Quadricuspid aortic valve: Difference between revisions
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'''Associate Editors-In-Chief:''' {{CZ}}; [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu] | '''Associate Editors-In-Chief:''' {{CZ}}; [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu] | ||
==Overview== | ==Overview== | ||
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Latest revision as of 14:10, 6 September 2012
| Quadricuspid aortic valve | |
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| Truncus Arteriosus With Subvalvular Interventricular Septal Defect: Gross natural color excellent view of lesion looking at opened aortic ring with quadricuspid aortic valve large subvalvular defect origin of pulmonary arteries is at forceps. Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Associate Editors-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Keri Shafer, M.D. [3]
Overview
Quadricuspid aortic valve is a rare congenital anomaly and usually found at surgery or diagnosed preoperatively during echocardiographic examination or aortography. [1]
The most common hemodynamic abnormality associated with this anomaly is aortic insufficiency.
Embryology
The semilunar valves are derived from mesenchymal swellings in the aortic and pulmonary trunk after the truncus arteriosus has been partitioned.
It is in the early stages of truncal separation that four subendothelial buds appear instead of three.
The presence of a corpus arnatii on all four cusps indicates that the valve resulted from abnormal embryogenesis. [2]
History
The first known case report was written by Balington in 1862. [3]
The prevalence of quadricuspid aortic valve is (by historical autopsy review) approximately 0.008%. [4] A modern echocardiography database review showed the prevalence to be somewhat higher, depending on the years reviewed (0.013%–0.043%). [5] [6]
Pathophysiology
On pathological descriptions the aortic valve has four cusps:
- a posterior cusp,
- a left coronary cusp,
- a right coronary cusp,
- an anterior supernumerary cusp.
Raphes join the supernumerary cusp with the left and right coronary cusps. The raphes have been described as shallow and chordlike. The supernumerary cusp can have multiple fenestrations.
The cusps can vary in;
- Size,
- Thickness
- Pliability
Classification
- Four equal cusp,
- Three equal large and one small cusp (the most common type),
- Two equal large and two equal small cusps,
- One large, two equal intermediate and one small cusps,
- Three equal small and one larger cusps,
- Two equal large and two unequal smaller cusps,
- Four unequal cusps. [7]
Associated cardiac abnormalities
- Displaced coronary ostia
- Single or accessory coronary ostia
- Isolated coronary ostium
- Patent ductus arteriosus
- Congenitally deficient mitral leaflets
- Ventricular septal defect
- Fibromuscular subaortic stenosis
- Non-obstructive hypertrophic cardiomyopathy
- Aortic insufficiency (the most common)
Diagnosis
Echocardiography
- Quadricuspid Aortic Valve 1
<googlevideo>3949482772610719752&hl=en</googlevideo>
- Quadricuspid Aortic Valve 2
<googlevideo>-751422357787911615&hl=en</googlevideo>
- Quadricuspid Aortic Valve 3
<googlevideo>7157030825633724404&hl=en</googlevideo>
- Quadricuspid Aortic Valve 4
<googlevideo>1729835828662359814&hl=en</googlevideo>
- Quadricuspid Aortic Valve 5
<googlevideo>966621058418025889&hl=en</googlevideo>
- Quadricuspid Aortic Valve 6
<googlevideo>7337985372630045044&hl=en</googlevideo>
- Quadricuspid Aortic Valve 7
<googlevideo>-5429693187408571619&hl=en</googlevideo>
- Quadricuspid Aortic Valve 8
<googlevideo>-2395092633273759677&hl=en</googlevideo>
- Quadricuspid Aortic Valve 9
<googlevideo>-1101037639424512577&hl=en</googlevideo>
Pathological Findings
-
Truncus arteriosus with Subvalvular Interventricular Septal Defect: Gross natural color excellent view of lesion looking at opened aortic ring with quadricuspid aortic valve large subvalvular defect origin of pulmonary arteries is at forceps.
References
- ↑ Timperley J, Milner R, Marshall AJ, Gilbert TJ. Quadricuspid aortic valves. Clin Cardiol. 2002 Dec; 25(12):548-52. PMID 12492123
- ↑ Am J Cardiol 1991;67:323-324
- ↑ Balington J, quoted by Robicsek F, Sanger PW, Daugherty HK, Montgomery CC. Congenital quadricuspid aortic valve with displacement of the left coronary orifice. Am J Cardiol 1969;23:288–90. PMID 5772948
- ↑ Tutarel O. The quadricuspid aortic valve: a comprehensive review. J Heart Valve Dis 2004;13(4):534–7. PMID 15311857
- ↑ Feldman BJ, Khandheria BK, Warnes CA, Seward JB, Taylor CL, Tajik AJ. Incidence, description and functional assessment of isolated quadricuspid aortic valves. Am J Cardiol. 1990 Apr 1;65(13):937-8. PMID 2181849
- ↑ Recupero A, Pugliatti P, Rizzo F, Arrigo F, Coglitore SQuadricuspid aortic valve: a rare cause of aortic insufficiency diagnosed by doppler echocardiography. Report of two cases and review of the literature. Ital Heart J. 2005 Nov; 6(11): 927-30. PMID 16320931
- ↑ Hurwitz LE, Roberts WC. Quadricuspid semilunar valve. Am J Cardiol. 1973 May;31(5):623-6 PMID 4698133