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Pubblicazioni recenti - pediatric cardiac
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Quality of life and comprehensive health supervision for children with Down syndrome in Thailand.
J Community Genet2020 Feb;():. doi: 10.1007/s12687-020-00458-4.
Rojnueangnit Kitiwan, Khaosamlee Penrawee, Chunsuwan Issarapa, Vorravanpreecha Nattariya, Lertboonnum Tanayoot, Rodjanadit Rungrote, Sriplienchan Pak,
Abstract
Children with Down syndrome often require several specialty doctors and multidisciplinary teams for their associated anomalies. This may impact their quality of life and creates gaps in treatment monitoring. No studies have yet been conducted in Thailand to measure their quality of life and level of comprehensive health supervision. Therefore, we aimed to study the quality of life among children with Down syndrome and determine if they receive comprehensive health supervision for their condition. In this descriptive research, data were collected from a medical record review of children with Down syndrome during a 1-year period in our Pediatric Outpatient Clinic; 50 children and 39 caregivers participated. Mean total quality of life score of the children was 67.9/100 points. The children had the highest scores (73.6?±?12.8) in emotional functioning and the lowest (57.2?±?25.6) in cognitive functioning. It appears that the quality of life may be lower in Down syndrome patients than in Thai children without it. Regarding health supervision, all 50 were screened for thyroid function, and 48 received cardiac evaluations. However, only 17 (34%) received "complete basic assessment" of 5 screening combinations with developmental evaluations and growth monitoring. Furthermore, none received "comprehensive" evaluations for all recommended conditions. While these findings show a need for health supervision improvement for children with Down syndrome within our hospital, they may also be indicative for most care facilities throughout Thailand.
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Assessment of out-of-field doses in radiotherapy treatments of paediatric patients using Monte Carlo methods and measurements.
Phys Med2020 Feb;71():53-61. doi: S1120-1797(20)30039-9.
Cravo Sá Ana, Barateiro Andreia, Bednarz Bryan, Borges Cecília, Pereira Joana, Baptista Mariana, Pereira Miguel, Zarza-Moreno Miriam, Almeida Pedro, Vaz Pedro, Madaleno Tiago, Romanets Yuriy,
Abstract
PURPOSE:
To assess out-of-field doses in radiotherapy treatments of paediatric patients, using Monte Carlo methods to implement a new model of the linear accelerator validated against measurements and developing a voxelized anthropomorphic paediatric phantom.
METHODS:
CT images of a physical anthropomorphic paediatric phantom were acquired and a dosimetric planning using a TPS was obtained. The CT images were used to perform the voxelization of the physical phantom using the ImageJ software and later implemented in MCNP. In order to validate the Monte Carlo model, dose measurements of the 6 MV beam and Linac with 120 MLC were made in a clinical setting, using ionization chambers and a water phantom. Afterwards TLD measurements in the physical anthropomorphic phantom were performed in order to assess the out-of-field doses in the eyes, thyroid, c-spine, heart and lungs.
RESULTS:
The Monte Carlo model was validated for in-field and out-of-field doses with average relative differences below 3%. The average relative differences between TLD measurements and Monte Carlo is 14,3% whilst the average relative differences between TLD and TPS is 55,8%. Moreover, organs up to 22.5 cm from PTV center show TLD and MCNP6 relative differences and TLD and TPS relative differences up to 21.2% and 92.0%, respectively.
CONCLUSIONS:
Our study provides a novel model that could be used in clinical research, namely in dose evaluation outside the treatment fields. This is particularly relevant, especially in pediatric patients, for studying new radiotherapy treatment techniques, since it can be used to estimate the development of secondary tumours.
Copyright © 2020. Published by Elsevier Ltd.
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Variability in chest compression rate calculations during pediatric cardiopulmonary resuscitation.
Resuscitation2020 Feb;():. doi: S0300-9572(20)30079-4.
Landis William P, Morgan Ryan W, Reeder Ron W, Graham Kathryn, Siems Ashley, Diddle J Wesley, Pollack Murray M, Maa Tensing, Fernandez Richard P, Yates Andrew R, Tilford Bradley, Ahmed Tageldin, Meert Kathleen L, Schneiter Carleen, Bishop Robert, Mourani Peter M, Naim Maryam Y, Friess Stuart, Burns Candice, Manga Arushi, Franzon Deborah, Tabbutt Sarah, McQuillen Patrick S, Horvat Christopher, Bochkoris Matthew, Carcillo Joseph A, Huard Leanna, Federman Myke, Sapru Anil, Viteri Shirley, Hehir David A, Notterman Daniel A, Holubkov Richard, Dean J Michael, Nadkarni Vinay M, Berg Robert A, Wolfe Heather A, Sutton Robert M, ,
Abstract
AIM:
The mathematical method used to calculate chest compression (CC) rate during cardiopulmonary resuscitation varies in the literature and across device manufacturers. The objective of this study was to determine the variability in calculated CC rates by applying four published methods to the same dataset.
METHODS:
This study was a secondary investigation of the first 200 pediatric cardiac arrest events with invasive arterial line waveform data in the ICU-RESUScitation Project (NCT02837497). Instantaneous CC rates were calculated during periods of uninterrupted CCs. The defined minimum interruption length affects rate calculation (e.g., if an interruption is defined as a break in CCs ? 2?s, the lowest possible calculated rate is 30?CCs/min). Average rates were calculated by four methods: 1) rate with an interruption defined as ? 1?s; 2) interruption ? 2?s; 3) interruption ? 3?s; 4) method #3 excluding top and bottom quartiles of calculated rates. American Heart Association Guideline-compliant rate was defined as 100-120?CCs/min. A clinically important change was defined as ±5?CCs/min. The percentage of events and epochs (30?s periods) that changed Guideline-compliant status was calculated.
RESULTS:
Across calculation methods, mean CC rates (118.7-119.5/min) were similar. Comparing all methods, 14 events (7%) and 114 epochs (6%) changed Guideline-compliant status.
CONCLUSION:
Using four published methods for calculating CC rate, average rates were similar, but 7% of events changed Guideline-compliant status. These data suggest that a uniform calculation method (interruption ? 1?s) should be adopted to decrease variability in resuscitation science.
Copyright © 2020. Published by Elsevier B.V.
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ISHLT consensus statement on donor organ acceptability and management in pediatric heart transplantation.
J Heart Lung Transplant2020 Jan;():. doi: S1053-2498(20)31363-2.
Kirk Richard, Dipchand Anne I, Davies Ryan R, Miera Oliver, Chapman Gretchen, Conway Jennifer, Denfield Susan, Gossett Jeffrey G, Johnson Jonathan, McCulloch Michael, Schweiger Martin, Zimpfer Daniel, Ablonczy László, Adachi Iki, Albert Dimpna, Alexander Peta, Amdani Shahnawaz, Amodeo Antonio, Azeka Estela, Ballweg Jean, Beasley Gary, Böhmer Jens, Butler Alison, Camino Manuela, Castro Javier, Chen Sharon, Chrisant Maryanne, Christen Urs, Danziger-Isakov Lara, Das Bibhuti, Everitt Melanie, Feingold Brian, Fenton Matthew, Garcia-Guereta Luis, Godown Justin, Gupta Dipankar, Irving Claire, Joong Anna, Kemna Mariska, Khulbey Sanjeev Kumar, Kindel Steven, Knecht Kenneth, Lal Ashwin K, Lin Kimberly, Lord Karen, Möller Thomas, Nandi Deipanjan, Niesse Oliver, Peng David M, Pérez-Blanco Alicia, Punnoose Ann, Reinhardt Zdenka, Rosenthal David, Scales Angie, Scheel Janet, Shih Renata, Smith Jonathan, Smits Jacqueline, Thul Josef, Weintraub Robert, Zangwill Steve, Zuckerman Warren A,
Abstract
The number of potential pediatric heart transplant recipients continues to exceed the number of donors, and consequently the waitlist mortality remains significant. Despite this, around 40% of all donated organs are not used and are discarded. This document (62 authors from 53 institutions in 17 countries) evaluates factors responsible for discarding donor hearts and makes recommendations regarding donor heart acceptance. The aim of this statement is to ensure that no usable donor heart is discarded, waitlist mortality is reduced, and post-transplant survival is not adversely impacted.
Copyright © 2020 International Society for Heart and Lung Transplantation. All rights reserved.
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Cardiac Magnetic Resonance Imaging Features in Hypertrophic Cardiomyopathy Diagnosed at <21 Years of Age.
Am J Cardiol2020 Jan;():. doi: S0002-9149(20)30068-0.
Bonura Erica D, Bos J Martijn, Abdelsalam Mahmoud A, Araoz Philip A, Ommen Steve R, Ackerman Michael J, Geske Jeffrey B,
Abstract
Hypertrophic cardiomyopathy (HC) is the most common inherited cardiomyopathy, with varied timing of phenotypic and clinical presentation. Literature describing cardiac magnetic resonance (CMR) imaging and late gadolinium enhancement (LGE) in young patients with HC is limited. This study included patients diagnosed with HC at young age (<21 years) between January 1990 and January 2015 who underwent transthoracic echocardiography and CMR with assessment of LGE at a single tertiary referral center. LGE was quantified via a method of 6 standard deviations and patients were grouped based upon presence or absence of LGE (?1% and >1% LGE, respectively). Sudden cardiac death (SCD) risk was assessed in patients >16 years of age using the European SCD risk score. A composite outcome of New York Heart Association class III-IV symptoms, aborted SCD, heart transplantation, and all-cause mortality was assessed via Kaplan-Meier curves with log-rank analysis. Overall, 126 patients were included (78 male; 62%). Median age of diagnosis was 15 (12 to 18) years. LGE was present in 81 (64%) patients, although only 4 (3%) patients had LGE >15%. Median age at CMR imaging was 19 (15 to 23) years. Patients with LGE had greater wall thickness (25 ± 8 mm vs 22 ± 7 mm, p?=?0.01). Median European SCD risk score was 4.7 (2.9 to 6.5). Median follow-up was 6.5 (2.5 to 13) years with 26 patients (21%) meeting the composite outcome. There were no significant differences in composite outcome since age of diagnosis when stratified by presence/absence of LGE (p?=?1.0). The presence of LGE in young HC patients was not an independent risk factor for cardiovascular morbidity and mortality. Wall thickness was greater in patients with LGE. There remains a need for further evaluation of this unique HC cohort.
Copyright © 2020 Elsevier Inc. All rights reserved.
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Understanding adherence to the CardioMEMS pulmonary artery pressure monitoring system for heart failure: A qualitative study.
Heart Lung2020 Feb;():. doi: S0147-9563(20)30011-X.
Haynes Sarah C, Pallin Rocco, Tong Kathleen, Henderson Stuart, Romano Patrick S,
Abstract
BACKGROUND:
The CardioMEMS HF system is a remote monitoring device that allows patients to transmit pulmonary artery pressure readings to providers, who are alerted when pressures increase above the patient's normal threshold.
OBJECTIVES:
The purpose of this study was to gain a better understanding of patient adherence to the CardioMEMS system and to compare patients' self-reported use of the system with actual adherence.
METHODS:
We conducted semi-structured interviews with patients who had a CardioMEMS device. Transcripts were analyzed using a combination of structural and process coding.
RESULTS:
Patients engaged with the CardioMEMS system in a reciprocal manner, continually learning from the feedback provided by the system. Self-concept influenced the way that patients adhere.
CONCLUSIONS:
Patients experience many benefits of the CardioMEMS system; however, it is not clear that these perceived or real benefits affect how a person adheres. Rather, our findings suggest that a person's self-concept may be a better predictor of adherence to telemonitoring systems.
Copyright © 2020 Elsevier Inc. All rights reserved.
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A case of bi-ventricular extensive calcification caused by multiple factors.
BMC Pediatr2020 Feb;20(1):85. doi: 10.1186/s12887-020-1973-x.
Tu Xiaoyan, Hu Zhihui, Yang Kevin, Hu Zhuoqing, Jiang Yi,
Abstract
BACKGROUND:
Extensive myocardial calcification has a low incidence rate, but when the patients do have extensive myocardial cases, the prognosis is usually poor. Several sepsis-related extensive myocardial calcification cases have been reported, but there are cases of biventricular calcifications that are caused by multiple cases besides bacteremia and the treatment for it has a low percentage of success.
CASE PRESENTATION:
A 9 year old girl had an extensive biventricular calcification which is caused by multiple factors including multiple organ failure (heart, lung, liver, and kidney), aseptic cardiomyopathy, systemic inflammatory response syndrome, pulmonary hemorrhage, viral encephalitis. In this case study, the massive myocardial calcification present in the patient was classified as dystrophic. After the patient was transferred to the Intensive care unit, a series of rescue treatments such as anti-inflammatory factor storm were implemented to protect the organs. In the end, the patient was rescued from the rescue treatment procedure. After 18?months of follow-up, it was observed that the patient's heart function returned to normal and it was observed that there was no change in myocardial calcification in the patient.
CONCLUSION:
In this case study, it showcased a case of the diffused biventricular calcification that caused by multiple factors. Furthermore, the precise role of calcification on cardiac function was largely unknown and there has to be further follow-up observation on the patient.
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Identification of Risk Factors for Early Fontan Failure.
Semin Thorac Cardiovasc Surg2020 Feb;():. doi: S1043-0679(20)30033-2.
Rochelson Ellis, Richmond Marc E, LaPar Damien J, Torres Alejandro, Anderson Brett R,
Abstract
Despite improvements in operative and perioperative care, the risk of significant morbidity and mortality for children undergoing Fontan procedures persists. Previous investigations have identified peri-Fontan characteristics that may predict early adverse events. The purpose of this study was to identify characteristics from throughout a patient's lifespan, including all perioperative stages, that might predict early Fontan failure - defined as death, Fontan takedown, or listing for cardiac transplantation before hospital discharge or within 30 postoperative days. A single-center retrospective study of all patients undergoing a Fontan procedure was performed. Patient and intervention-related characteristics were examined from birth through Fontan. Data were described using standard summary statistics. Univariable, logistic regression was used to examine associations with early Fontan failure. In total, 191 patients met inclusion criteria. The incidence of early Fontan failure was 4% (n?=?8: six deaths, two Fontan takedowns). Neonatal balloon atrial septostomy was the only patient characteristic significantly associated with Fontan failure. Patients who underwent balloon septostomy had 8.5-times higher odds of Fontan failure (CI 2.6 - 28.1, p < 0.001) than those who did not. Children who require balloon septostomy as neonates remain at higher risk of Fontan takedown, listing for heart transplantation, or death in the early post-Fontan period.
Copyright © 2020. Published by Elsevier Inc.
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Null: A novel ?-antitrypsin allele with in cis variants Glu366Lys and Ile100Asn.
Clin Biochem2020 Feb;():. doi: S0009-9120(19)31122-1.
Chen Sharon, DeMarco Mari L, Estey Mathew P, Kyle Barry, Parker Michelle L, Agbor Terence A, Kawada Patricia, Speevak Marsha, Nelson Tanya N, Mattman Andre,
Abstract
BACKGROUND:
?-Antitrypsin (A1AT) deficiency predisposes patients to pulmonary disease due to inadequate protection against human neutrophil elastase released during inflammatory responses. A1AT deficiency is caused by homozygosity or compound heterozygosity for A1AT variants; individuals with A1AT deficiency most commonly have at least one Z variant allele (c.1096G>A (Glu366Lys)). Null variants that result in complete absence of A1AT in the plasma are much rarer. With one recent exception, all reported A1AT variants are characterized by a single pathogenic variant.
CASE:
An 8 years old patient from Edmonton, Alberta, Canada, was investigated for A1AT deficiency. His A1AT phenotype was determined to be M (wild type)/Null by isoelectric focusing (IEF) but M/Z by targeted genotyping. Gene sequencing revealed two heterozygous variants: Z and Ile100Asn (c.299T>A). The Ile100Asn substitution is predicted to disrupt the secondary structure of an ?-helix in which it resides and the neighbouring tertiary structure, resulting in intracellular degradation of A1AT prior to hepatocyte secretion.
METHODS:
Family testing was conducted to verify potential inheritance of an A1AT allele carrying the two mutations in cis, as this arrangement of the mutations would explain "Z" detection by genotyping but not by IEF. Molecular modeling was used to assess the effect of the variants on A1AT structure and stability.
DISCUSSION:
Carrier status for a novel variant Null with in cis mutations (c.[299T>A;1096G>A], p.[(Ileu100Asn;Glu366Lys)]) was confirmed. A sibling was identified as having A1AT deficiency on the basis of compound heterozygosity for two alleles: Null and the common Z allele. A separate pedigree from the Maritimes was subsequently recognized as carrying Null.
CONCLUSION:
In cis mutations such as Null may be more common than previously described due to failure to detect such mutations using historical testing methods. Combined approaches that include gene sequencing and segregation studies allow recognition of rare A1AT variants, including in cis mutations.
Copyright © 2020. Published by Elsevier Inc.
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Growth hormone treatment in a patient with deletion of the long arm of chromosome 18: An 8-year observation.
Neuro Endocrinol Lett2019 Nov;40(4):169-174.
Jackowski Tomasz, Petriczko El?bieta, Horodnicka-Jozwa Anita, Biczysko-Mokosa Agnieszka, Szalecki Mieczys?aw, Walczak Mieczys?aw,
Abstract
BACKGROUND:
Deletion of the long (q) arm of chromosome 18 causes a rare genetic disease termed 18q- syndrome. This syndrome has varying clinical presentation, depending on the extent of the deletion and the percentage of cells with abnormal chromosomes. One of the most common disorders in children affected by the disease is short stature, usually associated with growth hormone deficiency. Numerous reports on patients with 18q- syndrome show growth hormone treatment has significant therapeutic benefits, not only in terms of final body height but also cognitive functions and psychosocial development.
CASE PRESENTATION:
Here we describe the case of a 10-year-old girl with 18q- syndrome treated with recombinant human growth hormone from the age of 2. This is the first report of such a patient in Poland. After 8 years of observation, the child showed a clear benefit from recombinant human growth hormone treatment in terms of height and possibly mental development. The girl remains under cardiac care due to congenital heart disease and under neurological care for epilepsy.
CONCLUSIONS:
This case indicates the need for early diagnosis and multidisciplinary action to achieve satisfactory quality of life in patients with 18q- syndrome.
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Chemical Cardioversion of Atrial Flutter and Fibrillation in the Pediatric Population with Ibutilide.
Pacing Clin Electrophysiol2020 Feb;():. doi: 10.1111/pace.13890.
Dasgupta Soham, Dave Ishaan, Whitehill Robert, Fischbach Peter,
Abstract
INTRODUCTION:
Atrial flutter (AFL) and atrial fibrillation (AF) are common in pediatric patients with congenital heart disease (CHD) and structurally normal hearts as well. Chemical cardioversion is attractive for patients with AFL/AF for a short period of time because of the ability to avoid sedation. We review a single center's experience with Ibutilide in pediatric patients in an effort to report on its safety and efficacy.
METHODS:
We performed a retrospective chart review of pediatric patients (0-21 years) who underwent chemical cardioversion for AFL/AF with Ibutilide (January 2011-February 2019). Patients on another anti-arrhythmic medication or attempted chemical cardioversion with another drug were excluded.
RESULTS:
There were 21 patients who met inclusion criteria. 13/21 (62%) patients were successfully cardioverted with ibutilide (10/13 had AF and 4/13 required a 2 dose). There were no significant differences in baseline characteristics between those who were successfully cardioverted compared to those who were not. Administration of magnesium prior to administration did not appear to have an effect on the success rate. There was a significant increase in QTc intervals post Ibutilide administration which returned to baseline prior to discharge. One patient had symptomatic bradycardia needing intravenous fluids and another had torsades requiring electrical cardioversion during Ibutilide administration.
CONCLUSIONS:
The success rate of chemical cardioversion with Ibutilide was similar in our experience as compared to studies in the adult population and the other lone pediatric study. While adverse events were uncommon, Ibutilide administration warrants close monitoring and fully defining its efficacy warrants further pediatric experience. This article is protected by copyright. All rights reserved.
This article is protected by copyright. All rights reserved.
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Alteration of CTCF-associated chromatin neighborhood inhibits TAL1-driven oncogenic transcription program and leukemogenesis.
Nucleic Acids Res2020 Feb;():. doi: gkaa098.
Li Ying, Liao Ziwei, Luo Huacheng, Benyoucef Aissa, Kang Yuanyuan, Lai Qian, Dovat Sinisa, Miller Barbara, Chepelev Iouri, Li Yangqiu, Zhao Keji, Brand Marjorie, Huang Suming,
Abstract
Aberrant activation of the TAL1 is associated with up to 60% of T-ALL cases and is involved in CTCF-mediated genome organization within the TAL1 locus, suggesting that CTCF boundary plays a pathogenic role in T-ALL. Here, we show that -31-Kb CTCF binding site (-31CBS) serves as chromatin boundary that defines topologically associating domain (TAD) and enhancer/promoter interaction required for TAL1 activation. Deleted or inverted -31CBS impairs TAL1 expression in a context-dependent manner. Deletion of -31CBS reduces chromatin accessibility and blocks long-range interaction between the +51 erythroid enhancer and TAL1 promoter-1 leading to inhibition of TAL1 expression in erythroid cells, but not T-ALL cells. However, in TAL1-expressing T-ALL cells, the leukemia-prone TAL1 promoter-IV specifically interacts with the +19 stem cell enhancer located 19 Kb downstream of TAL1 and this interaction is disrupted by the -31CBS inversion in T-ALL cells. Inversion of -31CBS in Jurkat cells alters chromatin accessibility, histone modifications and CTCF-mediated TAD leading to inhibition of TAL1 expression and TAL1-driven leukemogenesis. Thus, our data reveal that -31CBS acts as critical regulator to define +19-enhancer and the leukemic prone promoter IV interaction for TAL1 activation in T-ALL. Manipulation of CTCF boundary can alter TAL1 TAD and oncogenic transcription networks in leukemogenesis.
© The Author(s) 2020. Published by Oxford University Press on behalf of Nucleic Acids Research.
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Inequalities in cardiovascular risks among Swedish adolescents (ABIS): a prospective cohort study.
BMJ Open2020 Feb;10(2):e030613. doi: 10.1136/bmjopen-2019-030613.
Andersson White Pär, Ludvigsson Johnny, Jones Michael P, Faresjo Tomas,
Abstract
OBJECTIVES:
To investigate if socioeconomic status (SES) is predictive of cardiovascular risk factors among Swedish adolescents. Identify the most important SES variable for the development of each cardiovascular risk factor. Investigate at what age SES inequality in overweight and obesity occurs.
DESIGN:
Longitudinal follow-up of a prospective birth cohort.
SETTING:
All Babies in Southeast Sweden (ABIS) study includes data from children born between October 1997 and October 1999 in five counties of south east Sweden.
PARTICIPANTS:
A regional ABIS-study subsample from three major cities of the region n=298 adolescents aged 16-18 years, and prospective data from the whole ABIS cohort for overweight and obesity status at the ages 2, 5, 8 and 12 years (n=2998-7925).
OUTCOME MEASURES:
Blood pressure above the hypertension limit, overweight/obesity according to the International Obesity Task Force definition, low high-density lipoproteins (HDL) or borderline-high low-density lipoproteins according to National Cholesterol Education Program expert panel on cholesterol levels in children.
RESULTS:
For three out of four cardiovascular risk outcomes (elevated blood pressure, low HDL and overweight/obesity), there were increased risk in one or more of the low SES groups (p<0.05). The best predictor was parental occupational class (Swedish socioeconomic classification index) for elevated blood pressure (area under the receiver operating characteristic (ROC) curve 0.623), maternal educational level for overweight (area under the ROC curve 0.641) and blue-collar city of residence for low HDL (area under the ROC curve 0.641). SES-related differences in overweight/obesity were found at age 2, 5 and 12 and for obesity at age 2, 5, 8 and 12 years (all p<0.05).
CONCLUSIONS:
Even in a welfare state like Sweden, SES inequalities in cardiovascular risks are evident already in childhood and adolescence. Intervention programmes to reduce cardiovascular risk based on social inequality should start early in life.
© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY. Published by BMJ.
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Nationwide Frequency, Sequential Trends, and Impact of Co-morbid Mental Health Disorders on Hospitalizations, Outcomes, and Healthcare Resource Utilization in Adult Congenital Heart Disease.
Am J Cardiol2020 Jan;():. doi: S0002-9149(20)30065-5.
Desai Rupak, Patel Krupa, Dave Hitanshu, Shah Kaushal, DeWitt Natalie, Fong Hee Kong, Varma Yash, Varma Kaksha, Mansuri Zeeshan, Sachdeva Rajesh, Khanna Amber, Kumar Gautam,
Abstract
Despite the growing prevalence of adult congenital heart disease (ACHD), data on trends in prevalence of mental health disorders (MHD) among patients with ACHD remain limited. The National Inpatient Sample (2007 to 2014) was queried to identify the frequency and trends of MHD among ACHD hospitalizations (stratification by age, sex, and race); demographics and co-morbidities for ACHD cohorts, with (MHD+) versus without MHD (MHD-); the rate and trends of all-cause in-hospital mortality, disposition, mean length of stay, and hospitalization charges among both cohorts. A total of 11,709 (13.8%, mean age: 49.1 years, 56.0% females, 78.7% white) out of 85,029 ACHD patient encounters had a coexistent MHD (anxiety, depression, mood disorder, or psychosis). ACHD-MHD+ cohort was more often admitted nonelectively (38.1% vs 32.8%, p <0.001) and had a higher frequency of cardiac/extra-cardiac co-morbidities. The trends in prevalence of coexistent MHD increased from 10.3% to 17.5% (70% relative increase) from 2007 to 2014 with a consistently higher prevalence among females (from 13% to 20.3%) compared to males (from 7.6% to 15.5%) (p <0.001). The hospitalization trends with MHD increased in whites (12.1% to 19.8%) and Hispanics (5.9% to 12.7%). All-cause mortality was lower (0.7% vs 1.1%, p?=?0.002) in ACHD-MHD+; however, mean length of stay (?5.7 vs 4.9 days, p <0.001) was higher without significant difference in charges ($97,710 vs $96,058, p?=?0.137). ACHD-MHD+ cohort was less often discharged routinely (declining trend) and more frequently transferred to other facilities and required home healthcare (rising trends). In conclusion, this study reveals increasing trends of MHD, healthcare resource utilization and a higher frequency of co-morbidities in patients with ACHD.
Copyright © 2020 Elsevier Inc. All rights reserved.
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Proposed pathway for patients undergoing enhanced recovery after spinal surgery: protocol for a systematic review.
Syst Rev2020 Feb;9(1):39. doi: 10.1186/s13643-020-1283-2.
Licina Ana, Silvers Andrew, Laughlin Harry, Russell Jeremy, Wan Crispin,
Abstract
BACKGROUND:
The best evidence-enhanced recovery care pathway is yet to be defined for patients undergoing spinal surgery. Minimally invasive surgery, multimodal analgesia, early mobilization, and early postoperative nutrition have been considered as critical components of enhanced recovery in spinal surgery (ERSS). The objective of this study will be to synthesize the evidence underpinning individual components of a proposed multidisciplinary enhanced recovery pathway for patients undergoing spinal surgery.
METHODS:
This is the study protocol for a systematic review of complex interventions. Our team identified 22 individual care components of a proposed pathway based on clinical practice guidelines and published reviews. We will include systematic reviews and meta-analysis, randomized controlled trials, non-randomized controlled trials, and observational studies in adults or pediatric patients evaluating any one of the pre-determined care components. Our primary outcomes will be all-cause mortality, morbidity outcomes (e.g., pulmonary, cardiac, renal, surgical complications), patient-reported outcomes and experiences (e.g., pain, quality of care experience), and health services outcomes (e.g., length of stay and costs). We will search the following databases (1990 onwards) MEDLINE, EMBASE, and Cochrane Library (Cochrane Database of Systematic Reviews and CENTRAL). Two reviewers will independently screen all citations, full-text articles, and abstract data. Potential conflicts will be resolved through discussion. The risk of bias for individual studies will be appraised using appropriate tools. A narrative synthesis will be provided with the information presented in the text and tables to summarize and explain the characteristics and findings of the included studies. Due to clinical and methodological heterogeneity, we do not anticipate to conduct meta-analyses. Confidence in cumulative evidence for each component of care will be classified according to the GRADE system.
DISCUSSION:
This systematic review will identify, evaluate, and integrate the evidence underpinning individual components of a pathway for patients undergoing spinal surgery. The formation of an evidence-based pathway will allow for the standardization of clinical care delivery within the context of enhanced recovery in spinal surgery.
SYSTEMATIC REVIEW REGISTRATION:
PROSPERO CRD42019135289.
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Ghrelin protects against obesity-induced myocardial injury by regulating the lncRNA H19/miR-29a/IGF-1 signalling axis.
Exp Mol Pathol2020 Feb;():104405. doi: S0014-4800(19)30778-6.
Liu Yang, Xu Xin-Yue, Shen Yang, Ye Chun-Feng, Hu Na, Yao Qing, Lv Xiu-Zi, Long Sheng-Lan, Ren Chao, Lang Yuan-Yuan, Liu Yan-Ling,
Abstract
BACKGROUND:
Obesity is associated with the impairment of cardiac fitness and consequent ventricular dysfunction and heart failure. Ghrelin has been largely documented to be cardioprotective against ischaemia/reperfusion injury. However, the role of ghrelin in obesity-induced myocardial injury is largely unknown. This study sought to determine the cardiac effect of ghrelin against obesity-induced injury and the underlying mechanisms.
METHODS:
The effect of ghrelin was evaluated in a mouse model of obesity and a palmitic acid (PA)-treated cardiomyocyte cell line with or without ghrelin transfection. Gene and protein expression levels were determined by real-time PCR and western blot, respectively. Cell apoptosis was measured by flow cytometry analysis.
RESULTS:
In the present study, we found that both a high-fat diet (HFD) and PA treatment caused myocardial injury by increasing apoptosis and the expression of inflammatory cytokines. Overexpression of ghrelin reversed the effects induced by HFD or PA treatment. Knockdown of lncRNA H19 or overexpression of miR-29a abrogated the cardioprotective effects of ghrelin against apoptosis and inflammation. We also found that IGF-1 was a target gene of miR-29a and that H19 regulated IGF-1 expression via miR-29a. Overexpression of IGF-1 partially reversed the apoptosis and inflammation promoting effects of miR-29a.
CONCLUSIONS:
Our findings suggested that ghrelin protected against obesity-induced myocardial injury by regulating the H19/miR-29a/IGF-1 signalling axis, providing further evidence for the clinical application of ghrelin.
Copyright © 2020. Published by Elsevier Inc.
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Pulmonary Artery Reconstruction Following Failed Pulmonary Artery Stents.
Ann Thorac Surg2020 Feb;():. doi: S0003-4975(20)30208-3.
Mainwaring Richard D, Collins R Thomas, MacMillen Kirstie L, Palmon Michal, Hanley Frank L,
Abstract
BACKGROUND:
Pulmonary artery stents are widely deployed in patients with stenoses ; in the branch pulmonary arteries. However, stents do not address more peripheral sites of ; stenosis and invariably develop in-stent restenosis. The purpose of this study was to review our ; experience with pulmonary artery reconstruction following failed pulmonary artery stents.
METHODS:
This was a retrospective study of 56 patients who underwent pulmonary artery reconstruction following failed pulmonary artery stents. These patients had undergone a median of two previous surgical procedures (range 0-5) and two pulmonary artery stents (range 1-4).
RESULTS:
The median age at stent surgery was 5 years (range 0.3 to 23.6 years). The majority of stents (79%) were completely removed and patch augmented. The minority of stents (21%) were felt to be unremovable and thus were split longitudinally and reconstructed using; pulmonary artery homograft. There was one operative mortality (1.8%). The mean pulmonary artery-to-aortic pressure ratio decreased from a pre-operative value of 0.91 ± 0.21 to a post-operative value of 0.31 ± 0.07 (p < 0.001). The median hospital length of stay was 10 days. The median duration of follow-up is 1.8 years. There has been no mid-term mortality. Six patients have undergone balloon dilation post-operatively for residual pulmonary artery stenosis.
CONCLUSIONS:
Pulmonary artery reconstruction resulted in a significant decrease in pulmonary artery-to-aortic pressure ratios. The subsequent need for re-intervention on the ; pulmonary arteries has been relatively low (11% to date). These results suggest that patients with ; pulmonary artery stents can be successfully treated with surgical reconstruction.
Copyright © 2020. Published by Elsevier Inc.
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Prenatal hypoxia increases susceptibility to kidney injury.
PLoS One2020 ;15(2):e0229618. doi: 10.1371/journal.pone.0229618.
Cargill Kasey R, Chiba Takuto, Murali Anjana, Mukherjee Elina, Crinzi Elizabeth, Sims-Lucas Sunder,
Abstract
Prenatal hypoxia is a gestational stressor that can result in developmental abnormalities or physiological reprogramming, and often decreases cellular capacity against secondary stress. When a developing fetus is exposed to hypoxia, blood flow is preferentially redirected to vital organs including the brain and heart over other organs including the kidney. Hypoxia-induced injury can lead to structural malformations in the kidney; however, even in the absence of structural lesions, hypoxia can physiologically reprogram the kidney leading to decreased function or increased susceptibility to injury. Our investigation in mice reveals that while prenatal hypoxia does not affect normal development of the kidneys, it primes the kidneys to have an increased susceptibility to kidney injury later in life. We found that our model does not develop structural abnormalities when prenatally exposed to modest 12% O2 as evident by normal histological characterization and gene expression analysis. Further, adult renal structure and function is comparable to mice exposed to ambient oxygen throughout nephrogenesis. However, after induction of kidney injury with a nephrotoxin (cisplatin), the offspring of mice housed in hypoxia exhibit significantly reduced renal function and proximal tubule damage following injury. We conclude that exposure to prenatal hypoxia in utero physiologically reprograms the kidneys leading to increased susceptibility to injury later in life.
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Feasibility of selective cardiac ventricular electroporation.
PLoS One2020 ;15(2):e0229214. doi: 10.1371/journal.pone.0229214.
Sugrue Alan, Vaidya Vaibhav R, Livia Christopher, Padmanabhan Deepak, Abudan Anas, Isath Ameesh, Witt Tyra, DeSimone Christopher V, Stalboerger Paul, Kapa Suraj, Asirvatham Samuel J, McLeod Christopher J,
Abstract
INTRODUCTION:
The application of brief high voltage electrical pulses to tissue can lead to an irreversible or reversible electroporation effect in a cell-specific manner. In the management of ventricular arrhythmias, the ability to target different tissue types, specifically cardiac conduction tissue (His-Purkinje System) vs. cardiac myocardium would be advantageous. We hypothesize that pulsed electric fields (PEFs) can be applied safely to the beating heart through a catheter-based approach, and we tested whether the superficial Purkinje cells can be targeted with PEFs without injury to underlying myocardial tissue.
METHODS:
In an acute (n = 5) and chronic canine model (n = 6), detailed electroanatomical mapping of the left ventricle identified electrical signals from myocardial and overlying Purkinje tissue. Electroporation was effected via percutaneous catheter-based Intracardiac bipolar current delivery in the anesthetized animal. Repeat Intracardiac electrical mapping of the heart was performed at acute and chronic time points; followed by histological analysis to assess effects.
RESULTS:
PEF demonstrated an acute dose-dependent functional effect on Purkinje, with titration of pulse duration and/or voltage associated with successful acute Purkinje damage. Electrical conduction in the insulated bundle of His (n = 2) and anterior fascicle bundle (n = 2), was not affected. At 30 days repeat cardiac mapping demonstrated resilient, normal electrical conduction throughout the targeted area with no significant change in myocardial amplitude (pre 5.9 ± 1.8 mV, 30 days 5.4 ± 1.2 mV, p = 0.92). Histopathological analysis confirmed acute Purkinje fiber targeting, with chronic studies showing normal Purkinje fibers, with minimal subendocardial myocardial fibrosis.
CONCLUSION:
PEF provides a novel, safe method for non-thermal acute modulation of the Purkinje fibers without significant injury to the underlying myocardium. Future optimization of this energy delivery is required to optimize conditions so that selective electroporation can be utilized in humans the treatment of cardiac disease.
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Dobutamine stress testing in patients with Fontan circulation augmented by biomechanical modeling.
PLoS One2020 ;15(2):e0229015. doi: 10.1371/journal.pone.0229015.
Ruijsink Bram, Zugaj Konrad, Wong James, Pushparajah Kuberan, Hussain Tarique, Moireau Philippe, Razavi Reza, Chapelle Dominique, Chabiniok Radomír,
Abstract
Understanding (patho)physiological phenomena and mechanisms of failure in patients with Fontan circulation-a surgically established circulation for patients born with a functionally single ventricle-remains challenging due to the complex hemodynamics and high inter-patient variations in anatomy and function. In this work, we present a biomechanical model of the heart and circulation to augment the diagnostic evaluation of Fontan patients with early-stage heart failure. The proposed framework employs a reduced-order model of heart coupled with a simplified circulation including venous return, creating a closed-loop system. We deploy this framework to augment the information from data obtained during combined cardiac catheterization and magnetic resonance exams (XMR), performed at rest and during dobutamine stress in 9 children with Fontan circulation and 2 biventricular controls. We demonstrate that our modeling framework enables patient-specific investigation of myocardial stiffness, contractility at rest, contractile reserve during stress and changes in vascular resistance. Hereby, the model allows to identify key factors underlying the pathophysiological response to stress in these patients. In addition, the rapid personalization of the model to patient data and fast simulation of cardiac cycles make our framework directly applicable in a clinical workflow. We conclude that the proposed modeling framework is a valuable addition to the current clinical diagnostic XMR exam that helps to explain patient-specific stress hemodynamics and can identify potential mechanisms of failure in patients with Fontan circulation.
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