A 63-year-old white male presented with altered mental status after falling off a couch. Initially, he was found in hemorrhagic shock as a result of splenic laceration and acute upper gastrointestinal bleed. He was fluid resuscitated and stabilized. Past medical history was significant for end-stage renal disease on hemodialysis, alcoholic cirrhosis, hyperlipidemia, and hypertension. Hospitalization was complicated by Enterococcus faecalis sepsis, and appropriate antibiotics were started. A concern for endocarditis has been raised. An echocardiogram was performed, as shown below.
Video 1
What is the most likely diagnosis?
Show Answer
The correct answer is: E. Aortic regurgitation and mitral regurgitation with A1/A2 perforation
A transesophageal echocardiogram with 2D imaging and color flow Doppler shows moderate-to-severe mitral regurgitation with perforation of the A1/A2 segment of the anterior leaflet of the mitral valve. It also shows an aortic valve vegetation and severe aortic regurgitation, suggesting endocarditis.
The inter-ventricular septum and tricuspid valve were intact, which excludes answers A and B. There is no fistula connecting the aorta and the left atrium, which excludes answer C. Although infective endocarditis (IE) can extend from the aortic valve to the subaortic structures consisting of the mitral-aortic intervalvular fibrosa and the anterior mitral leaflet, there was no evidence that the layers of the mitral leaflet had separated to form a pseudoaneurysm, which excludes answer D.
The case illustrates progressive native valve endocarditis secondary to Enterococcus species, which affected both the aortic and mitral valves. Enterococcus faecalis is the third main cause of IE worldwide, and it is gaining relevance, especially among healthcare encounters. Left-sided native valve IE accounts for fewer than 6.2 cases per 100,000 person-years in the developed world.1,5 Valve perforation following IE can lead to severe valve destruction, severe valve incompetence, intractable heart failure, and subsequent high hospital mortality. Although its frequency is not well recognized, it was reported in earlier autopsy studies2 and subsequent echocardiography reports3 with incidence in less than 35% in the latter. Perforation of the mitral valve is more common than perforation of the aortic valve and is correlated with hemodialysis, severe valvular insufficiency, and significant morbidity and mortality.4,5 Because physical examination in this condition may be misleading, echocardiography serves as the cornerstone for early diagnosis and early definitive surgery because antibiotics are very unlikely to yield any improvement in valvular regurgitation resulting from a significant perforation.
References
Mylonakis E, Calderwood SB. Infective endocarditis in adults. N Engl J Med 2001;345:1318-30.
Buchbinder NA, Roberts WC. Left-sided valvular active infective endocarditis. A study of forty-five necropsy patients. Am J Med 1972;53:20-35.
De Castro S, d'Amati G, Cartoni D, et al. Valvular perforation in the left-sided infective endocarditis: a prospective echocardiographic evaluation and clinical outcome. Am Heart J 1997;134:656-64.
Bachour K, Zmily H, Kizilbash M, et al. Valvular perforation in left-sided native valve infective endocarditis. Clin Cardiol 2009;32:E55-62.
Luk A, Kim ML, Ross HJ, Rao V, David TE, Butany J. Native and prosthetic valve infective endocarditis: clinicopathologic correlation and review of the literature. Malays J Pathol 2014;36:71-81.