Unicuspid Unicommissural Aortic Valve: An Extremely Rare Congenital Anomaly
Sukhjeet SinghPuneet GhayalAtish MathurMargaret MysliwiecConstantinos LovoulosPallavi SolankiMarc KlapholzJames Maher
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Abstract Unicuspid aortic valve is a rare congenital malformation that usually presents in the 3rd to 5th decade of life—and usually with severe aortic stenosis or regurgitation. It often requires surgical correction. Diagnosis can be made with 2- or 3-dimensional transthoracic or transesophageal echocardiography, cardiac computed tomography, or cardiac magnetic resonance imaging. We report the case of a 31-year-old man who presented with dyspnea on exertion due to severe aortic stenosis secondary to a unicuspid unicommissural aortic valve. After aortic valve replacement, this patient experienced complete heart block that required the placement of a permanent pacemaker.Bicuspid valve
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This study aimed to evaluate the clinical outcomes of treating high-risk patients presenting with severe aortic regurgitation (AR) or aortic stenosis (AS) using transcatheter aortic valve replacement (TAVR).This retrospective study included 290 consecutive patients with symptomatic severe aortic regurgitation or aortic stenosis. All patients who underwent TAVR with J-Valve (Med Tech College LLC, Fort Wayne, IN, USA) at our institution between March 2014 and July 2019. Preoperative demographic data, clinical and echocardiographic parameters, procedural parameters, postoperative clinical outcomes, and echocardiographic parameters were recorded retrospectively.The study included a total of 290 participants. 161 patients had severe aortic regurgitation and 129 patients had severe aortic stenosis. The baseline characteristics of the two groups were similar. The device success rate was high for both aortic regurgitation and aortic stenosis groups (95.0% vs. 93.0%, P=0.47). All-cause mortality of both groups was similar at 30 days (1.9% vs. 3.9%, P=0.48). Patients treated for aortic regurgitation had a higher incidence of pacemaker implantation compared to the aortic stenosis group (8.3% vs. 0.8%, P<0.01) at 30 days postoperation. There was no significant difference between the groups in moderate or severe paravalvular leakage (1.9% vs. 0, P=0.13). The use of larger prostheses was more common in the aortic regurgitation group compared to the aortic stenosis group (66.5% vs. 13.2%, P<0.01). Mean pressure gradient was lower in the aortic stenosis group (8.5±2.9 vs. 12.9±6.6, P<0.01), but structural valve deterioration was more common in the aortic stenosis group (9.7% vs. 0, P<0.01) at 30 days postop.In this study, we found that the prognosis of patients with aortic regurgitation is comparable to that of patients with aortic stenosis after TAVR with J-valve. For patients with severe aortic regurgitation or aortic stenosis, TAVR is an effective therapeutic option. Pacemaker rate in the aortic regurgitation group was higher, but structural valve deterioration was more common in aortic stenosis patients.
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Aortic valve stenosis is a potentially serious condition. Progression from mild to severe aortic stenosis is well-recognized but there are few data as to the likely rate of progression. Clinical outcome and cardiac catheterization data were reviewed for 65 patients with valvar aortic stenosis. Each patient had been investigated by cardiac catheterization on at least two occasions, the interval between studies ranging between 1 and 17 years (mean 7 years). In 60 cases the aortic valve gradient had increased, from a median of 10 mmHg (range 0–60) to a median of 52 mmHg (range 15–120). The mean rate of increase of gradient was 6.5 mmHg per year, and was significantly faster in patients in whom there was aortic valve calcification or aortic regurgitation present at the first catheter study (P < 0.02).
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Rate-corrected left ventricular ejection time was measured from the aortic pressure tracings of 171 catheterised patients with aortic valve area less than or equal to 1.2 cm2. In 50 patients with pure aortic stenosis, left ventricular ejection time in increased with decreasing valve area and was significantly higher (468 +/- 5 ms, mean +/- SEM) than in 13 normal subjects (435 +/- 5 ms). Additional aortic regurgitation in 72 patients further increased the left ventricular ejection time to 484 +/- 4 ms. Significant mitral stenosis (mitral valve are less than or equal to 1.2 cm2) in 6 patients with aortic stenosis and 33 patients with aortic stenosis and regurgitation reduced the left ventricular ejection time to normal. Similarly, severe mitral regurgitation in 3 patients with aortic stenosis and regurgitation reduced left ventricular ejection time to normal, though slight or moderate mitral regurgitation in 4 of these patients did not. These data show that the prolonged left ventricular ejection time in aortic valve disease may be restored to normal in the presence of coexisting significant mitral disease.
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Analysis of aortic valve gradients by transseptal technique: Implications for noninvasive evaluation
Abstract The peak instantaneous aortic valve gradient derived from Doppler echocardiography is commonly used to predict the severity of aortic stenosis. Peak instantaneous gradient should not be equated with the mean gradient or “peak to peak” gradient measured at cardiac catheterization. The primary purpose of this study is to assess the relationship between the aortic valve gradients, using a two‐catheter transseptal technique in 102 patients with aortic stenosis, mixed aortic stenosis and regurgitation, and following aortic valve replacement. These cases were drawn from a series of 111 consecutive transseptal procedures for patients with isolated aortic valve disease. No major complications occurred, and the most common reason for technical failure was inability to engage the atrial septum in postoperative patients. Although the peak instantaneous gradient correlates well with the mean gradient in aortic stenosis (r = .94, P < .001), mixed stenosis and regurgitation (r = .95, P < .001), and after aortic valve replacement (r = .86, P < .001), it systematically overestimates both the mean gradient and the peak to peak gradient. Neither the peak instantaneous nor the mean gradient correlates highly with aortic valve area in aortic stenosis (r = −.48, P < .01 peak; r = −.58, P < .001 mean gradient), mixed aortic stenosis and regurgitation (r = −.39, P NS peak; r = −.42, P NS mean gradient) or following aortic valve replacement (r = −.26, P NS peak; r = −.53, P < .01 mean gradient). Systolic time intervals also were analyzed from the simultaneous left ventricular and ascending aortic pressure tracings. The systolic ejection period, time to peak gradient, time to peak left ventricular pressure, and time to peak aortic pressure correlated poorly with aortic valve area. This study indicates that the mean gradient, which is consistently smaller than the peak instantaneous gradient, can be estimated by a simple regression formula: mean gradient = 0.70 peak instantaneous gradient. The mean gradient, which is usually less than the peak to peak gradient over 58 mm Hg and greater than the peak to peak gradient under 58 mm Hg, can be estimated by: mean gradient = 0.71 peak to peak gradient + 17 mm Hg. The limitations of isolated gradients in predicting aortic valve area indicate the importance of accurate flow measurements for optimal determination of the severity of aortic valve obstruction.
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In this study, we analyzed the extent and pattern of regression of left ventricular (LV) hypertrophy after aortic valve replacement in patients with aortic stenosis (AS) and compared the results with those of another group of patients with aortic regurgitation (AR). Seventy patients who underwent isolated aortic valve replacement were divided into 2 groups. Group 1 was comprised of 29 patients who underwent aortic valve replacement for aortic stenosis, and Group 2 of 41 patients who underwent aortic valve replacement for aortic regurgitation. A third group of 10 healthy subjects served as a healthy control group. Echocardiographic studies were done before the operation and 5 years postoperatively. At follow-up, a significant reduction in the left ventricular mass was found in both groups, but it remained significantly greater than in the healthy control group. The ratio of LV wall thickness to radius (th/r) in Group 1 decreased significantly, and at follow-up it was within the normal value. In Group 2, the th/r ratio increased, and at follow-up it was within the normal value. After aortic valve replacement, the wall thickness remained significantly greater than normal for patients with AS, and the chamber radius remained significantly greater than normal for patients with AR. For these reasons, LV hypertrophy still existed in both groups at postoperative follow-up. The actuarial survival rate was 85.3% at 16 years for Group 1 and 83.4% at 18 years for Group 2. There was no significant difference in the long-term survival rates between the 2 groups. Actuarial freedom from valve-related events was 91.9% at 16 years for Group 1 and 82% at 18 years for Group 2. There was no significant difference in the valve-related event free curves between groups. After 5 years of follow-up, th/r reached normal for both groups, indicating remodeling of the LV geometry after aortic valve replacement.
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