Summary
Definition
History and exam
Other diagnostic factors
- dyspnea
- decreased exercise tolerance
- diastolic murmur
- orthopnea
- paroxysmal nocturnal dyspnea
- 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
- transthoracic Doppler echocardiogram (TTE)
- CXR
Investigations to consider
- transesophageal Doppler echocardiogram (TEE)
- MRI chest
- CT scan chest
- cardiac MRI
Treatment algorithm
Contributors
Authors

Interventional Cardiology
Prairie Heart Institute at St John's Hospital
Springfield
IL
Disclosures
SSG declares that he has no competing interests.
Interventional Cardiology Fellow
Department of Cardiovascular Medicine
Cleveland Clinic
Cleveland
OH
Disclosures
CPH declares that he has no competing interests.

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.
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
Consultant in Paediatric Cardiology and Adults with Congenital Heart Disease
John Radcliffe Hospital
Oxford
UK
Disclosures
SA declares that he has no competing interests.
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.
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.
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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