Adult Congenital Heart Patients Research Articles

Impact of new allocation policy on waitlist and transplant outcomes of adult congenital heart patients supported with ECMO.

The use of ECMO as a bridge to heart transplantation has been growing rapidly in all heart transplant recipients since the implementation of the new UNOS allocation policy; however, the impact on adult congenital heart disease (ACHD) patients is not known. We analyzed the UNOS data (2015-2021) for ACHD patients supported with extracorporeal membrane oxygenation (ECMO) during the waitlist, before and after October 2018, to assess the impact on the waitlist and posttransplant outcomes. We compared the characteristics and outcomes of ACHD patients with or without ECMO use during the waitlist and pre- and postpolicy changes. A total of 23 821 patients underwent heart transplantation, and only 918 (4%) had ACHD. Out of all ACHD patients undergoing heart transplants, 6% of patients in the prepolicy era and 7.6% in the postpolicy era were on ECMO at the time of listing. Those on ECMO were younger and sicker compared to the rest of the ACHD cohort. Those on ECMO had similar profiles pre- and postpolicy change; however, there was a very significant decrease in the waitlist time [136 days (IQR 29-384) vs. 38 days (IQR 11-108), p = 0.01]. There was no difference in waitlist mortality; however, competing risk analyses showed a higher likelihood of transplantation (51% vs. 29%) and a lower likelihood of death or deterioration (31% vs. 42%) postpolicy change. Long-term outcomes posttransplant for those supported with ECMO compared to the non-ECMO cohort are similar for ACHD patients, although there was higher attrition in the first year for the ECMO cohort. The new allocation policy has resulted in shorter waitlist times and a higher likelihood of transplantation for ACHD patients supported by ECMO. However, the appropriate use of ECMO and the underuse of durable circulatory support devices in this population need further exploration.

Towards improving the lived experience of adult congenital heart patients: suggestions for health care practitioners.

What steps may clinicians looking after adult congenital heart patients take to improve the care they provide? An adult with complex congenital heart disease (CHD) who required a Fontan circulation has developed a discussion paper with the help of his attending clinicians. Drawing on his personal experience and that of others that he has represented, the paper highlights the problems in communication between such patients and their physician, followed by suggestions as to how to improve patient engagement, empowerment, and enablement. Attention to those suggestions may well benefit all in the care provided for this growing population.

Utility of urinary liver-type fatty acid-binding protein as a prognostic marker in adult congenital heart patients hospitalized for acute heart failure.

Progression to acute kidney injury (AKI) under treatment in adult congenital heart disease (ACHD) patients with heart failure is associated with poor prognosis, early detection and interventions are necessary. We aimed to explore the utility of urinary liver-type fatty acid binding protein (L-FABP) in ACHD patients hospitalized for acute decompensated heart failure (ADHF). We prospectively evaluated hemodynamic, biochemical data, and urinary biomarkers including urinary L-FABP in ACHD patients hospitalized in our institution from June 2019 to March 2022. The primary outcomes were the development of AKI and death. AKI was defined as serum creatinine level increased by 0.3mg/dl or more within 5days after hospitalization. A total of 104 ADHF patients aged 31 (36-51) years were enrolled. 26 cases (25% of ADHF patients) developed AKI during hospitalization and 4 died after hospital discharge. Serum creatinine (sCr), serum total bilirubin, brain natriuretic peptide (BNP), and urinary L-FABP in AKI patients were significantly higher than in non-AKI patients, whereas systemic oxygen saturation of the peripheral artery (SpO2) and estimated glomerular filtration ratio in AKI patients were lower than non-AKI patients. There was no difference in the intravenous diuretic dose on admission and during hospitalization between the two groups. In the receiver operating characteristic (ROC) analysis, the maximum area under the curve (AUC) of urinary biomarkers in AKI patients was urinary L-FABP (AUC = 0.769, p < 0.001) with a cutoff value of 4.86µg/gCr. Urinary L-FABP level on admission was associated with a predictor for AKI development during hospitalization after adjusting for sCr, BNP and SpO2. Urinary L-FABP was a useful predictor for the development of AKI in ACHD patients hospitalized for ADHF. Monitoring of urinary L-FABP allows us to detect a high-risk patient earlier than the conventional biomarkers.

Cardiac Society of Australia and New Zealand (CSANZ) Position Statement on the Follow-Up of Cardiovascular Implantable Electronic Devices 2022.

Recognising the need for a national approach for the recommended best practice for the follow-up of implanted cardiac rhythm devices to ensure patient safety, this document has been produced by the Cardiac Society of Australia and New Zealand (CSANZ). It draws on accepted practice standards and guidelines of international electrophysiology bodies. It lays out methodology, frequency, and content of follow-up, including remote monitoring; personnel, including physician, allied health, nursing and industry; paediatric and adult congenital heart patients; and special considerations including magnetic resonance imaging scanning, perioperative management, and hazard alerts.

Ablation of intra-atrial reentrant tachycardias in adults with congenital heart disease: islands, isthmuses, channels, and walls.

The adult congenital heart patient population is rapidly growing due to increasing survival rates, and they often face chronic physiologic challenges as sequalae of both their defects and repairs. Among the most common sequalae are arrhythmias. Here we describe intra-atrial reentrant tachycardia (IART), one of the most commonly seen arrhythmias in the adult congenital heart population, and the approaches to successful ablation in adult congenital heart patients. IART has increasing ablation success rates due to the increasing exposure of electrophysiologists to congenital cases, advances in technology, and the increasing application of both our pediatric congenital knowledge and adult acquired knowledge to the adult congenital population. IART is a frequently seen arrhythmia in the adult congenital population, and it can have life-threatening consequences in the setting of congenital disease. Ablation techniques and treatment rates have improved over time, despite the challenges these cases present to clinicians; success of the ablation depends on careful, often creative, preplanning, and understanding of the complex individualized anatomy and circuits of the patient.

Lessons learnt from COVID-19 in adult congenital heart patient in Tehran: a survey-based study of prevention, exposure, susceptibility, and outcomes.

COVD-19 pandemic has overwhelmed many healthcare systems worldwide. Underlying cardiovascular disease predisposes to greater disease susceptibility and more complications including mortality. Such data is unverified in adults with congenital heart disease (ACHD). The aim of the study is to report the Tehran experience with respect to preventative self-care measures, disease exposure, susceptibility, and outcomes after COVD-19 infection in ACHD patients. A telephone-based survey was conducted in ACHD patients, focusing on new-onset symptoms that might indicate COVID-19 infection, prevention measures, confirmed infection rates, and outcomes. Three-hundred and nine ACHD patients, with a mean age of 29.13 years (range from 14 to 72 years, SD = 10.64), and 170 (55%) women were assessed. The majority (86.7%) had moderate or complex ACHD. Two-thirds (67.3%) of the patients practiced high-level preventative self-care measures. After community exposure, 33.3% developed COVID-19, and after household exposure, 43.7% developed COVID-19. There was only one mortality in a post-operative patient. Thirty-seven patients (12%) reported new symptoms including cough (10%), fatigue (8%), fever (7%), and new dyspnoea (6.5%). Amongst 18 (6%) with confirmed COVID-19, there was only 1 mortality in a post-operative patient. Age (adjusted OR = 1.19, 95% CI: 1.07-1.31, p = 0.001), contact with confirmed COVID-19 cases (adjusted OR = 59.34, 95% CI: 3.68-955.10, p = 0.004) were independently associated with COVID-19 infection. Mortality risk associated with COVID-19 infection in ACHD patients with moderate or severe disease appears to be relatively low, similar to the general population. Such risk appears to act through conventional risk factors, and in this cohort, we demonstrated age as a significant risk factor in addition to exposure to the development of COVID-19 infection. Preventative self-care measures are a potentially significant and impactful intervention target for intervention and for improving outcomes.

The Real-World Utility of the LINQ Implantable Loop Recorder in Pediatric and Adult Congenital Heart Patients.

This study sought to determine the practical use of the recently introduced LINQ implantable loop recorder (LINQ-ILR) in a cohort of pediatric and adult congenital arrhythmia patients. Correlating symptoms to a causative arrhythmia is a key aspect of diagnosis and management in clinical electrophysiology. Retrospective review of clinical data, implantation indications, findings, and therapeutic decisions in patients who underwent LINQ-ILR implantation from April 1st, 2014 to January 30th, 2017 at Boston Children's Hospital. A total of 133 patients were included, of which 76 (57%) were male. The mean age at implantation was 15.7 ± 9.1 years with a duration of follow-up of 11.8 months. Congenital heart disease was present in 34 patients (26%), a confirmed genetic diagnosis in 50 (38%), and cardiomyopathy in 22 (26%), and the remainder were without a previous diagnosis. Syncope was the most common indication for LINQ-ILR implantation, occurring in 59 patients (44%). The median time to diagnosis was 4.5 months, occurring in 78 patients (59%). Cardiac device placement occurred in 17 patients (22%), a medication change in 9 (12%), electrophysiology study/ablation in 5 (6%), or LINQ-ILR explantation in 42 (54%). Infection or erosion occurred in 5 patients. Syncope was correlated with a diagnostic transmission (54% vs. 31%, p= 0.01). The LINQ-ILR is an important diagnostic tool, providing useful data in more than one-half of patients in<6 months. Adverse events are low. Patient selection is critical and undiagnosed syncope represents an important presenting indication for which a LINQ-ILR implant should be considered.

Acute Myocardial Infarction with Isolated Congenitally Corrected Transposition of the Great Arteries

Congenital cardiac abnormalities diagnosed at the time of acute coronary syndrome are rare. A 43-year-old man presented to the emergency department complaining of recurring, severe chest pain. Subsequent emergent coronary angiography demonstrated unusual coronary anatomy: 1) one small caliber bifurcating vessel originating from the right sinus of Valsalva; 2) one very large vessel arising from the posterior sinus; and 3) no coronary artery from the normal left sinus of Valsalva. The large vessel from the posterior sinus was totally occluded in its midportion and was treated with intravascular ultrasound-guided percutaneous coronary intervention. Further diagnostic workup, including two-dimensional transthoracic echocardiogram and computed tomographic coronary angiography, demonstrated isolated corrected transposition of the great arteries with a dilated systemic ventricle and systolic dysfunction with an ejection fraction of 30%. The patient's clinical course was complicated by recurrent nonsustained ventricular tachycardia, treated with medical therapy and a dual-chamber implantable cardioverter defibrillator. This case is an example of a common clinical presentation with a very uncommon congenital heart disorder. Similar cases may become more frequent as the number of adult congenital heart patients increases in the population.

Multiprofessional and Intrahospital Experience for Diagnosis and Treatment of Pulmonary Arterial Hypertension

Referral centres for pulmonary hypertension will provide care by a multiprofessional team, which should as a minimum comprise: consultant physicians with a special interest in PH, clinical nurse specialist, radiologist, cardiologist with expertise in echocardiography. this study sought to determine whether the experience of the establishment of a clinic for pulmonary arterial hypertension, initially created only for the treatment and diagnosis of heart failure, may be considered positive. From 1 July 2008 to January 1, 2012 we evaluated 80 patients in our ambulatory dedicated to the diagnosis and treatment of PAH. All patients were performed to clinical evaluation, ECG, and echocardiography with estimation of the sPAP. Then we evaluated the functional capacity through cardiopulmonary exercise testing or six minute walking test (6MWT). RHC was required to confirm the diagnosis of pulmonary arterial hypertension. 80 patients (mean age: 50.9 +/- 18.68 years, 31 males) were evaluated in our center; the largest groups subjected to screening were thalassemia (21 subjects), rheumatologic patients (18 patients), respirators, suspected of "out-of Proportion" (12 patients) and 4 patients with OSAS. 8 adult congenital heart patients. A diagnosis of PAH after right heart catheterization was possible in 25 cases. In particular, among patients with pulmonary arterial hypertension, 8 had a rheumatic etiology (systemic sclerosis), 2 post-thromboembolic disease, 5 patients had congenital heart disease, 1 patient with HIV infection, 1 patient with thalassemia major, 1 chronic lymphocytic leukemia and 1 with myelodysplasia. The initial experience of our center and network within our hospital may be considered positive, because it permitted to patients easy access to hospital services, to undertake a comprehensive prognostic stratification and to recognize the early signs of worsening in subsequent tests.

Echocardiographic evaluation of right ventricular function in congenital heart disease.

This review aims to provide an overview of conventional and novel indices used in clinical and research arenas for evaluation of right ventricular (RV) function in congenital heart diseases with a dual-chambered circulation. Articles cited in this review were selected using PubMed search of publications in English with no date limits. The search terms included "echocardiography", "right ventricle", "RV function", "cardiac function", and "congenital heart disease". Key references were also searched for additional publications. Articles related to description of echocardiographic techniques in the evaluation of subpulmonary or systemic RV function and their applications in congenital cardiac malformations were retrieved and reviewed. Three approaches have been used to evaluate subpulmonary and systemic RV function: (1) assessment of changes in RV size in the cardiac cycle, (2) determination of Doppler-derived velocities and systolic and diastolic time intervals, and (3) quantification of myocardial velocities and deformation. Conventional and novel echocardiographic techniques enable the evaluation of subpulmonary and systemic RV function. Novel echocardiographic techniques have further allowed quantification of RV volumes and direct interrogation of myocardial deformation. These new techniques show promise in a more comprehensive evaluation beyond "eye-balling" of RV function in the growing population of adolescent and adult congenital heart patients.

Contemporary transcatheter interventions in adult congenital heart disease.

in 1966, William rashkind performed the first balloon atrial septostomy on a patient with transposition of the great arteries,1 and on that fateful day, the field of congenital and pediatric interventional cardiology was born. since then, the complexity and capability of the discipline has grown explosively with the advent of balloon aortic and pulmonary valvuloplasty,2 device closure of atrial and ventricular septal defects and patent ductus arteriosus, and the Melody® transcatheter pulmonary valve solution3 (Medtronic, inc., Minneapolis, Mn). the successes of rashkind’s cardiac surgical colleagues over the last 50 years has given rise to a population of nearly 1.3 million adult congenital heart patients in the united states.4 since this population is expected to grow by 5% annually, the sustained cardiovascular care of such a heterogeneous and complex group of patients will require ongoing evolution as physicians continue to learn how best to treat them. likewise, the role of transcatheter intervention continues to evolve and grow as an adjunct to cardiac surgery in managing mechanical issues—especially in patients who conceivably live well into their 70s or 80s when just 50 years ago they may not have survived their first year. Because each patient is unique in pathophysiology, surgical repair, comorbidities, and postsurgical pathophysiology, there are few medical devices that have been approved specifically for the treatment of congenital heart disease. as such, the practice of transcatheter congenital intervention is characterized by continuous on-the-fly innovation—adapting catheters, wires, and devices from other disciplines and applications and building new devices. in this issue of the Methodist deBakey Cardiovascular Journal, we focus on the depth and breadth of transcatheter interventions in congenital heart disease. drs. Balzer, forbes, holoshitz, kenny, tarui, and colleagues present a number of unique innovations that characterize the field’s rapid evolution. the use of novel 3-dimensional imaging techniques both before and during catheter intervention is described by fagan and colleagues to optimize complex interventions. finally, Bergersen and colleagues discuss the unfathomably complex task of defining standards of quality in the heterogeneous field of congenital interventions. While few of our readers likely care for adult patients with congenital heart disease on a regular basis, we hope that this issue will serve to stimulate curiosity, innovation, collaboration, and awareness of this rapidly growing medical need. C.H. Lin, M.D., Ph.D. D.T. Balzer, M.D.

Gerbode defect misinterpreted as pulmonary hypertension

Survival into adulthood is now routine for patients with congenital heart disease (CHD). Adult cardiology practices increasingly encounter these patients, however, are often challenged by the complexity of the cardiac lesions, prior surgical interventions, and associated conditions. We describe a case of a 64-year-old woman with history of CHD and multiple operations, including surgical aortic valve replacement, who presented for routine evaluation. During transthoracic echocardiogram she was believed to have idiopathic severe pulmonary hypertension (tricuspid regurgitation velocity of 4.6m/s) and a ventricular septal defect (VSD). Consequently, she was referred to a pediatric CHD center. On noninvasive evaluation it became clear that a flow jet through a Gerbode defect, from left ventricle to right atrium, had led to the initial misdiagnosis of severe pulmonary hypertension. The patient was reassured that the VSD was small, restrictive, and that the pulmonary pressures were normal.<Learning objective: Recognition of a left ventricle-to-right atrial shunt (Gerbode defect) is challenging in the adult congenital heart patient. However, this diagnosis can be made with a noninvasive imaging strategy in the context of a high clinical suspicion: A combination of transthoracic echocardiography and cardiac MRI findings of a high velocity “tricuspid regurgitation jet” but normal appearing right ventricular pressures in someone with prior subaortic membrane and aortic valve surgery led to the diagnosis of a Gerbode defect in our patient.>.

Should complex ACHD patients have periodic Holter monitoring?

This article stems from a debate held at the Adult Congenital Heart Meetings at Cincinnati in the summer of 2011 where Dr. Moodie and Broberg debated whether complex adult congenital heart patients should have regular Holter monitor studies done. Dr. Moodie took the pro position and Dr. Broberg the con and this article is a result of that debate.

Development of Pediatric and Congenital Heart Surgery in Latin America

In the last 70 years, congenital heart surgery has dramatically evolved, and Latin America has completed this journey with unique regional features. Since the first ligation of a patent arterial duct by Enrique Finochietto in 1941 in Buenos Aires, the development of congenital heart surgery was deeply influenced by funding restrictions and scarcity of technology. However, the determined work of cardiovascular surgery pioneers as Hugo Filipozzi, Euriclides Zerbini, and Adib Jatene in Brazil; Helmut Jaeger in Chile; Hugo Baz and Clemente Robles in Mexico; Alberto Bejarano in Colombia; and Mario Brea and Fernando Tricerri in Argentina made cardiac surgery with cardiopulmonary bypass available by the late 1950s. In the following five decades new generations of cardiovascular surgeons received the legacy of these outstanding leaders and made several important contributions to the field in tetralogy of Fallot, transposition of the great arteries, tricuspid atresia, single ventricle, truncus arteriosus, heart transplantation, and many others. Many centers in Latin America routinely perform congenital heart disease surgery with excellent results, covering the entire spectrum from the newborn to the adult congenital heart patient. The most important challenge that remains is to provide access to care to all children with congenital heart disease in Latin America, since currently only 42% of them receive surgical treatment.

Adult Congenital Heart Surgery: Adult or Pediatric Facility? Adult or Pediatric Surgeon?

One of the current controversies in the field of adult congenital heart disease is whether patients should be cared for at an adult or pediatric facility and by an adult or pediatric heart surgeon. After transitioning our program from the children's hospital to the adult hospital, we analyzed our experience with each system. Between 2000 and 2007, 303 operations were performed on adults (age >or= 18 years) with congenital heart disease. One hundred eighty-five operations were performed in an adult hospital and 118 in a pediatric hospital. Forty-six operations were performed by an adult heart surgeon and 257 by a congenital heart surgeon. Mean age, coexisting medical problems, and preoperative risk factors were higher in both the adult hospital group and adult surgeon group compared with the respective pediatric groups. Mortality was similar at the adult and pediatric hospitals (4.3% versus 5.1%), but was markedly higher in the adult surgeon group compared with the pediatric surgeon group (15.2% versus 2.7%; p = 0.0008). By multivariate analysis, risk factors for mortality included older age at the time of surgery (p = 0.028), surgery performed at a children's hospital (p = 0.013), and surgery performed by an adult heart surgeon (p = 0.0004). Congenital heart surgery can be performed in adults with reasonable morbidity and mortality. Caring for an anticipated aging adult congenital population with increasingly numerous coexisting medical problems and risk factors is best facilitated in an adult hospital setting. Also, when surgery becomes necessary, these adult patients are best served by a congenital heart surgeon.

Advances in pediatric electrophysiology.

The pediatric electrophysiology literature during the past year has addressed several topics that are particularly relevant for children and other patients with congenital heart disease. This paper reviews selected studies germane to physicians and health care personnel who treat pediatric and adult congenital heart patients with arrhythmias and electrophysiologic disorders. Advances in arrhythmia diagnostics have been reported in pediatrics using loop monitoring, both external and implanted. Diagnostic criteria and risk stratification strategies have been refined for the congenital and inherited rhythm disorders such as cardiomyopathies and long QT syndrome. The use of therapeutic procedures such as catheter ablation for complex arrhythmias in congenital heart disease is discussed. Finally, a summary of articles on implanted devices in pediatrics and congenital heart disease is reviewed, including implantable defibrillators, atrial antitachycardia pacemakers, and cardiac resynchronization therapy in pediatrics. Pediatric electrophysiology is a rapidly changing field, with advances seen in diagnostic evaluation of arrhythmia, refinement of risk-stratification testing, and therapeutic options such as catheter ablation and cardiac rhythm management devices. The evolution of pediatric electrophysiology from a diagnostic specialty into a therapeutic and interventional subspecialty has advanced the treatment options for children with cardiac arrhythmias and conduction disorders.

The surgical management of congenital heart disease in the adult

There are an increasing number of adults with congenital heart disease due to greater success with palliative and corrective operations, along with improvements in the medical management of operated and unoperated patients. There are approximately 1 million adult corrected, palliated or uncorrected congenital heart patients; which is estimated to increase by 5% per year w1x The growing importance of this area of cardiology and cardiac surgery is reflected by subspecialty training and education, such as the recent 32nd Bethesda Conference on ‘The Care of Adults with Congenital Heart Disease’ w2x. Surgery on adult congenital heart patients is unique in that the surgical and perioperative care team (cardiology and critical care in particular) should have a thorough understanding of the particular anatomy and physiology involved. In our own experience 260 patients over the age of 18 years have undergone an operation to correct a congenital heart defect over the past 5 years (Table 1). Table 1 represents roughly 15% of our overall operated congenital heart population. These lesions range widely in complexity and include aortic valve disease that has close similarity with acquired heart disease and management to the more complex single ventricle physiology that requires a dedicated congenital heart team. The types of cardiac lesions encountered in adult patients, such as those listed in Table 1 can be divided into lesions by increasing complexity and likelihood of reaching adult age and requiring surgery. Previously operated individuals who require further surgery represent the largest group by far. Need for further intervention is based on a number of factors that include electrophysiologic, valvular (native), prosthetic materials (valves, patches and conduits), myocardial, vascular and neurologic w3x.

Diagnosis and management of congenital heart disease in the adult.

Currently, there are 1 million people in the United States over the age of 20 with congenital heart disease. These adult congenital heart patients can "slip through the cracks of our medical system" and many are too old to be cared for in most pediatric institutions by pediatric cardiologists, and, unfortunately, most adult cardiologists are not trained in congenital heart disease. Therefore, it is important to understand what the common lesions are in adult congenital heart disease and how they should be managed. Acyanotic congenital heart disease in the adult population primarily involves left-to-right shunts, such as atrial septal defect, ventricular septal defect, patent ductus arteriosus, and obstructive lesions such as aortic coarctation of the aorta. The most common form of cyanotic congenital heart disease in adults is tetralogy of Fallot. Other complex conditions seen in adults include univentricular hearts, Ebstein's anomaly of the tricuspid valve, and corrected transposition of the great vessels. Most patients with congenital heart disease will need to undergo surgery, catheterization, or catheterization intervention. Results are excellent in the adult population. Long-term follow-up is needed for any adult congenital heart patient receiving care in institutions that are well organized and well equipped, as we learn more about the natural and unnatural history of these conditions.

Effects of atrial flutter on exercise tolerance in adult congenital heart patients Wei Li, Michael Y. Henein, Jane Somerville. Royal Brompton Hospital, London, United Kingdom

Effects of atrial flutter on exercise tolerance in adult congenital heart patients Wei Li, Michael Y. Henein, Jane Somerville. Royal Brompton Hospital, London, United Kingdom

Adult congenital heart disease.

There are approximately 500,000 adults in the United States with congenital heart disease, and this group is growing at 5% per year. Adult cardiologists are, for the most part, poorly trained in the treatment of congenital heart disease; pediatric cardiologists, on the other hand, work in children's hospitals where it is difficult to care for adults. It is important, therefore, to review the current literature as it relates to adult congenital heart disease. This report details the long-term follow-up of patients who were operated on in childhood for congenital heart disease and are now adults. In addition, it reviews information related to the social adaptation of adult congenital heart patients. There are a number of interesting new reports on specific adult congenital heart anomalies. Atrial septal defects as well as the relationship between a patent foramen ovale and stroke are also reviewed.