SHA24/028005

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Repair of Congenital Aortic Valve Abdulaziz Alkhaldi, MD, MSc, FRCSC Consultant Adult & Pediatric Cardiac Surgery, King Abdulaziz Medical City Assistant Professor, King Saud bin Abdulaziz University for Health Sciences Principal Investigator, King Abdullah International Medical Research Center



Secondary Changes in Congenital Aortic Valve Disease



Quadricuspid AV • Most rare variant (< 200 cases reported). • More common with Truncal valve. • Commonly found as incidental finding by Echo (incidence = 0.013 – 0.043%).


Unicuspid AV • Incidence = 0.02% by Echocardiography • Two sub-types: – One commissure with or without raphe. – No commissures with central opening • Typically presents early with stenosis or late with calcification.


Bicuspid Aortic Valve (BAV) • Incidence = 0.4 – 2.0 % (most common congenital cardiovascular malformation) • Accounts for ~ 50% of adult aortic valve diseases.


Limited Opening

Reduced Free margin length / Annular attachment length

Poor Coaptation

Reduced height of inter-leaflet triangle and reduced depth of cusps.


Bicuspid Aortic Valve Conjoint cusp

Reference cusp Conjoint cusp / Reference cusp ratio = 1:1 to 2:1



Are Outcomes of Surgical Versus Transcatheter Balloon Valvotomy Equivalent in Neonatal Critical Aortic Stenosis? Brian W. McCrindle, Eugene H. Blackstone, William G. Williams, Rekwan Sittiwangkul, Thomas L. Spray, Anthony Azakie, Richard A. Jonas and the members of the Congenital Heart Surgeons Society Circulation. 2001;104:I-152-I-158 doi: 10.1161/hc37t1.094837

Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright Š 2001 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539

The online version of this article, along with updated information and services, is located on the World Wide Web at: http://circ.ahajournals.org/content/104/suppl_1/I-152


Newer adjunct techniques for surgical repair of congenital AS

Tolan MJ et al. Ann Thorac Surg 1997;63:465-469


MORPHOLOGIC DETERMINANTS FAVORING SURGICAL AORTIC VALVULOPLASTY VERSUS PULMONARY AUTOGRAFT AORTIC VALVE REPLACEMENT IN CHILDREN

Jacques A. M. van Son, MD*, V. Mohan Reddy, MD, Michael D. Black, MD, Hiranya Rajasinghe, MD, Gary S. Haas, MD, Frank L. Hanley, MD J Thorac Cardiovasc Surg 1996;111:1149-1157

J Thorac Cardiovasc Surg. 1996 Jun;111(6):1149-56



Circular aortic annuloplasty

Commissural plication







Precise fitting cusp augmentation

Smith PC, et al. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 1999; 2: 83–94

Standard shape cusp augmentation

Kalangos A, et al. Aortic valve repair by cusp extension with the use of fresh autologous pericardium in children with rheumatic aortic insufficiency. J Thorac Cardiovasc Surg 1999; 118: 225–236



Rao V, et al. Aortic valve repair for adult congenital heart disease. A 22-year experience. Circulation 2000;102(Suppl. III):III40 – 3.


Univariate predictors of reoperation or recurrent aortic insufficiency after surgical repair of congenital aortic valve

Rao V, et al. Aortic valve repair for adult congenital heart disease. A 22-year experience. Circulation 2000;102(Suppl. III):III40 – 3.


Valve Configuration Determines Long-Term Results After Repair of the Bicuspid Aortic Valve Configuration Determines Long-Term Results After Repair of the Bicuspid Aortic Valve Diana Aicher, Takashi Kunihara, OmarValve Abou Issa, Brigitte Brittner, Stefan Gräber and Diana Aicher, Takashi Kunihara, Hans-Joachim Omar Abou Issa, Brigitte Brittner, Stefan Gräber and Schäfers Hans-Joachim Schäfers Circulation. 2011;123:178-185; originally published online January 3, 2011; Circulation. 2011;123:178-185; originally published online January 3, 2011; doi: 10.1161/CIRCULATIONAHA.109.934679 doi:by10.1161/CIRCULATIONAHA.109.934679 Circulation is published the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231

Circulation is published by the Heart Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © American 2011 American Association, Inc. All rights reserved. Copyright © 2011 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539 Print ISSN: 0009-7322. Online ISSN: 1524-4539

The online version of this article, along with updated information and services, is located on the The online version of this article, along World with updated information and services, is located on the Wide Web at: World Wide Web at: http://circ.ahajournals.org/content/123/2/178 http://circ.ahajournals.org/content/123/2/178


Aicher D et al. Circulation 2011;123:178-185


Summary • Surgical repair of congenital aortic valve disease (Stenosis / Insufficiency) is a feasible and attractive option in children and young patients. • Durability of the repair is variable and in some pathologies limited.


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