Approach Considerations

Laboratory evaluation usually is not helpful.

Oximetry provides information on possible right-to-left shunting in borderline cyanotic lesions or in patients with anemia but does not identify the cause of the shunt (pulmonary, interatrial, interventricular, great arterial).^[15]

Although arterial blood gases (ABG) analysis usually is not needed, one notable exception is the hyperoxia test in newborns with cyanosis of undetermined origin.

Administered 100% FIO₂ generally does not increase the partial pressure of oxygen to levels much greater than 100 mm Hg in patients with a cyanotic congenital heart defect.

Electrocardiography is not diagnostic, and in mild to moderate pulmonic valvular stenosis will yield normal results. In severe pulmonic valvular stenosis, right axis deviation and right ventricular hypertrophy may occur.

According to the American College of Cardiology/American Heart Association guidelines for the management of adults with congenital heart disease, cardiac catheterization is unnecessary for diagnosis and should only be utilized when percutaneous catheter intervention is considered.^[2]

Chest radiography

Chest radiography may demonstrate a prominent main pulmonary artery segment but a normal heart size; however, it may also present as congestive heart failure (CHF), with cardiomegaly secondary to enlarged right atria and ventricle.

Computed tomography scanning and magnetic resonance imaging

Modalities such as computed tomography (CT) scanning and magnetic resonance imaging (MRI) can show structural cardiac abnormalities.^{[24, 25]}They are the preferred modality when echocardiogram cannot be used.

One case report described the identification of a patient with isolated subvalvular pulmonary stenosis using whole-heart MRI.^[26]This imaging tool is noninvasive and has the added benefit of creating a 3-dimensional representation of the heart and surrounding structures. Other reports have described the use of whole-heart MRI as a presurgical adjunct.^[24]

Cardiac CT has been beneficial for noninvasive presurgical evaluation of someone with known coronary artery disease. The radiation exposure is minimal, and the reconstructions of a 64-slice CT can yield very accurate images of myocardium changes associated with outflow tract obstruction.^[25]

Echocardiography

Echocardiography is safer and more effective in the diagnosis of pulmonic valvular stenosis (PVS) compared with cardiac catheterization. It can determine flow gradients across the pulmonic valve, which is an indicator of disease severity.^[7]Multiple views and measurements increase the accuracy. Additionally, in the parasternal short axis view, "doming" can be seen, which is evidence of a thickened pulmonic valve with restricted systolic motion.

The transthoracic approach provides valuable information about the site of obstruction and other possible congenital abnormalities.^{[18, 27, 28, 29]}The transesophageal approach can also be used if the views are suboptimal, or if assessing for infective endocarditis.^[2]Practice guidelines use echocardiography to determine definitive management in pulmonic valvular stenosis and as part of patient follow up.^[30]

In a study that compared live/real-time three-dimensional transesophageal echocardiography (3D-TEE) with two-dimensional transesophageal echocardiography (2D-TEE) to determine whether there are advantages to using 3D-TEE on patients with pulmonary stenosis, investigators prospectively enrolled 16 consecutive adult patients with pulmonary stenosis and indications of TEE. They found evidence of the incremental value of using 3D-TEE instead of 2D-TEE during assessments of pulmonary stenosis, specifically in cases where special conditions cause inaccuracy in recordings of the transvalvular peak gradient. The investigators concluded that during routine echocardiographic examinations, 3D-TEE should be used as a complementary imaging tool to 2D-TEE.^[31]

Most children with pulmonary stenosis do not require further evaluation beyond echocardiography.

Treatment & Management

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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.