Objective:To extend our knowledge on tolerance of acute high-altitude exposure and hemodynamic response to exercise in adolescents with congenital heart disease(AscCHD)without meaningful clinical or functional restric...Objective:To extend our knowledge on tolerance of acute high-altitude exposure and hemodynamic response to exercise in adolescents with congenital heart disease(AscCHD)without meaningful clinical or functional restriction.Methods:A symptom limited cardiopulmonary exercise stress test and a non-invasive cardiac output measurement during steady state exercise were performed at 540 m and at 3454 m a.s.l.Symptoms of acute mountain sickness were noted.Results:We recruited 21 healthy controls and 16 AscCHD(59%male,mean age 14.7±1.1 years).Three subjects(2 controls,1 AscCHD)presented light symptoms of acute mountain sickness(dizziness and headache).During the symptom limited exercise test at lowland,control subjects showed a significantly higher power to weight index(3.5±0.6 W/kg vs.3.0±0.7 W/kg,p<0.001),heart rate(188.8±10.41/min vs.179.4±13.11/min,p<0.050)and ventilation(92.8±22.9 l/min vs.75.4±18.6 l/min,<0.050).At altitude,power to weight index only remained significantly higher in the control group(2.8±0.6 W/kg vs.2.6±0.6 W/kg,p<0.001).Pulmonary blood flow(PBF)at lowland showed no difference between the control and the AscCHD group,neither at rest(5.4±0.8 l/min vs.5.1±0.9 l/min,p=0.308),nor during the steady state test(10.6±2.4 l/min vs.10.5±2.0 l/min,p=0.825).At high altitude,PBF increased by 110%and 112%,respectively(12.8±2.32 l/min vs.12.5±3.0 l/min;intergroup difference:p=0.986).Conclusions:High altitude exposure was well tolerated in an unselected group of AscCHD.No significant difference in the cardio-pulmonary adaptation to a control group was noted during a steady state exercise.Symptoms of minor acute mountain sickness did occur,which should however not be misinterpreted as signs of hemodynamic maladaptation.展开更多
Congenital heart disease(CHD) is the most common type of birth defect. Traditionally, a polygenic model defined by the interaction of multiple genes and environmental factors was hypothesized to account for different ...Congenital heart disease(CHD) is the most common type of birth defect. Traditionally, a polygenic model defined by the interaction of multiple genes and environmental factors was hypothesized to account for different forms of CHD. It is now understood that the contribution of genetics to CHD extends beyond a single unified paradigm. For example, monogenic models and chromosomal abnormalities have been associated with various syndromic and non-syndromic forms of CHD. In such instances, genetic investigation and testing may potentially play an important role in clinical care. A family tree with a detailed phenotypic description serves as the initial screening tool to identify potentially inherited defects and to guide further genetic investigation. The selection of a genetic test is contingent upon the particular diagnostic hypothesis generated by clinical examination. Genetic investigation in CHD may carry the potential to improve prognosis by yielding valuable information with regards to personalized medical care, confidence in the clinical diagnosis, and/or targeted patient followup. Moreover, genetic assessment may serve as a tool to predict recurrence risk, define the pattern of inheritance within a family, and evaluate the need for further family screening. In some circumstances, prenatal or preimplantation genetic screening could identify fetuses or embryos at high risk for CHD. Although genetics may appear to constitute a highly specialized sector of cardiology, basic knowledge regarding inheritance patterns, recurrence risks, and available screening and diagnostic tools, including their strengths and limitations, could assist the treating physician in providing sound counsel.展开更多
Background Cardiorespiratory fitness(CRF)is inversely associated with mortality in apparently healthy subjects and in some clinical populations,but evidence for the association between CRF and all-cause and/or cardiov...Background Cardiorespiratory fitness(CRF)is inversely associated with mortality in apparently healthy subjects and in some clinical populations,but evidence for the association between CRF and all-cause and/or cardiovascular disease(CVD)mortality in patients with established CVD is lacking.This study aimed to quantify this association.Methods We searched for prospective cohort studies that measured CRF with cardiopulmonary exercise testing in patients with CVD and that examined all-cause and CVD mortality with at least 6 months of follow-up.Pooled hazard ratios(HRs)were calculated using random-effect inverse-variance analyses.Results Data were obtained from 21 studies and included 159,352 patients diagnosed with CVD(38.1%female).Pooled HRs for all-cause and CVD mortality comparing the highest vs.lowest category of CRF were 0.42(95%confidence interval(95%CI):0.28–0.61)and 0.27(95%CI:0.16–0.48),respectively.Pooled HRs per 1 metabolic equivalent(1-MET)increment were significant for all-cause mortality(HR=0.81;95%CI:0.74–0.88)but not for CVD mortality(HR=0.75;95%CI:0.48–1.18).Coronary artery disease patients with high CRF had a lower risk of all-cause mortality(HR=0.32;95%CI:0.26–0.41)than did their unfit counterparts.Each 1-MET increase was associated with lower all-cause mortality risk among coronary artery disease patients(HR=0.83;95%CI:0.76–0.91)but not lower among those with heart failure(HR=0.69;95%CI:0.36–1.32).Conclusion A better CRF was associated with lower risk of all-cause mortality and CVD.This study supports the use of CRF as a powerful predictor of mortality in this population.展开更多
Background:In patients with cyanotic congenital heart disease(CHD),cerebral oxygenation may be maintained by elevations in hematocrit(Hct).Hemodilution accompanying cardiopulmonary bypass(CPB),however,can disrupt cere...Background:In patients with cyanotic congenital heart disease(CHD),cerebral oxygenation may be maintained by elevations in hematocrit(Hct).Hemodilution accompanying cardiopulmonary bypass(CPB),however,can disrupt cerebral oxygen balance,leading to fluctuations in cerebral oxygen saturation(ScO_(2)).The present study investigated the effects of Hct changes on the fluctuation of ScO_(2)during CPB in cyanotic CHD using performance measurement(PM).Methods:Children with CHD(51 acyanotic and 46 cyanotic)who had undergone cardiac surgery using CPB were enrolled.Median performance error(MDPE),median absolute performance error(MDAPE),and wobble parameters of ScO_(2)were calculated before(reference value),during,and after CPB.Correlations of PM parameters with Hct and reductions in Hct(ΔHct)were also evaluated.Results:Before CPB,patients with cyanotic CHD had lower MDPE and larger wobble than those with acyanotic CHD,although mean ScO_(2)did not differ significantly between the two groups.During CPB,ScO_(2)of acyanotic CHD increased asΔHct increased,but PM variables were not associated withΔHct.In cyanotic CHD,MDPE(r=−0.324,p=0.032)and MDAPE(r=0.339,p=0.024)correlated significantly withΔHct during CPB.After CPB,MDPE(r=0.574,p=0.025)and MDAPE(r=−0.543,p=0.036)were significantly correlated with Hct in children with cyanotic CHD who underwent palliative surgery.Conclusion:Therefore,ScO_(2)fluctuation during CPB in children with cyanotic CHD may be affected by the decrease in Hct,suggesting that excessive hemodilution can negatively influence the maintenance of cerebral oxygenation in these patients.展开更多
Background:The purpose of this study was to investigate whether patients with adult congenital heart disease(ACHD)benefit from exercise-based cardiac rehabilitation(CR)short-and long-term with regard to improvement of...Background:The purpose of this study was to investigate whether patients with adult congenital heart disease(ACHD)benefit from exercise-based cardiac rehabilitation(CR)short-and long-term with regard to improvement of cardiorespiratory fitness.Methods:Cardiopulmonary exercise tests(CPET)completed by ACHD patients between January 2000 and October 2019 were analysed retrospectively.Linear mixed models were performed for peak oxygen consumption(VO_(2))with patients as random effect and age,sex,disease classification,preceding surgery(≤3 months)and preceding CR(≤4 weeks for short term and>4 weeks for long term)as fixed effects.Results:1056 CPETs of 311 ACHD patients with simple(7),moderate(188)or great(116)complexity heart defects were analysed.The 59 patients who completed a CR(median age 27 yrs,38%females)increased peak VO_(2)from before to after CR by a median of 2.7(IQR–0.6 to 5.5)ml/kg/min.However,in the multivariate mixed model,peak VO_(2)was non-significantly increased short-term after CR(β0.8,95%CI–0.7 to 2.4),not maintained long-term after CR(β0.0,95%CI–1.7 to 1.6)but significantly reduced after surgery(β–5.1,95%CI–7.1 to–3.1).The 20 CR patients after surgery increased their peak VO_(2)by 6.2(IQR 3.6–9.5)ml/kg/min,while the 39 CR patients without preceding surgery increased it by 0.9(IQR–1.5 to 3.1)ml/kg/min.Conclusions:The increase in peak VO_(2)with CR was mainly due to recovery from surgical intervention.The small independent benefit from CR was not maintained long-term,highlighting the potential to improve current CR concepts in ACHD populations.展开更多
Cardiopulmonary exercise testing (CPX) has become the cornerstone of risk stratification for heart failure patients. Peak oxygen consumption (VO2) was the first CPX variable to demonstrate prognostic value and is stil...Cardiopulmonary exercise testing (CPX) has become the cornerstone of risk stratification for heart failure patients. Peak oxygen consumption (VO2) was the first CPX variable to demonstrate prognostic value and is still the most frequently analyzed variable in clinical practice. More recently, several investigations have shown that ventilatory efficiency, typically expressed as the minute ventilation/carbon dioxide production (VE/VCO2) slope, is a strong prognostic marker in patient with HF. The majority of studies report the VE/VCO2 slope to be prognostically superior to peak VO2 which underscore the clinical importance of assessing ventilatory efficiency in HF patients. Other expressions of ventilatory inefficiency like exercise oscillatory breathing (EOB), oxygen uptake efficiency slope (OUES), end-tidal carbon dioxide pressure (PET CO2) at rest, and haemodynamic responses such as heart rate recovery (HRR) are strong predictors of outcomes in patients with heart failure (HF). So there is a need for simplified approaches that integrate the additive prognostic information from cardiopulmonary exercise testing.展开更多
There is a growing population of adults with congenital heart disease(CHD) due to improved survival beyond childhood.It has been suggested that adults with CHD may be at increased risk for mental health problems,parti...There is a growing population of adults with congenital heart disease(CHD) due to improved survival beyond childhood.It has been suggested that adults with CHD may be at increased risk for mental health problems,particularly depression.The reported incidence of depression in CHD varies from 9% to 30%.This review examines the evidence for a higher depression rate in CHD vs general population.Possible explanations are offered from a variety of disease models,ranging from brain injury to the psychoanalytical approach.Risk factors for an abnormal emotional adjustment and depression include early exposure to stress from illness and medical interventions in infancy,separation from the parents during hospitalizations and brain organic syndromes.Later in life,patients often have to cope with physical limitations.Recent improvements in care may be protective.Current patients may benefit from an earlier age at first surgical intervention,fewer reoperations and inclusion to the mainstream schooling,among other factors.At this point,there is little systematic knowledge about evidence-based therapeutic interventions for depression in adults with CHD.Health care providers of patients with CHD should be aware of mental health challenges and may take a more proactive approach to identifying patients at risk for depression.展开更多
AIM:To find out whether serum oxidizability potential correlates with exercise test(EXT) parameters and predicts their results in chronic ischemic heart disease(IHD) patients.METHODS:Oxidizability potential was determ...AIM:To find out whether serum oxidizability potential correlates with exercise test(EXT) parameters and predicts their results in chronic ischemic heart disease(IHD) patients.METHODS:Oxidizability potential was determined in a group of chronic IHD patients who underwent a symptom limited EXT upon initiation of a cardiac rehabilitation program.The thermo-chemiluminescence(TCL) assay was used to assess serum oxidizability potential.This assay is based on heat-induced oxidation of serum,leading to the formation of electronically excited species in the form of unstable carbonyls,which further decompose into stable carbonyls and light energy(low chemiluminescence).Measured photons emission is represented by a kinetic curve which is described by its amplitude and slope(= ratio).We assessed the correlations of TCL ratio with exercise duration,metabolic equivalents(METS),maximal heart rate(mHR),maximal systolic BP,> 1 mm S-T depression,diabetes,hypertension,smoking,left ventricular ejection fraction(LVEF) > or < 40%,previous myocardial infarction,and aorto-coronary bypass surgery and compared to the TCL ratio measured in a group of healthy controls.RESULTS:A high TCL ratio(%) correlated well with METS(r = 0.84),with mHR(r = 0.79) and with exercise induced S-T segment shift(r = 0.87,P < 0.05).A lower serum oxidizability potential,expressed as a low TCL ratio,thus suggestive of a previous high oxidative stress,was found in IHD patients compared to healthy controls,and,in particular,in patients with low LVEF%.The TCL ratio(%) in IHD patients was 193 ± 21,compared to 215 ± 13 in controls(P < 0.05),and was 188 ± 14.7 in patients with LVEF < 40% as compared to 200 ± 11.9 in those with LVEF > 40%(P < 0.01).A trend for lower TCL ratio(%) was found in diabetic,hypertensive,and post-coronary bypass surgery patients.A paradoxically low TCL ratio(low oxidizability potential) was observed in patients without S-T depression compared to patients with S-T depression(189 ± 22 vs 201 ± 15,P = NS),due to the fact these patients had a much lower LVEF% and a lower exercise capacity.CONCLUSION:Serum oxidizability potential is associated with EXT parameters,results,and IHD severity.TCL ratio is an "easy-to-measure marker" that might be incorporated into risk assessment and prediction in chronic IHD patients.展开更多
The prognostic role of cardiopulmonary exercise test (CPET) in elderly women with chronic heart failure (HF) has not yet been clarified. We assessed the incremental value of CPET variables for risk stratification in f...The prognostic role of cardiopulmonary exercise test (CPET) in elderly women with chronic heart failure (HF) has not yet been clarified. We assessed the incremental value of CPET variables for risk stratification in female HF patients with preserved or reduced left ventricular ejection fraction (LVEF). We prospectively followed up 131 female HF outpatients aged 72 [interquartile range 62 - 77] years after a symptom limited CPET. 34% had ischemic heart disease and 14% permanent atrial fibrillation, 24% were in NYHA class III. LVEF was 50% [interquartile range 36 - 62], peak oxygen consumption was 11.3 [interquartile range 9.2 - 13.5] ml/kg/min;the slope of the regression line relating ventilation to CO2 output was 33.9 [interquartile range 30.3 - 44.9];40% of patients showed exercise oscillatory breathing during CPET. During a median follow-up of 18 months [interquartile range 8 - 54], overall 39 patients (29.7%) met the combined end-point of cardiovascular mortality or HF admission using a time-to-first event approach. Moderate to severe mitral regurgitation, slope, exercise oscillatory breathing were independently associated to cardiovascular mortality or HF admission. When CPET ventilatory variables were added to clinical and echocardiographic parameters, prediction of the combined point improved significantly (AUC 0.755 (95% CI 0.662 to 0.832) vs 0.634 (95% CI 0.536 to 0.725), p = 0.016). In conclusion, among elderly female HF patients the CPET derived parameters EOB and VE/VCO2 slope emerged as strong prognostic markers, with additive predictive value to clinical and echocardiographic parameters in patients with both reduced and preserved LVEF.展开更多
The beneficial effects of the programs of cardiac rehabilitation in adults are widely known. Nevertheless, the accomplishment of these programs in patients with congenital heart is scarcely developed. In this study, w...The beneficial effects of the programs of cardiac rehabilitation in adults are widely known. Nevertheless, the accomplishment of these programs in patients with congenital heart is scarcely developed. In this study, we want to evaluate the preliminary results of a program of cardiac rehabilitation. Patients and methods: Thirty-three patients aged 8 to 40 completed the rehabilitation program. Thirty-one of them had an operated congenital heart disease, one had an implantable cardioverter defibrillator, and another was a patient with arterial hypertension and obesity. Data before and after the program were compared. Results: Improvements were found in all of the measured parameters. We found a statistically significant difference in maximum heart rate, exercise duration, foreseen heart rate’s percentage and increase of metabolic equivalents. Twenty-seven of 33 patients noticed a significant improvement in their quality of life at the end of the program. Conclusion: Initial results seem to be encouraging and we consider the experience very positive since the program achieved an improvement in the functional capacity of our patients. In our opinion, these observations and the results of our study provide strong arguments for the incorporation of cardiac rehabilitation into the care of many patients with congenital heart disease.展开更多
Purpose:To perform ophthalmic examinations to evaluate the ocular characteristics of children living in the Tibet plateau and diagnosed with congenital heart disease.Methods:.Children with congenital heart disease und...Purpose:To perform ophthalmic examinations to evaluate the ocular characteristics of children living in the Tibet plateau and diagnosed with congenital heart disease.Methods:.Children with congenital heart disease underwent a conventional ocular examination including distant acuity test,slit-lamp examination,.fundus examination,.non-contact intraocular pressure measurement,.measurement of corneal thickness, and fundus photography.Results: Forty-two Tibetan children, aged between 4 and 18 years and diagnosed with congenital heart disease,.were enrolled in this study..The percentage of low visual acuity was4.76%, mean intraocular pressure was(13.67±2.15) mm Hg,average corneal thickness was(492.55±33.79) μm,.96.43%had an anterior chamber depth of 1 / 2 corneal thickness(CT),and 35.7% had an obvious fundus vascular tortuosity.Conclusion:.This study adds to the understanding of the ocular status of the population living in the Tibet plateau, thereby offering clinical evidence for the prevention and treatment of eye diseases in this area.展开更多
BACKGROUND Vascular endothelial dysfunction is an underlying pathophysiological feature of chronic heart failure(CHF).Patients with CHF are characterized by impaired vasodilation and inflammation of the vascular endot...BACKGROUND Vascular endothelial dysfunction is an underlying pathophysiological feature of chronic heart failure(CHF).Patients with CHF are characterized by impaired vasodilation and inflammation of the vascular endothelium.They also have low levels of endothelial progenitor cells(EPCs).EPCs are bone marrow derived cells involved in endothelium regeneration,homeostasis,and neovascularization.Exercise has been shown to improve vasodilation and stimulate the mobilization of EPCs in healthy people and patients with cardiovascular comorbidities.However,the effects of exercise on EPCs in different stages of CHF remain under investigation.AIM To evaluate the effect of a symptom-limited maximal cardiopulmonary exercise testing(CPET)on EPCs in CHF patients of different severity.METHODS Forty-nine consecutive patients(41 males)with stable CHF[mean age(years):56±10,ejection fraction(EF,%):32±8,peak oxygen uptake(VO2,mL/kg/min):18.1±4.4]underwent a CPET on a cycle ergometer.Venous blood was sampled before and after CPET.Five circulating endothelial populations were quantified by flow cytometry:Three subgroups of EPCs[CD34+/CD45-/CD133+,CD34+/CD45-/CD133+/VEGFR2 and CD34+/CD133+/vascular endothelial growth factor receptor 2(VEGFR2)]and two subgroups of circulating endothelial cells(CD34+/CD45-/CD133-and CD34+/CD45-/CD133-/VEGFR2).Patients were divided in two groups of severity according to the median value of peak VO2(18.0 mL/kg/min),predicted peak VO2(65.5%),ventilation/carbon dioxide output slope(32.5)and EF(reduced and mid-ranged EF).EPCs values are expressed as median(25th-75th percentiles)in cells/106 enucleated cells.RESULTS Patients with lower peak VO2 increased the mobilization of CD34+/CD45-/CD133+[pre CPET:60(25-76)vs post CPET:90(70-103)cells/106 enucleated cells,P<0.001],CD34+/CD45-/CD133+/VEGFR2[pre CPET:1(1-4)vs post CPET:5(3-8)cells/106 enucleated cells,P<0.001],CD34+/CD45-/CD133-[pre CPET:186(141-361)vs post CPET:488(247-658)cells/106 enucleated cells,P<0.001]and CD34+/CD45-/CD133-/VEGFR2[pre CPET:2(1-2)vs post CPET:3(2-5)cells/106 enucleated cells,P<0.001],while patients with higher VO2 increased the mobilization of CD34+/CD45-/CD133+[pre CPET:42(19-73)vs post CPET:90(39-118)cells/106 enucleated cells,P<0.001],CD34+/CD45-/CD133+/VEGFR2[pre CPET:2(1-3)vs post CPET:6(3-9)cells/106 enucleated cells,P<0.001],CD34+/CD133+/VEGFR2[pre CPET:10(7-18)vs post CPET:14(10-19)cells/106 enucleated cells,P<0.01],CD34+/CD45-/CD133-[pre CPET:218(158-247)vs post CPET:311(254-569)cells/106 enucleated cells,P<0.001]and CD34+/CD45-/CD133-/VEGFR2[pre CPET:1(1-2)vs post CPET:4(2-6)cells/106 enucleated cells,P<0.001].A similar increase in the mobilization of at least four out of five cellular populations was observed after maximal exercise within each severity group regarding predicted peak,ventilation/carbon dioxide output slope and EF as well(P<0.05).However,there were no statistically significant differences in the mobilization of endothelial cellular populations between severity groups in each comparison(P>0.05).CONCLUSION Our study has shown an increased EPCs and circulating endothelial cells mobilization after maximal exercise in CHF patients,but this increase was not associated with syndrome severity.Further investigation,however,is needed.展开更多
objective To study how to improve the accuracy of treadmill exercise testing(TET).Methods TET was performed before coronary angiography (CAG) for 147 subjects admitted into our departmentbecause of suspicion of angina...objective To study how to improve the accuracy of treadmill exercise testing(TET).Methods TET was performed before coronary angiography (CAG) for 147 subjects admitted into our departmentbecause of suspicion of angina or coronary artery disease (CAD), then: (1) Every subject was inquired about hissymptoms and physically examined in detail before the testing. Chest pain was divided into ischemic (ICP) ornon - ischemic (NICP) pattern. (2) Informations got from TET, like angina, ST segment depression, and SPB ratio,were analyzed in detail. Results (1) When the patients showed chest pain of ICP pattern and positive TETresults, the sensitivity of TET would be 95%, for patients with NICP chest pain, the negative result of TET meansthat their coronary arteries is normal with the accurate possibility of 98%. (2) By our new criteria of TET, thesensitivity and specificity of TET were 89% and 95% respectively, both higher than those in theliterature. Conclusion in this report, we found TET was more sensitive and accurate if we knew the symptomsprior, especially in detail the characteristics of chest pain, and consider different parameters in combination.展开更多
Rationale PH is a pathophysiological disorder involving multiple clinical conditions and is often concomitant with other cardiopulmonary diseases,and related differential diagnosis is quite tricky for its various etio...Rationale PH is a pathophysiological disorder involving multiple clinical conditions and is often concomitant with other cardiopulmonary diseases,and related differential diagnosis is quite tricky for its various etiologies.Patient characteristics A 49-year-old man presented with 1-year history of nocturnal paroxysmal dyspnea and was transferred to our hospital.展开更多
文摘Objective:To extend our knowledge on tolerance of acute high-altitude exposure and hemodynamic response to exercise in adolescents with congenital heart disease(AscCHD)without meaningful clinical or functional restriction.Methods:A symptom limited cardiopulmonary exercise stress test and a non-invasive cardiac output measurement during steady state exercise were performed at 540 m and at 3454 m a.s.l.Symptoms of acute mountain sickness were noted.Results:We recruited 21 healthy controls and 16 AscCHD(59%male,mean age 14.7±1.1 years).Three subjects(2 controls,1 AscCHD)presented light symptoms of acute mountain sickness(dizziness and headache).During the symptom limited exercise test at lowland,control subjects showed a significantly higher power to weight index(3.5±0.6 W/kg vs.3.0±0.7 W/kg,p<0.001),heart rate(188.8±10.41/min vs.179.4±13.11/min,p<0.050)and ventilation(92.8±22.9 l/min vs.75.4±18.6 l/min,<0.050).At altitude,power to weight index only remained significantly higher in the control group(2.8±0.6 W/kg vs.2.6±0.6 W/kg,p<0.001).Pulmonary blood flow(PBF)at lowland showed no difference between the control and the AscCHD group,neither at rest(5.4±0.8 l/min vs.5.1±0.9 l/min,p=0.308),nor during the steady state test(10.6±2.4 l/min vs.10.5±2.0 l/min,p=0.825).At high altitude,PBF increased by 110%and 112%,respectively(12.8±2.32 l/min vs.12.5±3.0 l/min;intergroup difference:p=0.986).Conclusions:High altitude exposure was well tolerated in an unselected group of AscCHD.No significant difference in the cardio-pulmonary adaptation to a control group was noted during a steady state exercise.Symptoms of minor acute mountain sickness did occur,which should however not be misinterpreted as signs of hemodynamic maladaptation.
基金Supported by A Canada research chair in electrophysiology and adult congenital heart disease(Paul Khairy)
文摘Congenital heart disease(CHD) is the most common type of birth defect. Traditionally, a polygenic model defined by the interaction of multiple genes and environmental factors was hypothesized to account for different forms of CHD. It is now understood that the contribution of genetics to CHD extends beyond a single unified paradigm. For example, monogenic models and chromosomal abnormalities have been associated with various syndromic and non-syndromic forms of CHD. In such instances, genetic investigation and testing may potentially play an important role in clinical care. A family tree with a detailed phenotypic description serves as the initial screening tool to identify potentially inherited defects and to guide further genetic investigation. The selection of a genetic test is contingent upon the particular diagnostic hypothesis generated by clinical examination. Genetic investigation in CHD may carry the potential to improve prognosis by yielding valuable information with regards to personalized medical care, confidence in the clinical diagnosis, and/or targeted patient followup. Moreover, genetic assessment may serve as a tool to predict recurrence risk, define the pattern of inheritance within a family, and evaluate the need for further family screening. In some circumstances, prenatal or preimplantation genetic screening could identify fetuses or embryos at high risk for CHD. Although genetics may appear to constitute a highly specialized sector of cardiology, basic knowledge regarding inheritance patterns, recurrence risks, and available screening and diagnostic tools, including their strengths and limitations, could assist the treating physician in providing sound counsel.
基金AGH is a Miguel Servet Fellow at the Instituto de Salud Carlos III(CP18/0150)RRV is funded in part by a Postdoctoral Fellowship(Resolution ID 420/2019)from the Universidad Pública de Navarra.
文摘Background Cardiorespiratory fitness(CRF)is inversely associated with mortality in apparently healthy subjects and in some clinical populations,but evidence for the association between CRF and all-cause and/or cardiovascular disease(CVD)mortality in patients with established CVD is lacking.This study aimed to quantify this association.Methods We searched for prospective cohort studies that measured CRF with cardiopulmonary exercise testing in patients with CVD and that examined all-cause and CVD mortality with at least 6 months of follow-up.Pooled hazard ratios(HRs)were calculated using random-effect inverse-variance analyses.Results Data were obtained from 21 studies and included 159,352 patients diagnosed with CVD(38.1%female).Pooled HRs for all-cause and CVD mortality comparing the highest vs.lowest category of CRF were 0.42(95%confidence interval(95%CI):0.28–0.61)and 0.27(95%CI:0.16–0.48),respectively.Pooled HRs per 1 metabolic equivalent(1-MET)increment were significant for all-cause mortality(HR=0.81;95%CI:0.74–0.88)but not for CVD mortality(HR=0.75;95%CI:0.48–1.18).Coronary artery disease patients with high CRF had a lower risk of all-cause mortality(HR=0.32;95%CI:0.26–0.41)than did their unfit counterparts.Each 1-MET increase was associated with lower all-cause mortality risk among coronary artery disease patients(HR=0.83;95%CI:0.76–0.91)but not lower among those with heart failure(HR=0.69;95%CI:0.36–1.32).Conclusion A better CRF was associated with lower risk of all-cause mortality and CVD.This study supports the use of CRF as a powerful predictor of mortality in this population.
文摘Background:In patients with cyanotic congenital heart disease(CHD),cerebral oxygenation may be maintained by elevations in hematocrit(Hct).Hemodilution accompanying cardiopulmonary bypass(CPB),however,can disrupt cerebral oxygen balance,leading to fluctuations in cerebral oxygen saturation(ScO_(2)).The present study investigated the effects of Hct changes on the fluctuation of ScO_(2)during CPB in cyanotic CHD using performance measurement(PM).Methods:Children with CHD(51 acyanotic and 46 cyanotic)who had undergone cardiac surgery using CPB were enrolled.Median performance error(MDPE),median absolute performance error(MDAPE),and wobble parameters of ScO_(2)were calculated before(reference value),during,and after CPB.Correlations of PM parameters with Hct and reductions in Hct(ΔHct)were also evaluated.Results:Before CPB,patients with cyanotic CHD had lower MDPE and larger wobble than those with acyanotic CHD,although mean ScO_(2)did not differ significantly between the two groups.During CPB,ScO_(2)of acyanotic CHD increased asΔHct increased,but PM variables were not associated withΔHct.In cyanotic CHD,MDPE(r=−0.324,p=0.032)and MDAPE(r=0.339,p=0.024)correlated significantly withΔHct during CPB.After CPB,MDPE(r=0.574,p=0.025)and MDAPE(r=−0.543,p=0.036)were significantly correlated with Hct in children with cyanotic CHD who underwent palliative surgery.Conclusion:Therefore,ScO_(2)fluctuation during CPB in children with cyanotic CHD may be affected by the decrease in Hct,suggesting that excessive hemodilution can negatively influence the maintenance of cerebral oxygenation in these patients.
基金This project was awarded a contribution from the Swiss Heart Foundation.
文摘Background:The purpose of this study was to investigate whether patients with adult congenital heart disease(ACHD)benefit from exercise-based cardiac rehabilitation(CR)short-and long-term with regard to improvement of cardiorespiratory fitness.Methods:Cardiopulmonary exercise tests(CPET)completed by ACHD patients between January 2000 and October 2019 were analysed retrospectively.Linear mixed models were performed for peak oxygen consumption(VO_(2))with patients as random effect and age,sex,disease classification,preceding surgery(≤3 months)and preceding CR(≤4 weeks for short term and>4 weeks for long term)as fixed effects.Results:1056 CPETs of 311 ACHD patients with simple(7),moderate(188)or great(116)complexity heart defects were analysed.The 59 patients who completed a CR(median age 27 yrs,38%females)increased peak VO_(2)from before to after CR by a median of 2.7(IQR–0.6 to 5.5)ml/kg/min.However,in the multivariate mixed model,peak VO_(2)was non-significantly increased short-term after CR(β0.8,95%CI–0.7 to 2.4),not maintained long-term after CR(β0.0,95%CI–1.7 to 1.6)but significantly reduced after surgery(β–5.1,95%CI–7.1 to–3.1).The 20 CR patients after surgery increased their peak VO_(2)by 6.2(IQR 3.6–9.5)ml/kg/min,while the 39 CR patients without preceding surgery increased it by 0.9(IQR–1.5 to 3.1)ml/kg/min.Conclusions:The increase in peak VO_(2)with CR was mainly due to recovery from surgical intervention.The small independent benefit from CR was not maintained long-term,highlighting the potential to improve current CR concepts in ACHD populations.
文摘Cardiopulmonary exercise testing (CPX) has become the cornerstone of risk stratification for heart failure patients. Peak oxygen consumption (VO2) was the first CPX variable to demonstrate prognostic value and is still the most frequently analyzed variable in clinical practice. More recently, several investigations have shown that ventilatory efficiency, typically expressed as the minute ventilation/carbon dioxide production (VE/VCO2) slope, is a strong prognostic marker in patient with HF. The majority of studies report the VE/VCO2 slope to be prognostically superior to peak VO2 which underscore the clinical importance of assessing ventilatory efficiency in HF patients. Other expressions of ventilatory inefficiency like exercise oscillatory breathing (EOB), oxygen uptake efficiency slope (OUES), end-tidal carbon dioxide pressure (PET CO2) at rest, and haemodynamic responses such as heart rate recovery (HRR) are strong predictors of outcomes in patients with heart failure (HF). So there is a need for simplified approaches that integrate the additive prognostic information from cardiopulmonary exercise testing.
文摘There is a growing population of adults with congenital heart disease(CHD) due to improved survival beyond childhood.It has been suggested that adults with CHD may be at increased risk for mental health problems,particularly depression.The reported incidence of depression in CHD varies from 9% to 30%.This review examines the evidence for a higher depression rate in CHD vs general population.Possible explanations are offered from a variety of disease models,ranging from brain injury to the psychoanalytical approach.Risk factors for an abnormal emotional adjustment and depression include early exposure to stress from illness and medical interventions in infancy,separation from the parents during hospitalizations and brain organic syndromes.Later in life,patients often have to cope with physical limitations.Recent improvements in care may be protective.Current patients may benefit from an earlier age at first surgical intervention,fewer reoperations and inclusion to the mainstream schooling,among other factors.At this point,there is little systematic knowledge about evidence-based therapeutic interventions for depression in adults with CHD.Health care providers of patients with CHD should be aware of mental health challenges and may take a more proactive approach to identifying patients at risk for depression.
文摘AIM:To find out whether serum oxidizability potential correlates with exercise test(EXT) parameters and predicts their results in chronic ischemic heart disease(IHD) patients.METHODS:Oxidizability potential was determined in a group of chronic IHD patients who underwent a symptom limited EXT upon initiation of a cardiac rehabilitation program.The thermo-chemiluminescence(TCL) assay was used to assess serum oxidizability potential.This assay is based on heat-induced oxidation of serum,leading to the formation of electronically excited species in the form of unstable carbonyls,which further decompose into stable carbonyls and light energy(low chemiluminescence).Measured photons emission is represented by a kinetic curve which is described by its amplitude and slope(= ratio).We assessed the correlations of TCL ratio with exercise duration,metabolic equivalents(METS),maximal heart rate(mHR),maximal systolic BP,> 1 mm S-T depression,diabetes,hypertension,smoking,left ventricular ejection fraction(LVEF) > or < 40%,previous myocardial infarction,and aorto-coronary bypass surgery and compared to the TCL ratio measured in a group of healthy controls.RESULTS:A high TCL ratio(%) correlated well with METS(r = 0.84),with mHR(r = 0.79) and with exercise induced S-T segment shift(r = 0.87,P < 0.05).A lower serum oxidizability potential,expressed as a low TCL ratio,thus suggestive of a previous high oxidative stress,was found in IHD patients compared to healthy controls,and,in particular,in patients with low LVEF%.The TCL ratio(%) in IHD patients was 193 ± 21,compared to 215 ± 13 in controls(P < 0.05),and was 188 ± 14.7 in patients with LVEF < 40% as compared to 200 ± 11.9 in those with LVEF > 40%(P < 0.01).A trend for lower TCL ratio(%) was found in diabetic,hypertensive,and post-coronary bypass surgery patients.A paradoxically low TCL ratio(low oxidizability potential) was observed in patients without S-T depression compared to patients with S-T depression(189 ± 22 vs 201 ± 15,P = NS),due to the fact these patients had a much lower LVEF% and a lower exercise capacity.CONCLUSION:Serum oxidizability potential is associated with EXT parameters,results,and IHD severity.TCL ratio is an "easy-to-measure marker" that might be incorporated into risk assessment and prediction in chronic IHD patients.
文摘The prognostic role of cardiopulmonary exercise test (CPET) in elderly women with chronic heart failure (HF) has not yet been clarified. We assessed the incremental value of CPET variables for risk stratification in female HF patients with preserved or reduced left ventricular ejection fraction (LVEF). We prospectively followed up 131 female HF outpatients aged 72 [interquartile range 62 - 77] years after a symptom limited CPET. 34% had ischemic heart disease and 14% permanent atrial fibrillation, 24% were in NYHA class III. LVEF was 50% [interquartile range 36 - 62], peak oxygen consumption was 11.3 [interquartile range 9.2 - 13.5] ml/kg/min;the slope of the regression line relating ventilation to CO2 output was 33.9 [interquartile range 30.3 - 44.9];40% of patients showed exercise oscillatory breathing during CPET. During a median follow-up of 18 months [interquartile range 8 - 54], overall 39 patients (29.7%) met the combined end-point of cardiovascular mortality or HF admission using a time-to-first event approach. Moderate to severe mitral regurgitation, slope, exercise oscillatory breathing were independently associated to cardiovascular mortality or HF admission. When CPET ventilatory variables were added to clinical and echocardiographic parameters, prediction of the combined point improved significantly (AUC 0.755 (95% CI 0.662 to 0.832) vs 0.634 (95% CI 0.536 to 0.725), p = 0.016). In conclusion, among elderly female HF patients the CPET derived parameters EOB and VE/VCO2 slope emerged as strong prognostic markers, with additive predictive value to clinical and echocardiographic parameters in patients with both reduced and preserved LVEF.
文摘The beneficial effects of the programs of cardiac rehabilitation in adults are widely known. Nevertheless, the accomplishment of these programs in patients with congenital heart is scarcely developed. In this study, we want to evaluate the preliminary results of a program of cardiac rehabilitation. Patients and methods: Thirty-three patients aged 8 to 40 completed the rehabilitation program. Thirty-one of them had an operated congenital heart disease, one had an implantable cardioverter defibrillator, and another was a patient with arterial hypertension and obesity. Data before and after the program were compared. Results: Improvements were found in all of the measured parameters. We found a statistically significant difference in maximum heart rate, exercise duration, foreseen heart rate’s percentage and increase of metabolic equivalents. Twenty-seven of 33 patients noticed a significant improvement in their quality of life at the end of the program. Conclusion: Initial results seem to be encouraging and we consider the experience very positive since the program achieved an improvement in the functional capacity of our patients. In our opinion, these observations and the results of our study provide strong arguments for the incorporation of cardiac rehabilitation into the care of many patients with congenital heart disease.
文摘Purpose:To perform ophthalmic examinations to evaluate the ocular characteristics of children living in the Tibet plateau and diagnosed with congenital heart disease.Methods:.Children with congenital heart disease underwent a conventional ocular examination including distant acuity test,slit-lamp examination,.fundus examination,.non-contact intraocular pressure measurement,.measurement of corneal thickness, and fundus photography.Results: Forty-two Tibetan children, aged between 4 and 18 years and diagnosed with congenital heart disease,.were enrolled in this study..The percentage of low visual acuity was4.76%, mean intraocular pressure was(13.67±2.15) mm Hg,average corneal thickness was(492.55±33.79) μm,.96.43%had an anterior chamber depth of 1 / 2 corneal thickness(CT),and 35.7% had an obvious fundus vascular tortuosity.Conclusion:.This study adds to the understanding of the ocular status of the population living in the Tibet plateau, thereby offering clinical evidence for the prevention and treatment of eye diseases in this area.
基金Greece and the European Union(European Social Fund-ESF)through the Operational Programme“Human Resources Development,Education and Lifelong Learning”in the context of the project“Strengthening Human Resources Research Potential via Doctorate Research”(MIS-5000432),implemented by the State Scholarships Foundation(ΙΚΥ)the special account for research grants of the National and Kapodistrian University of Athens,Athens,Greece.
文摘BACKGROUND Vascular endothelial dysfunction is an underlying pathophysiological feature of chronic heart failure(CHF).Patients with CHF are characterized by impaired vasodilation and inflammation of the vascular endothelium.They also have low levels of endothelial progenitor cells(EPCs).EPCs are bone marrow derived cells involved in endothelium regeneration,homeostasis,and neovascularization.Exercise has been shown to improve vasodilation and stimulate the mobilization of EPCs in healthy people and patients with cardiovascular comorbidities.However,the effects of exercise on EPCs in different stages of CHF remain under investigation.AIM To evaluate the effect of a symptom-limited maximal cardiopulmonary exercise testing(CPET)on EPCs in CHF patients of different severity.METHODS Forty-nine consecutive patients(41 males)with stable CHF[mean age(years):56±10,ejection fraction(EF,%):32±8,peak oxygen uptake(VO2,mL/kg/min):18.1±4.4]underwent a CPET on a cycle ergometer.Venous blood was sampled before and after CPET.Five circulating endothelial populations were quantified by flow cytometry:Three subgroups of EPCs[CD34+/CD45-/CD133+,CD34+/CD45-/CD133+/VEGFR2 and CD34+/CD133+/vascular endothelial growth factor receptor 2(VEGFR2)]and two subgroups of circulating endothelial cells(CD34+/CD45-/CD133-and CD34+/CD45-/CD133-/VEGFR2).Patients were divided in two groups of severity according to the median value of peak VO2(18.0 mL/kg/min),predicted peak VO2(65.5%),ventilation/carbon dioxide output slope(32.5)and EF(reduced and mid-ranged EF).EPCs values are expressed as median(25th-75th percentiles)in cells/106 enucleated cells.RESULTS Patients with lower peak VO2 increased the mobilization of CD34+/CD45-/CD133+[pre CPET:60(25-76)vs post CPET:90(70-103)cells/106 enucleated cells,P<0.001],CD34+/CD45-/CD133+/VEGFR2[pre CPET:1(1-4)vs post CPET:5(3-8)cells/106 enucleated cells,P<0.001],CD34+/CD45-/CD133-[pre CPET:186(141-361)vs post CPET:488(247-658)cells/106 enucleated cells,P<0.001]and CD34+/CD45-/CD133-/VEGFR2[pre CPET:2(1-2)vs post CPET:3(2-5)cells/106 enucleated cells,P<0.001],while patients with higher VO2 increased the mobilization of CD34+/CD45-/CD133+[pre CPET:42(19-73)vs post CPET:90(39-118)cells/106 enucleated cells,P<0.001],CD34+/CD45-/CD133+/VEGFR2[pre CPET:2(1-3)vs post CPET:6(3-9)cells/106 enucleated cells,P<0.001],CD34+/CD133+/VEGFR2[pre CPET:10(7-18)vs post CPET:14(10-19)cells/106 enucleated cells,P<0.01],CD34+/CD45-/CD133-[pre CPET:218(158-247)vs post CPET:311(254-569)cells/106 enucleated cells,P<0.001]and CD34+/CD45-/CD133-/VEGFR2[pre CPET:1(1-2)vs post CPET:4(2-6)cells/106 enucleated cells,P<0.001].A similar increase in the mobilization of at least four out of five cellular populations was observed after maximal exercise within each severity group regarding predicted peak,ventilation/carbon dioxide output slope and EF as well(P<0.05).However,there were no statistically significant differences in the mobilization of endothelial cellular populations between severity groups in each comparison(P>0.05).CONCLUSION Our study has shown an increased EPCs and circulating endothelial cells mobilization after maximal exercise in CHF patients,but this increase was not associated with syndrome severity.Further investigation,however,is needed.
文摘objective To study how to improve the accuracy of treadmill exercise testing(TET).Methods TET was performed before coronary angiography (CAG) for 147 subjects admitted into our departmentbecause of suspicion of angina or coronary artery disease (CAD), then: (1) Every subject was inquired about hissymptoms and physically examined in detail before the testing. Chest pain was divided into ischemic (ICP) ornon - ischemic (NICP) pattern. (2) Informations got from TET, like angina, ST segment depression, and SPB ratio,were analyzed in detail. Results (1) When the patients showed chest pain of ICP pattern and positive TETresults, the sensitivity of TET would be 95%, for patients with NICP chest pain, the negative result of TET meansthat their coronary arteries is normal with the accurate possibility of 98%. (2) By our new criteria of TET, thesensitivity and specificity of TET were 89% and 95% respectively, both higher than those in theliterature. Conclusion in this report, we found TET was more sensitive and accurate if we knew the symptomsprior, especially in detail the characteristics of chest pain, and consider different parameters in combination.
文摘Rationale PH is a pathophysiological disorder involving multiple clinical conditions and is often concomitant with other cardiopulmonary diseases,and related differential diagnosis is quite tricky for its various etiologies.Patient characteristics A 49-year-old man presented with 1-year history of nocturnal paroxysmal dyspnea and was transferred to our hospital.