Summary
Definition
History and exam
Key diagnostic factors
- history of acute rheumatic fever (ARF) during childhood
- dyspnea
- elevated jugular venous pressure with prominent a-wave
- low-frequency presystolic (diastolic) murmur at lower left sternal border
Other diagnostic factors
- age: 20-39 years
- exercise intolerance
- fatigue
- jugular pulsations
- abdominal swelling and discomfort
- edema
- cyanosis or hypoxemia
- atrial fibrillation
- hepatomegaly
- ascites, edema, anasarca
- age: infancy or childhood
- episodic facial flushing, watery diarrhea, or bronchoconstriction
- absent right ventricular lift or heave
- opening snap
- endocarditis stigmata (e.g., splinter hemorrhages, Osler nodes, Janeway lesions)
Risk factors
- group A streptococcal (GAS) pharyngitis
- metastatic carcinoid tumors
- artificial tricuspid valve
- intravenous drug use
- pacemaker/defibrillator leads crossing tricuspid valve orifice
- genetic predisposition and environmental factors
Diagnostic tests
1st tests to order
- ECG
- chest x-ray
- 2D transthoracic echocardiogram
- Doppler transthoracic echocardiogram
- liver function tests
- blood biochemistry
- CBC
- blood cultures
- 24-hour urinary excretion of 5-hydroxy-indole acetic acid (5-HIAA)
Tests to consider
- cardiac catheterization
- cardiac MRI
- 3D transthoracic echocardiogram
- cardiac CT angiography (CTA)
- [18F] fluorodeoxyglucose (FDG)-PET/CT
Treatment algorithm
congenital
with carcinoid heart disease
with rheumatic fever sequelae
with infective endocarditis
Contributors
Authors
John R. Charpie, MD, PhD
Professor
Pediatrics and Communicable Diseases
Division Director
Pediatric Cardiology
Medical Director
Pediatric Cardiothoracic ICU
University of Michigan Congenital Heart Center
Ann Arbor
MI
Disclosures
JRC declares that he has no competing interests.
Jeffrey D. Zampi, MD
Associate Professor
Pediatrics and Communicable Diseases
Director, Interventional Pediatric Cardiology
University of Michigan Congenital Heart Center
Ann Arbor
MI
Disclosures
JZ is a consultant for Medtronic Inc and Gore Medical. JZ serves on the data safety monitoring board for a clinical trial sponsored by Encore Medical. JZ has served as medical expert for several legal cases.
Amanda D. McCormick MD,
Assistant Professor
Pediatrics and Communicable Diseases
University of Michigan Congenital Heart Center
Ann Arbor
MI
Disclosures
ADM receives grant funding from the NIH.
Acknowledgements
Dr John R. Charpie, Dr Jeffrey D. Zampi, and Dr Amanda D. McCormick would like to gratefully acknowledge Dr Martin L. Bocks, a previous contributor to this topic.
Disclosures
MLB declares that he has no competing interests.
Peer reviewers
Lokesh Tejwani, MD, FACC
Assistant Professor of Clinical Medicine
University of Missouri Hospitals and Clinics
Harry S. Truman VA Hospital
Columbia
MO
Disclosures
LT declares that he has no competing interests.
John Coltart, MD, FRCP, FACC, FESC, MRCS
Consultant Cardiologist
Cardio-thoracic Unit
Guy's and St Thomas' Hospital
London
UK
Disclosures
JC declares that he has no competing interests.
References
Key articles
WHO guideline on the prevention and diagnosis of rheumatic fever and rheumatic heart disease [Internet]. Geneva: World Health Organization; 2024.Full text Abstract
Vahanian A, Beyersdorf F, Praz F, et al. 2021 ESC/EACTS guidelines for the management of valvular heart disease. EuroIntervention. 2022 Feb 4;17(14):e1126-96.Full text Abstract
Writing Committee Members., Otto CM, Nishimura RA, et al. 2020 ACC/AHA Guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2021 Feb 2;77(4):e25-e197.Full text Abstract
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
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