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Hypertrophic cardiomyopathy - is an inherited disease characterized by pronounced genetic and phenotypic heterogeneity. There are two most common anatomic variants of cardiac hypertrophy: subaortic and submitral phenotypes. Subaortic phenotype is characterized by hypertrophy of the basal parts of the heart, mainly in the interventricular septum (IVS), manifesting by a high pressure gradient in the LVOT. Submitral phenotype is characterized by localization of hypertrophic zone downward to the apex and apical phenotype is without a pressure gradient in the LVOT.
The morphology, nature of hemodynamic abnormalities not well studied in patients with apical phenotype of HCM, and surgical treatment are controversial, and for those patients with advanced stage of the HF the orthotopic heart transplantations (HTx) is usually considered.
One of the surgical techniques available for this category of patients is apical myectomy. The main goal of this intervention is increasing the left ventricular volume and improving of the LV compliance with an increase of the diastolic relaxation. Limited data of such procedures in HCM patients were already published but it still requires further investigation on larger cohort of patients.
In this study, the investigators hypothesize that along with left ventricular septal hypertrophy, a small cavity is formed in patients with submittal-apical phenotype due to an increased number of hypertrophied papillary muscles. They are displaced to the apex and tightly fixed both among themselves and to the left ventricular walls. This causes a significant reduction in diastolic volume and left ventricular relaxation capacity. The present study will analyze the experience of performing resection of hypertrophied trabeculae and mobilization of papillary muscles performed through the aorta. Throw this approach procedure can be done without the need for traumatic access and suturing in the apex of the left ventricle.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Patients with hypertrophic apical cardiomyopathy undergoing surgical interventions | Patients who underwent surgery for apical hypertrophic cardiomyopathy without significant left ventricular outflow tract obstruction. Patients with transapical surgical access will not be included in the study. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Surgical transaortic left ventricular cavity remodeling in patients with apical hypertrophic cardiomyopathy without left ventricular outflow tract obstruction | Procedure | The proposed intervention is a variation of classical myectomy, but unlike it, the main substrate for resection is not only the hypertrophied interventricular septum, but the abnormal papillary muscles and interpapillary trabeculae in the left ventricular cavity |
| Measure | Description | Time Frame |
|---|---|---|
| Hospital mortality after undergoing trans aortic surgical left ventricular remodeling | Binary value: of alive/dead | Assessment by medical records during the first 28 days after surgical intervention |
| Measure | Description | Time Frame |
|---|---|---|
| left ventricular diastolic dysfunction | Diastolic left ventricular function relying on echo protocols. E/A ratio as an equation of an early transmitral flow (E wave) and a late flow with atrial contraction (A wave). An E/A ratio less than 0.75 or greater than 1.5 indicates dyastolic disfunction. | Perioperative/Periprocedural |
| Measure | Description | Time Frame |
|---|---|---|
| Search for mutations in full genomic DNA by sequencing method | Pathogenicity assessment of candidate genetic variants is performed according to the ACMG recommendations (2015) Will be performed on patients who have a preserved tissue sample suitable for whole genome sequencing | through study completion, an average of 1 year |
Inclusion Criteria:
Exclusion Criteria:
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The study will include all patients meeting the inclusion criteria treated in the period from 2017 to 2024 at the Petrovsky National Research Centre of Surgery and the Federal Cardiac Surgery Center in Astrakhan
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| Name | Affiliation | Role |
|---|---|---|
| Sergey L Dzemeshkevich, MD, PhD, Prof. | Petrovsky National Research Centre of Surgery | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Petrovsky National Research Centre of Surgery | Moscow | Moscow | 119991 | Russia |
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venous blood
|
| Heart Failure (NYHA) |
Functional class of heart failure according to New York Heart Association (NYHA) Functional Classification of heart failure |
| 1 month after surgery |
| Need for mechanical circulation in the postoperative period | Binary value: yes/no | Perioperative/Periprocedural |
| Freedom from re-interventions after surgical left ventricular remodeling | Binary value yes/no According to available medical records for the entire follow-up period | hrough study completion, an average of 1 year |
| ID | Term |
|---|---|
| D000092183 | Apical Hypertrophic Cardiomyopathy |
| D002312 | Cardiomyopathy, Hypertrophic |
| D016757 | Death, Sudden, Cardiac |
| ID | Term |
|---|---|
| D017379 | Hypertrophy, Left Ventricular |
| D006332 | Cardiomegaly |
| D006331 | Heart Diseases |
| D002318 | Cardiovascular Diseases |
| D009202 | Cardiomyopathies |
| D001020 | Aortic Stenosis, Subvalvular |
| D001024 | Aortic Valve Stenosis |
| D000082862 | Aortic Valve Disease |
| D006349 | Heart Valve Diseases |
| D006323 | Heart Arrest |
| D003645 | Death, Sudden |
| D003643 | Death |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |
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