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Address reprint requests to Paul S. Pagel, MD, PhD, Clement J. Zablocki Veterans Affairs Medical Center, Anesthesia Service, 5000 West National Avenue, Milwaukee, WI 53295
A 32-YEAR-OLD, 87-kg, 173-cm man with a past medical history of a congenital bicuspid
aortic valve was admitted to the authors' hospital for evaluation of dyspnea on exertion.
The patient had been a frequent participant in strenuous athletic activities including
full-court basketball. He reported that his stamina during these activities had declined
substantially in recent months. The patient also described unusual episodes of fatigue
while performing his job as a biomedical engineer. He denied a history of angina pectoris,
syncope, palpitations, orthopnea, paroxysmal nocturnal dyspnea, and peripheral edema.
The physical examination was notable for a grade III of VI holodiastolic murmur heard
best along the left sternal border. An Austin Flint murmur
was not appreciated. The remainder of the physical examination was noncontributory.
Noninvasive measurements of arterial blood pressure indicated the presence of a widened
pulse pressure (75-80 mmHg). A plasma brain natriuretic peptide concentration was
normal. Transesophageal echocardiography (TEE) was performed as part of the evaluation
and confirmed the presence of a bicuspid aortic valve with thickened anterior-lateral
(left and right coronary cusp fusion; type A)
and posterior-medial leaflets of approximately equal size. The TEE examination also
revealed that the middle scallop of the anterior mitral leaflet (A2) was essentially immobile throughout the cardiac cycle (Fig 1, Fig 2 and Video 1 [supplementary videos are available online]). What is the cause of this anterior
mitral leaflet immobility?
Fig 1The midesophageal long-axis view obtained during systole.
Fig 2The midesophageal long-axis view obtained during early diastole; the location and
shape of the middle scallop of the anterior mitral leaflet (A2) are essentially unchanged compared with Figure 1.
in: Savage R.M. Aronson S. Comprehensive Textbook of Intraoperative Transesophageal Echocardiography. Williams, and Wilkins,
Philadelphia, Lippincott2005: 205-218
Reduced aortic elasticity and dilatation are associated with aortic regurgitation and left ventricular hypertrophy in nonstenotic bicuspid aortic valve patients.