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PURPOSE: While sleep apnea (SA) gets more prevalent with advancing age, the impact of age on the association between SA and health outcomes is not well known. We assessed the association between the severity of SA and all-cause mortality in different age groups using large longitudinal data. METHOD: We applied a Natural Language Processing pipeline to extract the apnea-hypopnea index (AHI) from the physicians' interpretation of sleep studies performed at the Veteran Health Administration (FY 1999-2022). We categorized the participants as no SA (n-SA, AHI< 5) and severe SA (s-SA, AHI≥30). We grouped the cohort based on age: Young≤40; Middle-aged:40-65; and Older adults≥65; and calculated the odds ratio (aOR) of mortality adjusted for age, sex, race, ethnicity, BMI, and Charlson-Comorbidity Index (CCI) using n-SA as the reference. RESULTS: We identified 146,148 participants (age 52.23 ± 15.02; BMI 32.11 ± 6.05; male 86.7 %; White 66 %). Prevalence of s-SA increased with age. All-cause mortality was lower in s-SA compared to n-SA in the entire cohort (aOR,0.56; 95%CI: 0.54,0.58). Comparing s-SA to n-SA, the all-cause mortality rates (Young 1.86 % vs 1.49 %; Middle-aged 12.07 % vs 13.34 %; and Older adults 26.35 % vs 40.18 %) and the aOR diminished as the age increased (Young: 1.11, 95%CI: 0.93-1.32; Middle-aged: 0.64, 95%CI: 0.61-0.67; and Older adults: 0.44, 95%CI: 0.41-0.46). CONCLUSION: The prevalence of severe SA increased while the odds of all-cause mortality compared to n-SA diminished with age. SA may exert less harmful effects on the aged population. A causality analysis is warranted to assess the relationship between SA, aging, and all-cause mortality.
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STUDY OBJECTIVES: Excessive daytime sleepiness (EDS) is prevalent and overwhelmingly stems from disturbed sleep. We hypothesized that age modulates the association between EDS and increased all-cause mortality. METHODS: We utilized the Veterans' Health Administration data from 1999-2022. We enrolled participants with sleep related ICD9/10 codes or sleep services. A natural language processing (NLP) pipeline was developed and validated to extract the Epworth Sleepiness Scale (ESS) as a self-reported tool to measure EDS from physician progress notes. The NLP's accuracy was assessed through manual annotation of 470 notes. Participants were categorized into Normal-ESS, n-ESS, (ESS 0-10) and high-ESS, h-ESS, (ESS 11-24). We created three age groups: < 50 years; 50 to < 65 years; and ≥ 65 years. The adjusted odds ratio (aOR) of mortality was calculated for age, BMI, sex, race, ethnicity, and the Charlson Comorbidity Index (CCI), using n-ESS as the reference. Subsequently, we conducted age stratified analysis. RESULTS: The first ESS records were extracted from 423,087 veterans with a mean age of 54.8 (±14.6), mean BMI of 32.6 (±6.2), and 90.5% male. The aOR across all ages was 17% higher (1.15,1.19) in the h-ESS category. The aORs only became statistically significant for individuals aged ≥ 50 years in the h-ESS compared to the n-ESS category (< 50 years: 1.02 [0.96,1.08], 50 to < 65 years 1.13[1.10,1.16]; ≥ 65 years: 1.25 [1.21-1.28]). CONCLUSIONS: High ESS, predicted increased mortality only in participants aged 50 and older. Further research is required to identify this differential behavior in relation to age.
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We investigated the accuracy of International Classification of Diseases (ICD) codes for the identification of veterans with rapid eye movement sleep behavior disorder. The charts of 139 randomly sampled veterans with ≥ 1 ICD-9 and ICD-10 code(s) for rapid eye movement sleep behavior disorder were reviewed for documentation of a suspected, previous, or current diagnosis; clinical symptoms; and/or empiric treatments for this disorder. Notably, 71 (51.1%) patients with rapid eye movement sleep behavior disorder electronic diagnoses had not undergone polysomnography, and 29 (20.9%) had polysomnography reports without commentary on rapid eye movement sleep without atonia. Sleep centers are therefore encouraged to include a brief sentence in polysomnography report templates commenting on the presence/absence of rapid eye movement sleep without atonia. CITATION: Jones MB, Schenck CH, Azarian M, Jorge RE, Sharafkhaneh A, Razjouyan J. Validation of electronic diagnostic codes for rapid eye movement sleep behavior disorder. J Clin Sleep Med. 2024;20(8);1387-1389.
Subject(s)
International Classification of Diseases , Polysomnography , REM Sleep Behavior Disorder , Veterans , Humans , REM Sleep Behavior Disorder/diagnosis , REM Sleep Behavior Disorder/physiopathology , Male , Polysomnography/methods , Female , Veterans/statistics & numerical data , Middle Aged , Reproducibility of Results , Aged , AdultABSTRACT
OBJECTIVE: Posttraumatic stress disorder (PTSD) and traumatic brain injury (TBI), which are prevalent conditions among post-9/11 veterans, increase risks of rapid eye movement (REM) sleep behavior disorder (RBD) and degenerative synucleinopathy. Rates and predictors of RBD symptoms were investigated by screening post-9/11 veterans for RBD with a validated questionnaire. METHODS: In this cross-sectional analysis, consecutive patients in the Houston Translational Research Center for TBI and Stress Disorders (TRACTS) were screened with the English translation of the RBD Questionnaire-Hong Kong (RBDQ-HK). In addition to data from the standard TRACTS battery, systematic chart review was used to identify known sleep disorders mimicking or manifesting RBD. RESULTS: Of the 119 patients with available RBDQ-HK scores, 71 (60%) and 65 (55%) screened positive for RBD, when a total score ≥21 and a factor 2 score ≥8 were used as cutoff scores, respectively. Univariable analyses with both cutoffs showed consistent associations between a positive RBDQ-HK screen and global sleep quality, number of TBI exposures, and PTSD severity. Multivariable logistic regression with total score ≥21 as a cutoff indicated that PTSD severity (odds ratio=1.06, 95% CI=1.02-1.10) and number of TBIs (odds ratio=1.63, 95% CI=1.16-2.41) were independent predictors of a positive screen, whereas global sleep quality was no longer significant. Multivariable logistic regression with factor 2 score ≥8 as a cutoff showed similar results. CONCLUSIONS: Interdisciplinary parasomnia assessment, further validation of RBD screens, and standardized reporting of REM sleep without atonia could provide necessary information on the pathophysiological relationships linking PTSD, TBI, RBD symptoms, and ultimately synucleinopathy risk among post-9/11 veterans.
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BACKGROUND: Physical violence and aggression (PVA), defined as behaviors with the potential to cause bodily injury, are unfortunate risks in the management of all-cause neurodegenerative dementias. While dementia in Parkinson's disease (PD) may not be evident for many years after clinical onset, neuropsychiatric disturbances occur at all stages of the disease. At issue is whether PVA in PD is associated with clinical factors that can be targets for prevention and management in the absence of a prevailing dementia syndrome. OBJECTIVE: This systematic review examined the extent to which PVA in PD without dementia is a clinically significant concern and whether it is associated with factors that could warrant proactive management. METHODS: A systematic search of 9 electronic databases used MeSH headings and equivalent terms for PD, aggression, and violence. Eligible manuscripts were original articles that were published in peer-reviewed journals and reported on adults with PD in the awake state with PVA as possible outcomes. Extracted data included study design, PD ascertainment methods and characteristics, PVA assessment methods, subject demographics, psychiatric and medical comorbidities, and pertinent results. Inciting and confounding factors were extracted from case reports. Quality assessment tools were applied in accordance with the study design (e.g., observational, qualitative, or case report). RESULTS: The search identified 10 manuscripts: 2 observational quantitative studies (total n with PD = 545), 1 qualitative study (n with PD = 20), and 7 case reports (n = 7). The observational studies suggested that PVA is less common than other neuropsychiatric disturbances, but heterogeneous methods and quality concerns prevented further conclusions. In the case reports, all patients were male, and most were early onset. In 6 of the reports, PVA occurred in the context of bilateral subthalamic nucleus deep brain stimulation. CONCLUSIONS: PVA, while relatively rare in PD, can be a significant management issue that is associated with select premorbid characteristics and antiparkinsonian motor treatments. As PVA may be under-reported, further understanding of its frequency, causes, risk factors, and outcomes would benefit from its systematic assessment, ideally using self-report and informant-based questionnaires.
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OBJECTIVE: A systematic review was conducted to answer whether adult-onset post-traumatic stress disorder (PTSD) is associated with increased risk of Parkinson's disease (PD) and related synucleinopathies. DESIGN: A systematic search of Medline (Ovid), Embase (Elsevier), PsycInfo (Ovid), Cochrane Library (Wiley), and Web of Science (Clarivate) was performed using MeSH headings and equivalent terms for PTSD, PD, DLB, and related disorders. SETTING: No restrictions. PARTICIPANTS: Eligible articles were published in peer-reviewed journals, sampled adult human populations, and treated PTSD and degenerative synucleinopathies as exposures and outcomes, respectively. MEASUREMENTS: Extracted data included diagnostic methods, sample characteristics, matching procedures, covariates, and effect estimates. Bias assessment was performed with the Newcastle-Ottawa scale. Hazard ratios were pooled using the random effects model, and the Hartung-Knapp adjustment was applied due to the small number of studies. RESULTS: A total of six articles comprising seven unique samples (total n = 1,747,378) met eligibility criteria. The risk of PD was reported in three retrospective cohort studies and one case-control study. Risk of DLB was reported in one retrospective cohort, one case-control, and one prospective cohort study. No studies addressed potential relationships with multiple system atrophy or pure autonomic failure. Meta-analysis of hazard ratios from four retrospective cohort studies supported the hypothesis that incident PTSD was associated with PD and DLB risk (pooled HR 1.88, 95% C.I. 1.08-3.24; p = 0.035). CONCLUSIONS: The sparse literature to-date supports further investigations on the association of mid- to late-life PTSD with Parkinson's and related neurodegenerative disorders.
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Infants and children born with CHD are at significant risk for neurodevelopmental delays and abnormalities. Individualised developmental care is widely recognised as best practice to support early neurodevelopment for medically fragile infants born premature or requiring surgical intervention after birth. However, wide variability in clinical practice is consistently demonstrated in units caring for infants with CHD. The Cardiac Newborn Neuroprotective Network, a Special Interest Group of the Cardiac Neurodevelopmental Outcome Collaborative, formed a working group of experts to create an evidence-based developmental care pathway to guide clinical practice in hospital settings caring for infants with CHD. The clinical pathway, "Developmental Care Pathway for Hospitalized Infants with Congenital Heart Disease," includes recommendations for standardised developmental assessment, parent mental health screening, and the implementation of a daily developmental care bundle, which incorporates individualised assessments and interventions tailored to meet the needs of this unique infant population and their families. Hospitals caring for infants with CHD are encouraged to adopt this developmental care pathway and track metrics and outcomes using a quality improvement framework.
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Critical Pathways , Heart Defects, Congenital , Infant, Newborn , Infant , Child , Humans , Public Opinion , Heart Defects, Congenital/complications , Heart Defects, Congenital/therapy , Heart Defects, Congenital/diagnosisABSTRACT
Neuroimaging is widely utilized in studying traumatic brain injury (TBI) and post-traumatic stress disorder (PTSD). The risk for PTSD is greater after TBI than after non-TBI trauma, and PTSD is associated with worse outcomes after TBI. Studying the neuroimaging correlates of TBI-related PTSD may provide insights into the etiology of both conditions and help identify those TBI patients most at risk of developing persistent symptoms. The objectives of this systematic review were to examine the current literature on neuroimaging in TBI-related PTSD, summarize key findings, and highlight strengths and limitations to guide future research. A Preferred Reporting Items for Systematic Review and Meta-Analysis Protocols (PRISMA) compliant literature search was conducted in PubMed (MEDLINE®), PsycINFO, Embase, and Scopus databases prior to January 2022. The database query yielded 4486 articles, which were narrowed based on specified inclusion criteria to a final cohort of 16 studies, composed of 854 participants with TBI. There was no consensus regarding neuroimaging correlates of TBI-related PTSD among the included articles. A small number of studies suggest that TBI-related PTSD is associated with white matter tract changes, particularly in frontotemporal regions, as well as changes in whole-brain networks of resting-state connectivity. Future studies hoping to identify reliable neuroimaging correlates of TBI-related PTSD would benefit from ensuring consistent case definition, preferably with clinician-diagnosed TBI and PTSD, selection of comparable control groups, and attention to imaging timing post-injury. Prospective studies are needed and should aim to further differentiate predisposing factors from sequelae of TBI-related PTSD.
Subject(s)
Brain Injuries, Traumatic , Stress Disorders, Post-Traumatic , Humans , Stress Disorders, Post-Traumatic/diagnostic imaging , Stress Disorders, Post-Traumatic/etiology , Brain Injuries, Traumatic/complications , Brain Injuries, Traumatic/diagnostic imaging , Neuroimaging , BrainABSTRACT
BACKGROUND: Behavioral and emotional dyscontrol commonly occur following traumatic brain injury (TBI). Neuroimaging and electrophysiological correlates of dyscontrol have not been systematically summarized in the literature to date. OBJECTIVE: To complete a systematic review of the literature examining neuroimaging and electrophysiological findings related to behavioral and emotional dyscontrol due to TBI. METHODS: A Preferred Reporting Items for Systematic Reviews and Meta-Analyses-compliant literature search was conducted in PubMed (MEDLINE), PsycINFO, EMBASE, and Scopus databases prior to May 2019. The database query yielded 4392 unique articles. These articles were narrowed based on specific inclusion criteria (e.g., clear TBI definition, statistical analysis of the relationship between neuroimaging and dyscontrol). RESULTS: A final cohort of 24 articles resulted, comprising findings from 1552 patients with TBI. Studies included civilian (n = 12), military (n = 10), and sport (n = 2) samples with significant variation in the severity of TBI incorporated. Global and region-based structural imaging was more frequently used to study dyscontrol than functional imaging or diffusion tensor imaging. The prefrontal cortex was the most common neuroanatomical region associated with behavioral and emotional dyscontrol, followed by other frontal and temporal lobe findings. CONCLUSIONS: Frontal and temporal lesions are most strongly implicated in the development of postinjury dyscontrol symptoms although they are also the most frequently investigated regions of the brain for these symptom categories. Future studies can make valuable contributions to the field by (1) emphasizing consistent definitions of behavioral and emotional dyscontrol, (2) assessing premorbid dyscontrol symptoms in subjects, (3) utilizing functional or structural connectivity-based imaging techniques, or (4) restricting analyses to more focused brain regions.
Subject(s)
Brain Injuries, Traumatic , Brain Injuries , Humans , Diffusion Tensor Imaging , Brain Injuries, Traumatic/diagnostic imaging , Neuroimaging , Emotions , Brain Injuries/pathologyABSTRACT
BACKGROUND: Hospital readmissions and outpatient visits contribute to the significant burden on healthcare systems. Obstructive sleep apnea (OSA) is a chronic medical condition that is associated with cardiovascular comorbidities and other chronic conditions. Inpatient and outpatient healthcare utilization rates in patients with OSA following hospitalization are unclear. METHODS: This. retrospective case-control cohort study utilized a nationwide database to assess if OSA is associated with higher healthcare utilization post-hospitalization. We compared healthcare utilization among patients with OSA versus without OSA between 2009 and 2014 after matching for demographic variables, geographic location, hospital environment, reason for admission, and severity of illness during hospitalization. We measured future healthcare utilization by the number of ICU admissions, hospital admissions, emergency room visits, and outpatient visits after being discharged from the index hospitalization. RESULTS: Two equal-sized cohorts comprised of 85,912 matched pairs were obtained. The OSA cohort demonstrated significantly higher rates of future ICU admissions, hospital admissions, emergency room visits, and outpatient visits. Matching for comorbid cardiovascular conditions continued to demonstrate higher healthcare utilization in the OSA group. Short-term outcomes during the index hospitalization were relatively similar between groups. CONCLUSIONS: This retrospective database study demonstrated that OSA may be an independent marker of higher future healthcare utilization. On the other hand, the length of stay during the index hospitalization was not elevated. Prospective studies are needed to confirm these findings and investigate the impact of directing additional resources to inpatients with OSA.
Subject(s)
Patient Readmission , Sleep Apnea, Obstructive , Case-Control Studies , Humans , Outpatients , Retrospective Studies , Sleep Apnea, Obstructive/complications , Sleep Apnea, Obstructive/epidemiology , Sleep Apnea, Obstructive/therapyABSTRACT
The investigators aimed to draw attention to current debates surrounding the etiologies of dream enactment behaviors in patients with posttraumatic stress disorder (PTSD). The phenomenological overlap between PTSD-related nocturnal symptoms, rapid eye movement sleep behavior disorder (RBD), and trauma-associated sleep disorder (TASD) is discussed. Strategies used to diagnose and manage dream enactment behaviors, whether due to RBD or another confounding sleep disorder, are considered. Finally, the need for further research on the pathophysiological overlap and integrated treatment of PTSD, RBD, and, possibly, TASD is highlighted.
Subject(s)
REM Sleep Behavior Disorder , Sleep Wake Disorders , Stress Disorders, Post-Traumatic , Humans , REM Sleep Behavior Disorder/complications , REM Sleep Behavior Disorder/diagnosis , Sleep , Stress Disorders, Post-Traumatic/complicationsABSTRACT
BACKGROUND: Traumatic brain injury (TBI) can precipitate new-onset psychiatric symptoms or worsen existing psychiatric conditions. To elucidate specific mechanisms for this interaction, neuroimaging is often used to study both psychiatric conditions and TBI. This systematic review aims to synthesize the existing literature of neuroimaging findings among patients with anxiety after TBI. METHODS: We conducted a Preferred Reporting Items for Systematic Review and Meta-Analyses-compliant literature search via PubMed (MEDLINE), PsychINFO, EMBASE, and Scopus databases before May, 2019. We included studies that clearly defined TBI, measured syndromal anxiety as a primary outcome, and statistically analyzed the relationship between neuroimaging findings and anxiety symptoms. RESULTS: A total of 5982 articles were retrieved from the systematic search, of which 65 studied anxiety and 13 met eligibility criteria. These studies were published between 2004 and 2017, collectively analyzing 764 participants comprised of 470 patients with TBI and 294 non-TBI controls. Imaging modalities used included magnetic resonance imaging, functional magnetic resonance imaging, diffusion tensor imaging, electroencephalogram, magnetic resonance spectrometry, and magnetoencephalography. Eight of 13 studies presented at least one significant finding and together reflect a complex set of changes that lead to anxiety in the setting of TBI. The left cingulate gyrus in particular was found to be significant in 2 studies using different imaging modalities. Two studies also revealed perturbances in functional connectivity within the default mode network. CONCLUSIONS: This is the first systemic review of neuroimaging changes associated with anxiety after TBI, which implicated multiple brain structures and circuits, such as the default mode network. Future research with consistent, rigorous measurements of TBI and syndromal anxiety, as well as attention to control groups, previous TBIs, and time interval between TBI and neuroimaging, are warranted. By understanding neuroimaging correlates of psychiatric symptoms, this work could inform future post-TBI screening and surveillance, preventative efforts, and early interventions to improve neuropsychiatric outcomes.
Subject(s)
Brain Injuries, Traumatic , Diffusion Tensor Imaging , Anxiety/diagnostic imaging , Anxiety/etiology , Brain Injuries, Traumatic/complications , Brain Injuries, Traumatic/diagnostic imaging , Humans , Magnetic Resonance Imaging , Neuroimaging/methodsABSTRACT
Treatment options for Achromobacter xylosoxidans are limited. Eight cystic fibrosis patients with A. xylosoxidans were treated with 12 cefiderocol courses. Pretreatment in vitro resistance was seen in 3 of 8 cases. Clinical response occurred after 11 of 12 treatment courses. However, microbiologic relapse was observed after 11 of 12 treatment courses, notably without emergence of resistance.
Subject(s)
Achromobacter denitrificans , Cystic Fibrosis , Gram-Negative Bacterial Infections , Adult , Anti-Bacterial Agents/therapeutic use , Cephalosporins , Child , Cystic Fibrosis/complications , Cystic Fibrosis/drug therapy , Gram-Negative Bacterial Infections/drug therapy , Humans , CefiderocolABSTRACT
The roles played by nurse practitioners and physician assistants have expanded exponentially during the last decade. Although existing professional advancement models for nurse practitioners/physician assistants have led to enhanced integration in different patient care units, the development of a distinctive professional identity formation is lacking. This perspective proposes a new framework to guide the planning of an educational program that provides not only clinical knowledge and technical skills but also opportunities for enhancing leadership and research skills, along with strong career mentorship. Such a program will lead to formation of a distinctive identity for critical care nurse practitioners and physician assistants, which in turn can improve job satisfaction and employee retention.
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Nurse Practitioners , Physician Assistants , Critical Care , Humans , Job Satisfaction , LeadershipABSTRACT
OBJECTIVE: Veterans with posttraumatic stress disorder (PTSD) frequently report dream enactment behavior (DEB). Although DEBs are associated with PTSD symptoms, their relationship with other sleep disorders, including REM behavior disorder, warrants reexamination of their clinical correlates. METHODS: The investigators used a cross-sectional, exploratory analysis to compare demographic and clinical characteristics of veterans endorsing regularly occurring DEB compared with those endorsing no or infrequent DEB. The participants comprised a convenience sample of servicemembers who were previously deployed to Operation Enduring Freedom/Operation Iraqi Freedom/Operation New Dawn (OEF/OIF/OND) and enrolled in an ongoing cohort study. RESULTS: Of the 78 eligible participants, 19 (24.4%) endorsed DEBs occurring at least once per week in the past month. Compared with participants who reported no or infrequent DEBs, participants with regularly occurring DEBs had poorer sleep quality, greater PTSD severity, a higher number of reported mild traumatic brain injuries (mTBI) with loss of consciousness, and a higher likelihood of being diagnosed with sleep disorders. After adjustment for global sleep quality, a significant association persisted between DEBs and the number of mTBI with loss of consciousness but not between DEBs and the severity of PTSD symptoms. CONCLUSIONS: These results suggest that mTBI may disrupt neural circuits regulating sleep among OIF/OEF/OND veterans. Prospective, polysomnographic assessment of muscle tone and behavioral events during REM sleep is needed to characterize the physiology of DEBs in this population.
Subject(s)
Brain Concussion/epidemiology , Combat Disorders/epidemiology , Dreams , Parasomnias/epidemiology , Sleep Initiation and Maintenance Disorders/epidemiology , Stress Disorders, Post-Traumatic/epidemiology , Unconsciousness/epidemiology , Veterans/statistics & numerical data , Adult , Afghan Campaign 2001- , Cross-Sectional Studies , Humans , Iraq War, 2003-2011 , Male , United States/epidemiologyABSTRACT
As survivable outcomes among patients with complex congenital heart disease (cCHD) have continued to improve over the last several decades, more attention is being dedicated to interventions that impact not just survival but quality of life among patients with cCHD. In particular, patients with cCHD are at risk for impaired neurodevelopmental outcomes. In this review summarizing select presentations given at the 14th Annual Pediatric Cardiac Intensive Care Society's Annual Meeting in 2019, we discuss the neurodevelopmental phenotype of patients with cCHD, patients at greatest risk of impaired development, and three specific modifiable risk factors impacting development.
Subject(s)
Heart Defects, Congenital/complications , Neurodevelopmental Disorders/etiology , Quality of Life , Risk Assessment , Child , Global Health , Heart Defects, Congenital/psychology , Humans , Incidence , Neurodevelopmental Disorders/epidemiology , Neurodevelopmental Disorders/psychology , Risk FactorsABSTRACT
The care of children with hypoplastic left heart syndrome is constantly evolving. Prenatal diagnosis of hypoplastic left heart syndrome will aid in counselling of parents, and selected fetuses may be candidates for in utero intervention. Following birth, palliation can be undertaken through staged operations: Norwood (or hybrid) in the 1st week of life, superior cavopulmonary connection at 4-6 months of life, and finally total cavopulmonary connection (Fontan) at 2-4 years of age. Children with hypoplastic left heart syndrome are at risk of circulatory failure their entire life, and selected patients may undergo heart transplantation. In this review article, we summarise recent advances in the critical care management of patients with hypoplastic left heart syndrome as were discussed in a focused session at the 12th International Conference of the Paediatric Cardiac Intensive Care Society held on 9 December, 2016, in Miami Beach, Florida.
Subject(s)
Fontan Procedure/methods , Hypoplastic Left Heart Syndrome , Palliative Care , Prenatal Diagnosis/methods , Child, Preschool , Female , Global Health , Humans , Hypoplastic Left Heart Syndrome/diagnosis , Hypoplastic Left Heart Syndrome/epidemiology , Hypoplastic Left Heart Syndrome/surgery , Infant , Infant, Newborn , Morbidity/trends , Pregnancy , Survival Rate/trendsABSTRACT
BACKGROUND: Acquired von Willebrand syndrome (AvWS) in the setting of congenital heart disease is an under-recognized cause of bleeding in the pediatric cardiac critical care unit. METHODS: Fourteen patients diagnosed with AvWS admitted to the cardiac intensive care unit at the Children's National Health System between December 2009 and September 2015 were identified with subsequent chart review and case analysis. RESULTS: Of the 14 patients included in this study, 4 patients were on ventricular-assist devices, 6 patients were on extracorporeal membrane oxygenation, and 4 were patients with congenital heart disease not receiving any mechanical circulatory support. All patients identified manifested persistent severe bleeding, despite appropriate management of anticoagulation and blood product administration based on the established protocols. Detailed hemostatic testing including quantitative von Willebrand factor (vWF) multimer analysis revealed decreased high-molecular-weight multimers (HMWMs) and absent ultra-HMWM, consistent with AvWS in all patients. Eight patients received treatment with vWF concentrate, one patient with desmopressin, and five recovered without specific treatment. Bleeding ceased in all but one patient. CONCLUSIONS: Acquired von Willebrand syndrome is an uncommon but important cause of bleeding in pediatric patients with cardiac disease. A high index of clinical suspicion with knowledge of the characteristic clinical scenario in addition to low levels of vWF multimers is required to manage and diagnose AvWS. Although the optimal management of AvWS in this patient population is unclear, vWF concentrates are available and appear to be efficacious for controlling life-threatening bleeding.
Subject(s)
Cardiac Surgical Procedures/adverse effects , Heart Defects, Congenital/surgery , Intensive Care Units , Postoperative Hemorrhage/etiology , von Willebrand Diseases/complications , Adolescent , Child , Child, Preschool , Female , Heart Defects, Congenital/complications , Heart-Assist Devices/adverse effects , Humans , Infant , Infant, Newborn , Male , Postoperative Hemorrhage/blood , Postoperative Hemorrhage/diagnosis , von Willebrand Diseases/blood , von Willebrand Diseases/diagnosis , von Willebrand Factor/metabolismABSTRACT
OBJECTIVE: The objectives of this review are to describe the education and critical thinking skills that characterize pediatric critical care nursing and how these skills impact patient care and outcomes in pediatric cardiac critical care. DATA SOURCE: MEDLINE and PubMed. CONCLUSIONS: Pediatric cardiac critical care nurses manage complex and vulnerable patients requiring various levels of support. Effective care of these patients requires knowledge about the complex anatomy and physiology associated with congenital and acquired heart disease, as well as the effects of mechanical ventilation, mechanical circulatory support, and vasoactive medications. Strong physical examination skills, accurate interpretation of hemodynamic and laboratory data, active participation in bedside rounds, excellent communication skills, meticulous care of invasive monitoring catheters and tubes, and compassionate support of families are among the skills that distinguish a cardiac critical care nurse.