Type D Personality Associated With Increased Risk for Mortality in Adults With Congenital Heart Disease

Background Type D personality has been previously shown to increase the risk for mortality in patients with acquired heart disease.ObjectiveWe aimed to compare mortality in adult patients with congenital heart disease (CHD) with and without type D.Methods Survival was assessed using prospective data from the Dutch national Congenital Corvitia registry for adults with CHD. Patients were randomly selected from the registry and characterized at inclusion in 2009 for the presence of type D using the DS14 questionnaire.Results One thousand fifty-five patients, with 484 (46%) males, a mean (SD) age of 41 (14) years, 613 (58%) having mild CHD, 348 (33%) having moderate CHD, and 94 (9%) having severe CHD, were included. Type D personality was present in 225 patients (21%). Type D was associated with an increased risk for all-cause mortality independent of age, sex, New York Heart Association class, number of prescribed medications, depression, employment status, and marital status (hazard ratio, 1.94; 95% confidence interval, 1.05–3.57; P = .033).Conclusion Type D personality was associated with an increased risk for all-cause mortality in adult patients with CHD

An implantable loop recorder or smartphone based single-lead electrocardiogram to detect arrhythmia in adults with congenital heart disease?

BackgroundThe European Society of Cardiology (ESC) guidelines for the management of adult congenital heart disease (ACHD) recommend screening in patients at risk for arrhythmic events. However, the optimal mode of detection is unknown.MethodsBaseline and follow-up data of symptomatic ACHD patients who received an implantable loop recorder (ILR) or who participated in a smartphone based single-lead electrocardiogram study were collected. The primary endpoint was time to first detected arrhythmia.ResultsIn total 116 ACHD patients (mean age 42 years, 44% male) were studied. The ILR group (n = 23) differed from the smartphone based single-lead electrocardiogram group (n = 93) in having a greater part of males and had more severe CHD and (near) syncope as qualifying diagnosis. In the smartphone based single-lead electrocardiogram group history of arrhythmia and palpitations were more frequent (all p < 0.05). Monitoring was performed for 40 and 79 patient-years for the ILR- and smartphone based single-lead electrocardiogram group, respectively. Arrhythmias occurred in 33 patients with an equal median time for both groups to first arrhythmia of 3 months (HR of 0.7, p = 0.81). Furthermore, atrial fibrillation occurred most often (n = 16) and common therapy changes included medication changes (n = 7) and implantation of pacemaker or Implantable Cardioverter Defibrillator (ICD) (N = 4). Symptoms or mode of detection were not a determinant of the first event.ConclusionNon-invasive smartphone based single-lead electrocardiogram monitoring could be an acceptable alternative for ILR implantation in detecting arrhythmia in symptomatic ACHD patients in respect to diagnostic yield, safety and management decisions, especially in those without syncope

How to initiate eHealth in congenital heart disease patients?

Patients with congenital heart disease (CHD) are a vulnerable subgroup of cardiac patients. These patients have a high morbidity and high mortality rate. As the number of patients with CHD keeps growing, while also getting older, new tools for the care and follow-up of these vulnerable patients are warranted. eHealth has an enormous potential to revolutionize health care, and particularly for CHD patients, by expanding care beyond hospital walls and even moving some of the provided care to the comfort of home. As new eHealth tools continue to grow in number, such as invasive eHealth tools, health care delivered through eHealth continues to evolve. This teaching series summarizes current insights and discusses challenges yet to be overcome. Importantly, none of them are insurmountable. This all lays ground for a promising future for eHealth in the care of patients with CHD

A successful crowdfunding project for eHealth research on grown-up congenital heart disease patients

Background: Scarce data on crowdfunding report a maximal funding of €10.000,-, and state that research is needed to attract attention of larger granting organizations. The aims of this project were 1) to fund an eHealth study in grown-up congenital heart disease (GUCH) patients 2) to contemplate on critical success factors. Methods: After peer review of the Dutch Heart Foundation a project was published at a donation platform, which was open for donations during a predetermined period of two months. Copywriters were hired to create an easy-to-understand message to donors. A video teaser was created with a motivated patient, and rewards were available. The crowdfunding targeted €25.000 and the Dutch Heart Foundation doubled the donations to €50.000, and return of donations were guaranteed in case this was not met. Results: Initially, donations came from the investigators' private inner circle. In total, 44 potential donors were contacted, but refused to donate originally. Multiple (social) media campaigns were published to promote the project, and an offline mailing was sent to contributors to the Dutch Heart Foundation. During the project support emerged, resulting in extra donations and public awareness. In the lasts three weeks, after sufficient private donations, five major donors decided to support the project. The project became a big success: the predetermined target was exceeded and a total of €74.450,- was raised. Conclusion: Innovative crowdfunding gave the opportunity to start eHealth research in GUCH patients. Critical success factors include support of a professional organization, support of stakeholders, and easy-to-understand messages

22q11.2 deletion syndrome is associated with increased mortality in adults with tetralogy of Fallot and pulmonary atresia with ventricular septal defect

Background: 22q11.2 Deletion syndrome (22q11.2DS) is common in patients with tetralogy of Fallot (TOF) or pulmonary atresia with ventricular septal defect (PA/VSD) and is associated with worse outcomes in children. Whether this impaired prognosis is also translated into adulthood is unknown, as data in adult patients are limited. We aimed to compare long-term outcomes in adults with TOF or PA/VSD both with and without 22q11.2DS. Methods: This study prospectively followed a nationwide multicenter cohort of TOF or PA/VSD patients with genetically confirmed presence or absence of 22q11.2DS, from inclusion in the Dutch national CONCOR registry for adults with congenital heart disease (CHD) onward. Outcome measures included all-cause mortality, cardiac mortality, need for pulmonary valve replacement (PVR), ventricular arrhythmias (VA), pacemaker implantation, and ICD implantation. Results: In total, 479 patients were included (277 (58%) male, median age 28 [IQR; 21–37] years, 62 (13%) with PA/VSD, 34 (7%) with 22q11.2DS). During a median follow-up of 11 [IQR; 6–13] years, 52 (11%) patients died (8 with 22q11.2DS and 44 without 22q11.2DS). Patients with 22q11.2DS had significant decreased survival after 12 years (76% [95% CI; 62–93]) compared to patients without 22q11.2DS (89% [95% CI; 86–92], p = 0.008). 22q11.2DS was associated with increased risk of all-cause mortality and cardiac-mortality, independent of age, sex, and PA/VSD. No association was found between 22q11.2DS and late complications i.e. PVR, VA, pacemaker, or ICD implantation. Conclusions: Adults with TOF or PA/VSD with 22q11.2DS have a significantly worse survival than adults without this deletion. In patients with TOF or PA/VSD, genetic analysis for the presence of 22q11.2DS is important for risk stratification and genetic counseling

An implantable loop recorder or smartphone based single-lead electrocardiogram to detect arrhythmia in adults with congenital heart disease?

Background: The European Society of Cardiology (ESC) guidelines for the management of adult congenital heart disease (ACHD) recommend screening in patients at risk for arrhythmic events. However, the optimal mode of detection is unknown. Methods: Baseline and follow-up data of symptomatic ACHD patients who received an implantable loop recorder (ILR) or who participated in a smartphone based single-lead electrocardiogram study were collected. The primary endpoint was time to first detected arrhythmia. Results: In total 116 ACHD patients (mean age 42 years, 44% male) were studied. The ILR group (n = 23) differed from the smartphone based single-lead electrocardiogram group (n = 93) in having a greater part of males and had more severe CHD and (near) syncope as qualifying diagnosis. In the smartphone based single-lead electrocardiogram group history of arrhythmia and palpitations were more frequent (all p < 0.05). Monitoring was performed for 40 and 79 patient-years for the ILR- and smartphone based single-lead electrocardiogram group, respectively. Arrhythmias occurred in 33 patients with an equal median time for both groups to first arrhythmia of 3 months (HR of 0.7, p = 0.81). Furthermore, atrial fibrillation occurred most often (n = 16) and common therapy changes included medication changes (n = 7) and implantation of pacemaker or Implantable Cardioverter Defibrillator (ICD) (N = 4). Symptoms or mode of detection were not a determinant of the first event. Conclusion: Non-invasive smartphone based single-lead electrocardiogram monitoring could be an acceptable alternative for ILR implantation in detecting arrhythmia in symptomatic ACHD patients in respect to diagnostic yield, safety and management decisions, especially in those without syncope

An implantable loop recorder or smartphone based single-lead electrocardiogram to detect arrhythmia in adults with congenital heart disease?

Background: The European Society of Cardiology (ESC) guidelines for the management of adult congenital heart disease (ACHD) recommend screening in patients at risk for arrhythmic events. However, the optimal mode of detection is unknown. Methods: Baseline and follow-up data of symptomatic ACHD patients who received an implantable loop recorder (ILR) or who participated in a smartphone based single-lead electrocardiogram study were collected. The primary endpoint was time to first detected arrhythmia. Results: In total 116 ACHD patients (mean age 42 years, 44% male) were studied. The ILR group (n = 23) differed from the smartphone based single-lead electrocardiogram group (n = 93) in having a greater part of males and had more severe CHD and (near) syncope as qualifying diagnosis. In the smartphone based single-lead electrocardiogram group history of arrhythmia and palpitations were more frequent (all p < 0.05). Monitoring was performed for 40 and 79 patient-years for the ILR- and smartphone based single-lead electrocardiogram group, respectively. Arrhythmias occurred in 33 patients with an equal median time for both groups to first arrhythmia of 3 months (HR of 0.7, p = 0.81). Furthermore, atrial fibrillation occurred most often (n = 16) and common therapy changes included medication changes (n = 7) and implantation of pacemaker or Implantable Cardioverter Defibrillator (ICD) (N = 4). Symptoms or mode of detection were not a determinant of the first event. Conclusion: Non-invasive smartphone based single-lead electrocardiogram monitoring could be an acceptable alternative for ILR implantation in detecting arrhythmia in symptomatic ACHD patients in respect to diagnostic yield, safety and management decisions, especially in those without syncope