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This article is available as a 'Standalone BJC Learning module' CPD activity

Background

Cardiac amyloidosis (CA) is a disorder of protein misfolding with resultant accumulation within the myocardium ultimately leading to clinical heart failure. It is subcategorised, according to the type of misfolded protein, into primary light-chain amyloidosis (AL) and transthyretin amyloidosis (ATTR). AL-CA is the result of cardiac infiltration of misfolded light-chain proteins; whereas ATTR-CA is caused by cardiac deposition of the misfolded thyroid hormone transport protein, transthyretin. ATTR-CA can be further subcategorised into hereditary (hATTR) versus wild-type (wtATTR).^1,2 Although the exact prevalence of ATTR-CA is unknown, it has become increasingly accepted as a common cause of heart failure, particularly in the elderly. Despite this knowledge, ATTR-CA remains a widely underdiagnosed cause of heart failure, and there are frequently delays to achieving the accurate diagnosis. Prompt recognition and early diagnosis of ATTR-CA is critical to decreasing morbidity and mortality, particularly as disease-modifying therapies emerge in the treatment of ATTR-CA. We present a case of ATTR-CA that remained undiagnosed until later disease stages and we will subsequently review the diagnostic evaluation of patients with suspected ATTR-CA.

This article is available as a 'Standalone BJC Learning module' CPD activity

Transthyretin amyloidosis (ATTR) is a progressive, fatal disease in which deposition of amyloid derived from either mutant or wild-type transthyretin (wtATTR) causes progressive and severe organ dysfunction. The two key clinical phenotypes are transthyretin amyloid cardiomyopathy (ATTR-CM) or transthyretin amyloid polyneuropathy (ATTR-PN), which both carry significant morbidity and mortality. Hereditary ATTR amyloidosis frequently presents with a mixed phenotype (ATTR-mixed).

ATTR-CM is an infiltrative, restrictive cardiomyopathy characterised by congestive cardiac failure, often with preserved left ventricular ejection fraction, and significant risk of conduction disease.

Treatment focuses on supportive care, reduction and ideally elimination of transthyretin (TTR) from the plasma, stabilisation of the tetramic structure of TTR and dissolution of the existing ATTR amyloid matrix.

Liver transplantation, to remove variant TTR production remains a treatment option for a select cohort of patients with hereditary ATTR-PN, although it is likely to be replaced by novel RNA-targeting therapies aimed at reducing TTR production. TTR stabilisers, such as tafamidis and acoramidis, may offer disease-modifying therapy to the majority of elderly patients with wild-type ATTR-CM. The rapidly evolving landscape of treatment options for ATTR amyloidosis, particularly among older patients with wtATTR, validates improved efforts to diagnose ATTR-CM.

This article is available as a 'Standalone BJC Learning module' CPD activity

Introduction

Hyperkalaemia (HK) in heart failure and chronic kidney disease patients limits the use of renin–angiotension–aldosterone system (RAAS) inhibitors, and successful intervention may allow patients to remain on optimal RAAS therapy. The management of HK is an established practice, but the increasing popularity of novel potassium binders may represent an effective and better-tolerated alternative compared with conventional therapy, such as sodium polystyrene sulfonate.¹

This article reviews the efficacy, and safety profile of patiromer and sodium zirconium cyclosilicate (SZC), and summarises the National Institute for Health and Care Excellence (NICE) guidance for both agents.^2,3

The world is changing or is it? Science is changing or is it?

The concept of race based on skin colour, is an entirely social construct and its harbinger, segregation and slavery, projects itself into our modern day as racism. Perhaps more recently, it is acknowledged that racism remains a clear and present danger in today’s world. It is deeply rooted within the fabric of society and can only be tackled by active and persistent engagement.

In scientific circles, what is whispered but not openly spoken about is the cumulative acts of indifference that contribute to racial disparities in healthcare within our society. This comes in the form of implicit and subconscious biases that affect healthcare allocation and worse, delivery, in the form of differential treatment of patients.¹ This is as deadly as it is silent. As clinicians and academics who contribute to healthcare, we can either pretend this doesn’t exist or we can educate ourselves, and others, to foster health equity for all.

Around 7.4 million people in the UK have heart and cardiovascular disease, coronary artery disease (CAD) being the most common type. The Driving and Vehicle Licensing Agency (DVLA) has guidance for medical professionals to aid assessment of cardiac patients with respect to driving. The guidance is different for personal, Public Carriage Office (PCO) and goods vehicles. It remains the doctors’ responsibility to advise patients of any driving restrictions, as certain cardiac conditions can limit patients’ ability to drive. This gains importance especially after certain procedures. A retrospective review of discharge summaries from electronic medical records was undertaken for a period of three months to review the number of patients getting appropriate advice. It was noted that frequently no written driving advice was recorded on discharge, neglecting an important element of patient safety. Steps were taken to counteract the lack of proper driving advice and documentation, which were effective on second review. Therefore, measures similar to ones outlined here should be put in place to ensure safe discharge and knowledge of the clinicians in accordance with the DVLA guidance.