Interventional treatment for congenital heart disease and its severe complications

Background To approach the incidence, cause and possible treatment of severe complications induced during or after congenital heart disease interventions. Methods Interventional procedures of congenital heart disease were performed in 654 patients from January 2003 to October 2009, which were divided into four groups, i.e. patent ductus arteriosus （PDA）, atrial septum defect （ASD）, pulmonary stenosis（PS）, ventricular septum defect（VSD）, among them 32 patients with severe complications were retrospectively analyzed. Results A total of 654 cases accepted the treatment of intrusion. There was one death in all the patients, ten patients failed in the operation. The overall severe complication rate was 4.89% （32/654）, in which 2.29%（4/175） in PDA group, 5.26%（10/190） in ASD group, 5.77% （3/52） in PS group, 6.33%（15/237） in VSD group, respectively. Conclusions The severe complication rates of interventional therapy for congenital heart disease are low, it is a relatively safe interventional method, and careful supervision is necessary during or after procedure.     

Interventional therapy for neonates with critical congenital heart disease

Transcatheter interventional therapies for children continue to evolve at a remarkable rate. Perhaps no where are these procedures more important than in the critically ill newborn. In this core curriculum review article several of the most commonly performed interventions performed in critically ill newborns are discussed with regards to technique and outcomes. © 2008 Wiley‐Liss, Inc.     

Racial and ethnic disparities in coronary,vascular, structural,and congenital heart disease

Cardiovascular disease (CVD) remains the leading cause of death in the United States. However, percutaneous interventional cardiovascular therapies are often underutilized in Blacks, Hispanics, and women and may contribute to excess morbidity and mortality in these vulnerable populations. The Society for Cardiovascular Angiography and Interventions (SCAI) is committed to reducing racial, ethnic, and sex-based treatment disparities in interventional cardiology patients. Accordingly, each of the SCAI Clinical Interest Councils (coronary, peripheral, structural, and congenital heart disease [CHD]) participated in the development of this whitepaper addressing disparities in diagnosis, treatment, and outcomes in underserved populations. The councils were charged with summarizing the available data on prevalence, treatment, and outcomes and elucidating potential reasons for any disparities. Given the huge changes in racial and ethnic composition by age in the United States (Figure 1), it was difficult to determine disparities in rates of diagnosis and we expected to find some racial differences in prevalence of disease. For example, since the average age of patients undergoing transcatheter aortic valve replacement (TAVR) is 80 years, one may expect 80% of TAVR patients to be non-Hispanic White. Conversely, only 50% of congenital heart interventions would be expected to be performed in non-Hispanic Whites. Finally, we identified opportunities for SCAI to advance clinical care and equity for our patients, regardless of sex, ethnicity, or race.     

Results of the society of cardiac angiography and interventions survey of physicians and training directors on procedures for structural and valvular heart disease

Background: Minimal information is available on the number and type of procedures being performed for structural and valvular heart disease, the physicians who perform these procedures, and on the training requirements for this emerging field. Methods: Surveys were performed using an online survey of members of the Society of Cardiac Angiography and Interventions (SCAI), including its Council on Structural Heart Disease and the Congenital Heart Disease Committee. The responses of 107 US‐based interventional cardiologists were analyzed. A second questionnaire of a purposive sample of 10 training directors of US interventional cardiology programs was also performed. Results: Although many procedures (e.g., transseptal puncture, PFO, and ASD closure) are commonly performed by most respondents, others are limited to a significant minority of respondents (e.g., alcohol septal ablation, transcatheter valve repair, and implantation). In addition, the number of procedures performed varies greatly as does the training directors' estimate of the number necessary to gain proficiency. There is no single method being used to gain the requisite skills. A number of factors that limit the more widespread growth of this field were identified. Conclusions: The field of intervention for structural and valvular heart disease is new, growing rapidly, and will require a core knowledge base and new didactic methods. The cardiovascular community will be challenged to devise new training standards and credentialing approaches to serve interventionalists interested in this field. © 2010 Wiley‐Liss, Inc.     

经导管介入治疗小儿先天性心脏病复合畸形

目的　探讨经皮动脉导管未闭 (PDA)、房间隔缺损 (ASD)、室间隔缺损 (VSD)封堵术以及肺动脉瓣成形术(PBPV)在小儿复合先天性心脏病介入治疗中联合应用的可行性。方法　自 1998年 11月至 2 0 0 3年 12月 ,共 2 8例患有复合先天心血管畸形的住院患儿施行联合介入治疗 ,其中联合进行ASD封堵和PBPV术 15例 ,PDA封堵 (其中包括弹簧圈法和蘑菇伞法 )和PBPV术 7例 ,ASD和PDA封堵术 5例 ,VSD封堵和PBPV术 1例。结果　本组病例均为一次心导管术程中同时进行 2种介入治疗 ,成功率为 10 0 % (2 8/ 2 8) ,取得满意的治疗效果 ,无严重并发症 ,经 3个月至 4年随诊证实疗效确切。结论　在病例选择恰当的情况下 ,对小儿先天性心脏病复合畸形进行联合介入治疗是安全、可行的     

Opportunities for training to advance the care for adults with congenital heart disease with advanced circulatory failure

Heart failure is an emerging issue with important implications in adult patients with congenital heart disease. Practitioners with expertise in both adult congenital heart disease and heart failure are needed to manage this growing and often complex population. In the United States, the optimal training pathway to enable practitioners to best care for these patients is ill‐defined. This article explores possibilities and issues that interested trainees may encounter during their training experience.     

结构性心脏病室性心动过速治疗方法进展

结构性心脏病室性心动过速（室速）的治疗面临巨大挑战。主要是室速基质复杂、部位深在或靠近心外膜和血管、发作时血流动力学不稳定,上述因素导致药物治疗和消融治疗成功率较低,复发率高,远期预后差。本文就近年来室速的非药物治疗进展进行综述。     

Body mass index in adults with congenital heart disease

Objective: To investigate the status of body mass index (BMI) in adult people with congenital heart disease (ACHD).
Methods: Five hundred thirty‐nine adults with CHD (53.8% men) were seen in the outpatient clinic from 2013 to 2015 and compared to a reference population (n = 1737). The severity of CHD was categorized as mild, moderate, and severe ac‐ cording to standard guidelines. Patients were categorized based on BMI as under‐ weight (<18.5), overweight (25‐30), or obese (>30). Echocardiography and magnetic resonance imaging were used to measure ventricular function while exercise capac‐ ity was estimated via cardiopulmonary exercise test.
Results: Adults with CHD had slightly lower BMI than the reference group (24.1 ± 4.3 vs 24.6 ± 4.3; P = .012). Men in the mild and severe group (23.9 ± 3.6; 23.3 ± 4.4 vs 25.1 ± 3.7; P = .007; P = .023) and women in the severe group (21.6 ± 3.3 vs 24.2 ± 4.7; P < .001) had lower BMI compared to the reference group. In the subgroups, men with ventricular septal defect, coarctation of aorta/ventricular septal defect and Fontan cir‐ culation and women with Fontan circulation had lower BMI than the reference group. Underweight was more prevalent in women with severe lesions compared to the refer‐ ence group (22.2% vs 3.8%; P < .001). BMI was associated with age and exercise ca‐ pacity in patients with mild and moderate lesions, while higher BMI was related to better ventricular function in women with Fontan circulation.
Conclusion: Underweight was more prevalent in ACHD patients with severe lesions. Special attention should be paid to the possible existence of underweight‐related comorbidities.     

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.     

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.     

Obesity trends in children,adolescents, and young adults with congenital heart disease

Objectives: To determine the prevalence, age of onset, and risk factors for overweight and obesity in children with congenital heart disease (CHD).
Study Design: Children with CHD who were seen at our institution from 1996 to 2017 were studied. Patients were full-time residents of the United States and were receiving all cardiac care at our institution. Patients were categorized by age and CHD diagnosis. The date of last normal weight for age and the date of first recorded weight in the range of overweight and obese were documented.
Results: Nine hundred sixty-eight patients with CHD were included. The prevalence of overweight and obesity was 31.5% and 16.4%, respectively. For patients who became overweight or obese, the last recorded normal weight was between 6 and 10 years of age. Electrophysiologic disease and older age were risk factors for obesity.
Conclusions: Children with CHD have an increasing risk of becoming overweight and obese in early childhood. This study provides important information and identifies critical period to implement preventative measures and counsel families about the risk of obesity in CHD.     

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.     

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

     

Psychopathology in young adults with congenital heart disease. Follow-up results

Aims The aim of the present study was to assess the occurrence ofa wide range of behavioural and emotional problems long-term(>9 years) after surgical correction for congenital heart diseasein infancy and childhood. Methods The problem scores on the Young Adult Self-Report of 166 19–25-year-oldadults with congenital heart disease were compared with thoseof subjects of similar age from the general population. Results On most Young Adult Self-Report scales no differences were foundbetween the mean problem scores of the congenital heart disease-adultsand reference peers. On only two Young Adult Self-Report scales(i.e. Somatic Complaints and Strange) and the total problemscore were significant though small differences found betweenthe mean problem scores of the congenital heart disease adultsand reference peers. No significant relationship was found betweencardiac diagnosis and problem behaviours in congenital heartdisease adults. No relationship was found between IQ scoresand problem behaviours in congenital heart disease adults. Conclusion Overall, it can be concluded that the results of the congenitalheart disease adults were not unfavourable, since the differencesin mean (total) problem scores between the congenital heartdisease sample and reference group were small and limited toonly two specific problem areas.     

Transesophageal echocardiography for congenital heart disease

Surface echocardiographic imaging of small children is routinely successful in defining anatomical details and Doppler flow patterns with even the most complex congenital cardiac malformations. However, in larger children or adults, imaging is frequently limited. A recent expansion of the role of echocardiography is intraoperative epicardial imaging. Epicardial and postoperative imaging, however, have significant limitations. To avoid some of these limitations, transesophageal echocardiography has increasingly been used in the arena of congenital heart disease. The more recent development of small sized gastroscopic probes has allowed transesophageal echocardiographic assessment of congenital heart disease in children down to newborn size. As detailed studies of individual lesions are reported, it has become clear that the mere presence of a congenital heart defect is not an indication for transesophageal echocardiography in most children if imaging can be accomplished by surface examination. However, transesophageal echocardiography may be indicated for the intraoperative or postoperative assessment of that defect, particularly when repair has been difficult or is known to be associated with significant residual abnormalities. Cardiac structures encountered with horizontal and vertical imaging plane transducers have been described and should be completely familiar to the examining echocardiographer.     

Anticoagulation practices in adults with congenital heart disease and atrial arrhythmias in Switzerland

Background: In adults with congenital heart disease (CHD) and atrial arrhythmias, recommendations for thromboprophylaxis are vague and evidence is lacking. We aimed to identify factors that influence decision-making in daily practice.
Methods: From the Swiss Adult Congenital HEart disease Registry (SACHER) we identified 241 patients with either atrial fibrillation (Afib) or atrial flutter/intraatrial reentrant tachycardia (Aflut/ IART). The mode of anticoagulation was reviewed. Logistic regression models were used to assess factors that were associated with oral anticoagulation therapy.
Results: Compared with patients with Aflut/IART, patients with Afib were older (51 ± 16.1 vs 37 ± 16 years, P < .001) and had a higher CHA₂DS₂-VASc (P < .001) and HAS-BLED scores (P = .005). Patients with Afib were more likely on oral anticoagulation than patients with Aflut/IART (67% vs 43%, P < .001). In a multivariate logistic regression model, age [odds ratio (OR) 1.03 per year, 95%CI (1.01-1.05), P = .019], atrial fibrillation [OR 2.75, 95%CI (1.30-5.08), P = .007], nonparoxysmal atrial arrhythmias [OR 5.33, 95%CI (2.21-12.85)], CHA2DS2-VASc-Score >1 [OR 2.93, 95%CI (1.87-4.61), P < .001], and Fontan palliation [OR 17.5, 95%CI (5.57-54.97), P < .001] were independently associated with oral anticoagulation treatment, whereas a HAS-BLED score >1 was associated with absence of thromboprophylaxis [OR 0.32, 95%CI (0.17-0.60), P < .001].
Conclusions: In this multicenter study, age, type, and duration of atrial arrhythmias, CHA2DS2- VASc and HAS-BLED scores as well as a Fontan palliation had an impact on the use of thromboprophylaxis in adult CHD patients with atrial arrhythmias. In daily practice, anticoagulation strategies differ between patients with Afib and those with Aflut/IART. Prospective observational studies are necessary to clarify whether this attitude is justified.