Hypertrophic Cardiomyopathy in "Real-World" Community Cardiology Practice.

“真实世界” 社区心脏病学实践中的肥厚型心肌病。

  • 影响因子:2.86
  • DOI:10.1016/j.amjcard.2020.01.040
  • 作者列表:"Rowin EJ","Maron MS","Bhatt V","Gillam L","Maron BJ
  • 发表时间:2020-05-01

:Differences in presentation and natural history of hypertrophic cardiomyopathy (HC) between community cardiology practice and referral centers has been a source of considerable uncertainty. We report here a cross-sectional analysis of 253 consecutive HC patients from a "real-world" clinical cardiology setting. When compared with a highly selected referral center cohort, patients in clinical practice proved to be similar with regard to disease expression such as left ventricular (LV) wall thickness, outflow obstruction, and natural history, including stable and largely benign clinical course with no or mild symptoms (61% in community practice vs. 55% in referred patients, p = 0.23), occurrence of atrial fibrillation (22% vs. 24%, p = 0.75) and nonfatal sudden death (SD) events (3% vs. 4%, p = 0.8). In contrast, progressive heart failure symptoms were most common in the referral cohort (36% vs. 26%, p = 0.04). In clinical practice, SD was prevented by prophylactic implatable cardioverter defibrillators (ICD) in 5 of 44 patients (11%), although risk was overestimated in 6 patients who were implanted with ICDs in the absence of risk markers (14%). In 16 of 61 (26%) severely symptomatic drug-refractory patients with LV outflow obstruction, recommendation for surgical myectomy (or alcohol septal ablation) was delayed. In conclusion, clinical characteristics and course of HC patients in community practice were generally similar to those in HC referral centers. Community cardiologists managed HC patients predominantly in concert with guideline-based strategies, although risk for SD could be overestimated, and the significance of outflow obstruction with timely reversal of refractory heart failure by intervention was underappreciated.


: 社区心脏病学实践和转诊中心之间肥厚型心肌病 (HC) 的表现和自然史的差异是相当大的不确定性的来源。我们在这里报告了来自 “真实世界” 临床心脏病学环境的253例连续HC患者的横断面分析。当与高度选择的转诊中心队列相比时,临床实践中的患者被证明在疾病表达方面相似,例如左心室 (LV) 壁厚度,流出道梗阻和自然史,包括稳定且基本上良性的临床过程,没有症状或轻微症状。(61% 社区实习vs. 55%,转诊病人,p  =   0.23),发生心房颤动 (房颤),差异有统计学意义 (22% vs. 24%,p  =   0.75) 和非致死性猝死 (SD) 事件 (3% 比4%,p  =   0.8).相反,在转诊队列中,进行性心力衰竭症状最常见 (36% 对26%,p = 0.04)。在临床实践中,预防性心脏复律除颤器 (ICD) 预防了44例患者中的5例 (11%) 的SD,尽管在没有风险标记的情况下植入ICD的6例患者 (14%) 的风险被高估。在61例严重有症状的药物难治性LV流出道梗阻患者中,16例 (26%) 延迟了手术肌切除术 (或酒精间隔消融术) 的建议。总之,社区实践中HC患者的临床特征和病程与HC转诊中心相似。社区心脏病专家主要根据基于指南的策略管理HC患者,尽管SD风险可能被高估,并且通过干预及时逆转难治性心力衰竭的流出道梗阻的重要性被低估.



作者列表:["Cui H","Schaff HV","Abel MD","Helder MRK","Frye RL","Ommen SR","Nishimura RA"]

METHODS:OBJECTIVE:There has been debate on the importance and pathophysiologic effects of the dynamic subaortic pressure gradient in hypertrophic obstructive cardiomyopathy. The study was conducted to elucidate the hemodynamic abnormalities associated with the dynamic pressure gradient in hypertrophic obstructive cardiomyopathy. METHODS:Eight patients with hypertrophic obstructive cardiomyopathy and 7 patients with valvular aortic stenosis underwent a detailed hemodynamic study of pressure flow relationships before and after myectomy or aortic valve replacement during operation. RESULTS:In aortic stenosis, the increased gradient after premature ventricular contraction was associated with an increase in peak flow (325 ± 122 mL/s to 428 ± 147 mL/s, P = .002) and stroke volume (75.0 ± 27.3 mL to 88.0 ± 24.0 mL, P = .004), but in hypertrophic obstructive cardiomyopathy peak flow remained unchanged (289 ± 79 mL/s to 299 ± 85 mL/s, P = .334) and stroke volume decreased (45.9 ± 18.7 mL to 38.4 ± 14.4 mL, P = .04) on the postpremature ventricular contraction beat. After myectomy, the capacity to augment stroke volume on the postpremature ventricular contraction beats was restored in patients with hypertrophic obstructive cardiomyopathy (45.6 ± 14.4 mL to 54.4 ± 11.8 mL, P = .002). CONCLUSIONS:The pressure flow relationship in hypertrophic obstructive cardiomyopathy supports the concept of true obstruction to outflow, with a low but continued flow during late systole, when the ventricular-aortic pressure gradient is the highest. Septal myectomy can abolish obstruction and restore the ability to augment stroke volume, which may explain the mechanism of symptomatic improvement after operation.

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作者列表:["Gülsaran SK","Baysal M","Demirci U","Baş V","Kirkizlar HO","Umit E","Demir AM"]

METHODS:INTRODUCTION:Ibrutinib, an oral inhibitor of Bruton's tyrosine kinase, has altered the treatment perspective of chronic lymphocytic leukemia and showed modest activity against several types of non-Hodgkin's lymphomas. According to phase studies and real-world data, reported serious adverse effects included atrial fibrillation, diarrhea, and bleeding diathesis. However, heart failure was not reported to be a probable adverse effect linked with ibrutinib. CASE REPORT:In this paper, we present a 66-year-old female chronic lymphocytic leukemia patient who developed significant and symptomatic left ventricular dysfunction at the 13th month of ibrutinib treatment. MANAGEMENT AND OUTCOME:Following cessation of ibrutinib, ejection fraction and clinical findings of the left ventricular dysfunction alleviated. DISCUSSION:Although the use of ibrutinib is generally well tolerated, cardiac functions should be monitored occasionally in all patients.

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作者列表:["Ishigaki T","Shingu Y","Katoh N","Wakasa S","Katoh H","Ooka T","Kubota S","Matsui Y"]

METHODS:OBJECTIVES:The slope in the preload recruitable stroke work relationship is a highly linear, load-insensitive contractile parameter. However, the perioperative change of the slope has not been reported before. We examined the perioperative slope from a steady-state single beat in patients with functional mitral regurgitation and assessed the correlation with brain natriuretic peptide (BNP) levels. METHODS:The study included 16 patients with non-ischemic dilated cardiomyopathy and refractory heart failure: 10 patients underwent mitral valve plasty and left ventricular plasty (MVP + LVP group) and 6 patients who underwent mitral valve replacement and papillary muscle tugging approximation (MVR + PMTA group). The left ventricular ejection fraction was assessed by the modified Simpson method; the slope was assessed by the single-beat technique using transthoracic echocardiography. BNP levels were measured by chemiluminescent immunoassay. RESULTS:The left ventricular ejection fraction and slope did not significantly change from pre- to early post-surgery in the MVP + LVP group. Both the left ventricular ejection fraction and slope significantly increased 6 months after surgery in the MVR + PMTA group. Postoperative BNP level was low in the MVR + PMTA group. While the postoperative left ventricular ejection fraction did not correlate with BNP levels, the postoperative slope significantly correlated with BNP level after surgery in the MVP + LVP group and in the total functional mitral regurgitation group. CONCLUSIONS:The change of slope was dependent on surgical procedures. In functional mitral regurgitation, the slope may be a more sensitive parameter in reflecting the left ventricular contractile function than the left ventricular ejection fraction.

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