Supravalvular Aortic Stenosis – Tubular Type

  • Hakimeh Sadeghian
  • Zahra Savand-Roomi


A 13-year-old boy presented with frequent episodes of syncopal attacks. Physical examination showed a systolic ejection murmur (grade IV/VI) in the aortic area with radiation into the jugular notch and along the carotid vessels. An ejection click was not heard, and the blood pressure was 120/80 mmHg in the left arm and 140/80 mmHg in the right arm. The patient had regular but unequal upper extremity pulses with prominence in the right upper extremity. He was referred to us for an evaluation of the cardiac cause of the syncopal attacks. Transthoracic echocardiography revealed a relatively small left ventricular cavity and a normal systolic function as well as concentric left ventricular hypertrophy.

Supplementary material

Video 105.1

(MP4 5847 kb)


  1. 2.
    Webb GD, Smallhorn J, Therrien J, Redington AN. Congenital heart disease. In: Bonow RO, Mann D, Zipes DP, Libby P, Breunwald E, editors. Braunwald heart disease, a text book of cardiovascular medicine. 9th ed. Philadelphia: Elsevier Saunders; 2012. p. 1411–67.Google Scholar
  2. 87.
    Lopez L. Abnormalities of left ventricular outflow. In: Eidem BW, Cetta F, O’Leary PW, editors. Echocardiography in pediatric and adult congenital heart disease. Philadelphia: Lippincott Williams and Wilkins; 2010. p. 215–36.Google Scholar
  3. 118.
    Schneider DJ, Moore J. Aortic stenosis. In: Allen HA, Driscoll D, Shaddy RE, Feltes TF, editors. Moss and Adams’ heart disease in infants, children, and adolescents. 8th ed. Philadelphia: Lippincott Williams & Wilkins; 2013. p. 1023–43.Google Scholar

Copyright information

© Springer International Publishing Switzerland 2015

Authors and Affiliations

  • Hakimeh Sadeghian
    • 1
  • Zahra Savand-Roomi
    • 2
  1. 1.Department of EchocardiographyTehran University of Medical Sciences Tehran Heart CentreTehranIran
  2. 2.Department of EchocardiographyKowsar HospitalShirazIran

Personalised recommendations