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After the recent celebrations of the 50th anniversary of the modern description of hypertrophic cardiomyopathy (HCM) by Teare and Lord Brock, the time is ripe to reflect on what remains to be discovered. With the full realization ...
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After the recent celebrations of the 50th anniversary of the modern description of hypertrophic cardiomyopathy (HCM) by Teare and Lord Brock, the time is ripe to reflect on what remains to be discovered. With the full realization that a massive amount of information relating to the disease has already been uncovered, and paying tribute to all those involved in this process, it is essential to concentrate on the gaps in our knowledge that require concerted efforts to advance the field, particularly in relation to patient management, which continues to be perceived as less than optimal. We believe that this is largely due to the partial disconnect between basic research, and an incomplete understanding of the fundamental mechanisms molding a continuously, often insidiously changing phenotype. A thorough comprehension of these processes requires a translational approach based on long-term clinical observation of large HCM cohorts, coupled with basic scientific research, and represents an essential step toward the development of innovative therapies which need to be both disease- and patient-specific.
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The presence of intraventricular obstruction is a powerful predictor of outcome in patients with hypertrophic cardiomyopathy (HCM) and, when associated with severe, drug-refractory symptoms, should be managed aggressively. Resting...
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The presence of intraventricular obstruction is a powerful predictor of outcome in patients with hypertrophic cardiomyopathy (HCM) and, when associated with severe, drug-refractory symptoms, should be managed aggressively. Resting left ventricular outflow obstruction is found in approximately 20% of the patients, classically occurs at the subaortic level, and is associated with mitral valve systolic anterior motion (SAM). In a minority of patients, however, the impedance to flow occurs at midventricular level, unrelated to SAM. Symptomatic midventricular obstruction represents a clinical challenge, and its treatment is not standardized. In these patients, both surgical myectomy and alcohol septal ablation (ASA) are technically feasible. A rational approach to the management of these patients depends on accurate characterization of the pathophysiology, coupled with comparison of the results of different management strategies. To illustrate these points, the details of a patient who first underwent percutaneous ASA and subsequently required redo surgical treatment are described here, with special emphasis on the implications to the management of midventricular obstruction, as well as to the more global issue of obstructive HCM.
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In hypertrophic cardiomyopathy, the plasma levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) correlate with functional capacity. However, their prognostic relevance remains unresolved. We followed up 183 stable outpat...
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In hypertrophic cardiomyopathy, the plasma levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) correlate with functional capacity. However, their prognostic relevance remains unresolved. We followed up 183 stable outpatients with hypertrophic cardiomyopathy (age 50 ± 17 years, 64% men) for 3.9 ± 2.8 years after NT-proBNP measurement. The primary end point included cardiovascular death, heart transplantation, resuscitated cardiac arrest, and appropriate implantable cardioverter-defibrillator intervention. The secondary end point (SE) included heart failure-related death or hospitalization, progression to end-stage disease, and stroke. The median NT-proBNP level was 615 pg/ml (intertertile range 310 to 1,025). The incidence of the primary end point in the lower, middle, and upper tertiles was 0%, 1.3%, and 2.1% annually, respectively (overall p = 0.01). On multivariate analysis, the only independent predictors of the primary end point were NT-proBNP (hazard ratio for log-transformed values 5.8, 95% confidence interval 1.07 to 31.6; p = 0.04) and a restrictive left ventricular filling pattern (hazard ratio 5.19, 95% confidence interval 1.3 to 21.9; p = 0.02). The NT-proBNP cutoff value of 810 pg/ml had the best sensitivity for the primary end point (88%), but the specificity was low (61%). The incidence of the SE in the lower, middle, and upper NT-proBNP tertiles was 4.6%, 12.0%, and 11.2% annually, respectively (overall p = 0.001). An NT-proBNP level of <310 pg/ml was associated with a 75% reduction in the rate of SE compared with a level of ≥310 pg/ml (hazard ratio 0.25, 95% confidence interval 0.11 to 0.57; p = 0.001), independent of age, left ventricular outflow tract obstruction, or atrial fibrillation. In conclusion, in stable outpatients with hypertrophic cardiomyopathy, plasma NT-proBNP proved a powerful independent predictor of death and heart failure-related events. Although the positive predictive accuracy of an elevated NT-proBNP level was modest, low values reflected true clinical stability, suggesting the possibility of avoiding or postponing aggressive treatment options.
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Hypertrophic cardiomyopathy (HCM) is an epidemiologically relevant, worldwide spread condition which is frequently perceived as a rare disease. This misconception might be supported by some characteristics of HCM such as its incom...
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Hypertrophic cardiomyopathy (HCM) is an epidemiologically relevant, worldwide spread condition which is frequently perceived as a rare disease. This misconception might be supported by some characteristics of HCM such as its incomplete penetrance and variable age at onset and by the fact that many patients remain asymptomatic for a long course of the disease and are thus unlikely to seek for medical evaluation. Multiple evidences suggest that early diagnosis of HCM is important, not only because it allows the patients to be addressed to appropriate diagnostic work-out and to adequate therapeutical options but because it may trigger the screening of family members with the potential of further, new diagnosis of HCM in previously unsuspected individuals. Increased awareness of the disease among physicians working in community-based hospitals and in outpatients facilities, and a facilitated communication and access to tertiary referral centers, will result into a wider knowledge of the spectrum of the disease, a better access to the state-of-the-art management options for patients, and to a more diffuse practice of genetic evaluation of HCM families.
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BACKGROUND: A 10-year-old boy presented with a history of severe angina on exertion. A two-dimensional echocardiogram showed mild asymmetric left ventricular (LV) hypertrophy localized to the interventricular septum, consistent wi...
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BACKGROUND: A 10-year-old boy presented with a history of severe angina on exertion. A two-dimensional echocardiogram showed mild asymmetric left ventricular (LV) hypertrophy localized to the interventricular septum, consistent with nonobstructive hypertrophic cardiomyopathy. A maximal treadmill exercise test was terminated early owing to marked downsloping of the ST-T segment on all precordial leads, associated with mild chest discomfort. Cardiac MRI and coronary angiography showed that the left anterior descending (LAD) artery was 'tunneled' from its origin to the junction of the middle and lower segments, causing systolic obliteration. PET showed diffusely blunted myocardial blood flow after dipyridamole infusion. A beating-heart technique was used to perform surgical mobilization of the superficial and lateral surfaces of the LAD artery. The patient was free from angina at 6 months after surgery. A repeat exercise test showed considerable improvement in exercise tolerance, which was associated witha marked decrease in ST-T changes on exertion. INVESTIGATIONS: Physical examination, laboratory tests, 12-lead electrocardiography, two-dimensional echocardiography, exercise testing, cardiac MRI, coronary angiography, PET, Holter electrocardiographic monitoring. DIAGNOSIS: Angina caused by extensive myocardial tunneling of the LAD artery in nonobstructive hypertrophic cardiomyopathy. MANAGEMENT: Bisoprolol therapy and surgical mobilization of the tunneled LAD artery.
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Subcutaneous ICD (S-ICD) is a promising option for Hypertrophic Cardiomyopathy (HCM) patients at risk of Sudden Cardiac Death (SCD). However, its effectiveness in terminating ventricular arrhythmias in HCM is yet unresolved.
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Whether treatment with β blockers (BBs) is of benefit to patients with hypertrophic cardiomyopathy (HC) and provocable outflow obstruction (with none or with only mild heart failure symptoms) is largely unresolved. Thus, we prosp...
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Whether treatment with β blockers (BBs) is of benefit to patients with hypertrophic cardiomyopathy (HC) and provocable outflow obstruction (with none or with only mild heart failure symptoms) is largely unresolved. Thus, we prospectively studied 27 patients with HC (age 36 ± 15 years; 81% men) with New York Heart Association class I or II, without obstruction at rest, but with exercise-induced left ventricular outflow tract (LVOT) gradient of <30 mm Hg. Patients underwent exercise echocardiography at baseline and after treatment with nadolol (n = 18; 40 to 80 mg/day) or bisoprolol (n = 9; 5 to 10 mg/day), according to a prespecified protocol. Without the BBs, the postexercise LVOT gradient was 87 ± 29 mm Hg and >50 mm Hg in 25 patients (93%). After a 12 ± 4-month period of BB treatment, the postexercise LVOT gradient had decreased to 36 ± 22 mm Hg (p <0.001) and was virtually abolished (to 0 or <30 mm Hg) in 14 patients (52%), substantially blunted (<20 mm Hg reduction) in 9 (33%), and unchanged in only 4 (15%). Severe postexercise obstruction (range 58 to 80 mm Hg) persisted in 6 patients (22% compared to 93% without BBs; p <0.001). Nonresponders (residual postexercise gradient of <30 mm Hg with BBs) were characterized by an increased body mass index (hazard ratio 2.03/1 kg/m 2, 95% confidence interval 1.2 to 3.4; p <0.05). In conclusion, in patients with HC with mild or no symptoms, treatment with BBs can prevent the development of LVOT obstruction triggered by physiologic exercise. These findings provide a rationale for the novel strategy of early prophylactic pharmacologic treatment with standard, well-tolerated doses of BBs in physically active patients with provocable gradients, aimed at effective prevention of the hemodynamic burden associated with dynamic obstruction.
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OBJECTIVES: The relevance of iatrogenic left coronary artery fistulas complicating surgical myectomy in patients with hypertrophic cardiomyopathy is not known. We prospectively defined the echocardiographic features, prevalence, a...
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OBJECTIVES: The relevance of iatrogenic left coronary artery fistulas complicating surgical myectomy in patients with hypertrophic cardiomyopathy is not known. We prospectively defined the echocardiographic features, prevalence, and clinical significance of left coronary artery fistulas in 40 consecutive patients with hypertrophic cardiomyopathy undergoing extended septal myectomy. METHODS: Echocardiographic analysis was performed preoperatively and 1 and 6 months after surgical intervention. Diagnosis of left coronary artery fistulas required evidence of diastolic flow draining from the left ventricular wall into the left ventricular cavity according to prespecified criteria. RESULTS: Left coronary artery fistulas were detected in 9 (23%) of the 40 study patients as a single occurrence in all except 1 patient, who had multiple fistulas. At 6 months, left coronary artery fistulas could still be detected in only 2 of the 9 patients. Of these, 1 patient remained asymptomatic but continued to show left coronary artery fistula persistence at 37 months postoperatively. The other, a woman with prior alcohol septal ablation, had progressive severe symptoms that required percutaneous closure of the fistula with a covered stent after angiographic identification of a large first septal branch fistula associated with distal left anterior descending coronary artery steal. CONCLUSIONS: In patients with hypertrophic cardiomyopathy, left coronary artery fistulas are common in the early period after surgical myectomy, although their echocardiographic prevalence is dependent on operator awareness. Most left coronary artery fistulas heal spontaneously. Occasionally, however, fistulas can persist and cause symptoms requiring therapeutic intervention.
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Contemporary therapeutic options have led to substantial improvement in survival of patients with heart failure. However, limited evidence is available specifically on idiopathic dilated cardiomyopathy. We thus examined changes in...
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Contemporary therapeutic options have led to substantial improvement in survival of patients with heart failure. However, limited evidence is available specifically on idiopathic dilated cardiomyopathy. We thus examined changes in prognosis of a large idiopathic dilated cardiomyopathy cohort systematically followed during the past 30 years.From 1977 to 2011, 603 consecutive patients (age, 53±12 years; 73% men; left ventricular ejection fraction, 32±10%) fulfilling World Health Organization criteria for idiopathic dilated cardiomyopathy, including negative coronary angiography, were followed up for 8.8±6.3 years. Patients were subdivided in 4 enrollment periods on the basis of heart failure treatment eras: (1) 1977-1984 (n=66); (2) 1985-1990 (n=102); (3) 1991-2000 (n=197); (4) 2001-2011 (n=238). Rates of patients receiving angiotensin-converting enzyme inhibitors/angiotensin receptors blockers, β-blockers, and devices at final evaluation increased from 56%, 12%, 8% (period 1) to 97%, 86%, 17% (period 4), respectively (P<0.05). There was a trend toward enrollment of older patients with less severe left ventricular dilatation and dysfunction during the years. During follow-up, 271 patients (45%) reached a combined end point including death (heart failure related, n=142; sudden death, n=71; and noncardiac, n=22) or cardiac transplant (n=36). A more recent enrollment period represented the most powerful independent predictor of favorable outcome {period 2 versus 1 (hazard ratio [HR], 0.64; P=0.04), period 3 versus 1 (HR, 0.35; P<0.001), period 4 versus 1 (HR, 0.14; P<001)}. Each period was associated with a 42% risk reduction versus the previous one (HR, 0.58; 95% confidence interval, 0.50-0.67; P<0.001), reflecting marked decreases in heart failure-related mortality and sudden death (period 4 versus 1: HR, 0.10; P<001 and HR, 0.13; P<0.0001, respectively).Evidence-based treatment has led to dramatic improvement in the prognosis of idiopathic dilated cardiomyopathy during the past 3 decades. The benefits of controlled randomized trials can be replicated in the real world, emphasizing the importance of tailored follow-up and long-term continuity of care.
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The first use of immunoglobulin therapy, historically, was in 1890 when Emil von Behring developed effective antiserum against diphtheria toxin, but only in the early 1970s technological advancements in the fractionation of plasma...
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The first use of immunoglobulin therapy, historically, was in 1890 when Emil von Behring developed effective antiserum against diphtheria toxin, but only in the early 1970s technological advancements in the fractionation of plasma lead to the production of Ig preparations which could be administered intravenously. Intravenous Ig products are a mainstay for disorders such as: primary immunodeficiency, serious infections, autoimmune and inflammatory disorders. During autoimmune and systemic inflammatory disease IVIg exhibits a number of immune modulatory activities such as: Fc Receptor-mediated effects, modulation of complement, modulation of cytokine production, superantigens neutralization, antibodies neutralization by idiotype network, increased catabolism of IgG, but also biologic effects of other molecules present in IVIg preparations. Recent understanding about IVIg composition and mechanism of action can explain its therapeutic effect in autoimmune and inflammatory disorders. Nevertheless it is important to underline that IVIg is a heterogeneous product and it is difficult to determine the exact mechanism of its activities in every disease. The increased use of IVIg in the treatment of autoimmune disorders outlined the issue of tolerability. Undesiderable effects to IVIg occurs in less than 5% of patients.
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