The Natural History of Left Ventricular Geometry in the Community: Clinical Correlates and Prognostic Significance of Change in LV Geometric Pattern

ObjectivesThis study sought to evaluate pattern and clinical correlates of change in left ventricular (LV) geometry over a 4-year period in the community; it also assessed whether the pattern of change in LV geometry over 4 years predicts incident cardiovascular disease (CVD), including myocardial infarction, heart failure, and cardiovascular death, during an additional subsequent follow-up period.BackgroundIt is unclear how LV geometric patterns change over time and whether changes in LV geometry have prognostic significance.MethodsThis study evaluated 4,492 observations (2,604 unique Framingham Heart Study participants attending consecutive examinations) to categorize LV geometry at baseline and after 4 years. Four groups were defined on the basis of the sex-specific distributions of left ventricular mass (LVM) and relative wall thickness (RWT) (normal: LVM and RWT <80th percentile; concentric remodeling: LVM <80th percentile but RWT ≥80th percentile; eccentric hypertrophy: LVM ≥80th percentile but RWT <80th percentile; and concentric hypertrophy: LVM and RWT ≥80th percentile).ResultsAt baseline, 2,874 of 4,492 observations (64%) had normal LVM and RWT. Participants with normal geometry or concentric remodeling progressed infrequently (4% to 8%) to eccentric or concentric hypertrophy. Change from eccentric to concentric hypertrophy was uncommon (8%). Among participants with concentric hypertrophy, 19% developed eccentric hypertrophy within the 4-year period. Among participants with abnormal LV geometry at baseline, a significant proportion (29% to 53%) reverted to normal geometry within 4 years. Higher blood pressure, greater body mass index (BMI), advancing age, and male sex were key correlates of developing an abnormal geometry. Development of an abnormal LV geometric pattern over 4 years was associated with increased CVD risk (140 events) during a subsequent median follow-up of 12 years (adjusted-hazards ratio: 1.59; 95% confidence interval: 1.04 to 2.43).ConclusionsThe longitudinal observations in the community suggest that dynamic changes in LV geometric pattern over time are common. Higher blood pressure and greater BMI are modifiable factors associated with the development of abnormal LV geometry, and such progression portends an adverse prognosis.     

Echocardiography for Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is a genetic cardiomyopathy. The prevalence of phenotypic expression, in the absence of another systemic or cardiac disease causing increased left ventricular (LV) wall thickness, is estimated to be 1:500. The frequency of clinical presentation is far less, highlighting the need for a non-invasive diagnostic imaging tool. Echocardiography is readily available and allows for structural characterization and hemodynamic assessment of the hypertrophic heart and to screen patients at-risk for HCM, such as first degree relatives of affected individuals, and differentiate HCM from the athletic heart. Echocardiography can also be used to assess for anatomic abnormalities of the mitral valve apparatus that may exacerbate LV outflow track obstruction and to further risk stratify patients during exercise. Finally, echocardiography plays an integral role in guiding alcohol septal ablation procedures.     

The Global Burden of Aortic Stenosis

Degenerative, calcific valvular aortic stenosis (AS), caused by an active process of atherosclerosis, calcification and ossification, is the most common cause of AS in industrialized nations. The prevalence of calcific AS is age-dependent, and thus is expected to increase due to demographic aging of the global population. It is well recognized that severe AS carries a poor prognosis if left untreated. Despite this recognition, many patients are inappropriately denied surgery because of perceived risk. This article will examine the etiology, prevalence, and current trends in the treatment of degenerative AS focusing on indications for surgical aortic valve replacement.     

高血压病血压昼夜节律与左室肥厚关系的研究

目的 探讨血压负荷(BPL)与高血压靶器官损伤程度的关系,以及左室重量指数(LVMI)与动态血压(Ambulatory Blood Pressure,ABP)各参数之间的相关性。方法 采用无创性携带式动态血压监测仪对30例正常血压者,1级和2级高血压患者各25例进行了动态血压监测,同时用超声心动图检测左室重量指数。结果(1)正常组与1级高血压组之间血压负荷(SBP:4.83±2.95％vs59.75±22.12％;DBP:3.75±2.05％vs 61.75±18.24％)比较有显著性差异(P均<0.01);1级高血压组昼夜负荷差较大,呈杓型改变。2级高血压组的血压负荷(SBP:94.94±5.08％vs59.75±22.12％;DBP:91.75±10.08％vs61.75±18.24％)明显高于1级组(P均<0.01)。昼夜负荷呈非杓型改变。2级组左室肥厚(LVH)异常检出率76％明显高于1级组20％(P<0.01)。(2)高血压组LVMI与夜间SBP和DBP均显著正相关(P均<0.01),与夜间SBP和DBP下降率均显著负相关(P均<0.01)。结论 血压负荷对高血压靶器官损害程度的评价和预测有临床价值。夜间SBP与LVMI的相关性比DBP与LVMI的相关性更佳。     

The effect of enzyme replacement therapy on clinical outcomes in female patients with Fabry disease – A systematic literature review by a European panel of experts

BackgroundHeterozygous females with Fabry disease have a wide range of clinical phenotypes depending on the nature of their mutation and their X-chromosome inactivation pattern; it is therefore important to examine outcomes of enzyme replacement therapy (ERT) in the female patient population specifically. This paper presents the findings of a systematic literature review of treatment outcomes with ERT in adult female patients.MethodsA comprehensive systematic literature review was conducted through January 2017 to retrieve published papers with original data on ERT in the treatment of Fabry disease. The review included all original articles that presented ERT outcomes data on patients with Fabry disease, irrespective of the study type.ResultsClinical evidence for the efficacy of ERT in female patients was available from 67 publications including six clinical trial publications, and indicates significant reductions in plasma and urine globotriaosylceramide (GL-3) accumulation (in female patients with elevated pre-treatment levels) and improvements in cardiac parameters and quality of life (QoL). To date, data are insufficient to conclude on the effects of ERT on the nervous system, gastrointestinal manifestations, and pain in female patients with Fabry disease.ConclusionsThis review of available literature data demonstrates that ERT in adult female patients with Fabry disease has a beneficial effect on GL-3 levels and cardiac outcomes. The current evidence also suggests that ERT may improve QoL in this patient population, though further studies are needed to examine these results.     

缬沙坦与氨氯地平联合治疗中、重度高血压伴左心室肥厚的临床观察

目的：探讨缬沙坦联合氨氯地平治疗中、重度原发性高血压伴左心室肥厚的临床疗效。方法：72例中、重度原发性高血压伴左心室肥厚患者给予缬沙坦联合氨氯地平口服治疗,疗程6个月。治疗期间监测血压和心率,超声心动图检测左室舒张末期内径（LVDd）、舒张末期室间隔厚度（IVSd）、左室后壁厚度（LVPWd）、左室重量指数（LVMI）、射血分数（EF）、二尖瓣口舒张早期最大峰值流速与舒张晚期最大峰值流速比值（E／A）,并进行治疗前后对比分析。结果：患者血压较前均明显好转,总有效率93．06％;超声心动图复查IVSd、LVPWd及LVMI均降低,EF及E／A均增高,与治疗前相比差异均有统计学意义（P〈0．05）。结论：缬沙坦联合氨氯地平可以有效地降低中、重度原发性高血压及改善左心室肥厚。