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Pediatric Supravalvar Aortic Stenosis Clinical Presentation

  • Author: Anita Krishnan, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
Updated: Aug 26, 2015


Symptoms caused by SVAS usually develop in childhood. Rarely, symptoms may develop in infancy; in some cases, symptoms develop in the second or third decade of life.

Most pediatric patients present because of a heart murmur or the features of Williams syndrome. Patients with Williams syndrome may also develop systemic hypertension and involvement of joints, peripheral pulmonary artery stenosis, coarctation of aorta, and mitral insufficiency.[13]

Dyspnea on exertion, angina, and syncope develop in the course of the disease if SVAS is untreated. These symptoms indicate at least a moderate degree of LVOT obstruction. Because of the coronary artery involvement, angina may arise early and more often than in other obstructive LVOT lesions. Because of the risk of sudden death in SVAS, the development of angina and syncope should prompt immediate investigation.


Physical Examination

The physical examination focuses on upper extremity pulses, the precordium, heart sounds, and heart murmurs.

Asymmetrical upper extremities pulses

Discrepancies between carotid pulsations and upper extremity pulses and blood pressure are the characteristic clinical findings in SVAS. The discrepancies occur because the jet of blood flow from SVAS has a preferential trajectory into the brachiocephalic (innominate) artery (ie, Coanda effect).


The precordium is usually hyperdynamic, and the apex of the heart is displaced laterally and inferiorly because of ventricular hypertrophy. A thrill in the suprasternal notch is usually felt because of the trajectory of the blood flow jet from SVAS.

Heart sounds

The first heart sound is generally normal. A narrowly split, single, or paradoxically split second heart sound and a fourth heart sound are present in severe SVAS.

Heart murmurs

The characteristic systolic murmur of SVAS is crescendo-decrescendo in shape, low pitched, and best heard at the base of the heart, sited higher than in valvular aortic stenosis. It mainly radiates to the right carotid artery and tends to peak during the last two thirds of ventricular systole if the obstruction is severe.

A high-pitched, short, early diastolic aortic regurgitation murmur is uncommon in SVAS unless the aortic valve has become damaged due to the supravalve obstruction and has become regurgitant.  An ejection click is absent.

Contributor Information and Disclosures

Anita Krishnan, MD Assistant Professor of Pediatrics, George Washington University School of Medicine; Attending Physician, Division of Cardiology, Children’s National Medical Center

Disclosure: Nothing to disclose.


Gautam K Singh, MD, , MRCP Professor of Pediatrics, Division of Cardiology, Director of Noninvasive Imaging Research, Co-Director of Echocardiography Laboratory, Washington University in St Louis School of Medicine; Attending Faculty, Department of Pediatrics, Division of Cardiology, St Louis Children's Hospital

Gautam K Singh, MD, , MRCP is a member of the following medical societies: American College of Cardiology, American Heart Association, American Society of Echocardiography, Royal College of Physicians

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

John W Moore, MD, MPH Professor of Clinical Pediatrics, Section of Pediatic Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital

John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Chief Editor

Howard S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children's Hospital

Howard S Weber, MD, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Received income in an amount equal to or greater than $250 from: St. Jude Medical.

Additional Contributors

Charles I Berul, MD Professor of Pediatrics and Integrative Systems Biology, George Washington University School of Medicine; Chief, Division of Cardiology, Children's National Medical Center

Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, Heart Rhythm Society, Cardiac Electrophysiology Society, Pediatric and Congenital Electrophysiology Society, American College of Cardiology, American Heart Association, Society for Pediatric Research

Disclosure: Received grant/research funds from Medtronic for consulting.

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Two-dimensional suprasternal echocardiographic image of supravalvar aortic stenosis.
Aortogram of a patient with supravalvar aortic stenosis and dilated sinus of Valsalva.
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