Physical activity is associated with improved aerobic exercise capacity over time in adults with congenital heart disease

Background

Impaired exercise capacity is common in adults with congenital heart disease (ACHD). This impairment is progressive and is associated with increased morbidity and mortality. We studied the influence of the frequency of at least moderately strenuous physical activity (PhysAct) on changes in exercise capacity of ACHD patients over time.

Methods

We studied ACHD patients ≥ 21 years old who had repeated maximal (RER ≥ 1.09) cardiopulmonary exercise tests within 6 to 24 months. On the basis of data extracted from each patient's clinical records, PhysAct frequency was classified as (1) Low: minimal PhysAct, (2) Occasional: moderate PhysAct < 2 times/week, or (3) Frequent: moderate PhysAct ≥ 2 times/week.

Results

PhysAct frequency could be classified for 146 patients. Those who participated in frequent exercise tended to have improved pVO₂ (?pVO₂ = + 1.63 ± 2.67 ml/kg/min) compared to those who had low or occasional activity frequency (?pVO₂ = + 0.06 ± 2.13 ml/kg/min, p = 0.003) over a median follow-up of 13.2 months. This difference was independent of baseline clinical characteristics, time between tests, medication changes, or weight change. Those who engaged in frequent PhysAct were more likely to have an increase of pVO₂ of ≥ 1SD between tests as compared with sedentary patients (multivariable OR = 7.4, 95%CI 1.5–35.7). Aerobic exercise capacity also increased for patients who increased activity frequency from baseline to follow-up; 27.3% of those who increased their frequency of moderately strenuous physical activity had a clinically significant (at least + 1SD) increase in pVO₂ compared to only 11% of those who maintained or decreased activity frequency.

Conclusions

ACHD patients who engage in frequent physical activity tend to have improved exercise capacity over time.     

Risky business: insuring adults with congenital heart disease.

Accurate prognostication in congenital heart disease is vital for purposes of obtaining insurance, yet can be problematic for patients, physicians and insurers. This article discusses the scope of the problem, and describes the process of evaluating life insurance. Mortality data as well as predictors of adverse outcomes for individual congenital heart lesions are reviewed. Practical tips for patients and their physicians are given to aid in successful application for insurance. To expand the possibility of future patients obtaining insurance coverage, the ongoing reporting and constant updating of very long-term survival data in congenital heart disease is emphasized.     

Measures of exercise capacity in adults with congenital heart disease

Background

Exercise capacity in grown-ups with congenital heart disease (GUCH) is mostly reported by peak oxygen consumption (peak VO₂). Our aim was to evaluate the maximal character of exercise tests, and to investigate submaximal measures of exercise capacity.

Methods

Adults with Coarctation of the Aorta (COA, n = 155), Tetralogy of Fallot (TOF, n = 98), dextro-Transposition of the Great Arteries (dTGA, n = 68) and Univentricular Heart (UVH, n = 10), and 122 healthy adults performed cardiopulmonary exercise testing until exhaustion. Gas exchange was measured breath by breath. The maximal performance of the test was evaluated by respiratory exchange ratio (RER), ventilatory equivalent for oxygen and Borg scale. Oxygen uptake efficiency slope (OUES), VE/VCO₂ slope and VO₂/WR slope were calculated and ventilatory anaerobic threshold (VAT) was defined. Correlations of these measures with peak VO₂ were calculated.

Results

GUCH showed significantly lower peak VO₂ than controls (p < 0.001), declining from 80% in COA, 74% in TOF, 64% in dTGA, to 55% in UVH. Compared to suggested criteria, mean peak RER and median Borg scale indicated a maximal effort in GUCH, however these results were significantly lower than controls (p < 0.05). OUES, VO₂/WR slope and VAT were significantly lower in patients compared to controls. OUES (r = 0.853) and VAT (r = 0.840) correlated best with peak VO₂; VO₂/WR slope (r = 0.551) and VE/VCO₂ slope (r = −0.421) correlated to a lesser degree (p < 0.001).

Conclusion

The investigated GUCH show reduced exercise tolerance compared to controls, related to the underlying heart defect. Different expressions of exercise tolerance clearly reveal the same differences in exercise capacity across groups of GUCH.     

Recommendations to organize care for adults with congenital heart disease in the Czech Republic

This paper was formed by the Expert committee for congenital heart disease (CHD) in adults – a division of Czech Society of Cardiology (ČKS). It was designed as an appendix to National cardiovascular programme ČKS created in 2013 and was based on Recommendations for organization of care for adults with congenital heart disease and for training in the subspecialty of ‘Grown-up Congenital Heart Disease’ in Europe: a position paper of the Working Group on Grown-up Congenital Heart Disease of the European Society of Cardiology created in 2014 [1].Aims of this paper are: To optimize medical care in all its aspects for adults with CHD in the Czech Republic, to facilitate easy transition between paediatric and adult medical care, to enable research in the field in order to create evidence based care, to support training of regional cardiologists and other specialists who are involved in monitoring adult patients with CHD, to help with communication with national institutions, to provide information for other medical workers and patients, to consolidate resources.     

The impact of interventional cardiology for the management of adults with congenital heart defects.

The objective of this study is to assess the impact of interventional cardiology procedures for the management of ACHD. The interventional approach to the management of CHD in the adult population is becoming increasingly recognized as the preferred treatment option for a wide number of congenital cardiac conditions. The files of all consecutive patients over 18 years of age who were hospitalized in our department from January 2000 to December 2004 were reviewed. Over the study period, 1,115 ACHD (583 women; mean age 41 +/- 13.8, years, range 18-72 years) were hospitalized in our department; 752 patients underwent cardiac catheterization and 82.4% of them had an interventional procedure carried out. ASD (329/620) and PFO (159/620) closure account for 78% of all the procedures carried out, with a 2.7% of major complications incidence (all of them closing ASDs). Other procedures such as stenting aortic coarctation (40/620), ventricular septal defect closure (33/620), patent ductus arteriosus embolization (30/620), pulmonary valvuloplasty (12/620), stenting pulmonary artery branches (8/620), etc (5/620) were carried out. The most important complication was one death, which occurred in the case of a 22-year-old woman after stent implantation for a recurrent aortic coarctation. A trivial residual shunt was detected in only 5% of the patients who had a 6-month follow-up after VSD closure; no residual shunt was found after PDA embolization during the 12-month follow-up. In conclusion, we believe that the interventional approach is a safe and successful treatment option for a wide number of congenital cardiac conditions. The increasing use of catheter interventions for these patients will be responsible for an increase of complex cases in surgery.     

Reduced health-related quality of life in older patients with congenital heart disease: A cross sectional study in 2360 patients

Objective

Health-related quality of life questionnaires are frequently used to involve patients' impressions and feelings in the outcome evaluation. In patients with congenital heart disease (CHD) methodological heterogeneities, assessment in different age and diagnostic groups led to controversial findings. This study aims to give a comprehensive answer to the health-related quality of life in patients with CHD.

Patients and methods

From July 2001 to June 2013, 2360 patients (1058 female, 28.6 ± 11.2 years, range 14–75 years) with various kinds of CHD underwent a quality of life assessment with the SF-36 questionnaire and underwent a cardiopulmonary exercise test as part of their routine follow-up.

Results

Physical component summary score (PCS) develops from 96.1 %predicted in patients younger than 20 years, to 96.2 %predicted in patients aged 20 to 30 years, 92.3 %predicted in patients aged 30 to 40 years, and 92.6 %predicted in patients 40 years or older (r = − .114; p < .001). The decline was more prominent in the mental component summary score (MCS) declining from 104.1 %predicted in patients younger than 20 years, to 103.4 %predicted, 99.9 %predicted, and 97.5 %predicted (r = − .132; p < .001). Exercise capacity was impaired with 80.1 ± 23.0 %predicted and also declined slowly with age (r = − .084; p < .001).

Conclusions

Health-related quality of life in patients with CHD is progressively reduced in older age-groups. The decline is small, but more prominent in the mental components. This should be considered in the interpretation of studies, and strategies have to be developed to reduce this effect in future.     

Genetics of congenital heart disease

Cardiovascular malformations are the most common type of birth defect and result in significant mortality worldwide. The etiology for the majority of these anomalies remains unknown but genetic factors are being recognized as playing an increasingly important role. Advances in our molecular understanding of normal heart development have led to the identification of numerous genes necessary for cardiac morphogenesis. This work has aided the discovery of an increasing number of monogenic causes of human cardiovascular malformations. More recently, studies have identified single nucleotide polymorphisms and submicroscopic copy number abnormalities as having a role in the pathogenesis of congenital heart disease. This review discusses these discoveries and summarizes our increasing understanding of the genetic basis of congenital heart disease.     

Transcatheter ablation of arrhythmias associated with congenital heart disease

The improvement of surgical techniques resulted in significant life prolongation of many young patients with congenital heart disease (CHD). However, as these patients reach adulthood, their risk for late complications associated with surgery is also increased. One of the most difficult challenges associated with CHD is the high incidence of cardiac arrhythmias that arise from either the myocardial substrate created by abnormal physiology (pressure/volume changes, septal patches, and suture lines) or the presence of surgical scar. Catheter ablation is proven to be effective in treating atrial and ventricular arrhythmias in structurally normal hearts, and has also been used to treat arrhythmias in adults with congenital heart disease. In this review we provide an overview about diagnostic challenges, mapping and ablation techniques and outcome of patients undergoing transcatheter ablation procedures.     

Indications for heart transplantation in congenital heart disease

In this review we have looked at indications for cardiac transplantation in congenital heart disease. An outline of the general principles of the use of transplant as a management strategy both as a first line treatment and following other surgical interventions is discussed. We explore the importance of the timing of patient referral and the evaluations undertaken, and how the results of these may vary between patients with congenital heart disease and patients with other causes of end-stage heart failure. The potential complications associated with patients with congenital heart disease need to be both anticipated and managed appropriately by an experienced team. Timing of transplantation in congenital heart disease is difficult to standardize as the group of patients is heterogeneous. We discuss the role and limitations of investigations such as BNP, 6 minute walk, metabolic exercise testing and self estimated physical functioning. We also discuss the suitability for listing. It is clear that congenital heart patients should not be considered to be at uniform high risk of death at transplant. Morbidity varies greatly in the congenital patient population with the failing Fontan circulation having a far higher risk than a failing Mustard circulation. However the underlying issue of imbalance between donor organ supply and demand needs to be addressed as transplant teams are finding themselves in the increasingly difficult situation of supporting growing numbers of patients with a diverse range of pathologies with declining numbers of donor organs.     

先天性心脏病介入治疗的主要并发症

我国是世界上先天性心脏病（简称先心病）发病率较高的国家之一,发病率约占全部活产婴儿的0．7％加．8％,每年有15万-17万先心病新生儿患者,其中约10万患儿需要手术治疗。常见的先心病,如室间隔缺损（ventricular septal defect,VSD）、动脉导管未闭（patent ductus arteriosus,PDA）、     

先天性心脏病的病因学研究

采用配对病例对照研究，探讨先天性心脏病（先心病）与风疹病毒、疱疹病毒、柯萨奇病毒感染和其他危险因素的关系。结果：病例组患儿、母亲的风疹病毒感染率分别比对照组患儿、母亲高，差异有显著性（分别Ｐ＜０．００５、＜０．０１）。单因素分析和多因素条件Ｌｏｇｉｓｔｉｃ回归分析均提示先心病与风疹病毒感染之间，病因联系强度具统计学意义（均Ｐ＜０．００１）。此外，母亲先兆流产史、孕期服药史也是先心病发病的危险因素。根据前述结果提出，预防先心病要尽快研制、推广育龄妇女风疹病毒疫苗接种。做好孕妇保健工作、避免感染、正确用药等也是重要环节。     

婴幼儿先天性心脏病476例外科治疗

目的 探讨婴幼儿先天性心脏病外科治疗经验.方法 2001年1月～2005年6月行婴幼儿先天性心脏病直视手术476例,男289例,女187例,年龄13日～36个月,体重3～16kg.非紫绀型先天性心脏病患儿307例,全部Ⅰ期根治,紫绀型先天性心脏病患儿169例,行Ⅰ期根治或分期手术.合并症有:中、重度肺动脉高压107例,中重营养不良37例,反复肺炎、抗心力衰竭药不能控制47例.结果 95.2%(453/476)痊愈出院.全组手术死亡率为4.8%(23/476),2004年以来死亡率2.9%(5/175);死亡病儿多为年龄小于1岁和复杂先天性心脏病,主要死因为低心输出量综合征和肺部并发症;限期手术死亡率6%(3/47).结论 婴幼儿先天性心脏病早期手术疗效良好.     

非编码RNA在先天性心脏病中的研究进展

先天性心脏病是心血管结构及心电传导系统先天性畸形疾病的总称,是常见并且严重的出生畸形。随着最新分子生物学及遗传生物学的深入研究,越来越多的证据表明 非编码RNA 与先天性心脏病的发生、发展相关。本文将就目前国内、外非编码RNA 与先天性心脏病的关系的研究进展进行综述。     

婴幼儿先天性心脏病1018例外科治疗

目的总结1018例婴幼儿行先天性心脏病(以下简称先心病)外科治疗的经验,探讨提高婴幼儿外科手术成功的方法和婴幼儿先心病的适当手术时机。方法对1992年12月至2004年7月间1018例3岁以内婴幼儿先天性心脏病外科治疗进行总结。1018例中男性613例占60.2%,女性405例占39.8%。年龄39h～36个月,平均(14.3±10.3)个月,其中新生儿8例(0.8%),婴儿514例(50.5%),幼儿496例(48.7%)。体质量(2.3～18.5)kg,平均(8.6±2.9)kg。术前反复发生呼吸道感染183例占16.8%。本组病人非紫绀型819例占80.5%,紫绀病人199例占19.5%。1018例病人非直视手术87例占8.6%,体外循环下直视手术931例占91.4%。结果住院死亡58例,死亡率5.7%。不同年龄段住院死亡率分别为:新生儿为50%,婴儿为9.6%,幼儿2.7%。不同体重死亡率分别为<5kg者15.3%,5～10kg者6.2%,11～15kg者2.1%,>15kg者无死亡。术后并发症发生率14.85%(150例),呼吸系统并发症占并发症的41.3%,循环系统占34.0%,其他并发症有切口感染、肺高压危象以及肝、肾功能不全等。出院的960例病人,758例获得随访,随访率78.5%,随诊时间2个月～10年6个月;晚期死亡1例,原因不明;室缺残余漏(VSD)6例,其中3例再次手术治疗,另3例因分流量小仍在观察中;智力障碍1例,为脑缺氧后遗症,慢性心力衰竭1例系法     

Individual quality of life in adults with congenital heart disease: a paradigm shift.

AIMS: During the last decade, a paradigm shift has emerged in the measurement of quality of life, from the use of standard questionnaires towards a more individualized approach. Therefore, this study examined individual quality of life in adults with congenital heart disease and explored potential differences with those reported by matched, healthy control subjects. METHODS AND RESULTS: We examined 579 adults with congenital heart disease. A subsample of 514 of these patients was matched for age, gender, educational level, and employment status with 446 healthy counterparts. Individual quality of life was assessed using the Schedule for the Evaluation of Individual Quality of Life-Direct Weighting (SEIQoL-DW). Twelve domains affecting patients' quality of life were identified. Family, job/education, friends, health, and leisure time were the most prominent quality of life domains. Significantly fewer patients than control subjects considered financial means and material well-being and future to be important determinants of quality of life. CONCLUSION: Assessment of quality of life in adults with congenital heart disease that focusses on the individual is appropriate for obtaining in-depth information on issues relevant for patients' quality of life. This represents a paradigm shift in the measurement of this concept.     

Mechanisms and outcomes of catheter ablation for biatrial tachycardia in adults with congenital heart disease

1. Download : Download high-res image (225KB)
2. Download : Download full-size image

     

右心声学造影在先天性心脏病诊断上的应用

心脏声学造影即心脏超声造影(又称造影超声心动图)1969 年由Gralniak 首先提出,在彩色多普勒超声心动图问世前在先天性心脏病的诊断方面发挥了重要作用.彩色多普勒(CDFI)的普及应用极大地提高了超声诊断先天性心脏病的准确性, 尤其对于左向右分流性先天性心脏病,CDFI能直观显示异常血流的起源、流向及流速,敏感性高,但在显示右向左分流时则敏感性较低[ 1 ].右心声学造影安全、无创、可重复性强,能提供更为详细的右向左分流信息,特别是在复杂心血管畸形的鉴别诊断中具有重要的诊断价值,可为临床提供丰富的解剖及血流动力学资料[ 2 ].即使在彩色多普勒普及的情况下,对右向左分流的先天性心脏病,右心声学造影仍不失为一种有效的诊断方法.