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Pulmonary stenosis

最后审阅: 21 Aug 2025
最后更新: 28 Jun 2024

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
完整详情

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
完整详情

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
完整详情

诊断性检查

首要检查

  • ECG
  • chest x-ray
  • echocardiography with Doppler assessment
完整详情

需考虑的检查

  • diagnostic cardiac catheterization
  • Hb and Hct
  • pulse oximetry
  • arterial blood gas
  • cardiac MRI
  • cardiac CT
  • exercise stress testing
完整详情

治疗流程

急症处理

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

Nuestros equipos internos de evidencia y editoriales colaboran con colaboradores expertos internacionales y revisores pares para garantizar que brindemos acceso a la información más clínicamente relevante posible.

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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