Common long-term complications of adult congenital heart disease: avoid falling in a H.E.A.P.

Advances in cardiology and cardiac surgery have transformed the outlook for patients with congenital heart disease (CHD) so that currently 85% of neonates with CHD survive into adult life. Although early surgery has transformed the outcome of these patients, it has not been curative. Heart failure, endocarditis, arrhythmias and pulmonary hypertension are the most common long term complications of adults with CHD. Adults with CHD benefit from tertiary expert care and early recognition of long-term complications and timely management are essential. However, it is as important that primary care physicians and general adult cardiologists are able to recognise the signs and symptoms of such complications, raise the alarm, referring patients early to specialist adult congenital heart disease (ACHD) care, and provide initial care.

In this paper, we provide an overview of the most commonly encountered long-term complications in ACHD and describe current state of the art management as provided in tertiary specialist centres.     

Adult congenital heart disease: intensive care management and outcome prediction

Objective Improved patient survival and increasingly complex surgery have expanded the requirement for specialist care for patients with adult congenital heart disease (ACHD). Despite the recent publications of management guidelines for ACHD, data concerning optimal patterns of care in the peri-operative/critical care period of this challenging population are sparse. The aims of the current study were to therefore to determine the pattern of intensive care unit (ICU) management, resource utilisation and predictors of mortality in critically ill ACHD patients. Design, setting and patients Data were collected prospectively for patients with ACHD stratified for complexity of disease admitted to the ICU of a tertiary cardiothoracic centre (1997–2002). Multivariate analysis of pre-operative indices as predictors of mortality was performed. Of 342 ACHD admissions (total mortality 4.4%, simple 0%, moderate/complex 10.6%), the requirement for specialist investigations and interventions was high, reflected in ICU admission costs per patient (simple $5391 ± 130, moderate $13218 ± 261, complex $30074 ± 689). Standard severity of illness scoring systems did not accurately predict mortality; however, abnormal pre-operative thyroid function (p = 0.0048), creatinine (p = 0.0032) and bilirubin (p = 0.0021) were highly predictive of mortality. Conclusions Peri-operative mortality in patients with ACHD is low overall but varies with disease complexity. Such patients have a high requirement for specialist ICU investigation/intervention. Although standard severity of illness scoring is unhelpful, simple pre-operative parameters may predict peri-operative mortality. These findings reflect the requirement for specialist care, and have implications for planning service provision, training and operative consent in ACHD patients.     

Health care transition in congenital heart disease: the providers' view point

Unfortunately, despite recommendations for educational training of ACHD health care providers and the goal to create regional ACHD centers, current needs still far outweigh appropriate available services. From a practical standpoint, we will need to work from our current models of health care delivery, which vary because of geographic and institutional issues and the availability of appropriate resources, toward the ideal goal of regional ACHD centers. Successful transition of adolescents and adults with CHD requires collaboration and planning between the pediatric health care team and the ACHD health care team. Good communication and an atmosphere of mutual respect are essential. All members of the ACHD health care team need to be committed to improving the process of transition for the adolescent and adult with CHD. The advanced practice nurse (CNS or ARNP) from both the pediatric program and the adult program are often key players in this process. As ACHD health care providers, we must work toward decreasing barriers to care and become organized advocates for our patients. Ultimately, our goal is not only to provide a smooth transition from one model of care to another, it is to create a health care delivery system that will maximize the lifelong potential and function of adults with congenital heart disease.     

Care of the patient with adult congenital heart disease

The adult congenital heart disease (ACHD) patient population has numerous and distinctive needs and comprise a rapidly growing group within the modern day healthcare arena. Current treatment provisions along with recommendations for future specialized multidisciplinary ACHD teams are discussed. The exclusive needs of this patient population have forced professional groups to reconsider the availability and appropriateness of current healthcare delivery to this group of complex patients, whose survivability and life expectancy have continued to improve as a direct result of enhanced research, technology, medical, surgical, and nursing care. General care considerations including psychosocial issues and patient education are presented, and reveal the challenges and strengths of caring for this adult patient population within a pediatric facility. Congenital heart disease is a lifelong illness that requires a dedicated, consistent approach to healthcare delivery in order to guarantee quality care throughout the lifespan.     

Adolescents in transition of adult care: why the concern?

This article has described issues associated with transition planning for youth with CHD. A number of approaches have been described to address the needs of this growing number of youth with CHD. Transition to adulthood has been and will continue to be a significant clinical concern as the adult survival rate increases. Health care professionals who are involved in transition planning will be focused on the health-related concerns of the transfer to an ACHD, insurability, and long-term CHD management, as well as issues related to employment, education, social relationships, and independent living. Successful transitioning is dependent on long-term planning,. youth-centered approaches, service coordination, and referral to adult agencies. As youth, families, adult survivors, and experts have discovered, long-term survival means more than the physical aspects of CHD.     

Short-term outcome of isolated and associated congenital heart defects in relation to antenatal ultrasound screening.

OBJECTIVE: To investigate the outcome of fetuses affected by congenital heart defects (CHD), either detected or undetected at ultrasound screening, according to their complexity and severity. DESIGN: The study group comprised 3633 malformed fetuses entered into the Eurofetus database of which 798 had CHD. We compared the short-term outcome in cases where a CHD was detected by ultrasound screening with that in cases where a CHD was not detected. Isolated and associated CHD (ICHD and ACHD) and the degree of severity of defects were considered separately. Outcome data included termination of pregnancy (TOP), intrauterine fetal death, neonatal death (< 6 days after birth), gestational age at diagnosis and at delivery, mode of delivery and birth weight. RESULTS: Of the 798 fetuses with CHD, 595 had ICHD and 203 had ACHD. The diagnosis of an anomaly was made significantly earlier in ACHD cases. TOP was chosen in 28% of cases with a prenatal diagnosis of CHD, 20% for ICHD and 37% for ACHD (P < 0.001). The survival rate of antenatally diagnosed fetuses was lower in those with ACHD than in those with ICHD (P < 0.001) and lower for fetuses with antenatal diagnosis than with postnatal diagnosis (P < 0.001); this was due to significant differences in the complexity and severity of the defect. Premature delivery (< 32 weeks) was more frequent in fetuses in which an antenatal diagnosis of CHD had been made. Severe CHD were diagnosed earlier and were associated with a higher rate of TOP and spontaneous loss. CONCLUSIONS: The severity of CHD has a positive influence on the sensitivity of screening but a negative influence on the outcome. CHD that are not diagnosed antenatally include a high proportion of benign malformations. This explains the apparent paradox of a poorer outcome in fetuses in which a CHD was detected prenatally compared to those fetuses in which the defect was undetected prenatally. However, prenatal diagnosis provides parents with the option of TOP or of preventive care in specialized centers.     

Integrating depression and chronic disease care among patients with diabetes and/or coronary heart disease: The design of the TEAMcare study

Diabetes and coronary heart disease (CHD) are two of the most prevalent medical illnesses in the US population and comorbid depression occurs in up to 20% of these patients. Guidelines for management of diabetes and CHD overlap for healthy lifestyle and disease-control recommendations. However, the majority of patients with these medical illnesses have been shown to have inadequate control of key risk factors such as blood pressure, LDL cholesterol, or blood sugar. Comorbid depression has been shown to adversely affect self-care of diabetes and CHD, and is associated with an increased risk of complications and mortality. Interventions that have improved quality and outcomes of depression care alone in patients with diabetes and CHD have not demonstrated benefits in self-care, improved disease control or morbidity and mortality. This paper describes the design and development of a new biopsychosocial intervention (TEAMcare) aimed at improving both medical disease control and depression in patients with poor control of diabetes and/or CHD who met the criteria for comorbid depression. A team approach is used with a nurse interventionist who receives weekly psychiatric and primary care physician caseload supervision in order to enhance treatment by the primary care physician. This intervention is being tested in an NIMH-funded randomized controlled trial in a large integrated health plan.     

Cost-effectiveness of nurse practitioner management of hypercholesterolemia following coronary revascularization

PURPOSE: To evaluate the cost-effectiveness of case management by a nurse practitioner (NP) to lower blood lipids in patients with coronary heart disease (CHD) from a managed care perspective. DATA SOURCES: A total of 228 consecutive, eligible adults with hypercholesterolemia and CHD were recruited during hospitalization after coronary revascularization. Patients were randomized to receive lipid management, including individualized lifestyle modification and pharmacologic intervention from an NP for 1 year after discharge in addition to their usual care (NURS) or to receive usual care (EUC) enhanced with feedback on lipids to their primary provider and/or cardiologist. A cost-effectiveness ratio was calculated using incremental costs of the NURS group per unit change and percent change in low-density lipoprotein cholesterol (LDL-C) for 1 year at 2004 values. CONCLUSIONS: The annual incremental cost-effectiveness of NP case management was 26.03 dollars per mg/dL and 39.05 dollars per percent reduction in LDL-C. When costs of NURS care for the second 6 months of management were compared to the first 6 months of management, nursing salary costs were lower as patients were established on cholesterol management regimens, but the reduction in costs was offset by the increase in incremental costs of drug treatment as the NP titrated the patient to higher drug dosages that were more costly. IMPLICATIONS FOR PRACTICE: The findings suggest that case management by an NP is a cost-effective approach for a managed care organization to consider in improving the care of patients with cardiovascular disease.     

Factors influencing satisfaction and well-being among parents of congenital heart disease children: development of a conceptual model based on the literature review

Aim and method: The treatment and management of congenital heart disease (CHD) has improved dramatically over the past 25 years, necessitating re-evaluation of satisfaction with care and well-being among CHD children and their parents (PCCHD). The present study reviews the published literature over the past 25 years on parental satisfaction with the paediatric care of CHD and well-being among the parents, with the specific aim of: (a) assessing the extent of psychosocial problems and grade of satisfaction with care and (b) modelling factors associated with satisfaction and well-being among the parents. Results: There is general agreement in the literature that PCCHD experience psychosocial morbidity to a higher degree than parents of children with other paediatric conditions and parents of healthy children. The research on satisfaction with care among PCCHD is not conclusive, though there is considerable agreement that a substantial proportion of PCCHD may not be receiving adequate information regarding the ill-child's condition, treatment and medical prognosis. Finally, based on the review of factors affecting satisfaction and well-being, a model is generated indicating that interactions between parental perception of CHD, psychosocial resources and social vulnerability may account for differences in well-being among PCCHD, which in turn may explain differences in satisfaction with care among them. Conclusion: A holistic approach to the care of CHD that acknowledges the role of parents' perception of CHD, need for psychosocial resources and social vulnerability in the adaptation process is recommended to improve parental satisfaction with the care of CHD.     

Interventional and surgical treatment of cardiac arrhythmias in adults with congenital heart disease

Arrhythmias are a major cause of morbidity, mortality and hospital admission in adults with congenital heart disease (CHD). The etiology of arrhythmias in this population is often multifactorial and includes electrical disturbances as part of the underlying defect, surgical intervention or hemodynamic abnormalities. Despite the numerous existing arrhythmia management tools including drug therapy, pacing and ablation, management of arrhythmias in adults with CHD remains difficult and challenging. Owing to improvement in mapping and ablation techniques, ablation and arrhythmia surgery are being performed more frequently in adults with CHD. However, there is little information on the long-term results of these treatment strategies. The purpose of this article is therefore to review the available data on nonpharmacological treatment of cardiac arrhythmias in adult patients with CHD and to give an overview of the available data on the early and late outcomes of these treatment strategies.     

成人先天性心脏病伴肺动脉高压患者血浆VEGF与TGF-β1浓度变化的相关研究

【目的】探讨成人先天性心脏病（CHD）并肺动脉高压（PAH）患者血浆中血管内皮生长因子（VEGF）和转化生长因子-β1TGF-β1）的浓度水平在疾病过程中的临床意义及价值。【方法】选择本院心内科、心胸外科收治的78例成人左向右分流型CHD患者均在右心导管检查中直接测定肺动脉平均压（mPAP）,按mPAP水平分为无PAH组（mPAP≤25mm-Hg）20例、轻度PAH组（25mmHg〈mPAP≤35mmHg）21例、中度PAH组（35mmHg〈mPAP≤45mmHg）14例及重度PAH组（mPAP〉45mmHg）23例。取患者主肺动脉血。选择同期心内科收治的预行射频消融术患者10例为正常对照组。对照组于清晨空腹状态下行射频消融术时抽股静脉血4m1。采用酶联免疫吸附法（ELISA）测定其肺动脉血浆VEGF及TGF-β1水平,分析二者与mPAP的关系。【结果】CHD组血浆VEGF及TGF-β1均显著高于对照组（P〈0．01）。四组患者肺动脉血浆VEGF水平和TGF-β1水平组间比较差异均有统计学意义（F=449．078,P〈0．05）,（F=57．347,P〈0．05）;肺动脉血浆VEGF浓度水平与mPAP呈正相关（r=0．814,P〈0．05）;肺动脉血浆TGF-β1浓度水平与mPAP呈正相关（r=0．723,P〈0．05）;患者血浆VEGF与TGF-β1水平呈正相关（r=0．758,P〈0．05）。【结论】VEGF及TGFBl参与了左向右分流型CHD并PAH的病理生理过程,其血浆水平可作为评估左向右分流型cHD患者PAH严重程度的参考指标之一。VEGF及TGF-β1对监测PAH发生发展动态变化过程有一定意义及临床价值。     

Interventional and surgical treatment of cardiac arrhythmias in adults with congenital heart disease

Arrhythmias are a major cause of morbidity, mortality and hospital admission in adults with congenital heart disease (CHD). The etiology of arrhythmias in this population is often multifactorial and includes electrical disturbances as part of the underlying defect, surgical intervention or hemodynamic abnormalities. Despite the numerous existing arrhythmia management tools including drug therapy, pacing and ablation, management of arrhythmias in adults with CHD remains difficult and challenging. Owing to improvement in mapping and ablation techniques, ablation and arrhythmia surgery are being performed more frequently in adults with CHD. However, there is little information on the long-term results of these treatment strategies. The purpose of this article is therefore to review the available data on nonpharmacological treatment of cardiac arrhythmias in adult patients with CHD and to give an overview of the available data on the early and late outcomes of these treatment strategies.     

Why there is a need to discuss pulmonary hypertension other than pulmonary arterial hypertension?

Pulmonary hypertension(PH) is a condition characterized by the elevation of the mean pulmonary artery pressure above 25 mm Hg and the pulmonary vascular resistance above 3 wood units. Pulmonary arterial hypertension(PAH) is an uncommon conditionwith severe morbidity and mortality, needing early recognition and appropriate and specific treatment. PH is frequently associated with hypoxemia, mainly chronic obstructive pulmonary disease and DPLD and/or left heart diseases(LHD), mainly heart failure with reduced or preserved ejection fraction. Although in the majority of patients with PH the cause is not PAH, a significant number of published studies are still in regard to group Ⅰ PH, leading to a logical assumption that PH due to other causes is not such an important issue. So, is there a reason to discuss PH other than PAH? Chronic lung diseases, mainly chronic obstructive lung disease and DPLD, are associated with a high incidence of PH which is linked to exercise limitations and a worse prognosis. Although pathophysiological studies suggest that specific PAH therapy may benefit such patients, the results presented from small studies in regard to the safety and effectiveness of the specific PAH therapy are discouraging. PH is a common complication of left heart disease and is related to disease severity, especially in patients with reduced ejection fraction. There are two types of PH related to LHD based on diastolic pressure difference(DPD, defined as diastolic pulmonary artery pressure- mean PAWP): Isolated post-capillary PH, defined as PAWP 15 mm Hg and DPD 7 mm Hg, and combined post-capillary PH and pre-capillary PH, defined as PAWP 15 mm Hg and DPD ≥ 7 mm Hg. The potential use of PAH therapies in patients with PH related to left heart disease is based on a logical pathobiological rationale. In patients with heart failure, endothelial dysfunction has been proposed as a cause of PH and hence as a target for treatment, supported by the presence of increased endothelin-1 activity and impaired nitric oxide-dependent vasodilation. Unfortunately, so far, there is no evidence supporting the use of specific PAH therapies in patients with PH related to left heart disease. In conclusion, the presence of PH in patients with conditions other than PAH contributes to the severity of the disease, affecting the outcome and quality of life. The disappointing results regarding the effectiveness of specific PAH therapies in patients withchronic lung diseases and LHD underline the need for seeking new underlying mechanisms and thus novel therapies targeting PH due to left heart disease and/or lung diseases.     

Pulmonary Hypertension: Evaluation and Management

Pulmonary hypertension (PH) is a hemodynamic state characterized by elevation in the mean pulmonary arterial pressure and pulmonary vascular resistance leading to right ventricular failure and premature death. PH can be the result of a variety of diseases of different etiologies. Pulmonary arterial hypertension (PAH) should be distinctly differentiated from pulmonary venous hypertension (PVH) as a result of left heart disease. PAH is commonly caused by or associated with an underlying pulmonary, cardiac, or systemic disease (APAH). In the absence of an identifiable etiology or associated underlying disease, PAH is referred to as idiopathic (IPAH). IPAH, formerly known as primary pulmonary hypertension (PPH), is a rare disease most commonly seen in women of childbearing age. Presenting symptoms and signs are nonspecific and include dyspnea on exertion, fatigue, and a loud pulmonary component of the second heart sound. Transthoracic Doppler echocardiography is an excellent noninvasive test to detect the presence of pulmonary hypertension, although every patient should receive a right heart catheterization to confirm the diagnosis. A detailed work up, including laboratory tests and imaging studies, is also indicated to rule out known causes of pulmonary hypertension. Several targeted treatment options have become available in recent years and include parenteral and inhaled prostanoids, oral endothelin receptor antagonists, and oral phosphodiesterase type-5 inhibitors. As a result of their complex care, patients should be referred to centers with expertise in pulmonary hypertension.     

Organizational and medical aspects of transition of juveniles with congenital heart defects to adult cardiology care

A growing, heterogeneous group of children with congenital heart disease is surviving into adulthood due to advances in medicine. These patients including those with simple and complex congenital heart disease and operated on during childhood are facing long-term complications. Superspecialist care and expertise are required during their life to deal with their unique problems the most common being ventricular failure, arrhythmias, valve and conduit longevity. Teenagers and adolescents disappear from both medical and parental care because of the lack of transition programs. Transition of care from pediatric to adult cardiologists must be organized in each country and must reflect regional history, regional politics and realities. Transition of care requires goodwill from parents, adolescents, pediatric and adult cardiologists. Transition clinics being held jointly by pediatric and adult cardiologists between the age of 16 and 18 years are essential to encourage the adolescents to take charge of their own life and health issues. Adequate information about their heart defect, their operations and their residual lesions may help them understand the implications for the future and improve their compliance. A transition program must include counseling on education, career, endocarditis prophylaxis, insurance and lifestyle issues such as sexuality and reproduction (including anticonception, pregnancy), cardiovascular risk factors and sports activities. Medical reports including operative reports and heart catheterization reports must be transferred to the adult cardiologists. In Switzerland, care of adults with congenital heart disease is based on three levels: 1) primary caregivers including general practiioners, internists and community cardiologists; 2) cardiologists with special commitment and expertise to patients with congenital heart disease who organize regional outpatient clinics; 3) supraregional referral centers with cardiologists trained in pediatric and adult congenital heart disease and experienced in the special needs, problems and management of this unique population. A close collaboration between pediatric and adult cardiologists participating jointly in the care of congenital heart disease patients is very important. An interdisciplinary team offers all non-invasive and invasive facilities for diagnostic and therapeutic procedures in a supraregional referral center. A close collaboration among all physicians is crucial for optimal care and management. Integration of adolescents with congenital heart disease into an adult medical system improves both compliance and quality of care and supports the patient's esteem because many medical aspects and lifestyle issues are completely different from those during childhood.     

Inequalities in socio‐economic status and invasive procedures for coronary heart disease: a comparison between the USA and the UK

Background: Coronary heart disease (CHD) is a serious health problem in the USA and UK. Low socio‐economic status (SES) has been associated with an increased prevalence of CHD and also with inequalities in related health outcomes. Rates of utilisation of invasive coronary procedures (ICPs), which improve CHD outcomes and quality of life, can be employed as indicators of quality of medical care. Objectives: To investigate and compare inequalities in care experienced by low SES CHD patients in the US Medicaid programme and the UK National Health Service (NHS) in relation to waiting times for, and access to, ICPs. Possible ways of addressing SES inequalities are proposed. Setting/Subject: Coronary heart disease patients eligible for ICPs in the US Medicaid programme and the UK NHS. Methods: A systematic literature search was performed for relevant SES inequalities. Data from 43 sources were analysed. Results: Both countries exhibited differences in waiting times for coronary angiography (CA) and percutaneous transluminal coronary angioplasty/coronary artery bypass graft (PTCA)/(CABG). Low SES patients waited longer than high SES patients within each country. The disparity in CHD care between low and high SES patients within each country appeared to be similar. Low SES patients in both countries experienced reduced rates of CA and CABG/PTCA. Implications/Conclusion: Despite differences between the US third‐party payer system and the UK socialised, primary care‐oriented system, each country faces the same SES inequalities regarding waiting time for and access to the ICPs. Understanding the reasons behind these inequalities is vital to address them.     

Safety and Imaging Quality of MRI in Pediatric and Adult Congenital Heart Disease Patients with Pacemakers

Background: Magnetic resonance imaging (MRI) is a standard of care in evaluating many disease processes. Given concerns about device damage or movement, programming changes, lead heating, inappropriate pacing, and image artifact, MRI is contraindicated in pacemaker patients. Despite this, studies have demonstrated safety and efficacy of MRI in adults with acquired heart disease and endocardial pacing leads. We sought to evaluate MRI use in congenital heart disease (CHD) patients with predominantly epicardial pacing leads.
Methods: From July 2007 to October 2008, MRI (1.5 Tesla) was performed in 11 patients without alternative imaging modality who were not pacemaker dependent or possessing abandoned leads. Pacing was disabled during MRI. An electrophysiologist monitored electrocardiogram and hemodynamic parameters throughout each study. Device and lead function were evaluated before and after MRI, and at subsequent clinic visits.
Results: Eleven MRIs (four cardiac, seven noncardiac) were performed in eight patients. Mean patient age was 16.5 ± 9.2 years (range 1.7–24.5) with five patients under the age of 16 years. Diagnoses included structural CHD in six patients and long QT syndrome and congenital heart block in one each. There were three dual- and five single- (three atrial, two ventricular) chamber devices, two endocardial, and nine epicardial leads. No inappropriate pacing or significant change in generator or lead parameters was noted. All MRI studies were of diagnostic quality.
Conclusion: Diagnostic quality MRI can be performed safely in nonpacemaker-dependent CHD patients with predominantly epicardial leads. Further studies will define safe practice measures in this population, as well as in CHD patients with pacemaker dependency.     

Echocardiographic Follow-up of Grown-ups with Congenital Heart Disease: Update 2013

There is a burgeoning population of adults with congenital heart disease (CHD) due to successful medical and surgical intervention in childhood. This group of patients requires careful echocardiographic evaluation and follow-up of the residua and sequellae of their cardiac anomalies. This review addresses the guidelines for evaluation and management of the grown-up with CHD, the contributions of 3D echo in their assessment, and the current status and new developments in noninvasive determination of ventricular volumes and function. The contribution of stress and contrast echocardiography for the adult with CHD is considered. The application of transcatheter interventions for this population is included with particular regard for the role of echocardiography pre- and postprocedure. Particular problems presented by pregnancy for the adult CHD patient are also reviewed.     

改善我国先天性心脏病诊疗体系的思考:外科区域化治疗

手术是先天性心脏病的主要治疗手段之一。目前，我国顶尖先天性心脏病外科中心的诊治水平已接近国际一流标准，大量患者得到了规范救治。然而，我国先天性心脏病患者的整体预后仍有待提高。本文从外科医疗资源分布和医疗可及性等角度，分析我国与西方发达国家的差距，并针对外科区域化治疗的理念，提出改善我国先天性心脏病诊疗体系的可行路径。