case study rheumatic heart disease

An official website of the United States government

The .gov means it’s official. Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

The site is secure. The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

• Publications
• Account settings

Preview improvements coming to the PMC website in October 2024. Learn More or Try it out now .

• Advanced Search
• Journal List
• BMJ Case Rep

Case Report

Rheumatic fever with severe carditis: still prevalent in the south west pacific, taran kaur nandra.

1 Medicine, King's College London, London, UK

Nigel J Wilson

2 Auckland City Hospital, Auckland, New Zealand

John Artrip

Bruno pagis.

3 Centre Hospitalier de la Polynesie Francaise, Papeete, French Polynesia

Rheumatic heart disease (RHD) has a worldwide prevalence of 33 million cases and 270 000 deaths annually, making it the most common acquired heart disease in the world. There is a disparate global burden in developing countries. This case report aims to address the minimal RHD coverage by the international medical community. A Tahitian boy aged 10 years was diagnosed with advanced heart failure secondary to RHD at a local clinic. Previous, subtle symptoms of changes in handwriting and months of fever had gone unrecognised. Following a rapid referral to the nearest tertiary centre in New Zealand, urgent cardiac surgery took place. He returned home facing lifelong anticoagulation. This case highlights the RHD burden in Oceania, the limited access to paediatric cardiac services in countries where the RHD burden is greatest and the need for improved awareness of RHD by healthcare professionals, and the general public, in endemic areas.

Case presentation

Patient A, a normally fit Tahitian boy aged 10 years, presented to his community clinic with a 1-week history of fever, worsening breathlessness and nausea leading to loss of appetite in August 2016. On clinical examination, he was sweating, tachycardic and a murmur of grade 3/6 was noted. He also stated he had a sore throat 3 months previously, which was dismissed as trivial due to its intermittent nature. His mother highlighted a rash on the upper aspects of both thighs. He had intermittent hip and knee pain for the 12 months, resulting in limping and reduced mobility—emphasised by the fact he was visiting his neighbour much less than usual. He commented on a 3-month history of a change in handwriting at school, and a greater tendency to drop objects, suggestive of a subtle chorea. 1 There was no significant medical history. The birth history includes a 2-week stay in hospital after birth due to ongoing fevers. The social history includes residing with three other family members in a two-bedroom apartment; however, there are occasions when family members stay for periods of time and the household can exceed nine persons. Overcrowding is a risk factor for developing acute rheumatic fever (ARF), as there is increased risk of transmission of group A streptococcus. 1 Patient A's mother provides the primary household income; she left school at 20 years and works in a manual occupation, emphasising rheumatic heart disease (RHD) as a disease of poverty.

He was referred to a regional adult cardiologist in French Polynesia (FP) 3 days after his presentation to the local clinic. The cardiology team performed ECG that detected sinus tachycardia (128 bpm) and left atrial (LA) and left ventricular (LV) hypertrophy. Transthoracic echocardiography (TTE) revealed severe mitral-aortic disease with predominant mitral incompetence and pulmonary arterial hypertension. There was evidence of LA and LV dilation, with preserved ejection fraction. Blood cultures were negative. The primary differential diagnosis was ARF meeting the Jones criteria 2 with carditis, raised inflammatory markers, temperature and elevated group A streptococcal titres. Following a diagnosis of ARF and secondary heart failure, he was started on steroids, diuretics and penicillin and placed on bed rest. Given the advanced carditis found on the TTE, a semiurgent referral was made to Starship Children's Hospital, New Zealand, which provides tertiary cardiac care for children in the South Pacific.

Three weeks after presentation to his local clinic, patient A arrived in Auckland. His weight was 54 kg (>95th centile) and height 154 cm (>94th centile). Initial observations indicated lethargy and heavy sweating, with symptoms of sore throat and nausea. There were no signs of finger clubbing or infective endocarditis with a regular radial pulse. The jugular venous pulse was 2 cm elevated. On auscultation, there was a hyperdynamic praecordium with a displaced apex beat to the sixth intercostal space, in the midclavicular line. There was a parasternal heave and a pansystolic murmur, radiating to the axilla. A diastolic rumble was audible at the lower left sternal edge (LLSE), with diastolic murmur over the aortic area. There was reduced air entry at the lung bases. The abdomen examination revealed right upper quadrant tenderness. The liver edge was palpable 2 cm below the right subcostal margin. Initial blood test results showed haemoglobin 89 g/L, ESR 114 mm in 1 hour and CRP 178 mg/L. The antistreptolysin O titre was 885 IU/mL (normal limit 240 IU/mL) and anti-DNAse antibodies were 1200 U/mL (normal limit <680 U/mL). Peripheral blood cultures showed no growth. Transoesophageal echocardiography replicated the findings of the TTE that was performed in FP 2 weeks earlier: the mitral valve showed mixed stenosis and regurgitation. The mitral valve was thickened and the posterior mitral valve leaflet restricted in motion. There was a visible coaptation defect with severe mitral regurgitation. Severe aortic regurgitation was also present. There was dilation of the LA and LV; LVEDD 6.6 cm with Z score +5.1 and LVEDS 4.4 cm with Z score +4.4. The ejection fraction was preserved. The diagnosis of ARF with carditis seemed secure; a differential diagnosis of infective endocarditis was eliminated by negative blood cultures.

Cardiac surgery took place 2 weeks after arrival at Starship and was indicated due to continued cardiac failure and pulmonary hypertension. The valves were not amenable to valve repair with the mitral valve showing acute and chronic changes. He received a size 25 mm On-X prosthetic mitral valve replacement and a size 21 mm On-X prosthetic aortic valve replacement and underwent a tricuspid valve annuloplasty. There were no perioperative complications. Postoperative TTE indicated LV modelling with LV dysfunction; this is expected to improve in time. The prosthetic aortic and mitral valves were functioning normally, with a mild prosthetic valve gradient. Postoperative management included optimising pain relief through a patient-controlled analgesia device, drug treatment for congestive heart failure with spironolactone, furosemide and captopril, improving compliance with drug treatments (eg, exchanging paracetamol tablets for syrup) and physiotherapy/play therapy. He was started on anticoagulation with Coumadin, a warfarin substitute used in FP, and continued on intramuscular benzathine penicillin every 28 days as secondary prevention for ARF. He was discharged from hospital after 7 days and followed in outpatients for INR control before returning to FP after 4 weeks.

Global health problem list

• Awareness of signs and symptoms of ARF
• Missed opportunity to diagnose ARF earlier as hip and knee pain symptoms seemed minor to the family. Symptoms and signs of Sydenham's chorea can very subtle.
• Disease burden of RHD in Oceania
• Access to cardiac surgery in for those in remote regions and lower middle-income countries (LMIC)

Global health problem analysis

RHD is the most common acquired heart disease in the world, with a disparate global burden affecting children in developing countries. 3 In wealthier countries, the burden of RHD tends to be confined to indigenous populations, such as the Aborigine population of Australia and the Maori population of New Zealand. 4 The 2013 Global Burden of Diseases study estimates a worldwide prevalence of 33 million cases, causing 9 million disability-adjusted life years lost and 270 000 deaths annually. 5 In 2016, the ARF rate in FP was 42/100 000, though it is unclear how the various cultures in FP, for example, Maori, Cook islanders and Samoans, contribute to this. The global health problems associated with RHD are: the disproportionate burden in LMIC, delay in the recognition of ARF/RHD and limited access to cardiac surgery for ARF/RHD. RHD has minimal reportage from the worldwide medical community; this report is an effort to raise awareness of a disease conferring immense cardiovascular mortality and morbidity in childhood and young adulthood.

What is rheumatic fever?

Rheumatic fever is caused by a group A β haemolytic streptococcal infection, and initially presents as a sore throat. Approximately 2–4 weeks later, symptoms manifest as fever, polyarthralgia, polyarthritis, chorea and erythema marginatum. 2 Acute rheumatic carditis occurs when there is a cross-reaction between the bacterial carbohydrate cell wall and the valve tissue (antigenic mimicry), causing chronic damage to valve tissue, 6 which is then termed RHD. Risk factors include poverty, overcrowding, lack of access to medical care and young age. 7

Disease burden and recognition of ARF in FP and LMIC

FP, population 270 000, is a collection of 118 Pacific islands—one of which is Tahiti, where our patient is from. The WHO estimates RHD incidence in FP school children at 8/1000. 8 An ongoing rise in incidence has been attributed to improved record keeping. 9 Despite the ease of prophylaxis, delayed symptom recognition by professionals and patients (as with our case), and poor antibiotic compliance have been linked to the existence of this preventable disease. 10

Another country in Oceania with a high RHD prevalence is Fiji; a recent study of RHD-associated deaths there found that RHD is responsible for a significant premature loss of human life. 10 The 50% RHD mortality under 40 years in Fiji directly affects economic productivity in young adulthood, 10 the age group on which economies thrive. A particularly poignant case by Steer et al 11 indicates the importance of healthcare professional training to recognise RHD in Fiji. A Fijian girl aged 7 years died from RHD-associated severe valve deformation, after being misdiagnosed 2 years earlier with asthma. The nurse who recognised her symptoms on her last, fatal presentation had attended an RHD workshop the previous day. 11 In a similar presentation to patient A, joint pains and irregular movements went undiagnosed, despite ongoing fevers for several preceding months. Irregular movements can manifest as changes in handwriting, 1 which our patient also commented on. His mother was not aware of this, as ARF symptoms can be subtle unless arthritis is severe. This suggests improved community ARF education is needed to enable early presentation to healthcare services; in our case, the patient's mother could have recognised symptoms earlier. Furthermore, healthcare physicians and nurses from Europe working abroad are not aware of, or have not seen ARF cases, hence training may be required to enable symptom recognition as in the Steer et al case.

The international RHD burden in LMIC is also worth noting; the Global Rheumatic Heart Disease Registry (the ‘REMEDY’ study) found the average age of an individual in Africa to be affected by RHD is 28 years, with over 66.7% women. 12 The disparate burden on women's health in the reproductive age illustrates the need for adult and paediatric RHD services and improved obstetric care, especially as RHD is a major cause of maternal death in Africa. 13

Access to cardiac surgery

The access to cardiac surgery and postoperative care in geographically remote regions (as for our patient) and in LMIC is notoriously difficult. Despite free public clinics, some FP regions on outer islands have only a nurse or a health assistant for primary healthcare. There is no paediatric cardiac surgeon on site in Tahiti, but close communication between the island doctors and colleagues in New Zealand meant patient A's treatment plan was agreed via email correspondence. He was booked on a flight to New Zealand with a medical escort (BP, personal communication). Although patients in FP are fortunate to have a health system that offers transfer to nearby countries, this is not an international standard. In Western countries, there is 1 congenital heart surgeon per 3.5 million inhabitants, whereas in Africa, services vary from 1 per 35 million 14 to 2 paediatric cardiologists serving an entire country. 15 It has been recommended as a ‘global priority’ that an improved multidisciplinary approach to valve repair requires specialist surgeons, high-quality echocardiography and maintenance of therapy in the local populations. 16

Reduced access to cardiac care is exacerbated by the postoperative need for anticoagulation. Children with RHD often have damaged valvular tissue, and surgeons make it a priority to repair rather than replace valves, 16 as 40% of patients from LMIC do not adhere to anticoagulation guidance after valve replacement. 17 The REMEDY study 12 demonstrated that if valve repair is not possible, cardiac centres use tissue rather than mechanical valve replacement to reduce postoperative thrombotic risk. 18 It is not clear whether non-concordance is due to lack of understanding by the patient, poor communication by healthcare professionals or lack of access to anticoagulative medications and monitoring facilities. 19 An encouraging study from Japan showed that point-of-care testing improves INR measurements, 20 though whether this intervention is applicable to LMIC populations is uncertain.

Historically, global health interventions for paediatric cardiac care (PCC) involved ‘surgical tourism’ (when a medical team of paediatric cardiologists and cardiac surgeons went to underserved area for short period of time), leaving little legacy. Alternatively, accessing healthcare from high-income countries was an option only available for wealthier families from developing countries, aggravating healthcare inequalities. Experts recommend promotion of partnerships between developed and underserved countries, which may enable LMIC creation of sustainable cardiac facilities. 21 It has also been demonstrated that improvement in one medical area within an LMIC can result in parallel upgrades in other hospital facilities and local infrastructure. 21 PCC can be overlooked in countries with a very limited healthcare budget, as treatments for more widespread diseases are prioritised. 22

Finally, a key determinant of whether PCC programmes succeed is funding. In this context, our patient can be said to be unlucky to have been affected by ARF and RHD, but fortunate to have had access to cardiac surgery. There is reasonable access to cardiac surgery for children in the South West Pacific mainly to New Zealand, and recently to Indian cardiac units. Patients from FP can also be treated in France. Some patients in Oceania have access to fly-in units. 19

Indications for cardiac surgery in ARF/RHD

Occasionally, a child with ARF may present in acute pulmonary oedema to ruptured chordae tendinae of the mitral valve, leading to a rapid rise in LA pressure and pulmonary oedema. Often mistaken for acute pneumonia, cardiac surgery in this scenario is lifesaving. 23 The more common scenario in ARF with severe carditis is cardiac compensation and remodelling of the LV size over months to years. There is a well-developed ARF/RHD register and good rates of secondary prophylaxis in FP (BP, personal communication). Cardiac surgery is indicated based on symptoms and LV size relation to body surface area. 1 24 Our patient also had severe dilation of the LV, which has been associated with reduced LV function due to increased preload and afterload. 24 In the case of our patient, who had mitral and aortic regurgitation, valvular replacement was a necessity as the heart valves were too damaged, and the consequences of a failed valve repair in the context of established heart failure were too hazardous. Postoperative care for our patient includes lifelong anticoagulation in a remote region of a well-resourced country. As well as the risk of subsequent thromboembolism, our patient may require reoperation when he reaches adult size if the prosthetic valves are too small.

There is international consensus on how to reduce the global health burden of RHD; current guidance focuses on secondary antibiotic prophylaxis, 3 primary prevention and primordial prevention by improving living conditions. 5 Themes reinforced in this case report include the need for improved training of healthcare professionals to detect ARF symptoms earlier, informing the population to attend a clinic at an early stage (sore throat, joint pains), access to echocardiography, surgical valve repair rather than replacement and particularly in the case of the latter, enhanced postoperative care.

Learning points

• Awareness of the global health problem of rheumatic heart disease and the disproportionate burden on low-income and middle-income countries (LMIC).
• Barriers to early recognition and diagnosis, for example, lack of knowledge of unusual symptoms of acute rheumatic fever such as irregular movements, as seen in patient A, as well as infrastructure issues such as poor quality of echocardiography and reduced access to cardiac care units for remote populations and in LMIC.
• International efforts to improve access to cardiac surgery.
• Indications for cardiac surgery in paediatric cases of RHD.

Contributors: TKN, NJW and JA are responsible for substantial contributions to the conception or design of the work; or the acquisition, analysis or interpretation of data for the work. TKN, NJW and BP are responsible for drafting the work or revising it critically for important intellectual content. TKN, NJW, BP and JA are responsible for final approval of the version to be published. TKN, NJW, BP and JA are responsible for agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Competing interests: None declared.

Patient consent: Obtained.

Provenance and peer review: Not commissioned; externally peer reviewed.

Log in using your username and password

• Search More Search for this keyword Advanced search
• Latest content
• Global health
• BMJ Journals More You are viewing from: Google Indexer

You are here

• Volume 2015, Issue
• Rheumatic heart disease
• Article Text
• Article info
• Citation Tools
• Rapid Responses
• Article metrics

• Christina Maria Steger
• Department of Pathology , Academic Teaching Hospital Feldkirch, Affiliation of the Medical University Innsbruck , Feldkirch , Austria
• Correspondence to Dr Christina Maria Steger, Christina.Steger{at}lkhf.at

https://doi.org/10.1136/bcr-2015-211943

Statistics from Altmetric.com

Request permissions.

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

Description

A 65-year-old man with a medical history of rheumatic fever in childhood reported thoracic pain while at work and collapsed. He died in the ambulance on the way to hospital due to ventricular fibrillation.

At autopsy, the heart showed signs of rheumatic heart disease with left ventricular dilation, eccentric left ventricular hypertrophy, posteromedial papillary muscle hypertrophy, and thickening and fusion of the chordae tendineae of the mitral valve ( figures 1 and 2 ; in comparison, a heart with regular chordae tendineae in figure 3 ). Chordae tendineae of the anterolateral papillary muscle measured up to 1.2 cm in length and 1.1 cm in diameter ( figure 4 ); chordae tendineae of the posteromedial papillary muscle measured up to 2 cm in length and 6 mm in diameter ( figure 5 ).

• Download figure
• Open in new tab
• Download powerpoint

Heart with left ventricular dilation, thickening and fusion of the chordae tendineae of the mitral valve, and hypertrophy of the posteromedial papillary muscle.

Close-up of a few of the chordae tendineae.

Regular heart with unremarkable chordae tendineae.

Chordae tendineae of the anterolateral papillary muscle.

Chordae tendineae of the posteromedial papillary muscle.

Histopathological analysis of the heart revealed extensive tendon fibrosis and sclerosis of the chordae tendineae without inflammation, small fibrous scars in the left and right ventricle and pericardial fibrosis, as evidence of prior myocarditis and pericarditis. At the time of death, no inflammatory infiltrates in atria, ventricles and heart valves were found.

Rheumatic heart disease is a complication of acute rheumatic fever, an inflammatory disease that can involve the heart, joints, skin and brain. Rheumatic fever typically develops 2–4 weeks after a throat infection caused by group A β-haemolytic Streptococcus . The diagnosis of acute rheumatic fever is established by the Jones criteria. 1 Heart involvement generally develops over time and may include mitral and aortic valve stenosis or insufficiency, pancarditis, arrhythmias and heart failure.

The mitral valve is most commonly and severely affected (65–70% of patients), followed by the aortic valve (25%) and the tricuspid valve (10%). Fusion of the valve apparatus develops 2–10 years after an episode of acute rheumatic fever, and recurrent episodes may cause progressive damage to the valves. Fusion occurs at the level of the valve commissures, cusps, chordal attachments, or any combination of these. Fibrosis and scarring of valve leaflets, commissures, cusps and chordae tendineae lead to abnormalities that can result in valve stenosis or regurgitation or a combination of stenosis and insufficiency. 2 Variables that correlate with severity of valve disease include the number of previous attacks of rheumatic fever, and the length of time between the onset of disease and the start of therapy.

Learning points

Rheumatic heart disease is a complication of acute rheumatic fever caused by group A β-haemolytic Streptococcus and develops over time.

Heart involvement can include mitral and aortic valve stenosis or insufficiency, pancarditis, arrhythmias and heart failure, and is characterised by fibrosis and scarring of the leaflets, commissures and cusps.

Treatment of rheumatic heart disease consists of medical treatment of congestive heart failure and heart valve surgery.

• ↵ [No authors listed] . Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association . JAMA 1992 ; 268 : 2069 – 73 . doi:10.1001/jama.1992.03490150121036 OpenUrl CrossRef PubMed Web of Science
• Brice EAW ,
• Commerford PW

Competing interests None declared.

Patient consent Obtained.

Provenance and peer review Not commissioned; externally peer reviewed.

Read the full text or download the PDF:

• Search Menu
• Sign in through your institution
• Author videos
• ESC Content Collections
• Supplements
• Author Guidelines
• Submission Site
• Open Access Options
• Self-Archiving Policy
• About European Heart Journal Supplements
• About the European Society of Cardiology
• ESC Publications
• Editorial Board
• Advertising and Corporate Services
• Journals Career Network
• Terms and Conditions
• Journals on Oxford Academic
• Books on Oxford Academic

Article Contents

• < Previous

SEVERE MITRAL INSUFFICIENCY FROM RHEUMATIC DISEASE: A CASE REPORT

• Article contents
• Figures & tables
• Supplementary Data

S Testa, M Ricci, S Gallina, G Renda, F Ricci, SEVERE MITRAL INSUFFICIENCY FROM RHEUMATIC DISEASE: A CASE REPORT, European Heart Journal Supplements , Volume 26, Issue Supplement_2, April 2024, Pages ii149–ii150, https://doi.org/10.1093/eurheartjsupp/suae036.372

• Permissions Icon Permissions

• antibiotics
• mitral valve insufficiency
• rheumatic heart disease
• rheumatic fever
• acute rheumatic fever
• echocardiography
• cardiovascular diseases
• left atrium
• rheumatic disorders
• heart valves
• mitral valve
• bacterial infections
• immune response
• deoxyribonuclease i
• deoxyribonucleases
• developed countries
• dilatation, pathologic
• streptococcal infections
• antistreptolysin
• left ventricular dilatation
• penicillin prophylaxis
• cardiovascular findings
• streptococcus pyogenes infection
• interalveolar fibrosis, rapidly progressive
• heart sound p2

Email alerts

Related articles in pubmed, citing articles via.

• Recommend to Your Librarian

Affiliations

• Online ISSN 1554-2815
• Print ISSN 1520-765X
• Copyright © 2024 European Society of Cardiology
• About Oxford Academic
• Publish journals with us
• University press partners
• What we publish
• New features  
• Open access
• Institutional account management
• Rights and permissions
• Get help with access
• Accessibility
• Advertising
• Media enquiries
• Oxford University Press
• Oxford Languages
• University of Oxford

Oxford University Press is a department of the University of Oxford. It furthers the University's objective of excellence in research, scholarship, and education by publishing worldwide

• Copyright © 2024 Oxford University Press
• Cookie settings
• Cookie policy
• Privacy policy
• Legal notice

This Feature Is Available To Subscribers Only

Sign In or Create an Account

This PDF is available to Subscribers Only

For full access to this pdf, sign in to an existing account, or purchase an annual subscription.

Create Free Account or

• Acute Coronary Syndromes
• Anticoagulation Management
• Arrhythmias and Clinical EP
• Cardiac Surgery
• Cardio-Oncology
• Cardiovascular Care Team
• Congenital Heart Disease and Pediatric Cardiology
• COVID-19 Hub
• Diabetes and Cardiometabolic Disease
• Dyslipidemia
• Geriatric Cardiology
• Heart Failure and Cardiomyopathies
• Invasive Cardiovascular Angiography and Intervention
• Noninvasive Imaging
• Pericardial Disease
• Pulmonary Hypertension and Venous Thromboembolism
• Sports and Exercise Cardiology
• Stable Ischemic Heart Disease
• Valvular Heart Disease
• Vascular Medicine
• Clinical Updates & Discoveries
• Advocacy & Policy
• Perspectives & Analysis
• Meeting Coverage
• ACC Member Publications
• ACC Podcasts
• View All Cardiology Updates
• Earn Credit
• View the Education Catalog
• ACC Anywhere: The Cardiology Video Library
• CardioSource Plus for Institutions and Practices
• ECG Drill and Practice
• Heart Songs
• Nuclear Cardiology
• Online Courses
• Collaborative Maintenance Pathway (CMP)
• Understanding MOC
• Image and Slide Gallery
• Annual Scientific Session and Related Events
• Chapter Meetings
• Live Meetings
• Live Meetings - International
• Webinars - Live
• Webinars - OnDemand
• Certificates and Certifications
• ACC Accreditation Services
• ACC Quality Improvement for Institutions Program
• CardioSmart
• National Cardiovascular Data Registry (NCDR)
• Advocacy at the ACC
• Cardiology as a Career Path
• Cardiology Careers
• Cardiovascular Buyers Guide
• Clinical Solutions
• Clinician Well-Being Portal
• Diversity and Inclusion
• Infographics
• Innovation Program
• Mobile and Web Apps

Diagnosis and Management of Rheumatic Heart Disease

The following are key points to remember from this review on rheumatic heart disease (RHD):

Epidemiology:

• The global burden of RHD continues to be significant. In 2017, there were an estimated 38-40.8 million cases of RHD globally, with the highest prevalence in Oceania, South Asia, and sub-Saharan Africa.
• Prevalence ranged from 3.4 cases/100,000 in non-endemic regions, to >1,000/100,000 cases in endemic areas.
• Data on RHD related morbidity and mortality are less robust, but the estimate is at least 260,000-300,000 deaths per year.
• A global registry of 3,300 RHD cases from 14 lower- to mid-income countries reveals that most patients with RHD are young (median age 28 years), female (66%), with moderate to severe multivalvular disease (64%) complicated by congestive heart failure (33%), pulmonary hypertension (29%), atrial fibrillation (22%), and stroke (7%).
• The World Heart Federation (WHF) has set forth an aim to reduce the burden of RHD by 25% in 2025.

RHD diagnosis:

• During acute rheumatic fever (ARF), rheumatic carditis can manifest as pericarditis or valvulitis. Rheumatic carditis will frequently progress to RHD (up to 70% in certain studies), although the initial ARF will have often been missed.
• The mitral or both mitral and aortic valves are most commonly affected. Isolated aortic valve, or right-sided valve involvement is rare. Acute mitral valvulitis can result in anterior leaflet prolapse, annular dilation, chordal elongation, and varying degrees of mitral regurgitation. Over time, chronic inflammation results in commissural fusion with involvement of the mitral valve apparatus, resulting in mitral stenosis.
• The WHF has well-defined minimal echocardiography criteria for the diagnosis of RHD, while the American Society of Echocardiography/American College of Cardiology/American Heart Association guidelines further outline well-recognized criteria for quantification of degree of regurgitation or stenosis. Research is being done on more simplified criteria that could be obtained on cheaper hand-held machines by less skilled/trained technicians.
• Echocardiographic assessment of the mitral valve apparatus should include leaflet mobility, valve thickening, subvalvular thickening, valvular calcification, commissural morphology, and leaflet displacement—all of which are needed to determine the likelihood of successful intervention with balloon mitral valvuloplasty.
• Improved identification of ARF and prompt initiation of treatment could reduce progression to RHD and is, therefore, of great interest. However, fever is nonspecific and up to one third of patients with ARF report no history of sore throat.
• Auscultation is neither sensitive nor specific for detection of RHD. However, limited prevalence data have made it difficult to support echocardiography-based screening.
• The two most suitable populations for echo-based screening are school-aged children, as they would still benefit from secondary prophylaxis, and pregnant women, given the potential consequences for both mother and baby.
• Echo-based screening has led to creation of multiple registries, which track the prevalence of RHD and its natural progression. These have established the need for long-term antibiotic treatment.
• The role of anti-streptolysin O titers in determining treatment or monitoring efficacy of prophylaxis in subclinical RHD remains unclear.
• The role of echocardiographic screening as a public health strategy for global reduction of the burden of RHD, its related morbidity and mortality, and estimates of number needed to treat are still unknown.

Management:

• The key to ARF/RHD management is secondary prevention with continuous antibiotic prophylaxis to prevent recurrent infection with Group A streptococcus. Benzathine penicillin G dosed every 3-4 weeks is superior to oral penicillin.
• Data on appropriate duration of treatment are based mostly on expert opinion and vary among different countries. Considerations include ARF presentation (age, time since last ARF, ± rheumatic carditis), and presence and severity of chronic RHD. Typical treatment durations are 5-10 years, or until age 21 (whichever is longer). For severe chronic RHD, treatment can be life-long, even after surgical intervention.
• For patients under age 35 years without a documented history of ARF, treatment durations are a minimum of 5 years or until age 40 (whichever is longer). Life-long prophylaxis is recommended following valve surgery.
• While typical guidelines for severe valvular heart disease stress surgical and catheter-based interventions, the majority of cases occur in regions of the world where these options may not be available. Typical agents such as diuretics, afterload reducers, and beta-blockers are recommended for symptomatic relief of heart failure.
• For atrial fibrillation or flutter, anticoagulation with oral vitamin K antagonists or direct oral anticoagulants is still recommended. However, the INVICTUS-VKA study is currently evaluating noninferiority of rivaroxaban to warfarin.
• For isolated mitral stenosis in symptomatic patients with favorable valve anatomy, balloon mitral valvuloplasty is generally preferred given the lower cost and rapid recovery time. While complications (such as tamponade or valve leaflet rupture) are rare (2-5%), on-site surgical back-up is typically still required. Long-term benefit after balloon mitral valvuloplasty is seen in about 75% of patients.
• While surgical mitral valve repair by experienced surgeons is feasible in >75% of cases, the most important consideration in RHD-endemic regions is limiting the risk of a redo operation. This makes valve replacement the more common practice, especially for double-valve surgery (with a subsequent need for lifetime anticoagulation).
• Access to surgeons remains the most important problem in RHD endemic areas, with three cardiothoracic surgeons per 1 million inhabitants in North Africa and one cardiothoracic surgeon per 3.3 million people in Sub-Saharan Africa. International declarations to improve access to surgery in endemic areas through global alliances and structured training of more cardiac surgeons will be essential.

Clinical Topics: Anticoagulation Management, Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Pericardial Disease, Prevention, Valvular Heart Disease, Anticoagulation Management and Atrial Fibrillation, Atrial Fibrillation/Supraventricular Arrhythmias, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Cardiac Surgery and VHD, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, Acute Heart Failure, Interventions and Imaging, Interventions and Structural Heart Disease, Echocardiography/Ultrasound, Mitral Regurgitation

Keywords: Anticoagulants, Anti-Bacterial Agents, Antibiotic Prophylaxis, Atrial Fibrillation, Balloon Valvuloplasty, Cardiac Surgical Procedures, Constriction, Pathologic, Dilatation, Diuretics, Diagnostic Imaging, Echocardiography, Heart Defects, Congenital, Heart Failure, Heart Valve Diseases, Mitral Valve Insufficiency, Mitral Valve Stenosis, Myocarditis, Penicillin G Benzathine, Pericarditis, Pregnancy, Rheumatic Fever, Rheumatic Heart Disease, Secondary Prevention, Stroke, Warfarin

You must be logged in to save to your library.

Jacc journals on acc.org.

• JACC: Advances
• JACC: Basic to Translational Science
• JACC: CardioOncology
• JACC: Cardiovascular Imaging
• JACC: Cardiovascular Interventions
• JACC: Case Reports
• JACC: Clinical Electrophysiology
• JACC: Heart Failure
• Current Members
• Campaign for the Future
• Become a Member
• Renew Your Membership
• Member Benefits and Resources
• Member Sections
• ACC Member Directory
• ACC Innovation Program
• Our Strategic Direction
• Our History
• Our Bylaws and Code of Ethics
• Leadership and Governance
• Annual Report
• Industry Relations
• Support the ACC
• Jobs at the ACC
• Press Releases
• Social Media
• Book Our Conference Center

Clinical Topics

• Chronic Angina
• Congenital Heart Disease and     Pediatric Cardiology
• Diabetes and Cardiometabolic     Disease
• Hypertriglyceridemia
• Invasive Cardiovascular Angiography    and Intervention
• Pulmonary Hypertension and Venous     Thromboembolism

Latest in Cardiology

Education and meetings.

• Online Learning Catalog
• Products and Resources
• Annual Scientific Session

Tools and Practice Support

• Quality Improvement for Institutions
• Accreditation Services
• Practice Solutions

Heart House

• 2400 N St. NW
• Washington , DC 20037
• Contact Member Care
• Phone: 1-202-375-6000
• Toll Free: 1-800-253-4636
• Fax: 1-202-375-6842
• Media Center
• Advertising & Sponsorship Policy
• Clinical Content Disclaimer
• Editorial Board
• Privacy Policy
• Registered User Agreement
• Terms of Service
• Cookie Policy

© 2024 American College of Cardiology Foundation. All rights reserved.

Nursing Case Study for Rheumatic Heart Disease

Watch More! Unlock the full videos with a FREE trial

Included In This Lesson

Access More! View the full outline and transcript with a FREE trial

Ms. Patel, a 19-yr-old female who recently immigrated to the US from India due to poor conditions in her home village, presents to the Emergency Department (ED) with “fluttering” in the chest which is sometimes uncomfortable. She also complains of fatigue and occasional shortness of breath. She has no primary care provider and works at her family’s hotel primarily cleaning rooms. She says this issue is impacting her work.

What further nursing assessments need to be performed for Ms. Patel?

• Head-to-toe assessment with a focus on heart and lungs. 12 lead EKG and place on a heart monitor. O2 sats. Use facility-approved pain evaluation tool – since this may be cardiac related (“fluttering in the chest”) use the PQRST method. P=provacation/palliation, Q=quality, R=region/radiation, S=severity scale, T=timing)

What focused questions need to be included in the admission interview?

• The point is to assess for rheumatic fever history. What were the living conditions in India (crowded, poverty, no healthcare access)? Does she remember having very bad or frequent sore throat symptoms (checking for strep A infection history)? Rheumatic fever diagnosis or symptoms (fever, painful joints especially knees, ankles, elbows and wrists; pain that moves between different joints; fatigue, jerky uncontrollable body movements).

Upon further assessment, the patient has mild peripheral edema. Heart auscultation indicates a harsh pansystolic murmur and lung auscultation reveals crackles in all fields. Vital signs were as follows:

BP 134/84 mmHg SpO2 92% on Room Air HR 102 bpm and regular RR 12 bpm at rest, 30 with movement Temp 37.2°C

What orders does the nurse anticipate?

• Transthoracic (non-invasive) echocardiogram (echo) Cardiac labs Chest radiographs 12 lead EKG if not done in triage

Cardiac enzymes were drawn, results are still pending. EKG ordered, results: 12-lead EKG report reads: “Sinus tachycardia with mild to moderate mitral regurgitation.” A chest x-ray and transthoracic echocardiogram were ordered. Ms. Patel asks why she needs these tests. Pregnancy test was negative. The provider confides in the nurse that he suspects rheumatic heart disease.

How can the nurse best explain the need for the tests?

• Transthoracic (non-invasive) echocardiogram – checking the heart valves and cardiac health in case rheumatic fever caused damage Cardiac labs – to see if the problem is acute vs chronic Chest radiographs – best and least invasive way to get cardiac and lung data 12 lead EKG if not done in triage – to determine if there are dysrhythmias, to check for acute vs chronic pathology, a baseline for future treatment (patient is at risk for afib)
• Pregnancy test – sometimes when a woman becomes pregnant previously asymptomatic heart conditions may produce symptoms, heart damage can lead to pregnancy or delivery complications, and this should be assessed due to her being of childbearing age.

What potential risk factors does Ms. Patel have for rheumatic heart disease?

• Coming from impoverished conditions (“The disease persists among the rural poor and marginalized populations with little or no access to primary health care.”) If yes to rheumatic fever history screening, this is the cause of rheumatic heart disease (RHD)

The nurse has Ms. Patel on continuous cardiac monitoring per the provider’s order. He notices a change in the P wave on the monitor, however, the QRS complex remains narrow. He checks on the patient, and she reports no chest pain but feels a “flutter” in her chest. Cardiac enzymes were negative.

How does the nurse interpret this EKG finding? What does it mean? What may explain it?

• This could be premature atrial contractions that may come and go. They can be caused by structural problems or for no reason at all. She may have heart valve issues which is why it is important to tell the provider of any EKG changes.

The provider is advised of the EKG changes. He comes in to tell the patient about the chest x-ray which indicates cardiomegaly and mild interstitial pulmonary edema. An echocardiogram reveals mitral regurgitation, thickened mitral leaflets, and dilated left atrium and ventricle.

After the provider leaves, Ms. Patel asks what that means.

How can the nurse help explain what the provider said to Ms. Patel?

• Cardiomegaly = enlarged heart maybe from chronic valve problems and strain on the heart from rheumatic fever Mild interstitial pulmonary edema – fluid in lungs due to heart getting “backed up” from a valve not working correctly.
• An echocardiogram reveals mitral regurgitation, thickened mitral leaflets, and dilated left atrium and ventricle = heart valve has changes consistent with RHD, the heart is enlarged from increased work due to valve problems.

The provider consults cardiology for the patient. When the specialist arrives, he mentions possible heart surgery, according to the patient, but is not specific.

What type of surgery does the nurse consider researching more to educate the patient?

• The determination of the exact procedure is up to the specialist. However, discussing and educating about cardiac catheterization and/or valve replacement surgery options is appropriate. Heart valve replacement discussion should include biological and mechanical options.

Ms. Patel is discharged after an influenza vaccine with instructions to follow up with cardiology. Discharge medications include:

Spironolactone PO 50 mg daily Aspirin PO 81 mg daily Enalapril PO 2.5 mg daily

What Is the rationale for these medications?

• Flu shot – prevention is key especially with underlying cardiac patients Spironolactone PO 50 mg daily – diuretic to reduce preload Aspirin PO 81 mg daily – antiplatelet to help prevent coronary vessel thrombosis Enalapril PO 2.5 mg daily – ACE to reduce afterload because the patient has signs of heart dysfunction

View the FULL Outline

When you start a FREE trial you gain access to the full outline as well as:

• SIMCLEX (NCLEX Simulator)
• 6,500+ Practice NCLEX Questions
• 2,000+ HD Videos
• 300+ Nursing Cheatsheets

“Would suggest to all nursing students . . . Guaranteed to ease the stress!”

References:

View the full transcript, nursing case studies.

This nursing case study course is designed to help nursing students build critical thinking.  Each case study was written by experienced nurses with first hand knowledge of the “real-world” disease process.  To help you increase your nursing clinical judgement (critical thinking), each unfolding nursing case study includes answers laid out by Blooms Taxonomy  to help you see that you are progressing to clinical analysis.We encourage you to read the case study and really through the “critical thinking checks” as this is where the real learning occurs.  If you get tripped up by a specific question, no worries, just dig into an associated lesson on the topic and reinforce your understanding.  In the end, that is what nursing case studies are all about – growing in your clinical judgement.

Nursing Case Studies Introduction

Cardiac nursing case studies.

• 6 Questions
• 7 Questions
• 5 Questions
• 4 Questions

GI/GU Nursing Case Studies

• 2 Questions
• 8 Questions

Obstetrics Nursing Case Studies

Respiratory nursing case studies.

• 10 Questions

Pediatrics Nursing Case Studies

• 3 Questions
• 12 Questions

Neuro Nursing Case Studies

Mental health nursing case studies.

• 9 Questions

Metabolic/Endocrine Nursing Case Studies

Other nursing case studies.

Horrific nightmares may signal initial onset of these chronic diseases, study says

Sign up for CNN’s Sleep, But Better newsletter series.  Our seven-part guide has helpful hints to achieve better sleep .

The nightmares are intense and often horrifying, sometimes lasting well into the day.

“There’s a serial killer after me and the last few years I have the same one,” according to a Canadian patient. “He’s got my legs or something I can still feel something on my legs even when I’m then awake.”

Another English patient described nightmares “where I can’t breathe and where someone is sitting on my chest.” Yet another shared stories of “really nasty” violent visions in their sleep.

“Horrific, like murders, like skin coming off people,” said one Irish patient about his nightmares. “I think it’s like when I’m overwhelmed which could be the lupus being bad … so I think the more stress my body is under then the more vivid and bad the dreaming would be.”

Nightmares and “daymares,” dreamlike hallucinations that appear when awake, may be little-known signs of the onset of lupus and other systemic autoimmune diseases such as rheumatoid arthritis, according to a new study published Monday in the journal eClinicalMedicine.

Such unusual symptoms may also be a signal that an established disease may be about to intensely worsen or “flare” and require medical treatment, said lead study author Melanie Sloan, a researcher in the department of public health and primary care at the University of Cambridge in the United Kingdom.

“This is particularly the case in a disease like lupus, which is well known for affecting multiple organs including the brain, but we also found these patterns of symptoms in the other rheumatological diseases, like rheumatoid arthritis, Sjogren’s syndrome, and systemic sclerosis,” Sloan said in an email.

Lupus is a long-term disease in which the body’s immune system goes haywire, attacking healthy tissue and causing inflammation and pain in any part of the body, including blood cells, the brain, heart, joints and muscles, kidneys, liver, and lungs.

“Cognitive problems and many of these other neuropsychiatric symptoms we studied can have a huge influence on people’s lives, ability to work, to socialize, and just to have as much of a normal life as possible,” she said.

“These symptoms are often invisible and (currently) untestable but that shouldn’t make them any less important to be considered for treatment and support.”

Jennifer Mundt, an assistant professor of sleep medicine, psychiatry and behavioral sciences at Northwestern University’s Feinberg School of Medicine in Chicago who was not involved in the study, said in an email she was pleased the study focused on nightmares.

“Although nightmares are a very distressing problem in many medical and psychiatric conditions, they rarely get focused on except in the context of PTSD (post-traumatic stress syndrome),” Mundt said.

“A  recent study  showed that 18% of people with long-COVID have (frequent) nightmares, and this compares to a general population prevalence of about 5%,” she said. “Hearing the patient perspective is critical so that research and clinical care can be guided by what is most important to patients themselves.”

Doctors and patients need to know

While research in the field is rather new, a March 2019 study found patients with inflammatory arthritis and other autoimmune and inflammatory diseases also experienced nightmares and other REM sleep disorders such as sleep paralysis. REM is short for rapid eye movement, the stage of sleep in which people dream and information and information and experiences are consolidated and stored in memory.

In that study, one 57-year-old man recalled being “threatened by feral birds of prey” in his nightmares, while a 70-year-old woman dreamed her nephew was in grave danger but she could do nothing to help him.

The new study surveyed 400 doctors and 676 people living with lupus and also conducted detailed interviews with 50 clinicians and 69 people living with systemic autoimmune rheumatic diseases, including lupus.

Researchers found 3 in 5 lupus patients, and 1 in 3 patients with other rheumatology-related diseases, had increasingly vivid and distressing nightmares just before their hallucinations. These nightmares often involved falling or being attacked, trapped, or crushed or committing murder.

“I’d be riding a horse, going around cutting people out with my sword. One of them was somebody attacking me and I ended up slitting their throat,” the English patient said.

“I’m not a violent person at all. I don’t even kill an insect,” the patient continued. “And I came to the conclusion that’s probably me fighting my own (autoimmune) system. … I’m probably attacking myself, that’s the only thing I can logically make sense out of it.

Systemic autoimmune diseases often have a range of symptoms, called prodromes, that appear as signs of a sudden and possibly dangerous worsening of the condition. In lupus, for example, headaches, an increase in fatigue, painful, swollen joints, rashes, dizziness and a fever without an infection are well-known signs of an upcoming flare.

Recognizing these warning signs are important, Sloan said, because they allow “earlier detection and therefore treatment of flares, some of which can be organ damaging and even fatal in lupus patients.”

However, unique warning symptoms such as nightmares and daymares are not in the diagnostic criteria for lupus or other diseases, Sloan said. The study found doctors infrequently ask about such experiences, and patients often avoid talking about them to their physicians.

“We are strongly encouraging more doctors to ask about nightmares and other neuropsychiatric symptoms — thought to be unusual, but actually very common in systemic autoimmunity — to help us detect disease flares earlier,” said senior study author David D’Cruz, a consultant rheumatologist at Guy’s Hospital and Kings College London.

Connect the dots to autoimmune disease

On first glance, it would make sense that such neurological manifestations as nightmares would occur if the autoimmune disease impacts the brain, which lupus often does, Sloan said. But that’s not what the study uncovered.

“Interestingly, we found that lupus patients who were classified as having organ involvement other than the brain, such as kidneys or lungs, often also reported a variety of neuropsychiatric symptoms in the lead up to their kidney/lung flare,” Sloan said via email.

“This suggests that monitoring these symptoms — such as nightmares and changing mood — as well as the usual rashes and protein in the urine (due to inflammation in the kidneys ), etc., may help with earlier flare detection in many patients, not just those who go on to develop major brain involvement,” she said.

However, there is no reason for people with occasional nightmares or daytime dreams to be worried they may have an inflammatory autoimmune disease, said sleep disorder specialist Dr. Carlos Schenck, a professor and senior staff psychiatrist at the Hennepin County Medical Center at the University of Minnesota in Minneapolis.

“This study could alarm the general public into believing or worrying about whether they have lupus or a related autoimmune disorder if they have nightmares or hallucinations, which are what doctors call ‘nonspecific symptoms,’ meaning that a variety of conditions (medical and psychiatric) can manifest with these symptoms,” Schenck said in an email.

It is indeed “perfectly normal” to have occasional nightmares and even daymares, or hallucinations, which “are also more common than we think,” Sloan said.

However, if those are intense, upsetting and occur around other symptoms such as extreme fatigue, headaches and other signs of autoimmune disorders , they “should be discussed with a doctor,” Sloan said.

“People shouldn’t be afraid or embarrassed to talk about these symptoms,” she said. “In some cases, reporting these symptoms earlier, even if they seem strange and unconnected, may lead to the doctor being able to ‘join the dot’s’ to diagnose an autoimmune disease.”

For more CNN news and newsletters create an account at CNN.com