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The American Heart Association and American College of Cardiology published practice guidelines for the management of adult congenital heart disease in 2018 and the European Society of Cardiology published analogous guidelines in 2020. Although there are broad areas of consensus between the 2 documents, there are important differences that impact patient management. This review discusses key areas of agreement and disagreement between the 2 guidelines, with discussion of possible reasons for disagreement and potential implications.

Palliative care has potential to improve quality of life and goal-concordant care for patients with adult congenital heart disease (ACHD). However, it is rarely employed prior to critical illness because the best methods for implementation are not well-defined. We qualitatively evaluated ACHD patients' understanding of and opinions regarding palliative care and advance care planning (ACP) to better define the needs of this population. We conducted a thematic analysis of 25 semistructured interviews with patients with ACHD in which we assessed participants' perspectives on the need for, and barriers and facilitators to, the use of palliative care and ACP. In a group of participants with ACHD (mean age 38, 48% male) classified as simple (24%), moderate (32%), or complex (44%), we identified 4 major themes: (1) using knowledge to combat future uncertainties; (2) unfamiliarity with and limited exposure to palliative care and ACP; (3) facilitators and barriers to engaging in palliative care and ACP; and (4) importance of timing and presentation of ACP discussions. In conclusion, participants expressed a desire for knowledge about ACHD progression and treatment. They supported routine incorporation of palliative care and ACP and identified related facilitators and barriers to doing so. Importantly, timing and format of these discussions must be individualized using shared decision-making between clinicians, patients, and their families.

As the population of adult congenital heart disease patients ages and grows, so too does the burden of heart failure in this population. Despite the advances in medical and surgical therapies over the last decades, heart failure in adult congenital heart disease remains a formidable complication with high morbidity and mortality. This review focuses on the challenges in determining the true burden and management of heart failure in adult congenital heart disease. There is a particular focus on the need for developing a common language for classifying and reporting heart failure in adult congenital heart disease, the clinical presentation and prognostication of heart failure in adult congenital heart disease, the application of hemodynamic evaluation, and advanced heart failure treatment. A common case study of heart failure in adult congenital heart disease is utilized to illustrate these key concepts.

Fear of acquiring severe acute respiratory syndrome coronavirus-2 infection is a major contributor to underutilization of the health care system during the current pandemic. In this report, we describe 4 cases of unexpected deaths that occurred within a short time period in patients with adult congenital heart disease without warning symptoms. (Level of Difficulty: Intermediate.)

The COVID-19 pandemic poses risk for worsened quality of life in patients with adult congenital heart disease. In a qualitative thematic analysis of semi-structured interviews conducted July 2020 to August 2021, we examined the pandemic’s impact on participants’ (N = 25) experiences with self-perception and coping. All had moderate or complex disease; median age 32 years. The pandemic altered some participants’ self-perception, including increased vulnerability beyond heart-attributed risk. Restrictions frequently prevented participants from using their usual coping strategies, forcing use of alternative methods. For an already at-risk population, these findings suggest the need for increased mental health awareness, assessment, and support.

Background:Advance care planning and palliative care are gaining recognition as critical care components for adults with CHD, yet these often do not occur. Study objectives were to evaluate ACHD providers’ 1) comfort managing patients’ physical symptoms and psychosocial needs and 2) perspectives on the decision/timing of advance care planning initiation and palliative care referral.Methods:Cross-sectional study of ACHD providers. Six hypothetical patients were described in case format, followed by questions regarding provider comfort managing symptoms, initiating advance care planning, and palliative care referral.Results:Fifty providers (72% physicians) completed surveys. Participants reported low levels of personal palliative care knowledge, without variation by gender, years in practice, or prior palliative care training. Providers appeared more comfortable managing physical symptoms and discussing prognosis than addressing psychosocial needs. Providers recognised advance directives as important, although the percentage who would initiate advance care planning ranged from 18 to 67% and referral to palliative care from 14 to 32%. Barriers and facilitators to discussing advance care planning with patients were identified. Over 20% indicated that advance care planning and end-of-life discussions are best initiated with the development of at least one life-threatening complication/hospitalisation.Conclusions:Providers noted high value in advance directives yet were themselves less likely to initiate advance care planning or refer to palliative care. This raises the critical questions of when, how, and by whom discussion of these important matters should be initiated and how best to support ACHD providers in these endeavours.

[Extract] The 2018 ACHD guideline is a full revision of the “2008 ACC/AHA Guidelines for the Management of Adults with Congenital Heart Disease” (S1.4-1), which was the first U.S. guideline to be published on the topic. This revision uses the 2008 ACHD guideline as a framework and incorporates new data and growing ACHD expertise to develop recommendations. Congenital heart disease (CHD) encompasses a range of structural cardiac abnormalities present before birth attributable to abnormal fetal cardiac development but does not include inherited disorders that may have cardiac manifestations such as Marfan syndrome or hypertrophic cardiomyopathy. Also not included are anatomic variants such as patent foramen ovale. Valvular heart disease (VHD) may be congenital, so management overlaps with the “2014 AHA/ACC Guidelines for the Management of Patients With Valvular Heart Disease” (S1.4-2), particularly for bicuspid aortic valve (BAV) disease. Where overlap exists, this document focuses on the diagnosis and treatment of congenital valve disease when it differs from acquired valve disease, whether because of anatomic differences, presence of concomitant lesions, or differences to consider given the relatively young age of patients with ACHD. This guideline is not intended to apply to children (

The American Heart Association and American College of Cardiology published practice guidelines for the management of adult congenital heart disease in 2018 and the European Society of Cardiology published analogous guidelines in 2020. Although there are broad areas of consensus between the 2 documents, there are important differences that impact patient management. This review discusses key areas of agreement and disagreement between the 2 guidelines, with discussion of possible reasons for disagreement and potential implications.

STRUCTURED Abstract Background As clinical care and research in adult congenital heart disease (ACHD) increasingly include a focus on quality of life, hearing and reflecting on our patients’ experiences must be a priority. The objective of this study was to describe how living with this chronic illness has affected the adult lives of patients living with ACHD. Methods We conducted a qualitative study of 25 participants in which we asked about the impact of ACHD on their quality of life. We used thematic analysis to analyze the interview data and identified themes reflecting patients’ reported experiences. Results Study participants were a mean age of 38 years (range 21–63 years) and reported good functional status. ACHD was characterized as simple (24%), moderate (32%), or complex (44%). Three major themes arose: 1) impact of ACHD on identity, including perception of “normal,” self-esteem, and motivation; 2) sources of emotional distress; and 3) influence on adult decision-making, including careers and relationships. Conclusion Living with ACHD impacts important aspects related to self-perceived quality of life. Addressing these concerns is important to providing comprehensive, patient-centered ACHD care.

Background Patients with adult congenital heart disease (ACHD) report that advance care planning (ACP) is important, and that they want information about prognosis. However, recognizing importance and being willing to participate are different constructs, and how and when to begin ACP and palliative care discussions remains ill-defined. Methods We conducted a cross-sectional survey of 150 consecutive outpatients to assess willingness to participate in ACP, with whom, and important barriers and facilitators to these discussions. Results The majority of participants (69%) reported being willing to participate in ACP; 79% to have a meeting to discuss goals and care preferences; and 91% to speak to a clinician who specializes in palliative care. Being married and anticipating a shorter lifespan were associated with increased reported willingness to participate in ACP. The health care provider with whom most participants preferred to have these discussions was their ACHD clinician. Participants identified important barriers and facilitators to these discussions. Conclusion Patients with ACHD report being willing to participate in ACP and palliative care discussions. Patients prefer to have these discussions with their ACHD clinicians, thus ACHD clinicians need to be prepared to address these issues as part of routine care.

Background Heart failure is the leading cause of morbidity and mortality for adults with congenital heart disease (ACHD). Many patients are ineligible for transplantation, and those who are eligible often face long wait times with high wait-list morbidity. Durable mechanical circulatory support (MCS) may be an option for many patients. This systematic review evaluates the published literature on the use of durable MCS in teenagers and adults with congenital heart disease. Methods A comprehensive search of MEDLINE (PubMed), EMBASE, and the Cochrane Library was performed electronically in July 2015 and updated in March 2016, guided by the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. Results Individual case reports and several case series identified 66 patients with ACHD treated with durable MCS. More than half were INTERMACS 1 or 2 at the time of implantation. Patients with Fontan repairs were more frequently classified as INTERMACS 1 or 2 (89% compared to 59% or less among other groups). Cases published after 2010 showed a trend toward less severe INTERMACS status, and patients were less likely to have received transplants by the time of reporting (31% compared to 61% prior). Durable MCS was implanted as bridge-to-transplant in 77%. Patients with Fontan repair accounted for 14% of cases. Conclusion Reports of durable MCS utilization in patients with ACHD are becoming more frequent and devices are being implanted in more stable patients. Reports are mostly case reports or small case series so reporting bias is likely and prospective protocoled reporting is needed.

Background Anticoagulation for mechanical heart valves during pregnancy is essential to prevent thromboembolic events. Each regimen has drawbacks with regard to maternal or fetal risk. Objectives This meta-analysis sought to estimate and compare the risk of adverse maternal and fetal outcomes in pregnant women with mechanical heart valves who received different methods of anticoagulation. Methods Studies were identified using a Medline search including all publications up to June 5, 2016. Study inclusion required reporting of maternal death, thromboembolism, and valve failure, and/or fetal spontaneous abortion, death, and congenital defects in pregnant women treated with any of the following: 1) a vitamin K antagonist (VKA) throughout pregnancy; 2) low-molecular-weight heparin (LMWH) throughout pregnancy; 3) LMWH for the first trimester, followed by a VKA (LMWH and VKA); or 4) unfractionated heparin for the first trimester, followed by a VKA (UFH and VKA). Results A total of 800 pregnancies from 18 publications were included. Composite maternal risk was lowest with VKA (5%), compared with LMWH (16%; ratio of averaged risk [RAR]: 3.2; 95% confidence interval [CI]: 1.5 to 7.5), LMWH and VKA (16%; RAR: 3.1; 95% CI: 1.2 to 7.5), or UFH and VKA (16%; RAR: 3.1; 95% CI: 1.5 to 7.1). Composite fetal risk was lowest with LMWH (13%; RAR: 0.3; 95% CI: 0.1 to 0.8), compared with VKA (39%), LMWH and VKA (23%), or UFH and VKA (34%). No significant difference in fetal risk was observed between women taking ≤5 mg daily warfarin and those with an LMWH regimen (RAR: 0.9; 95% CI: 0.3 to 2.4). Conclusions VKA treatment was associated with the lowest risk of adverse maternal outcomes, whereas the use of LMWH throughout pregnancy was associated with the lowest risk of adverse fetal outcomes. Fetal risk was similar between women taking ≤5 mg warfarin daily and women treated with LMWH., Central Illustration

To identify predictors of morbidity and mortality in patients undergoing either transcatheter pulmonary valve replacement (TPVR) or surgical pulmonary valve replacement (SPVR) in an effort to quantify any early benefit of TPVR over SPVR.Using a risk-adjusted propensity score model, we compare early major morbidity and mortality between patients undergoing SPVR and TPVR at our institution between January 2006 and January 2014.145 patients in the SPVR cohort and 78 patients in the TPVR cohort were included. Primary pulmonary regurgitation was more common in the SPVR group (76.6% vs 23.1%, p0.001) and primary pulmonary stenosis was more common in the TPVR group (9.7% vs 44.9%, p0.001). In unadjusted analysis, major morbidity and mortality occurred in 11.7% of SPVR patients versus 3.8% of TPVR patients (p=0.04). However, following risk adjustment and inclusion of a propensity score, no significant difference was seen between the two modalities. A larger right ventricular end-diastolic dimension (RVEDVI) was the only preoperative variable associated with the primary end point (OR 1.013/10 mL/mFollowing risk adjustment, no significant differences were observed between SPVR and TPVR strategies. TPVR was associated with a shorter hospitalisation; however, total hospitalisation costs were similar between groups.

Review the recently updated guidelines for the management of patients with adult congenital heart disease (ACHD) with a focus on the changes between these guidelines and the prior guidelines. The 2018 guidelines for the management of patients with ACHD focused on utilizing the available data and limiting the number recommendations based only on expert opinion. These guidelines implement a new anatomic and physiological classification scheme to guide management of patients, which takes into account both the underlying anatomy as well as residual cardiac disease and symptoms. Given a lack of robust outcomes data for many types of CHD, the new guidelines provide fewer total recommendations than the prior version, emphasizing the data that is available and drawing attention to the need for additional data. The 2018 guidelines provide the field with a comprehensive update in the management of ACHD patients with an emphasis on the available data.

It has been 50 years since Francis Fontan pioneered the operation that today bears his name. Initially designed for patients with tricuspid atresia, this procedure is now offered for a vast array of congenital cardiac lesions when a circulation with 2 ventricles cannot be achieved. As a result of technical advances and improvements in patient selection and perioperative management, survival has steadily increased, and it is estimated that patients operated on today may hope for a 30-year survival of >80%. Up to 70 000 patients may be alive worldwide today with Fontan circulation, and this population is expected to double in the next 20 years. In the absence of a subpulmonary ventricle, Fontan circulation is characterized by chronically elevated systemic venous pressures and decreased cardiac output. The addition of this acquired abnormal circulation to innate abnormalities associated with single-ventricle congenital heart disease exposes these patients to a variety of complications. Circulatory failure, ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, protein-losing enteropathy, and plastic bronchitis are potential complications of the Fontan circulation. Abnormalities in body composition, bone structure, and growth have been detected. Liver fibrosis and renal dysfunction are common and may progress over time. Cognitive, neuropsychological, and behavioral deficits are highly prevalent. As a testimony to the success of the current strategy of care, the proportion of adults with Fontan circulation is increasing. Healthcare providers are ill-prepared to tackle these challenges, as well as specific needs such as contraception and pregnancy in female patients. The role of therapies such as cardiovascular drugs to prevent and treat complications, heart transplantation, and mechanical circulatory support remains undetermined. There is a clear need for consensus on how best to follow up patients with Fontan circulation and to treat their complications. This American Heart Association statement summarizes the current state of knowledge on the Fontan circulation and its consequences. A proposed surveillance testing toolkit provides recommendations for a range of acceptable approaches to follow-up care for the patient with Fontan circulation. Gaps in knowledge and areas for future focus of investigation are highlighted, with the objective of laying the groundwork for creating a normal quality and duration of life for these unique individuals. ispartof: CIRCULATION vol:140 issue:6 pages:E234-E284 ispartof: location:United States status: published

Author(s): Jenkins, Kathy J; Botto, Lorenzo D; Correa, Adolfo; Foster, Elyse; Kupiec, Jennifer K; Marino, Bradley S; Oster, Matthew E; Stout, Karen K; Honein, Margaret A

Advances in the management of pediatric patients with congenital heart disease (CHD) have led to an increased number of adults with CHD (ACHD). While many have undergone cardiac surgery, ACHD patients have premature morbidity and mortality and often die from cardiovascular events. In order to ensure appropriate follow-up for these patients, transition to a practice with experience managing ACHD patient is crucial. Ideally, these adolescents with CHD are placed on a seamless pathway for transition, but there are multiple barriers to this at the patient, provider, and health system levels. Repeated patient and family education is one intervention that has been shown to improve follow-up and cardiovascular outcomes.

The use of temporary mechanical circulatory support (tMCS) in cardiogenic shock has increased. Little is known about tMCS utility in adult congenital heart disease (ACHD) patients. National estimates of hospitalizations for ACHD patients were identified in the National Inpatient Sample. We

The number of adults with congenital heart disease (CHD) has increased markedly over the past few decades as a result of astounding successes in pediatric cardiac care. Nevertheless, it is now well understood that CHD is not cured but palliated, such that life-long expert care is required to optimize outcomes. All countries in the world that experience improved survival in CHD must face new challenges inherent to the emergence of a growing and aging CHD population with changing needs and medical and psychosocial issues. Founded in 1992, the International Society for Adult Congenital Heart Disease (ISACHD) is the leading global organization of professionals dedicated to pursuing excellence in the care of adults with CHD worldwide. Recognizing the unique and varied issues involved in caring for adults with CHD, ISACHD established a task force to assess the current status of care for adults with CHD across the globe, highlight major challenges and priorities, and provide future direction. The writing committee consisted of experts from North America, South America, Europe, South Asia, East Asia, and Oceania. The committee was divided into subgroups to review key aspects of adult CHD (ACHD) care. Regional representatives were tasked with investigating and reporting on relevant local issues as accurately as possible, within the constraints of available data. The resulting ISACHD position statement addresses changing patterns of worldwide epidemiology, models of care and organization of care, education and training, and the global research landscape in ACHD.

Durable ventricular assist device (VAD) support is uncommonly employed in adult congenital heart disease and often involves supporting a systemic right ventricle (RV). Ventricular assist device support of a subpulmonic RV is even more unusual.

A 28-year-old man with mild dyspnea on exertion presents for evaluation. He has a history of subaortic stenosis (SAS), bicuspid aortic valve (BAV), and coarctation of the aorta (CoA; Figure 1). He underwent repair of his CoA as a neonate with patch aortoplasty. At age 9, he was discovered to have SAS and had surgical resection of a subaortic membrane (Figure 2). His primary care provider referred him for evaluation after noting arterial hypertension and hearing a to-and-fro murmur on examination. Blood pressures suggested a recurrent coarctation with a 40 mm Hg gradient between the right arm and right leg. An echocardiogram demonstrated severe aortic regurgitation (AR; Figure 3) and recurrent SAS with a mean gradient of 31 mm Hg across the left ventricular outflow tract (LVOT). Cardiac magnetic resonance (CMR) confirmed recurrent CoA and estimated the aortic regurgitation fraction to be 30% (Figures 4 and 5). The patient underwent stenting of his CoA (Figure 6). Evaluation 6 months later revealed a mean gradient of 42 mm Hg across the LVOT with unchanged AR. Consequently, the patient underwent surgical aortic valve (AoV) replacement and SAS resection. Figure 1. Aortic coarctation by echocardiography. Echocardiogram in a 2-wk-old neonate with coarctation of the aorta. The 2-dimensional image ( A ) shows a tight stenosis at the isthmus (asterisk) with a posterior shelf (arrow) and mild hypoplasia of the aortic arch. The left common carotid artery (LCC) is dilated. The color Doppler image ( B ) demonstrates flow acceleration at the site of coarctation. Movie in the Data Supplement. AAo indicates ascending aorta; IA, innominate artery; and LSCA, left subclavian artery. Figure 2. Transesophageal echocardiogram of a discrete subaortic membrane. Transesophageal echocardiogram in the long-axis orientation showing a discrete subaortic membrane on the septal surface of the left ventricular (LV) outflow tract, 6 mm from the aortic valve leaflets. A , Two dimensional …

As the adult congenital heart disease (ACHD) population expands and ages, the incidence and prevalence of heart failure will rise. This poses several challenges, all complicated by our nascent understanding of heart failure epidemiology, pathophysiology and management in adults with congenital heart disease. Current definitions extrapolated from acquired heart failure often disregard the unique pathophysiology of heart failure in adults with congenital heart disease. Others have suggested that congenital heart disease is the ‘original heart failure syndrome’ implying that all ACHD patients are destined to manifest heart failure. Neither the adoption of acquired heart failure definitions nor the belief that heart failure is the common ultimate manifestation of ACHD has advanced the care of ACHD patients. The absence of a comprehensive definition that focuses on common themes while recognizing the unique manifestations of heart failure in ACHD stifles research progress and has translated to a paucity of ACHD specific recommendations in existing heart failure guidelines. Since many ACHD heart failure patients do not meet standard definitions of heart failure their access to potentially beneficial interventions such as cardiac rehabilitation is restricted by payers and regulators taking a narrow view of acquired heart failure guidelines. ACHD heart failure definitions that can be applied in the clinical and research setting are needed to guide treatment, facilitate communication between specialists, determine the prevalence and incidence of heart failure in AHCD, and improve ACHD patients' access to heart failure treatments. The purpose of this review is to understand how heart failure has been considered and defined in the existing ACHD literature and to highlight the need for a definition of heart failure applicable to ACHD.

Eric S. Williams, MD, MACC, Chair Jonathan L. Halperin, MD, FACC, Co-Chair James A. Arrighi, MD, FACC Eric H. Awtry, MD, FACC Eric R. Bates, MD, FACC Salvatore Costa, MD, FACC Lori Daniels, MD, FACC Akshay Desai, MD, FACC Douglas E. Drachman, MD, FACC Susan Fernandes, LPD, PA-C Rosario

The objective of this prospective multi-center study was to evaluate heart disease knowledge within the adult congenital heart disease (ACHD) population, pilot an educational intervention and assess interest in research participation among new patients at ACHD clinics.Many adults with congenital heart disease lack knowledge about their heart condition that may contribute to undesirable outcomes.Patients ≥18 years of age were recruited upon their first presentation to an ACHD clinic and underwent an educational intervention consisting of creation of a personal health information 'passport' and an introduction to web-based resources. Subjects were asked to complete initial and follow-up surveys documenting their perceived knowledge.Nine hundred twenty-two subjects were recruited from 12 ACHD centers, and 520 (57%) completed follow-up surveys. Patients who completed the follow-up survey were more likely to be women, have more education, and have mild heart disease. At follow-up, the ability of the subjects to name their heart condition improved (78% to 83%, p=0.002). Improvements were seen in mean Likert items regarding perceived knowledge of appropriate exercise (p0.0001), symptoms of heart rhythm problems or endocarditis (p0.0001), reasons for cardiac tests (p0.007), and birth control options and pregnancy safety (p0.0001). On follow-up, subjects reported a better understanding of medical research (p0.01), and higher interest in research participation (p0.003).This joint clinician-patient pilot program will help inform future efforts toward patient education and participation in research with a focus on standardization of protocols for life-long longitudinal follow-up and continued multi-center collaboration in the ACHD population.

Objectives The goal of this project was to quantify the prevalence of gaps in cardiology care, identify predictors of gaps, and assess barriers to care among adult congenital heart disease (adult CHD) patients. Background Adult CHD patients risk interruptions in care that are associated with undesired outcomes. Methods Patients (18 years of age and older) with their first presentation to an adult CHD clinic completed a survey regarding gaps in, and barriers to, care. Results Among 12 adult CHD centers, 922 subjects (54% female) were recruited. A >3-year gap in cardiology care was identified in 42%, with 8% having gaps longer than a decade. Mean age at the first gap was 19.9 years. The majority of respondents had more than high school education and knew their heart condition. The most common reasons for gaps included feeling well, being unaware that follow-up was required, and complete absence from medical care. Disease complexity was predictive of a gap in care with 59% of mild, 42% of moderate, and 26% of severe disease subjects reporting gaps (p Conclusions Adult CHD patients have gaps in cardiology care; the first lapse commonly occurred at age ∼19 years, a time when transition to adult services is contemplated. Gaps were more common among subjects with mild and moderate diagnoses and at particular locations. These results provide a framework for developing strategies to decrease gaps and address barriers to care in the adult CHD population.

Today, most female children born with congenital heart disease will reach childbearing age. For many women with complex congenital heart disease, carrying a pregnancy carries a moderate to high risk for both the mother and her fetus. Many such women, however, do not have access to adult congenital heart disease tertiary centers with experienced reproductive programs. Therefore, it is important that all practitioners who will be managing these women have current information not only on preconception counseling and diagnostic evaluation to determine maternal and fetal risk but also on how to manage them once they are pregnant and when to refer them to a regional center with expertise in pregnancy management.

Valvular heart disease constitutes considerable disease burden in the elderly and surgery remains the definitive treatment. Most valve dysfunction is chronic in nature and may not meet sufficient criteria for surgical consideration; however, additional stressors such as hypovolaemia, arrhythmia, or infection may lead to cardiovascular symptoms and haemodynamic compromise, necessitating intensive care unit management. Acute valve dysfunction is typically a surgical emergency, and medical therapy is selectively used to bridge to more definitive therapy. Some situations, such as mitral stenosis, may be effectively medically managed to delay a surgical procedure, but the majority of acute valve dysfunction that requires intensive care will eventually come to surgery.

While valvular heart disease encountered in developing countries is primarily rheumatic in aetiology, in industrialized countries it is largely comprised of degenerative valvular disease. Although less prevalent than ischaemic heart disease, its prevalence increases with older age and increased life expectancy, and therefore represents significant disease burden in aging populations. Transthoracic echocardiography remains the imaging modality of choice for timely delineation of the anatomy and severity of the lesion,although, once identified, may not correlate with symptoms due to clinical latency of disease onset to disease manifestation. Variations of disease severity, which may not meet criteria for intervention, lead to chronicity of disease, while clinically silent lesions may remain undiagnosed—both of these situations may lead to acute illness requiring intensive care management. Stabilization through medical intervention may be required, although many patients with severe disease will need emergent surgical repair, therefore collaborative involvement between intensivists, cardiologists, and cardiovascular surgeons is needed to minimize patient mortality and morbidity.

Described is a 42-year-old man with complex congenital heart disease with single-ventricle physiology who underwent a Fontan operation at 27 years of age. He presented with sudden-onset chest pain and dyspnea and was initially misdiagnosed with a pulmonary embolism. This case is described in order to highlight the challenges in the evaluation of common presentations in adults with congenital heart disease.

Interpretation of ECGs in athletes is complicated by physiological changes related to training. The purpose of this study was to determine the accuracy of ECG interpretation in athletes among different physician specialties, with and without use of a standised ECG criteria tool.Physicians were asked to interpret 40 ECGs (28 normal ECGs from college athletes randomised with 12 abnormal ECGs from individuals with known ciovascular pathology) and classify each ECG as (1) 'normal or variant--no further evaluation and testing needed' or (2) 'abnormal--further evaluation and testing needed.' After reading the ECGs, participants received a two-page ECG criteria tool to guide interpretation of the ECGs again.A total of 60 physicians participated: 22 primary care (PC) residents, 16 PC attending physicians, 12 sports medicine (SM) physicians and 10 ciologists. At baseline, the total number of ECGs correctly interpreted was PC residents 73%, PC attendings 73%, SM physicians 78% and ciologists 85%. With use of the ECG criteria tool, all physician groups significantly improved their accuracy (p0.0001): PC residents 92%, PC attendings 90%, SM physicians 91% and ciologists 96%. With use of the ECG criteria tool, specificity improved from 70% to 91%, sensitivity improved from 89% to 94% and there was no difference comparing ciologists versus all other physicians (p=0.053).Providing standised criteria to assist ECG interpretation in athletes significantly improves the ability to accurately distinguish normal from abnormal findings across physician specialties, even in physicians with little or no experience.

The role of antibiotic prophylaxis for prevention of infective endocarditis is unknown. Endocarditis prophylaxis is recommended for certain high-risk individuals prior to dental procedures. To our knowledge, this is the first case reported in the literature of a patient with complex congenital heart disease developing endocarditis in the period immediately following otherwise uncomplicated intrauterine device insertion.

As medical management and surgical techniques continue to improve, patients with congenital heart disease are surviving further into adulthood and seeking to participate in multiple activities. Given the increasing popularity of adventure recreation, it is likely that many of these individuals will express interest in travel to and activities at high altitude. At first glance, the hypoxia associated with acute altitude exposure would appear to pose high risks for patients with underlying cardiopulmonary disease, but few studies have systematically addressed these concerns in the adult congenital heart disease population. In this review, we consider the safety of high-altitude travel in these patients. After reviewing the primary cardiopulmonary responses to acute hypoxia and the risks of high altitude in all individuals regardless of their underlying health status, we consider the risks in adult congenital heart disease patients, in particular. We focus on broad concerns that should be considered in all patients such as whether they have underlying pulmonary hypertension, the adequacy of their ventilatory responses, and their ability to compensate for hypoxemia and right-to-left shunting. We then conclude by providing basic recommendations for pretravel assessment in patients with congenital heart disease of moderate or great complexity.

Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease (CHD). The history of surgical treatment of ToF is closely linked with the origins of cardiac surgery. Before the era of cardiac surgery, most ToF patients died in childhood; now well over 85% survive to adulthood, resulting in an increased prevalence of ToF in adults.1 Patients with “repaired” ToF pose unique challenges for primary care providers and cardiologists. This review will focus on the history and changing epidemiology of ToF in adults, the pathophysiology of repaired ToF, and emerging data for monitoring and treatment. The aorta and pulmonary artery form from septation of the distal bulbus cordis and truncus arteriosus and rotate to overlie the ventricles. Faulty rotation and septation with incomplete transfer of the aorta to a position above the left ventricle results in a malalignment ventricular septal defect (VSD) and an aorta that “overrides” the interventricular septum.w1 Right ventricle (RV) outflow tract obstruction, the other defining feature of ToF, is the result of one or more of the following abnormalities: subvalvar obstruction from septal deviation, hypertrophied muscular bands in the RV outflow tract, a hypoplastic pulmonary valve annulus, pulmonary valve stenosis or atresia, and main or branch pulmonary artery stenosis. The RV hypertrophies as a result of the outflow tract obstruction. Thus, the characteristic four features of ToF are an overriding aorta, RV outflow tract obstruction, malalignment VSD, and RV hypertrophy (fig 1). In addition, ToF is associated with other congenital cardiac abnormalities in about 40% of patients. Some of the more common anomalies are a right sided aortic arch (13–34%), atrial septal defect (∼15%), atrioventricular septal defect (1.7–7%), anomalous coronary arteries (most commonly a prominent conus artery or the left anterior descending artery from the right coronary artery or right sinus of Valsalva) …

This report describes the management of a 47-year-old patient with Ebstein's anomaly and severe right ventricular dysfunction. Her complicated postoperative course is discussed. In addition, age-related differences in the hemodynamic response to a one-and-one-half ventricle repair are reviewed.

Background Metabolic syndrome increases risk for atherosclerotic coronary artery disease, and its prevalence increases with increasing age and body mass index. Adults with congenital heart disease ( ACHD ) are now living longer and accruing coronary artery disease risk factors. However, the prevalence of metabolic syndrome in ACHD patients is unknown. Methods and Results We conducted a retrospective cohort study of ACHD patients at our center to quantify the prevalence of metabolic syndrome in an ACHD population. Using case‐control matching, we constructed a comparable control group from a population‐based sample of 150 104 adults. International Diabetes Federation criteria were used to define metabolic syndrome. We used logistic regression to compare the risk of metabolic syndrome across the resulting cohorts, which were composed of 448 ACHD patients and 448 controls matched by age and sex. Mean age of both groups was 32.4±11.3 years, and 51.3% were female. Obesity was present in 16.1% of the ACHD patients and 16.7% of the controls. Metabolic syndrome was more common in ACHD patients than in controls (15.0% versus 7.4%; odds ratio 1.82, 95% CI 1.25–2.65). Conclusions Our data suggest that metabolic syndrome is more common among adults with congenital heart disease than in the general population. Thus, patients with congenital heart disease should be screened for metabolic syndrome and risk factors mitigated where possible to prevent atherosclerotic coronary artery disease. Preventive cardiology should be included during routine ACHD care.

Introduction The past 60 years have brought remarkable advancements in the diagnosis and treatment of congenital heart disease (CHD). Early diagnosis and improvements in cardiac surgery and interventional cardiology have resulted in unprecedented survival of patients with CHD, even those with the most complex lesions. Despite remarkable success in treatments, many interventions are palliative rather than curative, and patients often develop cardiac complications, including heart failure (HF). HF management in the setting of CHD is challenged by the wide range of ages at which HF occurs, the heterogeneity of the underlying anatomy and surgical repairs, the wide spectrum of HF causes, the lack of validated biomarkers for disease progression, the lack of reliable risk predictors or surrogate end points, and the paucity of evidence demonstrating treatment efficacy. The purposes of this statement are to review the literature pertaining to chronic HF in CHD and to elucidate important gaps in our knowledge, emphasizing the need for specific studies of HF mechanisms and improving outcomes for those with HF. In this document, the definition of CHD severity is the definition common in CHD documents, including the American College of Cardiology (ACC)/American Heart Association (AHA) guidelines1 for the management of adults with CHD (Table 11–3). The definition of HF corresponds to that found in the multiple guidelines on diagnosis and management of HF. Although nuances and specific details may be controversial,4 the broad definition from the Heart Failure Society of America guidelines states the following: “In physiologic terms, HF is a syndrome characterized by either or both pulmonary and systemic venous congestion and/or inadequate peripheral oxygen delivery, at rest or during stress, caused by cardiac dysfunction.”5 The definition of chronic HF in this document concurs with that of the European Society of Cardiology guidelines, which emphasize chronic HF …

Dramatic evolution in the medical and surgical care of children with congenital heart disease (CHD) has led to a growing number of adults with late-onset complications, including heart failure (HF).1 In parallel with an overall increase in hospital admissions for adults with CHD (ACHD) and HF,2 CHD complexity has increased substantially in survivors over the past 2 decades.3 Heart transplant (HTx) specialists face the challenge of determining eligibility for advanced HF treatments among an increasingly complex population of CHD patients in whom guidelines for HTx and mechanical circulatory support (MCS) are scant. The purpose of this review is to provide a state-of-the-art update on HTx and MCS in CHD. HTx remains the surgical procedure of choice for eligible patients with severe advanced HF,4,5 with little change in the number of transplants performed yearly over the past decade. The body of information related to transplantation for CHD is derived almost entirely from registry and single-center–based outcome data; no randomized clinical trial or meta-analysis data are available. CHD presents additional challenges to successful HTx compared with HTx in patients with acquired HF. Many CHD patients require complex vascular reconstruction at the time of transplantation. The presence of antibodies to human leukocyte antigen (HLA) and ABO blood group sensitization are also impediments to timely transplantation. The ability of patients with single-ventricle physiology to survive during the waiting period is also limited by the additional burden of “outgrowing” their pulmonary blood flow and the resultant cyanosis. It is not surprising that CHD remains a risk factor during the waiting period and after transplantation. Therefore, although the management of the CHD patient with end-stage HF must include the option of HTx, its indication and timing are very different from that for acquired HF. Patients with ACHD represent an increasing …

Aortic stenosis is a common condition, particularly in the elderly. The treatment is surgical, and any patient with symptomatic severe aortic stenosis should be considered for aortic valve replacement. Aortic stenosis causes an increase in afterload to the left ventricle, which when severe can lead to hemodynamic instability. Although the therapy of aortic stenosis is valve replacement, determining whether a patient has symptoms and accurately assessing the severity of stenosis can be difficult. The management of patients with severe aortic stenosis in the intensive care unit setting can be very challenging, particularly when comorbid medical conditions make aortic valve replacement difficult. This article reviews the diagnosis of aortic stenosis, methods of assessing symptoms and severity, and management of severe symptomatic stenosis, particularly in the intensive care unit setting. Components of the history that suggest symptomatic aortic stenosis are presented. The role of physical examination is discussed, as are the echocardiographic means of determining stenosis severity. Other means of assessing severity are addressed, as are circumstances in which there can be difficulty in interpretation, such as severe aortic stenosis and left ventricular dysfunction. Management of patients, focusing on the intensive care unit setting, is reviewed, with a focus on the timing of aortic valve replacement.

Background Mortality for children with congenital heart disease (CHD) has declined with improved surgical techniques and neonatal screening; however, as these patients live longer, accurate estimates of the prevalence of adults with CHD are lacking. Methods To determine the prevalence and mortality trends of adults with CHD, we combined National Vital Statistics System data and National Health Interview Survey data using an integrative systems model to determine the prevalence of recalled CHD as a function of age, sex, and year (by recalled CHD, we mean positive response to the question “has a doctor told you that (name) has congenital heart disease?”, which is a conservative lower-bound estimate of CHD prevalence). We used Human Mortality Database estimates and US Census Department projections of the US population to calculate the CHD-prevalent population by age, sex, and year. The primary outcome was prevalence of recalled CHD in adults from 1970 to 2050; the secondary outcomes were birth prevalence and mortality rates by sex and women of childbearing age (15–49 years). Results The birth prevalence of recalled CHD in 2010 for males was 3.29 per 1,000 (95 % uncertainty interval (UI) 2.8–3.6), and for females was 3.23 per 1,000 (95 % UI 2.3–3.6). From 1968 to 2010, mortality among zero to 51-week-olds declined from 170 to 53 per 100,000 person years. The estimated number of adults (age 20–64 years) with recalled CHD in 1968 was 118,000 (95 % UI 72,000–150,000). By 2010, there was an increase by a factor of 2.3 (95 % UI 2.2–2.6), to 273,000 (95 % UI 190,000–330,000). There will be an estimated 510,000 (95 % UI: 400,000–580,000) in 2050. The prevalence of adults with recalled CHD will begin to plateau around the year 2050. In 2010, there were 134,000 (95 % UI 69,000–160,000) reproductive-age females (age 15–49 years) with recalled CHD in the United States. Conclusion Mortality rates have decreased in infants and the prevalence of adults with CHD has increased but will slow down around 2050. This population requires adult medical systems with providers experienced in the care of adult CHD patients, including those familiar with reproduction in women with CHD. Electronic supplementary material The online version of this article (doi:10.1186/s12963-015-0063-z) contains supplementary material, which is available to authorized users.

The number of adults with congenital heart disease (CHD) has increased markedly over the past few decades as a result of astounding successes in pediatric cardiac care. Nevertheless, it is now well understood that CHD is not cured but palliated, such that life-long expert care is required to optimize outcomes. All countries in the world that experience improved survival in CHD must face new challenges inherent to the emergence of a growing and aging CHD population with changing needs and medical and psychosocial issues. Founded in 1992, the International Society for Adult Congenital Heart Disease (ISACHD) is the leading global organization of professionals dedicated to pursuing excellence in the care of adults with CHD worldwide. Recognizing the unique and varied issues involved in caring for adults with CHD, ISACHD established a task force to assess the current status of care for adults with CHD across the globe, highlight major challenges and priorities, and provide future direction. The writing committee consisted of experts from North America, South America, Europe, South Asia, East Asia, and Oceania. The committee was divided into subgroups to review key aspects of adult CHD (ACHD) care. Regional representatives were tasked with investigating and reporting on relevant local issues as accurately as possible, within the constraints of available data. The resulting ISACHD position statement addresses changing patterns of worldwide epidemiology, models of care and organization of care, education and training, and the global research landscape in ACHD.