Last reviewed: 21 Oct 2024
Last updated: 06 Aug 2024
Summary
Definition
History and exam
Key diagnostic factors
- systolic ejection murmur
- fixed splitting of the second heart sound
Full details
Other diagnostic factors
- presence of risk factors
- mid-diastolic murmur
- congestive cardiac failure
- faltering growth
- symptoms of atrial arrhythmias
- cyanosis
- finger clubbing
Full details
Risk factors
- female sex
- maternal alcohol consumption
- positive family history
Full details
Diagnostic investigations
1st investigations to order
- echocardiogram
- ECG
- chest x-ray
Full details
Investigations to consider
- pulse oximetry
- chest CT/MRI
- cardiac catheterisation
Full details
Treatment algorithm
ACUTE
pulmonary vascular resistance (PVR) <5 Wood units (WU) and Qp:Qs <1.5
PVR <5 WU and Qp:Qs ≥1.5, or evidence of right atrial enlargement
PVR ≥5 WU
Contributors
Authors
Christoph Haller, MD
Staff Cardiovascular Surgeon
Division of Cardiovascular Surgery
Labatt Family Heart Centre
The Hospital for Sick Children
Assistant Professor
University of Toronto
Toronto
Canada
Disclosures
CH declares that he has no competing interests.
Acknowledgements
Dr Christoph Haller would like to gratefully acknowledge Dr Sachin Khambadkone, Dr Brandon Lane Phillips, Dr Frank Cetta, and Dr David J. Driscoll, previous contributors of this topic.
Disclosures
SK, BLP, FC, and DJD declare that they have no competing interests.
Peer reviewers
Chawki Elzein, MD
Professor of Surgery
University of Illinois at Chicago
Advocate Children's Hospital
Chicago
IL
Disclosures
CE declares that he has no competing interests.
Differentials
- Partial anomalous pulmonary venous drainage
- Ventricular septal defect
- Patent ductus arteriosus
More DifferentialsGuidelines
- ACR Appropriateness Criteria® congenital or acquired heart disease
- 2023 ESC guidelines for the management of infective endocarditis
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