Relationship of Left Ventricular Mass to Lean Body Mass in the Obese Pediatric Population.

Our primary aim was to investigate the relationship between LVM and anthropometric measures including lean body mass (LBM) in obese pediatric subjects compared to normal weight controls. A retrospective chart review identified subjects 2-18 years old who were normotensive and had normal echocardiograms between 1995 and 2020 at Boston Children's Hospital. LVM was calculated with the 5/6 area length rule from 2D echocardiograms. LBM was calculated with equations derived from dual-energy X-ray absorptiometry. Of the 2217 subjects who met inclusion criteria, 203 were obese and 2014 had normal weight. The median age was 11.9 (2.0-18.9); 46% were female. The median LVM was 94.5 g (59.3-134.3) in obese subjects vs. 78.0 g (51.5-107.7) in controls. The median LBM was 37.2 kg (18.9-50.6) in obese subjects vs. 30.5 kg (17.6-40.8) in controls. In control and obese subjects, LBM had the strongest correlation to LVM (R2 0.86, P < 0.001) and (R2 0.87, P < 0.001), respectively. There was at most a modest correlation between tissue Doppler velocity z-scores and LV mass, and the largest was Septal E' z-score in obese subjects (r = - 0.31, P = 0.006). In this cohort, LBM was found to have the strongest relationship to LVM in obese subjects. The largest correlation between tissue Doppler velocity z-scores and LV mass was Septal E' z-score. Future studies will evaluate which measurements are more closely aligned with clinical outcomes in obese children.

Nutrition for critically ill children with congenital heart disease.

Children with congenital heart disease often require admission to the cardiac intensive care unit at some point in their lives, either after elective surgical or catheter-based procedures or during times of acute critical illness. Meeting both the macronutrient and micronutrient needs of children in the cardiac intensive care unit requires complex decision-making when considering gastrointestinal perfusion, vasoactive support, and fluid balance goals. Although nutrition guidelines exist for critically ill children, these cannot always be extrapolated to children with congenital heart disease. Children with congenital heart disease may also suffer unique circumstances, such as chylothoraces, heart failure, and the need for mechanical circulatory support, which greatly impact nutrition delivery. Guidelines for neonates and children with heart disease continue to be developed. We provide a synthesized narrative review of current literature and considerations for nutrition evaluation and management of critically ill children with congenital heart disease.

Transcatheter Ductus Arteriosus Stenting for Acute Pediatric Pulmonary Arterial Hypertension is Associated with Improved Right Ventricular Echocardiography Strain.

BACKGROUND: Interventional therapies for severe pulmonary arterial hypertension (PAH) can provide right ventricular (RV) decompression and preserve cardiac output. Transcatheter stent placement in a residual ductus arteriosus (PDA) is one potentially effective option in critically ill infants and young children with PAH. We sought to assess recovery of RV function by echocardiographic strain in infants and young children following PDA stenting for acute PAH. METHODS: Retrospective review of patients < 2 years old who underwent PDA stenting for acute PAH. Clinical data were abstracted from the electronic medical record. RV strain (both total and free wall components) was assessed from echocardiographic images at baseline and 3, 6, and 12 months post-intervention, as well as at last echocardiogram. RESULTS: Nine patients underwent attempted ductal stenting for PAH. The median age at intervention was 38 days and median weight 3.7 kg. One-third (3of 9) of patients had PAH associated with a congenital diaphragmatic hernia. PDA stents were successfully deployed in eight patients. Mean RV total strain was - 14.9 ± 5.6% at baseline and improved to - 23.8 ± 2.2% at 6 months post-procedure (p < 0.001). Mean free wall RV strain was - 19.5 ± 5.4% at baseline and improved to - 27.7 ± 4.1% at 6 months (p = 0.002). Five patients survived to discharge, and four patients survived 1 year post-discharge. CONCLUSION: PDA stenting for severe, acute PAH can improve RV function as assessed by strain echocardiography. The quantitative improvement is more prominent in the first 6 months post-procedure and stabilizes thereafter.

Predictors of Mediastinal Exploration While on Extracorporeal Membrane Oxygenation After Pediatric Cardiac Surgery.

Cardiac surgical patients requiring extracorporeal membrane oxygenation (ECMO) are at increased risk for hemorrhage due to necessary anticoagulation, in-situ cannulas, and disturbed hemostasis. We performed a retrospective, cross-sectional study of patients 0-18 years old in our cardiac intensive care unit (CICU) cannulated to ECMO within 48 h of cardiopulmonary bypass. The 69 patients included in the study were divided into three analysis groups based on serial chest tube output per hour: no bleeding (NB) on admission to the CICU (21/69, 30%), bleeding stopped (BS) with medical management (26/69, 38%), bleeding requiring emergent mediastinal exploration (BME) (22/69, 32%). The NB group had a more favorable coagulation profile upon admission to the CICU (PTT 53 s NB, 105 s BS, 83 s BME p < 0.001, ACT 169 s NB, 225 s BS, 211 s BME, p =0.013). Only chest tube output during the first three postcannulation hours remained associated with the need for mediastinal exploration by multivariable analysis. An average chest-tube output of 11.6 mL/kg/h during the first three hours had the highest percentage of patients classified correctly (84%) for requiring mediastinal exploration during their ECMO run (sensitivity 91%, specificity 81%).

Anti-hypertensive treatment in the immediate post-operative period and 1 year after pediatric heart transplantation.

Hypertension is a known complication of pediatric heart transplantation. We sought to identify factors associated with anti-hypertensive use in pediatric heart transplant recipients immediately post-transplant and oral anti-hypertensive use at discharge and 1-year post-transplant. Retrospective chart review was conducted of patients ≤18 years who underwent heart transplantation at two major heart transplant centers between August 1, 2009 and December 31, 2017 with ≥1-year follow-up. Exclusion criteria included re-transplant, multi-organ recipients, survival <1 year, and comorbidities associated with hypertension. Anti-hypertensive use was recorded during initial ICU stay, at discharge, and 1-year post-transplant. Univariate and multivariate analyses determined associations of demographic and diagnostic factors and need for anti-hypertensives. There were 188 patients that met inclusion criteria. Anti-hypertensive infusions were required in the ICU post-transplant in 46 patients (24.5%) for a median of 3 days (1-21 days). Oral anti-hypertensives were required in 58 patients (30.9%) at discharge and 1-year post-transplant. Anti-hypertensive infusion in the ICU post-transplant was associated with donor-to-recipient weight ratio. Oral anti-hypertensive use at discharge was associated with weight ratio and pretransplant VAD use, and at 1-year, post-transplant was associated with age at transplant, steroid use at discharge, and oral anti-hypertensive use at discharge. Hypertension is common immediately following and 1-year post-transplant. Weight ratio was the only independent predictor of anti-hypertensive use in the early post-transplant period, whereas VAD use was also associated with anti-hypertensive use at discharge. Anti-hypertensive use 1-year post-transplant was not associated with those factors, but rather with age at transplant and steroid use.

Technical performance score as predictor for post-discharge reintervention in valve-sparing tetralogy of Fallot repair.

Recognition of late problems following repair of tetralogy of Fallot (TOF) with a transannular patch has stimulated modifications to preserve pulmonary valve (PV) function. This study assesses the ability of technical performance score (TPS) to determine the need for post-discharge reinterventions (RIs) in valve-sparing TOF repair. We retrospectively reviewed 157 patients following valve-sparing repair of TOF from 2007-2012. We assigned TPS as Class 1 (optimal), Class 2 (adequate), or Class 3 (inadequate) based on discharge echo and clinical criteria. Preoperative, discharge, and follow-up PV Z scores and post-discharge RIs were documented. Reasons for Class 2 or 3 designation were right ventricular outflow tract (RVOT) gradient in 52, pulmonary regurgitation in 13, residual ventricular septal defects in 7, both RVOT gradient and ventricular septal defects in 13, and both RVOT gradient and pulmonary regurgitation in 37 patients. Median follow-up was 19.6 (range: 0.1-86.1) months. Class 3 patients had a significantly longer median intensive care unit and hospital stay compared with Class 1 (3 vs 2 days [P = 0.015] and 7 vs 5 days [P < 0.001], respectively). Post-discharge RIs were significantly lower in Class 1 vs Class 2 and Class 3 (P = 0.003). Class 1 patients had significantly larger PV Z scores compared with Class 2 or Class 3 patients (P < 0.001). TPS is associated with post-discharge RI rate after valve-sparing TOF repair. Preoperative PV Z score is highly correlated with Class I TPS. Patient selection based on preoperative PV Z scores may help determine if valve-sparing approach is appropriate, thus minimizing the need for RIs.

Safety of cardiac catheterization at a center specializing in the care of patients with pulmonary arterial hypertension.

Cardiac catheterization is important for the management of patients with pulmonary arterial hypertension (PAH). It is used for diagnosis, assessment, and monitoring of PAH patients, as well as to perform interventions such as balloon atrial septostomy and coil embolization of collateral vessels. Although reports on the risks of catheterization in PAH patients are scarce, many centers hesitate to perform these procedures in such fragile patients. We performed a retrospective chart review of all cardiac catheterizations performed in PAH patients over 10 years at our pulmonary hypertension center. Demographic, hemodynamic, and outcome data were collected. Complication rates were determined, and multivariate proportional hazards modeling was performed to identify predictors of catheterization-related complications. There were 1,637 catheterizations performed in 607 patients over 10 years. Pediatric patients accounted for 50% of these cases, 48% were performed in patients with idiopathic PAH, and 49% were performed under general anesthesia. While the overall complication rate was 5.7%, the rate of major complications was only 1.2% ([Formula: see text]). Although there were 8 deaths during the admission following catheterization, only 4 of these were related to the procedure, yielding a catheterization-related mortality of 0.2%. In conclusion, when performed at a pulmonary hypertension center with expertise in the care of PAH patients, cardiac catheterization is associated with low complication rates and mortality, and it should remain an important tool in the management of these patients.

Effectiveness and safety of inhaled treprostinil for the treatment of pulmonary arterial hypertension in children.

The introduction of prostanoid therapy has revolutionized the treatment of pulmonary arterial hypertension (PAH). However, continuous intravenous prostacyclin infusion poses significant risks and challenges, particularly in children. Inhaled treprostinil has been shown to be safe and efficacious in adults. This study describes the safety and efficacy of inhaled treprostinil in children with PAH. A retrospective analysis of 29 children treated with inhaled treprostinil for ≥6 weeks was performed. Effects of inhaled treprostinil on exercise capacity, functional class, and echocardiographic and hemodynamic data were evaluated. Adverse events were documented. Patients received 3 to 9 breaths (6 µg/breath) of inhaled treprostinil 4 times/day. All were receiving background PAH therapy; 12 had previously received parenteral prostanoid. Inhaled treprostinil was discontinued in 4 patients because of symptoms including cough and bronchospasm (n = 3) and progression of PAH (n = 1). Mild side effects including cough (n = 9) and sore throat (n = 6) did not require discontinuation of therapy. World Health Organization functional class improved in 19 and was unchanged in 10; exercise capacity significantly improved with the 6-minute walk distance, improving on follow-up from 455.7 ± 71.5 to 498 ± 70 m (p = 0.01) and peak oxygen consumption increasing from 25.5 ± 10.2 to 27.4 ± 10 (p = 0.04). In conclusion, inhaled treprostinil was associated with improvement in exercise capacity and World Health Organization functional class when added to background targeted PAH therapy in children and had an acceptable safety profile. Based on these early data, further study of inhaled treprostinil appears warranted in pediatric patients with PAH.