Supravalvular aortic stenosis: Difference between revisions
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==Overview== | ==Overview== | ||
Supravalvular aortic stenosis is the most uncommon cause of [[left ventricular outflow tract obstruction]] ([[LVOT]]) accounting for 8% of congenital cases of [[LVOT obstruction]]. | |||
==Pathophysiology== | |||
#Obstruction occurs just above the coronary ostium at the level of the sinotubular junction: | #Obstruction occurs just above the coronary ostium at the level of the sinotubular junction: | ||
#*Hourglass type (the most common) | #*Hourglass type (the most common) | ||
#*Hypoplastic type: uniform narrowing of the ascending aorta. | #*Hypoplastic type: uniform narrowing of the ascending aorta. | ||
#Associated lesion is peripheral pulmonary arterial stenosis | #Associated lesion is peripheral [[pulmonary arterial stenosis]] | ||
#Because of high perfusion pressure of the coronary arteries there is premature | #Because of high perfusion pressure of the coronary arteries there is premature coronary artery disease. | ||
#Coronary arteries may be obstructed by an adjacent stenotic ring. | #Coronary arteries may be obstructed by an adjacent stenotic ring. | ||
== | ==Genetics== | ||
#One third of cases are transmitted as an autosomal dominant trait. | |||
==Diagnosis== | |||
#50% have a characteristically greater pulse and systolic blood pressure in the right carotid | ===Physical Examination== | ||
#50% have a characteristically greater pulse and systolic blood pressure in the right carotid and brachial arteries than in the left. | |||
#The systolic murmur is maximal below the right clavicle and radiates primarily to the right carotid artery. | #The systolic murmur is maximal below the right clavicle and radiates primarily to the right carotid artery. | ||
#No ejection click, no diastolic murmur. | #No ejection click is present, no diastolic murmur. | ||
===Echocardiography=== | |||
==References== | ==References== | ||
Revision as of 02:15, 10 April 2012
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Aortic Stenosis Microchapters |
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Diagnosis |
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Treatment |
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Percutaneous Aortic Balloon Valvotomy (PABV) or Aortic Valvuloplasty |
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Transcatheter Aortic Valve Replacement (TAVR) |
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Case Studies |
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Supravalvular aortic stenosis On the Web |
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American Roentgen Ray Society Images of Supravalvular aortic stenosis |
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Directions to Hospitals Treating Supravalvular aortic stenosis |
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Risk calculators and risk factors for Supravalvular aortic stenosis |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Supravalvular aortic stenosis is the most uncommon cause of left ventricular outflow tract obstruction (LVOT) accounting for 8% of congenital cases of LVOT obstruction.
Pathophysiology
- Obstruction occurs just above the coronary ostium at the level of the sinotubular junction:
- Hourglass type (the most common)
- Hypoplastic type: uniform narrowing of the ascending aorta.
- Associated lesion is peripheral pulmonary arterial stenosis
- Because of high perfusion pressure of the coronary arteries there is premature coronary artery disease.
- Coronary arteries may be obstructed by an adjacent stenotic ring.
Genetics
- One third of cases are transmitted as an autosomal dominant trait.
Diagnosis
=Physical Examination
- 50% have a characteristically greater pulse and systolic blood pressure in the right carotid and brachial arteries than in the left.
- The systolic murmur is maximal below the right clavicle and radiates primarily to the right carotid artery.
- No ejection click is present, no diastolic murmur.