Acute Rheumatic Fever and Rheumatic Heart Disease, 1e

Book
Copyright
Professor Bongani Mbawethu Mayosi
Professor Diana (Dinny) Lennon
List of Contributors
Preface
Acute Rheumatic Fever and Rheumatic Heart Disease
1. Epidemiology, Risk Factors, Burden and Cost of Acute Rheumatic Fever and Rheumatic Heart Disease
	INTRODUCTION
	EPIDEMIOLOGY OF ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE
		Incidence of Acute Rheumatic Fever
		Prevalence of Rheumatic Heart Disease
		Heart Failure due to Rheumatic Heart Disease
		Excess Mortality from Rheumatic Heart Disease
	RISK FACTORS FOR ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE
		Pathogen and Host Factors that Lead to Acute Rheumatic Fever and Rheumatic Heart Disease
			Factors specific to the streptococcal organism
			Host demographics
			Host genetic factors
			Host oral health status
			Host nutritional status
		Social and Environmental Conditions
			Household crowding, including bed sharing
			Household resources, including washing and laundry
			Housing conditions, including tenure, damp, and cold
			Healthcare utilization and health literacy
		Summary of Risk and Protective Factors for Acute Rheumatic Fever and Rheumatic Heart Disease
	MEASURES OF GLOBAL DISEASE BURDEN
	ECONOMIC CONSEQUENCES OF ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE
	REFERENCES
2. Pathogenesis of Acute Rheumatic Fever
	INTRODUCTION
	OVERVIEW OF THE PATHOGENESIS OF ACUTE RHEUMATIC FEVER
	MEDIATORS OF AUTOIMMUNE REACTIONS
	MOLECULAR MIMICRY
	CARDITIS AND RHEUMATIC HEART DISEASE
	T CELLS AND RHEUMATIC HEART DISEASE
	SYDENHAM CHOREA
	GENETIC SUSCEPTIBILITY TO ACUTE RHEUMATIC FEVER
	REFERENCES
3. Clinical Evaluation and Diagnosis of Acute Rheumatic Fever
	INTRODUCTION
	CLINICAL EVALUATION OF ACUTE RHEUMATIC FEVER
		Risk Factors for Acute Rheumatic Fever
	CLINICAL FEATURES OF ACUTE RHEUMATIC FEVER
		Major Manifestations
			Joint symptoms (arthritis and arthralgia)
			Carditis
			Chorea
			Erythema Marginatum
			Subcutaneous Nodules
		Minor Manifestations
			Fever
			Arthralgia
			Elevated Acute Phase Reactants
			First-Degree Heart Block
	OTHER CLINICAL MANIFESTATIONS OF ACUTE RHEUMATIC FEVER
	DIAGNOSTIC INVESTIGATIONS FOR ACUTE RHEUMATIC FEVER
		Laboratory Evidence of a Preceding Group A Streptococcus Infection
			Throat Culture
			Rapid Antigen Detection Testing
			Streptococcal Serology
		Chest X-ray
		Echocardiography
		Diagnosis of Rheumatic Carditis by Echocardiography
	DIAGNOSIS OF ACUTE RHEUMATIC FEVER
	RATIONALE FOR 2015 REVISION
		Possible Acute Rheumatic Fever
		Rheumatic Fever Recurrences
	INTERNATIONAL DIAGNOSTIC CRITERIA (AUSTRALIA, NEW ZEALAND, BRAZIL, INDIA)3,4,78,79
	DIFFERENTIAL DIAGNOSIS OF ACUTE RHEUMATIC FEVER
	FUTURE RESEARCH DIRECTIONS
		Working toward Universal Diagnostic Criteria
		The Search for a Molecular Signature in the Diagnosis of Acute Rheumatic Fever
		More Appropriate Ways of Diagnosing Acute Rheumatic Fever in the 21st Century
	REFERENCES
4. Management of Acute Rheumatic Fever
	INTRODUCTION
	ESTABLISHING THE DIAGNOSIS
	ERADICATION OF THE GROUP A STREPTOCOCCUS
	GENERAL MEASURES AND SYMPTOMATIC RELIEF
		Fever
		Bed Rest and Diet
	MAJOR MANIFESTATIONS
		Arthritis and/or Arthralgia
		Carditis and Heart Failure
			Carditis
			Advanced atrioventricular block
			Heart failure
				Heart-failure medications
				Heart-failure medications
				Antiinflammatory medications
				Antiinflammatory medications
				Heart valve surgery
				Heart valve surgery
		Sydenham Chorea
	DISCHARGE PROTOCOL
		Discharge Criteria
		Notification
		Patient and Family Education
		Prophylaxis
			Infective endocarditis
		Outpatient Follow-up
	REFERENCES
5. Clinical Evaluation and Diagnosis of Rheumatic Heart Disease
	INTRODUCTION
	EPIDEMIOLOGY OF RHEUMATIC HEART DISEASE
	DIAGNOSTIC CRITERIA FOR RHEUMATIC HEART DISEASE
		Diagnosis of Chronic Rheumatic Heart Disease in Individuals with a History of Acute Rheumatic Fever
		Diagnosis of Rheumatic Heart Disease in Individuals Without a History of Acute Rheumatic Fever
	CLINICAL ASSESSMENT AND FINDINGS
		Innocent Cardiac Murmurs
			Innocent murmurs in the pediatric population
		Pathological Murmurs in the Pediatric Population
		Pathological Murmurs in the Adult Population
		Mitral Regurgitation
			Clinical symptoms
			Clinical signs: mild-to-moderate mitral regurgitation
			Clinical signs: moderate-to-severe mitral regurgitation
			Electrocardiogram
			Chest X-ray
		Mitral Stenosis
			Clinical symptoms
			Clinical signs: mild to moderate
			Clinical signs: moderate to severe
			Electrocardiogram
			Chest X-ray
		Mixed Mitral Valve Disease
			Clinical symptoms
			Clinical signs: moderate to severe
			Electrocardiogram
			Chest X-ray
		Aortic Regurgitation
			Clinical symptoms
			Clinical signs: mild-to-moderate aortic regurgitation
			Clinical signs: moderate-to-severe aortic regurgitation
			Electrocardiogram
			Chest X-ray
		Aortic Stenosis
			Clinical symptoms
			Clinical signs: mild to moderate
			Clinical signs: moderate to severe
			Electrocardiogram
			Chest X-ray
		Tricuspid Regurgitation
			Clinical symptoms
			Clinical signs: mild to moderate
			Clinical signs: moderate to severe
			Electrocardiogram
			Chest X-ray
		Pulmonary Regurgitation
			Clinical symptoms
			Clinical signs: mild to moderate
			Clinical signs: moderate to severe
			ECG
			Chest X-ray
		Multivalve Rheumatic Heart Disease
			Clinical symptoms
			Clinical signs: severe multivalve disease
			ECG
			Chest X-ray
	ECHOCARDIOGRAPHY IN RHEUMATIC HEART DISEASE
		Grading the Severity of Valve Disease Using Echocardiography
		Mitral Valve Disease
			Echocardiography of isolated mitral regurgitation
			Echocardiographic findings in isolated mitral stenosis
			Echocardiography in mixed mitral valve disease
		Aortic Valve Disease
		Tricuspid Valve Disease
		Pulmonary Valve Disease
		Evaluation of Prosthetic Valves
		Monitoring Disease Progression and Resolution
		Guide to Nature and Timing of Cardiosurgical Intervention
		3D Echocardiography and Rheumatic Valve Disease
	OTHER IMAGING AND DIAGNOSTIC MODALITIES
		Cardiac Catheterization
		Computed Tomography
		Cardiac Magnetic Resonance Imaging
	EVALUATION OF HEART FAILURE
		Clinical Investigations for Chronic Heart Failure
			Full blood count
			Electrolytes and renal function testing
			Troponin
			Liver function testing and clotting profile
			Inflammatory markers
			Antibody testing
			Biomarkers for heart failure
		Exercise Stress Testing
	DISEASE PROGRESSION AND NATURAL HISTORY
	FUTURE DIRECTIONS
	REFERENCES
6. Medical Management of Rheumatic Heart Disease
	INTRODUCTION
	PRINCIPLES OF MANAGEMENT OF RHEUMATIC HEART DISEASE
	MEDICAL MANAGEMENT OF CHRONIC HEART FAILURE IN RHEUMATIC HEART DISEASE
		Overview of Heart Failure Treatment
		Heart Failure Managment (HFrEF)
			General management
			Pharmacological therapy
	MEDICAL MANAGEMENT OF INDIVIDUAL VALVE LESIONS
		Chronic Mitral Regurgitation
			Natural History and Pathophysiology of Mitral Regurgitation
			Mitral Regurgitation Medical Management
			Indications for Cardiac Surgery for Mitral Regurgitation
				Serial evaluation
				Serial evaluation
	MITRAL STENOSIS
		Natural History and Pathophysiology of Mitral Stenosis
		Medical Management of Mitral Stenosis
		Indications for Percutaneous Mitral Balloon Commissurotomy and Cardiac Surgery
			Serial evaluation
	CHRONIC AORTIC REGURGITATION
		Natural History and Pathophysiology of Aortic Regurgitation
		Medical Management of Chronic Aortic Regurgitation
		Indications for Cardiac Surgery in Chronic Aortic Regurgitation
			Serial evaluation
	AORTIC STENOSIS
		Natural History and Pathophysiology of Aortic Stenosis
		Medical Management of Aortic Stenosis
		Indications for Surgery in Aortic Stenosis
			Transcatheter Aortic Valve Implantation
			Serial evaluation
	OTHER CHRONIC RHEUMATIC HEART DISEASE ENTITIES
		Multivalve Disease and Mixed Valve Disease
		Tricuspid Valve Disease
		Prosthetic Valve Patients
			Mechanical valves
			Bioprosthetic valves
	GENERAL CARE PRINCIPALS FOR THE RHD PATIENT
		Secondary Prevention With Antibiotic Prophylaxis
		Antithrombotic Therapy in Rheumatic Heart Disease
			Antithrombotic Therapy for Prosthetic Valves
			Anticoagulation in Atrial Fibrillation
				Initiating warfarin therapy
				Initiating warfarin therapy
			Anticoagulation for patients in sinus rhythm and who have not had valve surgery
		Access to oral healthcare
		Infective Endocarditis Prophylaxis
	REFERENCES
7. Catheter-Based Evaluation and Treatment of Rheumatic Heart Disease
	INTRODUCTION
	INDICATIONS FOR CATHETER-BASED EVALUATION IN THE PATIENT WITH RHEUMATIC HEART DISEASE
	MEASUREMENTS IN THE CATHETERIZATION LABORATORY
		Basics of Cardiac Catheterization and Pressure Measurement
		Contour of Normal Pressure Waveforms and Common Artifacts
		Estimation of Pulmonary Capillary Wedge Pressure
		Estimation of Cardiac Output
	HEMODYNAMIC ASSESSMENT OF VALVE FUNCTION
		Mitral Stenosis
			The hemodynamic findings of mitral stenosis at cardiac catheterization
			Quantification of severity of mitral stenosis and calculation of valve area
		Mitral Regurgitation
			The hemodynamic findings in rheumatic mitral regurgitation at cardiac catheterization
		Mixed Mitral Valve Disease
		Aortic Valve Disease
			Aortic Stenosis
			Aortic Regurgitation
		Tricuspid Valve Disease
	CATHETHER-BASED INTERVENTIONS IN RHEUMATIC HEART DISEASE
		Mitral Stenosis
			Percutaneous mitral balloon commissurotomy
				Indications for Percutaneous Mitral Balloon Commissurotomy
				Indications for Percutaneous Mitral Balloon Commissurotomy
				Percutaneous Mitral Balloon Commissurotomy Technique
				Percutaneous Mitral Balloon Commissurotomy Technique
				Complications of Percutaneous Mitral Balloon Commissurotomy
				Complications of Percutaneous Mitral Balloon Commissurotomy
				Outcomes Following Percutaneous Mitral Balloon Commissurotomy
				Outcomes Following Percutaneous Mitral Balloon Commissurotomy
		Interventions in Rheumatic Aortic Valve Disease
	CONCLUSION
	REFERENCES
8. Surgical Management of Rheumatic Valvular Heart Disease
	INTRODUCTION
	HISTORICAL BACKGROUND
	ANATOMICAL FEATURES AND CLINICAL SPECTRUM OF SURGICAL RHEUMATIC HEART DISEASE
		Mitral Valve Disease
		Aortic Valve Disease
		Tricuspid Valve Disease
	PREOPERATIVE DIAGNOSTIC WORK-UP
		Echocardiography—What Are Surgeons Looking for?
			Mitral valve analysis
			Aortic valve analysis
			Tricuspid valve analysis
				Role of the preoperative three-dimensional and transesophageal echocardiogram
				Role of the preoperative three-dimensional and transesophageal echocardiogram
		Left and Right Catheterization—Coronary Angiography
		New Methods—CT Scan and Magnetic Resonance Imaging
	INDICATIONS FOR SURGERY AND SURGICAL TECHNIQUES
		The Young Rheumatic Patient
			Closed mitral commissurotomy for mitral stenosis
			Open mitral commissurotomy for mitral stenosis
			Repair for mitral valve regurgitation
			Mitral valve replacement
				Selection of valve prosthesis
				Selection of valve prosthesis
				Technique of valve replacement
				Technique of valve replacement
			Surgery for aortic valve disease
				Aortic valve repair
				Aortic valve repair
				Aortic valve replacement
				Aortic valve replacement
			Tricuspid valve disease
				Tricuspid regurgitation
				Tricuspid regurgitation
				Tricuspid stenosis
				Tricuspid stenosis
			Multi valve surgery
	EARLY AND LONG-TERM OUTCOMES OF RHEUMATIC VALVE SURGERY
		Perioperative Results
		Long-Term Outcomes: Repair Versus Replacement
			Mitral valve
			Aortic valve
		How Far Can We Go With Repair?
	THE ROLE OF NEW TECHNOLOGIES
	CONCLUSIONS AND RECOMMENDATIONS
	REFERENCES
9. Rheumatic Heart Disease in Pregnancy
	INTRODUCTION
	EPIDEMIOLOGY OF RHEUMATIC HEART DISEASE IN PREGNANCY
	MATERNAL CARDIAC PHYSIOLOGY
	PRECONCEPTION EVALUATION
		Risk Stratification
			Mitral Stenosis
			Aortic Stenosis
			Mitral and Aortic Regurgitation
			Tricuspid Regurgitation
			Mixed Valve Lesions
			Mitral/Aortic Valve Replacement
				Bioprosthetic Valves
				Bioprosthetic Valves
				Mechanical valves
				Mechanical valves
	CLINICAL EVALUATION DURING NORMAL PREGNANCY
	MANAGEMENT OF RHEUMATIC HEART DISEASE IN PREGNANCY
		Management of Heart Failure in Pregnancy
		Management of Specific Valve Lesions During and Before Pregnancy
			Mitral Stenosis
			Mitral and Aortic Regurgitation
			Aortic Stenosis
		Anticoagulation in Pregnancy
			Anticoagulation for Mechanical Prostheses
				Vitamin K Antagonists
				Vitamin K Antagonists
				Heparins
				Heparins
			Emergency Reversal of Anticoagulation
		Labor and Delivery
	CONTRACEPTIVE CHOICES IN WOMEN WITH RHEUMATIC HEART DISEASE
		Contraceptive Methods
			Progestogen-only methods
			Copper IUD
			Emergency contraception
	CONCLUSION
	REFERENCES
10. Primary Prevention of Acute Rheumatic Fever and Rheumatic Heart Disease
	PRIMORDIAL PREVENTION
	EFFECTIVENESS OF PRIMARY PREVENTION
	DIAGNOSIS OF GROUP A STREPTOCOCCAL PHARYNGITIS
		Streptococcal Antibody Tests
	TREATMENT OF GROUP A STREPTOCOCCAL PHARYNGITIS
		Delivery of Benzathine Penicillin G: Barriers and Potential Solutions
		Safe Administration of Benzathine Penicillin G
		Future Directions in the Treatment of Group A Streptococcal Infections
	COST-EFFECTIVENESS OF PRIMARY PREVENTION
	DELIVERY OF PRIMARY PREVENTION STRATEGIES
		Population-Based Strategies
		Health System-Based Strategies
	CONCLUSION
	REFERENCES
11. Secondary Prevention of Acute Rheumatic Fever and Rheumatic Heart Disease
	INTRODUCTION
		Definition of Secondary Prevention
		Historical Background
		Evidence of Effectiveness
		Limitations and Challenges in Delivery of Secondary Prophylaxis
	PENICILLIN FOR SECONDARY PROPHYLAXIS
		Penicillins and Pharmacokinetics
		Penicillin Supply
		Penicillin Adverse Reactions
	CLINICAL EVALUATION AND MANAGEMENT OF ANAPHYLAXIS
		Definition and Diagnosis
		Management of anaphylaxis
			Assessment of possible penicillin allergy
	SECONDARY PROPHYLAXIS RECOMMENDATIONS AND REGIMENS
		Antibiotic Dosing and Frequency
		Duration of Secondary Prophylaxis
		Stopping Secondary Prophylaxis
	IMPROVING ADHERENCE TO SECONDARY PROPHYLAXIS
		Reasons for Nonadherence
		Methods to Support Adherence
	PROGRAMMATIC APPROACHES TO SECONDARY PROPHYLAXIS DELIVERY
	INDIVIDUAL APPROACHES TO SECONDARY PROPHYLAXIS DELIVERY
	REDUCING THE PAIN OF BENZATHINE PENICILLIN G INJECTIONS
		Procedure for Preparation and Delivery of Benzathine Penicillin G Injection With Lidocaine
			Addition of 2% lidocaine to benzathine penicillin G pre-filled syringe
	ADMINISTERING THE INTRAMUSCULAR INJECTION
	SECONDARY PROPHYLAXIS IN SPECIAL CIRCUMSTANCES
		Secondary Prophylaxis During Pregnancy
		Secondary Prophylaxis while Breastfeeding
		Secondary Prophylaxis while on Oral Contraceptive
		Secondary Prophylaxis in Anticoagulated Patients
	REFERENCES
12. Rheumatic Heart Disease Control Programs, Registers, and Access to Care
	RHEUMATIC HEART DISEASE CONTROL PROGRAMS
		1900 – 1950s
		1950s–2000
		2000–2005
		2005–2018
			Contemporary recommendations for RHD control programs
	INTRODUCTION TO DISEASE REGISTERS WITH A FOCUS ON RHEUMATIC HEART DISEASE
		Introduction to Registers
		Issues Specific to Rheumatic Heart Disease Registers
	ACCESS TO CARE
	CONCLUSION
	REFERENCES
13. Echocardiographic Screening for Rheumatic Heart Disease
	INTRODUCTION
	THE RATIONALE FOR RHEUMATIC HEART DISEASE SCREENING
	THE EVOLUTION OF RHEUMATIC HEART DISEASE SCREENING
		Terminology
		Accuracy of Auscultation for Rheumatic Heart Disease
		Standardization of Echocardiographic Criteria
		Technical Considerations and Interpretation of the Guidelines
	CONTRIBUTION OF ECHOCARDIOGRAPHIC SCREENING FOR RHEUMATIC HEART DISEASE EPIDEMIOLOGY
	INNOVATIONS IN ECHOCARDIOGRAPHIC SCREENING
		Screening by Nonexpert Operators
		Innovative Technologies for Screening
		Abbreviated Protocols and Agreed Cut-Off Points
		Training Procedures
	FUTURE DIRECTIONS AND UNANSWERED QUESTIONS
		Disease Progression of Latent (Subclinical) Rheumatic Heart Disease
		Does Identification of Latent Rheumatic Heart Disease Improve Outcomes?
		Is Echocardiographic Screening Cost-Effective in Low Resource Areas?
		What Would it Take to Scale-Up Echocardiographic Screening to a National/Global Level?
		What are the Harms of a Screening Program?
		What is the Ideal Model/Test Strategy for Echocardiographic Screening?
	CONCLUSIONS
	REFERENCES
14. Group A Streptococcus Vaccines
	INTRODUCTION
	ISSUES IN GAS VACCINE DEVELOPMENT
	HISTORY OF GAS VACCINES
	21ST CENTURY GAS VACCINE DEVELOPMENT
		M-protein Vaccines
		Non-M-protein Vaccines
	FUTURE DEVELOPMENT ACTIVITIES
	REFERENCES
15. Awareness, Education, and Advocacy
	INTRODUCTION
	BASELINE AWARENESS
		Community
		Teachers
		People and Families Living with Acute Rheumatic Fever and Rheumatic Heart Disease
		Clinicians
		Policy Makers and Funders
	INTERVENTIONS TO IMPROVE AWARENESS
	MOVING FROM ORGANIC TO STRATEGIC APPROACHES
	LOCAL AND GLOBAL ADVOCACY EFFORTS
		Defining Advocacy
		What Can Be Achieved Through Successful Advocacy in Rheumatic Heart Disease?
		Moving the Needle
	WHAT NEXT FOR ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE?
	A FINAL WORD ON THE KEY ELEMENTS OF SUCCESS
	REFERENCES
16. Complications of Rheumatic Heart Disease and Acute Emergencies
	INTRODUCTION
	1: ACUTE HEART FAILURE
		Definition and Classification
		Epidemiology
		Etiology of Acute Heart Failure
			Acute rheumatic fever
			Rheumatic heart disease
		Evaluation and Management of the Patient With Acute Heart Failure
			Phase I: Early recognition, treatment, and triage of life-threatening conditions
			Phase II: Identification and treatment of acute etiologies or relevant triggers of AHF
				Acute valvular regurgitation
				Acute valvular regurgitation
				Acute presentation of chronic disease
				Acute presentation of chronic disease
				Prosthetic valve dysfunction (mechanical and bioprosthetic valves)
				Prosthetic valve dysfunction (mechanical and bioprosthetic valves)
					Prosthetic valve thrombosis management
					Prosthetic valve thrombosis management
					Prosthetic valve stenosis
					Prosthetic valve stenosis
					Other prosthetic valve complications
					Other prosthetic valve complications
				Pericardial tamponade
				Pericardial tamponade
				Tachyarrhythmia in chronic mitral stenosis
				Tachyarrhythmia in chronic mitral stenosis
				Advanced heart block
				Advanced heart block
			Phase III: Diagnostic work-up to confirm acute heart failure
			Phase IV: Defining the clinical profile and optimal management strategy
				Diuretics
				Diuretics
				Vasodilators
				Vasodilators
				Ongoing management
				Ongoing management
		Conclusion
	2: INFECTIVE ENDOCARDITIS
		Introduction
		Pathophysiology of Infective Endocarditis in Rheumatic Heart Disease
		Epidemiology of Infective Endocarditis and Rheumatic Heart Disease
			High income countries
			Infective Endocarditis in Rheumatic Heart Disease-endemic countries
		Presenting Clinical Features
			Examination findings
		Investigations
			Laboratory investigations
				Bacterial pathogen/causative organisms
				Bacterial pathogen/causative organisms
		Imaging
			Echocardiography and newer imaging modalities
		Diagnostic Criteria
		Differential Diagnosis
		Medical Management
			Principles of antibiotic therapy for infective endocarditis
		Clinical Course and Complications
			Stroke/intracranial hemorrhage/neurologic events
			Other vascular complications
			Uncontrolled sepsis and persistent bacteremia
				Cardiac surgical management
				Cardiac surgical management
		Prognosis
		Prevention and Antibiotic Prophylaxis
		Conclusions
	3: OVERANTICOAGULATION AND BLEEDING
		Introduction
		Management
	REFERENCES
Index
	A
	B
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	D
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	H
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	L
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