Pulmonary stenosis

Last reviewed: 30 Dec 2022
Last updated: 23 Dec 2022

Summary

Definition

History and exam

Key diagnostic factors

  • pathologic systolic ejection murmur with or without a systolic click
More key diagnostic factors

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
Other diagnostic factors

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
More risk factors

Diagnostic investigations

1st investigations to order

  • ECG
  • chest x-ray
  • echocardiography with Doppler assessment
More 1st investigations to order

Investigations to consider

  • diagnostic cardiac catheterization
  • Hb and Hct
  • pulse oximetry
  • arterial blood gas
  • cardiac MRI
  • cardiac CT
  • exercise stress testing
More investigations to consider

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.

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.

  • Pulmonary stenosis images
  • Differentials

    • Innocent murmur
    • Straight back syndrome
    • Idiopathic dilation of pulmonary artery
    More Differentials
  • Guidelines

    • Guidelines for cardiovascular intervention in adults with congenital heart disease
    • Guidelines for the management of adult congenital heart disease (previously grown-up congenital heart disease)
    More Guidelines
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