Last reviewed: November 2017
Last updated: November  2017

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)
Full details

Investigations to consider

  • cardiac catheterization
  • cardiac MRI
  • 3D transthoracic echocardiogram
Full details

Treatment algorithm

Contributors

Authors VIEW ALL

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

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 VIEW ALL

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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