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: 40s to 50s
- 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 investigations
1st investigations to order
- ECG
- CXR
- 2D transthoracic echocardiogram
- Doppler transthoracic echocardiogram
- LFTs
- blood biochemistry
- CBC
- blood cultures
- 24-hour urinary excretion of 5-hydroxy-indole acetic acid (5-HIAA)
Investigations to consider
- cardiac catheterization
- cardiac MRI
- 3D transthoracic echocardiogram
Treatment algorithm
Contributors
Authors

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.

Assistant Professor
Pediatrics and Communicable Diseases
University of Michigan Congenital Heart Center
Ann Arbor
MI
Disclosures
JDZ declares that he has no competing interests.
Dr John R. Charpie and Dr Jeffrey D. Zampi would like to gratefully acknowledge Dr Martin L. Bocks, a previous contributor to this monograph. MLB declares that he has no competing interests.
Peer reviewers
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.
Consultant Cardiologist
Cardio-thoracic Unit
Guy's and St Thomas' Hospital
London
UK
Disclosures
JC declares that he has no competing interests.
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