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Purpose: Mitral valve anomalies of insertion are not so rare but often misdiagnosed and confused with other pathologies (HCM, subaortic stenosis, storage diseases). Hypertrophic cardiomyopathy in particular is associated in 5-8% of the cases to mitral valve anomalies: in these patients differential diagnosis is mandatory. Five different types of mitral valve anomalies of insertion have been identified. The most frequent type is associated to left ventricular outflow gradient, significant mitral regurgitation and left ventricular hypertrophy. Medical therapy is not so beneficial and surgical therapy is decisive, hence recognition of such anomalies has a strict relation to prognosis for the patients affected. Our aim was to evaluate the importance of correctly identifying mitral valve anomalies of insertion as a possible cause of left ventricular hypertrophy, in order to decide the appropriate therapeutic approach for these patients.Methods: From 1990 to 2011 we have found 18 cases of patients with mitral valve anomalies of insertion during the daily routine activity in our Echocardiography Laboratory. These patients were previously diagnosed as affected by congenital hypertrophic cardiomyopathy or severe left ventricular hypertrophy without a clear etiology. All the patients had a significant left ventricular outflow gradient and different degrees of mitral regurgitation.Results: The specific anomalies in the 18 patients were: anomalous insertion of the mitral valve tissue on the ventricular wall in 13 patients and accessory valvular tissue in 5 patients. There were no other cardiac abnormalities associated. The identification of the specific cause avoided inappropriate therapies such as defibrillator implantation. Three patients underwent surgical repair: the simple disinsertion of the anomalous tissue allowed complete disappearance of the outflow gradient, complete or significant reduction of the mitral regurgitation and progressive reverse remodeling of the left ventricle with regression of the left ventricular hypertrophy, as shown in the post-operative echocardiograms.Conclusions: Correct identification of mitral valve anomalies as a possible etiology of ventricular hypertrophy is fundamental to address such patients to the correct therapeutic pathway, including surgical repair as a choice for a definitive solution. Most importantly, the diagnosis of these specific mitral valve abnormalities has a great prognostic relevance towards more severe diseases such as Hypertrophic cardiomyopathy, the latter having a totally different prognostic and therapeutic impact.