Health-related quality of life and physical activity in children with inherited cardiac arrhythmia or inherited cardiomyopathy: the prospective multicentre controlled QUALIMYORYTHM study rationale, design and methods.

BACKGROUND: Advances in paediatric cardiology have improved the prognosis of children with inherited cardiac disorders. However, health-related quality of life (QoL) and physical activity have been scarcely analysed in children with inherited cardiac arrhythmia or inherited cardiomyopathy. Moreover, current guidelines on the eligibility of young athletes with inherited cardiac disorders for sports participation mainly rely on expert opinions and remain controversial. METHODS: The QUALIMYORYTHM trial is a multicentre observational controlled study. The main objective is to compare the QoL of children aged 6 to 17 years old with inherited cardiac arrhythmia (long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, or arrhythmogenic right ventricular dysplasia), or inherited cardiomyopathy (hypertrophic, dilated, or restrictive cardiomyopathy), to that of age and gender-matched healthy subjects. The secondary objective is to assess their QoL according to the disease's clinical and genetic characteristics, the level of physical activity and motivation for sports, the exercise capacity, and the socio-demographic data. Participants will wear a fitness tracker (ActiGraph GT3X accelerometer) for 2 weeks. A total of 214 children are required to observe a significant difference of 7 ± 15 points in the PedsQL, with a power of 90% and an alpha risk of 5%. DISCUSSION: After focusing on the survival in children with inherited cardiac disorders, current research is expanding to patient-reported outcomes and secondary prevention. The QUALIMYORYTHM trial intends to improve the level of evidence for future guidelines on sports eligibility in this population. Trial registration ClinicalTrials.gov Identifier: NCT04712136, registered on January 15th, 2021 ( https://clinicaltrials.gov/ct2/show/NCT04712136 ).

Arrhythmia Risk During the 2016 US Presidential Election: The Cost of Stressful Politics.

Background Anger and extreme stress can trigger potentially fatal cardiovascular events in susceptible people. Political elections, such as the 2016 US presidential election, are significant stressors. Whether they can trigger cardiac arrhythmias is unknown. Methods and Results In this retrospective case-crossover study, we linked cardiac device data, electronic health records, and historic voter registration records from 2436 patients with implanted cardiac devices. The incidence of arrhythmias during the election was compared with a control period with Poisson regression. We also tested for effect modification by demographics, comorbidities, political affiliation, and whether an individual's political affiliation was concordant with county-level election results. Overall, 2592 arrhythmic events occurred in 655 patients during the hazard period compared with 1533 events in 472 patients during the control period. There was a significant increase in the incidence of composite outcomes for any arrhythmia (incidence rate ratio [IRR], 1.77 [95% CI, 1.42-2.21]), supraventricular arrhythmia (IRR, 1.82 [95% CI, 1.36-2.43]), and ventricular arrhythmia (IRR, 1.60 [95% CI, 1.22-2.10]) during the election relative to the control period. There was also an increase in specific types of arrhythmia, including atrial fibrillation (IRR, 1.50 [95% CI, 1.06-2.11]), supraventricular tachycardia (IRR, 3.7 [95% CI, 2.2-6.2]), nonsustained ventricular tachycardia (IRR, 1.7 [95% CI, 1.3-2.2]), and daily atrial fibrillation burden (P<0.001). No significant interaction was found for sex, race/ethnicity, device type, age ≥65 years, hypertension, coronary artery disease, heart failure, political affiliation, or concordance between individual political affiliation and county-level election results. Conclusions There was a significant increase in cardiac arrhythmias during the 2016 US presidential election. These findings suggest that exposure to stressful sociopolitical events may trigger arrhythmogenesis in susceptible people.

The INFluence of Remote monitoring on Anxiety/depRession, quality of lifE, and Device acceptance in ICD patients: a prospective, randomized, controlled, single-center trial.

BACKGROUND: Impact of telemedicine with remote patient monitoring (RPM) in implantable cardioverter-defibrillator (ICD) patients on clinical outcomes has been investigated in various clinical settings with divergent results. However, role of RPM on patient-reported-outcomes (PRO) is unclear. The INFRARED-ICD trial aimed to investigate the effect of RPM in addition to standard-of-care on PRO in a mixed ICD patient cohort. METHODS AND RESULTS: Patients were randomized to RPM (n = 92) or standard in-office-FU (n = 88) serving as control group (CTL). At baseline and on a monthly basis over 1 year, study participants completed the EQ-5D questionnaire for the primary outcome Quality of Life (QoL), the Hospital Anxiety and Depression Scale, and the Florida Patient Acceptance Survey questionnaire for secondary outcomes. Demographic characteristics (82% men, mean age 62.3 years) and PRO at baseline were not different between RPM and CTL. Primary outcome analysis showed that additional RPM was not superior to CTL with respect to QoL over 12 months [+ 1.2 vs. + 3.9 points in CTL and RPM group, respectively (p = 0.24)]. Pre-specified analyses could not identify subgroups with improved QoL by the use of RPM. Neither levels of anxiety (- 0.4 vs. - 0.3, p = 0.88), depression (+ 0.3 vs. ± 0.0, p = 0.38), nor device acceptance (+ 1.1 vs. + 1.6, p = 0.20) were influenced by additional use of RPM. CONCLUSION: The results of the present study show that PRO were not improved by RPM in addition to standard-of-care FU. Careful evaluation and planning of future trials in selected ICD patients are warranted before implementing RPM in routine practice.

The Role of Quality of Life Indices in Patient-Centred Management of Arrhythmia.

Clinicians obviously need to understand the impact of illness and its management on their patients' quality of life (QOL). Objective, "hard" outcomes have taken primacy in the assessment of treatment outcomes in cardiovascular care, at least in part because they are relatively easy to measure precisely and accurately. QOL, on the other hand, is a subjective and more nebulous concept. However, decades of clinical observation and research have clearly shown that what matters most to patients is their subjective QOL, and objective measures of disease burden are less salient to their concerns. It is possible to measure QOL objectively and quantitatively using validated methods and to incorporate these into both everyday clinical care and research to measure the effect of various treatments and strategies. Physician beliefs about patients' subjective states are often imprecise and imperfectly accurate. Patient-reported QOL measures can help to predict the potential benefits of treatment; using validated measures, an estimate of the relevance of a particular amount of change in QOL can be obtained; the efficacy of different treatments from the patient's perspective can be compared. In patient-centred care, the patient is the central focus of attention; this focus should be on patients and their subjective perceptions, more importantly than on the disease or associated laboratory measures of severity.

Quality of life and psychological co-morbidities in children and adolescents with cardiac pacemakers and implanted defibrillators: a cohort study in Eastern Germany.

INTRODUCTION: The implantation of a pacemaker or an implantable cardioverter-defibrillator during childhood may reduce quality of life and lead to mental health problems. This study aimed to evaluate potential mental health problems (i.e., depressive and anxiety symptoms) and quality of life in children with cardiac active devices in comparison to healthy peers. METHODS: We analysed data of children with pacemakers or implantable cardioverter-defibrillators aged 6-18 years. Quality of life, depressive and anxiety symptoms were assessed by standardised questionnaires. The results were compared to age-matched reference groups. RESULTS: Children with implantable cardioverter-defibrillator showed significant lower quality of life in comparison to reference group (p = 0.03), but there was no difference in quality of life between children with pacemaker and reference group. There was no significant difference in depressive symptoms between children with a cardiac rhythm device compared to reference group (self-report: p = 0.67; proxy report: p = 0.49). There was no significant difference in anxiety (p = 0.53) and depressive symptoms (p = 0.86) between children with pacemaker and children with implantable cardioverter-defibrillator. CONCLUSIONS: Living with an implantable cardioverter-defibrillator in childhood seems to decrease the patients' quality of life. Although children with pacemaker and implantable cardioverter-defibrillator don't seem to show more depressive and anxiety symptoms in comparison to their healthy peers, there still can be an increased risk for those children to develop mental health problems. Therefore, treating physicians should be aware of potential mental health problems and provide the patients and their families with appropriate therapeutic offers.

Medical and neurobehavioural phenotypes in carriers of X-linked ichthyosis-associated genetic deletions in the UK Biobank.

BACKGROUND: X-linked ichthyosis (XLI) is an uncommon dermatological condition resulting from a deficiency of the enzyme steroid sulfatase (STS), often caused by X-linked deletions spanning STS. Some medical comorbidities have been identified in XLI cases, but small samples of relatively young patients has limited this. STS is highly expressed in subcortical brain structures, and males with XLI and female deletion carriers appear at increased risk of developmental/mood disorders and associated traits; the neurocognitive basis of these findings has not been examined. METHODS: Using the UK Biobank resource, comprising participants aged 40-69 years recruited from the general UK population, we compared multiple medical/neurobehavioural phenotypes in males (n=86) and females (n=312) carrying genetic deletions spanning STS (0.8-2.5 Mb) (cases) to male (n=190 577) and female (n=227 862) non-carrier controls. RESULTS: We identified an elevated rate of atrial fibrillation/flutter in male deletion carriers (10.5% vs 2.7% in male controls, Benjamini-Hochberg corrected p=0.009), and increased rates of mental distress (p=0.003), irritability (p<0.001) and depressive-anxiety traits (p<0.05) in male deletion carriers relative to male controls completing the Mental Health Questionnaire. While academic attainment was unaffected, male and female deletion carriers exhibited impaired performance on the Fluid Intelligence Test (Cohen's d≤0.05, corrected p<0.1). Neuroanatomical analysis in female deletion carriers indicated reduced right putamen and left nucleus accumbens volumes (Cohen's d≤0.26, corrected p<0.1). CONCLUSION: Adult males with XLI disease-causing deletions are apparently at increased risk of cardiac arrhythmias and self-reported mood problems; altered basal ganglia structure may underlie altered function and XLI-associated psychiatric/behavioural phenotypes. These results provide information for genetic counselling of deletion-carrying individuals and reinforce the need for multidisciplinary medical care.

Quality of life of patients undergoing conventional vs leadless pacemaker implantation: A multicenter observational study.

BACKGROUND: Leadless pacemakers (L-PM) are an emerging effective and safe technology that offer an alternative to conventional pacemakers (C-PM) for right ventricular stimulation. However, there is little information about their potential benefits for quality of life (QoL) in patients with L-PM. We compared QoL between patients with L-PM and C-PM. METHODS: The study population comprised patients undergoing single chamber pacemaker implantation from December 2016 to March 2018. The SF-36 questionnaire was used to evaluate QoL at baseline and at 6 months of followup. We also used a questionnaire consisted of 10 specific questions related to the implant procedure. RESULTS: A total of 106 patients (64 C-PM; 42 L-PM) were included. There were no differences in baseline characteristics between the groups (C-PM vs L-PM), except for age (81.5 vs 77.3 years; P = .012) and diabetes (38% vs 17%; P = .021). Baseline SF-36 scores did not differ between the groups. At 6 months followup, patients in the L-PM group scored significantly higher on physical function (63 vs 42; P < .001), physical role (64 vs 36; P = .004), and mental health (75 vs 65; P = .017), even after adjusting for covariates. Pacemaker-related discomfort and physical restrictions were significantly lower for the L-PM group. CONCLUSION: L-PM is associated with better QoL than C-PM in both physical and mental health. Patients undergoing L-PM implantation reported less procedure-related discomfort, physical restriction, and preoccupation.

Patient-reported outcomes at hospital discharge among patients with arrhythmia: Results from the national DenHeart survey.

BACKGROUND: Patient-reported outcomes are important predictors of mortality, cardiovascular events and hospitalisation in patients with cardiac diseases, but differences in patient-reported outcomes between groups of patients with arrhythmia have not yet been investigated. AIMS: To describe and compare patient-reported outcomes at discharge among patients with different types of cardiac arrhythmia and to examine the associations between demographic characteristics, inhospital factors and patient-reported outcomes. METHODS: Data were derived from the national DenHeart study including patient-reported outcomes from the following questionnaires: the hospital anxiety and depression scale (HADS), HeartQoL, short form 12 (SF-12), current health status (EQ-5D), brief illness perception questionnaire (B-IPQ) and the Edmonton symptom assessment scale (ESAS). Clinical and demographic data were obtained from national registers. Multiple linear and logistic regression models were used to investigate the associations between the potential risk factors and the patient-reported outcomes. RESULTS: A total of 4251 patients diagnosed with arrhythmia completed the questionnaire. Across the arrhythmia subgroups, some differences were observed. In general, patient-reported outcome scores were worst among patients with 'ventricular arrhythmia' (e.g. highest depression scores and highest prevalence of feeling unsafe at discharge). Regression analysis revealed that longer hospital stay, female gender, being unmarried and having a short education were significantly associated with poor health for almost all arrhythmia subgroups. CONCLUSION: Differences exist in self-reported health, quality of life and symptom burden across arrhythmia groups with patients with ventricular arrhythmia reporting poorer patient-reported outcomes. Longer hospital stay, female gender, being unmarried and having a low level of education were significantly associated with worse outcomes among the total population.

Cognitive behavioural therapy significantly reduces anxiety in patients with implanted cardioverter defibrillator compared with usual care: Findings from the Screen-ICD randomised controlled trial.

AIM: The aim of this study was to investigate the effect of a cognitive behavioural therapy intervention provided by trained cardiac nurses plus usual care compared with usual care alone in patients with an implanted cardioverter defibrillator who, prior to randomization, had presented with symptoms of anxiety measured by the Hospital Anxiety and Depression Scale. BACKGROUND: Around 20-25% of all patients with an implanted cardioverter defibrillator experience anxiety. Among these patients anxiety is associated with mortality, presumably explained by risk behaviour and activation of the autonomic nervous system. We hypothesised that cognitive behavioural therapy provided by cardiac nurses in an out-patient setting would be an effective treatment of anxiety. METHODS: This was an investigator-initiated randomised clinical superiority trial with blinded outcome assessment, with 1:1 randomisation to cognitive behavioural therapy plus usual care or to usual care. The primary outcome was Hospital Anxiety and Depression Scale-Anxiety mean score at 16 weeks. Secondary outcomes included Hospital Anxiety and Depression Scale-Depression, Becks Anxiety Inventory, HeartQoL and Hamilton Anxiety Scale. Primary outcome analysis was based on the intention-to-treat principle. RESULTS: A total of 88 participants were included, 66% men, mean age 64.3 years and 61% had primary indication implantable cardioverter defibrillator. A significant difference after 16 weeks was found between groups: intervention group (Hospital Anxiety and Depression Scale-A mean (standard deviation) 4.95 points (3.30) versus usual care group 8.98 points (4.03) (p < 0.0001)). Cohen's d was -0.86, indicating a strong clinical effect. The effect was supported by secondary outcomes. CONCLUSION: Cognitive behavioural therapy provided by cardiac nurses to patients with a positive screening for anxiety had a statistically and clinically significant effect on anxiety compared with patients not receiving cognitive behavioural therapy.

An Easy and Reliable Way to Prevent Electrocardiographic Deteriorations of Patients Undergoing Off-Pump Coronary Artery Bypass Surgery: Preoperative Anxiolytic Treatment.

OBJECTIVE: To investigate the effects of preoperative anxiety relieving on electrophysiological changes in patients undergoing off-pump coronary artery bypass surgery. METHODS: A total of 61 patients at ASA III risk group in the age range of 18-65 years were enrolled in the present study. Patients were randomly divided into two groups. Group S (Sedation group) was administered 0.04 mg/kg lorazepam per os (PO) twice before the operation. Group C (control group) was not administered with any anxiolytic premedication. State Trait Anxiety Inventory (STAI-I) and Beck Anxiety Inventory (BAI) were used to evaluate the level of anxiety. Electrocardiography (ECG), pulse oximeter and standard monitoring were performed for each patient. QT and P dispersions in each derivation of all ECGs were calculated. RESULTS: Preoperative STAI-I scores were significantly lower in sedation group compared to the controls. Mean values of QT dispersion measured before induction, at the 1st minute of induction, 30th second of intubation and 4th minute of intubation in sedation group were significantly reduced compared to controls (P=0.024; P=0.027; P=0.001; P=0.033, respectively). The mean values of P dispersion measured before induction, at the 3rd minute of induction, 30th second of intubation and 4th minute of intubation in sedation group were significantly reduced compared to controls (P=0.001; P=0.020; P=0.023; P=0.005, respectively). CONCLUSION: Elevated anxiety levels in patients undergoing coronary bypass surgery have a negative effect through prolonged QT and P-wave dispersion times. Anxiolytic treatment before surgery may be useful to prevent ventricular and atrial arrhythmias and associated complications through decreasing the QT and P-wave dispersion duration.

An easy and reliable way to prevent electrocardiographic deteriorations of patients undergoing off-pump coronary artery bypass surgery: preoperative anxiolytic treatment

Abstract Objective: To investigate the effects of preoperative anxiety relieving on electrophysiological changes in patients undergoing off-pump coronary artery bypass surgery. Methods: A total of 61 patients at ASA III risk group in the age range of 18-65 years were enrolled in the present study. Patients were randomly divided into two groups. Group S (Sedation group) was administered 0.04 mg/kg lorazepam per os (PO) twice before the operation. Group C (control group) was not administered with any anxiolytic premedication. State Trait Anxiety Inventory (STAI-I) and Beck Anxiety Inventory (BAI) were used to evaluate the level of anxiety. Electrocardiography (ECG), pulse oximeter and standard monitoring were performed for each patient. QT and P dispersions in each derivation of all ECGs were calculated. Results: Preoperative STAI-I scores were significantly lower in sedation group compared to the controls. Mean values of QT dispersion measured before induction, at the 1st minute of induction, 30th second of intubation and 4th minute of intubation in sedation group were significantly reduced compared to controls (P=0.024; P=0.027; P=0.001; P=0.033, respectively). The mean values of P dispersion measured before induction, at the 3rd minute of induction, 30th second of intubation and 4th minute of intubation in sedation group were significantly reduced compared to controls (P=0.001; P=0.020; P=0.023; P=0.005, respectively). Conclusion: Elevated anxiety levels in patients undergoing coronary bypass surgery have a negative effect through prolonged QT and P-wave dispersion times. Anxiolytic treatment before surgery may be useful to prevent ventricular and atrial arrhythmias and associated complications through decreasing the QT and P-wave dispersion duration.

Psychometric evaluation of the Polish version of the Arrhythmia­Specific Questionnaire in Tachycardia and Arrhythmia: a new tool for symptom and health­related quality of life assessment.

BACKGROUND: Atrial fibrillation (AF) is the most common arrhythmia resulting in hospitalization. The assessment of symptoms and health­related quality of life (HRQoL) can provide valuable information before, during, and after health care interventions for AF. AIMS: We aimed to perform a translation and cultural adaptation of the Arrhythmia­Specific Questionnaire in Tachycardia and Arrhythmia (ASTA), and to evaluate the reliability and validity of its Polish version. METHODS: The standard forward­backward translation procedure to translate the ASTA questionnaire into Polish was used. A total of 244 patients with AF at a mean (SD) age of 70.7 (10.7) years completed the questionnaire and were included in the study. Reliability was tested using internal consistency (Cronbach α) and validity with an item­total correlation, exploratory factor analysis (EFA), and confirmatory factor analysis (CFA). RESULTS: The ASTA symptom scale had satisfactory psychometric properties (α = 0.718), and the corrected item­total correlation was sufficient for most items (0.361-0.506), except for cold sweats (0.156). The ASTA HRQoL scale showed good psychometric properties (α = 0.855). Initial CFA analyses showed that the 1- and 2­factor models had similar properties, with strong factor loadings and satisfactory goodness­of­fit values according to the comparative fit index (0.947 for the 1­factor model vs 0.988 for the 2­factor model). A comparison of the 1­and 2­factor models showed that the close fit for the root­mean­square error of approximation was better for the 2­factor model (0.387 vs 0.193). A 2­factor EFA model was produced, and for factor 1 (physical scale), the varimax low ranged between 0.470 and 0.804, and for factor 2 (the mental scale), it ranged between 0.597 and 0.873. CONCLUSIONS: The psychometric properties of the Polish version of the ASTA questionnaire were overall found to be satisfactory.

Cardiac biomarkers of disordered eating: A case for decreased mean R wave amplitude.

The purpose of this study was to identify cardiac biomarkers of disordered eating. Mean R wave amplitude (mV), mean T wave amplitude (mV), QRS interval (sec), QTc interval (sec), and Tpeak-Tend interval (sec) were assessed via electrocardiography among women with clinical (n = 53) and subclinical (n = 56) eating disorder symptoms versus asymptomatic controls (n = 32). QRS and QTc intervals were significantly longer and mean T and R wave amplitudes significantly lower among women with clinical symptoms compared to asymptomatic controls. QTc interval length was significantly longer and mean R wave amplitude was significantly lower among women with subclinical symptoms versus asymptomatic controls. Decreased mean R wave amplitude yielded a comparable effect size as QTc when differentiating between asymptomatic and subclinical groups and a larger effect size than QTc when differentiating between asymptomatic and clinical groups, representing a promising clinical biomarker.

Clinically significant ventricular arrhythmias and progression of depression and anxiety following an acute coronary syndrome.

BACKGROUND: Depression and anxiety are common and associated with worse clinical outcomes in patients who experience an acute coronary syndrome (ACS). We investigated the association between major ventricular arrhythmias (VAs) with the progression of depression and anxiety among hospital survivors of an ACS. METHODS: Patients were interviewed in hospital and by telephone up to 12 months after hospital discharge. The primary outcome was the presence of moderate/severe symptoms of depression and anxiety defined as a Patient Health Questionnaire (PHQ)-9 score ≥ 10 and a Generalized Anxiety Disorder (GAD)-7 score ≥ 10 at baseline and 1 month and PHQ-2 ≥ 3 and GAD-2 ≥ 3 at 3, 6, and 12 months. We used marginal models to examine the association between major VAs and the symptoms of depression or anxiety over time. RESULTS: The average age of the study population (n = 2074) was 61.1 years, 33.5% were women, and 78.3% were white. VAs developed in 105 patients (5.1%). Symptoms of depression and anxiety were present in 22.2% and 23.5% of patients at baseline, respectively, and declined to 14.1% and 12.6%, respectively, at 1-month post-discharge. VAs were not significantly associated with the progression of symptoms of depression (adjusted relative risk [aRR] = 1.29, 95% confidence interval [CI] = 0.94-1.77) and anxiety (aRR = 1.22, 95% CI = 0.86-1.72), or with change in average scores of PHQ-2 and GAD-2 over time, both before and after risk adjustment. CONCLUSION: The prevalence of symptoms of depression and anxiety was high after an ACS but declined thereafter and may not be associated with the occurrence of major in-hospital VAs.

Living with a pacemaker: patient-reported outcome of a pacemaker system.

BACKGROUND: The aim of this study was to assess among pacemaker patients their overall satisfaction with the pacemaker system, pain, soreness/discomfort, cosmetic results, restrictions due to impaired movement of the shoulder/arm/chest, related sleep disturbances, and concern about possible device malfunction. METHODS: The seven-item questionnaire was mailed to patients from a single center who had a pacemaker implant or replacement between 2006 and 2016. A higher score indicated worse outcome on a visual analog scale (VAS) of 0-100 mm. RESULTS: The response rate was 75.5% and 342 questionniares were analyzed. Median age of respondents was 77.6 years and 57.0% were males. In total, 65 complications requiring surgery (10 pocket corrections (2.9%), 5 in females) occurred during a median follow-up of 5.6 years.The distribution of the primary outcome had a median score of 5 while the 75th percentile was 13. Cosmetic appearance was significantly associated with reoperation (but not other variables). Overall scores for men and women were 5 vs. 6, respectively, which achieved significance (p = 0.042). Median ratings of pain, soreness/discomfort, cosmetic appearance, range of motion, sleep, and concern about device malfunction were all ≤5. Females reported worse outcomes for all questions, except for cosmetic results and concern about malfunction. CONCLUSIONS: The vast majority of patients report excellent overall satisfaction with the pacemaker system, and are not affected by pain, soreness/discomfort, or concern about device malfunction. They also reported favourable outcomes with respect to cosmetic results, shoulder movement, and sleep. However, some patients underwent a surgical correction of the pacemaker pocket.

The mental health of adolescents and pre-adolescents living with inherited arrhythmia syndromes: a systematic review of the literature.

Potentially fatal arrhythmias add to the mental health challenges of adolescence. This systematic review sought to summarise current knowledge regarding the mental health of adolescents and pre-adolescents diagnosed with inherited arrhythmia syndromes. Searches combining psychological problems with inherited cardiac arrhythmia diagnoses identified 16 studies with paediatric (<18 years) inherited arrhythmia patients. All studies were cross-sectional; 8/16 required an implantable cardioverter defibrillator. Methods were quantitative (n=11), qualitative (n=4), or mixed (n=1), with 14-100% of participants having an inherited arrhythmia syndrome. Mean/median age in 13/16 studies was 12-16 years. Patients and parents reported lower quality of life, particularly in relation to physical function, social relationships, restriction of peer activities, bodily pain, and mental and emotional health. Self-perceptions and behaviour were similar to healthy populations. Rates of anxiety and depression (15-33% of these patients) were not increased in these studies where patients were assessed 2+ years after diagnosis. Higher mental health risk occurred among patients who have a diagnosed sibling, those with cardiomyopathy, and those who report decreased quality of life. Mental health research among youth with inherited arrhythmias is extremely limited and of low quality. Data, primarily from patients 2-4 years after diagnosis or treatment with an implantable cardioverter defibrillator, indicate that quality of life may be decreased and 15-33% experience mental health issues. Future research is required to examine the mental health and quality of life of paediatric patients with inherited arrhythmia syndromes, whether or not they have an implantable cardioverter defibrillator, from time of diagnosis.

The Genetic Counselor in the Pediatric Arrhythmia Clinic: Review and Assessment of Services.

There are minimal data on the impact of genetic counselors in subspecialty clinics, including the pediatric arrhythmia clinic. This study aimed to describe the clinical encounters of a genetic counselor integrated into a pediatric arrhythmia clinic. In the 20 months between July 2015 and February 2017, a total of 1914 scheduled patients were screened for indications relevant for assessment by a genetic counselor. Of these, the genetic counselor completed 276 patient encounters, seeing 14.4% of all patients in clinic. The most expected and common indications for genetic counselor involvement were related to suspicion for primary heritable arrhythmia conditions, though patients seen in this clinic display a wide range of cardiac problems and many additional indications for genetic evaluation were identified. Roughly 75% (211/276) of encounters were for personal history of confirmed/suspected heritable disease, including cardiac channelopathies, cardiomyopathies, ventricular arrhythmias, and congenital heart defects, and 25% (65/276) were for family history of disease, including long QT syndrome and sudden unexplained death. Overall, this study shows that about 1 in 7 patients seen in a pediatric arrhythmia clinic have indications that likely benefit from genetic counselor involvement and care. Similar service delivery models embedding genetic counselors in pediatric arrhythmia clinics should be encouraged, and this model could be emulated to increase patient access to genetic counseling services.

Sleep disturbance in patients with an implantable cardioverter defibrillator: Prevalence, predictors and impact on health status.

BACKGROUND: Sleep disturbances are highly prevalent in patients with cardiac diseases and associated with poor health outcomes. However, little is known about sleep disturbance in patients with an implantable cardioverter defibrillator. AIMS: We examined the prevalence and predictors of sleep disturbance and the impact on perceived health status in a Dutch cohort of implantable cardioverter defibrillator patients. METHODS: Patients ( n=195) enrolled in the Web-based distress program for implantable cardioverter defibrillator patients (WEBCARE) trial completed questionnaires at the time of implantable cardioverter defibrillator implantation, three, six and 12 months afterwards. Sleep disturbance was assessed with the corresponding item #3 of the Patient Health Questionnaire 9. RESULTS: At baseline, 67% ( n=130) reported sleep disturbance (cut off ≥1). One year later, the prevalence was 57% ( n=112). Younger age (odds ratio=0.96, 95% confidence interval 0.92-0.99; p=0.012) and high negative affectivity/low social inhibition (odds ratio=4.47, 95% confidence interval 1.52-13.17; p=0.007) were associated with sleep disturbance at 12 months in adjusted analyses. Sleep disturbance was not associated with health status at 12 months. Charlson Comorbidity Index, anxiety, Type D personality and high negative affectivity/low social inhibition were associated with impaired health status at follow-up. CONCLUSIONS: Sleep disturbance was highly prevalent in patients with an implantable cardioverter defibrillator. Younger age and high negative affectivity predicted sleep disturbance 12 months post-implantation independent of other demographic, clinical, intervention and psychological covariates. Sleep disturbance was not associated with impaired health status at the 12-month follow-up.