Quadricuspid Aortic Valve: A Case Report and Review

Pramod Theetha Kariyanna¹, Jonathan Francois¹, Amog Jayarangaiah², Yuvraj Singh Chowdhury¹, Richard Grodman³, Moro O. Salifu¹ and Isabel M. McFarlane^1,

¹Department of Internal Medicine, State University of New York, Downstate Medical Center, Brooklyn NY 11203, USA

²Trinity School of Medicine, Ratho Mill, Ribishi, St. Vincent and Grenadines

³Richmond University Medical Center, Department of Cardiology, Staten Island, New York, United States

Cite this paper

Pramod Theetha Kariyanna, Jonathan Francois, Amog Jayarangaiah, Yuvraj Singh Chowdhury, Richard Grodman, Moro O. Salifu and Isabel M. McFarlane. Quadricuspid Aortic Valve: A Case Report and Review. American Journal of Medical Case Reports. 2020; 8(8):253-256. doi: 10.12691/AJMCR-8-8-11

Abstract

Quadricuspid aortic valve (QAV) is a rare congenital valvular abnormality with less than 200 cases reported to date. The first QAV was reported in 1862 by Balington. Clinical manifestations of patients with a QAV depend on the functional status of the QAV and the associated cardiac disorders. Most QAV are asymptomatic and are incidentally found. Severe aortic regurgitation and/or stenosis can develop overtime, requiring aortic valve replacement or repair. Transesophageal echocardiography is the preferred modality to diagnose QAV. We present a case of QAV which was incidentally diagnosed in a 43-year-old woman who presented with gastroenteritis.

Keywords

Quadricuspid aortic valve, congenital variant of aortic valve, Hurwitz and Robert’s classification

Copyright

This work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

References

[1]	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; 65: 937-8.
[2]	Timperley J, Milner R, Marshall AJ, Gilbert TJ. Quadricuspid aortic valves. Clin Cardiol 2002; 25: 548-52.
[3]	James KB, Centorbi LK, Novoa R. Quadricuspid aortic valve. Case report and review of the literature. Tex Heart Inst J 1991; 18: 141-3.
[4]	Khan SK, Tamin SS, Araoz PA. Quadricuspid aortic valve by cardiac magnetic resonance imaging: a case report and review of the literature. J Comput Assist Tomogr 2011; 35: 637-41.
[5]	Roberts WC. The congenitally bicuspid aortic valve. A study of 85 autopsy cases. Am J Cardiol 1970; 26: 72-83.
[6]	Falcone MW, Roberts WC, Morrow AG, Perloff JK. Congenital aortic stenosis resulting from a unicommisssural valve. Clinical and anatomic features in twenty-one adult patients. Circulation 1971; 44: 272-80.
[7]	Yotsumoto G, Iguro Y, Kinjo T, Matsumoto H, Masuda H, Sakata R. Congenital quadricuspid aortic valve: report of nine surgical cases. Ann Thorac Cardiovasc Surg 2003; 9: 134-7.
[8]	Olson LJ, Subramanian R, Edwards WD. Surgical pathology of pure aortic insufficiency: a study of 225 cases. Mayo Clin Proc 1984; 59: 835-41.
[9]	Tutarel O. The quadricuspid aortic valve: a comprehensive review. J Heart Valve Dis 2004; 13: 534-7.
[10]	Peretz DI, Changfoot GH, Gourlay RH. Four-cusped aortic valve with significant hemodynamic abnormality. Am J Cardiol 1969; 23: 291-3.
[11]	Hurwitz LE, Roberts WC. Quadricuspid semilunar valve. Am J Cardiol 1973; 31: 623-6.
[12]	Simonds J. Congenital malformation of aortic and pulmonary valves. American Journal of the Medical Sciences 1923; 166(4): 584-95.
[13]	Bogers AJJC, Gittenberger-de Groot AC, Poelmann RE, Péault BM, Huysmans HA. Development of the origin of the coronary arteries, a matter of ingrowth or outgrowth? Anatomy and Embryology 1989; 180: 437-441.
[14]	Nakamura Y, Taniguchi I, Saiki M, Morimoto K, Yamaga T. Quadricuspid aortic valve associated with aortic stenosis and regurgitation. Jpn J Thorac Cardiovasc Surg 2001; 49: 714-6.
[15]	Tutarel O. Quadricuspid aortic valves and anomalies of the coronary arteries. J Thorac Cardiovasc Surg 2004; 127: 897; author reply 897.
[16]	Mecozzi G, Pratali S, Milano A, Nardi C, Bortolotti U. Severe quadricuspid aortic valve stenosis after mediastinal irradiation. J Thorac Cardiovasc Surg 2003; 126: 1198-9.
[17]	Janssens U, Klues HG, Hanrath P. Congenital quadricuspid aortic valve anomaly associated with hypertrophic non-obstructive cardiomyopathy: a case report and review of the literature. Heart 1997; 78: 83-7.
[18]	Rosenkranz ER, Murphy DJ, Cosgrove DM. Surgical management of left coronary artery ostial atresia and supravalvar aortic stenosis. The Annals of Thoracic Surgery 1992; 54: 779-781.
[19]	Possati F, Calafiore AM, Di Giammarco G et al. [Quadricuspid aortic valve and pulmonary valve stenosis. A rare combination in the adult]. Minerva Cardioangiol 1984; 32: 815-8.
[20]	Robicsek F, Sanger PW, Daugherty HK, Montgomery CC. Congenital quadricuspid aortic valve with displacement of the left coronary orifice. Coll Works Cardiopulm Dis 1968; 14: 87-90.
[21]	Seol SH, Kim U, Cho HJ, Kim DK, Kim DI, Kim DS. Quadricuspid aortic valve with patent ductus arteriosus. Tex Heart Inst J 2010; 37: 726-7.
[22]	Matsukawa T, Yoshii S, Hashimoto R, Muto S, Suzuki S, Ueno A. Quadricuspid aortic valve perforation resulting from bacterial endocarditis--2-D echo- and angiographic diagnosis and its surgical treatment. Jpn Circ J 1988; 52: 437-40.
[23]	Takeda N, Ohtaki E, Kasegawa H, Tobaru T, Sumiyoshi T. Infective endocarditis associated with quadricuspid aortic valve. Jpn Heart J 2003; 44: 441-5.
[24]	Godefroid O, Colles P, Vercauteren S, Louagie Y, Marchandise B. Quadricuspid aortic valve: a rare etiology of aortic regurgitation. Eur J Echocardiogr 2006; 7: 168-70.
[25]	Tsang MY, Abudiab MM, Ammash NM et al. Quadricuspid Aortic Valve: Characteristics, Associated Structural Cardiovascular Abnormalities, and Clinical Outcomes. Circulation 2016; 133: 312-9.
[26]	Yuan SM. Quadricuspid Aortic Valve: A Comprehensive Review. Braz J Cardiovasc Surg 2016; 31: 454-460.
[27]	Luciani GB, Morjan M, Faggian G, Mazzucco A. Repair of quadricuspid aortic valve by bicuspidization: a novel technique. Interact Cardiovasc Thorac Surg 2010; 11: 348-50.
[28]	Kariyanna PT, Jayarangaiah A, Seshadri S, Adrah R, Sharma A, Rozin A. A rare case of quadricuspid aortic valve. Research 2016; 3: 1488.