Adult Congenital Heart Disease Research Articles

Comparison of advanced echocardiographic right ventricular functional parameters with cardiovascular magnetic resonance in adult congenital heart disease.

Advanced transthoracic echocardiography (TTE) using volumetric and deformational indices provides detailed quantification of right ventricular (RV) function in adults with congenital heart disease (ACHD). Two-dimensional multi-plane echocardiography (2D-MPE) has demonstrated regional wall differences in RV longitudinal strain (LS). This study aims to evaluate the association of these parameters with cardiovascular magnetic resonance (CMR). One-hundred stable ACHD patients with primarily affected RVs were included (age 50 ± 5 years; 53% male). Conventional and advanced echocardiographic RV functional parameters were compared with CMR-derived RV function. Advanced echocardiographic RV functional parameters were measurable in approximately one-half of the study cohort, while multi-wall LS assessment feasibility was lower. CMR RV ejection fraction (CMR-RVEF) was moderately correlated with deformational, area, and volumetric parameters [RV global LS (lateral wall and septum), n = 55: r = -0.62, P < 0.001; RV wall average LS, n = 34: r = -0.49, P = 0.002; RV lateral wall LS, n = 56: r = -0.45, P < 0.001; fractional area change, n = 67: r = 0.48, P < 0.001; 3D-RVEF, n = 48: r = 0.40, P = 0.005]. Conventional measurements such as TAPSE and RV S' correlated poorly. RV global LS best identified CMR-RVEF < 45% (area under the curve: 0.84, P < 0.001: cut-off value -19%: sensitivity 100%, specificity 57%). RVEF and LS values were significantly higher when measured by CMR compared with TTE (mean difference RVEF: 5 [-9 to 18] %; lateral (free) wall LS: -7 [7 to -21] %; RV global LS: -6 [5 to -16] %) while there was no association between respective LS values. In ACHD patients, advanced echocardiographic RV functional parameters are moderately correlated with CMR-RVEF, although significant differences exist between indices measurable by both modalities.

Cannabis Use: A New Risk Behaviour Among Adults With Congenital Heart Disease

BackgroundCannabis use has increased in Canada and can be associated with adverse cardiovascular events. Given increased use and accessibility to cannabis, there is a need among clinicians to better understand cannabis use in adults with congenital heart disease. MethodsA cross-sectional survey (May to September 2018) was used to investigate cannabis use among 252 patients with adult congenital heart disease in a quaternary care centre. ResultsOf the 252 patients, 53 (21%) reported using cannabis. The majority of cannabis users were men (62%), between the ages of 25 and 39 years (mean age = 32 ± 16 years), and more likely to use tobacco (n = 9, 17%; P = 0.001) and alcohol (n = 37, 60%; P = 0.001). Significant differences (P = 0.011) were found between the age of onset for tobacco use among cannabis users (mean age: 16 ± 8 years) and non-cannabis users (mean age: 20 ± 3 years). Users reported consuming cannabis for recreational purposes (n = 29, 55%), anxiety (n = 22, 42%), depression (n = 15, 28%), and pain management (n = 4, 8%). ConclusionsThis study supports our clinical experience that a high proportion of patients with adult congenital heart disease use cannabis. Cannabis users represent a patient population who may demonstrate less optimal health behaviours, including tobacco and alcohol use. Assessment of cannabis use should be an integral part of risk behaviour and cardiovascular risk profile at each clinic visit. Given the current legalization of cannabis in Canada and the growing increase of cannabis use, educational support should be provided to patients and caregivers.

The Use of Virtual Reality Learning on Transition Education in Adolescents with Congenital Heart Disease.

Improvement in congenital heart disease (CHD) outcomes has created a growing population of adolescents and young adults with unique health needs that require thoughtful transition planning and eventual transfer of care to an adult provider. Often, poor health literacy and limited resources can lead to interrupted care, which places them at risk for adverse health-related consequences. In 2019, the Wisconsin Adult Congenital Heart Disease transition program partnered with Stanford Virtual Heart (SVH), a virtual reality (VR) platform, to allow young adult patients to learn about their CHD in a clinic-based setting. We completed a single-center pilot study to evaluate these patients' experience and perceptions to using VR during their transition education. At an initial transition visit, we used an immediate post-VR experience survey, scored using Likert scales of 1-5 (1 = strongly disagree, 5 = strongly agree). Twenty-two patients (13 males) between the ages of 16 and 19 participated. Lesions included pulmonary stenosis, Tetralogy of Fallot, atrial and ventricular septal defect, coarctation, aortic stenosis, hypoplastic left heart syndrome, and patent ductus arteriosus. Likert averages were 4.7 for finding VR helped with understanding their heart lesion, 4.6 for finding VR helped with understanding their heart surgery, 4.7 for enjoying the VR heart simulation, and 4.6 for finding that it was a good use of time. This study demonstrates that adolescents enjoyed using SVH and found it helpful. Clinical implementation shows promise as a plausible adjunct tool for transition education.

Design and Harmonization Approach for the Multi-Institutional Neurocognitive Discovery Study (MINDS) of Adult Congenital Heart Disease (ACHD) Neuroimaging Ancillary Study: A Technical Note.

Dramatic advances in the management of congenital heart disease (CHD) have improved survival to adulthood from less than 10% in the 1960s to over 90% in the current era, such that adult CHD (ACHD) patients now outnumber their pediatric counterparts. ACHD patients demonstrate domain-specific neurocognitive deficits associated with reduced quality of life that include deficits in educational attainment and social interaction. Our hypothesis is that ACHD patients exhibit vascular brain injury and structural/physiological brain alterations that are predictive of specific neurocognitive deficits modified by behavioral and environmental enrichment proxies of cognitive reserve (e.g., level of education and lifestyle/social habits). This technical note describes an ancillary study to the National Heart, Lung, and Blood Institute (NHLBI)-funded Pediatric Heart Network (PHN) "Multi-Institutional Neurocognitive Discovery Study (MINDS) in Adult Congenital Heart Disease (ACHD)". Leveraging clinical, neuropsychological, and biospecimen data from the parent study, our study will provide structural-physiological correlates of neurocognitive outcomes, representing the first multi-center neuroimaging initiative to be performed in ACHD patients. Limitations of the study include recruitment challenges inherent to an ancillary study, implantable cardiac devices, and harmonization of neuroimaging biomarkers. Results from this research will help shape the care of ACHD patients and further our understanding of the interplay between brain injury and cognitive reserve.

Characteristics and outcomes of socioeconomically disadvantaged pregnant individuals with adult congenital heart disease presenting to a Cardio-Obstetrics Program

Outcomes of individuals with adult congenital heart disease (ACHD) who are socioeconomically disadvantaged and cared for in a cardio-obstetric programs are lacking. To describe clinical characteristics, maternal pregnancy outcomes and contraceptive uptake of individuals with ACHD in an urban cardio-obstetric program. Retrospective data were collected for individuals with ACHD seen in the Maternal Fetal Medicine-Cardiology Joint Program at Montefiore Health System between 2015-2021 and compared by modified World Health Organization (mWHO) class I,II vs. mWHO ≥II/III. Over 90% of individuals with ACHD were pregnant at the time of referral. mWHO I,II (n=77, 62.4% Black or Hispanic/Latina) had a total of 94 pregnancies and mWHO ≥II/III (n=49, 49.0% Black or Hispanic/Latina), a total of 56 pregnancies. Over 25% of individuals in each group had a body mass index ≥30 (p=0.78), as well as very low summary socioeconomic scores. mWHO ≥II/III were more likely to be anticoagulated in the first trimester than mWHO I, II (10.7% vs. 0.0%, p=0.002) and throughout pregnancy (14.3% vs 3.2% p=0.02). mWHO ≥II/III were more likely to require arterial monitoring during delivery than mWHO I, II (14.3% vs. 0.0%, p=0.001) or delivery under general anesthesia (8.9% vs. 1.1%, p=0.03), but had a comparable frequency of Cesarean delivery (35.8% vs. 41.3%, p=0.68). There were no in-hospital maternal deaths. There was no difference in the type of contraception recommended by mWHO class, however, mWHO ≥II/III were more likely to receive long acting types or permanent sterilization (35.6% vs 54.6%, p=0.045). In a socioeconomically disadvantaged cohort with ACHD from a historically marginalized community, those with mWHO ≥II/III had more complex antepartum and intrapartum needs, but similar maternal and obstetric outcomes as mWHO I,II. The multidisciplinary approach offered by a cardio-obstetrics program may contribute to successful outcomes in this high-risk cohort and these data are hypothesis generating.

Cardiac transplantation in adult congenital heart disease: a narrative review.

As more children with congenital heart disease survive to adulthood, adult congenital heart disease (ACHD) prevalence will increase (currently ~1 million US patients). Heart failure (HF) accounts for 26-42% of ACHD deaths. The rate of ACHD heart transplantations (ACHD HTx) is also increasing. We describe the ACHD HTx recipient/candidate cohort, analyze ACHD HTx outcomes, identify ACHD HTx specific challenges, and discuss opportunities to better serve more patients with ACHD HF. PubMed literature search including articles published from 2010-2023. Reviewed 89 studies, 67 included. Our search focused on the challenges of ACHD HTx and potential solutions. ACHD HTx recipients are young [median age 35 years, interquartile range (IQR): 24-46 years]. 87-95% of ACHD HTx recipients had prior cardiac surgery. The most common underlying diagnoses include transposition of the great arteries (31%) and Fontan/Glenn circulation (28%). 63% of listed ACHD HTx candidates received a transplant within one year of listing. Post-transplant 1-year survival is 80%, 5-year survival 74%, and 10-year survival 59%. There are 4 unique ACHD HTx challenges: (I) difficulty in assessing pulmonary hypertension, resulting in some centers selecting oversized donor hearts. However, selecting oversized hearts does not improve post-operative mortality and could prolong waitlist time. (II) Increased immunologic sensitization, increasing rejection risk. Desensitization therapy has enabled sensitized HTx recipients to enjoy outcomes similar to non-sensitized recipients. (III) Procedural complexity with ~30% of cases requiring additional surgical reconstruction. Detailed multidisciplinary planning, extensive imaging, and transferring the patient into the operating room early can help manage the complexities and reduce organ ischemic time. (IV) Increased intraoperative bleeding due to patients' surgical histories and circulatory collaterals. Preoperative collateral coil embolization and select utilization of hypothermic circulatory arrest can help reduce bleeding. Additional Fontan specific challenges include extensive great artery repair, liver failure, plastic bronchitis, and protein loss enteropathy. Finally, given limited donor heart availability, mechanical circulatory support is a promising technology for patients with ACHD HF. The prevalence of ACHD HTx is slowly but steadily increasing. The operational complexity of ACHD HTx can be managed, and the majority of recipients have excellent outcomes (59% 10-year survival).

Preprocedural Anxiety in Adults With Congenital Heart Disease: The PANIC Study

BackgroundPreprocedural anxiety may have detrimental effects both cognitively and physiologically. ObjectivesThe objective of this study was to determine the association between state (situational) and trait (persistent in everyday life) anxiety and differences between the adult congenital heart disease (ACHD) and acquired heart disease populations. MethodsThe State-Trait Anxiety Inventory and financial stress scale were administered to adults with acquired and CHD at 4 tertiary referral centers in the United States prior to cardiac catheterization. Student’s t-test and least absolute shrinkage and selection operator regression analyses were used to assess differences in anxiety between groups and identify the optimal model of predictors of anxiety. ResultsOf the 291 patients enrolled, those with CHD (n = 91) were younger (age 41.3 ± 16.3 years vs 64.7 ± 11.3 years, P < 0.001), underwent more cardiac surgeries (P < 0.001), and had higher levels of trait anxiety (t[171] = 2.62, P = 0.001, d = 0.33). There was no difference in state anxiety between groups (t[158.65] = 1.37, P = 0.17, d = 0.18). State anxiety was singularly associated with trait anxiety. Trait anxiety was negatively associated with age and positively associated with state anxiety and financial stress. Patients with CHD of great complexity were more trait (F[2,88] = 4.21, P = 0.02) and state anxious (F[2,87] = 4.59, P = 0.01), though with relatively small effect size. ConclusionsTrait anxiety levels are higher in the ACHD population and directly associated with state anxiety. Specialists caring for ACHD patients should not only recognize the frequency of trait anxiety but also high-risk subgroups that may benefit from psychological or social interventions to reduce preprocedural anxiety.

Survival After Cardiac Transplantation in Adults With Single-Ventricle Congenital Heart Disease

BackgroundWithout large-scale analyses of adults with single-ventricle congenital heart disease (CHD) undergoing heart transplantation, little evidence exists to guide listing practices and patient counseling. ObjectivesThis study aims to evaluate survival after heart transplantation in adults with single and biventricular CHD and compare it to that of non-CHD transplant recipients. MethodsIn this 15-year (2005-2020) retrospective analysis, outcome-blinded investigators used probability-linkage to merge the National (Nationwide) Inpatient Sample and Organ Procurement and Transplantation Network data sets. ResultsOf 382 adult (≥18 years of age) heart transplant recipients with CHD, 185 (48%) had single-ventricle physiology. Compared to biventricular CHD, single-ventricle patients showed significantly reduced survival at 1 (80% vs 91%; HR: 2.50; 95% CI: 1.40-4.49; P = 0.002) and 10 years (54% vs 71%; HR: 2.10; 95% CI: 1.38-3.18; P < 0.001). Among patients who survived the first post-transplantation year, biventricular CHD patients exhibited similar 10-year survival as single-ventricle patients, except for those with hypoplastic left heart syndrome (79% vs 71%; HR: 1.58; 95% CI: 0.85-2.92; P = 0.15). Additionally, biventricular CHD transplant recipients showed significantly better 10-year conditional survival compared to their non-CHD counterparts (79% vs 68%; HR: 0.73; 95% CI: 0.59-0.90; P = 0.003). ConclusionsAmong adult CHD transplant recipients, single-ventricle physiology correlated with higher short-term mortality. However, 10-year conditional survival was similar for biventricular and most single-ventricle CHD patients, and notably better for biventricular CHD patients compared to non-CHD heart transplant recipients. These findings have significant implications towards patient selection and listing strategies, easing concerns related to heart transplantation in adults with CHD and destigmatizing most subtypes of single-ventricle CHD.

Adult Congenital Heart Disease in the Aging and Elderly Population

Adult Congenital Heart Disease in the Aging and Elderly Population

An 18-year evolution of congenital heart disease in China: An echocardiographic database-based study

ObjectiveData on the evolution of congenital heart disease (CHD) in China remain scarce. Based on a Chinese echocardiography database, we analyzed the observed rate (OR) and spectrum changes of CHD over the past 18 years with a focus on the congenital aortic valve malformation (CAVM) and Adult CHD (ACHD). Methods and resultsThe transthoracic echocardiographic data of 682,565 records from 2003 to 2020 were retrospectively reviewed at Fujian Medical University Union Hospital, China. A total of 37,200 CHD cases were recruited in this study. Over the three periods (from 2003 to 2008, 2009–2014, to 2015–2020), the OR of Total CHD decreased (106.72, 90.64, and 67.43 per 1000 cases, respectively); the proportion of Simple CHD to Total CHD increased (80.96%, 83.41%, and 87.97%, respectively), with a decrease in the proportion of Complex CHD (18.11%, 15.51%, and 10.42%, respectively) (p < 0.05 for all). The proportion of ACHD increased in most types of CHD [Total CHD: 25.79%, 27.84%, and 31.43%; CAVM: 69.02%, 73.42%, and 78.16%; CAVM with aortic stenosis (AS): 67.42%, 70.73%, and 79.25%; respectively, p < 0.05 for all], with a much higher proportion in both CAVM and CAVM with AS than in the other CHD types. The proportion of CHD patients receiving intervention increased over the designated periods. ConclusionsThis study depicts the longitudinal changes of CHD in the Chinese population with a single-center echocardiographic data, revealing an increased proportion of Simple CHD, ACHD (including CAVM and CAVM with AS), and a decreased OR of Total CHD and proportion of Complex CHD.

Long-Term outcomes in adult patients with congenital heart disease considered for transplantation: A single center study.

Adult congenital heart disease (ACHD) patients pose unique challenges in identifying the time for transplantation and factors influencing outcomes. To identify hemodynamic, functional, and laboratory parameters that correlate with 1- and 10-year outcomes in ACHD patients considered for transplantation. A retrospective chart review of long-term outcomes in adult patients with congenital heart disease (CHD) evaluated for heart or heart + additional organ transplant between 2004 and 2014 at our center was performed. A machine learning decision tree model was used to evaluate multiple clinical parameters correlating with 1- and 10-year survival. We identified 58 patients meeting criteria. D-transposition of the great arteries (D-TGA) with atrial switch operation (20.7%), tetralogy of Fallot/pulmonary atresia (15.5%), and tricuspid atresia (13.8%) were the most common diagnosis for transplant. Single ventricle patients were most likely to be listed for transplantation (39.8% of evaluated patients). Among a comprehensive list of clinical factors, invasive hemodynamic parameters (pulmonary capillary wedge pressure (PCWP), systemic vascular pressure (SVP), and end diastolic pressures (EDP) most correlated with 1- and 10-year outcomes. Transplanted patients with SVP<14 and non- transplanted patients with PCWP<15 had 100% survival 1-year post-transplantation. For the first time, our study identifies that hemodynamic parameters most strongly correlate with 1- and 10-year outcomes in ACHD patients considered for transplantation, using a data-driven machine learning model.

Novel Therapeutic Strategies in Heart Failure in Adult Congenital Heart Disease: of Medicines and Devices.

This paper reviews the latest literature on the growing field of heart failure in the adult congenital heart disease population. After highlighting the increasing prevalence and a few of the unique potential causes, including the concept of early senescence, this review begins with novel medical management strategies such as the angiotensin II receptor blocker and neprilysin inhibitors and sodium glucose cotransporter-2 inhibitors. Then, it addresses the latest applications of percutaneous techniques like implantable hemodynamic monitoring, transcatheter pulmonary and aortic valve replacement, and mitral clips. Cardiac resynchronization therapy and novel lymphatic system imaging and intervention are then described. Finally, the use of mechanical support devices, temporary and durable, is discussed as well as heart and combined heart and liver transplantation. There have been recent exciting advances in the strategies used to manage adult congenital heart disease patients with heart failure. As this population continues to grow, it is likely we will see further rapid evolution in this field.

The Effects of Cardiac Implantable Electronic Device Personal Protective Equipment Use in Patients with Pediatric and Adult Congenital Heart Disease.

Pediatric and adult congenital heart disease (ACHD) patients encounter physical and emotional barriers. Cardiac implantable electronic devices (CIEDs), including pacemakers and implantable cardiac defibrillators (ICD) often compound these issues. Patient fear associated with damaging the CIED system can lead to avoidance of physical activity and reduced quality of life (QOL). CIED personal protective equipment (PPE) is a potential treatment for decreasing this fear. We sought to determine the effects of CIED PPE use in the pediatric and ACHD population. Patients 5 years or older with a CIED at a single pediatric and ACHD heart rhythm center received a CIED protector and customized athletic shirt. QOL was assessed using the PedsQL TM 4.0 SF15 prior to and after 6-12months. Of the 77 patients enrolled, 26 completed repeat assessment. The mean age at enrollment was 15.6years (range 5-36) with a mean device age of 2.2years (< 1-10years). Pacemakers were present in 77% and 23% had ICDs. The PPE was used in 92% with no PPE malfunction. Fear associated with physical activity was reduced, z = -4, p < 0.001, with a large effect size (r = 0.55). There was a trend toward increased physical activity. Total QOL scores improved, z = -2.771, P < 0.05, with a medium effect size (r = 0.4). This first study of CIED PPE in children shows that providing CIED PPE to pediatric and ACHD patients may decrease their fear of damaging their device system, increasing physical activity levels, and improving quality of life.

Sleep-disordered breathing and lung function abnormalities in adults with congenital heart disease

PurposeAdvances in treatment enables most patients with congenital heart diseases (CHD) to survive into adulthood, implying the need to address comorbid conditions in this growing cohort of patients. The aim of this study was to evaluate the prevalence of sleep-disordered breathing (SDB) and lung function abnormalities in patients with adult congenital heart disease (ACHD).MethodsPatients with ACHD underwent level 3 sleep testing (Embletta MPR polygraphy) and pulmonary function testing. Results were stratified by the underlying haemodynamic ACHD lesion group.ResultsPatients with ACHD (n = 100) were middle-aged (42.3 ± 14.6 years), 54% male and slightly overweight (BMI 25.9 ± 5.5 kg/m2). Polygraphy revealed a prevalence of sleep apnoea of 39% with 15% of patients presenting with predominantly obstructive apnoeic episodes, while 23% of patients presenting primarily with central sleep apnoea. The distribution of mild, moderate, and severe sleep apnoea in the total study population was 26%, 7% and 6%, respectively. Comparison of apnoea–hypopnoea index, presence of sleep apnoea, and apnoea severity did not offer significant differences between the four ACHD lesion groups (p = 0.29, p = 0.41 and p = 0.18, respectively). Pulmonary function testing revealed obstructive lung disease in 19 of 100 patients. Concomitant chronic obstructive pulmonary disease and obstructive sleep apnoea were diagnosed in 3% of patients and were associated with profound nocturnal desaturation.ConclusionThe findings suggest a mild propensity amongst patients with ACHD to develop SDB that seems to be unaffected by the specific underlying congenital lesion.

Extracorporeal Life Support for Cardiogenic Shock in Adult Congenital Heart Disease-An ELSO Registry Analysis.

There are minimal data on the use of venoarterial extracorporeal membrane life support (VA-ECLS) in adult congenital heart disease (ACHD) patients presenting with cardiogenic shock (CS). This study sought to describe the population of ACHD patients with CS who received VA-ECLS in the Extracorporeal Life Support Organization (ELSO) Registry. This was a retrospective analysis of adult patients with diagnoses of ACHD and CS in ELSO from 2009-2021. Anatomic complexity was categorized using the American College of Cardiology/American Heart Association 2018 guidelines. We described patient characteristics, complications, and outcomes, as well as trends in mortality and VA-ECLS utilization. Of 528 patients who met inclusion criteria, there were 32 patients with high-complexity anatomy, 196 with moderate-complexity anatomy, and 300 with low-complexity anatomy. The median age was 59.6 years (interquartile range, 45.8-68.2). The number of VA-ECLS implants increased from five implants in 2010 to 81 implants in 2021. Overall mortality was 58.3% and decreased year-by-year (β= -2.03 [95% confidence interval, -3.36 to -0.70], p = 0.007). Six patients (1.1%) were bridged to heart transplantation and 21 (4.0%) to durable ventricular assist device. Complications included cardiac arrhythmia/tamponade (21.6%), surgical site bleeding (17.6%), cannula site bleeding (11.4%), limb ischemia (7.4%), and stroke (8.7%). Utilization of VA-ECLS for CS in ACHD patients has increased over time with a trend toward improvement in survival to discharge.

Cardiogenic shock in adults with congenital heart disease: Insights from the FRENSHOCK registry

BackgroundData on cardiogenic shock in adults with congenital heart disease (ACHD) are scarce. AimWe sought to describe cardiogenic shock in ACHD patients in a nationwide cardiogenic shock registry. MethodsFrom the multicentric FRENSHOCK registry (772 patients with cardiogenic shock from 49 French centres between April and October 2016), ACHD patients were compared with adults without congenital heart disease (non-ACHD). The primary outcome was defined by all-cause mortality, chronic ventricular assist device or heart transplantation at 1year. ResultsOut of the 772 patients, seven (0.9%) were ACHD, who were younger (median age: 56 vs. 67years), had fewer cardiovascular risk factors, such as hypertension (14.3% vs. 47.5%) and diabetes (14.3% vs. 28.3%), and no previous ischaemic cardiopathy (0 vs. 61.5%). Right heart catheterization (57.1% vs. 15.4%), pacemakers (28.6% vs. 4.6%) and implantable cardioverter-defibrillators (28.6% vs. 4.8%) were indicated more frequently in the management of ACHD patients compared with non-ACHD patients, whereas temporary mechanical circulatory support (0 vs. 18.7%) and invasive mechanical ventilation (14.3% vs. 38.1%) were less likely to be used in ACHD patients. At 1year, the primary outcome occurred in 85.7% (95% confidence interval: 42.1–99.6) ACHD patients and 52.3% (95% confidence interval: 48.7–55.9) non-ACHD patients. Although 1-year mortality was not significantly different between ACHD patients (42.9%) and non-ACHD patients (45.4%), ventricular assist devices and heart transplantation tended to be more frequent in the ACHD group. ConclusionsCardiogenic shock in ACHD patients is rare, accounting for only 0.9% of an unselected cardiogenic shock population. Despite being younger and having fewer co-morbidities, the prognosis of ACHD patients with cardiogenic shock remains severe, and is similar to that of other patients.

Arrhythmias in adult congenital heart disease at the emergency department: ANMCO Tuscany clinical pathway

Arrhythmias are a common complication in the adult population with congenital heart disease (ACHD). Arrhythmias often lead to hemodynamic instability and, on the other hand, may be a marker of hemodynamic impairment in ACHD patients, both in natural history and after cardiac surgery. Treatment requires knowledge of basic anatomy and any previous cardiac surgery; the availability of patient's health records, if possible, is therefore crucial for therapeutic choices. In the emergency setting, the first target is represented by the patient's hemodynamic stabilization; mainly in moderate or high complexity ACHD, the connection with the referral center is recommended, to which patients should be entrusted for follow-up. A regional epidemiological observatory, aiming to assess the number, type and outcomes of emergency admissions of ACHD patients could be a useful tool for analyzing the effectiveness of the collaboration network between the different structures involved and for implementing organizational pathways.

Tachyarrhythmias in congenital heart disease patients: diagnosis, clinical presentation and treatment

Tachyarrhythmias are a major cause of morbidity and mortality in adult congenital heart disease patients. There is a strong association between tachyarrhythmias and the specific congenital heart disease and the patient's surgical history. Health care for adults with congenital heart disease and arrhythmias should be coordinated by adult congenital heart disease centers of excellence that serve as a resource for consultation and referral. A complete and multidisciplinary evaluation of patients with tachyarrhythmias, including a detailed hemodynamic and electrophysiological evaluation, is mandatory to define the best treatment strategy.

Novel strategy for postcardiotomy support in a patient with adult congenital heart disease.

Novel strategy for postcardiotomy support in a patient with adult congenital heart disease.

Analyzing the Content Found on Fellowship Websites for Adult Congenital Heart Disease.

The Adult Congenital Heart Disease (ACHD) fellowship is a two-year fellowship that can be done by physicians who have finished their internal medicine residency and cardiology fellowship. This study evaluated the accessibility and provided information on the websites of the ACHDfellowship programs to identify potential areas of improvement for future fellowship applicants. Analysis of 25 ACHD fellowship program websites was conducted based on 34 criteria under three main categories: recruitment information, education and research information, and incentive information. This study found that many evaluated ACHD program websites lacked information regarding recruitment. Specifically, information regardingmentorship opportunities, hospital statistics/number of beds, selection process, and interview dates, leaving out crucial details on what to expect during the matching process. Additionally, more information on education and research is beneficial for applicants to sufficiently compare ACHD fellowship programs and make more informed decisions about which programs they would like to apply to. Information on academic stipends, evaluation criteria, expected caseload, moonlighting opportunities, elective opportunities, rotation schedules, call requirements, and types of procedures were all limited across multiple websites. Lastly, incentive information was found to be insufficient across most ACHD fellowship websites. Incentive information included fellow wellness, harassment policies, parental leave, salary, benefits, and vacation/sick leave. This study shows that ACHD fellowship programs need to supply more information on their websites to provide applicants with details to help them choose the fellowship program that corresponds best with their career goals. Expanding upon information regarding recruitment, education, research, and incentives will provide applicants with a strong understanding of ACHD fellowship programs and what they can expect throughout their education. In return, this will help ACHD fellowship programs attract stronger applicants, ultimately improving the quality of their respective programs.