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Background: Echocardiography is regarded as the gold standard for diagnosis of bicuspid aortic valve (BAV), yet diagnostic accuracy has been evaluated previously only in single-center studies. We systematically evaluated the accuracy of BAV diagnosis in a large healthcare system of multiple echocardiography laboratories., Methods and Results: Aurora Health Care is a multihospital, multi-clinic system across the state of Wisconsin encompassing 33 inpatient and outpatient echocardiography laboratories with 39 cardiologist readers and 72 sonographers. As all laboratories store echocardiograms in one database, we queried all patients with "bicuspid aortic valve" diagnosis on echocardiography. Echocardiograms were classified as "BAV" or "possible BAV" based on initial reader confidence. An expert review panel categorized each as BAV, no BAV, or Indeterminate. Of the 745 cases identified, 589 (79.1%, initial reader interpretation: n = 494 "BAV," n = 95 "possible") were BAV. A high level of agreement (84%) was present in BAV diagnosis. There were 156 (20.9%) echocardiograms that were no BAV (8.4%) or Indeterminate (12.4%). We identified three common reasons for misdiagnosis: poor image quality (n = 70, 44.9%), leaflet calcium (n = 44, 28.2%), and oblique axis imaging (n = 33, 21.1%). A clear reason for misclassification was not elucidated in nine cases (n = 9, 5.7%)., Conclusions: This is the first study to evaluate BAV accuracy across a community health system with multiple echocardiography laboratories and a heterogeneous group of readers and sonographers. We establish a high degree of accuracy of echocardiography in BAV diagnosis and draw attention to common echocardiographic pitfalls that lead to BAV misclassification, highlighting opportunities for education and quality improvement., (© 2018 Wiley Periodicals, Inc.)

Aims Left ventricular outflow (LVOT) obstruction in patients with hypertrophic cardiomyopathy (HCM) is dynamic and sensitive to many variables that affect left ventricular preload, afterload, and contractility. The haemodynamic effect of normal respiration on LVOT obstruction has not been described. Methods and results We examined 20 patients with HCM who were noted to have phasic respiratory variation of LVOT obstruction on initial transthoracic 2D echocardiogram and Doppler examination. LVOT gradients were re-examined with simultaneous recording of a respirometer. LVOT gradients varied widely during the respiratory cycle; peak gradients were uniformly lowest during inspiration (50.8 mmHg + 25.6) and highest during expiration (90.1 mmHg + 41.8). On average, there was 82.4% ± 39.1 (P  ≤ 0.0001) incremental change from inspiration to expiration, in the severity of LVOT obstruction. In 11 patients with mitral annulus inflow, LV inflow (preload) was decreased during inspiration. In 16 patients with isovolumic relaxation time and ejection time measurements, decreased left atrial filling pressure was noted during inspiration, consistent with decreased LVOT obstruction. When compared with a control group of 20 HCM patients who did not have respiratory variation, the study group patients were more overweight (mean body mass index cases 35.1 ± 7.3 vs. control group 29.1 ± 5.1, P  = 0.0045) and more likely to have sleep-disordered breathing (n  = 15 study group, n  = 5 control group). Conclusions Counterintuitive respiratory-related fluctuations in LVOT gradients were observed in this case series of 20 HCM patients. These findings challenge traditional haemodynamic teaching and demonstrate the contribution of LV transmural pressure to LVOT obstruction in certain HCM patients. [ABSTRACT FROM AUTHOR]