Lipodystrophy – ABC CARDIOL

ABC

Editorial

Understanding Cardiac Alterations in Familial Partial Lipodystrophy: Insights from Echocardiography

Lipodystrophies are a diverse group of diseases characterized by a loss of fatty tissue in the body. Consequently, lipids are stored in non-fat cells, leading to major metabolic consequences, such as liver steatosis and insulin resistance, and increased risk of cardiovascular diseases due to premature atherosclerosis. Lipodystrophies can be classified as congenital or acquired, and generalized or partial, depending on the extent of fat distribution disturbance. Unlike congenital generalized lipodystrophy (CGL), patients with familial partial lipodystrophy (PL) do not have a widespread lack of fat throughout the body but rather a localized loss of fat in limbs and trunk. PL is a rare genetic disorder often accompanied by mutations in the lamin A/C gene (LMNA), belonging to the complex group of laminopathies that can lead to muscular and cardiac dystrophies, neuropathies, and syndromes of premature aging.^, Due to the rarity of the lipodystrophies and the phenotypic range of cardiac manifestations, only case reports and studies with limited numbers of patients, mostly restricted to individuals with CGL, are available.

Cardiomyopathy associated with familial PL remains underexplored despite its potential to cause early mortality. While premature atherosclerosis and cardiac arrhythmias are common, the direct impact of gene mutations on cardiac structure and function is unclear. Previous reports have described different phenotypes of cardiomyopathies in this group of patients, ranging from isolated left ventricular (LV) hypertrophy and hypertrophic cardiomyopathy to dilated cardiomyopathy.

[…]

Read Full Article

ABC

Original Article

Echocardiographic Alterations of Cardiac Geometry and Function in Patients with Familial Partial Lipodystrophy

Minna Moreira Dias Romano et al. André Timóteo Sapalo Natália Rossin Guidorizzi Henrique Turin Moreira … Paula Ananda Chacon Inês Lucas Candelária Kalil Maria Cristina Foss Francisco José Albuquerque de Paula

Abstract

Background:

Cardiomyopathy associated with partial lipodystrophy (PL) has not been well described yet.

Objective:

To characterize cardiac morphology and function in PL.

Methods:

Patients with familial PL and controls were prospectively assessed by transthoracic echocardiography and with speckle-tracking echocardiography (global longitudinal strain, GLS). The relationship between echocardiographic variables and PL diagnosis was tested with regression models, considering the effect of systolic blood pressure (SBP). Significance level of 5% was adopted.

Results:

Twenty-nine patients with PL were compared to 17 controls. They did not differ in age (p=0.94), gender or body mass index (p= 0.05). Patients with PL had statistically higher SBP (p=0.02) than controls. Also, PL patients had higher left atrial dimension (37.3 ± 4.4 vs. 32.1 ± 4.3 mm, p= 0.001) and left atrial (30.2 ± 7.2 vs. 24.9 ± 9.0 mL/m²,p=0.02), left ventricular (LV) mass (79.3 ± 17.4 vs. 67.1 ± 19.4, p=0.02), and reduced diastolic LV parameters (E’ lateral, p= 0.001) (E’ septal, p= 0.001), (E/E’ ratio, p= 0.02). LV ejection fraction (64.7 ± 4.6 vs. 62.2 ± 4.4 %, p= 0.08) and GLS were not statistically different between groups (-17.1 ± 2.7 vs. -18.0 ± 2.0 %, p= 0.25). There was a positive relationship of left atrium (β 5.6, p<0.001), posterior wall thickness, (β 1.3, p=0.011), E’ lateral (β -3.5, p=0.002) and E’ septal (β -3.2, p<0.001) with PL diagnosis, even after adjusted for SBP.

Conclusion:

LP patients have LV hypertrophy, left atrial enlargement, and LV diastolic dysfunction although preserved LVEF and GLS. Echocardiographic parameters are related to PL diagnosis independent of SBP.

Read Full Article

IJCS

Original Article

Self-reported HIV/HAART-associated Lipodystrophy and Modifiable Risk Factors for Cardiovascular Disease

Thiago Veiga Jardim et al. Rodrigo de Castro Cardoso Annelisa Silva e Alves de Carvalho Santos Marianne de Oliveira Falco Erika Aparecida Silveira

Abstract

Background

Patient self-report is the most common diagnostic tool in the literature to detect HIV/HAART-associated lipodystrophy. However, data on the association of cardiovascular risk factors with HIV/HAART-associated lipodystrophy assessed by self-report are still missing.

Objectives

To determine the prevalence of self-reported HIV/HAART-associated lipodystrophy and to identify independent associations between traditional modifiable cardiovascular risk factors and self-reported lipodystrophy.

Methods

We conducted a retrospective observational study at an outpatient infectious disease clinic in the Central-West of Brazil to identify the association between traditional modifiable cardiovascular risk factors and self-reported lipodystrophy. Sedentary lifestyle, smoking status, family history of cardiovascular disease, hypertension, diabetes, dyslipidemia, increased waist circumference and overweight were the cardiovascular risk factors assessed. Self-reported HIV/HART-associated lipodystrophy was categorized as: mild (noticeable by patients’ close inspection), moderate (easily noticeable by patient and physician) or severe (readily noticeable by a casual observer). Prevalence ratio (PR) and 95% confidence interval (CI95%) were calculated. Multivariate Poisson’s regression was used to analyze factors associated to HIV/HAART-associated lipodystrophy assessed by self-report considering a significance level of 5%.

Results

A total of 183 patients were included, with a mean age of 39.3±10.9 years. Most of the sample were male (77.6%), non-white (50.8%) and single (53.0%). The overall prevalence of HIV/HAART-associated lipodystrophy was 52.5% (95% CI 44.96 – 59.88). Severe lipodystrophy was observed in more than half patients (55.2%). No traditional modifiable cardiovascular risk factor was independently associated with lipodystrophy. Female sex (PR 1.49; 95% CI 1.15 – 1.95; p =0.003), time of HIV infection diagnosis of 1-3 years (PR 1.83; 95% CI 1.09 – 3.08; p =0.002) and a positive family history of CVD (PR 1.62; 95% CI 1.11 – 2.36; p <0.001) were independently associated with lipodystrophy.

Conclusion

HIV/HAART-associated lipodystrophy assessed by patient self-report was not associated with traditional modifiable cardiovascular risk factors. Int J Cardiovasc Sci. 2020; [online].ahead print, PP.0-0

Read Full Article

Understanding Cardiac Alterations in Familial Partial Lipodystrophy: Insights from Echocardiography

Echocardiographic Alterations of Cardiac Geometry and Function in Patients with Familial Partial Lipodystrophy

Abstract

Background:

Objective:

Methods:

Results:

Conclusion:

Self-reported HIV/HAART-associated Lipodystrophy and Modifiable Risk Factors for Cardiovascular Disease

Abstract

Background

Objectives

Methods

Results

Conclusion

Electronic Document Format (Vancouver)

Electronic Document Format (ABNT)

Electronic Document Format (APA)

Electronic Document Format (ISO)

Electronic Document Format (Vancouver)

Electronic Document Format (ABNT)

Electronic Document Format (APA)

Electronic Document Format (ISO)

Electronic Document Format (Vancouver)

Electronic Document Format (ABNT)

Electronic Document Format (APA)

Electronic Document Format (ISO)