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: 40 to 59 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
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
JDZ is and has been a consultant for Medtronic and Abbott.
Acknowledgements
Dr John R. Charpie and Dr Jeffrey D. Zampi 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.
Differentials
- Cardiac tumors
- Systemic lupus erythematosus (SLE)
- Constrictive pericarditis (CP)
More DifferentialsGuidelines
- 2021 ESC/EACTS guidelines for the management of valvular heart disease
- 2020 ACC/AHA guideline for the management of patients with valvular heart disease
More Guidelines
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