An Extremely Rare Congenital Association: Uni- cuspid Aortic Valve with Left Ventricular Noncom- paction (RCD code: III-5A.1.o)

Souzan Tatari

Full Text:



Unicuspid aortic valve is a rare congenital abnormality with a prevalence of 0.02% in the adult population. Left ventricular noncompaction is another congenital anomaly with an incidence of 0.05%. Unicuspid aortic valve with severe aortic regurgitation and left ventricular
noncompaction was found in a 15-year-old deaf male patient who presented with shortness of breath, orthopnoea and intermittent palpitations. JRCD 2019; 4 (4): 106–108.


rare disease; congenital heart disease; echocardiography; magnetic resonance imaging


Ward C. Clinical significance of the bicuspid aortic valve. Heart 2000; 83(1):81-5.

Novaro GM, Mishra M, Griffin BP. Incidence and echocardiographic features of congenital unicuspid aortic valve in an adult population. J Heart Valve Dis 2003; 12 (6):674-8.

Tutarel O. The quadricuspid aortic valve: a comprehensive review. J Heart Valve Dis 2004; 13(4):534-7.

Jenni R., Oechslin E.N., van der Loo B. (2007) Isolated ventricular non-compaction of the myocardium in adults. Heart 93:11–15

Maron B.J.,Towbin J.A., Thiene G., et al. (2006) Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation 113:1807–1816.

Samsa L.A., Yang B., Liu J. (2013) Embryonic cardiac chamber maturation: trabeculation, conduction and cardiomyocyte proliferation. Am J Med Genet C Semin Med Genet 163C:157– 168

Petersen S.E., Selvanayagam J.B., Wiesmann F., et al. (2005) Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol 46:101–105.

Jenni R., Oechslin E., Schneider J., et al. (2001) Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart 86:666–671.

Jacquier A., Thuny F., Jop B., et al. (2010) Measurement of trabeculated left ventricular mass using cardiac magnetic resonance imaging in the diagnosis of left ventricular non-compaction. Eur Heart J 31:1098–1104.

André F., Burger A., Loßnitzer D., et al. (2015) Reference values for left and right ventricular trabeculation and non-compacted myocardium. Int J Cardiol 185:240–247.

Sidhu M.S., Uthamalingam S., Ahmed W., et al (2014) Defining left ventricular noncompaction using cardiac computed tomography. J Thorac Imaging 29:60–66.

Roberts WC, Ko JM. Frequency by decades of unicuspid, bicuspid, and tricuspid aortic valves in adults having isolated aortic valve replacement for aortic stenosis, with or without associated aortic regurgitation. Circulation 2005; 111(7):920-5.

Brantley HP, Nekkanti R, Anderson CA, Kypson AP. Three-dimensional echocardiographic features of unicuspid aortic valve stenosis correlate with surgical findings. Echocardiography 2012; 29(8):E204-7.

Brantley HP, Nekkanti R, Anderson CA, Kypson AP. Threedimensional echocardiographic features of unicuspid aortic valve stenosis correlate with surgical findings. Echocardiography 2012; 29(8):E204-7.

Gibbs WN, Hamman BL, Roberts WC, Schussler JM. Diagnosis of congenital unicuspid aortic valve by 64-slice cardiac computed tomography. Proc (Bayl Univ Med Cent) 2008; 21 (2):139.

Debl K, Djavidani B, Buchner S, Poschenrieder F, Heinicke N, Schmid C, et al. Unicuspid aortic valve disease: a magnetic resonance imaging study [in German]. Rofo 2008; 180(11): 983-7.

Kang SD, Seol SH, Park BM, Kim DK, Kim KH, Kim DI, et al. Incidental diagnosis of the unicuspid aortic valve with ascending aortic aneurysm in an asymptomatic adult. J Cardiovasc Ultrasound 2011; 19 (2):102-4.

Mookadam F, Thota VR, Garcia-Lopez AM, Emani UR, Alharthi MS, Zamorano J, Khandheria BK. Unicuspid aortic valve in adults: a systematic review. J Heart Valve Dis 2010; 19 (1):79-85.



  • There are currently no refbacks.
Journal of Rare Cardiovascular Diseases (JRCD)
John Paul II Hospital in Kraków, 80 Prądnicka Str., 31-202 Kraków, Poland
Phone: +48 (12) 614 33 99, +48 (12) 614 34 88 Fax: +48 (12) 614 34 88
Published by SoftQ sp. z o.o.
ul. Oleandry 2, 30-063 Kraków, Poland
Phone: +48 (12) 444 1650 Fax: +48 (12) 444 1659