1,721,046 research outputs found

    The many faces of arterial hypertension in hypertrophic cardiomyopathy and its phenocopies: bystander, consequence, modifier

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    Arterial hypertension is the most prevalent cardiovascular (CV) risk factor worldwide, and a major preventable cause of CV, cerebrovascular and renal disease [1]. Arterial hypertension causes progressive myocardial remodeling, mainly characterized by left ventricular hypertrophy (LVH), which, once developed, strongly associates with adverse CV outcomes [2, 3]. At the molecular level, multiple alterations may be found in hypertensive hearts, including fbrosis, microvascular coronary circulation impairment, imbalance in the arterial-ventricular coupling and derangement of energetic mechanisms [2, 3]. As all these abnormalities may contribute to myocardial remodeling, it is now believed that LVH in arterial hypertension is not solely the result of an overload (haemodynamic) conditio

    The impossible quest to make cardiac amyloidosis diagnosis easy

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    Cardiac amyloidosis (CA) is an increasingly recognized myocardial infiltrative disease, causing pseudo-hypertrophy of the heart, diastolic dysfunction and heart failure. Growing efforts are ongoing to optimize screening and diagnostic algorithms for CA [1]. This is motivated by the surfacing of targeted therapies, which are supposed to be more effective the earlier the diagnosis of CA is made [2]. Echocardiography represents a fundamental methodology for the clinician to trigger the suspicion of CA [3]. Although echocardiography serves as a gatekeeper to CA diagnosis, features such as left ventricular hypertrophy (LVH) or diastolic dysfunction, which are common in CA, are frequently encountered in other more prevalent cardiovascular conditions within the ample spectrum of hypertrophic phenocopies [4,5], thus making differential diagnosis difficult. To this end, the work published by Aimo and colleagues in this issue of the European Journal of Clinical Investigation aims at simplifying the process by which a suspect of CA might be confirmed or refuted through the sole echocardiographic examination [6]. The present analysis builds on a previous recent work by the same Authors and others [7], in which two scores named "AL score" and "IWT score" were developed using differently weighted echocardiographic variables (i.e., relative wall thickness - RWT, E wave/e' wave ratio - E/e', tricuspid annular plane systolic excursion, longitudinal strain and septal longitudinal systolic apex-to-base ratio). Those scores were showed to allow a correct diagnosis of CA, respectively among patients with proven systemic light-chain amyloidosis and with unexplained LVH [7]. In the present analysis, a simplified score named "AMYLI score" is proposed, obtained by just multiplying RWT by E /e'. This new score is shown to have particularly good performance in ruling-out a diagnosis of CA when a value <2.2 is found in patients referred by hematologists and <2.36 in patients with unexplained LVH [6]. The AMYLI score is thus presented as a clinically helpful tool to this end. However, some shortcomings of the present work - and more in general, of the imaging-centric diagnostic approach to CA - should be acknowledged and deserve some discussion

    Urgent cesarean delivery following a spontaneous coronary artery dissection

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    Objective: Rare disease Background: Spontaneous coronary artery dissection is the most common etiology of pregnancy-associated myocardial infarction. It is characterized by high rates of maternal morbidity and mortality and may cause fetal complications and death as well. Case Report: A 44-year-old female (G2P1) suffered from pregnancy-related spontaneous coronary artery dissection with dissection of distal left anterior descending coronary artery. The patient was hemodynamically stable and did not required revascularization, but signs of fetal distress were detected and thus an urgent cesarean delivery was performed. This emergency procedure was undertaken in the catheterization laboratory (Cath-Lab) right after coronary angiography, thanks to a multidisciplinary team. Health conditions of the newborn were good. The patient instead suffered from a recurrence of spontaneous coronary artery dissection 6 days later, complicated by left ventricular apical thrombus and epistenocardial pericarditis. The dissection self-healed in 1 month. Conclusions: Careful evaluation of pregnancy-related spontaneous coronary artery dissection is needed to assess and manage both maternal and fetal complications. Under specific circumstances, a cesarean delivery may be required and be even performed in the Cath-Lab after coronary catheterization

    Yield of bone scintigraphy screening for transthyretin‐related cardiac amyloidosis in different conditions: Methodological issues and clinical implications

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    BACKGROUND: Transthyretin‐related cardiac amyloidosis (TTR‐CA) is thought to be particularly common in specific at‐risk conditions, including aortic stenosis (AS), heart failure with preserved ejection fraction (HFpEF), carpal tunnel syndrome (CTS) and left ventricular hypertrophy or hypertrophic cardiomyopathy (LVH/HCM). METHODS: We performed a systematic revision of the literature, including only prospective studies performing TTR‐CA screening with bone scintigraphy in the above‐mentioned conditions. Assessment of other forms of CA was also evaluated. For selected items, pooled estimates of proportions or means were obtained using a meta‐analytic approach. RESULTS: Nine studies (3 AS, 2 HFpEF, 2 CTS and 2 LVH/HCM) accounting for 1375 screened patients were included. One hundred fifty‐six (11.3%) TTR‐CA patients were identified (11.4% in AS, 14.8% in HFpEF, 2.6% in CTS and 12.9% in LVH/HCM). Exclusion of other forms of CA and use of genetic testing was overall puzzled. Age at TTR‐CA recognition was significantly older than that of the overall screened population in AS (86 vs. 83 years, p = .04), LVH/HCM (75 vs. 63, p < .01) and CTS (82 vs. 71), but not in HFpEF (83 vs. 79, p = .35). In terms of comorbidities, hypertension, diabetes and atrial fibrillation were highly prevalent in TTR‐CA‐diagnosed patients, as well as in those with an implanted pacemaker. CONCLUSIONS: Screening with bone scintigraphy found an 11–15% TTR‐CA prevalence in patients with AS, HFpEF and LVH/HCM. AS and HFpEF patients were typically older than 80 years at TTR‐CA diagnosis and frequently accompanied by comorbidities. Several studies showed limitations in the application of recommended TTR‐CA diagnostic algorithm, which should be addressed in future prospective studies
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