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Last reviewed: 9 Mar 2025
Last updated: 02 Apr 2025

Summary

Definition

History and exam

Key diagnostic factors

  • history of acute rheumatic fever (ARF) during childhood
  • dyspnoea
  • elevated jugular venous pressure with prominent a-wave
  • low-frequency pre-systolic (diastolic) murmur at lower left sternal border

Other diagnostic factors

  • age: 20-39 years
  • exercise intolerance
  • fatigue
  • jugular pulsations
  • abdominal swelling and discomfort
  • oedema
  • cyanosis or hypoxaemia
  • atrial fibrillation
  • hepatomegaly
  • ascites, oedema, anasarca
  • age: infancy or childhood
  • episodic facial flushing, watery diarrhoea, or bronchoconstriction
  • absent right ventricular lift or heave
  • opening snap
  • endocarditis stigmata (e.g., splinter haemorrhages, Osler nodes, Janeway lesions)

Risk factors

  • group A streptococcal (GAS) pharyngitis
  • metastatic carcinoid tumours
  • 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
  • chest x-ray
  • 2D transthoracic echocardiogram
  • Doppler transthoracic echocardiogram
  • liver function tests
  • blood biochemistry
  • FBC
  • blood cultures
  • 24-hour urinary excretion of 5-hydroxy-indole acetic acid (5-HIAA)

Investigations to consider

  • cardiac catheterisation
  • cardiac MRI
  • 3D transthoracic echocardiogram
  • cardiac CT angiography (CTA)
  • [18F] fluorodeoxyglucose (FDG)-PET/CT

Treatment algorithm

Contributors

Authors

John R. Charpie, MD, PhD
John R. Charpie

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
Jeffrey D. Zampi

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, null

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.

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