Last reviewed: 27 Jun 2021
Last updated: 05 Feb 2019

Summary

Definition

History and exam

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)
  • MRI chest
  • CT scan chest
  • cardiac MRI

Treatment algorithm

Contributors

Authors

Sachin S. Goel, MD
Sachin S. Goel

Interventional Cardiology

Prairie Heart Institute at St John's Hospital

Springfield

IL

Disclosures

SSG declares that he has no competing interests.

Chetan P. Huded, MD, MSc

Interventional Cardiology Fellow

Department of Cardiovascular Medicine

Cleveland Clinic

Cleveland

OH

Disclosures

CPH declares that he has no competing interests.

Samir Kapadia, MD
Samir Kapadia

Director of Cardiac Cath Lab

Professor of Medicine

Department of Cardiovascular Medicine

Cleveland Clinic

Cleveland

OH

Disclosures

SK declares that he has no competing interests.

Acknowledgements

Dr Sachin S. Goel, Dr Chetan P. Huded, and Dr Samir Kapadia would like to gratefully acknowledge Dr Mehdi H. Shishehbor, the previous contributor to this topic. MHS declares that he has 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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