Noncompaction and endocarditis in suspected mitochondrial disorder

Objectives To report the echocardiographic and autopsy findings of left ventricular hypertrabeculation (LVHT), also known as noncompaction, in a patient with central and peripheral nervous system disease, who died shortly after diagnosing noncompaction. Case report In a 75 year old woman with cognitive decline, Parkinson syndrome, stroke-like-episodes, basal ganglia calcification, neuromuscular disorder, cataract, diabetes, anemia, arterial hypertension, recurrent heart failure, left anterior hemiblock, and right bundle branch block, acute heart failure developed during treatment of a suspected urosepsis. Transthoracic echocardiography revealed severely reduced fractional shortening, valvular vegetations, and LVHT. Endocarditis was suspected. She died shortly afterwards from renal failure and ventricular fibrillation. Endocarditis and LVHT were confirmed at autopsy. Conclusions This case shows that endocarditis may occur together with LVHT and central and peripheral nervous system disease indicative of a mitochondrial disorder. Whether LVHT predisposes for endocarditis remains speculative.