Summary
Definition
History and exam
Key diagnostic factors
- systolic ejection murmur
- fixed splitting of the second heart sound
Other diagnostic factors
- mid-diastolic murmur
- congestive cardiac failure
- faltering growth
- symptoms of atrial arrhythmias
- cyanosis
- finger clubbing
Risk factors
- female sex
- maternal alcohol consumption
- positive family history
Diagnostic tests
1st tests to order
- echocardiogram
- ECG
- chest x-ray
Tests to consider
- pulse oximetry
- chest CT/MRI
- cardiac catheterization
Treatment algorithm
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.
References
Key articles
Jone PN, Ivy DD, Hauck A, et al. Pulmonary hypertension in congenital heart disease: a scientific statement from the American Heart Association. Circ Heart Fail. 2023 Jul;16(7):e00080.Full text Abstract
Baumgartner H, De Backer J, Babu-Narayan SV, et al; ESC Scientific Document Group. 2020 ESC guidelines for the management of adult congenital heart disease. Eur Heart J. 2021 Feb 11;42(6):563-645.Full text Abstract
Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on clinical practice guidelines. Circulation. 2019 Apr 2;139(14):e698-800.Full text Abstract
Abrahamyan L, Dharma C, Alnasser S, et al. Long-term outcomes after atrial septal defect transcatheter closure by age and against population controls. JACC Cardiovasc Interv. 2021 Mar 8;14(5):566-75.Full text Abstract
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
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
More GuidelinesLog in or subscribe to access all of BMJ Best Practice
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