Angiopoietin-2 in Adults with Congenital Heart Disease and Heart Failure

Background

Chronic heart failure is an important cause for morbidity and mortality in adults with congenital heart disease (ACHD). While NT-proBNP is an established biomarker for heart failure of non-congenital origin, its application in ACHD has limitations. The angiogenic factors Angiopoietin-1 and -2 (Ang-1, Ang-2), vascular endothelial growth factor (VEGF), and soluble receptor tyrosine kinase of the Tie family (sTie2) correlate with disease severity in heart failure of non-congenital origin. Their role in ACHD has not been studied.

Methods

In 91 patients Ang-2 and NT-proBNP were measured and related to New York Heart Association class, systemic ventricular function and parameters of cardiopulmonary exercise testing. Ang-1, VEGF, and sTie2 were also measured.

Results

Ang-2 correlates with NYHA class and ventricular dysfunction comparable to NT-proBNP. Further, Ang-2 showed a good correlation with parameters of cardiopulmonary exercise testing. Both, Ang-2 and NT-proBNP identified patients with severely limited cardiopulmonary exercise capacity. Additionally, Ang-2 is elevated in patients with a single ventricle physiology in contrast to NT-proBNP. VEGF, Ang-1, and sTie2 were not correlated with any clinical parameter.

Conclusion

The performance of Ang-2 as a biomarker for heart failure in ACHD is comparable to NT-proBNP. Its significant elevation in patients with single ventricle physiology indicates potential in this patient group and warrants further studies.     

A Predictive Study of Congenital Heart Disease and Need for Care

For long-term planning in the delivery of health care, prevalence data are essential for budget estimates in terms both of distribution and training of manpower and fiscal responsibility. From incidence figures, from the knowledge of the natural history of congenital heart disease and from predicted population estimates it is possible to construct a model that reflects the prevalence of congenital heart disease. This has been done for the state of California; the methods used and the data gathered should prove useful nationally.It is estimated that there were in 1975 in California 17,531 children under 21 years of age with congenital heart disease; 24 percent of these had ventricular septal defects and 23 percent had pulmonary stenosis, 11 percent had atrial septal defects and 9 percent had aortic stenosis; the other forms of congenital heart disease constituted the remaining 33 percent. Based on these estimates it is then possible to plan the medical resources necessary for optimal care.     

National Use of Safety-Net Clinics for Primary Care among Adults with Non-Medicaid Insurance in the United States

Objective

To describe the prevalence, characteristics, and predictors of safety-net use for primary care among non-Medicaid insured adults (i.e., those with private insurance or Medicare).

Methods

Cross-sectional analysis using the 2006–2010 National Ambulatory Medical Care Surveys, annual probability samples of outpatient visits in the U.S. We estimated national prevalence of safety-net visits using weighted percentages to account for the complex survey design. We conducted bivariate and multivariate logistic regression analyses to examine characteristics associated with safety-net clinic use.

Results

More than one-third (35.0%) of all primary care safety-net clinic visits were among adults with non-Medicaid primary insurance, representing 6,642,000 annual visits nationally. The strongest predictors of safety-net use among non-Medicaid insured adults were: being from a high-poverty neighborhood (AOR 9.53, 95% CI 4.65–19.53), being dually eligible for Medicare and Medicaid (AOR 2.13, 95% CI 1.38–3.30), and being black (AOR 1.97, 95% CI 1.06–3.66) or Hispanic (AOR 2.28, 95% CI 1.32–3.93). Compared to non-safety-net users, non-Medicaid insured adults who used safety-net clinics had a higher prevalence of diabetes (23.5% vs. 15.0%, p<0.001), hypertension (49.4% vs. 36.0%, p<0.001), multimorbidity (≥2 chronic conditions; 53.5% vs. 40.9%, p<0.001) and polypharmacy (≥4 medications; 48.8% vs. 34.0%, p<0.001). Nearly one-third (28.9%) of Medicare beneficiaries in the safety-net were dual eligibles, compared to only 6.8% of Medicare beneficiaries in non-safety-net clinics (p<0.001).

Conclusions

Safety net clinics are important primary care delivery sites for non-Medicaid insured minority and low-income populations with a high burden of chronic illness. The critical role of safety-net clinics in care delivery is likely to persist despite expanded insurance coverage under the Affordable Care Act.     

手术治疗先天性心脏病患者活动期心内膜炎的相关因素

目的:确定先天性心脏病活动期感染性心内膜炎(active infective endocarditis,AIE)的手术指征。方法:于2003-2011年从71个机构数据库中调查并采集247例患有感染性心内膜炎的儿童及成人先天性心脏病(congenital heart disease,CHD)患者数据,其中74例(30%)进行了AIE手术治疗。回顾性分析患者的年龄、性别、感染心内膜炎前对CHD的诊断、致病微生物和感染部位等数据。结果:与AIE手术治疗必要性显著相关的指标是感染性心内膜炎(infective endocarditis,IE)病发前对心脏异常的诊断缺乏、主动脉瓣IE、瓣周脓肿、心力衰竭以及抗生素发生变化。逐步逻辑回归方程分析结果表明瓣周脓肿、心力衰竭以及抗生素改变是先天性心脏病患者进行AIE手术治疗必要性的独立决定因素。结论:对IE合并CHD的患者而言,当心力衰竭、瓣周脓肿或抗生素变化发生时,手术可作为治疗AIE的一种手段。     

Amiodarone-Induced Thyrotoxicosis in Adults with Congenital Heart Disease -Clinical Presentation and Response to Therapy

ObjectiveThe development of amiodarone-induced thyrotoxicosis (AIT) can threaten the hemodynamic stability of adult patients with congenital heart disease (CHD). Here, we describe the natural history and treatment response of AIT in this at-risk population.MethodsWe studied retrospectively all cases of AIT that occurred in CHD patients at our institution after a minimum of 3 months on amiodarone. Subjects were identified from the cohort of adults with CHD who were treated at the Mayo Clinic Adult CHD clinic between 1987 and 2009.ResultsWe identified 23 cases of AIT: 7 were type 1, 13 were type 2, and 3 were undefined due to insufficient data. Most patients were symptomatic (17 of 23, 74%), with arrhythmia and weight loss as the most common symptoms. The majority (12 of 23, 52%) were initially observed; 10 patients (43%) were treated medically and 1 patient (5%) underwent thyroidectomy. Four patients from the observation group eventually required active treatment and 3 patients from the medical group required surgery. Asymptomatic patients tended to resolve under observation (5 of 7, 71.4%) rather than progress to active treatment (0 of 4) (P = .06). Discontinuation of amiodarone, AIT type, or use of perchlorate did not impact AIT duration.ConclusionAIT in CHD patients exhibits a wide range of severity and sensitivity to medical therapy. Asymptomatic patients display a trend toward AIT resolution with observation alone. Amiodarone continuation does not appear to impact management outcome or disease duration. Additional studies in this high-risk population could identify elements of pathophysiology that would point toward better disease prevention and treatment. (Endocr Pract. 2014;20:33-40)     

Seasonality and Coronary Heart Disease Deaths in United States Firefighters

United States firefighters have a high on‐duty fatality rate, and coronary heart disease is the leading cause. Seasonality affects the incidence of cardiovascular events in the general population, but its effects on firefighters are unknown. This study statistically examined the seasonal and annual variation of all on‐duty coronary heart disease deaths among US firefighters between 1994 and 2004 using the chi‐square distribution and Poisson regression model of the monthly fatality counts. It also examined the effect of ambient temperature (apparent as well as wind chill temperature) on coronary heart disease fatalities during the study span using a time‐stratified, case‐crossover study design. When grouped by season, we observed the distribution of the 449 coronary heart disease fatalities to show a relative peak in winter (32%) and relative nadir in spring (21%). This pattern was significantly different (p=0.005) from the expected distribution under the null hypothesis of season having no effect. The pattern persisted in additional analyses, stratifying the deaths by the type of duty in which the firefighters were engaged at the time of their deaths. In the Poisson regression model of the monthly fatality counts, the overall goodness‐of‐fit between the actual and predicted case counts was excellent (χ₄²=16.63; p=0.002). Two distinct peaks were detected: one in January–February and the other in August–September. Overall temperature was not associated with increased risk of on‐duty death. After allowing for different effects of temperature in mild/hot versus cold periods, a 1°C increase was not protective in cold weather; nor did it increase the risk of death in warmer weather. The findings of this study reveal statistical evidence for excess coronary heart disease deaths among firefighters during winter; however, the temporal pattern of coronary heart disease deaths was not linked to temperature variation. The seasonal pattern was also found to be independent of duty‐related risks.     

先天性心脏病相关综合征的遗传学研究

先天性心脏病(congenitalheartdisease,CHD)是儿科常见的疾病,现已发现约有300多种临床综合征伴有CHD.对Alagille综合征、CHARGE联合征、Holt-Oram综合征、Noonan综合征、Turner综合征、VACTERL联合征、Williams综合征、22q11缺失综合征和13、18、21三体综合征与CHD相关流行病学、临床表型、遗传病因和诊断及其再发风险进行了综述,为产前和产后临床诊断,了解疾病预后和再发概率提供资料.     

Geographic Analysis of Urologist Density and Prostate Cancer Mortality in the United States

Context

Financial and demographic pressures in US require an understanding of the most efficient distribution of physicians to maximize population-level health benefits. Prior work has assumed a constant negative relationship between physician supply and mortality outcomes throughout the US and has not addressed regional variation.

Methods

In this ecological analysis, geographically weighted regression was used to identify spatially varying relationships between local urologist density and prostate cancer mortality at the county level. Data from 1,492 counties in 30 eastern and southern states from 2006–2010 were analyzed.

Findings

The ordinary least squares (OLS) regression found that, on average, increasing urologist density by 1 urologist per 100,000 people resulted in an expected decrease in prostate cancer mortality of -0.499 deaths per 100,000 men (95% CI -0.709 to -0.289, p-value < 0.001), or a 1.5% decrease. Geographic weighted regression demonstrated that the addition of one urologist per 100,000 people in counties in the southern Mississippi River states of Arkansas, Mississippi, and Louisiana, as well as parts of Illinois, Indiana, and Wisconsin is associated with decrease of 0.411 to 0.916 in prostate cancer mortality per 100,000 men (1.6–3.6%). In contrast, the urologist density was not significantly associated with the prostate state mortality in the new England region.

Conclusions

The strength of association between urologist density and prostate cancer mortality varied regionally. Those areas with the highest potential for effects could be targeted for increasing the supply of urologists, as it associated with the largest predicted improvement in prostate cancer mortality.     

A Novel Atlas-Based Strategy for Understanding Cardiac Dysfunction in Patients with Congenital Heart Disease

Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease. Infants diagnosed with TOF require surgical interventions to survive into adulthood. However, as a result of postoperative structural malformations and long-term ventricular remodeling, further interventions are often required later in life. To help identify those at risk of disease progression, serial cardiac magnetic resonance (CMR) imaging is used to monitor these patients. However, most of the detailed information on cardiac shape and biomechanics contained in these large four-dimensional (4D) data sets goes unused in clinical practice for lack of efficient and comprehensive quantitative analysis tools. While current global metrics of cardiac size and function, such as indexed ventricular mass and volumes, can identify patients at risk of further complications, they are not adequate to explain the underlying mechanisms causing the postoperative malfunctions, and help cardiologists plan optimal personalized treatments. We are proposing a novel approach that uses 4D ventricular shape models derived from CMR imaging exams to generate statistical atlases of ventricular shape and finite-element models of ventricular biomechanics to identify specific features of cardiac shape and biomechanical properties that explain variations in ventricular function. This study has the potential to discover novel biomarkers that precede adverse ventricular remodeling and dysfunction.     

Implantation of Total Artificial Heart in Congenital Heart Disease

In patients with end-stage heart failure (HF), a total artificial heart (TAH) may be implanted as a bridge to cardiac transplant. However, in congenital heart disease (CHD), the malformed heart presents a challenge to TAH implantation. In the case presented here, a 17 year-old patient with congenital transposition of the great arteries (CCTGA) experienced progressively worsening HF due to his congenital condition. He was hospitalized multiple times and received an implantable cardioverter defibrillator (ICD). However, his condition soon deteriorated to end-stage HF with multisystem organ failure. Due to the patient''s grave clinical condition and the presence of complex cardiac lesions, the decision was made to proceed with a TAH. The abnormal arrangement of the patient''s ventricles and great arteries required modifications to the TAH during implantation.With the TAH in place, the patient was able to return home and regain strength and physical well-being while awaiting a donor heart. He was successfully bridged to heart transplantation 5 months after receiving the device. This report highlights the TAH is feasible even in patients with structurally abnormal hearts, with technical modification.     

Mortality from Congenital Heart Disease in Mexico: A Problem on the Rise

Background and Objectives

Temporal trends in mortality from congenital heart disease (CHD) vary among regions. It is therefore necessary to study this problem in each country. In Mexico, congenital anomalies were responsible for 24% of infant mortality in 2013 and CHD represented 55% of total deaths from congenital anomalies among children under 1 year of age. The objectives of this study were to analyze the trends in infant mortality from CHD in Mexico (1998 to 2013), its specific causes, age at death and associated socio-demographic factors.

Methods

Population-based study which calculated the compounded annual growth rate of death rom CHD between 1998 and 2013. Specific causes, age at which death from CHD occurred and risk factors associated with mortality were analyzed for the year 2013.

Results

Infant mortality from CHD increased 24.8% from 1998 to 2013 (114.4 to 146.4/ 100,000 live births). A total of 3,593 CHD deaths occurred in 2013; the main causes were CHD with left-to-right shunt (n = 487; 19.8/100,000 live births) and cyanotic heart disease (n = 410; 16.7/100,000). A total of 1,049 (29.2%) deaths from CHD occurred during the first week of life. Risk factors associated with mortality from CHD were, in order of magnitude: non-institutional birth, rural area, birth in a public hospital and male sex.

Conclusions

Mortality from CHD has increased in Mexico. The main causes were CHD with left-to-right shunt, which are not necessarily fatal if treated promptly. Populations vulnerable to death from CHD were identified. Approximately one-third of the CHD occurred during the first week of life. It is important to promote early diagnosis, especially for non-institutional births.     

287例患儿先天性心脏病的临床特点分析

目的:了解长春地区先天性心脏病的发病特点,分析影响手术效果的因素.方法:对2004年1月1日至2010年12月31日长春市儿童医院心胸外科收治的287例先天性心脏病患者的临床资料进行回顾性分析.结果:①未能确定体重是影响先天性心脏病患者术中循环时间和主动脉断流时间的因素.②尽量缩短体外循环时间和主动脉断流时间有助于降低死亡率.结论:术中尽量缩短体外循环时间和主动脉阻断时间,采取保护心肌措施,减少心脏缺血再灌注损伤.操作应精细,尽量减少手术操作对心肌的损伤;术后注意防治呼吸道并发症、低心排出量综合征和肺动脉高压危象,维持电解质和营养平衡,尽量减少患者的死亡率.