Korean J Radiol.  2016 Aug;17(4):463-471. 10.3348/kjr.2016.17.4.463.

Morphological and Functional Evaluation of Quadricuspid Aortic Valves Using Cardiac Computed Tomography

• Song I
• Park JA
• Choi BH
• Ko SM
• Shin JK
• Chee HK
• Kim JS

• KMID: 2455417
• DOI: http://doi.org/10.3348/kjr.2016.17.4.463

Abstract

OBJECTIVE
The aim of this study was to identify the morphological and functional characteristics of quadricuspid aortic valves (QAV) on cardiac computed tomography (CCT).
MATERIALS AND METHODS
We retrospectively enrolled 11 patients with QAV. All patients underwent CCT and transthoracic echocardiography (TTE), and 7 patients underwent cardiovascular magnetic resonance (CMR). The presence and classification of QAV assessed by CCT was compared with that of TTE and intraoperative findings. The regurgitant orifice area (ROA) measured by CCT was compared with severity of aortic regurgitation (AR) by TTE and the regurgitant fraction (RF) by CMR.
RESULTS
All of the patients had AR; 9 had pure AR, 1 had combined aortic stenosis and regurgitation, and 1 had combined subaortic stenosis and regurgitation. Two patients had a subaortic fibrotic membrane and 1 of them showed a subaortic stenosis. One QAV was misdiagnosed as tricuspid aortic valve on TTE. In accordance with the Hurwitz and Robert's classification, consensus was reached on the QAV classification between the CCT and TTE findings in 7 of 10 patients. The patients were classified as type A (n = 1), type B (n = 3), type C (n = 1), type D (n = 4), and type F (n = 2) on CCT. A very high correlation existed between ROA by CCT and RF by CMR (r = 0.99) but a good correlation existed between ROA by CCT and regurgitant severity by TTE (r = 0.62).
CONCLUSION
Cardiac computed tomography provides comprehensive anatomical and functional information about the QAV.

Keyword

Aortic valve; Valvular heart disease, congenital; Multidetector computed tomography; Echocardiography; Aortic regurgitation; Magnetic resonance imaging

MeSH Terms

Adult
Aged
Aortic Valve/*diagnostic imaging
Aortic Valve Insufficiency/*diagnosis/diagnostic imaging/pathology
Echocardiography
Female
Humans
Magnetic Resonance Imaging, Cine
Male
Middle Aged
Retrospective Studies
Severity of Illness Index
Tomography, X-Ray Computed
Young Adult

Figure

• 

  Fig. 1 Measurement of regurgitant orifice area (A) at mid-diastole, opening area (B) at early-systole, and individual valve cusp area (C) at mid-diastole in patient with quadricuspid aortic valve assessed by planimetry on cardiac computed tomography.

• 

  Fig. 2 Classification of quadricuspid aortic valve according to Hurwitz and Robert's classification.A. Four equal cusps. B. Three equal cusps and one smaller cusp. C. Two equal larger cusps and two equal smaller cusps. D. One large, two intermediate, and one small cusp. E. Three equal cusps and one larger cusp. F. Two equal larger cusps and two unequal smaller cusps. G. Four unequal cusps. Adapted from Hurwitz and Roberts. Am J Cardiol 1973;31:623-626 (2).

• 

  Fig. 3 52-year-old man with Hurwitz and Robert's type F quadricuspid aortic valve combined with dilatation of ascending aorta.A, B. Parasternal short-axis transthoracic echocardiographic images during diastole (A) and systole (B) show aortic valve. C. Parasternal long-axis color Doppler image during diastole shows moderate aortic regurgitation and moderate degree of aortic stenosis (mean pressure gradient = 38 mm Hg, not shown). D, E. Short-axis cardiac computed tomography (CCT) images of aortic valve during diastole (D) and systole (E) show thickened and calcified quadricuspid aortic valve with two equal larger cusps and two unequal smaller cusps. Areas of right cusp, left cusp, noncoronary cusp, and accessory cusp (arrows) are 2.44, 3.03, 2.92, and 0.31 cm2, respectively. Lack of coaptation of aortic valve (regurgitant orifice area = 0.1 cm2) was detected during diastole (D). F. Oblique coronal CCT image shows measurement of ascending aorta diameter at four different levels during early systole (annulus, 27 mm; sinuses of Valsalva, 36.7 mm; sinotubular junction, 26.7 mm; tubular portion, 51 mm). Patient underwent combined aortic valvuloplasty and ascending aorta wrapping. LCC = left coronary cusp, NCC = noncoronary cusp, RCC = right coronary cusp, 4th = accessory cusp

• 

  Fig. 4 19-year-old man with Hurwitz and Robert's type A quadricuspid aortic valve (QAV) with four equal-sized cusps shown on transthoracic echocardiography (TTE), cardiac computed tomography (CCT), and cardiac magnetic resonance (CMR) imaging.A. Parasternal short-axis TTE image of aortic valve during diastole shows QAV with four-leaf clover appearance. B. Short-axis CCT image of aortic valve during diastole demonstrates four equal aortic valve cusps. Areas of right cusp, left cusp, noncoronary cusp, and accessory cusp are 2.01, 2.13, 2.20, and 1.94 cm2, respectively. Lack of coaptation of aortic valve (arrow, regurgitant orifice area = 0.2 cm2) was detected during diastole, indicating aortic regurgitation. C. Oblique sagittal cine CMR image during diastole demonstrates central regurgitant jet (arrow) into left ventricular cavity. Quantitative analysis by phase-contrast CMR (not shown) yields regurgitant fraction of 35%, corresponding to moderate degree of aortic regurgitation (AR). LCC = left coronary cusp, NCC = noncoronary cusp, RCC = right coronary cusp, 4th = accessory cusp

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  Fig. 5 37-year-old woman with Hurwitz and Robert's type B quadricuspid aortic valve (QAV) combined with aortic regurgitation (AR) and subaortic stenosis due to subaortic membrane.A. Parasternal long-axis color Doppler image during diastole shows moderate AR. B. Short-axis cardiac computed tomography (CCT) image of aortic valve during diastole demonstrates QAV with three equal-sized cusps and one smaller cusp. Areas of right cusp, left cusp, noncoronary cusp, and accessory cusp are 1.62, 2.13, 1.77, and 0.80 cm2, respectively. Lack of coaptation of aortic valve was detected (arrow, regurgitant orifice area = 0.17 cm2) during diastole. C. Oblique sagittal CCT image shows subaortic membrane (black and white arrows) with left ventricular outflow tract obstruction. Patient underwent aortic valvuloplasty and resection of subaortic membrane. LCC = left coronary cusp, NCC = noncoronary cusp, RCC = right coronary cusp, 4th = accessory cusp

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