Summary
Definition
History and exam
Key diagnostic factors
- sopro sistólico de ejeção patológico com ou sem clique sistólico
Other diagnostic factors
- febre reumática
- dispneia
- fadiga
- dor torácica
- síncope
- características de dismorfismo da síndrome de Noonan
- características de dismorfismo na síndrome de Noonan com lentigos múltiplos
- características de dismorfismo da síndrome de Williams
- características de dismorfismo da síndrome de Alagille
- retardo do crescimento pôndero-estatural
- cianose
- sinais de insuficiência cardíaca direita
- ictus do ventrículo direito
- frêmito sistólico
Risk factors
- etnia negra
- Síndrome de Noonan
- Síndrome de Noonan com lentigos múltiplos
- Síndrome de Alagille
- Síndrome de Williams
- síndrome da rubéola congênita
- síndrome carcinoide
- endocardite infecciosa
- tumores miocárdicos
- compressão externa
Diagnostic tests
1st tests to order
- eletrocardiograma (ECG)
- radiografia torácica
- ecocardiografia com avaliação por Doppler
Tests to consider
- cateterismo cardíaco diagnóstico
- hemoglobina (Hb) e hematócrito (Hct)
- oximetria de pulso
- gasometria arterial
- ressonância nuclear magnética (RNM) cardíaca
- TC cardíaca
- teste ergométrico
Treatment algorithm
doença leve
doença moderada
doença grave a crítica
Contributors
Authors
Jeremy Asnes, MD
Associate Professor of Pediatrics (Cardiology)
Yale Pediatric Cardiology
Yale New Haven Children's Hospital
New Haven
CT
Disclosures
JA is a Principal Investigator for the Medtronic Harmony™ Transcatheter Pulmonary Valve clinical trial. JA has also been a paid consultant for Medtronic, the manufacturer of the Melody and Harmony transcatheter pulmonary valves.
Britton Keeshan, MD, MPH
Assistant Professor of Clinical Pediatrics
Yale Pediatric Cardiology
Yale New Haven Children's Hospital
New Haven
CT
Disclosures
BK declares that he has no competing interests.
Acknowledgements
Dr Asnes and Dr Keeshan would like to gratefully acknowledge Dr Ivandito Kuntjoro, Dr Edgar Tay, Dr James Yip, Dr Kian Keong Pho, Dr Low Ting Ting, Dr Peter S. Fischbach, Dr Cyrus Samai, and Dr David M. Drossner, the previous authors of this topic.
Disclosures
ET, IK, JY, KKP, LTT, PSF, CS and DD declared that they had no competing interests.
Peer reviewers
John Charpie, MD, PhD
Associate Professor of Pediatrics
Medical Director
Pediatric Cardiothoracic Intensive Care Unit
University of Michigan Congenital Heart Center
Ann Arbor
MI
Disclosures
JC declares that he has no competing interests.
James Gnanapragasam, MBBS, MSc, FRCP, FRCPCH
Consultant in Paediatric Cardiology
Southampton University Hospitals NHS Trust
Southampton
UK
Disclosures
JG declares that he has no competing interests.
Peer reviewer acknowledgements
BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.
Disclosures
Peer reviewer affiliations and disclosures pertain to the time of the review.
References
Key articles
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. J Am Coll Cardiol. 2018 Aug 10 [Epub ahead of print].Full text Abstract
Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA guideline for the management of patients with valvular heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2021 Feb 2;143(5):e35-71.Full text Abstract
European Society of Cardiology. 2020 ESC guidelines for the management of adult congenital heart disease (previously grown-up congenital heart disease). Aug 2020 [internet publication].Full text
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Differentials
- Sopro inocente
- Síndrome das costas retas
- Dilatação idiopática da artéria pulmonar
More DifferentialsGuidelines
- ACR appropriateness criteria: congenital or acquired heart disease
- Guidelines for cardiovascular intervention in adults with congenital heart disease
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