Autopsy in adults with congenital heart disease (ACHD).

The adult congenital heart diseases (ACHD) population is exceeding the pediatric congenital heart diseases (CHD) population and is progressively expanding each year, representing more than 90% of patients with CHD. Of these, about 75% have undergone surgical and/or percutaneous intervention for palliation or correction. Autopsy can be a very challenging procedure in ACHD patients. The approach and protocol to be used may vary depending on whether the pathologists are facing native disease without surgical or percutaneous interventions, but with various degrees of cardiac remodeling, or previously palliated or corrected CHD. Moreover, interventions for the same condition have evolved over the last decades, as has perioperative myocardial preservations and postoperative care, with different long-term sequelae depending on the era in which patients were operated on. Careful clinicopathological correlation is, thus, required to assist the pathologist in performing the autopsy and reaching a diagnosis regarding the cause of death. Due to the heterogeneity of the structural abnormalities, and the wide variety of surgical and interventional procedures, there are no standard methods for dissecting the heart at autopsy. In this paper, we describe the most common types of CHDs that a pathologist could encounter at autopsy, including the various types of surgical and percutaneous procedures and major pathological manifestations. We also propose a practical systematic approach to the autopsy of ACHD patients

Cardiac Arrhythmias In Congenital Heart Diseases

Arrhythmias figure prominently among the complications encountered in the varied and diverse population of patients with congenital heart disease, and are the leading cause of morbidity and mortality. The incidence generally increases as the patient ages, with multifactorial predisposing features that may include congenitally malformed or displaced conduction systems, altered hemodynamics, mechanical or hypoxic stress, and residual or postoperative sequelae. The safe and effective management of arrhythmias in congenital heart disease requires a thorough appreciation for conduction system variants, arrhythmia mechanisms, underlying anatomy, and associated physiology. We, therefore, begin this review by presenting the scope of the problem, outlining therapeutic options, and summarizing congenital heart disease-related conduction system anomalies associated with disorders of the sinus node and AV conduction system. Arrhythmias encountered in common forms of congenital heart disease are subsequently discussed. In so doing, we touch upon issues related to risk stratification for sudden death, implantable cardiac devices, catheter ablation, and adjuvant surgical therapy

Track 5: Cardiology and the imaging revolution - Part I

Cardiology and the imaging revolution

ESC core curriculum for the general cardiologist (2013)

[No abstract available

Hypoplastic Left Heart Syndrome Current Considerations and Expectations

In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current expectations are that 70% of newborns born today with HLHS may reach adulthood. Although the 3-stage treatment approach to HLHS is now well founded, there is significant variation among centers. In this white paper, we present the current state of the art in our understanding and treatment of HLHS during the stages of care: 1) pre-Stage I: fetal and neonatal assessment and management; 2) Stage I: perioperative care, interstage monitoring, and management strategies; 3) Stage II: surgeries; 4) Stage III: Fontan surgery; and 5) long-term follow-up. Issues surrounding the genetics of HLHS, developmental outcomes, and quality of life are addressed in addition to the many other considerations for caring for this group of complex patients

Defining long term morbidity and mortality in adult survivors of congenital heart disease

Over the past thirty years there has been a dramatic improvement in the survival of children with congenital heart disease, and the vast majority can now expect to reach adulthood. However it is rare that a lesion can truly be described as "cured" even with everything that modern surgery can offer. Often lesions are palliated instead, and this subjects patients to a variety of complications in later life. These include residual haemodynamic lesions necessitating further surgical or percutaneous intervention, cardiac arrhythmia, and heart failure. All of these are potentially lethal, and result in a steady decline in survival, functional status and quality of life in later years. In order to care for these patients a new medical subspecialty has emerged - that of Adults with Congenital Heart Disease (ACHD). Although the provision of highly specialist management for this population has allowed many significant clinical improvements to be made, healthcare practitioners working in this field face significant challenges. The long term prognosis for these patients is uncertain, making it difficult to know how to counsel young people who wish for employment, a family, and a future. Whilst powerful tools for the non-invasive assessment of cardiovascular structure and function exist, their role in clinical decision making is often poorly defined, and the nature and timing of repeat intervention are frequently based on expert opinion rather than robust evidence from large well conducted clinical trials, and consequently the true benefit of such procedures is unclear. Furthermore, to my knowledge, there has never been a serious attempt to characterise outcomes in ACHD patients in Scotland. Because of the sheer heterogeneity of congenital cardiac conditions I decided to focus on outcomes in two distinct, relatively easily defined cohorts of adult patients with repaired congenital heart disease - patients with a systemic right ventricle, and patients with repaired Tetralogy of Fallot (TOF), both characterised by the presence of an abnormal right ventricle. In chapter 3 I outline what is currently known about mortality and morbidity in each condition, based on what has been reported globally by other congenital cardiac centres. I then report the survival and adverse outcomes experienced by patients followed up by our centre: for patients with a systemic right ventricle in chapter 4, and for patients with repaired TOF in chapter 5. I assess these outcomes both in relation to the published literature internationally, and also to the general Scottish population. Finally, in chapter 6 I assess the clinical utility of parameters derived from cardiac MRI and cardiopulmonary exercise testing to establish whether this can improve clinical risk stratification for patients with repaired TOF