- 作者列表："Rowin EJ","Maron MS","Bhatt V","Gillam L","Maron BJ
: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.
METHODS::Relapsing polychondritis is a rare multi-system disease characterized by inflammation in cartilaginous structures and other connective tissues. Cardiovascular complications occur in 10-51% of the patients. We report a case of concomitant aortic valve replacement, mitral valve replacement, and coronary artery bypass grafting in a patient with relapsing polychondritis. A 71-year-old female with relapsing polychondritis on prednisolone (5 mg/day) for 15 years presented at our hospital for further evaluation of valvular disease. Severe aortic stenosis and severe mitral regurgitation were diagnosed. We performed aortic and mitral valve replacement. During surgery, we found connective tissue surrounding the intima of the sinus of Valsalva and stenosis of the right coronary artery ostium, which was not noted on preoperative coronary angiography. We removed the tissue and performed bypass grafting to the right coronary artery. Postoperative recovery was uneventful, and she was discharged 27 days after surgery.
METHODS:BACKGROUND:The effect of significant mitral regurgitation (MR) on outcomes after continuous flow left ventricular assist device (cfLVAD) implantation remains unclear. METHODS:We performed a retrospective review of prospectively collected data from 159 patients with preoperative severe MR who underwent cfLVAD implantation (2003-2017). Two-step cluster analysis using the log-likelihood distance for post-cfLVAD implantation parameters, which included right ventricular (RV) dysfunction, MR severity, and tricuspid regurgitation (TR) severity. Post-cfLVAD implantation echocardiographic parameters were obtained within the first month. RESULTS:Cluster analysis resulted in 3 groups. Group 1 (n = 67) had mild or less MR with moderate-severe RV dysfunction (RVD). Group 2 (n = 43) had moderate-severe MR with moderate-severe RVD. Group 3 (n = 49) had moderate MR with mild RVD. Group 2 had the largest proportion with Interagency Registry for Mechanically Assisted Circulatory Support score of 1 (30.2%) and 2 (41.9%). They were more likely to undergo temporary mechanical circulatory support (18.6%) and tricuspid valve procedure (62.8%). Group 2 had the highest rate of stroke (30.2%; P = .02), hemolysis (39.5%; P = .01), device thrombosis (30%; P = .01), and worst survival (46.5%; P = .01). Survival at 5 years for groups 1, 2, and 3 were 56.0%, 17.6%, and 55.8%. Regression analysis of the entire population showed that greater MR severity after cfLVAD was associated with RV failure (P < .05; odds ratio, 1.6) and RV assist device use (P = .09; odds ratio, 1.6). After excluding tricuspid valve repairs, MR severity had a positive correlation with TR severity (R = 0.33; P < .01). CONCLUSIONS:After cfLVAD implantation, moderate-severe MR and RVD predicted RV failure. Patients with preoperative moderate-severe MR and TR coupled with moderate-severe RVD might benefit the most from mitral and tricuspid valve intervention.
METHODS:BACKGROUND:Among elderly with severe aortic stenosis (AS), Comprehensive Geriatric Assessment (CGA) originally contributed to address to transcatheter aortic valve implantation (TAVI) patients not suitable to surgical aortic valve replacement(SAVR). Nevertheless, TAVI has recently been proposed also in lower surgical risk patients. AIMS:To evaluate predictors of TAVI procedure and clinical outcomes among these patients. METHODS:For each patient ≥ 65 years with severe AS referring to our Cardiac Surgery Division, CGA was performed, including functional and cognitive status, comorbidity burden, frailty, nutritional status, gait speed, hand-grip strength and number of medications. Surgical risk was evaluated according to the Society of Thoracic Surgeons (STS) score (low-risk < 4%). Post-procedural outcomes (30-day survival and post-procedural complications) were obtained by medical records and a one-year follow-up assessed survival, and functional and cognitive performance. RESULTS:Among 154 subjects (mean age 82.9 years), 52 were at low-risk according to STS score. 32 patients were addressed to TAVI, 20 to SAVR. Variables significantly associated with TAVI-approach were lower gait speed (p 0.030) and higher number of medications (p 0.015). Short and long-term outcomes did not differ between groups. DISCUSSION:Among CGA variables, gait speed and number of medications were associated with the decision to perform TAVI instead of SAVR, even in patients at low surgical risk. 30-day and one-year survival and one-year functional and cognitive decline were similar between groups, despite a relevant prevalence of frailty in the TAVI group. CONCLUSIONS:We suggest that gait speed and number of medications should be considered in selecting appropriate candidates to TAVI among low surgical risk patients.