History

Pulmonic valvular stenosis (PVS) in adults may present as an asymptomatic systolic murmur, or they may complain of decreased exercise tolerance.

In a functional and hemodynamic study of 19 Belgian patients with isolated mild-to-moderate pulmonary valve stenosis but no previous cardiac interventions, investigators reported significant differences in the following at-rest and exertional parameters between patients and their age- and sex-matched controls^[23]:

Higher resting heart rate (P = 0.001)
Lower peak power (P = 0.006)
Lower peak oxygen uptake (V_O2) (P = 0.011) and lower oxygen uptake efficiency slope (P = 0.007)
Higher ventilatory equivalent for carbon dioxide (VE/V_CO2) (P = 0.01)

The investigators indicated an observed linear increase in the peak pulmonary valve gradient may suggest a fixed valve area during exercise.^[23]They also reported no signs of right heart functional or morphologic changes during exercise, with good ventricular performance.

Physical Examination

For most adults with pulmonic valvular stenosis (PVS), there are no classic phenotypic findings, and disease is suspected during cardiac examination.

For suspected Noonan syndrome, phenotypes include the following:

Facial anomalies such as low set ears and hairline, hypertelorism, micrognathia, webbed neck
Chest deformities (pectus excavatum)
Short stature
Intellectual disability

Cardiac examination findings correlate with the severity of stenosis, valve pathology, and other associated cardiac lesions. Rarely, overt right heart failure is seen (unless end-stage disease).

On auscultation, the first heart sound is normal and followed by a systolic ejection click loudest over the left upper sternal border.^{[11, 15]}The murmur decreases with inspiration; this is secondary to the premature opening of the pulmonary valve by the atrial kick into a stiff right ventricle.^[2]

The second heart sound may have a wide split, especially in severe disease. This is due to delayed closing of the pulmonic valve at the end of systole. The pulmonic component of the second heart sound may be diminished in intensity, and a right sided fourth heart sound may be heard.

In more severe pulmonic valvular stenosis, a right ventricular lift with associated jugular venous distention may be appreciated, and the pulmonary ejection murmur is much louder and longer.

Myocardial infarction of hypertrophied right ventricle may occur.^[9]Cyanosis may occur with right-to-left shunting at the atrial level as with a patent foramen ovale or septal defect.^[15]

Differential Diagnoses

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Author

Victoria Zaccone, MD Resident Physician, Departments of Emergency Medicine and Internal Medicine, Kings County Hospital, SUNY Downstate Medical Center

Victoria Zaccone, MD is a member of the following medical societies: American College of Physicians, American Medical Student Association/Foundation, Emergency Medicine Residents' Association

Disclosure: Nothing to disclose.

Coauthor(s)

Bobak Zonnoor , MD, MMM Assistant Professor of Emergency Medicine, SUNY Downstate School of Medicine; Director, ED Observation Unit, Department of Emergency Medicine, Kings County Hospital; Volunteer Assistant Clinical Professor, University of California, Los Angeles, David Geffen School of Medicine; Clinical Faculty, University of California, Riverside, School of Medicine

Bobak Zonnoor , MD, MMM is a member of the following medical societies: American College of Emergency Physicians, Emergency Medicine Residents' Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Barry E Brenner, MD, PhD, FACEP Program Director, Emergency Medicine, Einstein Medical Center Montgomery

Barry E Brenner, MD, PhD, FACEP is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Chest Physicians, American College of Emergency Physicians, American College of Physicians, American Heart Association, American Thoracic Society, New York Academy of Medicine, New York Academy of Sciences, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Additional Contributors

David Eitel, MD, MBA Associate Professor, Department of Emergency Medicine, York Hospital; Physician Advisor for Case Management, Wellspan Health System, York

David Eitel, MD, MBA is a member of the following medical societies: American College of Emergency Physicians, American Society of Pediatric Nephrology, Society for Academic Emergency Medicine, Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Acknowledgements

Mert Erogul, MD Assistant Professor of Emergency Medicine, University Hospital of Brooklyn: Consulting Staff, Department of Emergency Medicine, Kings County Hospital Center

Mert Erogul, MD is a member of the following medical societies: American College of Emergency Physicians, American Medical Association, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Allysia M Guy, MD Staff Physician, Department of Emergency Medicine, State University of New York Downstate Medical Center

Disclosure: Nothing to disclose.

Kurt Pflieger, MD, FAAP Active Staff, Department of Pediatrics, Lake Pointe Medical Center

Kurt Pflieger, MD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Emergency Physicians, American Heart Association, and Texas Medical Association

Disclosure: Nothing to disclose.

David J Wallace, MD, MPH Resident, Assistant Professor of Clinical Medicine, Departments of Emergency Medicine and Internal Medicine, Kings County Hospital.

David J Wallace is a member of the following medical societies: Alpha Omega Alpha, American College of Emergency Physicians, American Medical Association, Emergency Medicine Residents Association, Society for Academic Emergency Medicine, and Society of Critical Care Medicine.

Disclosure: Nothing to disclose.