Clinical profiles of four large pedigrees with familial dilated cardiomyopathy: preliminary recommendations for clinical practice.

OBJECTIVES: This study aimed to characterize the clinical profile of familial dilated cardiomyopathy (FDC) in the families of four index patients initially diagnosed with idiopathic dilated cardiomyopathy (IDC) and to provide clinical practice recommendations for physicians dealing with these diseases. BACKGROUND: Recent evidence indicates that approximately one-half of patients diagnosed with IDC will have FDC, a genetically transmissible disease, but the clinical profile of families screened for FDC in the U.S. has not been well documented. Additionally, recent ethical guidelines suggest increased responsibilities in caring for patients with newly found genetic cardiovascular disease. METHODS: After identification of four families with FDC, we undertook clinical screening including medical history, physical examination, electrocardiogram and echocardiogram. Diagnostic criteria for FDC-affected status of asymptomatic family members was based on left ventricular enlargement (LVE). Subjects with confounding cardiovascular diagnoses or body mass indices >35 were excluded. RESULTS: We identified 798 living members from the four FDC pedigrees, and screened 216 adults and 129 children (age <16 years). Twenty percent of family members were found to be affected with FDC; 82.8% of those affected were asymptomatic. All four pedigrees demonstrated autosomal dominant patterns of inheritance. The average left ventricular end-diastolic dimension was 61.4 mm for affected and 48.4 mm for unaffected subjects, with an average age of 38.3 years (+/- 14.6 years) for affected and 32.1 years for unaffected subjects. The age of onset for FDC varied considerably between and within families. Presenting symptoms when present were decompensated heart failure or sudden death. CONCLUSIONS: We propose that with a new diagnosis of IDC, a thorough family history for FDC should be obtained, followed by echocardiographic-based screening of first-degree relatives for LVE, assuming their voluntary participation. If a diagnosis of FDC is established, we suggest further screening of first-degree relatives, and all subjects with FDC undergo medical treatment following established guidelines. Counseling of family members should emphasize the heritable nature of the disease, the age-dependent penetrance and the unpredictable clinical course.

Managed care and outcomes of hospitalization among elderly patients with congestive heart failure.

BACKGROUND: Little was known about the impact of the health maintenance organization-managed care on patients hospitalized for congestive heart failure. Understanding this issue is important with regards to the increasing prevalence of congestive heart failure among the elderly population as well as the growing enrollment of Medicare beneficiaries in managed care. OBJECTIVE: To examine the impact of the health maintenance organization-managed care on the outcomes of hospitalization among patients with congestive heart failure. PATIENTS AND METHODS: We analyzed the Oregon hospital discharge data set. Study subjects were all patients with congestive heart failure aged 65 years or older (N=5821) discharged from hospitals in 1995 and classified into 6 insurance groups: managed care, Medicare, Medicaid, commercial or private insurance, self-pay, and other. RESULTS: The percentage of patients admitted to hospitals via emergency departments was significantly higher in the managed care patients (69%) than in other health insurance coverage groups (29.0%-58.5%; P<.001). After adjusting for age, sex, and comorbidity, the managed care patients experienced a similar length of hospital stay (3.6 days) as the commercial or private insurance patients (3.7 days; P = .67), but a shorter length of hospital stay than the Medicare patients (4.0 days; P<.001), self-pay patients (4.5 days; P<.001), and other patients (4.8 days; P<.001). No difference in the in-hospital mortality rate was seen among the insurance groups (P = .37). The readmission rate was slightly higher in managed care patients (9.1%) than in commercial insurance patients (6.8%) and Medicare patients (7.5%). The differences, however, were not statistically significant after adjusting for the confounding factors (P = .59). CONCLUSIONS: Our results suggest no association between managed care and poor short-term outcomes of hospitalization in patients with congestive heart failure. Attention, however, needs to be paid to the increased use of emergency departments by managed care patients.

Analysis of trends in hospitalizations for heart failure.

BACKGROUND: Over the past 10 years, efforts have been made to control the cost of care for patients with congestive heart failure (CHF) through reducing hospitalizations and shortening lengths of stay. Few data are available regarding the effectiveness of these intervention strategies on a community basis. METHODS AND RESULTS: We analyzed the Oregon hospital discharge database. Multivariable methods were used to assess trends while controlling for confounding factors, such as age, sex, and comorbidity. The hospital admission rates for CHF were stable over time in all age groups. The age- and sex-standardized admission rate among people aged 65 years or older decreased slightly from 13.9/1,000 in 1991 to 12.9/1,000 in 1995. The annual hospital readmission rate remained constant over time, with an average rate of 15.3%. The average length of hospital stay decreased from 5.01 days in 1991 to 3.95 days in 1995. The in-hospital mortality rate decreased from 6.9% in 1991 to 4.7% in 1995, independent of length of stay. CONCLUSION: We observed stable hospital admission and readmission rates for CHF, accompanied by a decreasing trend in the length of hospital stay and in-hospital mortality. Our findings raise the possibility of improved care management for heart failure over time.

Comparative responsiveness of Short-Form 12 and Minnesota Living With Heart Failure Questionnaire in patients with heart failure.

BACKGROUND: The Short-Form 12 (SF-12) and Living With Heart Failure Questionnaire (LHFQ) are commonly used to measure quality of life (QOL) in heart failure outcomes research. Their comparative responsiveness, however, has not been documented. METHODS AND RESULTS: A prospective cohort study was conducted among patients attending a university-based heart failure clinic between April 1997 and September 1998. All patients received comprehensive heart failure care management. QOL of patients was assessed by the SF-12 and LHFQ at baseline and 3 months. Of 87 patients completing follow-up, the mean change score was 10.1 for the LHFQ and 5.8 for the SF-12 (both Ps < .001). The change scores of the instruments were correlated (r = 0.61; P < .001). The SF-12 had a greater ability than the LHFQ to statistically detect change in physical health but was less sensitive to changes in mental health. The LHFQ performed better than the SF-12 in the ability to distinguish the differences in perceived global health transition. CONCLUSION: The LHFQ is more responsive than the SF-12 to changes in QOL. The SF-12 should not be used alone to measure the changes in QOL of patients with heart failure.

Prospective evaluation of an outpatient heart failure management program.

BACKGROUND: Although considerable effort has been devoted to the follow-up of hospitalized patients, the effectiveness and process of heart failure outpatient management have not been well demonstrated. METHODS AND RESULTS: All new patients referred to the program from April 1997 to September 1998 were followed and managed by comprehensive strategies including preemptive hospitalization. Quality of life (QOL) and patients' self-care adherence behaviors were measured at baseline, 3 months, and 6 months. Clinical outcomes were compared for the 6 months before and 6 months after referral. A total of 108 patients were recruited. Patients' self-care knowledge score was improved over time (difference score = 0.9, P <.01). The proportion of patients weighing themselves daily increased by 24% (P =.02). The proportion of patients with New York Heart Association (NYHA) class III to IV was 67.6% at baseline and 49.1% at 6 months (P =.01). Compared with 6 months before referral, the program intervention was accompanied by a 52% reduction in the risk of hospitalization for cardiovascular causes (56.1% v 27.2%, P <.001) and a 72% reduction in emergency room visits (53.6% v 14.5%, P <.01). The total hospital admissions for cardiovascular causes decreased by 59% from 94 to 39; the total emergency room visits decreased by 77% from 83 to 19. The patients' QOL was improved over time with a change score of 11.2 (P <.001) at 3 months and 10.7 (P <.001) at 6 months. CONCLUSION: Our study shows the effectiveness of this heart failure outpatient management program.