Acute Rheumatic Fever and Rheumatic Heart Disease

Dr. Scott Dougherty, Jonathan Carapetis, Liesl Zuhlke, Nigel Wilson

• Publisher: Elsevier
• Year: 2020
• ISBN:
  9780323754606
• Topic: Medicina
• Subtopic: Reumatologia, ortopedia e protesiologia

Acute Rheumatic Fever and Rheumatic Heart Disease is a concise, yet comprehensive, clinical resource highlighting must-know information on rheumatic heart disease and acute rheumatic fever from a global perspective. Covering the major issues dominating the field, this practical resource presents sufficient detail for a deep and thorough understanding of the latest treatment options, potential complications, and disease management strategies to improve patient outcomes.

• Divided into four distinct sections for ease of navigation: Acute Rheumatic Fever, Rheumatic Heart Disease, Population-Based Strategies for Disease Control, and Acute and Emergency Presentations.
• International editors and chapter authors ensure a truly global perspective.
• Covers all clinical aspects, including epidemiology, pathophysiology, clinical features, diagnosis, management, and treatment.
• Includes key topics on population-based measures for disease control for effective primary, secondary, and tertiary prevention.
• Consolidates today's available information and guidance into a single, convenient resource.

Section II

Rheumatic Heart Disease

Outline

Chapter 5. Clinical Evaluation and Diagnosis of Rheumatic Heart Disease

Chapter 6. Medical Management of Rheumatic Heart Disease

Chapter 7. Catheter-Based Evaluation and Treatment of Rheumatic Heart Disease

Chapter 8. Surgical Management of Rheumatic Valvular Heart Disease

Chapter 9. Rheumatic Heart Disease in Pregnancy

Chapter 5

Clinical Evaluation and Diagnosis of Rheumatic Heart Disease

Ari Horton, Tom Gentles, and Bo Remenyi

Abstract

The clinical diagnosis of rheumatic heart disease (RHD) is challenging. Echocardiography has greatly improved diagnostic sensitivity and specificity and should be used during diagnostic evaluation when available. This chapter outlines the clinical and echocardiographic diagnosis of RHD. It considers individuals with and without a history of acute rheumatic fever (ARF), the latter being the most common scenario globally. We describe the various manifestations of RHD and how to assess and grade the severity of valvular dysfunction while also highlighting knowledge gaps in how we evaluate multivalve and mixed-valve disease. Finally, we discuss cardiac CT and MRI, which can provide supplementary information to guide cardiosurgical management strategies.

Keywords

Aortic regurgitation; Aortic stenosis; Aortic valve; Echocardiography; Mitral regurgitation; Mitral stenosis; Mitral valve; Rheumatic heart disease

Introduction

Until recently, the diagnosis and evaluation of rheumatic heart disease (RHD) was purely clinical in resource-poor settings where RHD has remained endemic. The advent of portable echocardiographic technology, however, now means that this valuable diagnostic tool is more widely available in resource-poor settings and in remote locations, thus transforming the diagnosis of both acute rheumatic fever (ARF) and RHD. In 2012, the first evidence-based echocardiographic guidelines were published to facilitate early diagnosis of mild RHD in individuals without a previous history of ARF. ¹ This was followed by revision of the Jones criteria in 2015 to include echocardiographic findings in the diagnosis of ARF. ² The most common manifestation of RHD in adults is multivalve and mixed valve disease. Current international guidelines focus on the evaluation of advanced single valve disease with either regurgitation or stenosis as the dominant pathology. A knowledge gap exists regarding the evaluation of multivalve and mixed valve diseases that characterize chronic RHD across the age spectrum.

A detailed review of the pathogenesis of ARF and RHD is presented in Chapter 2. RHD is the only significant long-term sequela of ARF and predominantly affects the left-sided cardiac valves. ^3,4 Approximately 60% of patients who experience at least one episode of ARF will develop RHD. ^5–9 Although acute rheumatic valvulitis is often reversible, ^10–13 severe single or repeated episodes of ARF often lead to permanent scarring and chronic valvular dysfunction known as chronic RHD. ¹⁴ Myocardial impairment and dilatation occur only in the setting of severe valve disease, and recovery can be expected if timely cardiosurgical correction of valvular dysfunction takes place. ^15–17 The initial or recurrent episode of ARF may lead to a prolonged phase of inflammation and requires a long period of rest and recovery. ^11,13 The pericardial effusion seen during the acute phase of ARF usually resolves with no long-term sequelae (see Chapter 3 for a more complete discussion of the clinical features of ARF).

Epidemiology of Rheumatic Heart Disease

In 2015, an estimated 33.4 million people worldwide had RHD, resulting in 319,400 deaths and 10.5 million disability-adjusted life-years lost per annum. ¹⁸ Today, RHD predominantly affects those young people who live in marginalized communities or resource-poor settings. ^18,19 Many affected populations live in rural and remote regions, some distance from diagnostic and specialist services. ^20,21

In childhood, by far the most common lesion in RHD is isolated mitral regurgitation (Fig. 5.1). ²² By adolescence and young adulthood, mixed multivalve disease involving both the aortic and mitral valves becomes the most common manifestation (Fig. 5.1). ²² Isolated aortic valve disease occurs in children but by adulthood it is rare, being more often associated with multivalve pathology (Fig. 5.1). ²² Pure mitral stenosis can occur as young as 10 years old but it is more common during the third and fourth decades of life. ²² Chronic RHD is more common in women than men across all ages with unrecognized RHD affecting many women during their childbearing years leading to preventable morbidity and mortality for both mother and child (Fig. 5.2). ^{22, 102,103,104}

Fig. 5.1 The pattern of native rheumatic valve disease in 2475 children and adults with no percutaneous or surgical intervention. MS, mitral stenosis; MR, mitral regurgitation; MMVD, mixed mitral valve disease; AVD, aortic valve disease; MAVD, mixed aortic valve disease; MMAVD, mixed mitral and aortic valve disease.
Reproduced with permission from Zühlke et al. ²²

Diagnostic Criteria for Rheumatic Heart Disease

The diagnosis of RHD often occurs late and usually because of complications of the illness, including heart failure (HF), infective endocarditis, arrhythmias, stroke, pregnancy-related complications, or sudden death. ^22,23 A long latent phase of asymptomatic valvular heart disease, often without any preceding history or symptoms of ARF, is the most common scenario. ²² The Global Rheumatic Heart Disease Registry study (REMEDY) showed that the proportion of patients with RHD who had a previous history of ARF is 22.3% in low-income countries, 44.3% in lower-middle-income countries, and 59% of upper-middle-income...