Adult Congenital Heart Disease Program Research Articles

Pregnancy-related cardiac outcomes among patients with congenital heart disease after formalization of a cardio-obstetrics program

The prevalence of pregnant patients with congenital heart disease (CHD) is increasing, and these patients are at high risk for cardiac morbidity. This study aimed to examine the pregnancy outcomes in patients with congenital heart disease before and after the establishment of formal cardio-obstetrics collaboration between adult congenital heart disease and maternal-fetal medicine programs. This was a retrospective cohort study of pregnant patients with congenital heart disease from 2002 to 2020 at a single urban academic institution in the United States. This study included patients with a singleton pregnancy who continued a pregnancy beyond 20 weeks of gestation. The primary outcome was a composite adverse maternal cardiac outcome, compared before (2002-2010) and after (2011-2020) the program. The secondary outcomes included gestational age at delivery, mode of delivery, rate of labor induction, use of diuresis after delivery, and a composite maternal morbidity outcome. The number of pregnant patients with congenital heart disease increased after formalization of the cardio-obstetrics program (200 [postprogram group] vs 84 [preprogram group]; 0.48% of all deliveries in the postprogram group vs 0.25% of all deliveries in the preprogram group; P<.001). The postprogram group was more likely to undergo labor induction than the preprogram group (126 [63%] vs 34 [41%], respectively; P<.001). There were fewer patients in the postprogram group than in the preprogram group who were New York Heart Association class II to IV (23 [12%] vs 17 [22%], respectively; P=.04) or with systemic ventricular dysfunction (8 [4%] vs 12 [16%], respectively; P=.001). There was no difference in the primary outcome (38 [19%] in the postprogram group vs 14 [17%] in the preprogram group; P=.64), even after adjusting for confounders, including New York Heart Association class >I and systemic ventricular dysfunction (adjusted odds ratio, 2.3; 95% confidence interval, 0.96-5.4). Patients in the postprogram group were more likely to receive diuresis after delivery than patients in the preprogram group, even in the absence of heart failure or pulmonary edema (9 [4.5%] vs 0 [0.0%], respectively; P=.04). In the period after the establishment of a formal cardio-obstetrics program between adult congenital heart disease and maternal-fetal medicine, the number of patients with congenital heart disease delivering at our institution increased significantly. Overall, fewer patients entered pregnancy with advanced-stage heart failure or systemic ventricular dysfunction, possibly suggesting improved prepregnancy cardiac care or improved preconception counseling. Composite maternal cardiac outcomes were similar, but the rates of postpartum diuresis increased significantly, suggesting increased attention to volume status in the postpartum period. Formalized collaboration between congenital heart disease and maternal-fetal medicine may help better optimize patients' care before conception, during pregnancy, and after delivery.

Organization and structure of intensive care management of adult congenital heart disease in the United States

BackgroundThe growing number of adults with congenital heart disease has led to the formation of dedicated centers to provide specialized care. There is a paucity of published data on the organization, staffing, and resources available when adult congenital heart disease (ACHD) patients are hospitalized in intensive care settings. This study aims to describe the delivery of intensive care for ACHD patients in the United States and identify key differences between accredited centers or those pending accreditation and non-accredited Adult Congenital Heart Association (ACHA) centers. MethodsA web-based cross-sectional survey targeting centers with specialized ACHD programs was conducted. ResultsData were obtained from 48 of 96 centers (50%). Twenty-seven (56%) were accredited by the ACHA and 8 (17%) were pending accreditation. Surgical ACHD patients were primarily co-managed by cardiologists and intensivists (n = 11, 23%) and treated within an adult cardiac surgical intensive care unit (n = 34, 72%). Compared to non-accredited centers, ACHA accredited centers or those pending accreditation were more likely to offer daily rounds with ACHD providers (30, 86% vs. 6, 46%, p=<0.01), were more likely to support an ACHD fellowship training program (16, 46% vs. 0, 0%, p=<0.01); and were more likely to have durable mechanical support, such as ventricular assist devices (35, 100% vs. 8, 62%, p < 0.01). ConclusionThis survey reports current trends in organization, staffing, and resources available to ACHD patients requiring intensive care. ACHA accredited centers or those pending accreditation were more likely to have dedicated ACHD rounds, fellowship training programs, and access to durable mechanical support.

Safety of SGLT-2 inhibitors in the management of heart failure in the adult congenital heart disease patient population

BackgroundSodium glucose transporter 2 inhibitors (SGLT-2i) have shown safety and efficacy in patients with heart failure (HF). However, evidence for the use of SGLT-2i in adult congenital heart disease (ACHD) patients with HF is limited. MethodsWe performed a retrospective, single center analysis of 18 patients (>18 years of age) with ACHD and a diagnosis of HF who were initiated on an SGLT-2i. Patient characteristics, including vital signs, laboratory values, concomitant medications, clinical outcomes, and echocardiograms, were obtained as part of standardized clinical care at our ACHD program before and 2–6 months after initiation of SGLT-2i. The primary outcome was to demonstrate safety of SGLT-2i initiation via potential changes in systolic blood pressure, serum sodium, and serum creatinine. ResultsOf the 18 patients, 11 (61%) had moderate complexity congenital heart disease while 7 (39%) had great complexity congenital heart disease. Post initiation, there were no significant differences in systolic blood pressure (121.8 ± 20.8 mmHg to 114.4 ± 14.9 mmHg, p = 0.06), sodium level (138.7 ± 2.9 mMol/L to 138.0 ± 2.2 mMol/L, p = 0.75), and creatinine level (0.85 ± 0.18 mg/dL to 0.89 ± 0.18 mg/dL, p = 0.07). There was a statistically significant decline in weight (78.9 ± 22.9 kg to 76.0 ± 23.0 kg, p = 0.0039) but without a statistically significant change in NT-pro NBP (1358.2 ± 2735.0 pg/mL to 601.6 ± 786.1 pg/mL, p = 0.36). ConclusionsWe demonstrated the use of SGLT-2i in a small cohort of ACHD population, including patients with complex congenital heart disease, appears safe and well tolerated. The safety and potential efficacy of SGLT-2i in patients with ACHD will require further evaluation in prospective multicenter studies.

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.

The association between cardiac magnetic resonance-derived aortic stiffness parameters and aortic dilation in young adults with bicuspid aortic valve: With and without coarctation of aorta

BackgroundBicuspid aortic valve (BAV) is associated with progressive aortic dilation. Studies in aortopathies have shown a correlation between increased aortic stiffness and aortic dilation. We aimed to evaluate aortic stiffness measures as predictors of progressive aortic dilation by cardiac magnetic resonance (CMR) in BAV patients. MethodsThis is a retrospective study of 49 patients with BAV (median age 21.1 years at first CMR visit) with ≥2 CMR at the Wisconsin Adult Congenital Heart Disease Program (WAtCH). Circumferential aortic strain, distensibility, and β-stiffness index were obtained from CMR-derived aortic root cine imaging, and aortic dimensions were measured at aortic root and ascending aorta. A linear mixed-model and logistic regression were used to identify important predictors of progressive aortic dilation. ResultsOver a median of 3.8 years follow-up, the annual growth rates of aortic root and ascending aorta dimensions were 0.25 and 0.16 mm/year, respectively. Aortic strain and distensibility decreased while β-stiffness index increased with age. Aortic root strain and distensibility were associated with progressive dilation of the ascending aorta. Baseline aortic root diameter was an independent predictor of >1 mm/year growth rate of the aortic root (adjusted OR 1.34, 95 % CI 1.03–1.74, p = 0.028). Most patients (61 %) had coexisting coarctation of aorta. Despite the higher prevalence of hypertension in patients with aortic coarctation, hypertension or coarctation had no effect on baseline aorta dimensions, stiffness, or progressive aortic dilation. ConclusionSome CMR-derived aortic stiffness parameters correlated with progressive aortic dilation in BAV and should be further investigated in larger and older BAV cohorts.

The Impact of and Barriers to Cardiac Rehabilitation Following Cardiac Surgery in the Adult With Congenital Heart Disease.

There is a paucity of literature evaluating the impact of and barriers to participation in cardiac rehabilitation (CR) in the adult congenital heart disease population. The aims of this study were to evaluate the impact of CR on physical activity and health-related quality of life, as well as to evaluate the barriers to participation in CR in a post-operative adult congenital heart disease population. Patients ≥18 yr of age seen in the Wisconsin Adult Congenital Heart Disease Program and post-open sternotomy surgery from 2010-2015 were eligible for inclusion. Subjects were mailed a novel physical activity survey and the validated EuroQOL-5D 3L health questionnaire. A retrospective medical record review was performed to extract demographic and clinical data. One hundred thirty-five patients underwent open sternotomy surgery from 2010-2015. Of these, 22 were excluded because of intellectual disability, three opted out, and three survey packets were returned to the sender. A total of 54 of the remaining 107 patients returned completed surveys. Of these, 47 (87%) were referred to CR. Thirty-five patients completed the entire CR program (74%). Those who completed CR were more likely to develop a home/independent exercise program (P = .027). Barriers to completing CR included insurance coverage, psychiatric disease, and a perception that CR would not be of benefit. Completing CR was associated with developing a home/independent exercise program in post-sternotomy adult patients with congenital heart disease. Barriers to participating in and completing CR in this population could lead to an improved completion rate if modified.

Developing an Interactive Geographic Information System for Adult Congenital Heart Disease Service Planning in Rural New South Wales

With medical advances greatly improving life expectancy, Adult Congenital Heart Disease (ACHD) patients have become a priority population requiring lifelong, specialised care. Guidelines specific to the Australian context suggest that a state-wide ACHD program should include a comprehensive ACHD centre that is supported by smaller, regional services [ [1] Celermajer D. Nicolae M. Hornung T. O’Donnell C. Justo R. Bullock A. et al. Adult congenital heart disease (ACHD) recommendations for standards of care: The Cardiac Society of Australia and New Zealand. http://www.csanz.edu.au/wp-content/uploads/2013/11/ACHD-Guidelines.pdfDate: 2013 Google Scholar ]. It is unclear what are the best locations for these regional ACHD services in New South Wales (NSW).

Do we have the ACHD physician resources we need to care for the burgeoning ACHD population?

Delivery of care to the adult congenital heart disease (ACHD) population has been limited by a shortage in the ACHD physician resources. There is limited data regarding the adequacy of the ACHD physician resources in the United States and our population estimates are extrapolated from Canadian data. Therefore, we proposed to evaluate the adequacy of ACHD physician: patient ratios in the United States at both national and regional levels. Data from the Adult Congenital Heart Association (ACHA) website along with metropolitan area and statewide population data from 2016 US Census Bureau estimates were analyzed. Physicians listed on the ACHA website were cross-referenced with ABIM to verify ACHD board certification status. There are 115 self-identified ACHD programs and 418 self-identified ACHD physicians listed in the ACHA website. There are 320 board-certified ACHD cardiologists in the United States today, including 161 not listed in the ACHA website. Regarding ratios of ACHD-certified physicians to patients, the best served metropolitan statistical area (MSA) is Raleigh-Cary, NC, and the worst served MSA is Riverside-San Bernardino-Ontario, CA. The best served State is Washington, DC, and the worst served State is Indiana. The ACHD population continues to grow, and the looming national physician shortage is likely to greatly affect the ability to meet the complex needs of this growing population. In order to bring the ACHD patient: physician ratio to 1000:1, a minimum of 170 additional ACHD board-certified physicians are needed now.

Relationship Between Types of Social Support, Coping Strategies, and Psychological Distress in Individuals Living With Congenital Heart Disease.

Many people with congenital heart disease (CHD) experience psychological distress related to medical complications and psychosocial issues related to the disease. Although studies show that social support and coping strategies are closely associated with psychological distress in people struggling with different chronic health challenges, very little is known about whether the same factors hold true for the psychological distress of people living with CHD. The purpose of this study was to examine the relationships between types of social support, coping strategies, and psychological distress for individuals living with CHD. A cross-sectional survey design with self-report questionnaires was used. A convenience sample of 272 participants was obtained from the Adult Congenital Heart Disease program at a tertiary care hospital in Western Canada. Structural equation modeling was conducted to examine hypothesized relationships among study variables. Perceived social support was directly related to both anxiety and depression. Received social support influenced anxiety and depression, but its effect was through perceived social support. Wishful-thinking coping strategies mediated the relationships between perceived social support and both anxiety and depression. Individuals with CHD who have low perceived and received social support are vulnerable to experiences of psychological distress. Assessments of social support and facilitation of positive coping strategies are integral to nursing care for adults with CHD.

Contemporary ACHD training and the reality of the field in the United States

BackgroundCare delivery for the growing population of adults living with congenital heart disease (CHD) has been met with challenges due to a shortage of physicians trained to care for this population. To meet this urgent need, restructuring and standardization of the training programs were implemented in 2015. The consequences of such a system on the graduating fellows have not been examined. MethodsA 25-question electronic survey was distributed to early career physicians who graduated following training in adult CHD (ACHD) care between 2015 and 2017 and are currently practicing in the United States. The survey results were anonymous. ResultsOf the 30 physicians who trained in ACHD between 2015 and 2017 in the U.S., 21 (70%) responded to the survey. The majority completed a 2-year ACHD program, practice at an adult hospital, are happy with their current job, spend most of their time in ACHD-related activities, make on average around 250,000 USD for entry level positions, and prioritize supportive leadership and colleagues. Their training was adequate for their job requirements. However, the acquisition of an additional skill, in addition to clinical ACHD care, allowed them to secure a more ideal job. A sizeable number of jobs required program building or expansion with only 9.5% of trainees comfortable doing so immediately after graduation. ConclusionsThe new ACHD training curriculum successfully meets most of the needs for ACHD jobs. Integration of specialty tracks, ensuring uniformity in the quality of training between programs, and promoting leadership skills may improve career prospects.

Identifying self-reported neurocognitive deficits in the adult with congenital heart disease using a simple screening tool.

Children with congenital heart disease (CHD) and adults with acquired heart disease are at an increased risk of neurocognitive impairment. The objective of this study was to determine the prevalence of self-reported neurocognitive impairment and its risk factors in the adult congenital heart disease (ACHD) population. The Wisconsin Adult Congenital Heart Disease Program recently began screening ACHD patients to identify those with significant self-perceived neurocognitive impairments. Screening consists of using a validated neuro-oncology screening instrument that has been modified for the ACHD population. Patients who answer this survey in a predetermined fashion consistent with significant self-perceived neurocognitive deficits are referred for a formal neurocognitive evaluation. Demographic and clinical information are obtained by chart review. Three hundred ten patients (49% males) completed the screening process. The average age was 30years (range: 17-69years). For the cohort, 57 (18%) patients had no prior cardiac surgeries, 85 (28%) one surgery, 77 (25%) two, and 91 (29%) at least three surgeries. Of those screened, 106 (34%) met criteria for a formal neurocognitive evaluation. Patients who were referred had undergone a greater number of prior cardiac surgeries (2.2 vs 1.7, P = .008) and were more likely to have severe complexity CHD (P = .006). Of those patients who were referred, the worst perceived functioning was in math and attention. There is a high prevalence of ACHD patients with significant self-perceived neurocognitive deficits. Simple screening questionnaires may help identify those patients at high risk and allow for timely and appropriate referral for formal neurocognitive evaluation, diagnosis, and therapy.

Invasive and noninvasive hemodynamic assessment in adults with Fontan palliation

Background/objectivesAlthough echocardiographic-Doppler cardiac index (CI) assessment is widely used to guide heart failure management in patients with biventricular circulation, this application has not been studied in the Fontan population. The objective of this study was to: (1) determine the correlation between Doppler and cardiac catheterization CI calculation; (2) determine the association between Doppler CI and the occurrence of Fontan failure. MethodsRetrospective review of adult Fontan patients followed at Mayo Clinic Adult Congenital Heart Disease program, 1994–2015. Inclusion criteria were: systemic left ventricle and echocardiogram and cardiac catheterization performed within the same week. Fontan failure was defined as a composite of all-cause mortality, heart transplantation listing, and palliative care. Results59 patients (age 29±6years; men 32[54%]) underwent 97 studies. Of the 59, 41[69%] had atriopulmonary Fontan and 12 (20%) had cirrhosis. Compared to patients without cirrhosis, patients with cirrhosis had higher Doppler CI (3.6±0.6 vs 2.8±0.4L/min∗m2, p=0.039); Fick CI (3.3 [2.5–3.7] vs 2.4 [1.6–3.1] L/min/m2, p=0.028); lower systemic vascular resistance (20±3 vs 25±4 WU∗m2, p=0.04).There was a positive correlation between Doppler and Fick CI (r=0.52; p<0.0001). Fontan failure occurred in 13 patients (22%) within 7.5±2.1years. In patients without cirrhosis, Fick CI and Doppler CI <2.5L/min/m2 were associated with Fontan failure (odds ratio [OR] 1.58, p=0.046) and (OR 1.43, p=0.051) respectively. ConclusionsDoppler CI assessment in feasible in a selected group of Fontan patients and it is predictive of clinical outcomes. The application of this concept in systemic right ventricles deserves further research.

Transition of Care in Congenital Heart Disease: Ensuring the Proper Handoff

With great advances in medical and surgical care, most congenital heart disease patients are living in to adulthood and require lifelong surveillance and expert care for adult onset complications. Care lapse and lack of successful transfer from pediatric to adult care put young adults at risk for increased morbidity and premature death. Hence, transition and transfer from pediatric to adult care is a crucial and critical process to provide access to specialized care and lifelong surveillance. The aim of this article is to describe barriers to successful transition and transfer and to share practical strategies and concepts to overcome these barriers in order to successfully implement a transition program. There are patient-specific, local, and institutional specific barriers to establish a successful transition program which involves many stakeholders. Collaboration of the Pediatric and Adult Congenital Heart Disease programs is paramount; the understanding of the benefit and the need of a structured transition program, dedication, and a proactive approach are essential. Youth- and family-centered education improves healthcare knowledge, self-management, self-advocacy, and appropriate interdependence and helps young adults to take ownership of their health. Nurses play an integral role within the multidisciplinary team in supporting seamless, successful transition and transfer of CHD patients from pediatric to adult care thereby reducing loss to follow-up and lapses in care. Most experiences and recommendations are based on retrospective studies and expert consensus. It is imperative to evaluate the impact of structured and planned transition/transfer programs on the outcomes. Hence, prospective, randomized trials are required to document if implementation of structured intervention transition programs improve knowledge, patient experiences, and outcomes of congenital heart defect survivors.

Pattern of Adult Congenital Heart Disease in a Tertiary Care Hospital in Saudi Arabia

Background: As the growing number of patients with Congenital Heart Disease (CHD) is surviving into the adulthood, the availability of regional centers that specialize in the treatment and follow-up care of adults with CHD bears a great importance. The need is especially pronounced for the population in the Middle East where the defect may not be detected in timely manner. Methods: The clinical records of 30 patients referred to the Adult Congenital Heart Disease Program at a tertiary care hospital in Western Saudi Arabia were reviewed for the study. The patients were categorized into four groups based on their diagnoses: left to right shunt lesions; complex lesions; obstructive lesions; and arrhythmia. Following the diagnosis, four different type of treatments were provided to patients: 1) Interventional catheterization, 2) Surgery, 3) Medical treatment, or 4) No intervention. Results: The patients in the complex lesions group exhibited the most serious and the highest number of symptoms. Fifteen patients underwent the cardiac interventional catheterization, eleven patients underwent surgery, one patient was placed on a medical treatment, and one patient was deemed not to be in need of any treatment. Conclusion: Many of the patients with complex lesions were diagnosed relatively late in childhood and therefore had late first interventions. The regional facility equipped to early diagnose CHD and provide optimal treatment would increase patients’ quality of life and reduce the ultimate health care costs. An awareness of the importance of the early diagnosis and treatment on the part of the primary physician, along with the education of patients and family affected with congenital heart disease on the importance of continued follow-up care in the center that specializes in adult congenital heart disease cannot be over-emphasized.

Pregnancy causes a transient decrease in systemic ventricular function in women with pre-existing cardiac disease

Pregnancy is known to induce significant hemodynamic changes. The purpose of our study was to determine the effects of these hemodynamic changes during pregnancy on systemic ventricular function (SVF) in women with pre-existing cardiac disease.A retrospective review of women followed in the Wisconsin Adult Congenital Heart Disease program with a concurrent pregnancy from 2006 to 2015 was conducted. The SVF was measured in a baseline echocardiogram (obtained ≤12months prior to the estimated dated of conception (EDC)), an echocardiogram during pregnancy, and a post-partum echocardiogram. To verify stability of the SVF in the years prior to pregnancy, SVF was also measured on a control echocardiograms (obtained >12months prior to EDC) and compared to the baseline echocardiogram. Systemic ventricular function was assessed in each echocardiogram by measuring global peak longitudinal strain (GPLS) and ejection fraction (EF).A total of 46 pregnancies were included. The average age at the EDC was 25.7±5years and the median gestational age at time of delivery was 38.0weeks. The most common cardiac anomaly was coarctation of the aorta (26.1%) followed by tetralogy of Fallot (13%). The mean GPLS for the control echocardiograms, baseline echocardiograms, pregnancy echocardiograms and post-partum echocardiograms were as follows: −18.9±2.5, −18.0±3.4%, −15.9±2.9% and −17.9±3.6%. When measuring GPLS there was a significant drop in SVF during pregnancy (0=0.04) with a return to baseline in the post-partum period. When SVF was measured by EF there was a strong trend toward a significant drop in SVF during pregnancy (p=0.08).Our results show there is a significant drop in systemic ventricular function during pregnancy in women with pre-existing cardiac disease when measured by GPLS, which returns to baseline in the post-partum period.

An Integrated Adult Congenital Heart Disease Psychology Service.

North American adults with congenital heart disease (CHD) are at increased risk of mood and anxiety disorders, although there are no published data on psychological treatment. In order to provide guidance to adult CHD (ACHD) programs considering the integration of psychological care, the aim of this study was to provide a comprehensive description of a specialized ACHD psychological service. At a large tertiary hospital, a retrospective review of 100 ACHD clinic patient psychology files was performed. The following data were abstracted: sociodemographic and medical background, presenting psychological concerns, and the course and outcome of psychological intervention. Of 100 patients, the mean age was 33 ± 11 years and 51% were female. Prevalent psychological concerns were generalized anxiety (82%), health-/heart-related anxiety (71%), depressed mood (60%), and difficulty coping with a medical condition (49%); 65% of patients met diagnostic criteria for a mood or anxiety disorder. Following assessment, individual psychotherapy was offered to 87 patients, of whom 75 opted to proceed with treatment. The median number of psychotherapy sessions was 8 and treatment most frequently included cognitive therapy (92%), relaxation skills training (57%), and communication skills training (46%). Of 64 patients for whom a course of psychotherapy had ended at the time of data abstraction, 54 (88%) had reduced or absent psychological distress. Reduced psychological distress can be achieved among adults with CHD who receive targeted psychological intervention. Consistent with the emerging field of behavioral cardiology, other ACHD programs as well as general cardiovascular programs are encouraged to integrate cardiac psychology services in order to provide comprehensive patient care.

Fellows' perspectives on training in adult congenital heart disease: Results of a survey from the International Society for Adult Congenital Heart Disease (ISACHD)

a Department of Cardiology, Academic Medical Center Amsterdam, Amsterdam, the Netherlands b Interuniversity Cardiology Institute of the Netherlands, Utrecht, Netherlands c Adult Congenital Heart Disease Program, Knight Cardiovascular Institute, Oregon Health & Science University, Portland, OR, USA d Congenital Cardiac Centre for Adults, University Health Network, University of Toronto, Toronto, ON, Canada e Montreal Heart Institute and Universite de Montreal, Montreal, QC, Canada f Department of Cardiology, Heart Institute Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA

Electronic medical record integration with a database for adult congenital heart disease: Early experience and progress in automating multicenter data collection

BackgroundThe adoption of electronic health records (EHR) has created an opportunity for multicenter data collection, yet the feasibility and reliability of this methodology is unknown. The aim of this study was to integrate EHR data into a homogeneous central repository specifically addressing the field of adult congenital heart disease (ACHD). MethodsTarget data variables were proposed and prioritized by consensus of investigators at five target ACHD programs. Database analysts determined which variables were available within their institutions' EHR and stratified their accessibility, and results were compared between centers. Data for patients seen in a single calendar year were extracted to a uniform database and subsequently consolidated. ResultsFrom 415 proposed target variables, only 28 were available in discrete formats at all centers. For variables of highest priority, 16/28 (57%) were available at all four sites, but only 11% for those of high priority. Integration was neither simple nor straightforward. Coding schemes in use for congenital heart diagnoses varied and would require additional user input for accurate mapping. There was considerable variability in procedure reporting formats and medication schemes, often with center-specific modifications. Despite the challenges, the final acquisition included limited data on 2161 patients, and allowed for population analysis of race/ethnicity, defect complexity, and body morphometrics. ConclusionLarge-scale multicenter automated data acquisition from EHRs is feasible yet challenging. Obstacles stem from variability in data formats, coding schemes, and adoption of non-standard lists within each EHR. The success of large-scale multicenter ACHD research will require institution-specific data integration efforts.

Congenital heart disease in the adult: what should the adult cardiologist know?

There is an increasing population of adults with congenital heart disease (CHD) due to successful pediatric medical and surgical intervention, and commensurate with this increase is a rise in heart failure, hospital admissions, and hospital costs among adult CHD patients. This group of patients requires careful long-term evaluation and follow-up of the residua and sequelae of their cardiac anomalies that arise in adulthood to prevent late complications. This article addresses congenital heart defects that are encountered in a general adult cardiology practice and reviews clinical, anatomic, and imaging features of each lesion, fundamental management issues, indications for interventions (and often re-interventions), issues related to endocarditis prophylaxis, pregnancy, and appropriateness of referral to a dedicated adult CHD program for long-term care.

Current and Future Status of Adult Congenital Training in North America

Adult congenital heart disease (ACHD) presents a unique and growing patient population, but there are no data to suggest there are sufficient training programs available to train specialists to meet the needs of this population. The objective of this national survey was to determine the current status of ACHD fellowship curricula and training. An online questionnaire was provided to adult and pediatric cardiology fellowship programs to determine program demographics, status, duration, faculty teaching responsibilities, volume or patient exposure, and institutional obstacles. Of the 225 programs surveyed, 81 responded (36%). Nearly all were university affiliated (93%) with <1/3 (29%) reporting an ACHD fellowship program. Most offered either a 1- (42%) or a 2-year (47%) fellowship. ACHD fellowship funding was provided by hospital budget (68%). Half reported an increase in applicants to their fellowship. Applicants were more likely to have had previous training in adult cardiovascular medicine (56%). Respondents ranked "Volume of Outpatients/Year" and "Lack of Institutional Support" as the most important aspect of an ACHD program to applicants and greatest obstacle to ACHD training, respectively. After training, ACHD fellows obtained adult cardiology/ACHD positions (47%) and were within an academic center (88%). In conclusion, the results demonstrate a deficiency in the number of currently available ACHD fellowship programs. Measures should be taken to strengthen and standardize ACHD training to meet the increasing workforce requirements of this population.