2021;1(2) – ABC CARDIOL

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Case Report

Familial Transthyretin Cardiac Amyloidosis with Homozygous Val122Ile Mutation Mimicking Hypertrophic Cardiomyopathy

Caio Rebouças Fonseca Cafezeiro et al. Aristoteles Comte de Alencar Neto Bruno Vaz Kerges Bueno João Henrique Rissato … André Luiz Dabarian Viviane Tiemi Hotta Fábio Fernandes

Abstract

Systemic amyloidosis is a group of diseases caused by the deposition of an amyloid protein that forms fibrils and deposits in tissues. With respect to age and phenotype, patients with the Val122Ile transthyretin (TTR) mutation are similar to those with wild-type cardiac amyloidosis, causing a late-onset restrictive cardiomyopathy with minimal neuropathy, whose median age of onset is 69 years. Homozygous mutation is rare. We report the case of a male patient who had recent-onset heart failure and phenotype of hypertrophic cardiomyopathy. Technetium scintigraphy showed cardiac uptake and absence of circulating immunoglobulins, suggesting TTR cardiac amyloidosis. Genetic analysis confirmed cardiac amyloidosis caused by the homozygous Val122Ile mutant TTR protein.

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Cardiac Magnetic Resonance and Amyloidosis: How can it Assist Clinical Reasoning?

Ibraim Masciarelli F. Pinto

Evaluation of cardiac morphology

CA leads to changes in cardiac structure. In the atria, dilatation and increased thickness of the septum usually occur, while, in more advanced phases, there may be slow flow and thrombi, which, when arrhythmias are present, may be difficult to identify via CMR.

The ventricular myocardium usually shows increased thickness, typically at levels higher than those observed in patients with arterial hypertension; the levels are typically greater in forms of transthyretin amyloidosis than in light chain amyloidosis, and they may involve both ventricles.^,

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Case Report

Heart Failure Due to Cardiac Transthyretin Amyloidosis

Humberto Butzke da Motta et al. Guilherme Oliveira Magalhães Costa Andreia Biolo

Abstract

A 90-year-old male patient presented with complaints and physical examination compatible with decompensated heart failure. Electrocardiogram showed atrial fibrillation and low voltage in limb leads, and echocardiogram identified biatrial dilatation, in addition to mild increases in ventricular wall thickness and diameter. Magnetic resonance imaging confirmed suspicion of cardiac amyloidosis. With negative serum and urinary protein electrophoresis and positive pyrophosphate cardiac scintigraphy, diagnosis of transthyretin cardiac amyloidosis was confirmed. Transthyretin gene mutations were not identified on sequencing, characterizing wild-type transthyretin cardiac amyloidosis.

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Indications for Anticoagulation in Cardiac Amyloidosis

Marcelo Westerlund Montera

In cardiac amyloidosis, amyloid infiltrate at the atrial level promotes atrial dilation and contractile dysfunction as well as a higher prevalence of atrial fibrillation which, when associated with ventricular diastolic and systolic dysfunction, favors blood stasis and the consequent development of intracardiac thrombosis (ICT) and cardioembolic events.

In a study of 116 autopsies of patients with cardiac amyloidosis, conducted at the Mayo Clinic, the presence of ICT was demonstrated in 33% of hearts, with a significantly higher prevalence in patients with the AL form of amyloidosis than in those with other forms (56% versus 16%, p < 0.001).

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Disease Modifying Therapies for Transthyretin Amyloid Cardiomyopathy

Ariane Vieira Scarlatelli Macedo et al. Fábio Fernandes Renato Delascio Lopes

Transthyretin (TTR) is a tetrameric protein synthesized essentially by the liver. TTR molecules may misfold and store as amyloid fibrils in the heart and other organs, leading to TTR-related amyloidosis (ATTR) . ATTR can follow the deposition of either variant ATTR (ATTRv), which was previously known as mutant ATTR, or wild-type ATTR (ATTRwt). ATTR is an underdiagnosed disease, as well as a crucial determinant of morbidity and mortality. Cardiac involvement is common in ATTR, leading to transthyretin cardiomyopathy (ATTR-CA). ATTR-CA is a progressive disorder, and patients ultimately develop heart failure, arrhythmias, and cardiac conduction disturbances, resulting in decreased functional capacity, diminished quality of life, and death. Until recently, treatment of ATTR-CA was limited to treating symptoms and complications; however, the development of new specific therapies that delay or stop the progress of the cardiomyopathy has positively modified the outcomes . These specific treatments work by targeting the inhibition of TTR synthesis (inotersen or patisiran); tetramer stabilization (diflunisal, tafamidis, or acoramidis [AG10]); inhibition of oligomer aggregation and disruption (epigallocatechin-3-gallate); and degradation and reabsorption of amyloid fibers (doxycycline-tauroursodeoxycholic acid [TUDCA] or doxycycline-ursodeoxycholic acid [UDCA]).4

In patients with ATTR-CA, either ATTRv or ATTRwt, tafamidis is considered the agent of choice due to the proven clinical benefit in cardiovascular outcomes. Tafamidis is a kinetic TTR stabilizer that binds to the unoccupied thyroxine binding sites of tetrameric TTR and blocks the amyloidogenic cascade. It was first adopted for treatment of symptomatic ATTRv polyneuropathy, and it is the only therapy approved with effectiveness to treat ATTR-CA. This recommendation is essentially based on the results of a large, well-designed phase III randomized clinical trial (RCT), ATTR-ACT. In this study, tafamidis (20 and 80 mg, pooled) showed a decline in all-cause mortality and cardiovascular-related hospitalization, decreased decline in 6-minute walk test, slower deterioration in quality of life, and a minor increase of NT-proBNP in patients with biopsy-proven ATTRwt or ATTRv CA with heart failure and NYHA class I to III at 30 months. The safety profile was similar to placebo, and individuals with NYHA classes I and II reached the most meaningful benefit. ATTR-ACT was not designed to assess a specific dose; therefore, further analysis from ATTR-ACT and its long-term extension study support tafamidis 80 mg as the optimal dose to be used in clinical practice.

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What is the Importance of Clinical Clues, and How can we Avoid Mistakes in Following Them?

Salvador Rassi et al. Daniela do Carmo Rassi Aguinaldo Figueiredo Freitas Junior

Approximately half of patients with heart failure (HF) have preserved ejection fraction (EF), and approximately half of these patients have increased left ventricular (LV) wall thickness . This increase in LV wall thickness is usually attributed to left ventricular hypertrophy induced by arterial hypertension (AH). However, cardiac amyloid fibril infiltration also increases ventricular wall thickness, and it can lead to a clinical syndrome of HF with preserved EF (HFpEF).

Light chain amyloidosis (AL) cardiomyopathy occurs in patients with AL or primary amyloidosis, which is a hematologic disease with specific hematologic treatment. Both hereditary transthyretin (hATTR) and wild-type (ATTRwt) amyloidosis can cause transthyretin amyloid cardiomyopathy (ATTR-CM).

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Cardiac Scintigraphy with Bone Markers in Clinical Practice: When to Solicit the Exam? How to Interpret the Results?

Marcus Vinicius Simões

When to solicit cardiac scintigraphy with bone markers?

In view of the aspects mentioned above, it is important for investigation of the specific form of ATTR-CA, using bone marker scintigraphy, to begin after 2 key preliminary steps: 1. establishing high clinical suspicion of the presence of CA and 2. ruling out the presence of immunoglobulin light chains, which would lead us to diagnosis of AL-CA.

It is fundamental to establish high clinical suspicion, given that negative scintigraphy does not rule out the presence of CA in its various forms, particularly the AL form. In other words, in a significant percentage of cases with high clinical suspicion and negative scintigraphy, investigation should continue with tissue biopsy, including endomyocardial biopsy, in order to conclude diagnosis. It is clear that endomyocardial biopsy should only be performed in cases of high clinical suspicion, as it is an invasive method with risks that, although low, are inherent to the procedure.

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Genetic Testing in Amyloidosis: For Whom?

Paulo Vinicius Ramos Souza et al. Fabio Fernandes Felix José Alvarez Ramires

Over the past years, the recognition of cardiac involvement due to deposition of amyloid substances has received greater emphasis. Cardiac amyloidosis (CA), as it is known, has been shown to be a more prevalent cause of heart failure with preserved ejection fraction than in previous decades, although diagnosis remains a constant challenge.

With advances in pathophysiological knowledge of this disease, as well as relevant advances in diagnostic methods, particularly in the field of cardiovascular imaging, there has been a significant contribution to earlier identification of CA, as well as a consistent change in the natural course of the disease, given that we are witnessing the emergence of therapies that are capable of prolonging these patients’ survival.

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Case Report

Sustained Ventricular Tachycardia as an Isolated Presentation of Transthyretin Amyloidosis Cardiomyopathy – Val50Met

Edileide de Barros Correia et al. Larissa Ventura Ribeiro Bruscky Kelin Chen Priscila Cestari Quagliato … Yoná Afonso Francisco Líria Maria Lima da Silva Ana Cristina de Souza Murta

Introduction

Amyloidosis is characterized by extracellular deposition of insoluble proteins (amyloid deposition) in multiple organs and progressive organ dysfunction. It is classified by the type of protein that is deposited, including transthyretin amyloidosis (ATTR), which can occur due to a genetic variant and aging. Approximately 120 genetic variants have been recognized to cause ATTR; one of the most common is Val50Met. The phenotypic presentation of ATTR due to the Val50Met mutation is mostly neurological, known as familial amyloidotic polyneuropathy (FAP), but cardiac involvement is being increasingly recognized. Sporadic cases with strictly cardiac manifestation, causing ventricular wall hypertrophy, dysfunction diastolic, and conduction disturbances have already been described in the literature.

In this article, we report a case of ATTR due to the Val50Met mutation, with the phenotypic expression of cardiac involvement, with sustained ventricular tachycardia as an isolated presentation.

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Comprehensive Echocardiogram: What is it? For which Patients?

Marcelo Iorio Garcia

Amyloidosis occurs due to the gradual deposition of protein aggregates, which may infiltrate more than one organ, progressively evolving to organ dysfunction. Prognosis is determined by the organ involved and by the involvement of the heart; cardiac amyloidosis (CA) leads to the worst evolution. CA may be due to myocardial deposition of the protein transthyretin, which is derived from liver tissue; this condition is known as ATTR. The other form of myocardial deposition is secondary to immunoglobulin light chain proteins, which are derived from a clone of plasma cells; this is known as the systemic form or AL amyloidosis. The ATTR and AL forms need to be differentiated, because they have different treatments.

CA is a common cause of restrictive cardiomyopathy, and it should always be considered in the context of patients with heart failure with preserved ejection fraction (HFpEF). Symptoms encompass reduced effort threshold, fatigue, and edema of the lower limbs. It is common for the conduction system to be involved, and reduced voltage of the QRS complex on electrocardiogram (ECG) is one of the diagnostic clues. Evolution with arrhythmias and atrioventricular block is also characteristic.^–

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A Practical Approach to Differential Diagnosis of Cardiomyopathies with Infiltrative Phenotypes

Ligia Lopes Balsalobre Trevizan et al. Sandrigo Mangini

Introduction

Heart failure (HF) affects approximately 2% of the world population; heart failure with preserved ejection fraction (HFpEF) is responsible for approximately 50% of cases, and it has shown a progressive increase in prevalence with the aging of the population.

HFpEF is associated with high morbidity and mortality, and it is responsible for frequent hospitalizations, especially in the most elderly population. It has a complex pathophysiology and a diversified group of risk factors and etiologies. To date, few therapies have been shown to be effective in reducing cardiovascular outcomes in HFpEF; however, for specific etiologies, the possibility of disease-modifying therapeutic strategies exists.^–

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Review Article

Heart Failure with Preserved Ejection Fraction and Cardiac Amyloidosis: Implications for Treatment

Luiz Claudio Danzmann et al. João Ricardo Cambruzzi Zimmer Anna Paula Tscheika

Abstract

Heart failure with preserved ejection fraction (HFpEF) is a syndrome with multiple etiologies and varied phenotypic manifestations. Amyloidosis is a systemic disease that frequently affects the myocardium, and it may represent a specific etiology of HFpEF. The treatment of HFpEF associated with amyloidosis is not based on specific evidence, and the risk of hypotension as an adverse effect of pharmacological management is always a concern. In this context, this review addresses some strategies for controlling circulatory congestion, arrhythmias, cardiac conduction disorders, valvular heart diseases, and treatment of the specific type of amyloidosis based on available evidence as well as the latest recommendations and guidelines for managing cardiac amyloidosis.

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Review Article

How to Treat Heart Failure in Patients with Cardiac Amyloidosis

Fabiana Goulart Marcondes-Braga et al. Carlos Aurélio dos Santos Aragão

Abstract

Amyloidosis is a multisystem disease characterized by deposition of fibrillar proteins in different tissues, the heart being one of the sites of amyloid deposition. Cardiac amyloidosis may present as a restrictive syndrome with symptoms of heart failure (HF). Treatment includes prognosis-modifying medications in HF; their optimization, however, is a challenge due to the frequent adverse effects in this population. Renin-angiotensin-aldosterone system blockade should be carried out using low doses because, due to autonomic dysfunction, these drugs may cause hypotension and fatigue. The use of diuretics is necessary to maintain euvolemic status and optimized preload; however, due to the ventricular restriction present in cardiac amyloidosis, these medications should be used with caution. In patients with amyloidosis who progress to advanced HF, there are particularities in relation to indications for heart transplantation or long-term ventricular assist devices. The objective of this article is to review the treatment alternatives for HF in patients with cardiac amyloidosis.

Amyloidosis is a multisystem disease characterized by the deposition of fibrillar proteins with specific structural characteristics and a beta-pleated shape. The deposition of these proteins in the heart and the involvement of the autonomic nervous system make it challenging to manage heart failure (HF) in these patients. Given this scenario, This article aims to demonstrate how to manage HF in patients with cardiac amyloidosis.

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Editorial

ABPAR and the Needs of Patients with Amyloidosis in Brazil

Liana Cláudia Uriarte Ferronato

Revival of the ABPAR in 2011

The first reunion took place at Glaide Mara’s residence, on May 14, 2011, and a commitment was made to start promoting the association and aggregating new members. Several meetings were scheduled, raising contributions to begin with the revival of the association.

On September 18, 2011, the fifth meeting took place, according to the publication in the newspaper O Dia, the new board members of the ABPAR were elected, maintaining Roberto Beltrami as president, and Glaide was elected vice president. The bylaws of the ABPAR were registered in accordance with the new civil code.

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Original Article

Registry of Transthyretin Amyloidosis in the State of São Paulo (REACT-SP)

Fabio Fernandes et al. Caio Cafezeiro Renata Margarida do Val Alexandra Patrícia Zilli Vieira … Wilson Marques Edileide Barros Correia Alzira Alves Siqueira Carvalho Antonio Carlos Palandrini Chagas Acary Souza Bulle Oliveira Paulo Victor Sgobi de Souza Wladimir Bocca Vieira de Resende Pinto Ariane Vieira Scarlatelli Macedo Murillo Oliveira Antunes Pedro Vellosa Schwartzmann Sandrigo Mangini Marcus Vinicius Simões

Abstract

Background

Amyloidosis is a systemic disease that involves multiple organs, characterized by the deposition of amyloid fibrils. Knowledge regarding the epidemiological, clinical, and genetic profile of the population affected by amyloidosis throughout the country is of fundamental importance for establishing diagnostic and therapeutic strategies.

Objective

To evaluate the epidemiological, clinical, laboratory, imaging, and treatment variables of patients with TTR cardiac amyloidosis.

Methods

A multicenter, retrospective, prospective, and observational study based on collection of data on the natural history of patients with TTR amyloidosis, followed in the state of São Paulo.

Results

To make it possible to map the regional distribution of the disease, increasing knowledge about the disease among clinicians and specialists in different areas. To evaluate patients with hereditary and wild-type TTR amyloidosis, in addition to following individuals with positive genotype and negative phenotype.

Conclusion

The information collected may show greater awareness of the disease, development of new diagnostic and treatment flowcharts with a direct impact on knowledge of the natural history of the disease and patient prognosis.

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Editorial

Amyloidosis: Rare or Underdiagnosed Disease?

Mariano Napoli Llobera et al. Atilio Costa Vitali

Transthyretin amyloidosis

Transthyretin amyloidosis (ATTR) is caused by the deposition of transthyretin (TTR), a protein synthesized by the liver, which has a stable tetrameric structure and whose function is the transport of thyroid hormones and retinol. TTR can dissociate into monomers and oligomers and then be deposited as amyloid fibrils, in a natural (wtATTR or “wild type”, previously known as senile systemic amyloidosis) or genetic manner (hATTR or “hereditary”).

The number of patients diagnosed with ATTR has increased over the years, and it has come to be considered that it may be more prevalent than AL.

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Algorithms for Cardiac Amyloidosis Diagnosis: How to apply them in practice?

Diagnostic suspicion

The main obstacle to rapid and proper diagnosis of cardiac amyloidosis is the absence of early suspicion in patients who have clinical hints (red flags) that could – or should – trigger an investigation. This phenomenon becomes even more clear when it is documented that before the definitive diagnosis of amyloidosis or cardiac involvement due to amyloid deposition, investigation may take several months or years, usually involving many doctors from different specialties. In a study by the University of Toulouse, the average delay between the onset of symptoms and the diagnosis of amyloidosis was 8, 10 and 18 months, respectively,

for light chain amyloidosis (AL), wild-type transthyretin amyloidosis and hereditary transthyretin amyloidosis. Similarly, the number of health professionals that each patient needs to consult on their “journey” for the correct diagnosis may vary substantially. In interviews with family members and patients with amyloidosis, predominantly due to deposition

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Review Article

Cardiac Amyloidosis and Aortic Stenosis: When to Consider it and How to Treat it?

Marcelo Kirschbaum et al. Aristóteles Conte Alencar Neto Vitor Emer de Egypto Rosa Félix José Alvarez Ramires … Alexandre Antonio Cunha Abizaid Fábio Sandoli de Brito Junior Flávio Tarasoutchi Roberto Kalil Filho Fábio Fernandes

Abstract

Aortic stenosis is the most prevalent valve disease in clinical practice. The wild form of transthyretin amyloidosis has an increased incidence in elderly individuals, usually over 70 years of age. Amyloidosis and aortic stenosis are 2 diseases that affect similar populations; therefore, they may coexist in the same patient.

Patients with amyloidosis and aortic stenosis have disproportionately higher serum levels of troponin and NT-proBNP compared to patients with pure severe aortic stenosis in addition to greater ventricular hypertrophy. Therapeutic decisions for these patients must be individualized and promptly discussed by the heart team. In this article, we discuss this association and possible valve therapies, in addition to the possibility of specific treatment for amyloidosis.

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Case Report

Recurrent Syncope: Initial Presentation of Transthyretin Amyloidosis. Benefits of Disease-Modifying Treatment

Edileide de Barros Correia et al. Larissa Ventura Ribeiro Bruscky Berenice Cataldo Oliveira Valério Ana Cristina de Souza Murta … Fabiano Castro Albrecht Ibraim Masciarelli Francisco Pinto Paola Emanuela Poggio Smanio

Introduction

Syncope is a symptom that can occur in normal hearts, but it may also have multiple causes, both cardiological and neurological. It is not frequently reported as an initial manifestation of cardiac amyloidosis, but, when it occurs, it can be determined by severe atrioventricular block, ventricular tachyarrhythmia, or, as we will describe in the following case report, autonomic dysfunction and postural arterial hypotension. The objective of this study is to describe the case of a patient with transthyretin (TTR) cardiac amyloidosis, with the Val50met mutation, historically known as Val30met,, whose initial clinical presentation was syncope.

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Editorial

The Importance of the DEIC/GEMIC/SBC 2021 Position Statement on Diagnosis and Treatment of Cardiac Amyloidosis to Clinical Practice

Marcus Vinicius Simões et al. Fabio Fernandes

Cardiac amyloidosis is a frequent cause of heart failure with preserved ejection fraction

The main cardiological phenotype of CA is that of an infiltrative restrictive cardiomyopathy, whose more general aspects of symptoms, increased thickness of the ventricular walls, and concentric remodeling of the left ventricle, overlap with those of heart failure with preserved ejection fraction (HFpEF), produced by risk factors such as high blood pressure, obesity, diabetes, and aging. Thus, CA should be considered as a differential diagnosis in patients who exhibit HFpEF.

It is worth remembering that, among the different amyloidogenic proteins that can be deposited in the myocardium and cause CA, the wild type transthyretin form (ATTR-CAwt), which is closely linked to aging, is the most frequently reported in several series of specialized medical literature. This has been repeated both in large reference centers and in real world registers. Accordingly, a recent article has shown a very significant increase in CA as a cause of hospitalizations due to heart failure in several regions of the United States, between 2000 and 2012, exceeding the rate of 65 cases per 100,000 people/year, indicating that CA is not a rare disease according to the current criteria of the World Health Organization ( ).

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New Paradigms in Cardiac Amyloidosis: The Current Experience of the Northeast Region of Brazil

Tonnison Silva et al. Carlos Eduardo Lucena Montenegro Marcelo Dantas Tavares de Melo Luiz Ritt André Luiz Cerqueira Almeida

Cardiac amyloidosis (CA) is a severe and progressive infiltrative disease caused by the deposition of amyloid fibrils, with poor prognosis and limited therapeutic options. It can occur due to rare genetic variants or as a consequence of acquired conditions. Thanks to advances in imaging techniques and the possibility of obtaining non-invasive diagnosis, we now know that CA would perhaps be better classified as an underdiagnosed disease rather than as a rare one.^–

González-Lopez et al. demonstrated that, in a series of 120 patients with preserved ejection fraction heart failure, 13.3% had a diagnosis of transthyretin-associated amyloid cardiomyopathy (ATTR-CM). In patients with aortic stenosis, we found a prevalence of around 15% of ATTR-CM, and, in necropsy studies, amyloid protein deposition was found in 25% of patients over 85 years old, which shows the importance of this diagnosis in the spectrum of myocardial diseases.

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Case Report

First Combined Heart-Liver Transplant in Amyloidosis Due to Transthyretin Mutation in Brazil: Impact of the Liver on Reducing anti-HLA Antibodies

Sandrigo Mangini et al. Bianca de Cássia Sabbion Robinson Poffo Sérgio Paiva Meira Filho … Márcio Dias de Almeida Fernando Bacal

Introduction

Cardiac amyloidosis is characterized by the presence of deposits of amyloid material between myocardial fibers, leading to impaired function. In 95% of cases, it can be secondary to excessive production of abnormal light chain immunoglobulins or changes in transthyretin (TTR), either due to mutation or wild type. TTR is a protein produced mainly by the liver, and it is responsible for transporting retinol and thyroid hormone. Familial amyloidotic polyneuropathy (FAP) is an autosomal dominant genetic disease due to mutation in the TTR gene, and it has high morbidity and mortality, especially when there is cardiac involvement. More than 130 mutations have been described, and they determine phenotypes of neurological and/or cardiac predominance. Historically, liver transplantation was the standard treatment to reduce progression of FAP; in the presence of heart disease with advanced heart failure (HF), heart transplantation may be considered. Combined heart-liver transplant (CHLT), although uncommon, is a well-established indication for transthyretin amyloidosis (ATTR), with good outcomes; furthermore, the presence of the liver seems to minimize the risks of cellular or antibody-mediated rejection, which could be of interest for patients with high immunological risks, especially those with human leukocyte antigen (HLA) hypersensitivity.

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Editorial

ATTRv: a Multisystemic Disease Requires a Multiprofessional Approach

Carolina Lavigne Moreira et al. Wilson Marques Júnior

Pathogenic variants of the transtiretin gene (TTRv) result in the production of amyloidogenic fibrils that deposit in the extracellular space of many tissues, resulting in amyloidogenic multisystemic disease (ATTRv). Several tissues may be affected in different combinations, including the peripheral nerves and the cardiac system, which are usually the main targets, but also the kidneys, eyes, gastrointestinal system, central nervous system and musculoskeletal structures.

ATTRv is a progressive disease that results in death around 8 to 15 years after the onset of the disease, even more rapidly when the cardiac system is involved early. However, the natural history of the disease has changed with the introduction of treatments that, by different mechanisms, decrease the production of amyloid fibrils.

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Review Article

Ventricular Arrythmias in Cardiac Amyloidosis: It is Possible to Prevent Sudden Death?

Carlos A. Dumont et al. María Inés Sosa Liprandi

Abstract

Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy that occurs secondary to deposition of misfolded protein in the myocardium. The two most common subtypes are amyloid light chain amyloidosis and amyloid transthyretin amyloidosis. CA frequently results in congestive cardiac failure and arrhythmias, due to a disruption in cardiac substrate with subsequent electromechanical remodeling. Disease progression is usually demonstrated by development of progressive pump failure, which may be seen with a high arrhythmic burden, usually portending a poor prognosis. Arrhythmias are common, and many commonly used pharmacological therapies may be poorly tolerated and lead to clinical decompensation in this population, adding complexity to the co-management of these conditions. Studies are required to assess the risks and benefits of catheter ablation for ventricular tachycardia, with no current data showing a mortality benefit. The role of implantable cardioverter-defibrillator therapy is controversial, with benefits seen predominantly in early phases of the disease process. High-quality evidence and guideline recommendations are limited with regard to the management of arrhythmias. Providers are often left to clinical experience and expert consensus to aid in decision-making. This review summarizes both historical and contemporary data and describes evidence-based strategies for managing ventricular arrhythmias and their sequelae in patients with CA.

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