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Pulmonary stenosis

Last reviewed: 9 Apr 2025
Last updated: 28 Jun 2024

Summary

Definition

History and exam

Key diagnostic factors

  • pathologic systolic ejection murmur with or without a systolic click
Full details

Other diagnostic factors

  • 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
Full details

Risk factors

  • black ethnicity
  • Noonan syndrome
  • Noonan syndrome with multiple lentigines
  • Alagille syndrome
  • Williams syndrome
  • congenital rubella syndrome
  • carcinoid syndrome
  • infectious endocarditis
  • myocardial tumors
  • external compression
Full details

Diagnostic tests

1st tests to order

  • ECG
  • chest x-ray
  • echocardiography with Doppler assessment
Full details

Tests to consider

  • diagnostic cardiac catheterization
  • Hb and Hct
  • pulse oximetry
  • arterial blood gas
  • cardiac MRI
  • cardiac CT
  • exercise stress testing
Full details

Treatment algorithm

ACUTE

mild disease

moderate disease

severe to critical disease

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.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

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.
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