小结
定义
病史和体格检查
关键诊断因素
- pathologic systolic ejection murmur with or without a systolic click
其他诊断因素
- rheumatic fever
- dyspnea
- fatigue
- chest pain
- syncope
- dysmorphic features of Noonan syndrome
- dysmorphic features of Noonan syndrome with multiple lentigines
- dysmorphic features of Williams syndrome
- dysmorphic features of Alagille syndrome
- failure to thrive
- cyanosis
- signs of right heart failure
- right ventricular heave
- systolic thrill
危险因素
- black ethnicity
- Noonan syndrome
- Noonan syndrome with multiple lentigines
- Alagille syndrome
- Williams syndrome
- congenital rubella syndrome
- carcinoid syndrome
- infectious endocarditis
- myocardial tumors
- external compression
诊断性检查
首要检查
- ECG
- chest x-ray
- echocardiography with Doppler assessment
需考虑的检查
- diagnostic cardiac catheterization
- Hb and Hct
- pulse oximetry
- arterial blood gas
- cardiac MRI
- cardiac CT
- exercise stress testing
治疗流程
mild disease
moderate disease
severe to critical disease
撰稿人
作者
Jeremy Asnes, MD
Associate Professor of Pediatrics (Cardiology)
Yale Pediatric Cardiology
Yale New Haven Children's Hospital
New Haven
CT
利益声明
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
Divulgaciones
BK declares that he has no competing interests.
Agradecimientos
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.
Divulgaciones
ET, IK, JY, KKP, LTT, PSF, CS and DD declared that they had no competing interests.
Revisores por pares
John Charpie, MD, PhD
Associate Professor of Pediatrics
Medical Director
Pediatric Cardiothoracic Intensive Care Unit
University of Michigan Congenital Heart Center
Ann Arbor
MI
Divulgaciones
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
Divulgaciones
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.
Referencias
Artículos principales
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].Texto completo Resumen
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.Texto completo Resumen
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].Texto completo
Artículos de referencia
Una lista completa de las fuentes a las que se hace referencia en este tema está disponible para los usuarios con acceso a todo BMJ Best Practice.
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