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Recognition and Initial Management of Fulminant Myocarditis: A Scientific Statement From the American Heart Association.
Kociol RD, Cooper LT, Fang JC, Moslehi JJ, Pang PS, Sabe MA, Shah RV, Sims DB, Thiene G, Vardeny O, American Heart Association Heart Failure and Transplantation Committee of the Council on Clinical Cardiology
(2020) Circulation 141: e69-e92
MeSH Terms: American Heart Association, Arrhythmias, Cardiac, Extracorporeal Membrane Oxygenation, Female, Heart Transplantation, Humans, Multiple Organ Failure, Myocarditis, Practice Guidelines as Topic, Shock, Cardiogenic, United States
Show Abstract · Added January 15, 2020
Fulminant myocarditis (FM) is an uncommon syndrome characterized by sudden and severe diffuse cardiac inflammation often leading to death resulting from cardiogenic shock, ventricular arrhythmias, or multiorgan system failure. Historically, FM was almost exclusively diagnosed at autopsy. By definition, all patients with FM will need some form of inotropic or mechanical circulatory support to maintain end-organ perfusion until transplantation or recovery. Specific subtypes of FM may respond to immunomodulatory therapy in addition to guideline-directed medical care. Despite the increasing availability of circulatory support, orthotopic heart transplantation, and disease-specific treatments, patients with FM experience significant morbidity and mortality as a result of a delay in diagnosis and initiation of circulatory support and lack of appropriately trained specialists to manage the condition. This scientific statement outlines the resources necessary to manage the spectrum of FM, including extracorporeal life support, percutaneous and durable ventricular assist devices, transplantation capabilities, and specialists in advanced heart failure, cardiothoracic surgery, cardiac pathology, immunology, and infectious disease. Education of frontline providers who are most likely to encounter FM first is essential to increase timely access to appropriately resourced facilities, to prevent multiorgan system failure, and to tailor disease-specific therapy as early as possible in the disease process.
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11 MeSH Terms
Rapid resolution of hyperammonemia in neonates using extracorporeal membrane oxygenation as a platform to drive hemodialysis.
Robinson JR, Conroy PC, Hardison D, Hamid R, Grubb PH, Pietsch JB, Lovvorn HN
(2018) J Perinatol 38: 665-671
MeSH Terms: Ammonia, Cohort Studies, Extracorporeal Membrane Oxygenation, Female, Hospitals, Pediatric, Humans, Hyperammonemia, Infant, Newborn, Logistic Models, Male, Peritoneal Dialysis, Prognosis, Referral and Consultation, Renal Dialysis, Retrospective Studies, Risk Assessment, Severity of Illness Index, Survival Rate, Treatment Outcome
Show Abstract · Added June 27, 2018
OBJECTIVE - We aimed to clarify the impact of extracorporeal membrane oxygenation (ECMO) as a platform to drive hemodialysis (HD) for ammonia clearance on outcomes of neonates with severe hyperammonemia.
STUDY DESIGN - All neonates treated for hyperammonemia at a single children's hospital between 1992 and 2016 were identified. Patient characteristics and outcomes were compared between those receiving medical management or ECMO/HD.
RESULT - Twenty-five neonates were treated for hyperammonemia, of which 13 (52%) received ECMO/HD. Peak ammonia levels among neonates treated with ECMO/HD were significantly higher than those medically managed (1041 [IQR 902-1581] μmol/L versus 212 [IQR 110-410] μmol/L; p = 0.009). Serum ammonia levels in the ECMO/HD cohort declined to the median of medically managed within 4.5 (IQR 2.9-7.0) hours and normalized within 7.3 (IQR 3.6-13.5) hours. All neonates survived ECMO/HD, and nine (69.2%) survived to discharge.
CONCLUSION - ECMO/HD is an effective adjunct to rapidly clear severe hyperammonemia in newborns, reducing potential neurodevelopmental morbidity.
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19 MeSH Terms
Review of 1,000 consecutive extracorporeal membrane oxygenation runs as a quality initiative.
Lovvorn HN, Hardison DC, Chen H, Westrick AC, Danko ME, Bridges BC, Walsh WF, Pietsch JB
(2017) Surgery 162: 385-396
MeSH Terms: Adolescent, Adult, Age Factors, Cardiopulmonary Resuscitation, Child, Child, Preschool, Extracorporeal Membrane Oxygenation, Hospital Mortality, Hospitalization, Humans, Infant, Infant, Newborn, Patient Selection, Registries, Retrospective Studies, Young Adult
Show Abstract · Added November 30, 2020
BACKGROUND - Extracorporeal membrane oxygenation is a resource-intensive mode of life-support potentially applicable when conventional therapies fail. Given the initial success of extracorporeal membrane oxygenation to support neonates and infants in the 1980s, indications have expanded to include adolescents, adults, and selected moribund patients during cardiopulmonary resuscitation. This single-institution analysis was conducted to evaluate programmatic growth, outcomes, and risk for death despite extracorporeal membrane oxygenation across all ages and diseases.
METHODS - Beginning in 1989, we registered prospectively all extracorporeal membrane oxygenation patient data with the Extracorporeal Life Support Organization. We queried this registry for our institution-specific data to compare the parameter of "discharge alive" between age groups (neonatal, pediatric, adult), disease groups (respiratory, cardiac, cardiopulmonary resuscitation), and modes of extracorporeal membrane oxygenation (veno-venous; veno-arterial). Extracorporeal membrane oxygenation-specific complications (mechanical, hemorrhagic, neurologic, renal, cardiovascular, pulmonary, infectious, metabolic) were analyzed similarly. Descriptive statistics, Kaplan-Meier, and linear regression analyses were conducted.
RESULTS - After 1,052 extracorporeal membrane oxygenation runs, indications have expanded to include adults, to supplement cardiopulmonary resuscitation, to support hemodialysis in neonates and plasmapheresis in children, and to bridge all age patients to heart and lung transplant. Overall survival to discharge was 52% and was better for respiratory diseases (P < .001). Probability of individual survival decreased to <50% if pre-extracorporeal membrane oxygenation mechanical ventilation exceeded respectively 123 hours for cardiac, 166 hours for cardiopulmonary resuscitation, and 183 hours for respiratory diseases (P = .013). Complications occurred most commonly among cardiac and cardiopulmonary resuscitation runs (P < .001), the veno-arterial mode (P < .001), and in adults (P = .044).
CONCLUSION - Our extracorporeal membrane oxygenation program, an Extracorporeal Life Support Organization-designated Center of Excellence, has experienced substantial growth in volume and indications, including increasing age and disease severity. Considering the entire cohort, pre-extracorporeal membrane oxygenation ventilation exceeding 7 days was associated with an increased probability of death.
Copyright © 2017 Elsevier Inc. All rights reserved.
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Neurological complications and outcomes in the Berlin Heart EXCOR® pediatric investigational device exemption trial.
Jordan LC, Ichord RN, Reinhartz O, Humpl T, Pruthi S, Tjossem C, Rosenthal DN
(2015) J Am Heart Assoc 4: e001429
MeSH Terms: Child, Child, Preschool, Extracorporeal Membrane Oxygenation, Female, Follow-Up Studies, Heart Failure, Heart Transplantation, Heart-Assist Devices, Humans, Infant, Ischemic Attack, Transient, Kaplan-Meier Estimate, Logistic Models, Male, Multivariate Analysis, Pediatrics, Prospective Studies, Prosthesis Design, Risk Assessment, Stroke, Survival Rate, Treatment Outcome, Waiting Lists
Show Abstract · Added March 24, 2020
BACKGROUND - The Berlin Heart EXCOR(®) ventricular assist device has been approved for use in the United States as a bridge to heart transplantation in children. We sought to characterize neurological events in children supported with the Berlin Heart EXCOR(®) device.
METHODS AND RESULTS - The multicenter prospective cohort consisted of all 204 children implanted with the Berlin Heart EXCOR(®) device at 47 centers in North America between May 2007 and December 2010. There were 73 neurological events in 59 patients, with 29% of the cohort experiencing ≥1 neurological event. Events included 52 strokes in 43 patients (21% of the cohort). The neurological event rate was 0.51 events per 100 patient-days. Many of the neurological events occurred early in the course of support, with 30 events recorded during the first 14 days of support. The mortality rate in participants with at least 1 neurological event was 42% (25 of 59), significantly higher than the 18% mortality rate (26 of 145) for those who did not have a neurological event (P=0.0006). Risk-factor analysis did not identify significant preimplantation predictors of neurological injury.
CONCLUSIONS - Of children treated with the Berlin Heart EXCOR(®) device as a bridge to transplant, 29% experienced at least 1 neurological event. The majority of neurological events were ischemic strokes, and many of those occurred early in the course of support. Neurological injury was the leading cause of death after implantation of the Berlin Heart EXCOR(®) device. Risk stratification for stroke or neurological injury is not possible based on baseline preimplantation characteristics.
CLINICAL TRIAL REGISTRATION URL - www.clinicaltrials.gov. Unique Identifier: NCT00583661.
© 2015 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.
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Neonatal carotid repair at ECMO decannulation: patency rates and early neurologic outcomes.
Duggan EM, Maitre N, Zhai A, Krishnamoorthi H, Voskresensky I, Hardison D, Tice J, Pietsch JB, Lovvorn HN
(2015) J Pediatr Surg 50: 64-8
MeSH Terms: Brain, Carotid Arteries, Evoked Potentials, Auditory, Brain Stem, Extracorporeal Membrane Oxygenation, Female, Heart Defects, Congenital, Humans, Infant, Newborn, Ligation, Male, Postoperative Period, Retrospective Studies, Vascular Patency
Show Abstract · Added October 1, 2015
PURPOSE - Neonates placed on veno-arterial extracorporeal membrane oxygenation (VA-ECMO) undergo either carotid repair or ligation at decannulation. Study aims were to evaluate carotid patency rates after repair and to compare early neurologic outcomes between repaired and ligated patients.
METHODS - A retrospective study of all neonates without congenital heart disease (CHD) who had VA-ECMO between 1989 and 2012 was completed using our institutional ECMO Registry. Carotid patency after repair, neuroimaging studies, and auditory brainstem response (ABR) testing at time of discharge were examined.
RESULTS - 140 neonates were placed on VA-ECMO during the study period. Among survivors, 84% of carotids repaired and imaged remained patent at last study. No significant differences were observed between infants in the repaired and ligated groups regarding diagnosis, ECMO duration, or length of stay. A large proportion (43%) developed a severe brain lesion after VA-ECMO, but few failed their ABR testing. Differences in early neurologic outcomes between the two groups of survivors were not significant.
CONCLUSIONS - At this single institution, carotid patency is excellent following repair at ECMO decannulation. No increased incidence of severe brain lesions or greater neurosensory impairment in the repair group was observed. Further studies are needed to investigate the effects of ligation on longer-term neurocognitive outcomes.
Copyright © 2015 Elsevier Inc. All rights reserved.
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13 MeSH Terms
A pilot study of cerebrovascular reactivity autoregulation after pediatric cardiac arrest.
Lee JK, Brady KM, Chung SE, Jennings JM, Whitaker EE, Aganga D, Easley RB, Heitmiller K, Jamrogowicz JL, Larson AC, Lee JH, Jordan LC, Hogue CW, Lehmann CU, Bembea MM, Hunt EA, Koehler RC, Shaffner DH
(2014) Resuscitation 85: 1387-93
MeSH Terms: Adolescent, Cerebrovascular Circulation, Child, Child, Preschool, Extracorporeal Membrane Oxygenation, Female, Heart Arrest, Homeostasis, Humans, Infant, Male, Pilot Projects, Resuscitation
Show Abstract · Added March 24, 2020
AIM - Improved survival after cardiac arrest has placed greater emphasis on neurologic resuscitation. The purpose of this pilot study was to evaluate the relationship between cerebrovascular autoregulation and neurologic outcomes after pediatric cardiac arrest.
METHODS - Children resuscitated from cardiac arrest had autoregulation monitoring during the first 72h after return of circulation with an index derived from near-infrared spectroscopy in a pilot study. The range of mean arterial blood pressure (MAP) with optimal vasoreactivity (MAPOPT) was identified. The area under the curve (AUC) of the time spent with MAP below MAPOPT and MAP deviation below MAPOPT was calculated. Neurologic outcome measures included placement of a new tracheostomy or gastrostomy, death from a primary neurologic etiology (brain death or withdrawal of support for neurologic futility), and change in the Pediatric Cerebral Performance Category score (ΔPCPC).
RESULTS - Thirty-six children were monitored. Among children who did not require extracorporeal membrane oxygenation (ECMO), children who received a tracheostomy/gastrostomy had greater AUC during the second 24h after resuscitation than those who did not (P=0.04; n=19). Children without ECMO who died from a neurologic etiology had greater AUC during the first 48h than did those who lived or died from cardiovascular failure (P=0.04; n=19). AUC below MAPOPT was not associated with ΔPCPC when children with or without ECMO were analyzed separately.
CONCLUSIONS - Deviation from the blood pressure with optimal autoregulatory vasoreactivity may predict poor neurologic outcomes after pediatric cardiac arrest. This experimental autoregulation monitoring technique may help individualize blood pressure management goals after resuscitation.
Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.
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Residual lesions in postoperative pediatric cardiac surgery patients receiving extracorporeal membrane oxygenation support.
Agarwal HS, Hardison DC, Saville BR, Donahue BS, Lamb FS, Bichell DP, Harris ZL
(2014) J Thorac Cardiovasc Surg 147: 434-41
MeSH Terms: Cardiac Catheterization, Cardiac Surgical Procedures, Chi-Square Distribution, Early Diagnosis, Echocardiography, Extracorporeal Membrane Oxygenation, Female, Heart Defects, Congenital, Hemodynamics, Hospital Mortality, Humans, Infant, Infant, Newborn, Male, Postoperative Complications, Predictive Value of Tests, Retrospective Studies, Tennessee, Time Factors, Treatment Outcome
Show Abstract · Added February 22, 2016
OBJECTIVE - The objective of this study was to examine the incidence and clinical outcomes of residual lesions in postoperative pediatric cardiac surgery patients receiving extracorporeal membrane oxygenation (ECMO) support.
METHODS - A retrospective observational study was undertaken at a pediatric heart institution. Postoperative pediatric cardiac surgery patients receiving ECMO support within 7 days of surgery during the past 7 years (2005-2011) were studied. A hemodynamically significant cardiac lesion on ECMO support that required intervention to decannulate successfully was defined as a residual lesion. Demographic data, complexity of cardiac defect, surgical data, indications for ECMO, echocardiographic findings, and cardiac catheterization results were studied. Evaluation of residual lesions based on duration of ECMO support, interventions undertaken, and clinical outcomes were also examined.
RESULTS - Residual lesions were evaluated in 43 of 119 postoperative patients placed on ECMO support. Lesions were detected in 35 patients (28%), predominantly in branch pulmonary arteries (n = 10), shunts (n = 7), and ventricular outflow tracts (n = 9). Echocardiography detected 7 residual lesions (20%) and cardiac catheterization detected 28 residual lesions (80%). Earlier detection of residual lesions during the first 3 days of ECMO support in 24 patients improved their rate of decannulation significantly (P = .004) and survival to hospital discharge (P = .035), compared with later detection (after 3 days of ECMO support) in 11 patients.
CONCLUSIONS - Residual lesions are present in approximately one-quarter of postoperative cardiac surgery patients requiring ECMO support. All postoperative pediatric cardiac surgery patients unable to be weaned off ECMO successfully should be evaluated actively for residual lesions, preferably by cardiac catheterization imaging. Earlier detection of residual lesions and reintervention are associated with improved clinical outcome.
Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.
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20 MeSH Terms
Berlin Heart EXCOR pediatric ventricular assist device for bridge to heart transplantation in US children.
Almond CS, Morales DL, Blackstone EH, Turrentine MW, Imamura M, Massicotte MP, Jordan LC, Devaney EJ, Ravishankar C, Kanter KR, Holman W, Kroslowitz R, Tjossem C, Thuita L, Cohen GA, Buchholz H, St Louis JD, Nguyen K, Niebler RA, Walters HL, Reemtsen B, Wearden PD, Reinhartz O, Guleserian KJ, Mitchell MB, Bleiweis MS, Canter CE, Humpl T
(2013) Circulation 127: 1702-11
MeSH Terms: Body Size, Cause of Death, Child, Child, Preschool, Comorbidity, Compassionate Use Trials, Equipment Design, Extracorporeal Membrane Oxygenation, Female, Heart Defects, Congenital, Heart Diseases, Heart Transplantation, Heart-Assist Devices, Hemorrhage, Humans, Hyperbilirubinemia, Infant, Kidney Diseases, Liver Diseases, Male, Mortality, Multiple Organ Failure, Proportional Hazards Models, Risk, Stroke, Survival Rate, Treatment Outcome, Waiting Lists
Show Abstract · Added March 24, 2020
BACKGROUND - Recent data suggest that the Berlin Heart EXCOR Pediatric ventricular assist device is superior to extracorporeal membrane oxygenation for bridge to heart transplantation. Published data are limited to 1 in 4 children who received the device as part of the US clinical trial. We analyzed outcomes for all US children who received the EXCOR to characterize device outcomes in an unselected cohort and to identify risk factors for mortality to facilitate patient selection.
METHODS AND RESULTS - This multicenter, prospective cohort study involved all children implanted with the Berlin Heart EXCOR Pediatric ventricular assist device at 47 centers from May 2007 through December 2010. Multiphase nonproportional hazards modeling was used to identify risk factors for early (<2 months) and late mortality. Of 204 children supported with the EXCOR, the median duration of support was 40 days (range, 1-435 days). Survival at 12 months was 75%, including 64% who reached transplantation, 6% who recovered, and 5% who were alive on the device. Multivariable analysis identified lower weight, biventricular assist device support, and elevated bilirubin as risk factors for early mortality and bilirubin extremes and renal dysfunction as risk factors for late mortality. Neurological dysfunction occurred in 29% and was the leading cause of death.
CONCLUSIONS - Use of the Berlin Heart EXCOR has risen dramatically over the past decade. The EXCOR has emerged as a new treatment standard in the United States for pediatric bridge to transplantation. Three-quarters of children survived to transplantation or recovery; an important fraction experienced neurological dysfunction. Smaller patient size, renal dysfunction, hepatic dysfunction, and biventricular assist device use were associated with mortality, whereas extracorporeal membrane oxygenation before implantation and congenital heart disease were not.
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Extracorporeal membrane oxygenation support of a severe metabolic crisis in a child with methylmalonic acidemia.
Stark RJ, Naik-Mathuria BJ, Lam FW, Olutoye OO, Sutton VR, Shekerdemian LS
(2012) ASAIO J 58: 438-9
MeSH Terms: Amino Acid Metabolism, Inborn Errors, Child, Extracorporeal Membrane Oxygenation, Female, Humans, Methylmalonic Acid, Phenotype, Respiratory Insufficiency, Treatment Outcome, Vasoplegia
Show Abstract · Added July 28, 2015
A 9-year-old female, with mut phenotype of methylmalonic acidemia who developed severe vasoplegic shock during a metabolic crisis, was successfully supported with venoarterial extracorporeal membrane oxygenation.
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10 MeSH Terms
Fatal Thrombosis After Factor VII Administration During Extracorporeal Membrane Oxygenation.
Swaminathan M, Shaw AD, Greenfield RA, Grichnik KP
(2008) J Cardiothorac Vasc Anesth 22: 259-60
MeSH Terms: Adult, Extracorporeal Membrane Oxygenation, Factor VII, Fatal Outcome, Female, Humans, Intraoperative Complications, Thrombosis
Added October 20, 2015
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8 MeSH Terms