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Last reviewed: 18 Mar 2025
Last updated: 15 Mar 2024

Summary

Definition

History and exam

Key diagnostic factors

  • presence of risk factors

Other diagnostic factors

  • dyspnoea
  • decreased exercise tolerance
  • diastolic murmur
  • orthopnoea
  • paroxysmal nocturnal dyspnoea
  • palpitations
  • fatigue
  • diaphoresis
  • displaced point of maximal apical impulse
  • systolic murmur
  • signs of right-sided heart failure

Risk factors

  • pulmonary hypertension
  • surgical repair of tetralogy of Fallot, pulmonary stenosis, or pulmonary atresia
  • endocarditis
  • left-sided heart disease
  • previous Ross procedure (with prosthetic pulmonary valve, homograft valve replacement)
  • collagen vascular disease
  • malignancies that involve the main pulmonary artery

Diagnostic investigations

1st investigations to order

  • ECG
  • trans-thoracic Doppler echocardiogram (TTE)
  • CXR

Investigations to consider

  • trans-oesophageal Doppler echocardiogram (TEE)
  • cardiac MRI
  • MRI chest
  • CT scan chest

Treatment algorithm

Contributors

Authors

Karen K. Stout, MD, FACC

Professor of Medicine

Adjunct Professor of Pediatrics and Cardiology

Division of Cardiology

University of Washington

Seattle

WA

Disclosures

KKS is former chair of a prior ACC/AHA guidelines committee and an author of a guideline cited in this topic.

Andrew Pistner, MD

Assistant Professor of Medicine

Division of Cardiology

University of Washington

Seattle

WA

Disclosures

AP declares that he has no competing interests

Acknowledgements

Dr Karen K. Stout and Dr Andrew Pistner would like to gratefully acknowledge Dr Sachin S. Goel, Dr Chetan P. Huded, Dr Samir Kapadia, and Dr Mehdi H. Shishehbor, previous contributors to this topic.

Disclosures

SSG, CPH, SK, and MHS declare that they have no competing interests.

Peer reviewers

Satish Adwani, MD

Consultant in Paediatric Cardiology and Adults with Congenital Heart Disease

John Radcliffe Hospital

Oxford

UK

Disclosures

SA declares that he has no competing interests.

Sarabjeet Singh, MD

Cardiology Fellow

Adult Cardiovascular Fellowship

Department of Cardiology

Chicago Medical School (CMS) and affiliated hospitals

Chicago

IL

Disclosures

SS declares that he has no competing interests.

Debabrata Mukherjee, MD

Gill Foundation Professor of Interventional Cardiology

Director of Cardiac Catheterization Laboratories

Gill Heart Institute

Division of Cardiovascular Medicine

University of Kentucky

Lexington

KY

Disclosures

DM declares that he has no competing interests.

Syed Wamique Yusuf, MD, MRCPI, FACC

Associate Professor

University of Texas MD Anderson Cancer Center

Department of Cardiology

Houston

TX

Disclosures

SWY declares that he has no competing interests.

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