Differentiating Cardiac (Mal)adaptation in 2 Athletes With Bicuspid Aortic Valves.

Figures

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  Visual Summary. Disentangling Physiologic and Pathological Cardiac Remodeling in Endurance Athletes With a Bicuspid Aortic Valve Theoretical schema showing the linear relationship between left ventricular end-diastolic volume (LVEDV) and maximal oxygen uptake (Vo2max) in endurance athletes, with values for patients 1 and 2 overlaid. In an asymptomatic athlete, changes in LVEDV result in proportional changes in Vo2max. Dilation of the left ventricle independent of Vo2max (downward and right shift of this relationship) may be an early indication of maladaptive left ventricular remodeling to compensate for the excessive regurgitant load.

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  Figure 1. Cardiac Remodeling and Aortic Regurgitant Flow Patterns in 2 Endurance Athletes With Bicuspid Aortic Valves The top panels show the horizontal long-axis view obtained from cardiac magnetic resonance imaging for both patients, demonstrating profound left ventricular dilatation that vastly exceeds that of the right ventricle. The middle and lower panels show Doppler color flow mapping of the aortic valve for each patient in the apical long-axis (middle panels) and parasternal long-axis views (lower panels), demonstrating moderate aortic regurgitation in both patients.

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  Figure 2. Resting Echocardiographic Assessment of Diastolic Function, Global Longitudinal Strain, and Cardiac Dilatation in Patient 1 The top panels show flow and tissue Doppler assessment of diastolic function, revealing normal E/A ratio (2.8) and mildly elevated E/e′ (10.2). The lower left panel shows left ventricular strain assessment, with preserved global longitudinal strain values (−18.5%). The lower right panel shows apical 4-chamber view, illustrating moderate atrial and right ventricular dilatation, albeit to a lesser extent than the left ventricle.

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  Figure 3. Cardiac Magnetic Resonance Imaging Assessment of Delayed Enhancement in Patient 1 There were no signs of significant late gadolinium enhancement in any regions of the left ventricular myocardium in patient 1.

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  Figure 4. Left Ventricular Remodeling in Patient 1 After Aortic Valve Replacement Using the Ross Procedure (A-C) Left ventricular end-diastolic volume (LVEDV) assessment from 3-dimensional echocardiography shows significant reverse remodeling in patient 1 shortly after aortic valve replacement that persists 12 months after the procedure.

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  Figure 5. Longitudinal Changes in Exercise Capacity and Left Ventricular Remodeling in 2 Endurance Athletes With Bicuspid Aortic Valves Patient 1 showed a significant reduction in maximal oxygen uptake (Vo2max) and left ventricular end-diastolic volume (LVEDV) after aortic valve replacement, with a prolonged and gradual improvement in Vo2max over the following 4 years as he gradually returned to endurance training. Patient 2 underwent continued active surveillance (watchful waiting) with regular imaging and exercise testing, revealing stable LVEDV and Vo2max over ∼3.5 years of follow-up.

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  Figure 6. Resting Echocardiographic Assessment of Diastolic Function, Global Longitudinal Strain, and Cardiac Dilatation in Patient 2 The top panels show flow and tissue Doppler assessment of diastolic function, revealing normal E/A ratio (2.0) and mildly elevated E/e′ (6.30). The lower left panel shows left ventricular strain assessment, with preserved global longitudinal strain values (−22.3%) and no significant wall motion abnormalities. The lower right panel shows apical 4-chamber view illustrating moderate left atrial dilatation.

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  Figure 7. Cardiac Magnetic Resonance Imaging Assessment of Delayed Enhancement in Patient 2 There were no signs of significant late gadolinium enhancement in any regions of the left ventricular myocardium in patient 2.

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  Figure 8. Reduced Aortic Regurgitation at Peak Exercise in an Endurance Athlete With Bicuspid Aortic Valve (Patient 2) Doppler color flow mapping of the aortic valve in the apical long-axis view measured at rest (A) and immediately after maximal exercise (B) in Patient 2 reveals a significant reduction in aortic regurgitation with exertion.