Surgical Treatment of the Patients With Restrictive Phenotype of the Hypertrophic Cardiomyopathy Without LVOT Obstruction

NCT06977646 | Enrolling By Invitation

• 1 other identifier: ApHCM-001
• Study Type: observational
• Target: 50
• Locations: 1 country
• Sites: 1

Brief Summary

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.

Conditions

Apical Hypertrophic Cardiomyopathy

Keywords

Apical hypertrophic cardiomyopathy; Midventricular obstruction and cavity obliteration; Septal myectomy; hypertrophic cardiomyopathy; sudden cardiac death

Outcome Measures

Primary Outcomes (1)

• 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

Secondary Outcomes (4)

• left ventricular diastolic dysfunction

  Perioperative/Periprocedural

• Heart Failure (NYHA)

  1 month after surgery

• Need for mechanical circulation in the postoperative period

  Perioperative/Periprocedural

• Freedom from re-interventions after surgical left ventricular remodeling

  hrough study completion, an average of 1 year

Other Outcomes (1)

• Search for mutations in full genomic DNA by sequencing method

  through study completion, an average of 1 year

Study Arms (1)

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.

Procedure: Surgical transaortic left ventricular cavity remodeling in patients with apical hypertrophic cardiomyopathy without left ventricular outflow tract obstruction

Interventions

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

Patients with hypertrophic apical cardiomyopathy undergoing surgical interventions

Eligibility Criteria

• Age: 15 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

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

You may qualify if:

• Hypertrophic apical cardiomyopathy according to medical records
• Surgical left ventricular remodeling performed

You may not qualify if:

• LVOT gradient greater than 30 mmHg

Sponsors & Collaborators

• Petrovsky National Research Centre of Surgery: lead

Study Sites (1)

Petrovsky National Research Centre of Surgery

Moscow, Moscow, 119991, Russia

Location

Biospecimen

Retention: SAMPLES WITH DNA

venous blood

MeSH Terms

Conditions

Apical Hypertrophic Cardiomyopathy; Cardiomyopathy, Hypertrophic; Death, Sudden, Cardiac

Condition Hierarchy (Ancestors)

Hypertrophy, Left Ventricular; Cardiomegaly; Heart Diseases; Cardiovascular Diseases; Cardiomyopathies; Aortic Stenosis, Subvalvular; Aortic Valve Stenosis; Aortic Valve Disease; Heart Valve Diseases; Heart Arrest; Death, Sudden; Death; Pathologic Processes; Pathological Conditions, Signs and Symptoms

Study Officials

• Sergey L Dzemeshkevich, MD, PhD, Prof.

  Petrovsky National Research Centre of Surgery

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: CASE ONLY
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: May 7, 2025
• First Posted: May 18, 2025
• Study Start: June 1, 2025
• Primary Completion: September 1, 2025
• Study Completion: December 1, 2025
• Last Updated: July 2, 2025

Record last verified: 2025-07

Locations

RU (1)