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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