Clinical manifestations and diagnosis of rheumatic heart disease - UpToDate

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21/10/2019

Clinical manifestations and diagnosis of rheumatic heart disease - UpToDate

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Clinical manifestations and diagnosis of rheumatic heart disease Authors: Liesl Zühlke, ChB DCH FCPaeds Cert Card MPH FESC FACC MSc PhD, Ferande Peters, MBBCH FCP(SA) FESC FACC FRCP (London) Section Editor: Patricia A Pellikka, MD, FACC, FAHA, FASE Deputy Editor: Susan B Yeon, MD, JD, FACC All topics are updated as new evidence becomes available and our peer review process is complete. Literature review current through: Sep 2019. | This topic last updated: Feb 06, 2019.

INTRODUCTION Rheumatic heart disease (RHD) remains a major cause of cardiovascular disease in developing nations, although the prevalence of RHD has declined sharply in industrialized countries during the last century [1]. This topic will review the clinical manifestations and diagnosis of RHD. The management of RHD and the epidemiology, pathogenesis, diagnosis, treatment, and prevention of acute rheumatic fever are discussed separately. (See "Management and prevention of rheumatic heart disease" and "Acute rheumatic fever: Epidemiology and pathogenesis" and "Acute rheumatic fever: Clinical manifestations and diagnosis" and "Acute rheumatic fever: Treatment and prevention".)

EPIDEMIOLOGY RHD is by far the most important form of acquired heart disease in children and young adults living in developing countries (which are inhabited by 80 percent of the world’s population); RHD accounts for approximately 15 percent of all patients with heart failure (HF) in endemic countries [2,3]. A study of RHD cases estimated that in 2015, there were globally 33.4 million cases of RHD, 10.5 million disability-adjusted life-years due to RHD, and 319,400 deaths due to RHD [4]. The global mortality burden of RHD decreased by nearly 50 percent from 1990 to 2015, but the prevalence varied widely among countries and was highest in Oceania, central sub-Saharan Africa, and South Asia. Estimated age-standardized prevalence of RHD in 2015 was 3.4 cases per 100,000 https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-rheumatic-heart-disease/print?search=FEBRE REUMÁTICA&sourc…

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population in nonendemic countries and 444 cases per 100,000 population in endemic countries. Twenty countries with an endemic pattern of RHD had an age-standardized prevalence exceeding 1 percent [4]. RHD is a disease affecting predominantly those living in poverty with inadequate access to health care and unchecked exposure to group A streptococcus [5]. The impact of socioeconomic status is illustrated by a study from Kinshasa where the prevalence based on clinical examination was 22.2 per 1000 among children who lived in slums but only 4 per 1000 among children attending the city schools [6]. A later report outlined the increased risk of RHD in association with overcrowding and unemployment as well as overcrowding and distance from the nearest health center [7]. The importance of socioeconomic factors is further underscored by the virtual disappearance of RHD in industrialized countries since the mid-20th century, which started well before the introduction of penicillin. By contrast, RHD is still endemic in Africa, Asia, South America, and developing communities of Australasia [4,8-12]. Echocardiographic studies have revealed high prevalence rates in developing countries. A robust systematic review and meta-analysis calculated the prevalence of clinically silent RHD (21.1 per 1000 people, 95% CI 14.1-31.4) to be approximately seven to eight times higher than that of clinically manifest disease (2.7 per 1000 people, 95% CI 1.6-4.4), with prevalence increasing with age, from 4.7 per 1000 children (95% CI 0.0-11.2) 5 years of age to 21.0 per 1000 children (95% CI 6.8-35.1) 16 years of age; prevalence is positively associated with increasing Gini coefficient for social inequality [13]. Based on these data, it is estimated that the RHD burden could as much as double that in the Global Burden of Disease (GBD) study [4]. Based upon the premise that children in sub-Saharan Africa represent 6 to 7 percent of total global RHD burden [4], there may be an estimated 50 to 80 million persons currently affected with RHD worldwide [14]. RHD causes symptomatic valvular heart disease in children who are younger than five years of age in Africa and Asia, and the burden of disease rises with age. In South Africa, the incidence of HF due to RHD increases from 30 per 100,000 per year in the 14- to 19-year age group to over 53 per 100,000 per year in older adults, resulting in an average incidence of 23 per 100,000 per year [15]. These rates are in contrast to the incidence of rheumatic fever in developed countries of 2 metabolic equivalents. Cannot carry out activities listed above (specific activity scale III).

NYHA: New York Heart Association. References: 1. The Criteria Committee of the New York Heart Association. Nomenclature and Criteria for Diagnosis of Diseases of the Heart and Great Vessels, 9 th ed, Little, Brown & Co, Boston, 1994. p.253. 2. Campeau L. Grading of angina pectoris. Circulation 1976; 54:522. https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-rheumatic-heart-disease/print?search=FEBRE REUMÁTICA&sour…

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3. Goldman L, Hashimoto B, Cook EF, Loscalzo A. Comparative reproducibility and validity of systems for assessing cardiovascular functional class: Advantages of a new specific activity scale. Circulation 1981; 64:1227. Graphic 52683 Version 17.0

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Morphological features of rheumatic heart disease Features in the MV AMVL thickening* ≥3 mm (age-specific) ¶ Chordal thickening Restricted leaflet motion Δ Excessive leaflet tip motion during systole ◊

Features in the AV Irregular or focal thickening § Coaptation defect Restricted leaflet motion Prolapse MV: mitral valve; AMVL: anterior mitral valve leaflet; AV: aortic valve; RHD: rheumatic heart disease. * AMVL thickness should be measured during diastole at full excursion. Measurement should be taken at the thickest portion of the leaflet, including focal thickening, beading, and nodularity. Measurement should be performed on a frame with maximal separation of chordae from the leaflet tissue. Valve thickness can only be assessed if the images were acquired at optimal gain settings without harmonics and with a frequency ≥2.0 MHz. ¶ Abnormal thickening of the AMVL is age-specific and defined as follows: ≥3 mm for individuals aged ≤20 years; ≥4 mm for individuals aged 21 to 40 years; ≥5 mm for individuals aged >40 years. Valve thickness measurements obtained using harmonic imaging should be cautiously interpreted and a thickness up to 4 mm should be considered normal in those aged ≤20 years. Δ Restricted leaflet motion of either the anterior or the posterior MV leaflet is usually the result of chordal shortening or fusion, commissural fusion, or leaflet thickening. ◊ Excessive leaflet tip motion is the result of elongation of the primary chords, and is defined as displacement of the tip or edge of an involved leaflet towards the left atrium resulting in abnormal coaptation and regurgitation. Excessive leaflet tip motion does not need to meet the standard echocardiographic definition of MV prolapse disease, as that refers to a different disease process. This feature applies to only those aged