Preliminary report on the cost effectiveness of ventricular assist devices.

The aim of the present study was to perform a cost-effectiveness analysis (CEA) of ventricular assist devices (VAD) implantation surgery in the Japanese medical reimbursement system. The study group consisted of thirty-seven patients who had undergone VAD implantation surgery for dilated cardiomyopathy (n = 25; 67.6 %) or hypertrophic cardiomyopathy (n = 4; 10.8 %), and others (n = 8; 21.6 %). Quality-adjusted life years (QALYs) were calculated using the utility score and years of life. Medical reimbursement bills were chosen as cost indices. The observation period was the 12-month period after surgery. Then, the incremental cost-effectiveness ratio was calculated according to the VAD type. In addition, the prognosis after 36 months was estimated on the basis of the results obtained using the Markov chain model. The mean preoperative INTERMACS profile score was 2.35 ± 0.77. Our results showed that the utility score, which indicates the effectiveness of VAD implantation surgery, improved by 0.279 ± 0.188 (ΔQALY, p < 0.05). The cost of VAD implantation surgery was 313,282 ± 25,275 (ΔUS$/year) on the basis of medical reimbursement bills associated with therapeutic interventions. The calculated result of CEA was 364,501 ± 190,599 (ΔUS$/QALY). The improvement in the utility score was greater for implantable versus extracorporeal VADs (0.233 ± 0.534 vs. 0.371 ± 0.238) and ICER was 303,104 (ΔUS$/ΔQALY). Furthermore, when we estimated CEA for 36 months, the expected baseline value was 102,712 (US$/QALY). Therefore, VAD implantation surgery was cost effective considering the disease specificities.

Epidemiology and cost of heart failure in children.

Heart failure in children is a complex disease process, which can occur secondary to a variety of aetiologies, including CHD, cardiomyopathy, or acquired conditions as well. Although the overall incidence of disease is low, the associated morbidity and mortality are high. Mortality may have decreased slightly over the last decade, and this is likely due to our ability to shepherd patients through longer periods of significant morbidity, with lasting effects. Costs of heart failure are significant - on the order of $1 billion annually as hospital charges for inpatient admissions alone. The value, or benefit to patient life and quality of life at this cost, is not well delineated. Further research is needed to optimise not only outcomes for these patients but also the high costs associated with them.

Social determinants of health in the setting of hypertrophic cardiomyopathy.

INTRODUCTION: Social determinants of health play an important role in explaining poor health outcomes across many chronic disease states. The impact of the social gradient in the setting of an inherited heart disease, hypertrophic cardiomyopathy (HCM), has not been investigated. This study sought to profile the socioeconomic status of patients attending a specialized multidisciplinary clinic and to determine the impact on clinical factors, psychosocial wellbeing and adherence to medical advice. METHODS: Patients with HCM and at-risk relatives attending a specialized multidisciplinary clinic in Sydney Australia between 2011 and 2013 were included. Clinical, socioeconomic, geographic remoteness and adherence data were available. A broader clinic and registry-based group completed a survey including psychological wellbeing, health-related quality of life, Morisky Medication Adherence Scale and individual-level socioeconomic information. RESULTS: Over a 3-year period, 486 patients were seen in the specialized clinic. There was an over-representation of patients from socioeconomically advantaged and the least geographically remote areas. Socioeconomic disadvantage was associated with comorbidities, poor psychological wellbeing and health-related quality of life, lower understanding of HCM and more complex clinical management issues such as NYHA class, atrial fibrillation and left ventricular outflow tract obstruction. Approximately 10% of patients were non-adherent to medical advice, and poor medication adherence was seen in 30% of HCM patients with associated factors being younger age, minority ethnicity, anxiety and poor mental quality of life. CONCLUSIONS: Of all the patients attending a specialized cardiac genetic clinic, there is an overrepresentation of patients from very advantaged and major metropolitan areas and suggests that those most in need of a multidisciplinary approach to care are not accessing it.

Should axis deviation or atrial enlargement be categorised as abnormal in young athletes? The athlete's electrocardiogram: time for re-appraisal of markers of pathology.

AIMS: The 2010 European Society of Cardiology (ESC) guidelines for electrocardiogram (ECG) interpretation in athletes are associated with a relatively high false positive rate and warrant modification to improve the specificity without compromising sensitivity. The aim of this study was to investigate whether non-specific anomalies such as axis deviation and atrial enlargement in isolation require further assessment in highly trained young athletes. METHOD AND RESULTS: Between 2003 and 2011, 2533 athletes aged 14-35 years were investigated with 12-lead ECG and echocardiography. Electrocardiograms were analysed for non-training-related (Group 2) changes according to the 2010 ESC guidelines. Results were compared with 9997 asymptomatic controls. Of the 2533 athletes, 329 (13%) showed Group 2 ECG changes. Isolated axis deviation and isolated atrial enlargement comprised 42.6% of all Group 2 changes. Athletes revealed a slightly higher prevalence of these anomalies compared with controls (5.5 vs. 4.4%; P = 0.023). Echocardiographic evaluation of athletes and controls with isolated axis deviation or atrial enlargement (n = 579) failed to identify any major structural or functional abnormalities. Exclusion of axis deviation or atrial enlargement reduced the false positive rate from 13 to 7.5% and improved specificity from 90 to 94% with a minimal reduction in sensitivity (91-89.5%). CONCLUSION: Isolated axis deviation and atrial enlargement comprise a high burden of Group 2 changes in athletes and do not predict underlying structural cardiac disease. Exclusion of these anomalies from current ESC guidelines would improve specificity and cost-effectiveness of pre-participation screening with ECG.

Obtaining insurance after DNA diagnostics: a survey among hypertrophic cardiomyopathy mutation carriers.

Hypertrophic cardiomyopathy (HCM) is a common hereditary heart disease associated with increased mortality. Disclosure of DNA test results may have social implications such as low access to insurance. In The Netherlands, insurance companies are restricted in the use of genetic information of their clients by the Medical Examination Act. A cross-sectional survey was used to assess the frequency and type of problems encountered by HCM mutation carriers applying for insurance, and associations with carriers' characteristics. The response rate was 86% (228/264). A total of 66 carriers (29%) applied for insurance of whom 39 reported problems (59%) during an average follow-up of 3 years since the DNA test result. More problems were encountered by carriers with manifest disease (P<0.001) and carriers with symptoms of HCM (P=0.049). Carriers identified after predictive DNA testing less frequently experienced problems (P=0.002). Three carriers without manifest HCM reported problems (5% of applicants). Frequently reported problems were higher premium (72%), grant access to medical records (62%), and complete rejection (33%). In conclusion, HCM mutation carriers frequently encounter problems when applying for insurances, often in the case of manifest disease, but the risk assessment of insurance companies is largely justified. Still, 5% of carriers encounter potentially unjustified problems, indicating the necessity to monitor the application of the existing laws and regulations by insurance companies and to educate counselees on the implications of these laws and regulations.

Life expectancy gains and cost-effectiveness of implantable cardioverter/defibrillators for the primary prevention of sudden cardiac death in patients with hypertrophic cardiomyopathy.

BACKGROUND: Sudden cardiac death (SCD) is a devastating complication of hypertrophic cardiomyopathy (HCM). The optimal strategy for the primary prevention of SCD in HCM remains controversial. METHODS: Using a Markov model, we compared the health benefits and cost-effectiveness of 3 strategies for the primary prevention of SCD: implantable cardioverter/defibrillator (ICD) insertion, amiodarone therapy, or no therapy. We modeled hypothetical cohorts of 45-year-old patients with HCM with no history of cardiac arrest but at significant risk of SCD (3%/y). RESULTS: Over a lifetime, compared with no therapy, ICD therapy increased quality-adjusted survival by 4.7 quality-adjusted life years (QALYs) at an additional cost of $142,800 ($30,000 per QALY), whereas amiodarone increased quality-adjusted survival by 2.8 QALYs at an additional cost of $104,900 ($37,300 per QALY). Compared with no therapy, ICD therapy would cost < $50,000 per QALY for patients (i) aged 25, with > or = 1 risk factors for SCD, and (ii) aged 45 or 65, with > or = 2 risk factors for SCD. CONCLUSIONS: An ICD strategy is projected to yield the greatest increase in quality-adjusted life expectancy of the 3 treatment strategies evaluated. Combined consideration of age and the number of risk factors for SCD may allow more precise tailoring of ICD therapy to its expected benefits.

The epidemiologic evolution and present perception of hypertrophic cardiomyopathy.

Patients with rare diseases are confronted by limited attention from the scientific community, a delayed diagnosis, a limited availability of resources and high costs of treatment. Although hypertrophic cardiomyopathy (HCM) does not meet the criteria for rare diseases, many physicians are uncomfortable with the disease, and patients still pay a price for the perceived "rarity" of HCM. In the year 2000, as part of a large research project on cardiovascular disease prevention (Progetto Cuore), the Italian Institute of Health has approved and funded a National Registry of HCM. The aims of the registry included: the collection of clinical data for HCM patients in different geographical areas; the creation of a network of cardiologists involved in the care of HCM patients; and an improved access of patients to the most advanced treatment options. It is our hope that the registry will be imitated by other countries, and that it will contribute to overcome the limitations of "rarity" in HCM research.

Five-year follow-up of Friedreich's ataxia cardiomyopathy.

Ten patients with Friedreich's ataxia were reexamined at a five-year follow-up with electrocardiography and echocardiography. The three patients who initially had had the smallest left ventricular diastolic dimensions relative to their body surface areas (decreased 23% to 25% below predicted dimensions) had undergone dilatation of their left ventricles and atria, with decreasing fractional shortening of the left ventricle, but stable to decreasing ventricular wall thickness. The other seven patients, whose ventricular diastolic dimensions were initially closer to those predicted for their body surface areas, had not undergone significant dilatation. However, their left ventricular posterior walls and interventricular septa had thickened at a mean rate of 0.019 mm/mo. The interplay of these tendencies to hypertrophy and dilatation may explain the disagreement about the type of hypertrophic cardiomyopathy in Friedreich's ataxia.