Epidemiology of hypertrophic cardiomyopathy-related death: revisited in a large non-referral-based patient population.

BACKGROUND: Death resulting from hypertrophic cardiomyopathy (HCM), particularly when sudden, has been reported to be largely confined to young persons. These data emanated from tertiary HCM centers with highly selected referral patterns skewed toward high-risk patients. METHODS AND RESULTS: The present analysis was undertaken in an international population of 744 consecutively enrolled and largely unselected patients more representative of the overall HCM spectrum. HCM-related death occurred in 86 patients (12%) over 8+/-7 years (mean+/-SD). Three distinctive modes of death were as follows: (1) sudden and unexpected (51%; age, 45+/-20 years); (2) progressive heart failure (36%; age, 56+/-19 years); and (3) HCM-related stroke associated with atrial fibrillation (13%; age, 73+/-14 years). Sudden death was most common in young patients, whereas heart failure- and stroke-related deaths occurred more frequently in midlife and beyond. However, neither sudden nor heart failure-related death showed a statistically significant, disproportionate age distribution (P=0.06 and 0.5, respectively). Stroke-related deaths did occur disproportionately in older patients (P=0.002). Of the 45 patients who died suddenly, most (71%) had no or mild symptoms, and 7 (16%) participated in moderate to severe physical activities at the time of death. CONCLUSIONS: HCM-related cardiovascular death occurred suddenly, or as a result of heart failure or stroke, largely during different phases of life in a prospectively assembled, regionally based, and predominantly unselected patient cohort. Although most sudden deaths occurred in adolescents and young adults, such catastrophes were not confined to patients of these ages and extended to later phases of life. This revised clinical profile suggests that generally held epidemiological tenants for HCM have been influenced considerably by skewed reporting from highly selected populations. These data are likely to importantly affect risk stratification and treatment strategies importantly for the prevention of sudden death in HCM.

Is vaginal birth after cesarean less expensive than repeat cesarean delivery?

OBJECTIVE: This study was undertaken to compare total medical costs of trial of labor after cesarean with those of elective repeat cesarean without labor, with both short- and long-term neonatal costs associated with such procedures taken into account. STUDY DESIGN: Costs associated with All Patient Refined diagnosis-related groups and Current Procedural Terminology for a large not-for-profit health care system were applied to an algorithm describing maternal and neonatal outcomes of trial of labor. Perinatal morbidity rates and cost estimates for long-term neurologic damage associated with uterine rupture were derived from published literature. RESULTS: If a 70% vaginal birth rate for women undergoing a trial of labor and delivery in a tertiary center with a mean uterine rupture to delivery time of 13 minutes is assumed, the net cost differential ranged from a saving of $149 to a loss of $217, depending on morbidity assumptions. For vaginal birth after cesarean success rates <70%, trial of labor in the presence of two previous scars, and institutional factors increasing the perinatal morbidity rate by just 4% with respect to that seen in tertiary centers, trial of labor resulted in a net financial loss to the health care system regardless of all other assumptions made. CONCLUSIONS: When costs as opposed to charges are considered and the cost of long-term care for neurologically injured infants is taken into account, trial of labor after previous cesarean is unlikely to be associated with a significant cost saving for the health care system. Recent government-mandated length-of-stay requirements are likely to make the economic benefit of vaginal birth after cesarean even less favorable. Factors other than cost must govern decisions regarding trial of labor or repeat cesarean.

Cardiac evaluation of infants. The first year of life.

Many structural congenital heart defects present at birth, and most are diagnosed by 1 year of age. Arrhythmias also are common during this time and may be particularly problematic. Identification of infants who may have a serious underlying congenital heart defect is important because many require prompt medical and surgical treatment. This article is a guide to the clinical findings that may assist the practitioner in the evaluation of infants with potential cardiac problems.

T cell antigen receptor V gene usage. Increases in V beta 8+ T cells in Crohn's disease.

Crohn's disease represents part of a spectrum of inflammatory bowel diseases characterized by immune regulatory defects and genetic predisposition. T cell antigen receptor V gene usage by T lymphocytes was investigated using four MAbs specific for various V gene products. One MAb (Ti3a), reactive with V beta 8 gene products, detected increased numbers of T cells in a subset of Crohn's disease patients as compared with normal controls and ulcerative colitis patients. In family studies there was no apparent inherited predisposition to the use of V beta 8 genes, and there was no association between a restriction fragment length polymorphism of the V beta 8.1 gene and Crohn's disease. The V beta 8+ T cells were concentrated in the mesenteric lymph nodes draining the inflammatory lesions and belonged to both the CD4+ and CD8+ T cell subsets. In contrast, lamina propria and intraepithelial T cells were not enriched in V beta 8+ T cells, suggesting that these cells were participating in the afferent limb of a gut-associated immune response. The expanded V beta 8+ T cells in Crohn's disease appear to result from an immune response to an as yet unknown antigen.

T cell receptor variable gene expression: analysis in ragweed-sensitive patients during allergen exposure.

Four monoclonal antibodies (MAb) to V region determinants of the alpha/beta-chain of the T cell antigen receptor (TCR) were used, by cytofluorography, to detect discrete populations of peripheral blood T cells (PBT). Together they identify 10-15% of circulating CD3+ T cells. Each MAb is known to detect specific V regions of the beta-chain. Thus V beta 5 gene products are recognized by MAb C37, V beta 6 by OT145, V beta 8 by Ti3a, and V beta 12 by MAb S511. In previous studies, we found that the percentages of PBT detected by these MAb show little variation over time in normal individuals. In order to determine if there is a change in TCR V gene usage during an immune response to an environmental antigen, 12 atopic patients with known ragweed sensitivity by history and skin test were followed for a 6-month period encompassing the ragweed season. No shifts in V gene usage that could be correlated with the ragweed season were consistently observed. The patients could be arbitrarily divided into two groups: in group I little variation over time was observed in the T cell populations identified by the MAb used, while group II was characterized by marked variation of the same T cell populations over time. In group II individuals, the population of Ti3a+ T cells showed the most variation over time. Failure to observe shifts in PBT subpopulations, identified by expression of different TCR V regions, during exposure to an allergen to which an IgE response has been made may mean that such shifts do not occur or that they occur primarily at the tissue site of antigen exposure and not in the peripheral circulation or that they occur in T cell subpopulations not identified by the reagents used.