Quadricuspid Aortic Valve Complicated with Severe Aortic Regurgitation and Left-Sided Inferior Vena Cava

A 72-year-old man with hypertension was referred to our hospital for severe aortic regurgitation probably associated with quadricuspid aortic valve on transthoracic echocardiogra-phy. He felt general fatigue on effort. On physical examination , blood pressure was 166/54 mm Hg and cardiac ausculta-tion indicated a systolic murmur at the aortic area together with a diastolic murmur at the left parasternal border. Value of brain natriuretic peptide was 258.5 pg/mL, and values of cardiac enzymes were within normal limits. An electrocardio-gram showed left ventricular high voltage in addition to ST-segment depression in II, III, aVF, and V6 leads. Subsequent transthoracic echocardiography revealed decreased motion in the inferoposterior wall of the dilated left ventricle (end-dia-stolic and end-systolic diameters of 6.3 and 4.6 cm, respectively ; ejection fraction 49 %, Simpson's method) and severe aortic regurgitation (vena contracta 6.7 mm, pressure half time 279 msec, regurgitant volume 77 mL on the proximal isove-locity surface area method, and effective regurgitant orifice area 0.38 cm 2) with probable quadricuspid aortic valve. Two-and three-dimensional transesophageal echocardiography clearly depicted a quadricuspid asymmetric aortic valve with a large right coronary cusp, two intermediate cusps including left coronary cusp and non-coronary cusp, and a smaller accessory cusp (Hurwitz and Roberts' classification, type D), and a severe aortic regurgitation due to the central coaptation defect (Fig. 1A, B, and C, Supplementary movie 1, 2, and 3). Pre-operative cardiac catheterization was performed (Fig. 2). Coronary angiography showed a severe stenosis in the distal segment of the which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. right coronary artery (Fig. 2A). Left coronary artery was normal (Fig. 2B). On aortography, aortic regurgitation of Sellers grade 3, left ventricular end-diastolic volume of 263 mL, end-systolic volume of 142 mL, ejection fraction of 46%, and hy-pokinesis in inferior were observed (Fig. 2C and D). During right heart catheterization, course of Swan-Ganz catheter suggested a presence of left-sided inferior vena cava (IVC). Hemo-dynamics study showed left ventricular end-diastolic pressure of 14 mm Hg, mean pulmonary capillary wedge pressure of 15 mm Hg, mean pulmonary artery pressure of 19 mm Hg, and cardiac index of 2.38 L/min/m 2. Computed tomography (CT) prior to the surgery confirmed a left-sided IVC (Fig. 3). Based on the diagnosis of severe aortic regurgitation with quad-ricuspid aortic valve and coronary artery disease, the patient underwent an aortic valve replacement using a bioprosthetic valve …