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1.
Martin S. Maron Winnie Xin Katherine B. Sims Rita Butler Tammy S. Haas Ethan J. Rowin Robert J. Desnick Barry J. Maron 《The American journal of medicine》2018,131(2):200.e1-200.e8
Background
Fabry disease is an X-linked lysosomal storage disorder caused by the deficient activity of α-galactosidase A due to mutations in the GLA gene, which may be associated with increased left ventricular wall thickness and mimic the morphologic features of hypertrophic cardiomyopathy. Management strategies for these 2 diseases diverge, with Fabry disease–specific treatment utilizing recombinant α-galactosidase A enzyme replacement therapy.Methods
We studied a prospectively assembled consecutive cohort of 585 patients (71% male) from 2 hypertrophic cardiomyopathy tertiary referral centers by screening for low α-galactosidase A activity in dried blood spots. Male patients with low α-galactosidase A activity levels and all females were tested for mutations in the GLA gene.Results
In 585 patients previously diagnosed with hypertrophic cardiomyopathy, we identified 2 unrelated patients (0.34%), both with the GLA mutation encoding P.N215S, the most common mutation causing later-onset Fabry disease phenotype. These patients were both asymptomatic, a man aged 53 years and a woman aged 69 years, and demonstrated a mild cardiac phenotype with symmetric distribution of left ventricular hypertrophy. After family screening, a total of 27 new Fabry disease patients aged 2-81 years were identified in the 2 families, including 12 individuals who are now receiving enzyme replacement therapy.Conclusions
These observations support consideration for routine prospective screening for Fabry disease in all patients without a definitive etiology for left ventriclar hypertrophy. This strategy would likely result, through cascade family testing, in the earlier identification of new Fabry disease–affected males and female heterozygotes who may benefit from monitoring and/or enzyme replacement therapy. 相似文献2.
Iliana S. Hurtado Rendón Diego Alcivar Juan Pablo Rodriguez-Escudero Kevin Silver 《The American journal of medicine》2018,131(2):202-205
Background
Stress cardiomyopathy is a transient cardiac syndrome characterized by reversible left ventricular systolic dysfunction precipitated by emotional or physiologic stress. The presence of obstructive coronary artery disease has been noted in stress cardiomyopathy.Methods
We describe 3 case reports of patients with acute coronary syndrome and transient wall motion abnormalities not usually seen in the distribution of coronary artery disease.Results
In these 3 cases of acute myocardial infarction, the distribution of the culprit coronary occlusion was not concordant with the territory of transient wall motion abnormality. Follow-up demonstrated resolution of the wall motion abnormalities without intervention in these territories.Conclusion
We believe that the physiologic stress of the acute coronary syndrome may have precipitated the stress cardiomyopathy as presented by these patients. This is the first demonstration that stress cardiomyopathy may be precipitated by acute coronary syndrome. 相似文献3.
Sophie Wells Ethan J. Rowin Griffin Boll Hassan Rastegar Wendy Wang Martin S. Maron Barry J. Maron 《The American journal of medicine》2018,131(6):e235-e239
Background
Surgical myectomy reverses heart failure symptoms in the vast majority of obstructive hypertrophic cardiomyopathy patients. However, a small subgroup fails to experience sustained postoperative improvement despite relief of obstruction. Clinical profile of such patients has not been well defined.Methods
Consecutive obstructive hypertrophic cardiomyopathy patients undergoing myectomy at Tufts Medical Center for drug-refractory New York Heart Association III/IV heart failure symptoms, 2004 to 2017, were followed postoperatively for 2.5 ± 2.8 years and assessed for outcome.Results
Of the 503 patients, there were 4 postoperative deaths (0.8%); 480 patients (96%) had sustained improvement to New York Heart Association classes I or II (responders), but 19 (3.8%) developed advanced symptoms (classes III or IV) in the absence of obstruction (nonresponders). Compared with responders, nonresponders were younger (40 ± 13 vs 53 ± 14 years; P < .001) and had greater septal thickness (25 ± 9 vs 20 ± 4 mm; P < .001). Massive hypertrophy (≥30 mm) was 5-fold more common in nonresponders (P < .01). Seven nonresponders developed systolic dysfunction (ejection fraction 20%-47%), 2 days to 6.1 years postoperatively. Four nonresponders underwent heart transplant 3.4 to 9.2 years after myectomy, and 2 others have been listed.Conclusions
Surgical myectomy is highly effective at reversing heart failure symptoms in the vast majority of patients with obstructive hypertrophic cardiomyopathy. However, a small minority experience persistent functional limitation despite surgical relief of outflow obstruction. Predictors of adverse postoperative course were substantial/massive septal thickness and youthful age. Patients who failed to respond symptomatically to myectomy were considered for advanced heart failure treatment, including heart transplantation. 相似文献4.
5.
Mona A. Abed Nidal F. Eshah Debra K. Moser 《Heart & lung : the journal of critical care》2018,47(3):226-230
Background
In developing countries, the number of adults who develop myocardial infarction (MI) at a young age is high. The popularity of waterpipe smoking (WPS) has increased among the same age group. It is unknown if WPS contributes to the incidence of early-onset MI.Objective
To study the association of WPS with early-onset MI, which is defined as first MI occurring in individuals 18 ≥ age ≤ 45 years compared to those older than 45 years.Methods
This was a cross-sectional study. The association of WPS with first-time MI was compared between younger and older adults (N = 225).Results
Twenty-five percent of all participants developed an acute MI before the age of 46 years. Both cigarette and WPS were more common among younger first-time MI patients than older first-time MI patients.Conclusions
WPS is one risk factor that distinguishes the risk profile of young adults with early-onset MI. 相似文献6.
Morgan E. Whitaker Vineet Nair Shripad Sinari Parinita A. Dherange Balaji Natarajan Lindsey Trutter Evan L. Brittain Anna R. Hemnes Eric D. Austin Kumar Patel Stephen M. Black Joe G.N. Garcia Jason X. Yuan MD PhD Rebecca R. Vanderpool Franz Rischard Ayako Makino Edward J. Bedrick Ankit A. Desai 《The American journal of medicine》2018,131(6):702.e7-702.e13
Background
Diabetes mellitus is associated with left ventricular hypertrophy and dysfunction. Parallel studies have also reported associations between diabetes mellitus and right ventricular dysfunction and reduced survival in patients with pulmonary arterial hypertension. However, the impact of diabetes mellitus on the pulmonary vasculature has not been well characterized. We hypothesized that diabetes mellitus and hyperglycemia could specifically influence right ventricular afterload and remodeling in patients with Group I pulmonary arterial hypertension, providing a link to their known susceptibility to right ventricular dysfunction.Methods
Using an adjusted model for age, sex, pulmonary vascular resistance, and medication use, associations of fasting blood glucose, glycated hemoglobin, and the presence of diabetes mellitus were evaluated with markers of disease severity in 162 patients with pulmonary arterial hypertension.Results
A surrogate measure of increased pulmonary artery stiffness, elevated pulmonary arterial elastance (P = .012), along with reduced log(pulmonary artery capacitance) (P = .006) were significantly associated with the presence of diabetes mellitus in patients with pulmonary arterial hypertension in a fully adjusted model. Similar associations between pulmonary arterial elastance and capacitance were noted with both fasting blood glucose and glycated hemoglobin. Furthermore, right ventricular wall thickness on echocardiography was greater in pulmonary arterial hypertension patients with diabetes, supporting the link between right ventricular remodeling and diabetes.Conclusion
Cumulatively, these data demonstrate that an increase in right ventricular afterload, beyond pulmonary vascular resistance alone, may influence right ventricular remodeling and provide a mechanistic link between the susceptibility to right ventricular dysfunction in patients with both diabetes mellitus and pulmonary arterial hypertension. 相似文献7.
Morgan Humphrey Sonia Everhart Desiree Kosmisky William E. Anderson 《Heart & lung : the journal of critical care》2018,47(4):387-391
Background
Sedation of mechanically ventilated patients should optimize comfort and safety while avoiding over-sedation and adverse outcomes. To our knowledge, characteristics associated with attaining target sedation are unknown.Objectives
Evaluate current sedation practice at a single center and explore which patient characteristics are associated with attaining target sedation.Methods
This is a single-center, retrospective chart review of sedated, ventilated patients in a medical/surgical ICU. Demographic and clinical data were collected. Univariate and multivariate logistic regression analyses were used with attaining target sedation as the dependent variable.Results
Of the 100 patients included (median 60.5 years), 50 attained target sedation. Univariate analyses (a = 0.10) revealed factors associated with target sedation were age (P = 0.08), history of alcohol abuse (P = 0.08), multiple comorbidities (P = 0.09), and delirium monitoring (P = 0.002). Multivariate analysis revealed an association between delirium monitoring/documentation and attaining target sedation (P = 0.005; OR 9.2; 95% CI 2.3–36.8).Conclusions
Patients without appropriate delirium monitoring/documentation had significantly reduced likelihood of achieving target sedation. 相似文献8.
Marilyn Schallom Donna Prentice Carrie Sona Cassandra Arroyo John Mazuski 《Heart & lung : the journal of critical care》2018,47(2):93-99
Background
In critically ill patients, clinicians can have difficulty obtaining accurate oximetry measurements.Objective
To compare the accuracy of nasal alar and forehead sensor measurements and incidence of pressure injury.Methods
43 patients had forehead and nasal alar sensors applied. Arterial samples were obtained at 0, 24, and 120 hours. Oxygen saturations measured by co-oximetry were compared to sensor values. Skin was assessed every 8 hours.Results
Oxygen saturations ranged from 69.8%-97.8%, with 18% of measures < 90%. Measurements were within 3% of co-oximetry values for 54% of nasal alar compared to 35% of forehead measurements. Measurement failures occurred in 6% for nasal alar and 22% for forehead. Three patients developed a pressure injury with the nasal alar sensor and 13 patients developed a pressure injury with the forehead sensor (χ2 = 7.68; p = .006).Conclusions
In this group of patients with decreased perfusion, nasal alar sensors provided a potential alternative for continuous monitoring of oxygen saturation. 相似文献9.
Nancy M. Albert James F. Bena Denise Buxbaum Linda Martensen Shannon L. Morrison Marilyn A. Prasun Kelly D. Stamp 《Heart & lung : the journal of critical care》2018,47(3):184-191
Background
Research findings on the value of nurse certification were based on subjective perceptions or biased by correlations of certification status and global clinical factors. In heart failure, the value of certification is unknown.Objectives
Examine the value of certification based nurses' decision-making.Methods
Cross-sectional study of nurses who completed heart failure clinical vignettes that reflected decision-making in clinical heart failure scenarios. Statistical tests included multivariable linear, logistic and proportional odds logistic regression models.Results
Of nurses (N = 605), 29.1% were heart failure certified, 35.0% were certified in another specialty/job role and 35.9% were not certified. In multivariable modeling, nurses certified in heart failure (versus not heart failure certified) had higher clinical vignette scores (p = 0.002), reflecting higher evidence-based decision making; nurses with another specialty/role certification (versus no certification) did not (p = 0.62).Conclusions
Heart failure certification, but not in other specialty/job roles was associated with decisions that reflected delivery of high-quality care. 相似文献10.
Zachary L. Cox Pikki Lai Connie M. Lewis JoAnn Lindenfeld Sean P. Collins Daniel J. Lenihan 《Heart & lung : the journal of critical care》2018,47(4):290-296
Background
Nationally-derived models predicting 30-day readmissions following heart failure (HF) hospitalizations yield insufficient discrimination for institutional use.Objective
Develop a customized readmission risk model from Medicare-employed and institutionally-customized risk factors and compare the performance against national models in a medical center.Methods
Medicare patients age ≥ 65 years hospitalized for HF (n = 1,454) were studied in a derivation cohort and in a separate validation cohort (n = 243). All 30-day hospital readmissions were documented. The primary outcome was risk discrimination (c-statistic) compared to national models.Results
A customized model demonstrated improved discrimination (c-statistic 0.72; 95% CI 0.69 – 0.74) compared to national models (c-statistics of 0.60 and 0.61) with a c-statistic of 0.63 in the validation cohort. Compared to national models, a customized model demonstrated superior readmission risk profiling by distinguishing a high-risk (38.3%) from a low-risk (9.4%) quartile.Conclusions
A customized model improved readmission risk discrimination from HF hospitalizations compared to national models. 相似文献11.
Ubolrat Piamjariyakul Noreen C. Thompson Christy Russell Carol E. Smith 《Heart & lung : the journal of critical care》2018,47(3):211-215
Background
African Americans with heart failure (HF) have the highest rates of depression among all ethnicities in the USA.Objectives
To compare the effects by race on depressive symptoms and topics discussed in the first clinic appointment after HF hospitalization.Methods
This study is a secondary analysis of data from a randomized clinical trial testing a patient group discussion of HF self-management with 93 Caucasians and 77 African Americans.Results
Reduction in depressive symptoms was significantly greater among African American patients within the intervention group (F = 3.99, p = .047) than controls. There were significant differences by race in four topics (dietitian referral, appointment date, help preparing discussion questions, and advice on worsening HF symptoms) concerning patient-physician discussions.Conclusion
The intervention showed greater effect in reducing depressive symptoms among African Americans than Caucasians. Preparing patients for discussions at physician appointments on diet, depressive symptoms, and HF symptoms is recommended. 相似文献12.
Quin E. Denfeld James O. Mudd Wohaib Hasan Jill M. Gelow Shirin O. Hiatt Kerri Winters-Stone Christopher S. Lee 《Heart & lung : the journal of critical care》2018,47(4):281-284
Background
The relationship between physical heart failure (HF) symptoms and pathophysiological mechanisms is unclear.Objective
To quantify the relationship between plasma β-adrenergic receptor kinase-1 (βARK1) and physical symptoms among adults with HF.Methods
We performed a secondary analysis of data collected from two studies of adults with HF. Plasma βARK1 was quantified using an enzyme-linked immunosorbent assay. Physical symptoms were measured with the HF Somatic Perception Scale (HFSPS). Generalized linear modeling was used to quantify the relationship between βARK1 and HFSPS scores.Results
The average age (n = 94) was 54.5 ± 13.1 years, 76.6% were male, and a majority (83.0%) had Class III or IV HF. βARK1 was significantly associated with HFSPS scores (β = 0.22 ± 0.10, p = 0.038), adjusting for other predictors of physical symptoms (model R2 = 0.250, F(7, 70) = 3.34, p = 0.004).Conclusions
Higher βARK1 is associated with worse physical HF symptoms, pinpointing a potential pathophysiologic underpinning. 相似文献13.
Dongmei He Min Ye Liwen Zhang Binghu Jiang 《Heart & lung : the journal of critical care》2018,47(2):122-126
Background
Late gadolinium enhancement (LGE) on cardiac MRI indicates the myocardial fibrosis in hypertrophic cardiomyopathy (HCM), and the prognostic value of LGE in HCM has been described in several studies, but controversy exists given the limited power of these studies to predict future adverse cardiac events. The objective of this study was to perform a meta-analysis to systematically evaluate the predictive value of LGE on cardiac magnetic resonance (CMR) for future adverse cardiac events.Methods
We systematically searched multiple database including PubMed, EMBASE, and Cochrane Library for cohort studies of the effects of LGE on clinical outcomes (sudden cardiac death (SCD)/aborted SCD, all cardiac death, and all-cause mortality) in patients with HCM. We performed a meta-analysis to determine pooled odds ratios (OR), weighted average annualized event rates, and summary receiver-operating characteristic (SROC) curves for these clinical events.Results
We identified nine clinical studies, examining 1734 patients with LGE and 2036 without LGE, and an average follow-up of 2.9 years. The weighted average annualized event rates of SCD/aborted SCD in patients with HCM (positive LGE versus negative LGE) was 1.28% versus 0.32% (p < 0.001), and the pooled OR was 3.40 (95% CI: 1.90, 6.08; p < 0.001). The sensitivity and specificity of predicting future cardiac events were 0.83 (95% CI: 0.66, 0.93) and 0.45 (95% CI: 0.31, 0.59), respectively. The 5-year risk of SCD/aborted SCD was 6.4% in patients with LGE. The all cardiac death and all-cause mortality were also significantly increased in patients with LGE. However, the extent of LGE was not significantly related to the risk of SCD/aborted SCD.Conclusions
LGE is significantly associated with SCD/aborted SCD risk, all cardiac death and all-cause mortality in patients with HCM. Implantable cardioverter defibrillators (ICD) can be considered for those patients with LGE. 相似文献14.
Jonathan F. Plehn Keren Hasbani Inez Ernst Kenneth D. Horton Bart E. Drinkard Nicholas A. Di Prospero 《Journal of cardiac failure》2018,24(10):672-679
Background
Identification of a subclinical cardiomyopathy in pediatric patients with Friedreich's ataxia (FA) has not been well-described.Methods
We performed echocardiography (Echo), cardiac magnetic resonance imaging (cMRI), and neurologic assessment in a cross-sectional analysis of 48 genetically confirmed FA subjects aged 9–17 years with moderate neurologic impairment but without a cardiovascular history. Echo- and cMRI-determined left ventricular mass were indexed (LVMI) to height in grams/m2.7. LV remodeling was categorized as concentric remodeling (CR), concentric hypertrophy (CH), or eccentric hypertrophy based upon Echo- determined relative LV wall thickness.Results
Echo LVMI exceeded age-based normal values in 85% of subjects, and cMRI-determined LVMI correlated with depression of both diastolic and systolic tissue Doppler velocity (E′: r?=?–0.65, P?<?.001, S′: r = –0.46, P?<?.001) as well as increased early diastolic Doppler flow velocity/tissue velocity ratio (r =?0.55, P?<?.001), a marker of elevated LV filling pressure. Similar associations were found with echo-determined LV mass. Evidence of depressed LV relaxation and increased LV stiffness were observed in 88% and 71%, of subjects, respectively, despite a normal LV ejection fraction in almost all cases (mean?=?60%?+?7%). CR and CH were present in 40% and 44% of the study group, respectively, although significant depressions of E′ and S′ were observed only in subjects with CH (P?<?.005).Conclusions
A subclinical hypertrophic cardiomyopathy is common in pediatric FA patients and CH is associated with both diastolic and systolic dysfunction. 相似文献15.
Robyn Gallagher Sue Randall Stella H.M. Lin Janice Smith Alexander M. Clark Lis Neubeck 《Heart & lung : the journal of critical care》2018,47(5):471-476
Background
The mechanisms contributing to the success of cardiac rehabilitation (CR) are poorly understood and may include assessment, monitoring and review activities enabled by continuity of care and this is investigated in this study.Objectives
To identify active assessment components of CR.Methods
A qualitative study using focus groups and individual interviews. CR staff (n = 39) were recruited via professional association email and network contacts and organised into major themes.Results
CR staff assessment strategies and timely actions undertaken provided a sophisticated post-discharge safety net for patients. Continuity of care enabled detection of adverse health indicators, of which medication issues were prominent. Interventions were timely and personalised and therefore likely to impact outcomes, but seldom documented or reported and thus invisible to audit.Conclusion
CR staff assessment and intervention activities provide an unrecognised safety net of activities enabled by continuity of care, potentially contributing to the effectiveness of CR. 相似文献16.
Elizabeth C. Parsons Catherine L. Hough Michael V. Vitiello Brian Palen Douglas Zatzick Dimitry S. Davydow 《Heart & lung : the journal of critical care》2018,47(2):87-92
Background
There is no insomnia screening tool validated in intensive care unit (ICU) survivors.Objectives
To examine the validity of a single item from the PTSD checklist-Civilian version (PCL-C) to detect insomnia by Insomnia Severity Index (ISI)Methods
We performed a secondary analysis of data from a longitudinal investigation in 120 medical-surgical ICU survivors. At 1 year post-ICU, patients completed ISI, PCL-C, and Medical Short-Form 12 (SF-12) by telephone. A single PCL-C item rates difficulty initiating or maintaining sleep over the past month. We compared performance characteristics of this PCL-C item to ISI-defined insomnia (ISI ≥15).Results
A score of ≥3 on the PCL-C sleep item exhibited 91% sensitivity and 67% specificity for ISI-defined insomnia (ISI ≥ 15), and it demonstrated construct validity by correlation to related QOL indices.Conclusions
A single PCL-C sleep item score ≥ 3 is a reasonable screen to identify insomnia symptoms in ICU survivors. 相似文献17.
Tania T. Von Visger Kristin K. Kuntz Gary S. Phillips Vedat O. Yildiz Namita Sood 《Heart & lung : the journal of critical care》2018,47(2):115-121
Background
Pulmonary arterial hypertension (PAH) has a delay in diagnosis that makes time since diagnosis of interest in this population.Objectives
To assess psychological conditions, perceived stress, QOL, and interpersonal support and to explore whether these factors may correlate with time since diagnosis in patients with PAH.Methods
Participants at an academic medical center (n = 108) completed psychological questionnaires (Cambridge Pulmonary Hypertension Outcome Review, Patient Health Questionnaire-9, Perceived Stress Scale-10, and Interpersonal Support Evaluation List-Short Form).Results
Prevalence of psychiatric disorder, major depression, and “other depressive disorder” were 29.6%, 15.7%, and 9.3%, respectively. Participants reported adequate social support, high perceived stress, and average quality of life. Time since diagnosis was positively associated with greater perceived social support (ρ = 0.174, p = .075) and greater perceived stress (ρ = 0.191, p = .048), but no other psychological factor.Conclusions
Routine psychological assessment and timely referral for mental health services are suggested. Social support may buffer patients from stress. 相似文献18.
Jayne Rosenberger Susan McCrudden Carol McCullough Lu Wang Joni Kime Nancy M. Albert 《Heart & lung : the journal of critical care》2018,47(2):100-106
Background
Experts recommend obtaining one-time dual- (inter)-arm blood pressure (BP) measurements to predict cardiovascular morbidity risk.Objectives
To determine differences in inter-arm systolic (S)/diastolic (D) BPs obtained simultaneously and sequentially and examine associations between patient factors and clinical outcomes and inter-arm BP differences.Method
A comparative study of adults treated in intensive care; multivariable logistic models were created to determine the extent that inter-arm BP differences predicted outcomes.Results
Of 427 adults in intensive care units, 31.8% had differences of >10 mmHg on simultaneous measurement and 35.1% had differences of >10 mmHg on sequential measurement; differences >15 mmHg were 17.9% and 19.8%, respectively. After controlling for patient factors, simultaneous inter-arm DBP differences >15 mmHg were associated with shorter hospital and longer intensive care length of stay (p = 0.031 and 0.029, respectively) and a 79% reduction in the likelihood of discharge to home (p = 0.009).Conclusions
Simultaneous inter-arm DBP differences >15 mmHg were associated with clinical outcomes. 相似文献19.
Marcin Waligóra Anna Tyrka Tomasz Miszalski-Jamka Małgorzata Urbańczyk-Zawadzka Piotr Podolec Grzegorz Kopeć 《Heart & lung : the journal of critical care》2018,47(3):237-242
Background
Right atrial (RA) enlargement is a common finding in patients with pulmonary arterial hypertension (PAH) and an important predictor of mortality, however its relation to the risk of atrial arrhythmias has not been assessed.Objectives
To assess whether RA enlargement is associated with supraventricular arrhythmias (SVA) and whether it predicts new clinically significant SVA (csSVA).Methods
Patients with PAH were recruited between January 2010 and December 2014 and followed until January 2017. csSVA was diagnosed if it resulted in hospitalization. To assess predictors of new csSVA, only patients without a history of SVA at baseline were analyzed.Results
Among 97 patients, any SVA was observed in 45 (46.4%) and included permanent atrial fibrillation(AF, n = 8), paroxysmal AF (n = 10), permanent atrial flutter (AFl, n = 1), paroxysmal AFl (n = 2) or other types of supraventricular tachycardia (n = 24). Patients with SVA as compared to patients without SVA were characterized by older age, lower distance in a 6-minute test, higher NT-proBNP, higher RA area index (RAai), left atrial area index, mean right atrial pressure (mRAP) and were more commonly treated with β-blocker. Eighty five patients who were in sinus rhythm at baseline assessment and had no history of significant SVA were observed for 37 ± 19.9 months. During that time csSVA occurred in 15.3%. In univariate models, the occurrence of csSVA were predicted by age, right ventricular ejection fraction, right ventricular end diastolic index, RAai and mRAP, but in multivariate model only RAai remained significant predictor for csSVA (HR of 1.23, 95%CI: 1.11–1.36, p < 0.001). The optimal threshold for RA enlargement as discriminator of csSVA was 21.7 cm2/m2.Conclusions
In PAH patients RA enlargement is associated with increased prevalence of SVA. RAai is an independent predictor of hospitalization due to csSVA. 相似文献20.
Wenjia Guo Tingting Lv Fei She Guobin Miao Yuanwei Liu Rong He Yajun Xue Nang Kham Nu Jing Yang Kun Li Ping Zhang 《Heart & lung : the journal of critical care》2018,47(5):516-524