Lindsay M. Ryerson

Presentation, Diagnosis, and Medical Management of Heart Failure in Children: Canadian Cardiovascular Society Guidelines

Pediatric heart failure (HF) is an important cause of morbidity and mortality in childhood. This article presents guidelines for the recognition, diagnosis, and early medical management of HF in infancy, childhood, and adolescence. The guidelines are intended to assist practitioners in office-based or emergency room practice, who encounter children with undiagnosed heart disease and symptoms of possible HF, rather than those who have already received surgical palliation. The guidelines have been developed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology, and are accompanied by practical Recommendations for their application in the clinical setting, supplemented by online material. This work does not include Recommendations for advanced management involving ventricular assist devices, or other device therapies.

Pediatric Extracorporeal Life Support Organization Registry International Report 2016

The purpose of this report is to describe the international growth, outcomes, complications, and technology used in pediatric extracorporeal life support (ECLS) from 2009 to 2015 as reported by participating centers in the Extracorporeal Life Support Organization (ELSO). To date, there are 59,969 children who have received ECLS in the ELSO Registry; among those, 21,907 received ECLS since 2009 with an overall survival to hospital discharge rate of 61%. In 2009, 2,409 ECLS cases were performed at 157 centers. By 2015, that number grew to 2,992 cases in 227 centers, reflecting a 24% increase in patients and 55% growth in centers. ECLS delivered to neonates (0–28 days) for respiratory support was the largest subcategory of ECLS among children <18-years old. Overall, 48% of ECLS was delivered for respiratory support and 52% was for cardiac support or extracorporeal life support to support cardiopulmonary resuscitation (ECPR). During the study period, over half of children were supported on ECLS with centrifugal pumps (51%) and polymethylpentene oxygenators (52%). Adverse events including neurologic events were common during ECLS, a fact that underscores the opportunity and need to promote quality improvement work.

Heterotaxy syndrome and intestinal rotation abnormalities: A survey of institutional practice

PURPOSE Abnormalities of intestinal rotation (IRA) are commonly associated with heterotaxy syndrome (HS). There is controversy whether asymptomatic infants with HS require screening for IRA and if present, whether a prophylactic Ladd procedure is indicated. The objective of this study is to determine institutional practice across North America in the management of asymptomatic infants with HS and IRA. METHODS We performed an international, multi-institutional web based survey to examine current practice and opinions in the management of IRA in HS patients. RESULTS Overall response rate was 30%. Of physicians surveyed, 84% believe that HS patients should be screened for IRA in the neonatal period. 61% of general surgeons, 50% of cardiovascular surgeons and 45% of cardiologists feel that all patients with HS and an asymptomatic IRA should have a prophylactic Ladd procedure. 55% of physicians stated they would be comfortable with conservative management for patients with HS and asymptomatic IRA. CONCLUSIONS The risk of midgut volvulus, morbidity and mortality from elective procedures and cardiovascular prognosis must be considered prior to an elective Ladd procedure on asymptomatic HS patients. There are practice variance among sub-specialists caring for these patients, a lack of expert consensus, and a paucity of evidence-based data for IRA in this population.

Rotating inotrope therapy in a pediatric population with decompensated heart failure.

Objective: To describe the clinical course of a group of patients who received a rotating inotrope regimen, including levosimendan, for decompensated congestive heart failure. Design: Case series. Setting: Pediatric intensive care unit in a tertiary care childrens hospital. Patients: Nine pediatric patients with severe, decompensated heart failure. Intervention: The study patients received a rotating inotrope regimen, including levosimendan, dobutamine, and, in some cases, milrinone. Measurements and Main Results: Six patients were weaned from positive-pressure ventilation. Eight patients were discharged from the intensive care unit, and seven survived to hospital discharge. Two patients were successfully bridged to orthotopic cardiac transplantation. The therapies were generally well tolerated. Conclusions: Rotating inotropes were safe and seemed to be effective in this heterogeneous population of infants and children with decompensated heart failure. This therapeutic regimen warrants prospective comparative analysis.

Pulmonary Interstitial Glycogenosis Associated With Pulmonary Hypertension and Hypertrophic Cardiomyopathy

A neonate with pulmonary interstitial glycogenosis, pulmonary hypertension, and hypertrophic cardiomyopathy is described. The fatal outcome for this patient contrasts with the reported favorable prognosis associated with isolated pulmonary interstitial glycogenosis. To the authors’ knowledge, the association of pulmonary interstitial glycogenosis and hypertrophic cardiomyopathy has not been reported previously. The authors have broadened the phenotype of pulmonary interstitial glycogenosis and demonstrate the diagnostic value of lung biopsy in cases of unexplained neonatal pulmonary hypertension.

Indications and outcomes in children receiving renal replacement therapy in pediatric intensive care.

PURPOSE We aimed to describe patient characteristics, indications for renal replacement therapy (RRT), and outcomes in children requiring RRT. We hypothesized that fluid overload, not classic blood chemistry indications, would be the most frequent reason for RRT initiation. MATERIALS AND METHODS A retrospective cohort study of all patients receiving RRT at a single-center quaternary pediatric intensive care unit between January 2004 and December 2008 was conducted. RESULTS Ninety children received RRT. The median age was 7 months (interquartile range, 1-83). Forty-six percent of patients received peritoneal dialysis, and 54% received continuous renal replacement therapy. The median (interquartile range) PRISM-III score was 14 (8-19). Fifty-seven percent had congenital heart disease, and 32% were on extracorporeal life support. The most common clinical condition associated with acute kidney injury was hemodynamic instability (57%; 95% confidence interval [CI], 46-67), followed by multiorgan dysfunction syndrome (17%; 95% CI, 10-26). The most common indication for RRT initiation was fluid overload (77%; 95% CI, 66-86). Seventy-three percent (95% CI, 62-82) of patients survived to hospital discharge. CONCLUSIONS Hemodynamic instability and multiorgan dysfunction syndrome are the most common clinical conditions associated with acute kidney injury in our population. In the population studied, the mortality was lower than previously reported in children and much lower than in the adult population.

Survival and Neurocognitive Outcomes After Cardiac Extracorporeal Life Support in Children Less Than 5 Years of AgeCLINICAL PERSPECTIVE

Background—Survival after pediatric cardiac extracorporeal life support (ECLS) is guarded, and neurological morbidity varies widely. Our objective is to report our 10-year experience with cardiac ECLS, including survival and kindergarten entry neurocognitive outcomes; to identify predictors of mortality or adverse neurocognitive outcomes; and to compare 2 eras, before and after 2005. Methods and Results—From 2000 to 2009, 98 children had venoarterial cardiac ECLS. Sixty-four patients (65%) survived to hospital discharge, and 50 (51%) survived ⩽5 years of age. Neurocognitive follow-up of survivors was completed at mean (SD) age of 52.9 (8) months using Wechsler Preschool and Primary Scale of Intelligence. Logistic regression analysis found the longer time (hours) for lactate to fall below 2 mmol/L on ECLS (hazard ratio, 1.39; 95% confidence interval, 1.05, 1.84; P=0.022), and the amount of platelets (mL/kg) given in the first 48 hours (hazard ratio, 1.18; 95% confidence interval, 1.06, 1.32; P=0.002) was independently associated with higher in-hospital mortality. Receiving ECLS after the year 2005 was independently associated with lower risk of in-hospital mortality (hazard ratio, 0.36; 95% confidence interval, 0.13, 0.99; P=0.048). Extracorporeal cardiopulmonary resuscitation was not independently associated with mortality or neurocognitive outcomes. Era was not independently associated with neurocognitive outcomes. The full-scale intelligence quotient of survivors without chromosomal abnormalities was 79.7 (16.6) with 25% below 2 SD of the population mean. Conclusions—Mortality has improved over time; time for lactate to fall on ECLS and volume of platelets transfused are independent predictors of mortality. Extracorporeal cardiopulmonary resuscitation and era were not independently associated with neurocognitive outcomes.

Anticoagulation Management and Monitoring during Pediatric Extracorporeal Life Support: A Review of Current Issues

Anticoagulation is an imperfect science and is even more complicated in neonates and young children. The addition of the extracorporeal life support (ECLS) foreign circuit adds an additional layer of complexity. Anticoagulation goals during ECLS are to maintain a clot-free circuit and a hemostatically balanced patient. Unfractionated heparin (UFH) is the default gold standard anticoagulant as no large studies have been performed on any other anticoagulants. This review will focus on the advantages and disadvantages of the various methods to monitor UFH anticoagulation, discuss alternative anticoagulants, and examine bleeding and thrombotic complications during ECLS.

Successful Semi-Ambulatory Veno-Arterial Extracorporeal Membrane Oxygenation Bridge to Heart–Lung Transplantation in a Very Small Child

Lung transplantation (LTx) may be denied for children on extracorporeal membrane oxygenation (ECMO) due to high risk of cerebral hemorrhage. Rarely has successful LTx been reported in children over 10 years of age receiving awake or ambulatory veno‐venous ECMO. LTx following support with ambulatory veno‐arterial ECMO (VA ECMO) in children has never been reported to our knowledge. We present the case of a 4‐year‐old, 12‐kg child with heritable pulmonary artery hypertension and refractory right ventricular failure. She was successfully bridged to heart–lung transplantation (HLTx) using ambulatory VA ECMO. Initial resuscitation with standard VA ECMO was converted to an ambulatory circuit using Berlin heart cannulae. She was extubated and ambulating around her bed while on VA ECMO for 40 days. She received an HLTx from an oversized marginal lung donor. Despite a cardiac arrest and Grade 3 primary graft dysfunction, she made a full recovery without neurological deficits. She achieved 104% force expiratory volume in 1 s 33 months post‐HLTx. Ambulatory VA ECMO may be a useful strategy to bridge very young children to LTx or HLTx. Patient tailored ECMO cannulation, minimization of hemorrhage, and thrombosis risks while on ECMO contributed to a successful HLTx in our patient.

Survival and Neurocognitive Outcomes After Cardiac Extracorporeal Life Support in Children Less Than 5 Years of Age A Ten-Year Cohort

Background—Survival after pediatric cardiac extracorporeal life support (ECLS) is guarded, and neurological morbidity varies widely. Our objective is to report our 10-year experience with cardiac ECLS, including survival and kindergarten entry neurocognitive outcomes; to identify predictors of mortality or adverse neurocognitive outcomes; and to compare 2 eras, before and after 2005. Methods and Results—From 2000 to 2009, 98 children had venoarterial cardiac ECLS. Sixty-four patients (65%) survived to hospital discharge, and 50 (51%) survived ⩽5 years of age. Neurocognitive follow-up of survivors was completed at mean (SD) age of 52.9 (8) months using Wechsler Preschool and Primary Scale of Intelligence. Logistic regression analysis found the longer time (hours) for lactate to fall below 2 mmol/L on ECLS (hazard ratio, 1.39; 95% confidence interval, 1.05, 1.84; P=0.022), and the amount of platelets (mL/kg) given in the first 48 hours (hazard ratio, 1.18; 95% confidence interval, 1.06, 1.32; P=0.002) was independently associated with higher in-hospital mortality. Receiving ECLS after the year 2005 was independently associated with lower risk of in-hospital mortality (hazard ratio, 0.36; 95% confidence interval, 0.13, 0.99; P=0.048). Extracorporeal cardiopulmonary resuscitation was not independently associated with mortality or neurocognitive outcomes. Era was not independently associated with neurocognitive outcomes. The full-scale intelligence quotient of survivors without chromosomal abnormalities was 79.7 (16.6) with 25% below 2 SD of the population mean. Conclusions—Mortality has improved over time; time for lactate to fall on ECLS and volume of platelets transfused are independent predictors of mortality. Extracorporeal cardiopulmonary resuscitation and era were not independently associated with neurocognitive outcomes.