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Pubblicazioni recenti - pediatric cardiac
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Acquired von Willebrand Syndrome and Platelet Function Defects during Extracorporeal Life Support (Mechanical Circulatory Support).
Hamostaseologie2020 Jun;40(2):221-225. doi: 10.1055/a-1150-2016.
Schlagenhauf Axel, Kalbhenn Johannes, Geisen Ulrich, Beyersdorf Friedhelm, Zieger Barbara,
Abstract
Patients with ventricular assist devices (VADs) and extracorporeal membrane oxygenation (ECMO) suffer from an increased risk for thromboembolic events as well as for hemorrhages. High shear stress in the mechanical device results in acquired von Willebrand syndrome (AVWS), characterized by a loss of high-molecular-weight multimers of von Willebrand factor (VWF) leading to an increased bleeding risk. Onset of AVWS occurs within hours, persists during the whole period of mechanical support, and subsides rapidly after explantation. Patients with the older HeartMate II exhibit more severe AVWS than those with the newer HeartMate III, thanks to lower shear stress in the latter. All ECMO and VAD patients exhibit thrombocytopathia and often thrombocytopenia which further increases the bleeding risk. Etiological models for AVWS are increased cleavage by the metalloproteinase ADAMSTS13, mechanical destruction of VWF, and shear-induced VWF binding to platelets. Platelet secretion defects may be caused by transient platelet activation leading to degranulation. AVWS can be diagnosed by detection of VWF multimers using gel-electrophoresis and functional assays of varying sensitivity (VWF ristocetin cofactor activity, VWF activity, VWF collagen binding). Platelet dysfunction is monitored using light transmission aggregometry and secretion defects are detectable using flow cytometry. Modest use of anticoagulants and a target-controlled therapy based on VWF parameters and other coagulation and platelet parameters are shown to be beneficial in this patient group. Persistent hemorrhages may be controlled with tranexamic acid and platelet concentrates. Prompt weaning from the device, when indicated, is the best therapeutic option to prevent recurrent bleeding.
Georg Thieme Verlag KG Stuttgart · New York.
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Intubation precautions in a pediatric patient with severe COVID-19.
J Pediatr Surg Case Rep2020 Jul;58():101495. doi: 10.1016/j.epsc.2020.101495.
Shaw Robert, Tighe Nathaniel, Odegard Kirsten C, Alexander Peta, Emani Sitaram, Yuki Koichi,
Abstract
We present the case of a child diagnosed with COVID-19 soon after open-heart surgery who required an urgent second surgery. The patient suffered from severe COVID-19 disease. The utility of preoperative COVID-19 testing, determination of recovery by an array of inflammatory markers and perioperative management are described.
© 2020 The Authors.
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Lamin A/C Cardiomyopathy with E203K Pathogenic Mutation.
Cureus2020 Apr;12(4):e7761. doi: 10.7759/cureus.7761.
Sheikh Fahad N, Hassan Syed Adeel, Alam Dilnaz, Kundi Maryam, Hassan Moeez,
Abstract
Lamin A/C (LMNA) cardiomyopathy is an adult-onset, autosomal dominant, rapidly progressive cardiomyopathy which belongs to a spectrum of familial idiopathic cardiomyopathies. It is the most common type of familial dilated cardiomyopathy that is associated with conduction defects. A 76-year-old African American female with second-degree atrioventricular (AV) block presented for evaluation of persistent fatigue. Her family history was significant for sudden deaths of her son and brother at the age of 6 and 48 years, respectively, and AV block in her sister with a pacemaker implant at the age of 64 years. Physical examination was within normal limits. Electrocardiogram showed a Mobitz type II, second-degree AV block. Mild dilated cardiomyopathy was present on echocardiogram. Stress echocardiography had to be stopped due to premature ventricular contractions. Cardiac catheterization, coronary angiography, and cardiac MRI revealed no significant etiology for rhythm disturbance. Holter monitoring revealed intermittent bradycardia with a heart rate falling as low as 28 beats per minute, which led to the decision of dual-chamber pacemaker implantation. RhythmNext genetic testing (Ambry Genetics, Aliso Viejo, CA) was done due to the significant family history of sudden death; it revealed a heterozygous E203K pathologic mutation in the LMNA gene. Sudden death is the most common mode of death in LMNA cardiomyopathy; hence, the implantation of intracardiac cardioverter-defibrillator for primary prophylaxis was discussed with the patient. Clinicians should suspect LMNA cardiomyopathy in patients with rhythm disorders and family history of sudden death, which can help to identify individuals at risk and prevent sudden death by appropriate interventions.
Copyright © 2020, Sheikh et al.
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Prevalence and Risk Factors of Childhood Hypertension in Urban-Rural Areas of China: A Cross-Sectional Study.
Int J Hypertens2020 ;2020():2374231. doi: 10.1155/2020/2374231.
Liang Xiaohua, Xiao Lun, Luo Yetao, Xu Jiapei,
Abstract
Objective:
The increased blood pressure level in children and adolescents is recognized as an essential predictor of adulthood cardiovascular disease. This study aimed to ascertain the prevalence and the urban-rural disparity of childhood hypertension in the southwest of China.
Methods:
Using stratified cluster sampling in urban and rural areas, a total of 13597 primary school children aged 6?12 years living in the Southwest of China were included. The prevalence of hypertension was analyzed. The risk factors were collected by questionnaires, and the risk factors of childhood hypertension were analyzed by the logistic regression model.
Results:
The prevalence of hypertension was 13.75%, 9.02%, and 17.47% in total, urban, and rural children, respectively, and the urban-rural difference was 8.44% (95%CI: 7.32%, 9.56%). Children with obesity, maternal gestational hypertension, >10 months of breastfeeding, or low family income had a significantly increased prevalence of hypertension (29.4%, 20.00%, 16.31%, and 16.25%, respectively). Rural residence, intake of more pickle (in rural), maternal gestational hypertension (in urban), low birth weight (in rural), obesity, increased heart rate, and red blood cell counts were the risk factors of childhood hypertension. The adjusted values were 13.61%, 23.25%, 10.88%, 11.12%, 12.23%, and 25.04% in the full models excluding and including serum indexes for total, urban, and rural children, respectively.
Conclusions:
The prevalence of childhood hypertension is significant in the Southwest of China and alarming in rural areas, which requires community intervention. Children living in rural areas combined with obesity, low social economic status, dietary imbalance, and abnormal lipid metabolism were associated with an increased risk of hypertension, and routine care programs should be conducted to prevent childhood hypertension.
Copyright © 2020 Xiaohua Liang et al.
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Intelligent Diagnosis of Heart Murmurs in Children with Congenital Heart Disease.
J Healthc Eng2020 ;2020():9640821. doi: 10.1155/2020/9640821.
Wang Jiaming, You Tao, Yi Kang, Gong Yaqin, Xie Qilian, Qu Fei, Wang Bangzhou, He Zhaoming,
Abstract
Heart auscultation is a convenient tool for early diagnosis of heart diseases and is being developed to be an intelligent tool used in online medicine. Currently, there are few studies on intelligent diagnosis of pediatric murmurs due to congenital heart disease (CHD). The purpose of the study was to develop a method of intelligent diagnosis of pediatric CHD murmurs. Phonocardiogram (PCG) signals of 86 children were recorded with 24 children having normal heart sounds and 62 children having CHD murmurs. A segmentation method based on the discrete wavelet transform combined with Hadamard product was implemented to locate the first and the second heart sounds from the PCG signal. Ten features specific to CHD murmurs were extracted as the input of classifier after segmentation. Eighty-six artificial neural network classifiers were composed into a classification system to identify CHD murmurs. The accuracy, sensitivity, and specificity of diagnosis for heart murmurs were 93%, 93.5%, and 91.7%, respectively. In conclusion, a method of intelligent diagnosis of pediatric CHD murmurs is developed successfully and can be used for online screening of CHD in children.
Copyright © 2020 Jiaming Wang et al.
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Surgical Management of an Empyema in a 3-Year-Old Child.
Case Rep Med2020 ;2020():5179292. doi: 10.1155/2020/5179292.
Kanakis Meletios A, Petsios Konstantinos Th, Giannopoulos Nicholas M, Bobos Dimitrios, Hatzianastasiou Sofia, Sianidou Lida C, Lioulias Achilleas,
Abstract
empyema in nonimmunocompromised children is rare. A case of surgical management of invasive aspergillosis in a previously healthy 3-year-old child is presented. The patient was initially admitted to a hospital with severe respiratory deterioration and clinical instability, originally attributed to sepsis. After surgical intervention and the diagnosis of invasive aspergillosis, intravenous therapy with voriconazole was initiated. During postoperative care, the patient's condition remained stable with mild functional respiratory deficits. The diagnosis and treatment of empyema remains challenging, especially in cases that the recognition of aspergillosis is delayed and urgent surgical management of the empyema is required due to rapid clinical deterioration of the patient. The early initiation, prolonged administration, and close monitoring of high-dose antifungal treatment are highly recommended.
Copyright © 2020 Meletios A. Kanakis et al.
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Towards personalized medicine in maternal and child health: integrating biologic and social determinants.
Pediatr Res2020 May;():. doi: 10.1038/s41390-020-0981-8.
Stevenson David K, Wong Ronald J, Aghaeepour Nima, Maric Ivana, Angst Martin S, Contrepois Kevin, Darmstadt Gary L, Druzin Maurice L, Eisenberg Michael L, Gaudilliere Brice, Gibbs Ronald S, Gotlib Ian H, Gould Jeffrey B, Lee Henry C, Ling Xuefeng B, Mayo Jonathan A, Moufarrej Mira N, Quaintance Cecele C, Quake Stephen R, Relman David A, Sirota Marina, Snyder Michael P, Sylvester Karl G, Hao Shiying, Wise Paul H, Shaw Gary M, Katz Michael,
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Creatinine filtration kinetics in critically Ill neonates.
Pediatr Res2020 May;():. doi: 10.1038/s41390-020-0977-4.
Rios Danielle R, Rhee Christopher J, Elizondo Leah, Brady Kenneth M, Rusin Craig G, Acosta Sebastian,
Abstract
BACKGROUND:
Creatinine values are unreliable within the first weeks of life; however, creatinine is used most commonly to assess kidney function. Controversy remains surrounding the time required for neonates to clear maternal creatinine.
METHODS:
Eligible infants had multiple creatinine lab values and were admitted to the neonatal intensive care unit (NICU). A mathematical model was fit to the lab data to estimate the filtration onset delay, creatinine filtration half-life, and steady-state creatinine concentration for each subject. Infants were grouped by gestational age (GA) [(1) 22-27, (2) >27-32, (3) >32-37, and (4) >37-42 weeks].
RESULTS:
A total of 4808 neonates with a mean GA of 34.4?±?5 weeks and birth weight of 2.34?±?1.1?kg were enrolled. Median (95% confidence interval) filtration onset delay for Group 1 was 4.3 (3.71, 4.89) days and was significantly different than all other groups (p?
CONCLUSIONS:
We quantified the observed kidney behavior in a large NICU population as a function of day of life and GA using creatinine lab results. These results can be used to interpret individual creatinine labs for infants to detect those most at risk for acute kidney injury.
IMPACT:
One of the largest cohorts of premature infants to describe the evolution of kidney development and function over their entire hospitalization.New concept introduced of the kidney filtration onset delay, the time needed for the kidney to begin clearance of creatinine, and that it can be used as an early indicator of kidney function.The smallest premature infants from 22 to 27 weeks gestation took the longest time to begin and complete maternal creatinine clearance.Clinicians can easily compare the creatinine level of their patient to the normative curves to improve understanding of kidney function at the bedside.
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Extracorporeal Blood Purification and Organ Support in the Critically Ill Patient during COVID-19 Pandemic: Expert Review and Recommendation.
Blood Purif2020 May;():1-11. doi: 10.1159/000508125.
Ronco Claudio, Bagshaw Sean M, Bellomo Rinaldo, Clark William R, Husain-Syed Faeq, Kellum John A, Ricci Zaccaria, Rimmelé Thomas, Reis Thiago, Ostermann Marlies,
Abstract
Critically ill COVID-19 patients are generally admitted to the ICU for respiratory insufficiency which can evolve into a multiple-organ dysfunction syndrome requiring extracorporeal organ support. Ongoing advances in technology and science and progress in information technology support the development of integrated multi-organ support platforms for personalized treatment according to the changing needs of the patient. Based on pathophysiological derangements observed in COVID-19 patients, a rationale emerges for sequential extracorporeal therapies designed to remove inflammatory mediators and support different organ systems. In the absence of vaccines or direct therapy for COVID-19, extracorporeal therapies could represent an option to prevent organ failure and improve survival. The enormous demand in care for COVID-19 patients requires an immediate response from the scientific community. Thus, a detailed review of the available technology is provided by experts followed by a series of recommendation based on current experience and opinions, while waiting for generation of robust evidence from trials.
© 2020 S. Karger AG, Basel.
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Purkinje System Hyperexcitability and Ventricular Arrhythmia Risk in Type 3 Long QT Syndrome.
Heart Rhythm2020 May;():. doi: S1547-5271(20)30453-7.
Barake Walid, Giudicessi John R, Asirvatham Samuel J, Ackerman Michael J,
Abstract
BACKGROUND:
Gain-of-function variants in the SCN5A-encoded Nav1.5 sodium channel cause type 3 long QT syndrome (LQT3) and multifocal ectopic Purkinje-related premature contractions (MEPPC). Although the Purkinje system is uniquely sensitive to the action potential prolonging effects of LQT3-causative variants, the existence of additional Purkinje phenotype(s) in LQT3 is unknown.
OBJECTIVE:
To determine the prevalence and clinical implications of frequent fascicular/Purkinje-related premature ventricular contractions (PVCs) and short-coupled ventricular arrhythmias (VAs), suggestive of Purkinje system hyperexcitability (PSH), in a single-center LQT3 cohort.
METHODS:
Retrospective analysis of 177 SCN5A-positive patients was performed to identify individuals with a LQT3 phenotype. Available electrocardiographic, electrophysiology (EP) study, device, and genetic data from 91 LQT3 individuals were reviewed for evidence of presumed fascicular PVCs and short-coupled VAs. The relationship between PSH and ventricular fibrillation (VF) events was assessed by Kaplan-Meier and Cox regression analyses.
RESULTS:
Overall, 30/91 (33%) patients with LQT3 exhibited evidence of presumed PSH [fascicular PVCs 30/30 (100%); short-coupled VAs 17/30 (56%)]. Kaplan-Meier and Cox regression analyses demonstrated an increased risk of VF events in individuals with LQT3 and PSH (log rank p<0.03; HR=3.95, 95% CI 1.15-15.7, p=0.03). Interestingly, variants in the voltage-sensing domain regions of Nav1.5 were more frequently observed in LQT3 patients with PSH than those without [19/30 (63%) vs. 9/61 (15%); p<0.0001].
CONCLUSIONS:
This study demonstrates that a discernible Purkinje phenotype is present in one- third of LQT3 cases and increases the risk of potentially lethal VAs. Further study is needed to determine if a distinct cellular EP phenotype underlies this phenomenon.
Copyright © 2020. Published by Elsevier Inc.
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Epidemiology, Treatments, and Cardiac Complications in Patients with Kawasaki Disease: The Nationwide Survey in Japan, 2017-2018.
J Pediatr2020 May;():. doi: S0022-3476(20)30625-9.
Ae Ryusuke, Makino Nobuko, Kosami Koki, Kuwabara Masanari, Matsubara Yuri, Nakamura Yosikazu,
Abstract
OBJECTIVE:
To report the epidemiologic characteristics, treatments, and cardiac complications of KD, using data from the nationwide survey in Japan.
STUDY DESIGN:
The nationwide KD survey in Japan has been conducted biennially since 1970. The most recent survey was completed in 2019, obtaining information for patients who developed KD during 2017-2018. Survey respondents were hospitals specializing in pediatrics and those with ?100 beds and a pediatric department throughout Japan, where patients with Kawasaki disease were eventually hospitalized.
RESULTS:
The survey identified 32528 patients with Kawasaki disease, which consisted of 15164 (46.6%) in 2017 and 17364 (53.4%) in 2018. The highest annual incidence rate was recorded in 2018 (359 per 100,000 children aged 0-4 years). After 1982, patients with ?4 principal KD signs gradually increased, resulting in 6847 (21.1%) patients diagnosed during 2017-2018. Among 30784 patients receiving initial intravenous immunoglobulin administration, 6061 (19.7%) did not respond. Within 30 days of KD onset, 9.0% of patients were diagnosed with cardiac complications, and consequently, 2.6% of patients developed cardiac sequelae after the acute illness.
CONCLUSIONS:
The annual number of patients developing KD in Japan increased from 1970 through 2018, while the proportion of patients with Kawasaki disease with cardiac complications decreased in the most recent two decades. Early diagnosis of KD as well as advances in initial treatments could explain these findings.
Copyright © 2020. Published by Elsevier Inc.
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A 20/20 view of ANT function in mitochondrial biology and necrotic cell death.
J Mol Cell Cardiol2020 May;():. doi: S0022-2828(20)30194-2.
Bround Michael J, Bers Donald M, Molkentin Jeffery D,
Abstract
The adenosine nucleotide translocase (ANT) family of proteins are inner mitochondrial membrane proteins involved in energy homeostasis and cell death. The primary function of ANT proteins is to exchange cytosolic ADP with matrix ATP, facilitating the export of newly synthesized ATP to the cell while providing new ADP substrate to the mitochondria. As such, the ANT proteins are central to maintaining energy homeostasis in all eukaryotic cells. Evidence also suggests that the ANTs constitute a pore-forming component of the mitochondrial permeability transition pore (MPTP), a structure that forms in the inner mitochondrial membrane that is thought to underlie regulated necrotic cell death. Additionally, emerging studies suggest that ANT proteins are also critical for mitochondrial uncoupling and for promoting mitophagy. Thus, the ANTs are multifunctional proteins that are poised to participate in several aspects of mitochondrial biology and the greater regulation of cell death, which will be discussed here.
Copyright © 2019. Published by Elsevier Ltd.
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Assessment of anomalous coronary arteries by imagers and surgeons: Comparison of imaging modalities.
Ann Thorac Surg2020 May;():. doi: S0003-4975(20)30742-6.
Farooqi Kanwal M, Nees Shannon N, Smerling Jennifer, Senapathi Sri H, Lorenzoni Raymond, Pavlicova Martina, Einstein Andrew J, Bacha Emile A, Kalfa David, Chai Paul J,
Abstract
BACKGROUND:
Anomalous aortic origin of a coronary artery (AAOCA) is associated with sudden cardiac death. High risk characteristics are most commonly assessed using two dimensional (2D) echocardiogram (echo) or cardiac computed tomography (CT). We hypothesize that these characteristics will be more accurately assessed when they are presented in the form of a 3D digital model.
METHODS:
14 participants including cardiothoracic surgeons and cardiac imaging specialists assessed image representations including echo, CT images and a 3D digital model, from six patients who had undergone AAOCA repair. Accuracy of assessment was evaluated by comparing responses with operative findings, i.e. the "gold standard".
RESULTS:
The reported type of AAOCA was most accurately assessed on CT (100%) and 3D models (92.31%) as compared to echo (80.77%). The accuracy of the AAOCA course was highest on CT (91.03%), 80.77% on 3D model and lowest on echo (61.54%). The accuracy of intramurality was low across all imaging modalities (17.95% echo, 29.49% CT and 21.79% 3D model). Accurate assessment of a separate AAOCA ostium was highest on 3D models (97.40%). Ostial stenosis was more accurately assessed on 3D models (56.41%). When accuracy was separated by subspecialty, CT and 3D models were more accurately assessed by all participants regardless of training.
CONCLUSIONS:
Cardiac imagers and congenital cardiothoracic surgeons most accurately assessed AAOCA presence, type and course on cardiac CT and 3D models. 3D models were superior in representation of ostial characteristics. CT and 3D models are overall more accurately assessed by specialists regardless of training.
Copyright © 2020. Published by Elsevier Inc.
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Modified Ozaki procedure including annular enlargement for small aortic annuli in young patients.
Ann Thorac Surg2020 May;():. doi: S0003-4975(20)30754-2.
Marathe Supreet P, Chávez Mariana, Sleeper Lynn A, Marx Gerald, Del Nido Pedro J, Baird Christopher W,
Abstract
BACKGROUND:
Aortic valve neo-cuspidization (AVNeo, Ozaki procedure) has excellent mid-term results in adults. Outcomes in patients with a small native aortic annulus are unknown. We report early outcomes in young patients with small native aortic valve annuli.
METHODS:
Retrospective review of patients undergoing AVNeo between 2015-2019 were reviewed. Patients with native aortic annulus ?21 mm undergoing 3-leaflet AVNeo were included.
RESULTS:
51 patients were identified (median age 7.9 years, weight 21 kg). 80% patients were ?12 years age. Pre-operative indication was AR (n=23), AS (n=22) or AS/AR (n=6). Baseline anatomy was: quadricuspid (n=1), tricuspid (n=23), bicuspid (n=15) or unicuspid (n=12) valve. Pre-operative peak gradient for AS and AS/AR patients was 55.36 mmHg. Median native annulus diameter was 17 mm; sinus and annular enlargements were required in 22 and 9 patients, respectively. Median ICU and hospital length of stay were 2.0 and 7.2 days, respectively. There were no re-interventions and one hospital mortality unrelated to aortic valve. At discharge, 94% of patients had ?mild AR and median peak gradient was 18 mmHg. At mean follow-up of 11.9 months, 80% and 82% of patients had0 at follow-up.
CONCLUSIONS:
The Ozaki procedure has acceptable short-term results in young patients with small aortic annuli. A larger aortic annulus can be achieved with surgical annular enlargement. Long-term follow-up is necessary to determine late valve function and potential continued annular growth.
Copyright © 2020. Published by Elsevier Inc.
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Dexmedetomidine for Prolonged Sedation in the PICU: A Systematic Review and Meta-Analysis.
Pediatr Crit Care Med2020 Mar;():. doi: 10.1097/PCC.0000000000002325.
Daverio Marco, Sperotto Francesca, Zanetto Lorenzo, Coscini Nadia, Frigo Anna Chiara, Mondardini Maria Cristina, Amigoni Angela,
Abstract
OBJECTIVES:
We aimed to systematically describe the use of dexmedetomidine as a treatment regimen for prolonged sedation in children and perform a meta-analysis of its safety profile.
DATA SOURCES:
PubMed, EMBASE, Cochrane Library, Scopus, Web of Science, ClinicalTrials.gov, and CINAHL were searched from inception to November 30, 2018.
STUDY SELECTION:
We included studies involving hospitalized critically ill patients less than or equal to 18 years old receiving dexmedetomidine for prolonged infusion (? 24 hr).
DATA EXTRACTION:
Data extraction included study characteristics, patient demographics, modality of dexmedetomidine use, associated analgesia and sedation details, comfort and withdrawal evaluation scales, withdrawal symptoms, and side effects.
DATA SYNTHESIS:
Literature search identified 32 studies, including a total of 3,267 patients. Most of the studies were monocentric (91%) and retrospective (88%); one was a randomized trial. Minimum and maximum infusion dosages varied from 0.1-0.5 µg/kg/hr to 0.3-2.5 µg/kg/hr, respectively. The mean/median duration range was 25-540 hours. The use of a loading bolus was reported in eight studies (25%) (range, 0.5-1 µg/kg), the mode of weaning in 11 (34%), and the weaning time in six of 11 (55%; range, 9-96 hr). The pooled prevalence of bradycardia was 2.6% (n = 10 studies; 14/387 patients; 95% CI, 0.3-7.3; I = 75%), the pooled prevalence incidence of bradycardia was 2.6% (n = 10 studies; 14/387 patients; 95% CI, 0.3-7.3; I = 75%), the pooled incidence of hypotension was 6.1% (n = 8 studies; 19/304 patients; 95% CI, 0.8-15.9; I = 84%). Three studies (9%) reported side effects' onset time which in all cases was within 12 hours of the infusion starting.
CONCLUSIONS:
High-quality data on dexmedetomidine use for prolonged sedation and a consensus on correct dosing and weaning protocols in children are currently missing. Infusion of dexmedetomidine can be considered relatively safe in pediatrics even when longer than 24 hours.
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Central Venous Catheter Utilization and Complications in the Pediatric Cardiac ICU: A Report From the Pediatric Cardiac Critical Care Consortium (PC4).
Pediatr Crit Care Med2020 Mar;():. doi: 10.1097/PCC.0000000000002306.
DiPietro Lisa M, Gaies Michael, Banerjee Mousumi, Donohue Janet E, Zhang Wenying, DeSena Holly C, Graham Eric M, Sasaki Jun, Moga Michael-Alice, Prodhan Parthak, Goldstein Stuart L, Tabbutt Sarah, Cooper David S,
Abstract
OBJECTIVES:
Current central venous catheter utilization in patients within pediatric cardiac ICUs is not well elucidated. We aim to describe current use of central venous catheters in a multi-institutional cohort and to explore the prevalence and risk factors for central line-associated thrombosis and central line-associated bloodstream infections.
DESIGN:
Observational analysis.
SETTING:
Pediatric Cardiac Critical Care Consortium hospitals.
PATIENTS:
Hospitalizations with at least one cardiac ICU admission from October 2013 to July 2016.
INTERVENTIONS:
None.
MEASUREMENTS AND MAIN RESULTS:
There were 17,846 hospitalizations and 69% included greater than or equal to one central venous catheter. Central venous catheter use was higher in younger patients (86% neonates). Surgical hospitalizations included at least one central venous catheter 88% of the time compared with 35% of medical hospitalizations. The most common location for central venous catheters was internal jugular (46%). Central venous catheters were in situ a median of 4 days (interquartile range, 2-10). There were 248 hospitalizations (2% overall, 1.8% medical, and 2.1% surgical) with at least one central line-associated thrombosis (271 total thromboses). Thrombosis was diagnosed at a median of 7 days (interquartile range, 4-14) after catheter insertion. There were 127 hospitalizations (1% overall, 1.4% medical, and 1% surgical) with at least one central line-associated bloodstream infection (136 total infections) with no association with catheter type or location. Central line-associated bloodstream infection was diagnosed at a median of 19 days (interquartile range, 8-36) after catheter insertion. Significant risk factors for central line-associated thrombosis and central line-associated bloodstream infection were younger age, greater surgical complexity, and total catheter days.
CONCLUSIONS:
Utilization of central venous catheters in pediatric cardiac ICUs differs according to indication for hospitalization. Although thrombosis and central line-associated bloodstream infection are infrequent complications of central venous catheter use in cardiac ICU patients, these events can have important short- and long-term consequences for patients. Total central venous catheter line days were the only modifiable risk factor identified. Future study must focus on understanding central venous catheter practices in high-risk patient subgroups that reduce the prevalence of thrombosis and central line-associated bloodstream infection.
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Methylene Blue for Refractory Shock in Children: A Systematic Review and Survey Practice Analysis.
Pediatr Crit Care Med2020 Mar;():. doi: 10.1097/PCC.0000000000002295.
Otero Luna Andrea V, Johnson Rachel, Funaro Melissa, Canarie Michael F, Pierce Richard W,
Abstract
OBJECTIVES:
Shock refractory to fluid and catecholamine therapy has significant morbidity and mortality in children. The use of methylene blue to treat refractory shock in children is not well described. We aim to collect and summarize the literature and define physicians' practice patterns regarding the use of methylene blue to treat shock in children.
DESIGN:
We conducted a systematic search of MEDLINE, Embase, PubMed, Web of Science, Cochrane for studies involving the use of methylene blue for catecholamine-refractory shock from database inception to 2019. Collected studies were analyzed qualitatively. To describe practice patterns of methylene blue use, we electronically distributed a survey to U.S.-based pediatric critical care physicians. We assessed physician knowledge and experience with methylene blue. Survey responses were quantitatively and qualitatively evaluated.
SETTING:
Pediatric critical and cardiac care units.
PATIENTS OR SUBJECTS:
Patients less than or equal to 25 years old with refractory shock treated with methylene blue.
INTERVENTIONS:
None.
MEASUREMENTS AND MAIN RESULTS:
One-thousand two-hundred ninety-three abstracts met search criteria, 139 articles underwent full-text review, and 24 studies were included. Studies investigated refractory shock induced by a variety of etiologies and found that methylene blue was generally safe and increased mean arterial blood pressure. There is overall lack of studies, low number of study patients, and low quality of studies identified. Our survey had a 22.5% response rate, representing 125 institutions. Similar proportions of physicians reported using (40%) or never even considering (43%) methylene blue for shock. The most common reasons for not using methylene blue were unfamiliarity with this drug, its proper dosing, and lack of evidentiary support.
CONCLUSIONS:
Methylene blue appears safe and may benefit children with refractory shock. There is a stark divide in familiarity and practice patterns regarding its use among physicians. Studies to formally assess safety and efficacy of methylene blue in treating pediatric shock are warranted.
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Novel frameshift variant in MYL2 reveals molecular differences between dominant and recessive forms of hypertrophic cardiomyopathy.
PLoS Genet2020 May;16(5):e1008639. doi: 10.1371/journal.pgen.1008639.
Manivannan Sathiya N, Darouich Sihem, Masmoudi Aida, Gordon David, Zender Gloria, Han Zhe, Fitzgerald-Butt Sara, White Peter, McBride Kim L, Kharrat Maher, Garg Vidu,
Abstract
Hypertrophic cardiomyopathy (HCM) is characterized by the thickening of the ventricular muscle without dilation and is often associated with dominant pathogenic variants in cardiac sarcomeric protein genes. Here, we report a family with two infants diagnosed with infantile-onset HCM and mitral valve dysplasia that led to death before one year of age. Using exome sequencing, we discovered that one of the affected children had a homozygous frameshift variant in Myosin light chain 2 (MYL2:NM_000432.3:c.431_432delCT: p.Pro144Argfs*57;MYL2-fs), which alters the last 20 amino acids of the protein and is predicted to impact the most C-terminal of the three EF-hand domains in MYL2. The parents are unaffected heterozygous carriers of the variant and the variant is absent in control cohorts from gnomAD. The absence of the phenotype in carriers and the infantile presentation of severe HCM is in contrast to HCM associated with dominant MYL2 variants. Immunohistochemical analysis of the ventricular muscle of the deceased patient with the MYL2-fs variant showed a marked reduction of MYL2 expression compared to an unaffected control. In vitro overexpression studies further indicate that the MYL2-fs variant is actively degraded. In contrast, an HCM-associated missense variant (MYL2:p.Gly162Arg) and three other MYL2 stop-gain variants (p.E22*, p.K62*, p.E97*) that result in loss of the EF domains are stably expressed but show impaired localization. The degradation of the MYL2-fs can be rescued by inhibiting the cell's proteasome function supporting a post-translational effect of the variant. In vivo rescue experiments with a Drosophila MYL2-homolog (Mlc2) knockdown model indicate that neither the MYL2-fs nor the MYL2:p.Gly162Arg variant supports normal cardiac function. The tools that we have generated provide a rapid screening platform for functional assessment of variants of unknown significance in MYL2. Our study supports an autosomal recessive model of inheritance for MYL2 loss-of-function variants in infantile HCM and highlights the variant-specific molecular differences found in MYL2-associated cardiomyopathy.
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