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Advances in the Treatment of Cardiac Amyloidosis.

Opinion statementCardiac amyloidosis is associated with a high mortality rate, a long delay between the first signs and the diagnosis but a short interval between diagnosis and death. This scenario has changed recently due to improved disease awareness among doctors and significant progress in diagnosis thanks to multimodal imaging and a multidisciplinary approach. Therefore, during the last few years, we have had access to specific therapies for those patients. Those therapies are quite different depending on the type of amyloidosis, but there has been real progress. Systemic light chain amyl

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… Implantable cardiac defibrillator and cardiac resynchronization therapy The role of implantable cardiac defibrillator (ICD) in cardiac amyloidosis is not well established. … Regarding cardiac resynchronization therapy (CRT), little evidence supports this indication in cardiac amyloidosis patients. …

Autonomic control of ventricular function in health and disease: current state of the art.

PurposeCardiac autonomic dysfunction is one of the main pillars of cardiovascular pathophysiology. The purpose of this review is to provide an overview of the current state of the art on the pathological remodeling that occurs within the autonomic nervous system with cardiac injury and available neuromodulatory therapies for autonomic dysfunction in heart failure.MethodsData from peer-reviewed publications on autonomic function in health and after cardiac injury are reviewed. The role of and evidence behind various neuromodulatory therapies both in preclinical investigation and in-use in clini

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… Cardiac resynchronization therapy Cardiac resynchronization therapy (CRT) has become an established non-pharmacological therapy in heart failure patients with reduced ejection fraction and ventricular dyssynchrony (predominantly in the setting of left bundle branch block), reducing mortality. …

CSI position statement on management of heart failure in India.

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A Comprehensive Review of the Management of Light-Chain (AL) and Transthyretin (ATTR) Cardiac Amyloidosis.

Cardiac amyloidosis, once considered a rare and untreatable disorder, is now increasingly recognized as a significant cause of heart failure, particularly in older adults. The two most clinically relevant subtypes of cardiac amyloidosis-immunoglobulin light-chain amyloidosis (AL) and transthyretin-related amyloidosis (ATTR)-differ in pathogenesis, natural history, and management strategies, thereby necessitating a tailored approach to diagnosis and therapy. Advances in multimodality cardiac imaging, including echocardiography, cardiac magnetic resonance, and nuclear scintigraphy, have enabled

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… As for cardiac resynchronization therapy, Donnellan et al . [ 104 ] showed that cardiac resynchronization therapy in patients with ATTR cardiac amyloidosis and an indication for a pacemaker is associated with improvements in mitral regurgitation, NYHA class, and left ventricular ejection fraction, when … Decisions regarding device implantation should be individualized with ICDs generally reserved for secondary prevention in patients with hemodynamically significant ventricular arrhythmias, while cardiac resynchronization therapy may provide symptomatic and functional improvement in selected patients …

Postoperative cognitive dysfunction: spotlight on light, circadian rhythms, and sleep.

Postoperative cognitive dysfunction (POCD) is a neurological disorder characterized by the emergence of cognitive impairment after surgery. A growing body of literature suggests that the onset of POCD is closely tied to circadian rhythm disruption (CRD). Circadian rhythms are patterns of behavioral and physiological change that repeat themselves at approximately, but not exactly, every 24 h. They are entrained to the 24 h day by the daily light-dark cycle. Postoperative CRD affects cognitive function likely by disrupting sleep architecture, which in turn provokes a host of pathological process

Internet-based device-assisted remote monitoring of cardiovascular implantable electronic devices: an evidence-based analysis.

OBJECTIVE: The objective of this Medical Advisory Secretariat (MAS) report was to conduct a systematic review of the available published evidence on the safety, effectiveness, and cost-effectiveness of Internet-based device-assisted remote monitoring systems (RMSs) for therapeutic cardiac implantable electronic devices (CIEDs) such as pacemakers (PMs), implantable cardioverter-defibrillators (ICDs), and cardiac resynchronization therapy (CRT) devices. The MAS evidence-based review was performed to support public financing decisions. CLINICAL NEED: CONDITION AND TARGET POPULATION: Sudden cardi…

Cardiac Amyloidosis: Tribulations and New Frontiers.

This review article aims to provide an overview of the pathophysiology, diagnosis, and contemporary management of cardiac amyloidosis (CA) as well as identify the knowledge gaps and areas of potential research. CA can be divided into two main groups: transthyretin cardiac amyloidosis (ATTR-CA) and light chain cardiac amyloidosis (AL-CA). The former further separates into wild-type transthyretin (ATTRwt) and hereditary transthyretin (ATTRv). African Americans, males, and people older than 75 are the most common demographics affected by this disease. Thanks to an increased understanding of this

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… In the presence of prolonged QRS either due to left bundle branch block (LBBB) or right bundle branch block (RBBB), the benefits of cardiac resynchronization therapy (CRT) has been investigated. … Cardiovascular outcomes after cardiac resynchronization therapy in cardiac amyloidosis. ESC Heart Fail. 2021;9:740–750. doi: 10.1002/ehf2.13663. 119. Cordes S., Dispenzieri A., Lacy M.Q., Hayman S.R., Buadi F.K., Dingli D., Kumar S.K., Hogan W.J., Gertz M.A. …

Diagnosis and treatment of cardiac amyloidosis: position statement of the German Cardiac Society (DGK).

Systemic forms of amyloidosis affecting the heart are mostly light-chain (AL) and transthyretin (ATTR) amyloidoses. The latter is caused by deposition of misfolded transthyretin, either in wild-type (ATTRwt) or mutant (ATTRv) conformation. For diagnostics, specific serum biomarkers and modern non-invasive imaging techniques, such as cardiovascular magnetic resonance imaging (CMR) and scintigraphic methods, are available today. These imaging techniques do not only complement conventional echocardiography, but also allow for accurate assessment of the extent of cardiac involvement, in addition t

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… For cardiac resynchronization therapy (CRT), there are no reliable data for patients with cardiac amyloidosis. …

The Multidisciplinary Heart Team in Cardiovascular Medicine: Current Role and Future Challenges.

Cardiovascular multidisciplinary heart teams (MDHTs) have evolved significantly over the past decade. These teams play a central role in the treatment of a wide array of cardiovascular diseases affecting interventional cardiology, cardiac surgery, interventional imaging, advanced heart failure, adult congenital heart disease, cardio-oncology, and cardio-obstetrics. To meet the specific needs of both patients and heart programs, the composition and function of cardiovascular MDHTs have had to adapt and evolve. Although lessons have been learned from multidisciplinary cancer care, best practices

Position Statement on the Use of Myocardial Strain in Cardiology Routines by the Brazilian Society of Cardiology's Department Of Cardiovascular Imaging - 2023.

Central Illustration : Position Statement on the Use of Myocardial Strain in Cardiology Routines by the Brazilian Society of Cardiology's Department Of Cardiovascular Imaging - 2023 Proposal for including strain in the integrated diastolic function assessment algorithm, adapted from Nagueh et al.67 Am: mitral A-wave duration; Ap: reverse pulmonary A-wave duration; DD: diastolic dysfunction; LA: left atrium; LASr: LA strain reserve; LVGLS: left ventricular global longitudinal strain; TI: tricuspid insufficiency. Confirm concentric remodeling with LVGLS. In LVEF, mitral E wave deceleration time

Between Charybdis and Scylla-an Odyssey in AL amyloidosis: insights and learnings from a narrative review and case report series.

Being "between Scylla and Charybdis" is an idiom derived from Greek mythology to mean "between a rock and a hard place" and clinicians managing amyloid light-chain (AL) amyloidosis often find themselves in this predicament. AL amyloidosis is caused by monoclonal light chains, most commonly produced by CD-38 positive plasma cells in target organs. The disease usually involves significant cardiac and/or renal involvement, but the systemic nature of the disease often leads to variable and non-specific manifestations that can critically delay early diagnosis and treatment. Here, we present a case

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… After a few cycles, his overall clinical condition substantially improved (apart from ageusia and abdominal pain); his pacemaker was upgraded, and he had cardiac resynchronization therapy implantation that improved his LV contractility. His EF was ~50% and GLS −12%. … Finally, this case suggests that VVI pacing can be harmful in some patients, particularly those with LV dysfunction and a restrictive pattern, and a personalized decision regarding cardiac resynchronization therapy pacemaker implantation is advisable. …

Diagnosis and treatment of cardiac amyloidosis: an interdisciplinary consensus statement.

The prevalence and significance of cardiac amyloidosis have been considerably underestimated in the past; however, the number of patients diagnosed with cardiac amyloidosis has increased significantly recently due to growing awareness of the disease, improved diagnostic capabilities and demographic trends. Specific therapies that improve patient prognosis have become available for certain types of cardiac amyloidosis. Thus, the earliest possible referral of patients with suspicion of cardiac amyloidosis to an experienced center is crucial to ensure rapid diagnosis, early initiation of treatmen

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… If there is an indication for pacemaker treatment in a patient for whom a high right ventricular stimulation fraction is anticipated or who already shows reduced ventricular function, implantation of a cardiac resynchronization therapy (CRT) system or HIS bundle pacing appears reasonable [ 118 ]. …

Arrhythmias, conduction disorders and sudden cardiac death in cancer patients and survivors: expert opinion of the working groups on cardio-oncology and on electrophysiology of the hellenic cardiac society.

Advances in cancer therapeutics have significantly improved patient survival; however, cardiotoxic effects-including arrhythmias-have emerged as a growing clinical concern. This expert opinion, developed by the Working Groups on Cardio-Oncology and Electrophysiology of the Hellenic Cardiological Society, aims to raise awareness of the increasing burden of arrhythmias in cancer patients and survivors. This document explores the multifactorial etiology of arrhythmias in this population, including patient-related factors, the direct effects of malignancy, and the arrhythmogenic potential of thera

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… As for the role of cardiac resynchronization therapy, it seems to remain a valuable therapeutic option in patients with chemotherapy induced cardiomyopathy fulfilling the criteria of implantation (reduced left ventricular ejection fraction ≤ 35%, New York Heart Association class II-IV HF symptoms, and … Association of Cardiac Resynchronization Therapy With Change in Left Ventricular Ejection Fraction in Patients With Chemotherapy-Induced Cardiomyopathy. JAMA. 2019;322(18):1799–805. 102. Fradley MG, Viganego F, Kip K, Martin A, Patel AA, Ismail-Khan R, et al. …

Interdisciplinary Multidimensional Assessment of Transthyretin Amyloidosis before and after Tafamidis.

BackgroundClinically, there is considerable heterogeneity in the presentation of transthyretin amyloidosis (ATTR), which ranges from primarily cardiac and primarily neurologic to mixed disease, among other manifestations. Because of this complex presentation, the diagnosis and management of patients with ATTR are often challenging and should be performed in interdisciplinary centers specialized in amyloidosis. Here, we aimed to increase awareness of ATTR detection and pathophysiology through a multidimensional multiorgan approach.Case reportWe reported on a 60-year-old man with wild-type ATTR

Cardiac amyloidosis: evolving pathogenesis, multimodal diagnostics, and principles of treatment.

Amyloidosis is a protein deposition disorder in which insoluble fibril structures accumulate in the bodily tissues damaging the organ function. Cardiac amyloidosis is a severe but under-reported medical condition characterized by the accumulation of amyloid in the extracellular area of the myocardium, which results in thickening and stiffening of ventricular walls. Cardiac amyloidosis has recently gained much attention with its slowly surging incidence. With this study, we seek to comprehensively compile the pathophysiology and clinical picture of cardiac amyloidosis subtypes, extending a clin

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… The most commonly indicated management for conduction disorder in CA is permanent pacemaker implantation and cardiac resynchronization therapy. … In a study conducted by Donnellan et al. it was observed that the implementation of cardiac resynchronization therapy led to enhancements in several aspects, including the severity of mitral regurgitation, NYHA functional class, and left ventricular ejection fraction. …

Progress and challenges in the treatment of cardiac amyloidosis: a review of the literature.

Cardiac amyloidosis is a restrictive cardiomyopathy determined by the accumulation of amyloid, which is represented by misfolded protein fragments in the cardiac extracellular space. The main classification of systemic amyloidosis is determined by the amyloid precursor proteins causing a very heterogeneous disease spectrum, but the main types of amyloidosis involving the heart are light chain (AL) and transthyretin amyloidosis (ATTR). AL, in which the amyloid precursor is represented by misfolded immunoglobulin light chains, can involve almost any system carrying the worst prognosis among amyl

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… ACEi, angiotensin‐converting enzyme inhibitor; ASO, antisense oligonucleotide; ATTR, transthyretin amyloidosis; BB, beta‐blocker; DOAC, direct anticoagulant; CRT, cardiac resynchronization therapy; ICD, implantable cardioverter defibrillator; TTR, transthyretin; TUDCA, tauroursodeoxycholic acid; VKA, … ACCF/HRS/AHA/ASE/HFSA/SCAI/SCCT/SCMR 2013 appropriate use criteria for implantable cardioverter‐defibrillators and cardiac resynchronization therapy: a report of the American College of Cardiology Foundation Appropriate Use Criteria Task Force, Heart Rhythm Society, American Heart Association, American …

Left Ventricular Hypertrophy: Etiology-Based Therapeutic Options.

Determining the etiologies of left ventricular hypertrophy (LVH) can be challenging due to the similarities of the different manifestations in clinical presentation and morphological features. Depending on the underlying cause, not only left ventricular mass but also left ventricular cavity size, or both, may increase. Patients with LVH remain asymptomatic for a few years, but disease progression will lead to the development of systolic or diastolic dysfunction and end-stage heart failure. As hypertrophied cardiac muscle disrupts normal conduction, LVH predisposes to arrhythmias. Distinguishin

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… Heart failure symptoms in patients with HCM, in the absence of LVOT obstruction, should be treated similarly to other patients with heart failure symptoms, including the consideration of advanced treatment options (e.g., cardiac resynchronization therapy, LV assist device, transplantation). … After reevaluation for symptoms, if the patients have New York Heart Association (NYHA) class II to ambulatory class IV functional capacity, left bundle branch block (LBBB), and EF < 50%, cardiac resynchronization therapy (CRT) for symptom reduction is reasonable. …

Ventricular Arrhythmia in Cancer Patients: Mechanisms, Treatment Strategies and Future Avenues.

Cardiovascular disease and cancer are the leading causes of morbidity and mortality in the US. Despite the significant progress made in cancer treatment leading to improved prognosis and survival, ventricular arrhythmias (VA) remain a known cardiovascular complication either exacerbated or induced by the direct and indirect effects of both traditional and novel cancer treatments. Although interruption of cancer treatment because of VA is rarely required, knowledge surrounding this issue is essential for optimising the overall care of patients with cancer. The mechanisms of cancer-therapeutic-i

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… Association of cardiac resynchronization therapy with change in left ventricular ejection fraction in patients with chemotherapy-induced cardiomyopathy. JAMA. 2019;322:1799–805. doi: 10.1001/jama.2019.16658. …

State of the Art of Cardiac Amyloidosis.

Cardiac amyloidosis is defined by extracellular deposition of misfolded proteins in the heart. The most frequent cases of cardiac amyloidosis are caused by transthyretin and light chain amyloidosis. This condition is underdiagnosed, and its incidence has been continuously rising in recent studies because of the aging of the population and the development of noninvasive multimodal diagnostic tools. Amyloid infiltration affects all cardiac tunics and causes heart failure with preserved ejection fraction, aortic stenosis, arrythmia, and conductive disorder. Innovative, specific therapeutic approa

Management of patients with an electrical storm or clustered ventricular arrhythmias: a clinical consensus statement of the European Heart Rhythm Association of the ESC-endorsed by the Asia-Pacific Heart Rhythm Society, Heart Rhythm Society, and Latin-American Heart Rhythm Society.

Electrical storm (ES) is a state of electrical instability, manifesting as recurrent ventricular arrhythmias (VAs) over a short period of time (three or more episodes of sustained VA within 24 h, separated by at least 5 min, requiring termination by an intervention). The clinical presentation can vary, but ES is usually a cardiac emergency. Electrical storm mainly affects patients with structural or primary electrical heart disease, often with an implantable cardioverter-defibrillator (ICD). Management of ES requires a multi-faceted approach and the involvement of multi-disciplinary teams, but

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… resynchronization therapy (CRT) may influence the occurrence of ES. … A, atrial signal; AF, atrial fibrillation; Aring, ring pole of atrial electrode; Atip, tip pole of atrial electrode; ATP, anti-tachycardia pacing; CRT, cardiac resynchronization therapy; EGM, intra-cardiac electrogram; F, ventricular fibrillation detected; ICD, implantable cardioverter-defibrillator; …