Apical Hypertrophic Cardiomyopathy
Conditions
Keywords
Apical hypertrophic cardiomyopathy, Midventricular obstruction and cavity obliteration, Septal myectomy, hypertrophic cardiomyopathy, sudden cardiac death
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.
Interventions
PROCEDURESurgical transaortic left ventricular cavity remodeling in patients with apical hypertrophic cardiomyopathy without left ventricular outflow tract obstruction
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
Sponsors
Petrovsky National Research Centre of Surgery
Study design
Observational model
CASE_ONLY
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
15 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Hypertrophic apical cardiomyopathy according to medical records * Surgical left ventricular remodeling performed
Exclusion criteria
* LVOT gradient greater than 30 mmHg
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Hospital mortality after undergoing trans aortic surgical left ventricular remodeling | Assessment by medical records during the first 28 days after surgical intervention | Binary value: of alive/dead |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| left ventricular diastolic dysfunction | Perioperative/Periprocedural | 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. |
| Heart Failure (NYHA) | 1 month after surgery | Functional class of heart failure according to New York Heart Association (NYHA) Functional Classification of heart failure |
| Need for mechanical circulation in the postoperative period | Perioperative/Periprocedural | Binary value: yes/no |
| Freedom from re-interventions after surgical left ventricular remodeling | hrough study completion, an average of 1 year | Binary value yes/no According to available medical records for the entire follow-up period |
Other
| Measure | Time frame | Description |
|---|---|---|
| Search for mutations in full genomic DNA by sequencing method | through study completion, an average of 1 year | 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 |
Countries
Russia
Outcome results
None listed