Abstract

Approximately, 75% of patients with infective endocarditis have underlying structural heart disease at the time of diagnosis. Aortic valve disease is associated with up to a quarter of patients, with aortic regurgitation being the most common abnormality. A unicuspid aortic valve (UAV) is an extremely rare congenital anomaly with a prevalence of 0.02%. We present a unique case of UAV with a devastating association. Case Summary: A 32-year-old male intravenous drug abuser presented with weakness after 18-hours of symptom onset. He was afebrile but tachycardic, with left-sided hemiparesis and sensory loss. Brain CT-scan revealed a hypodensity within the right cerebral hemisphere, a CTA of head-neck revealed an acute right MCA infarct with malignant cerebral edema. Due to deteriorating clinical condition, he was intubated for airway protection and underwent emergent hemi-craniectomy. Blood cultures grew enterococcus faecalis. Transthoracic echocardiogram showed abnormal aortic valve with severe regurgitation. Transesophageal echocardiography (TEE) demonstrated unicuspid unicommissural aortic valve with a small mobile echodensity attached to the left ventricular outflow tract aspect. Decision Making: He received a 6-weeks course of ceftriaxone and vancomycin with negative repeat blood cultures. Given poor clinical state, he was deemed poor candidate for valve replacement and was discharged to long-term rehabilitation facility. Four-months later, he was re-admitted with fevers. Repeat TEE demonstrated multiple aortic valve vegetations. He subsequently underwent successful minimally invasive aortic valve replacement. Conclusion: Unicuspid unicommisural aortic valve is an extremely rare condition that can be complicated by aortic stenosis more often than regurgitation. Infective endocarditis has been reported in 11% among UAV patients and can lead to valvular destruction with severe regurgitation which can be catastrophic in some patients

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