Cardiac Fabry's disease: an unusual cause of left ventricular hypertrophy.

Nat Clin Pract Cardiovasc Med

Faculdade de Medicina do Porto, Alameda Prof Hernâni Monteiro, 4200 Porto, Portugal.

Published: November 2007

Background: A 64-year-old male was observed as an outpatient with atypical, non-exercise-induced chest pain and palpitations. He had arterial hypertension and marked concentric left ventricular hypertrophy. After 2.5 years of antihypertensive drug therapy the patient's blood pressure had returned to normal, but his left ventricular hypertrophy was unchanged.

Investigations: Electrocardiography, transthoracic echocardiography, myocardial perfusion scintigraphic imaging, measurement of alpha-galactosidase A activity, gene sequencing, brain MRI, carotid artery ultrasonography, biochemical renal evaluation and cardiac Doppler tissue imaging.

Diagnosis: Cardiac Fabry's disease.

Management: Losartan, hydrochlorothiazide, low-dose aspirin and bisoprolol. The patient is expected to begin enzyme replacement therapy.

Download full-text PDF

Source
http://dx.doi.org/10.1038/ncpcardio1012DOI Listing

Publication Analysis

Top Keywords

left ventricular
12
ventricular hypertrophy
12
cardiac fabry's
8
fabry's disease
4
disease unusual
4
unusual left
4
hypertrophy background
4
background 64-year-old
4
64-year-old male
4
male observed
4

Similar Publications

Background: Long-term consumption of Western Diet (WD) is a well-established risk factor for the development of cardiovascular disease (CVD); however, there is a paucity of studies on the long-term effects of WD on the pathophysiology of CVD and sex-specific responses.

Methods: Our study aimed to investigate the sex-specific pathophysiological changes in left ventricular (LV) function using transthoracic echocardiography (ECHO) and LV tissue transcriptomics in WD-fed C57BL/6 J mice for 125 days, starting at the age of 300 through 425 days.

Results: In female mice, consumption of the WD diet showed long-term effects on LV structure and possible development of HFpEF-like phenotype with compensatory cardiac structural changes later in life.

View Article and Find Full Text PDF

Previous studies demonstrated that dexmedetomidine (Dex) posttreatment aggravated myocardial dysfunction and reduced survival in septic mice. Yet, whether Dex elicits similar effects in septic patients as defined by Sepsis-3 remains unknown. This study sought to assess the effects of Dex-based sedation on mortality and cardiac dysfunction in septic patients defined by Sepsis-3 and to further reveal the mechanisms in septic rats.

View Article and Find Full Text PDF

Background: Interventricular dyssynchrony derived from the classic non-physiological stimulation (n-PS) of the right ventricle (RV) is a known cause of left ventricular dysfunction (LVDys).

Methods: This was a prospective descriptive single-center study. We analyzed patients who develop LVDys with n-PS, and the results after upgrading to conduction system pacing (CSP).

View Article and Find Full Text PDF

Fibrosis is the main pathological feature of aortic stiffness, which is a common extracardiac comorbidity of heart failure with preserved ejection fraction (HFpEF) and a contributor to left ventricular (LV) diastolic dysfunction. Systemic low-grade inflammation plays a crucial role in the pathogenesis of HFpEF and the development of vascular fibrosis. In this study, we investigate the inflammatory mechanism of aortic fibrosis in HFpEF using a novel mouse model.

View Article and Find Full Text PDF

The correlation between Fischer's ratio and the risk of cardiac dysfunction in heart failure patients.

BMC Cardiovasc Disord

December 2024

Department of Cardiovascular Medicine, Rui Jin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200025, People's Republic of China.

Backgrounds: Due to the high mortality and hospitalization rate in chronic heart failure (HF), it is of great significance to study myocardial nutrition conditions. Amino acids (AAs) are essential nutrient metabolites for cell development and survival. This study aims to investigate the associations and prognostic value of plasma branched-chain amino acid/aromatic amino acid ratio (Fischer's ratio, FR) in patients with left ventricular ejection fraction (LVEF) ≤ 50%.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!