Successful Transcatheter Aortic Valve Implantation for Severe Stenosis of a Quadricuspid Aortic Valve

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  Visual Summary. Successful Transcatheter Aortic Valve Implantation in Quadricuspid Aortic Valve With Severe Stenosis AV = aortic valve; CRT-D = cardiac resynchronization therapy with defibrillator; HFrEF = heart failure with reduced ejection fraction; LBBB = left bundle branch block; LVEF = left ventricular ejection fraction; STEMI = ST-segment elevation myocardial infarction; TAVI = transcatheter aortic valve implantation.

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  Figure 1. Transthoracic Echocardiography Short-Axis Parasternal View During Diastole Showing a Characteristic “X” Shape of the Quadricuspid Aortic Valve

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  Figure 2. Computed Tomography Short-Axis View During Diastole Confirming the Quadricuspid Aortic Valve A computed tomography scan with a dedicated TAVI protocol was performed and excluded significant aortic root or ascending aorta anomalies (mean ascending aorta diameter: 33.7 mm). Detailed measurements showed an annular perimeter of 71.1 mm (perimeter-derived Ø: 22.6 mm); annular area of 379.0 mm2 (area-derived Ø: 22.0 mm); annular eccentricity of 0.20; left ventricular outflow tract diameter of 21.7 mm; sinuses of Valsalva 29.5 mm (left), 28.2 mm (right), and 28.9 mm (noncoronary); sinus height of 16.1 mm; and sinotubular junction diameter of 27.0 mm. Coronary ostia heights were 12.8 mm (left) and 13.9 mm (right), considered sufficient to minimize coronary obstruction risk during TAVI. LC = left coronary cusp; NC = noncoronary cusp; RC = right coronary cusp; TAVI = transcatheter aortic valve implantation.

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  Figure 3. Transthoracic Echocardiography Short-Axis View During Systole Showing a Rectangular Opening, Which Is Typical for Quadricuspid Aortic Valve

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  Figure 4. Computed Tomography Short-Axis View During Systole

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  Figure 5. Nakamura5 Simplified Classification of Quadricuspid Aortic Valve L = left coronary cusp; N = noncoronary cusp; S = supernumerary cusp.

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  Figure 6. Hurwitz and Roberts3 Classification of Quadricuspid Aortic Valve

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  Figure 7. Classification of Quadricuspid Aortic Valve in the Present Case The quadricuspid valve in our case corresponds to type D according to the Hurwitz and Roberts classification,3 featuring one large cusp, two midsized-cusps, and one smaller cusp. According to the Nakamura classification,5 it is type II, with the accessory cusp located between the noncoronary and right coronary cusps. A = accessory cusp; L = left coronary cusp; N = noncoronary cusp; R = right coronary cusp.

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  Figure 8. Severe, High-Gradient Aortic Stenosis Apical 5-chamber view with continuous Doppler revealing an aortic valve area of 0.81 cm2 (0.63 cm2/m2), a mean transaortic gradient of 40 mm Hg, and a peak velocity of 3.96 m/s.