Quadricuspid Aortic Valve and Anomalous Aortic Origin of the Right Coronary Artery

Quadricuspid aortic valve (QAV) is rare, with an incidence of 0.033%, as reported in a study on 6,000 autopsies.¹ It is rarer for QAV to occur in combination with anomalous aortic origin of a coronary artery (AAOCA).² Herein we report a case in which preoperative electrocardiogram-gated 3-dimensional computed tomography (3D-CT) diagnosed QAV and anomalous origin of the right coronary artery (AAORCA).

A 60-year-old man with no overt cardiac symptoms was referred to Juntendo Hospital due to a diastolic heart murmur. A transthoracic echocardiogram revealed severe aortic regurgitation. Electrocardiogram-gated 3D-CT revealed precise anatomical configurations of the morphology; QAV and right AAOCA (Figure A,B). The right coronary artery (RCA) originated from the left coronary cusp and ran between the aorta and the pulmonary artery (Figure C). The proximal part of the RCA ran in the aortic wall and its intramural segment showed 25% stenosis (Figure D). Because the intramural course was lower and passed under the commissure, unroofing was not indicated for this case.

Figure.

(A) Electrocardiogram-gated 3-dimensional computed tomography and (B) multiplanar reconstruction revealed a quadricuspid aortic valve. (C) The right coronary artery originated from the left coronary cusp and ran between the aorta and the pulmonary artery. (D) The intramural segment (arrow) showed 25% stenosis.

Accurate evaluation by electrocardiogram-gated 3D-CT is helpful in selecting a surgical procedure, such as unroofing, fenestration, reimplantation technique, and coronary artery bypass grafting, for AAOCA with QAV.³

Sources of Funding

This study did not receive any specific grant from any funding agency, commercial, or non-profit sectors.

Disclosures

All authors declare that there are no conflicts of interest.

IRB Information

This study was approved by Juntendo Hospital Ethics Review Board (No. JHS20-014).

References

• 1.   Hurwitz LE, Roberts WC. Quadricuspid semilunar valve. Am J Cardiol 1973; 31: 623–626.
• 2.   Tsang MY, Abudiab MM, Ammash NM, Naqvi TZ, Edwards WD, Nkomo VT, et al. Quadricuspid aortic valve: Characteristics, associated structural cardiovascular abnormalities, and clinical outcomes. Circulation 2016; 133: 312–319.
• 3.   Sato Y, Inoue F, Matsumoto N, Tani S, Takayama T, Yoda S, et al. Detection of anomalous origin of the coronary artery by means of multislice computed tomography. Circ J 2005; 69: 320–324.