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Burned-out Phase in Apical Hypertrophic Cardiomyopathy (Echocardiographic Data of 230 Patients with Apical HCM)

AUTHORS

avatar Mohammad Ali Sadr Ameli ORCID 1 , avatar Azin Alizadehasl ORCID 2 , avatar Zohre Rahbar ORCID 2 , * , avatar Maryam Maharlou ORCID 3 , avatar Sepehr Jamalkhani ORCID 4 , avatar Davood Khoda-Amorzideh ORCID 2 , avatar kamran roudini ORCID 5 , avatar Hooman Bakhshandeh ORCID 6 , avatar Mahdi Khalili ORCID 4 , avatar Zahra Shahidzadeh ORCID 4 , avatar Marzie Bazzi ORCID 4 , avatar maedeh Barahman ORCID 7 , avatar Nima Sarisarraf ORCID 4 , avatar Masood Shekarchizadeh ORCID 4 , avatar Behshid Ghadrdoost ORCID 1

1 Department of Cardiovascular Intervention, Rajaie Cardiovascular Medical and Research Center, Tehran, IR Iran

2 Department of Cardio-oncology, Rajaie Cardiovascular Medical and Research Center, Tehran, IR Iran

3 Department of Echocardiography, Rajaie Cardiovascular Medical and Research Center, Tehran, IR Iran

4 Rajaei Cardiovascular Medical and Research Center, Tehran, IR Iran

5 Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, IR Iran

6 Heart Research Center, Rajaie Cardiovascular, Medical and Research Center, Tehran, IR Iran

7 Firoozgar Hospital, Iran University of Medical Sciences, Tehran, IR Iran

How to Cite: Sadr Ameli M A, Alizadehasl A, Rahbar Z, Maharlou M, Jamalkhani S, et al. Burned-out Phase in Apical Hypertrophic Cardiomyopathy (Echocardiographic Data of 230 Patients with Apical HCM). Int Cardio Res J. 2022;16(2):e122923.

ARTICLE INFORMATION

International Cardiovascular Research Journal: 16 (2); e122923
Published Online: June 15, 2022
Article Type: Research Article
Received: January 24, 2022
Revised: March 14, 2022
Accepted: April 24, 2022
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Abstract

Background: Apical Hypertrophic Cardiomyopathy (ApHCM) is a rare form of hypertrophic
cardiomyopathy with sarcomere protein gene mutations, which predominantly affects
the apex of the left ventricle. Sudden Cardiac Death (SCD) and cardiovascular morbidity
are less likely to occur in patients with isolated ApHCM. However, recent data suggested
annual cardiac death rates of 0.5 - 4%, approaching those for classic HCM.
Objectives: The present study aimed to assess the prevalence of burned-out ApHCM and
its predictors.
Methods: In this retrospective cross-sectional study, echocardiographic data and
electrocardiography features of 230 patients with ApHCM including premature
ventricular contraction and atrial fibrillation were gathered and analyzed at baseline
and after a mean follow-up of five years. All data were obtained from the data registry
of Rajaie Cardiovascular Medical and Research Center, Tehran, Iran. The data were
included in a retrospective study, which was approved by the local Ethics Committee.
The patients were divided into two groups as follows: 1- Left Ventricular Ejection Fraction
(LVEF) ≥ 50% and 2- LVEF < 50% known as burned-out ApHCM. Data analysis was also
based on LVEF. Generalized Estimating Equation (GEE) regression was performed to
assess the association between the patients’ characteristics and burned-out ApHCM. The
clinical features demonstrating P < 0.05 in the univariate GEE analysis were included in
a multivariate model to identify the independent predictors.
Results: The prevalence of burned-out ApHCM, defined as LVEF < 50%, was 13.9%.
There was no significant difference between males and females in this regard (P = 0.48).
After a mean follow-up of five years, atrial fibrillation was significantly more common
in the patients with LVEF < 50% (48.7% vs. 24.4%, P = 0.007). Additionally, left atrial
enlargement was identified as the most important predictor of BO-ApHCM (odds ratio
= 2.4, P = 0.003). Moreover, right ventricular dysfunction was more severe in the patients
with burned-out ApHCM (P < 0.001).
Conclusions: The prevalence of burned-out HCM was higher in the present study than
in the previous studies (13.9%). Besides, right ventricular systolic dysfunction and left
atrial enlargement were two main predictors of the ApHCM progression.

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References

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© 2022, Shiraz University of Medical Sciences.
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