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1 . 2023

Left ventricular remodeling and mitral reconstruction in patient with hypertrophic cardiomyopathy and inflow obstruction

Abstract

Diffuse generalized phenotype of of hypertrophic cardiomyopathy (HCM) is one of the most difficult variants for surgical reconstruction. Surgical correction consists not only of extended myectomy of the interventricular septum (IVS) but also of sub-mitral area reconstruction in the left ventricle. Selection of patients for such a reconstruction is particularly difficult since there is no significant pressure gradient in the left ventricle outflow tract (LVOT), no severe mitral regurgitation but significant diastolic dysfunction is present. Despite medical treatment the natural course of the disease is progredient, and hemodynamics is worsening continuously. Such patients suffer from progressive heart failure and are considered as candidates for orthotopic heart transplantation.

In this publication we demonstrate the clinical case of a patient with severe inflow obstruction but not in LVOT, who required a combined surgical intervention: LV cavity remodeling and plastic reconstruction of the dysplastic mitral valve. In 1-year of follow-up after surgery she has a significant clinical and hemodynamic improvement with increasing of the LV diastolic filling and decreased myocardial mass. Here there is the first demonstration of the electronic microscopy features of the myocardial samples of this particular and relatively uncommon HCM phenotype associated with nonsense mutation in the ALPK3 gene.

Keywords:hypertrophic cardiomyopathy; non-obstructive HCM; inflow obstruction, mitral dysplasia; ALPK3; electronic microscopy; diastolic dysfunction

Funding. Genetic study was supported by Russian Science Foundation project N 22-75-00134, https://rscf.ru/project/22-75-00134.
Conflict of interest. The authors declare no conflict of interest.
For citation: Dzemeshkevich S.L., Motreva A.P., Areshidze D.A., Kozlova M.A., Chernikov V.P., Mershina E.A., Martyanova Yu.B., Kalmykova O.V., Tber M., Petrova O.V., Balashova M.S., Sadekova M.A., Zaklyazminskaya E.V. Left ventricular remodeling and mitral reconstruction in patient with hypertrophic cardiomyopathy and inflow obstruction. Clinical and Experimental Surgery. Petrovsky Journal. 2023; 11 (1): 129–37. DOI: https://doi.org/10.33029/2308-1198-2023-11-1-129-137  (in Russian)

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CHIEF EDITOR
CHIEF EDITOR
Sergey L. Dzemeshkevich
MD, Professor (Moscow, Russia)

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