVC% (<80 %) of which 13 (57 %) had mild (FVC% 60–79 %), 6 (26 %) had moderate (FVC% 40–59 %), and 4 (17 %) had severe (FVC <40 %) reductions. Mean RVEF was 58.3 ± 3.7 %. Patients with abnormal FVC% were older and had lower RVEF and RVEDVI. Both RVEF and RVEDVI were significantly associated with FVC% (r = 0.31, p = 0.02 and r = 0.39, p = 0.003, respectively). In a large DMD cohort, RVEF and RVEDVI were related to FVC%. Worsening respiratory status may guide monitoring of cardiac function in these patients.     

Airway foreign bodies in pediatric patients: anatomic location of foreign body affects complications and outcomes

Background

Airway foreign bodies (FB) are a common medical emergency within the pediatric population. While deaths are not uncommon, the in-hospital mortality rates and correlation with anatomic location of the airway foreign body have not been previously reported.

Methods

The KID database was reviewed for 2003, 2006, 2009, and 2012 for pediatric patients with a discharge diagnosis of airway foreign body using ICD-9 codes (933.1, 934.x).

Results

11,793 patients, ages 0–17, were found to have an airway FB. Of patients admitted for airway FB 21.2 % required mechanical ventilation during their hospitalization, and the overall mortality rate was 2.5 %. Location of the airway FB was dependent on age (p < 0.01). Use of mechanical ventilation was dependent on the location of the airway FB (p < 0.01) and being transferred from another hospital (OR 2.59, p < 0.01). Univariate analysis demonstrated differences in in-hospital mortality based on location (p < 0.01), use of a ventilator during hospitalization (OR 24.4, p < 0.01), and transfer from another hospital (OR 2.11, p < 0.01).

Conclusions

The in-hospital mortality rate for airway foreign bodies is 2.5 %. The anatomic location of airway FB in pediatric patients varies by age, and affects the need for mechanical ventilation and in-hospital mortality.

     

A comparison of the characteristics and precision of needle driving for right-handed pediatric surgeons between right and left driving using a model of infant laparoscopic diaphragmatic hernia repair

Purpose

We compared the characteristics and precision of right and left needle driving for right-handed pediatric surgeons using a laparoscopic diaphragmatic repair model.

Methods

Eighteen right-handed pediatric surgeons performed three needle driving maneuvers using both hands. We evaluated the required time and conducted an image analysis. The total path length, velocity, and acceleration of the needle driving were also evaluated.

Results

Obtained results show the findings for the required time (s, Rt 310.78 ± 148.93 vs. Lt 308.61 ± 122.53, p = 0.93), sum of needle driving balances (mm, Rt 5.23 ± 2.44 vs. Lt 5.05 ± 3.17, p = 0.83), the gap of the needle driving interval (Rt 1.2 ± 0.93 vs. Lt 2.17 ± 1.67, p = 0.04), total path length (mm, Rt 594.03 ± 205.29 vs. Lt 1641.07 ± 670.68, p < 0.01), and average velocity (mm/s, Rt 1.92 ± 0.54 vs. Lt 5.3 ± 1.39, p < 0.01).

Conclusion

For right-handed pediatric surgeons, left needle driving showed almost same quality of right needle driving as regarding the precision. But left needle driving also showed too fast but not economical movement unfortunately, implying rough and risky forceps manipulation. Non-dominant hand training is necessary to avoid organ injury.

     

Clinical Factors Associated with Dose of Loop Diuretics After Pediatric Cardiac Surgery: Post Hoc Analysis

A post hoc analysis of a randomized controlled trial comparing the clinical effects of furosemide and ethacrynic acid was conducted. Infants undergoing cardiac surgery with cardiopulmonary bypass were included in order to explore which clinical factors are associated with diuretic dose in infants with congenital heart disease. Overall, 67 patients with median (interquartile range) age of 48 (13–139) days were enrolled. Median diuretic dose was 0.34 (0.25–0.4) mg/kg/h at the end of postoperative day (POD) 0 and it significantly decreased (p = 0.04) over the following PODs; during this period, the ratio between urine output and diuretic dose increased significantly (p = 0.04). Age (r ?0.26, p = 0.02), weight (r ?0.28, p = 0.01), cross-clamp time (r 0.27, p = 0.03), administration of ethacrynic acid (OR 0.01, p = 0.03), and, at the end of POD0, creatinine levels (r 0.3, p = 0.009), renal near-infrared spectroscopy saturation (?0.44, p = 0.008), whole-blood neutrophil gelatinase-associated lipocalin levels (r 0.30, p = 0.01), pH (r ?0.26, p = 0.02), urinary volume (r ?0.2755, p = 0.03), and fluid balance (r 0.2577, p = 0.0266) showed a significant association with diuretic dose. At multivariable logistic regression cross-clamp time (OR 1.007, p = 0.04), use of ethacrynic acid (OR 0.2, p = 0.01) and blood pH at the end of POD0 (OR 0.0001, p = 0.03) was independently associated with diuretic dose. Early resistance to loop diuretics continuous infusion is evident in post-cardiac surgery infants: Higher doses are administered to patients with lower urinary output. Independently associated variables with diuretic dose in our population appeared to be cross-clamping time, the administration of ethacrynic acid, and blood pH.     

Evaluation of Cardiovascular Changes in Children with BAVs

The objectives of this study were to investigate left ventricular (LV) function, aortic dilation, and atherosclerosis in children with mildly deteriorated isolated bicuspid aortic valve (BAV) function using echocardiographic studies and biochemical markers of atherosclerosis and to correlate results with normal children. Biochemical analyses indicating cardiovascular risk of atherosclerosis and vascular changes in the aorta in relation to BAV were performed in 41 children aged 5–15 years old with isolated BAV and in 25 children with tricuspid aortic valves. Evaluations of aortic valve structures and functions; examinations of the LV M-mode and ascending aorta Doppler; and measurements of the LV Tei index (MPI), propagation velocity, ascending aorta at four levels, and carotid intima–media thickness (CIMT) were performed. There were no statistically significant differences in CIMTs, plasma matrix metalloproteinase-9, tissue metalloproteinase inhibitor-1 levels, or other biochemical parameters indicating cardiovascular risk or atherosclerosis between study and control groups. Deterioration of LV function, which could not be seen with M-mode echocardiography, was evident by MPI. MPI values in the study versus control groups were 0.46 ± 0.080 versus 0.40 ± 0.086 (p < 0.05). Diameters of the aorta in the study and control groups were 19.7 ± 4.7 and 17.2 ± 2.8 mm (p < 0.05) at the sinotubular junction level and 20.6 (14.4–40.5) and 18.3 (12.4–24) mm at the ascending aorta level (p < 0.05). Increased aortic valve insufficiency was related to increased aortic diameter. No sign of atherosclerosis was detected in children with BAV. Deterioration of LV function was seen using MPI, and aortic dilation was related to the severity of aortic valve insufficiency.