Emotional and behavioral problems in children with dilated cardiomyopathy.

BACKGROUND: Dilated cardiomyopathy (DCM) in children is an important cause of severe heart failure and carries a poor prognosis. Adults with heart failure are at increased risk of anxiety and depression and such symptoms predict adverse clinical outcomes such as mortality. In children with DCM, studies examining these associations are scarce. AIMS: We studied whether in children with DCM: (1) the level of emotional and behavioral problems was increased as compared to normative data, and (2) depressive and anxiety problems were associated with the combined risk of death or cardiac transplantation. METHODS: To assess emotional and behavioral problems in children with DCM, parents of 68 children, aged 1.5-18 years (6.9±5.7 years), completed the Child Behavior Checklist. RESULTS: Compared to normative data, more young children (1.5-5 years) with DCM had somatic complaints (24.3% vs. 8.0%; p < .001), but fewer had externalizing problems (5.4% vs. 17.0%; p = .049). Overall internalizing problems did not reach significance. Compared to normative data, more older children (6-18 years) showed internalizing problems (38.7% vs. 17.0%; p = .001), including depressive (29.0% vs. 8.0%; p < .001) and anxiety problems (19.4% vs. 8.0%; p = .023), and somatic complaints (29.0% vs. 8.0%; p < .001). Anxiety and depressive problems, corrected for heart failure severity, did not predict the risk of death or cardiac transplantation. CONCLUSION: Children of 6 years and older showed more depressive and anxiety problems than the normative population. Moreover, in both age groups, somatic problems were common. No association with outcome could be demonstrated.

Depression and health behaviors in women with Peripartum Cardiomyopathy.

BACKGROUND: Depression and health behavior engagement are a critical issue for recovery and secondary prevention in heart failure patients. No prior studies have examined these important clinical outcomes in young women diagnosed with Peripartum Cardiomyopathy (PPCM). We sought to characterize the prevalence of depression and health behaviors in PPCM patients and examine whether depression is associated with reduced engagement in health behaviors. METHODS: A nation-wide, cohort of 177 PPCM patients (mean age of 34.8 ± 5.7 years; median time since diagnosis of 3.0 ± 4.3 years) from a web-based quality of life registry completed questionnaires about depression (Patient Health Questionnaire; a cutoff score ≥10 was used for depression screening) and health behaviors. T-tests, chi-square and linear regression were used to compare clinical characteristics and health behaviors among depressed and non-depressed women. RESULTS: The prevalence of clinical depression at enrollment was 32.3% and was associated with use of antihypertensive medications, disability insurance status, higher BMI, history of arrhythmia and current or past use of psychotropic medication. Health behavior engagement for diet, physical activity, and tobacco cessation were low in the overall sample and depressed PPCM patients were significantly less likely to attend medical appointments than non-depressed women. CONCLUSIONS: Nearly 1 in 3 PPCM survivors reported symptoms of clinical depression which was associated with worse attendance at medical follow-up visits. Further research is needed to develop risk stratification models and patient-centered interventions to improve clinical outcomes for PPCM survivors.

Parent reports of health-related quality of life and heart failure severity score independently predict outcome in children with dilated cardiomyopathy.

BACKGROUND: Dilated cardiomyopathy in children causes heart failure and has a poor prognosis. Health-related quality of life in this patient group is unknown. Moreover, results may provide detailed information of parents' sense of their child's functioning. We hypothesised that health-related quality of life, as rated by parents, and the paediatric heart failure score, as assessed by physicians, have both predictive value on outcome. Methods and results In this prospective study, health-related quality of life was assessed by parent reports: the Infant Toddler Quality of Life questionnaire (0-4 years) or Child Health Questionnaire-Parent Form 50 (4-18 years) at 3-6-month intervals. We included 90 children (median age 3.8 years, interquartile range (IQR) 0.9-12.3) whose parents completed 515 questionnaires. At the same visit, physicians completed the New York University Pediatric Heart Failure Index. Compared with Dutch normative data, quality of life was severely impaired at diagnosis (0-4 years: 7/10 subscales and 4-18 years: 8/11 subscales) and ⩾1 year after diagnosis (3/10 and 6/11 subscales). Older children were more impaired (p<0.05). After a median follow-up of 3 years (IQR 2-4), 15 patients underwent transplantation. Using multivariable time-dependent Cox regression, "physical functioning" subscale and the Heart Failure Index were independently predictive of the risk of death and heart transplantation (hazard ratio 1.24 per 10% decrease of predicted, 95% confidence interval (CI) 1.06-1.47 and hazard ratio 1.38 per unit, 95% CI 1.19-1.61, respectively). CONCLUSION: Physical impairment rated by parents and heart failure severity assessed by physicians independently predicted the risk of death or heart transplantation in children with dilated cardiomyopathy.

Dynamics of cerebral blood flow in patients with mild non-ischaemic heart failure.

AIMS: Heart failure (HF) is associated with tissue hypoperfusion and congestion leading to organ dysfunction. Although cerebral blood flow (CBF) is preserved over a wide range of perfusion pressures in healthy subjects, it is impaired in end-stage HF. We aimed to compare CBF, autoregulation, and cognitive function in patients with mild non-ischaemic HF with healthy controls. METHODS AND RESULTS: Fifteen patients with mild idiopathic dilated cardiomyopathy and 15 matched healthy controls were studied. Co-existing cerebrovascular disease was excluded. All subjects, except five patients with an implantable cardioverter defibrillator, underwent magnetic resonance imaging for measurements of both CBF by arterial spin labelling and quantitative volume flow entering the brain. Cardiocerebral vascular function was assessed with Doppler techniques testing cerebral dynamic autoregulation and vasomotor reactivity. Cognitive analysis was performed by neuropsychological testing. Global and regional CBF did not differ between HF patients (44.3 mL/100 g.min) and controls (42.1 mL/100 g.min). Basilar but not carotid artery inflow was reduced in patients (1.95 mL/s vs. 2.51 mL/s, P = 0.009). Testing autoregulation revealed fewer dampened blood flow fluctuations in HF patients vs. controls (0.96% vs. 0.67%, P < 0.001). Vasomotor reactivity in HF patients showed a reduced CBF velocity (48.4% vs. 61.0%, P = 0.05) and regional cerebral oxygen saturation (18.3% vs. 23.8%, P = 0.02). Cognitive function overall was not affected. CONCLUSION: Although global CBF was unaffected in patients with mild HF, significant changes in basilar inflow volume, cerebral autoregulation and vasomotor reactivity were observed. We describe a model of dynamic cerebral mechanisms required to compensate for the impaired haemodynamics in early-stage HF.

Health Status and Psychological Distress in Patients with Non-compaction Cardiomyopathy: The Role of Burden Related to Symptoms and Genetic Vulnerability.

BACKGROUND: Non-compaction cardiomyopathy (NCCM) is a cardiomyopathy characterized by left ventricular tribeculae and deep intertrabecular recesses. Because of its genetic underpinnings and physical disease burden, noncompaction cardiomyopathy is expected to be associated with a lower health status and increase in pscyhological distress. PURPOSE: This study determined the health status and psychological distress in NCCM patients. We also examined the potential contribution of genetic predisposition and cardiac symptoms to health status and distress in NCCM, by comparing NCCM patients with (1) patients with familial hypercholesterolemia (FH) and (2) patients with acquired dilated cardiomyopathy (DCM). METHODS: Patients were recruited from the Erasmus Medical Center, Rotterdam, The Netherlands. Using a case-control design, NCCM patients (N = 45, mean age 46.7 ± 15.1 years, 38 % male) were compared with 43 FH patients and 42 DCM patients. Outcome measures were health status (Short Form Health Survey-12), anxiety (Generalized Anxiety Disorder 7-item scale) and depression (Patient Health Questionnaire 9-item scale). RESULTS: NCCM patients showed significantly worse health status (Physical Component Score F(1,84) = 9.58, P = .003; Mental Component Score F(1,84) = 16.65, P < .001), anxiety (F(1,85) = 9.63, P = .003) and depression scores (F(1,82) = 5.4, P = .023) compared to FH patients, also after adjusting age, sex, comorbidity, educational level and time since diagnosis. However, NCCM patients did not differ from DCM patients (Physical Component Score F(1,82) = 2,61, P = .11; Mental Component Score F(1,82) = .55, P = .46), anxiety (F(1,82) = 1.16, P = .28) and depression scores (F(1,82) = 1,95, P = .17). CONCLUSION: Cardiac symptoms are likely to play a role in the observed poor health status and elevated levels of anxiety and depressive symptoms in NCCM, whereas the burden of having a genetic condition may contribute less to these health status and psychological measures.

A group approach to genetic counselling of cardiomyopathy patients: satisfaction and psychological outcomes sufficient for further implementation.

The introduction of next-generation sequencing in everyday clinical genetics practise is increasing the number of genetic disorders that can be confirmed at DNA-level, and consequently increases the possibilities for cascade screening. This leads to a greater need for genetic counselling, whereas the number of professionals available to provide this is limited. We therefore piloted group genetic counselling for symptomatic cardiomyopathy patients at regional hospitals, to assess whether this could be an acceptable alternative to individual counselling. We performed a cohort study with pre- and post-counselling patient measurements using questionnaires, supplemented with evaluations of the group counselling format by the professionals involved. Patients from eight regional hospitals in the northern part of the Netherlands were included. Questionnaires comprised patient characteristics, psychological measures (personal perceived control (PPC), state and trait anxiety inventory (STAI)), and satisfaction with counsellors, counselling content and design. In total, 82 patients (mean age 57.5 year) attended one of 13 group sessions. Median PPC and STAI scores showed significantly higher control and lower anxiety after the counselling. Patients reported they were satisfied with the counsellors, and almost 75% of patients were satisfied with the group counselling. Regional professionals were also, overall, satisfied with the group sessions. The genetics professionals were less satisfied, mainly because of their perceived large time investment and less-than-expected group interaction. Hence, a group approach to cardiogenetic counselling is feasible, accessible, and psychologically effective, and could be one possible approach to counselling the increasing patient numbers in cardiogenetics.

Myocardial fibrosis and quality of life in patients with non-ischemic cardiomyopathy: a cardiovascular magnetic resonance imaging study.

Myocardial fibrosis (MF) is a common pathophysiologic endpoint in non-ischemic cardiomyopathy and may be identified by late gadolinium enhancement (LGE) MRI. While associated with future cardiovascular events in hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) the influence of MF on interim quality of life (QOL) has not been explored. In this study we investigate for associations between MF and validated indices of QOL in patients with HCM and DCM. Ninety-eight patients with known cardiomyopathy (n = 56-HCM/n = 42-DCM) underwent LGE-MRI in addition to standardized testing for QOL using the disease-specific Minnesota Living With Heart Failure (MLWHF) and the generic SF-12 questionnaires. LGE-MRI images were blindly analyzed for the presence and volume of MF using validated techniques. All analyses were stratified according to cardiomyopathy sub-type. The mean age of the population was 56.8 ± 12.9 years. MF was identified in 82 % of patients with HCM and 74 % of patients with DCM with respective mean MF burdens of 20.0 and 13.7 % of the left ventricular mass (p = 0.008). QOL scores for those with HCM or DCM, as assessed by both MLWHF and SF-12, were not significantly different between those with versus those without MF, and showed no association with MF burden by quantitative signal analysis. In this study we identified no association between QOL and MF burden by LGE-MRI in patients with HCM or DCM. Therefore, the severity of underlying myocardial tissue disease, a recognized substrate for ventricular arrhythmia, cannot and should not be inferred from the patient's symptom status or QOL.

Burden and well-being among a diverse sample of cancer, congestive heart failure, and chronic obstructive pulmonary disease caregivers.

CONTEXT: Three important causes of death in the U.S. (cancer, congestive heart failure, and chronic obstructive pulmonary disease) are preceded by long periods of declining health; often, family members provide most care for individuals who are living with serious illnesses and are at risk for impaired well-being. OBJECTIVES: To expand understanding of caregiver burden and psychosocial-spiritual outcomes among understudied groups of caregivers-cancer, congestive heart failure, and chronic obstructive pulmonary disease caregivers-by including differences by disease in a diverse population. METHODS: The present study included 139 caregiver/patient dyads. Independent variables included patient diagnosis and function; and caregiver demographics, and social and coping resources. Cross-sectional analyses examined distributions of these independent variables between diagnoses, and logistic regression examined correlates of caregiver burden, anxiety, depressive symptoms, and spiritual well-being. RESULTS: There were significant differences in patient functioning and caregiver demographics and socioeconomic status between diagnosis groups but few differences in caregiver burden or psychosocial-spiritual outcomes by diagnosis. The most robust social resources indicator of caregiver burden was desire for more help from friends and family. Anxious preoccupation coping style was robustly associated with caregiver psychosocial-spiritual outcomes. CONCLUSION: Caregiver resources, not patient diagnosis or illness severity, are the primary correlates associated with caregiver burden. Additionally, caregiver burden is not disease specific to those examined here, but it is rather a relatively universal experience that may be buffered by social resources and successful coping styles.

One-year results after implantation of the CorCap for dilated cardiomyopathy and heart failure.

BACKGROUND: We conducted a prospective study of the clinical outcomes and health-related quality of life after implantation of the CorCap support device (Acorn Cardiovascular Inc, St Paul, MN) for dilated cardiomyopathy. METHODS: The criteria adopted for CorCap implantation were dilated cardiomyopathy (left ventricular [LV] end-diastolic diameter≥60 mm, LV ejection fraction≤0.30 and >0.10), and New York Heart Association functional class II or III despite maximal medical therapy. Echocardiographic follow-up and evaluation with the Short Form-36 questionnaire were performed. RESULTS: Included were 39 patients: 5 in New York Heart Association class II and 32 in class III. At 13.3±2.5 months of follow-up, a statistically significant improvement was evident in mean LV volume (LV end-systolic volume from 202±94 to 138±72 ml. p=0.005) and systolic function (LV ejection fraction from 0.26±0.05 to 0.36±0.05, p<0.001). The mean LV sphericity index was significantly increased at the end of the follow-up (p=0.009). Ischemic etiology, diabetes, advanced age, and LV ejection fraction of less than 0.15 predicted lesser reversal of the LV alterations. Operative mortality was 5.1%. Cumulative follow-up mortality was 10.2%. The average Physical Health domain scores (Physical Functioning, Role Physical, General Health) were improved. Average Mental Health domain scores were also increased. CONCLUSIONS: The cardiac support device obtains reverse remodelling of the LV and is useful to improve the quality of life of patients with dilated cardiomyopathy and New York Heart Association class III symptoms of heart failure. The integration of different and complementary strategies (cardiac support device and resynchronization therapy) may represent the key to success for more complex patients, although further studies are required.

Relation of right ventricular dilation, age of repair, and restrictive right ventricular physiology with patient-reported quality of life in adolescents and adults with repaired tetralogy of fallot.

The present study aimed to determine the predictors of patient-reported quality of life and restrictive right ventricular (RV) physiology in adolescents and adults with repaired tetralogy of Fallot. A total of 62 patients (median age 28.5 years, range 14 to 69) undergoing cardiovascular magnetic resonance imaging completed the Short Form 36-item questionnaire, version 2, a validated quality of life assessment. RV inflow curves were generated from the sum of tricuspid inflow and pulmonary insufficiency. The patient-reported quality of life was comparable to population norms. Patients repaired after 1 year of age showed a strong trend toward a greater likelihood of physical component summary age-adjusted z-score ≤-1 (odds ratio 7.50, 95% confidence interval 0.90 to 62.3, p = 0.06). Patients with a RV ejection fraction of <45% reported decreased physical component summary (p = 0.02) and physical functioning (p = 0.02) scores. The RV end-diastolic volume, pulmonary regurgitation, and diastolic indexes did not predict the quality of life. The indexed RV end-diastolic volume was related to diastolic abnormalities, correlating with a greater peak early filling rate (r = 0.71, p <0.0001), ratio of peak early to atrial filling rates (r = 0.45, p = 0.006), and showing a strong trend with the end-diastolic forward flow in the pulmonary trunk (odds ratio 2.67 for moderate dilation and 3.50 for severe dilation, p = 0.06). Patients who underwent repair before 1 year old were more likely to have end-diastolic forward flow (15 of 17 vs 25 of 42, p = 0.03). In conclusion, the RV ejection fraction and age of repair were the best predictors of quality of life in this population, in whom end-diastolic forward flow and associated diastolic parameters appeared to reflect an overdistended ventricle, which might suggest a role for early pulmonary valve replacement.

Acute ischemic cardiomyopathy after extreme emotional stress in a child.

Ischemic cardiomyopathy is rare in children. It is usually caused by congenital anomalies of coronary arteries, coronary anomalies after coronary artery transfer, or Kawasaki disease. In recent years, a new cardiac syndrome-named "Tako-Tsubo cardiomyopathy" for the particular shape of the end systolic ventricle-has been described in adults. In the absence of coronary artery obstruction, it mimics acute myocardial infarction with chest pain and typical electrocardiography changes. Emotional or physical stress usually precedes this cardiomyopathy. At present, this entity has only been described in adults, with a strong predominance in postmenopausal women. We report a case of acute ischemic cardiomyopathy after extreme stress in a child that may share the same pathophysiology.

Effect of trimetazidine on quality of life in elderly patients with ischemic dilated cardiomyopathy.

INTRODUCTION: Elderly patients have an increased incidence of ischemic dilated cardiomyopathy, often related to diffuse coronary artery disease. Data have been cumulated to suggest that trimetazidine improves myocardial ischemia in patients with ischemic heart disease and improves left ventricular function in elderly patients with ischemic cardiomyopathy. The purpose of the present study was to assess the effects of trimetazidine in addition to standard cardiovascular therapy on left ventricular function and quality of life (QOL) parameters in elderly patients with ischemic heart disease and reduced left ventricular function. METHODS: Patients were randomized to receive either trimetazidine (twice daily) or placebo (twice daily) in addition to standard therapy, and were evaluated at baseline and after 6 months. RESULTS: Forty-seven patients completed the study (40 male, seven female; mean [+/-SD] age 78+/-3.4 years). Demographic data were comparable between the two groups with respect to sex, age, and race. At 6 months there was a significant improvement in the number of angina episodes per week in the trimetazidine group (-2.3+/-1, P=0.023). The overall assessment of QOL by a visual analog scale showed an improvement in patients randomized to trimetazidine at 6 months (from 4.1+/-0.6 to 6.4+/-0.8, P<0.01) and no changes in patients randomized to placebo (from 4.3+/-0.7 to 4.2+/-0.9, P>0.05). Physical QOL, evaluated by a MacNew Quality of Life After Myocardial Infarction questionnaire (MacNewQLMI), improved in patients randomized to trimetazidine but not in those allocated to placebo (32%+/-5% vs. -1%+/-3%, P<0.01). Similar results were obtained on social QOL evaluated by MacNewQLMI with trimetazidine compared with placebo (39%+/-4% vs. -2%+/-5%, P<0.01). CONCLUSION: In elderly patients with ischemic heart disease and reduced ventricular function, trimetazidine improves clinical condition and QOL.

[Concerning study of life quality in patients with postinfarction cardiosclerosis].

Myocardial infarction, which often occurs as a result of emotional stress, represents a severe somatic disease. This disease creates a substantial stress which would affect the quality of patient's life. Other factors like age, gender and education level affect psychological condition of the patients who had previously suffered from myocardial infarction. Thorough examination of interrelation between life quality, severity of disease and accompanying pathology would help to identify distinctive pattern of the disease clinical course, which in turn would serve as a contributing factor to optimize medical treatment and rehabilitation of the patients that fall into this category.

Effects of functional electrical stimulation on quality of life and emotional stress in patients with chronic heart failure secondary to ischaemic or idiopathic dilated cardiomyopathy: a randomised, placebo-controlled trial.

OBJECTIVE: Functional electrical stimulation (FES) improves exercise capacity and endothelial function in chronic heart failure (CHF) patients. This study evaluates the impact of FES on quality of life and emotional stress in patients with moderate to severe CHF. METHODS: Thirty patients with stable CHF (24 men; NYHA class II-III; left ventricular ejection fraction <35%) were randomly assigned (2:1) to a 6-week FES training program (n=20) or placebo (n=10). Questionnaires addressing quality of life [Kansas City Cardiomyopathy Questionnaire (KCCQ), functional and overall], and emotional stress [Zung self-rating depression scale (SDS), Beck Depression Inventory (BDI)], as well as plasma B-type natriuretic peptide (BNP) and 6-min walking distance test (6MWT) were assessed at baseline and after completion of training protocol. RESULTS: A significant improvement in KCCQ functional (F=76.666, p<0.001), KCCQ overall (F =41.508, p<0.001), BDI (F =17.768, p<0.001) and Zung SDS (F =27.098, p<0.001) was observed in the FES group compared to placebo. Patients in the FES group had also a significant increase in 6MWT (F =19.413, p<0.001) and a trend towards reduction in plasma BNP (F =4.252, p=0.053) compared to placebo. CONCLUSION: FES seems to have a beneficial effect on quality of life, exercise capacity and emotional stress in patients with moderate to severe CHF.

Symptom distress and quality of life in patients with advanced congestive heart failure.

Little is known about the burden of illness associated with advanced congestive heart failure (CHF). Understanding the needs of this population requires further information about symptoms and other factors related to quality of life. We studied a convenience sample of 103 community-dwelling patients with New York Heart Association Class III/IV CHF. The primary outcome, quality of life, was measured with the Multidimensional Index of Life Quality. Potential correlates of quality of life included overall symptom burden (Memorial Symptom Assessment Scale, MSAS), including global symptom distress (MSAS Global Distress Index, GDI); psychological state (Mental Health Inventory-5); functional status (Sickness Impact Profile); spirituality (Functional Assessment of Chronic Illness Therapy-Spirituality Scale); and co-morbid conditions (Charlson Comorbidity Index). Patients had a mean age of 67.1 years (SD=12.1); were mostly white (72.8%), male (71.8%), and married (51.5%); and had a mean ejection fraction of 22.3% (SD=6.8). The most prevalent symptoms were lack of energy (66%), dry mouth (62%), shortness of breath (56%), and drowsiness (52%). Pain was reported by about one-third of patients. For each of these symptoms, high symptom-related distress was reported by 14.1%-54.1%. Quality of life was moderately compromised (Multidimensional Index of Life Quality composite, median=56, possible range 12-84). Impairment in quality of life was strongly associated with global symptom distress (MSAS GDI; r=0.74, P<0.001); burden of comorbid conditions (r = -0.32, P=0.002), female sex (r=-0.22, P=0.03), functional impairment, particularly psychological impairment (r=-0.55, P<0.001), and poorer psychological well-being (r=0.68, P<0.001). In multivariate analyses, impairment in quality of life was significantly related to high symptom distress, poorer psychological well-being, and poor functional mobility (R2=0.67; P=0.002 for all). Distressful symptoms related to impaired quality of life included lack of energy (P=0.04), irritability (P=0.03), and drowsiness (P=0.02). Community-dwelling patients with advanced CHF experience numerous symptoms, significant symptom distress, and a compromised quality of life. Overall quality of life was strongly associated with symptom distress, psychological well-being and functional status. A focus on ameliorating prevalent physical symptoms and psychological distress, along with supportive measures that promote functional mobility, may lead to an improvement in the overall quality of life in this patient population.