Interventional fellowship in structural and congenital heart disease for adults

Training for structural and adult congenital heart disease interventions remains undeveloped. With the advent of recent percutaneous interventions for the treatment of structural and valvular heart disease, such as transcatheter aortic and pulmonary valve implantation, mitral valve repair, and the expansion of shunt closure procedures, there is a clear need to define the training requirements for this category of procedures. The training needs to be aligned with the goals and priorities of a basic or advanced level and be categorized into acquired and congenital. This document will define the training needs and knowledge base for the developing field of structural heart disease intervention. © 2010 Wiley‐Liss, Inc.     

Transcatheter Aortic Valve Replacement in Patients With Multivalvular Heart Disease

As transcatheter aortic valve replacement becomes a more dominant treatment option across all risk profiles, the frequency of encountering patients with multivalvular disease will increase. Furthermore, percutaneous interventions to treat other valvular lesions are also evolving. Understanding the clinical implications and treatment options for a second valvular lesion is becoming increasingly important to guide heart team decisions, and this paper aims to review the evidence around these situations. Diagnosis of multivalvular disease can be challenging because of changes in physiology. There are little randomized data to guide therapy in multivalvular disease. Multidisciplinary heart team decisions can be invaluable in integrating the plethora of clinical, hemodynamic, and imaging data on which an optimal management strategy can be planned. Prospective studies to assess the role of structural valve interventions in the transcatheter aortic valve replacement era would greatly help improve outcomes for structural heart patients.     

Evolving Trends in Interventional Cardiology: Endovascular Options for Congenital Disease in Adults

As increasing numbers of patients with congenital heart disease enter adulthood, there is a growing need for minimally invasive percutaneous interventions, primarily to minimize the number of repeated surgeries required by these patients. The use of percutaneous devices is commonplace for the treatment of simple lesions, such as atrial septal defect, patent foramen ovale, patent duct arteriosus, and abnormal vascular connections. There is also substantial experience with device closure of membranous and muscular ventricular septal defects, as well as more complex shunts such as baffle leaks after atrial switch repair and ventricular pseudoaneurysms. An increasing use of covered stents has improved the safety of aortic coarctation, conduit, and branch pulmonary stenosis interventions. Percutaneous pulmonary valve implantation now has an established role in the setting of dysfunctional right ventricle-pulmonary artery conduits or failing bioprosthetic pulmonary valves. Many patients remain unsuitable for percutaneous pulmonary valve implantation because of large diameter “native” outflow tracts, however, various techniques have emerged and multiple devices are in development to provide solutions for these unique anatomic challenges. Hybrid approaches involving use of surgical and transcatheter techniques are increasingly common, serving to optimize efficacy and safety of certain procedures; they depend on a collaborative and collegial relationship between cardiac surgeons and interventionalists that is primarily patient-centred.     

Same day discharge after structural heart disease interventions in the era of the coronavirus-19 pandemic and beyond

With recent advancements in imaging modalities and techniques and increased recognition of the long-term impact of several structural heart disease interventions, the number of procedures has significantly increased. With the increase in procedures, also comes an increase in cost. In view of this, efficient and cost-effective methods to facilitate and manage structural heart disease interventions are a necessity. Same-day discharge (SDD) after invasive cardiac procedures improves resource utilization and patient satisfaction. SDD in appropriately selected patients has become the standard of care for some invasive cardiac procedures such as percutaneous coronary interventions. This is not the case for the majority of structural heart procedures. With the coronavirus disease 2019 pandemic, safely reducing the duration of time spent within the hospital to prevent unnecessary exposure to pathogens has become a priority. In light of this, it is prudent to assess the feasibility of SDD in several structural heart procedures. In this review we highlight the feasibility of SDD in a carefully selected population, by reviewing and summarizing studies on SDD among patients undergoing left atrial appendage occlusion, patent foramen ovale/atrial septal defect closure, Mitra-clip, and trans-catheter aortic valve replacement procedures.     

Transfer of cardiac catheterisation from a paediatric to an adult cardiological centre: results at 3 years

The advances of surgical and interventional treatment of congenital heart diseases have allowed a large number of patients with congenital heart disease to reach adult age. This population involves almost 0.3/1000 of total population in West Europe and North America and can be estimated around 200000 patients in France. Patients with operated Tetralogy of Fallot, benign forms of pulmonary atresia with ventricular septal defect, simple or complex transposition of the great arteries usually survive beyond childhood. These patients can need repeated interventions to treat lesions of native or reconstructed pulmonary arteries and/or aortic arch, to occlude residual shunts, to treat pulmonary incompetence. More complex heart diseases such as single ventricle, rarely allow survival until the adult age. The majority of these patients undergo heart transplant, often made difficult by multiple cardiac surgeries, anomalies of pulmonary arteries, chronic cyanosis, aorto-pulmonary shunts. Patients with relatively simple or complex congenital heart diseases need to be followed-up in specialized units, like those created more than twenty years ago in the United States, Canada, and United Kingdom. Interventional cardiac catheterisation play a major role in the management of this population. The results of 3 years of activity in a new centre treating GUCH patients are illustrated.     

Heart transplantation in biventricular congenital heart disease: indications, techniques, and outcomes

Heart transplantation is an accepted therapeutic modality for end-stage congenital heart disease for both biventricular and univentricular anomalies. Many transplant centers have pushed the limits of transplantation to include patients with high pulmonary vascular resistance, high panel reactive antibodies, positive cross-matches, and ABO-incompatibility. Excellent results have been possible, particularly with the development of improved diagnostic and therapeutic algorithms to prevent and treat rejection, infection, and post-transplant lymphoproliferative disease. Late graft failure and chronic rejection remain vexing problems. The vast majority of patients with biventricular congenital heart disease have undergone prior cardiac surgical procedures. Indications for transplantation in this subgroup are primarily progressive refractory heart failure following prior cardiac surgical reconstructive procedures. Contraindications to transplantation mimic those for other forms of end-stage heart disease. A determination of pulmonary vascular resistance is important in listing patients with biventricular congenital heart disease for heart transplantation. Modifications in the implant technique are necessary and vary depending on underlying recipient anatomy. Risk factors for perioperative outcomes in patients with biventricular congenital heart disease include the need for reoperation, the degree of anatomic reconstruction necessary during the implant procedure, and the degree of antibody sensitization, in addition to a number of other recipient and donor factors. Postoperative outcomes and survival are very good but remain inferior to those with cardiomyopathy in most series. In conclusion, patients with end-stage biventricular congenital heart disease represent a complex group of patients for heart transplantation, and require careful evaluation and management to ensure optimal outcomes.     

Cardiac Surgery in HIV Patients: State of the Art

The clinical status of HIV infection has changed dramatically with the introduction of combined antiretroviral therapy. Patients with HIV are now living long enough to be susceptible to chronic illnesses, such as coronary disease and nonischemic cardiomyopathy, which can be consequences of the combined antiretroviral therapy treatment itself. Cardiovascular diseases are a major source of morbidity and mortality in HIV-positive patients. Increasingly, such patients might be candidates for the full range of cardiac surgical interventions, including coronary bypass, valve surgery, and heart transplantation. There has been a shift from offering palliative procedures such as pericardial window and balloon valvuloplasty, to more conventional and durable surgical therapies in HIV-positive patients. We herein provide an overview of the contemporary outcomes of cardiac surgery in this complex and unique patient population. We review some of the ethical issues around the selection and surgical care of HIV-positive patients. We also discuss strategies to best protect the surgical treatment team from the risks of HIV transmission. Finally, we highlight the need for involvement of dedicated infectious disease professionals in a multidisciplinary heart team approach, aiming at the comprehensive care of these unique and complex patients.     

Echocardiographic assessment of pulmonary arterial pressure in children with complete right bundle branch block

The time interval between tricuspid valve closure and pulmonary valve opening, termed the isovolumic contraction time of the right ventricle, was evaluated echographically in 38 normal children and within 24 hours of cardiac catheterization in 53 children with congenital heart disease and normal conduction as assessed with the electrocardiogram. In the 53 patients with congenital heart disease, isovolumic contraction time was strongly influenced by right ventricular afterload, as defined by pulmonary arterial end-diastolic pressure (r = 0.87). It was possible to utilize isovolumic contraction time to separate patients with normal or elevated values for pulmonary arterial end-diastolic pressure. Similar correlations were demonstrated between isovolumic contraction time and mean pulmonary arterial pressure and calculated pulmonary vascular resistance. Evaluation of 15 children with complete right bundle branch block revealed values for isovolumic contraction time that did not significantly differ from those of patients with similar pulmonary arterial end-diastolic pressure but no conduction abnormalities. These findings indicate that serial echographic evaluation of the interval from tricuspid valve closure to pulmonary valve opening can give an accurate reproducible assessment of right ventricular afterload in many children with congenital heart disease and complete right bundle branch block.     

Percutaneous valve replacement: Fact or fiction?

Percutaneous catheter-based interventions are an emerging area in the treatment of valvular heart disease. Despite many limitations related to device technology, valve durability and stability, and anatomical obstacles, the early results of percutaneous catheter-based valve repair and replacement are promising. The procedures are currently aimed at high-risk patients who cannot undergo conventional surgical management. The first percutaneous heart valve replacement occurred in 2002 in the pulmonary position. Subsequently, the early experience in the literature has reported over 50 human cases. The anatomical complexities of the aortic root have made success more elusive for percutaneous aortic valve replacement. Only a few cases have been reported, with variable success described. Percutaneous mitral valve replacement has not been reported to date; however, clinical trials are underway evaluating catheter-based techniques of mitral valve repair. Present enthusiasm should be tempered because scarcely more than 100 patients worldwide have undergone percutaneous valve interventions. Nonetheless, it is inevitable that as technology develops to overcome the present limitations and develop safe and effective techniques, percutaneous heart valve repair and replacement will undoubtedly increase in frequency. Scrupulously designed clinical trials must be performed to definitively determine the short- and long-term results of these procedures as compared with the gold standard of open surgical repair, as well as to define the appropriate patient population that will benefit the most.     

Historical Prospective: Therapeutic Catheterization Procedures in Congenital Heart Disease

The development of pediatric and congenital therapeutic ("interventional") catheterization procedures is traced from the earliest attempts to the current procedures. Therapeutic cardiac catheterization procedures for pediatric and congenital heart disease have grown from a dream to a reality during only the last three decades. Although some of the technology has come from the adult interventions and has paralleled the development of adult catheter interventions, most of the therapeutic procedures for congenital lesions were developed by a dedicated and persistent group of pediatric cardiologists and originated in the pediatric cardiac catheterization laboratories. Many of the common congenital lesions, which three decades ago required cardiac surgery for treatment, now, as standard accepted therapy, are managed in the catheterization laboratory. The most successful advances have been in the opening of stenotic valves, opening and fixing stenotic vessels with intravascular stents, and, at the opposite extreme, the closure of PDAs, secundum ASDs, some VSDs, and numerous miscellaneous abnormal communications. Although many of these therapies are now the standard of care, all of the catheter therapies in pediatric and congenital lesions are in the infancy of their development and will undergo many improvements and refinements in the future.     

Guidelines for the management of common congenital heart diseases in India: A consensus statement on indications and timing of intervention

IntroductionA number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition.ProcessGuidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on the 10th and 11th of August, 2018 at the All India Institute of Medical Sciences.ObjectivesThe aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases and (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases.RecommendationsEvidence-based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts, obstructive lesions, and cyanotic congenital heart diseases. In addition, protocols for follow-up of postsurgical patients are also described.     

Myocardial performance after anatomic or physiologic corrective surgery

Data on ventricular performance following “corrective” surgery for congenital heart disease at the present time are limited in number and scope. In isolated right and left heart pressure and volume overload lesions, information is available indicating suboptimal cardiac performance in many patients following successful corrective surgery. Patients with complex cyanotic lesions such as tetralogy of Fallot or transposition of the great arteries also have evidence for impaired myocardial function following “corrective” procedures. Histologic data show focal and diffuse myocardial fibrosis in most patients with long-standing hypertrophy. This fibrosis is probably a result of a discrepancy between myocardial oxygen demands and supply in patients with hypertrophy, and it may be the anatomical correlate of abnormal myocardial performance. Despite laboratory evidence for mild to moderate degrees of mechanical cardiac dysfunction, most patients are asymptomatic, lead useful lives, and are able to tolerate at least moderate levels of exercise without difficulty. Many questions which are of vital importance to the patient, the cardiologist, the primary physician, and the surgeon remain unanswered. What is the time reference over which irreversible changes in myocardial structure and function takes place for each congenital heart lesion? For what lesions will infant surgery prevent important residual cardiac-dysfunction? What are the long-term effects of outflow tract patches, ventriculotomy, pulmonary insufficiency, and residual pulmonary or aortic stenosis on cardiovascular function? Does hypothermia and total circulatory arrest ever permanently affect cardiac function? What are the time limits for anoxic arrest to prevent residual cardiac dysfunction in severe pressure overload lesions? Are mild degrees of abnormal pump function (ejection fraction 0.45–0.55) clinically important as regards morbidity, longevity, and “quality” of life for patients following surgery for congenital heart disease? These are only a few of the general questions for which very little data are available to attempt to answer. Obviously, each specific lesion and procedure will require study in an attempt to provide specific answers regarding optimal therapy. It is apparent that close liaisons must be formed and maintained between physicians who care primarily for children with heart disease and adult cardiologists in order to provide adequate longitudinal studies to evaluate current therapy for all patients with congenital heart disease.     

Percutaneous heart valves demonstrating long-term durability: A case series of Melody valves in the pulmonary position lasting up to 19 years

It is uncertain how long catheter delivered percutaneous heart valves may last. In congenital cardiology, stenosis and regurgitation of right ventricular to pulmonary artery conduits and valves is common, leading to repeated operations for young patients with concomitant mortality and morbidity. It has also been unclear whether percutaneous pulmonary valves last as long as surgical pulmonary valves. When the current generation of the percutaneous pulmonary valve was first implanted in the United Kingdom from 2003, randomized trials were initially not performed, decided on a case-by-case basis in congenital cardiology, nor long-term registries kept. We describe three cases where such percutaneous heart valves have lasted up to 19 years. All valves were working without significant stenosis and minor degrees of regurgitation on long-term echocardiographic follow-up, patients being asymptomatic. This demonstrates that percutaneous pulmonary valves can achieve long-term durability and may prevent the need for otherwise high-risk surgery in congenital cardiac patients.     

Heart Failure in Adult Congenital Heart Disease: From Advanced Therapies to End-of-Life Care

There is mounting recognition that some of the most urgent problems of adult congenital heart disease (ACHD) are the prevention, diagnosis, and management of heart failure (HF). Recent expert consensus and position statements not only emphasize a specific and pressing need to tackle HF in ACHD (ACHD-HF) but also highlight the difficulty of doing so given a current sparsity of data. Some of the challenges will be addressed by this review. The authors are from 3 different centres; each centre has an established subspeciality ACHD-HF clinic and is able to provide heart transplant, multiorgan transplant, and mechanical support for patients with ACHD. Appropriate care of this complex population requires multidisciplinary ACHD-HF teams evaluate all possible treatment options. The risks and benefits of nontransplant ACHD surgery, percutaneous structural and electrophysiological intervention, and ongoing conservative management must be considered alongside those of transplant strategies. In our approach, advanced care planning and palliative care coexist with the consideration of advanced therapies. An ethos of shared decision making, guided by the patient’s values and preferences, strengthens clinical care, but requires investment of time as well as skilled communication. In this review, we aim to offer practical real-world advice for managing these patients, supported by scientific data where it exists.     

Tricuspid and pulmonary valve disease evaluation and management

The clinical detection and quantification of tricuspid valve disease, although important, is not entirely accurate. Diagnostic evaluation is based on echocardiography, and color flow Doppler is useful for quantifying tricuspid regurgitation. Echocardiography provides information on heart chamber dimensions, right ventricular function, and the degree of pulmonary hypertension. In addition, tricuspid stenosis can be accurately assessed using mean and end-diastolic pressure gradient measurements. The treatment options for tricuspid stenosis include balloon valvuloplasty and surgical valve repair. Functional tricuspid regurgitation associated with left heart disease may require surgical attention during an operation to treat the left heart disease. Severe tricuspid regurgitation usually requires surgery to be performed in association with mitral valve surgery. Mild-to-moderate tricuspid regurgitation requires surgery when annular dilatation or severe pulmonary hypertension is present. The surgical options include tricuspid valve repair, with or without an annuloplasty ring. In patients with a primary anatomic deformity of the tricuspid valve, replacement of the valve with a bioprosthesis or mechanical valve may be considered. Intermediate and long-term results favor annuloplasty valve repair over valve replacement. Pulmonary valve disease is predominantly congenital, and generally takes the form of pulmonary stenosis. Pulmonary regurgitation often results from surgical or balloon valvuloplasty and is associated with deleterious long-term sequelae. The recent development of percutaneous valve replacement was a major advance.     

Insights on Atrial Fibrillation in Congenital Heart Disease

Patients with congenital heart disease (CHD) have been surviving late into adulthood, with atrial arrhythmias being the most common long-term complication. In recent reports, atrial fibrillation (AF) tended to be the most common form of arrhythmias among groups of patients with adult CHD (ACHD) older than 50 years of age. When compared with their adult counterparts without CHD, AF in patients with ACHD has been characterized by a higher incidence and prevalence, younger age of onset, and a greater risk of progression to persistent AF. Risk factors for the development of AF are not well known but include older age, left atrial dilation, systemic hypertension, and multiple cardiac surgeries. Data on management options such as optimal antiarrhythmic drug therapy, indications for anticoagulation, and efficacy and safety of catheter ablation are limited. There is a crucial need for further research exploring management, prevention, and monitoring strategies for the growing ACHD patient population with AF. This report will provide a contemporary review of the epidemiology, pathophysiology, and management options for AF in this complex patient population.     

The adult patient with eisenmenger syndrome: a medical update after dana point part I: epidemiology, clinical aspects and diagnostic options

Eisenmenger syndrome is the most severe form of pulmonary arterial hypertension and arises on the basis of congenital heart disease with a systemic-to-pulmonary shunt. Due to the chronic slow progressive hypoxemia with central cyanosis, adult patients with the Eisenmenger syndrome suffer from a complex and multisystemic disorder including coagulation disorders (bleeding complications and paradoxical embolisms), renal dysfunction, hypertrophic osteoarthropathy, heart failure, reduced quality of life and premature death.For a long time, therapy has been limited to symptomatic options or lung or combined heart-lung transplantation. As new selective pulmonary vasodilators have become available and proven to be beneficial in various forms of pulmonary arterial hypertension, this targeted medical treatment has been expected to show promising effects with a delay of deterioration also in Eisenmenger patients. Unfortunately, data in Eisenmenger patients suffer from small patient numbers and a lack of randomized controlled studies.To optimize the quality of life and the outcome, referral of Eisenmenger patients to spezialized centers is required. In such centers, specific interdisciplinary management strategies of physicians specialized on congenital heart diseases and PAH should be warranted. This medical update emphasizes the current diagnostic and therapeutic options for Eisenmenger patients with particularly focussing on epidemiology, clinical aspects and specific diagnostic options.     

Update in fetal cardiac intervention

Opinion statement The severity of fetal heart disease progresses during gestation and may lead to significant in utero or postnatal morbidity and mortality. Fetal echocardiography allows us to detect heart disease early enough in pregnancy to perform fetal cardiac intervention that may change the natural history of some diseases. The principal aims are the prevention of hydrops due to congenital heart disease, recruitment of hypoplastic ventricles, remodeling of the fetal pulmonary vascular bed, or creation of a two-ventricular circulation after birth without risk to the mother. The initial fetal interventions for valvuloplasty and atrial septostomy showed a high mortality and did not achieve significant change in the final outcome of patients. Nevertheless, some technique modifications have improved the outcome of these patients, making fetal cardiac intervention a promising treatment for congenital heart disease. Conversely, different assessment tools for selection of the correct patient have been studied in some series to improve the success of these procedures and thus the survival odds. We believe that percutaneous procedures with local anesthesia are preferable.     

Percutaneous valve replacement: a paradigm shift

PURPOSE OF REVIEW: Valvular heart disease is often associated with significant morbidity and mortality. Surgical treatment of stenotic or regurgitant valvular lesions can alter the natural history of the disease process, yielding excellent short and long-term results, meaning that this has been accepted as the gold standard for therapy of valvular disease. Surgical procedures can be associated with a significant rate of perioperative complications, however. These procedures are particularly associated with unacceptably high short and long-term morbidity and mortality in the elderly - a population group that has grown steadily over the years. Elderly patients often have significant comorbidities that preclude a safe and durable surgical correction of the valvular lesions in up to 31% of these high-risk cases. This review provides an overview of recent developments in percutaneous valve therapeutics. RECENT FINDINGS: Over the last decade, numerous technical developments in valvular interventions have focused on percutaneous valve replacement. There are percutaneous valve replacements on the horizon that promise to offer a novel approach to correct valvular lesions, especially in this high-risk surgical population. SUMMARY: Several devices are presently being evaluated in feasibility trials, and many new ones are being developed. Results with semilunar valves have generally been better than the atrioventricular valves. Long-term results of these treatment modalities are still unknown.     

Technological Advances in Arrhythmia Management Applied to Adults With Congenital Heart Disease

Arrhythmias are a leading cause of morbidity in adults with congenital heart disease. Numerous challenges to managing arrhythmias include vascular access issues, intracardiac shunts, unconventional locations of the conduction system, and anatomical complexities. Technological advances are improving our ability to diagnose and treat arrhythmias. Implantable loop recorders and various technologies developed for consumers to record electrocardiographic tracings using smartphone applications, watches, and dedicated portable devices are expanding our armamentarium of diagnostic tools. Remote magnetic-guided catheter ablation is enhancing access to otherwise difficult to reach arrhythmia substrates. Cryoablation allows perinodal arrhythmias to be safely treated in patients with displaced or difficult to predict locations of the atrioventricular conduction system. Interventions that minimize radiation exposure to near 0 levels are gaining interest and pulmonary vein isolation procedures to treat atrial fibrillation are increasingly performed. Cardiac implantable electronic devices compatible with magnetic resonance imaging have become the norm. Subcutaneous defibrillators and leadless pacemakers are providing effective solutions to patients in whom transvenous leads are contraindicated or not desired. His-bundle pacing is emerging as a viable option to provide more physiological pacing. Progressive advances in multicomponent communicating leadless devices carry the promise of providing leadless dual chamber pacing and cardiac resynchronization therapy in the near future. The safety of transvenous lead extraction procedures is likely to improve with advances such as the low-pressure balloon to manage superior vena cava lacerations. Awareness of these developments and referral to sites with dedicated expertise could contribute to further improving outcomes in adults with congenital heart disease.