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Aortic valve calcium (AVC) is a strong predictor of aortic stenosis (AS) severity and is typically calculated by multidetector computed tomography (MDCT). We propose a novel method using pixel density quantification software to objectively quantify AVC by two-dimensional (2D) transthoracic echocardiography (TTE) and distinguish severe from non-severe AS. A total of 90 patients (mean age 76 ± 10 years, 75% male, mean AV gradient 32 ± 11 mmHg, peak AV velocity 3.6 ± 0.6 m/s, AV area (AVA) 1.0 ± 0.3 cm 2 , dimensionless index (DI) 0.27 ± 0.08) with suspected severe aortic stenosis undergoing 2D echocardiography were retrospectively evaluated. Parasternal short axis aortic valve views were used to calculate a gain-independent ratio between the average pixel density of the entire aortic valve in short axis at end diastole and the average pixel density of the aortic annulus in short axis (2D-AVC ratio). The 2D-AVC ratio was compared to echocardiographic hemodynamic parameters associated with AS, MDCT AVC quantification, and expert reader interpretation of AS severity based on echocardiographic AVC interpretation. The 2D-AVC ratio exhibited strong correlations with mean AV gradient (r = 0.72, p < 0.001), peak AV velocity (r = 0.74, p < 0.001), AVC quantified by MDCT (r = 0.71, p <0.001) and excellent accuracy in distinguishing severe from non-severe AS (area under the curve = 0.93). Conversely, expert reader interpretation of AS severity based on echocardiographic AVC was not significantly related to AV mean gradient (t = 0.23, p = 0.64), AVA (t = 2.94, p = 0.11), peak velocity (t = 0.59, p = 0.46), or DI (t = 0.02, p = 0.89). In conclusion, these data suggest that the 2D-AVC ratio may be a complementary method for AS severity adjudication that is readily quantifiable at time of TTE., (Published by Elsevier Inc.)

Unicuspid aortic valve (UAV) is a rare malformation that is often difficult to distinguish from a bicuspid aortic valve (BAV) with commissural fusion by echocardiography or intraoperative surgical inspection. This study assessed the accuracy of intraoperative surgical inspection and two-dimensional echocardiography in diagnosing UAV compared to a gold standard of pathological diagnosis. The Mayo Clinic echocardiographic database, tissue registry database and electronic medical record were searched for all patients assigned a diagnosis of UAV by any technique. Transthoracic (TTE), transesophageal (TEE) echocardiographic, and surgical diagnoses were compared to pathological diagnosis as the standard. A clinical diagnosis of UAV was applied to 380 patients by 1 or more method and in 196 (52%) a pathologic evaluation was available to compare to the clinical description given by TTE, TEE, or surgical inspection. Of these 196 patients, only 58 (30%) had a pathological diagnosis of UAV; the majority were found to be BAVs by pathologic evaluation (n = 132, 67%). For diagnosing UAV, the sensitivity and specificity were 15% and 87% for TTE, 28%, and 82% for TEE, and 52% and 51% for surgical inspection, respectively. Valves with bicuspid morphology and extensive commissural fusion were frequently misclassified as UAV by all methods. In conclusion, intraoperative surgical inspection and echocardiography have limitations for diagnosing UAV due to difficulties in accurately assigning a correct morphological diagnosis, which suggests that the current understanding of the natural history of UAV may be inaccurate., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Keywords: aortic valve; aortic valve stenosis; cardiac surgical procedures; echocardiography; heart valve diseases Unicuspid aortic valve pathology characteristics and long-term outcomes.A, Surgically resected unicuspid (unicommissural) aortic valve (UAV) that exhibits 2 discrete raphes (asterisks) and a single commissure (arrow). Aortic valve, aortic valve stenosis, cardiac surgical procedures, echocardiography, heart valve diseases. [Extracted from the article]

Introduction: Unicuspid aortic valve (UAV) is a rare disease typically diagnosed by surgical inspection. This study assessed the natural history and outcomes of pathologically confirmed UAV. Methods: The institutional databases (echocardiographic, pathology) and medical record were searched for demographic, echocardiographic and outcome data of all UAV diagnoses. Only UAVs pathologically confirmed post-surgery or at autopsy were included. Results: 81 patients (mean±SD age, 29 ±18 years (range 0-81); male sex, 62%) were identified with pathologically confirmed UAV (83% post-surgery, 17% at autopsy). 67% had ascending aorta dilatation. Aortic stenosis (AS) or aortic regurgitation (AR) of any degree, mixed AR/AS, pure AS, and pure AR was present in 77%, 74%, 58%, 21 %, and 18% of patients, respectively (Table 1). Additional abnormalities included patent foramen ovale (5%), patent ductus arteriosus (5%) and coarctation (4%). At follow up (median 9.9 years) 67 (83%) and 34 (42%) patients had aortic valve surgery (mean ±SD age at surgery, 37 ± 14 years (range 0.1-81); 85% aortic valve replacement (AVR), 4% Ross procedure, 4% heart transplant, 6% unknown type) and ascending aorta surgery (mean ±SD age at aorta surgery, 43 ± 11 years (range 19-67); 82% aortic graft, 9% aortic repair, 9% Ross procedure), respectively (Table 1). 13% of patients had valvotomy prior to surgery. Mean ±SD time to AVR and aorta surgery from diagnosis was 3 ± 9 years and 8 ± 13 years, respectively. Surgical indications were AS (44%), mixed AS/AR (31%), AR (12%) and ascending aortic dilatation (7%). AVR alone and AVR with aorta surgery had similar outcomes (post-surgical survival 100% and 92% for both at 5 and 10 years, respectively (p = 0.32)). Conclusions: To our knowledge this is the largest and most comprehensive natural history and outcomes study of pathologically confirmed UAV. AS and AR were common in UAV and AS was the most common surgery indication. AVR alone and AVR with aorta surgery had similar outcomes. [ABSTRACT FROM AUTHOR]