Are missed appointments in an outpatient clinic for adults with congenital heart disease the harbinger for care gaps?

AIMS: Life-long follow-up is vital for patients with congenital heart disease (CHD) to safeguard longevity. Missing scheduled appointments might be prodromal to subsequent care gaps, but our understanding of their occurrence and impact is limited. This study determined the occurrence and predictors of missed appointments (MA), assess its predictive value for care gaps, and explored if MA or care gaps are associated with increased mortality. METHODS AND RESULTS: From 2007 to 2009, the occurrence of MA in 2075 CHD patients was documented at an adult CHD clinic. The frequency of outpatient visits in 1363 adults with moderate to complex CHD was calculated from 2009 to 2012. Patients without a visit were considered to have a care gap. Data on mortality were collected from 2013 to 2017. Missed appointments occurred in 11% of patients and were more common in men [odds ratio (OR) = 1.57; 95% confidence interval (CI): 1.18-2.08], without a history of cardiac procedures (OR = 1.46; 95% CI: 1.08-1.97), and for morning visits (OR = 1.45; 95% CI: 1.10-1.92). Care gaps were identified in 6% of moderate to complex CHD. A MA was significantly associated with an increased likelihood of care gaps (OR = 19.55; 95% CI: 11.92-32.07; R2 = 26.5%). In moderate to complex CHD, no difference in mortality rates was related to the occurrence of discontinued care. CONCLUSION: The occurrence of MA was related to patients' gender, no history of cardiac interventions, and time of day of the outpatient visits. Care gaps were associated by the preceding MA. No association with increased mortality rates was observed. Interventions reducing missed appointments, such as SMS-reminders prior to visits, need investigation.

Recall of patients discharged from follow-up after repair of isolated congenital shunt lesions.

BACKGROUND: Discharge from follow-up after closure of isolated congenital shunt lesions in childhood was common practice in the past. The aim of the present study was to recall these patients to evaluate their current status. METHODS: Patients included in the database of pediatric and congenital heart disease of our tertiary center with repaired secundum atrial septal defect (ASD) or ventricular septal defect (VSD) before the age of 18years, and discharged from follow-up, were invited for clinical and echocardiographic check-up. RESULTS: Forty-six ASD patients (age 30±7years, 37% male) responded. Median age at ASD repair was 6 (IQR 4-8) years. All but one functioned in NYHA class I. Eight (17%) patients reported palpitations. No patient developed pulmonary hypertension (PH). Right ventricular (RV) dilatation was present in 7 (15%). RV fractional area change (FAC) was <35% in 7 (15%), TAPSE <17mm in 12 (26%). Forty-seven VSD patients (age 34 (IQR 29-40) years, 57% male) participated. Median age at VSD repair was 4 (IQR 1-5) years. Six (13%) patients functioned in NYHA class II. Seventeen (36%) patients reported palpitations. Four (9%) patients presented PH. Left ventricular dilatation was present in 4 (9%), RV dilatation in 6 (13%). RV FAC was <35% in 7 (15%), TAPSE <17mm in 17 (36%). Seven (15%) patients had dilated ascending aorta. CONCLUSIONS: Patients with closure of isolated secundum-type ASD in childhood do well, but some have persistent RV dilatation and dysfunction. By contrast, more patients after VSD closure were symptomatic and presented with RV dilatation and dysfunction, PH, and a dilated ascending aorta.

Effectiveness of structured education on knowledge and health behaviors in patients with congenital heart disease.

OBJECTIVE: To investigate whether a single educational session increased the level of knowledge and changed the prevalence of health risk behaviors in young people with congenital heart disease (CHD). STUDY DESIGN: We conducted a longitudinal study of patients transferred to adult CHD care who received a single educational session (n = 201) at a tertiary care center. Their knowledge level and prevalence of health risk behaviors were assessed via the Leuven Knowledge Questionnaire CHD and the Health Behavior Scale CHD, respectively. A general linear model for longitudinal measurements was used to analyze the natural progression of patients' knowledge during a 27-month period and the effect of one educational session on outcomes. RESULTS: Participating in an educational session resulted in a small-to-moderate, but significant, increase in total knowledge level and better understanding of deterioration symptoms, and rationale and frequency of follow-up; however, it did not improve patients' health behaviors. CONCLUSIONS: This type of education did improve knowledge but did not improve the patients' tendency to engage in better health behaviors. Future studies should assess the effect of repetitive exposure to educational sessions dealing with CHD.

Effectiveness of structured patient education on the knowledge level of adolescents and adults with congenital heart disease.

BACKGROUND: Patients with congenital heart disease (CHD) have poor understanding of their heart condition, treatment and prevention of complications. To improve their level of health-related knowledge, a structured education program was implemented in the adult congenital heart disease program. This study aimed (a) to evaluate the level of knowledge of patients who received structured CHD education as compared to patients who did not receive this education; (b) to explore if the provision of structured education is an independent determinant of knowledge; and (c) to evaluate whether patients who received structured education reached the educational target (>80% correct answers). METHODS AND RESULTS: A total of 317 patients were included: 226 in the education group, and 91 in the comparison group. Knowledge was assessed using the 'Leuven Knowledge Questionnaire for Congenital Heart Disease'. The mean total knowledge score in the education group (57%) was significantly higher as compared to the comparison group (43%) (p<0.001). However, only 24 patients (11%) in the education group reached the educational target of the program. After adjusting for patient's age, educational level and disease complexity, hierarchical multivariable linear regression analysis showed that the provision of structured CHD education was an independent determinant of higher levels of knowledge. CONCLUSION: A structured education program was associated with a higher level of knowledge. However, the educational target for sufficient knowledge was reached in a very limited number of patients. Hence, continuous efforts in educating patients and developing alternative education methods are needed.

Transfer of adolescents with congenital heart disease from pediatric cardiology to adult health care: an analysis of transfer destinations.

OBJECTIVES: The transfer of adolescents with congenital heart disease from pediatric to adult care was examined. The aims were to investigate where these adolescents received adult-centered care, to determine the proportion of patients with no follow-up and with no appropriate follow-up after leaving pediatric cardiology, and to explore the determinants of no follow-up and no appropriate follow-up. BACKGROUND: Even after successful treatment, many patients require lifelong cardiac surveillance by specialized practitioners. Although guidelines describe the most appropriate level of follow-up, this is not always implemented in practice. METHODS: A descriptive, observational study was performed, including 794 patients with congenital heart disease examined and/or treated at a tertiary care center. RESULTS: Overall, 58 of the 794 patients included (7.3%) were not in follow-up. Cessation of follow-up was found in 2 of 74 patients with complex (2.7%), 31 of 448 patients with moderate (6.9%), and 25 of 272 patients with simple (9.2%) heart defects. Moreover, 684 patients (86.1%) remained in specialized follow-up. According to international guidelines, 81 patients (10.2%) did not receive the minimal level of cardiac care. Multivariable logistic regression revealed that male sex and no prior heart surgery were associated with no follow-up. Male sex, no prior heart surgery, and greater complexity of congenital heart disease were associated with no appropriate level of cardiac follow-up. CONCLUSIONS: The proportion of patients in this study lost to follow-up was substantially lower than in other Western countries. Because only patient-related factors were examined with respect to loss to follow-up, further examination of patient-related, hospital-related, and healthcare-related determinants of lack of follow-up is needed.

Patient knowledge of and adherence to oral anticoagulation therapy after mechanical heart-valve replacement for congenital or acquired valve defects.

OBJECTIVE: This study sought to determine (1) the level of knowledge that mechanical heart-valve patients (because of congenital heart disease or acquired heart-valve defects) have about oral anticoagulation therapy; and (2) to what extent these patients adhere to this therapy. METHODS: This descriptive, cross-sectional study included 57 patients. Knowledge was measured using the Knowledge of Oral Anticoagulation Tool. Adherence was assessed with a visual analogue scale and the Swiss HIV Cohort Study Adherence Questionnaire. RESULTS: Patients poorly understood symptoms relevant to over-anticoagulation and the effects of alcohol and vitamins on oral anticoagulants. The knowledge level of patients with congenital heart disease and acquired heart-valve defects did not differ significantly. Three-quarters of patients claimed to be 100% adherent to oral anticoagulant therapy. CONCLUSION: Most patients lack knowledge about oral anticoagulants, and one fourth of patients do not fully adhere to therapy.

Adolescents' understanding of their congenital heart disease on transfer to adult-focused care.

Adolescents with congenital heart disease (CHD) must take responsibility for their life and care. This requires that they have sufficient knowledge about their heart disease, treatment, and preventive measures. Thus, CHD-related education should be directed to adolescents. Research on adolescents' understanding and knowledge of CHD is limited. It is unknown what adolescents with CHD know about their heart defect, treatment, and preventive measures necessary to avoid complications. We addressed these questions in a descriptive cross-sectional study of 91 adolescents with CHD (53% males; median age 17 years). In the present study, we assessed the subjects' knowledge of CHD using the Leuven Knowledge Questionnaire for Congenital Heart Disease. The results showed that the patients had adequate knowledge (>80% correct answers) about the need for regular follow-up, their required diet, past treatment, and dental practices. They had moderate knowledge (50% to 80% correct answers) about the frequency of follow-up, occupational choices, medication regimen, and sexual activities. However, the patients had poor knowledge (<50% correct answers) of the name of their heart defect; the reasons for follow-up; the effects of competitive sports; the symptoms that reflect deterioration of their heart disease; the definition, characteristics, and risk factors of endocarditis; the possibility of recurrent episodes of endocarditis during their lifetime; the effect of smoking and alcohol on their heart disease; the hereditary nature of their condition; the suitability of intrauterine devices as contraceptives; the appropriateness of oral contraceptives; and the risks of pregnancy. In conclusion, the results of the present study have showed that the level of knowledge of adolescents with CHD has significant gaps.

Behavior of Ebstein's anomaly: single-center experience and midterm follow-up.

OBJECTIVES: Ebstein's anomaly, characterized by an apical displacement of the tricuspid valve into the right ventricle, occurs in approximately 1/200,000 live births. Because long-term follow-up data of adults with Ebstein's anomaly are scarce, we evaluated the outcome of our Ebstein's anomaly patients. METHODS: All patients >16 years and registered in the congenital heart disease database of our hospital with isolated Ebstein's anomaly were selected for the study. Records were reviewed for outcome. RESULTS: Forty-nine patients (21 males, mean age at diagnosis 29.1 ± 20.7 years) were followed for a mean time of 11.4 years (range 1.1-32.4). Twenty-five patients (51%) underwent tricuspid valve surgery (16 valvuloplasty and 9 valve replacement). Eight patients (32%) required redo tricuspid valve surgery. Twenty-six patients (52.1%) exhibited supraventricular arrhythmia, and the typical Wolff-Parkinson-White syndrome occurred in 15 patients (31.2%). Seventeen patients (34.7%) underwent ablation therapy and 5 patients (10.4%) required pacemaker implantation. CONCLUSIONS: Half of the patients with Ebstein's anomaly needed tricuspid valve surgery and redo surgery was not uncommon. Supraventricular arrhythmia occurred frequently and ablation therapy was often indicated. Careful follow-up is obligate, as some complications occur for the first time in adulthood.

Prevention of stroke by percutaneous left atrial appendage closure: short term follow-up.

Preliminary studies suggest that left atrial appendage (LAA) closure might be beneficial in the prevention of stroke in patients with atrial fibrillation (AF). We evaluated the preliminary clinical efficacy of percutaneous LAA closure using the PLAATO-device (ev3 Endovascular, Inc., North Plymouth, MN) and found that none of the patients suffered from thrombo-embolic events within a time period of 2 years after successful LAA closure. Therefore, we believe that percutaneous closure of the LAA might be a valuable technique for AF patients with contra-indications for or failure of oral anticoagulation to prevent thrombo-embolic events.

Attitude toward and current practice of transfer and transition of adolescents with congenital heart disease in the United States of America and Europe.

The objective of this study was to explore the current practice and attitudes of pediatric cardiologists in the United States and Europe on the transfer and transition of children with congenital heart disease (CHD). A survey among pediatric cardiology programs in the United States and Europe was undertaken. Sixty-nine centers completed and returned the 61-item questionnaire that was specifically devised for this survey. Of 69 participating centers, 74% reported that they transfer their patients to adult-focused care. When a center transfers its patients, 80% transfer them to a formalized Adult Congenital Heart Disease Program. The median age of transfer is 18 years. Comorbidities, pregnancy, and patient/family request to leave pediatric cardiology were identified as initiators for transfer. Complexity of the heart defect was relatively less important when deciding whether to transfer patients. Only one-third of the centers that transfer their patients provide a structured preparation for patients and family. Development of a formal transition program is planned at 59% of the centers that transfer patients. In conclusion, timely transfer and a structured transition process of children with CHD are not implemented in all pediatric cardiology programs. Health-care providers working in pediatric cardiology should make their transfer policies explicit and transition programs ought to be developed.

Expectations and experiences of adolescents with congenital heart disease on being transferred from pediatric cardiology to an adult congenital heart disease program.

PURPOSE: To investigate the expectations and experiences of adolescents on transferring from a pediatric cardiology program to an adult congenital heart disease program. METHODS: A qualitative, phenomenologic study was conducted, in which semi-structured, in-depth interviews were performed with 14 adolescents (aged 15-17 years) with congenital heart disease. Interviews were tape-recorded and transcribed verbatim. Data were analyzed using procedures described by Colaizzi. RESULTS: The study identified six themes that characterized adolescents' views of the transfer process: leaving pediatric cardiology was viewed as normal; leaving behind familiar surroundings; a positive wait-and-see attitude toward the adult congenital heart disease program; adjusting to a new environment; a need for better information; and a shift in roles between the adolescent and his parents. CONCLUSION: Although adolescents with congenital heart disease have a positive attitude toward transferring to an adult congenital heart disease program, they identified aspects of the transfer that healthcare professionals need to address. Patients and their families should be prepared for the transfer by informing them about the adult program and its healthcare providers. A formal transition program can play a role in this respect.

Behaviour of polyester grafts in adult patients with repaired coarctation of the aorta.

AIMS: Whatever the technique used for surgical or endovascular repair of a coarctation of the aorta (CA), long-term complications might occur. Aneurysm formation after patch angioplasty is not uncommon and may lead to a life-threatening condition. Therefore, we were interested in the long-term results of different types of tube grafts, from which a lower degree of dilatation is expected. METHODS AND RESULTS: All patients, currently older than 16 years, who underwent (redo) surgery for CA, and in whom a tube graft was inserted, were selected from the database of congenital heart disease of our hospital. Follow-up data were collected by reviewing the patients' files. The degree of graft dilatation was calculated for each patient. Fifty-three patients (41 males, median age 33.1 years, Q1-Q3 29.9-40.2 years) could be included in the study, in which 20 (38%) 'Gelseal', 12 (23%) 'Gelsoft', 8 (15%) 'Gelweave', and 13 (24%) older types of grafts were used. Twenty patients underwent a primary repair and in all others a tube graft was implanted after a previous patch angioplasty. The median graft diameter (manufacturer size) at implantation was 20 mm (Q1-Q3 16-22 mm). The median follow-up time of the grafts was 13.1 years (Q1-Q3 9.0-17.2 years). The graft size increased to a median value of 26 mm (Q1-Q3 22-30 mm) (median 50% increase in diameter, range 0-271%, P < 0.0001). The diameter of six grafts remained unchanged during follow-up. Three deaths occurred, of whom two were cardiac-related. False aneurysms occurred in four patients, graft aneurysm in two, endarteritis in two, and graft stenosis in one. CONCLUSION: Nearly, all tube grafts dilated up to 50% of the manufacturer diameter during follow-up. Re-interventions were inevitable in more than 10 per cent of the cases, primarily because of (false) aneurysm formation. Our series illustrates that late complications are not uncommon, so that rigorous follow-up of these CA patients remains mandatory.

Profile of adults with congenital heart disease having a good, moderate, or poor quality of life: a cluster analytic study.

AIMS: The purpose of the study was to assess the profile of adult patients with congenital heart disease who reported a good, moderate, or poor quality of life. METHODS: We conducted a secondary analysis of data from a large-scale quality-of-life study that included 627 patients. Demographic and clinical variables were retrieved from the medical records and functional status from patient interviews. Overall quality of life was measured using a Linear Analogue Scale. Using K-means cluster analysis, we categorized subjects into a 3-cluster solution: good, moderate, or poor quality of life. RESULTS: Four hundred ninety patients (78.1%) clustered into the good quality-of-life category; 126 patients (20.1%) clustered into the moderate quality-of-life category; and 11 patients (1.8%) clustered into the poor quality-of-life category. Poorer quality of life was associated with lower educational level, unemployment or disability, associated syndromes, instability of the heart disease, and a poorer functional status. CONCLUSION: Over three-quarters of the patients had a good quality of life, whereas only a small proportion had a poor quality of life. Specific demographic and clinical characteristics associated with a poor quality of life could assists in identifying patients at risk for developing a poor quality of life.

A modified technique of stent fenestration of the interatrial septum improves patients with pulmonary hypertension.

AIMS: A significant number of patients with pulmonary hypertension are resistant to medical therapy. We wanted to evaluate whether the modified technique of stent fenestration of the interatrial septum would be feasible and safe, and offer clinical benefit. METHODS AND RESULTS: The medical records of all patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension who underwent a stent fenestration of the interatrial septum between 2001 and 2008 were reviewed. In all fifteen patients (12 female, mean age 48.2 +/- 20.5 years) a successful fenestration procedure could be performed. Median follow-up time between diagnosis and fenestration was 2.3 years (range from 0.5 to 18.6 years). Mean event free survival since diagnosis and after septostomy was 9.8 +/- 2.9 and 3.2 +/- 0.8 years, respectively. When one extreme outlier was excluded, the 6 min walk distance improved significantly from 309 +/- 69 m immediately before fenestration to 374 +/- 84 m, 3-4 months after fenestration (n = 8, paired t-test, P = 0.03). No stent occlusion occurred. CONCLUSION: The modified stent fenestration technique is feasible and safe in patients with severe pulmonary hypertension. In a selected group of patients, functional capacity might improve although disease progression continues.

Implementation of transition programs can prevent another lost generation of patients with congenital heart disease.

Congenital heart disease is the most frequently occurring birth defect. To date, more than 90% of the children born with a heart defect reach adulthood. Since many patients are prone to residua and sequelae, lifelong specialized care is required. However, studies indicate that about one-half to three-quarters of the patients are lost to follow-up when they have grown up. This has resulted in a virtual lost generation. Lapse of care is associated with significant morbidity. Therefore, implementation of strategies to prevent patients from failing to continue regular follow-up is critical. It is argued that transition programs that inform patients about the rationale for ongoing follow-up and that teach them how to navigate the medical system can avoid another lost generation.

Percutaneous transcatheter ventricular septal defect closure in adults with Amplatzer septal occluders.

BACKGROUND: The interest in transcatheter ventricular septal defect (VSD) closure is continuously growing. Therefore, we report our experience in perimembranous (Pm) and postinfarction (Pi) VSD closure. METHODS: All patients, older than 16 years, were selected from a data base, in which Pm and Pi VSDs were registered. The patients' files were reviewed until the most recent follow-up date. RESULTS: Nine (7 male, 37.4 +/- 12.8 y) and 8 (6 male, 76.3 +/- 6.2 y) patients underwent a Pm (group A) and Pi VSD (group B) closure, respectively. One female patient was treated for a posttraumatic VSD (26 y). In group A, 7 patients were closed with the Amplatzer perimembranous VSD occluder, one with the muscular VSD occluder, and one patient with the atrial septal defect occluder. All patients in group B were treated with the muscular VSD occluder. In the post-traumatic VSD an Amplatzer patent foramen ovale occluder was used. Device implantation was feasible in all, except in two patients with extremely large VSDs (one Pm and one PiVSD). Total transcatheter closure or small residual leakage was achieved in 7/8 patients in group A, but one patient needed surgical VSD repair because of massive haemolysis, another patient died 9 months later. A small or moderate shunt was present immediately after the procedure in all patients of group B. No device-related complications were reported, but all, except one patient, died within 2 weeks after the procedure because of an extremely high co-morbidity (logistic Euroscore 70 +/- 25%). Total closure was achieved in the post-traumatic VSD. CONCLUSION: Transcatheter closure of Pm and Pi VSD with Amplatzer septal occluders in adults is feasible and safe, but the post-procedural prognosis totally depends on the aetiology of the VSD and its co-morbidity.

Homograft survival after tetralogy of Fallot repair: determinants of accelerated homograft degeneration.

AIMS: Homografts are frequently implanted in patients with tetralogy of Fallot (TOF). However, the lifespan of homografts is shorter than that of graft recipients, thus making surgical re-intervention unavoidable. Therefore, to determine variables that could influence their survival, we retrospectively studied the survival pattern of homografts used to treat TOF. METHODS AND RESULTS: Sixty-eight TOF patients, >14 years of age (mean age: 34 +/- 11; 71% male), were selected from our database of congenital cardiology cases. These patients underwent their first homograft implantation at a median age of 24 years (range: 14-49). The primary endpoint, homograft failure, was defined as homograft replacement or percutaneous balloon dilatation when the echocardiographic gradient reached more than 50 mmHg. Kaplan-Meier analysis revealed that the mean event-free survival time of first homografts was 14.6 years (CI, 12.9-16.2 years). The median increase in the homograft gradient was 1.1 mmHg/year (range: 0.0-22.1) for a median follow-up time of 8.4 years (range: 1.3-17.9). Stepwise regression analysis identified the homograft gradient at 1 month after surgery to be prognostic for homograft degeneration (R(2) = 0.23, beta = 0.26, P = 0.001). Immunological variables, gender, and post-operative inflammatory indicators were unrelated to the degree of homograft gradient increase. Finally, patient age at the time of first homograft implantation and previous palliative surgery was significantly associated with the gradient at 1 month (Spearman's rho = -0.41 and -0.29, respectively; P = 0.004 and 0.048, respectively). CONCLUSION: Homograft survival in patients with TOF repair is quite good. However, some patients develop accelerated homograft degeneration. We found that the gradient of the homograft 1 month after surgery is most indicative of accelerated homograft degeneration. We hypothesize that mechanical, not immunological, factors play an important role in homograft degeneration.