Cardioembolic stroke in cardiac amyloidosis. the real challenge lies beyond heart rhythm

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Old and new therapeutic solutions in the treatment of hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is a genetic disease of the myocardium that is relatively common in the general population, with an autosomal dominant inheritance as a genetic basis. Clinical and natural history pathways can be very different among patients with HCM. Treatment strategies have made very important advances in the last two decades, especially reducing cases of sudden death through effective risk stratification and the use of implantable defibrillators. Heart failure has become the predominant cause of morbidity and mortality in patients with HCM, being responsible for as many as 60% of disease-related deaths. HCM is most often characterized by the presence of left ventricular outflow tract (LVOT) obstruction, and this obstruction is the most frequent cause of impaired exercise tolerance in HCM and a strong independent predictor of heart failure progression and mortality. The different treatment strategies of LVOT obstruction in HCM are discussed below: surgical, invasive, and the more recent pharmacological

Cardiac metastasis presenting with an ischaemic electrocardiogram pattern mimicking anterior myocardial infarction

A cardiacmetastasis from a squamous cell carcinoma of the nasal cavities presented with an electrocardiogram showing ST-segment elevation and T-waves inversion in the anterior leads

Sudden death after valve-in-valve procedure due to delayed coronary obstruction: A case report

Background: Valve-in-valve transcatheter aortic valve implantation for degenerated aortic bioprostheses is an effective option for patients at high risk for redo surgery, even if it may be burdened by complications more common in specific settings, such as, coronary artery obstruction. Case presentation: We present the case of a Caucasic 84-year-old woman with degeneration of a previously implanted aortic Mitroflow bioprosthesis. She underwent a valve-in-valve transcatheter aortic valve implantation with a CoreValve\uae bioprosthesis. End-procedure coronary angiography demonstrated maintained perfusion of both coronary arteries. However, few hours later, she experienced sudden cardiac death. An autopsy showed that Mitroflow prosthesis leaflets were higher than the left main coronary ostium, and no other possible cause for the sudden death. Fatality was thus ascribed to left main coronary ostium obstruction due to apposition of the Mitroflow leaflet pushed upward by the late expansion of CoreValve\uae. Conclusions: Coronary artery obstruction is a frequently fatal complication which usually presents just after valve implantation, but, as reported in our case, it may also have a delayed presentation. Accurate patient's selection and intraoperative preventive measures can reduce this eventuality

Urgent cesarean delivery following a spontaneous coronary artery dissection

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

Anterior mitral valve aneurysm is an uncommon complication of aortic valve infective endocarditis: A case report

Objective: Rare disease Background: Mitral valve aneurysms (MVAs) are uncommon conditions frequently associated with aortic valve endocarditis. They may be complicated by perforation and severe mitral regurgitation (MR). Optimal treatment of MVA, and in particular the best timing for surgery, are uncertain. Case Report: A 62-year-old man with a recent history of dental surgery presented to the Emergency Department complaining relapsing fever. A first echocardiogram demonstrated infective endocarditis of the aortic valve. The patient was primarily managed with specific antibiotic therapy. Despite this, a few days later he suffered from splen-ic embolization and an MVA with MR was detected. Surgical replacement of the mitral and aortic valves was therefore performed. Conclusions: MVAs are infrequent but potentially severe complications of AV endocarditis. In the absence of definite treatment indication, the correct time for surgery should depend on concomitant clinical and infective features

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

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

Left ventricular remodeling in hypertrophic cardiomyopathy: an overview of current knowledge

While most patients with hypertrophic cardiomyopathy (HCM) show a relatively stable morphologic and clinical phenotype, in some others, progressive changes in the left ventricular (LV) wall thickness, cavity size, and function, defined, overall, as "LV remodeling", may occur. The interplay of multiple pathophysiologic mechanisms, from genetic background to myocardial ischemia and fibrosis, is implicated in this process. Different patterns of LV remodeling have been recognized and are associated with a specific impact on the clinical course and management of the disease. These findings underline the need for and the importance of serial multimodal clinical and instrumental evaluations to identify and further characterize the LV remodeling phenomenon. A more complete definition of the stages of the disease may present a chance to improve the management of HCM patients

Risk stratification in transthyretin-related cardiac amyloidosis

Transthyretin related cardiac amyloidosis (TTR-CA) is an infiltrative cardiomyopathy that cause heart failure with preserved ejection fraction, mainly in aging people. Due to the introduction of a non invasive diagnostic algorithm, this disease, previously considered to be rare, is increasingly recognized. The natural history of TTR-CA includes two different stages: a presymptomatic and a symptomatic stage. Due to the availability of new disease-modifying therapies, the need to reach a diagnosis in the first stage has become impelling. While in variant TTR-CA an early identification of the disease may be obtained with a genetic screening in proband's relatives, in the wild-type form it represents a challenging issue. Once the diagnosis has been made, in order to identifying patients with a higher risk of cardiovascular events and death it is necessary to focus on risk stratification. Two prognostic scores have been proposed both based on biomarkers and laboratory findings. However, a multiparametric approach combining information from electrocardiogram, echocardiogram, cardiopulmonary exercise test and cardiac magnetic resonance may be warranted for a more comprehensive risk prediction. In this review, we aim at evaluating a step by step risk stratification, providing a clinical diagnostic and prognostic approach for the management of patients with TTR-CA

Role of Arterial Hypertension and Hypertension-Mediated Organ Damage in Cardiotoxicity of Anticancer Therapies

Purpose of the review: Arterial hypertension (AH) is the most common cardiovascular (CV) risk factor in the community and in oncologic patients. It also represents the most important CV condition predisposing to anticancer treatment-related cardiotoxicity. This risk is heightened in the presence of cardiac AH-mediated organ damage (HMOD). Influence of AH and HMOD on the development of cardiotoxicity will be reviewed, with a focus on specific scenarios and implications for management of oncologic patients. Recent findings: Not adequately controlled AH before or during anticancer treatments and/or development of AH during or after completion of such therapies have detrimental effects on the clinical course of oncologic patients, particularly if HMOD is present. As overlooking CV health can jeopardize the success of anticancer treatments, the goal for clinicians caring for the oncologic patient should include the treatment of AH and HMOD