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1.
Front Cardiovasc Med ; 11: 1362692, 2024.
Article in English | MEDLINE | ID: mdl-38550516

ABSTRACT

Peripartum Cardiomyopathy (PPCM) is a polymorphic myocardial disease occurring late during pregnancy or early after delivery. While reduced systolic function and heart failure (HF) symptoms have been widely described, there is still a lack of reports about the arrhythmic manifestations of the disease. Most importantly, a broad range of unidentified pre-existing conditions, which may be missed by general practitioners and gynecologists, must be considered in differential diagnosis. The issue is relevant since some arrhythmias are associated to sudden cardiac death occurring in young patients, and the overall risk does not cease during the early postpartum period. This is why multimodality diagnostic workup and multidisciplinary management are highly suggested for these patients. We reported a series of 16 patients diagnosed with PPCM following arrhythmic clinical presentation. Both inpatients and outpatients were identified retrospectively. We performed several tests to identify the arrhythmic phenomena, inflammation and fibrosis presence. Cardiomyopathies phenotypes were reclassified in compliance with the updated ESC guidelines recommendations. Arrhythmias were documented in all the patients during the first cardiological assessment. PVC were the most common recorder arrhythmias, followed by VF, NSVT, AF, CSD.

2.
Healthcare (Basel) ; 12(6)2024 Mar 14.
Article in English | MEDLINE | ID: mdl-38540618

ABSTRACT

This review aims to delineate the potential impact of static meditation practice on cholesterol and triglyceride levels. PubMed, EMBASE, Web of Science, Cochrane Library, and Google Scholar were systematically screened up until December 2023 to identify pertinent studies. After searching the scientific literature, 16 clinical studies (11 trials and 5 observational experiments) met the criteria for inclusion, involving a total of 1147 participants. In general, Ayurvedic-based meditation techniques were predominantly associated with lower total cholesterol levels, mindfulness-based techniques demonstrated benefits in both total cholesterol and triglyceride levels, and Eastern meditation techniques with spiritual origins were primarily linked to improved serum concentrations of HDL cholesterol. Study participants mostly engaged in meditation on a daily basis, often practicing it once or even twice a day, spanning a duration ranging from a few weeks to several months. The meta-analysis shows an association between meditation practice in healthy or sub-healthy adults and reduced cholesterol levels, with an average decrease of approximately -14 mg/dL (MD = -13.91 [-23.35; -4.47] mg/dL; p = 0.02), alongside favorable and even more pronounced impacts on triglyceride levels (MD = -32.56 [-48.44; -16.68] mg/dL; p < 0.01). In summary, regular engagement in static meditation practices can be associated with lower triglyceride and, to a lesser extent, cholesterol levels. Further studies on the topic are recommended to better investigate the relationship between meditation practice and physiological parameters.

3.
J Clin Med ; 13(6)2024 Mar 21.
Article in English | MEDLINE | ID: mdl-38542026

ABSTRACT

Purpose: Myocarditis is frequently a sporadic disease, but may also occur in the context of genetic disorders which may increase susceptibility to cardiac inflammation. Cardiac involvement in Wolfram syndrome type 1 (WS1) has been scarcely characterized. To our knowledge, no cases of virus-negative myocarditis have been reported in the WS1 pediatric population. Methods: We report the description of a pediatric case of acute myocarditis in the context of WS1, followed by a literature review of cardiovascular involvement associated with wolframin variants, and discuss potential pathophysiological mechanisms and therapeutic options. Results: A young patient with WS1, treated with insulin and liraglutide, was admitted for acute chest pain. Cardiac magnetic resonance and endomyocardial biopsy were performed to confirm the clinical suspicion of myocarditis. While congenital heart diseases and arrhythmias have been described previously in patients with WS1, this is the first description of virus-negative myocarditis. Conclusions: Myocarditis may represent a possible manifestation of cardiovascular involvement in WS1. Cardiovascular screening may be considered in patients with WS1.

4.
J Cardiovasc Med (Hagerstown) ; 25(3): 179-185, 2024 Mar 01.
Article in English | MEDLINE | ID: mdl-38305146

ABSTRACT

AIMS: Coronary artery ectasia (CAE) has been linked to the occurrence of adverse events in patients with ischemia/angina and no obstructive coronary arteries (INOCA/ANOCA), while the relationship between CAE and myocardial infarction with nonobstructive coronary arteries (MINOCA) has been poorly investigated. In our study we aimed at assessing differences in clinical, angiographic and prognostic features among patients with CAE and MINOCA vs. INOCA/ANOCA presentation. METHODS: Patients with angiographic evidence of CAE were enrolled at the University Hospital of Parma and divided into MINOCA vs. INOCA/ANOCA presentation. Clinical and quantitative angiographic information was recorded and the incidence of major adverse cardiovascular events (MACE) was assessed at follow-up. RESULTS: We enrolled a total of 97 patients: 49 (50.5%) with MINOCA and 48 (49.5%) with INOCA/ANOCA presentation. The presentation with MINOCA was associated with a higher frequency of inflammatory diseases ( P  = 0.041), multivessel CAE ( P  = 0.030) and thrombolysis in myocardial infarction (TIMI) flow < 3 ( P  = 0.013). At a median follow-up of 38 months, patients with MINOCA had a significantly higher incidence of MACE compared with those with INOCA/ANOCA [8 (16.3%) vs. 2 (4.2%), P  = 0.045], mainly driven by a higher rate of nonfatal MI [5 (10.2%) vs. 0 (0.0%), P  = 0.023]. At multivariate Cox regression analysis, the presentation with MINOCA ( P  = 0.039) and the presence of TIMI flow <3 ( P  = 0.037) were independent predictors of MACE at follow-up. CONCLUSION: Among a cohort of patients with CAE and nonobstructive coronary artery disease, the presentation with MINOCA predicted a worse outcome.


Subject(s)
Coronary Aneurysm , Coronary Artery Disease , Myocardial Infarction , Humans , Coronary Artery Disease/complications , Coronary Artery Disease/diagnostic imaging , Coronary Artery Disease/epidemiology , Prognosis , Coronary Vessels/diagnostic imaging , Dilatation, Pathologic/complications , MINOCA , Coronary Angiography/adverse effects , Risk Factors , Myocardial Infarction/diagnostic imaging , Myocardial Infarction/epidemiology , Myocardial Infarction/etiology , Angina Pectoris
6.
Eur Heart J Acute Cardiovasc Care ; 12(12): 810-817, 2023 Dec 21.
Article in English | MEDLINE | ID: mdl-37708418

ABSTRACT

AIMS: Globally, nearly 20% of cardiovascular disease deaths were attributable to air pollution. Out-of-hospital cardiac arrest (OHCA) represents a major public health problem; therefore, the identification of novel OHCA triggers is of crucial relevance. The aim of the study was to evaluate the association between air pollution (short-, mid-, and long-term exposures) and OHCA risk, during a 7-year period in a highly polluted urban area in northern Italy, with a high density of automated external defibrillators (AEDs). METHODS AND RESULTS: Out-of-hospital cardiac arrests were prospectively collected from the 'Progetto Vita Database' between 1 January 2010 and 31 December 2017; day-by-day air pollution levels were extracted from the Environmental Protection Agency stations. Electrocardiograms of OHCA interventions were collected from the AED data cards. Day-by-day particulate matter (PM) 2.5 and 10, ozone (O3), carbon monoxide (CO), and nitrogen dioxide (NO2) levels were measured. A total of 880 OHCAs occurred in 748 days. A significant increase in OHCA risk with a progressive increase in PM2.5, PM10, CO, and NO2 levels was found. After adjustment for temperature and seasons, a 9% and 12% increase in OHCA risk for each 10 µg/m3 increase in PM10 (P < 0.0001) and PM2.5 (P < 0.0001) levels was found. Air pollutant levels were associated with both asystole and shockable rhythm risk, while no correlation was found with pulseless electrical activity. CONCLUSION: Short- and mid-term exposures to PM2.5 and PM10 are independently associated with the risk of OHCA due to asystole or shockable rhythm.


Subject(s)
Air Pollutants , Air Pollution , Out-of-Hospital Cardiac Arrest , United States , Humans , Out-of-Hospital Cardiac Arrest/epidemiology , Out-of-Hospital Cardiac Arrest/etiology , Particulate Matter/adverse effects , Particulate Matter/analysis , Nitrogen Dioxide/analysis , Air Pollution/adverse effects , Air Pollution/analysis , Air Pollutants/adverse effects , Air Pollutants/analysis
7.
Prog Brain Res ; 280: 103-144, 2023.
Article in English | MEDLINE | ID: mdl-37714570

ABSTRACT

This systematic review explores the influence of silence on the autonomic nervous system. The Polyvagal Theory has been used as a reference model to describe the autonomic nervous system by explaining its role in emotional regulation, social engagement, and adaptive physiological responses. PubMed, Scopus, PsycInfo, EMBASE, and Google Scholar were systematically searched up until July 2023 for relevant studies. The literature search yielded 511 results, and 37 studies were eventually included in this review. Silence affects the autonomic nervous system differently based on whether it is inner or outer silence. Inner silence enhances activity of the ventral vagus, favoring social engagement, and reducing sympathetic nervous system activity and physiological stress. Outer silence, conversely, can induce a heightened state of alertness, potentially triggering vagal brake removal and sympathetic nervous system activation, though with training, it can foster inner silence, preventing such activation. The autonomic nervous system response to silence can also be influenced by other factors such as context, familiarity with silence, presence and quality of outer noise, and empathy.


Subject(s)
Autonomic Nervous System , Emotional Regulation , Humans , Vagus Nerve , Empathy , Recognition, Psychology
8.
JACC Basic Transl Sci ; 8(7): 755-765, 2023 Jul.
Article in English | MEDLINE | ID: mdl-37547072

ABSTRACT

The authors present a clinical report focused on the overlap between myocarditis and genetic cardiomyopathies of the dilated and arrhythmogenic spectrum. Our cohort was composed of 25 patients undergoing extensive baseline characterization and prospective reassessment by a dedicated multidisciplinary disease unit during a median follow-up of 69 months. We showed that the use of multimodal imaging allowed both discrimination of specific genotypes and identification of myocardial inflammation proven using endomyocardial biopsy. In addition, we showed that the use of immunomodulatory therapy was beneficial for most patients.

9.
Healthcare (Basel) ; 11(11)2023 May 25.
Article in English | MEDLINE | ID: mdl-37297697

ABSTRACT

Happiness is receiving more and more interest both as a determinant of health and a measure of outcome in biomedical and psychological sciences. The main objective of this study was to assess how the levels of happiness vary in a large sample of Italian adults and to identify the socio-demographic conditions which impair happiness domains the most. The participants of this survey consisted of 1695 Italian adults (85.9% women; 14.1% men) who completed the Measure of Happiness (MH) questionnaire online. In this study, the differences between groups in total and single domain (life perspective, psychophysical status, socio-relational sphere, relational private sphere, and financial status) happiness levels were examined through a propensity score matching analysis with respect to socio-demographic conditions, including gender, age, annual income, relationship status, having children, and education level. The results show that low income has a negative impact on happiness levels, whereas being in a relationship has a positive effect. Having children appears to have a negative impact on male happiness. Males appear to be happier than females, especially with regard to the psychophysics status. This evidence emphasizes the urgency for Italian policymakers to take actions on removing obstacles to people's happiness, especially with regard to financial distress, parenthood, and gender gaps.

10.
Brain Sci ; 13(5)2023 May 12.
Article in English | MEDLINE | ID: mdl-37239263

ABSTRACT

Concentration and memory impairment (named "brain fog") represents a frequent and disabling neuropsychological sequela in post-acute COVID-19 syndrome (PACS) patients. The aim of this study was to assess whether neurocognitive function could improve after a multidisciplinary rehabilitation program enhanced with individualized neuropsychological treatment. A prospective monocentric registry of PACS patients consecutively admitted to our Rehabilitation Unit was created. The Montreal Cognitive Assessment (MoCA) was used to assess cognitive impairment at admission and discharge. A total of sixty-four (64) PACS patients, fifty-six (56) of them with brain fog, were treated with a day-by-day individualized psychological intervention of cognitive stimulation (45 min) on top of a standard in-hospital rehabilitation program. The mean duration of the acute-phase hospitalization was 55.8 ± 25.8 days and the mean in-hospital rehabilitation duration was 30 ± 10 days. The mean age of the patients was 67.3 ± 10.4 years, 66% of them were male, none had a previous diagnosis of dementia, and 66% of the entire sample had experienced severe COVID-19. At admission, only 12% of the patients had normal cognitive function, while 57% showed mild, 28% moderate, and 3% severe cognitive impairment. After psychological treatment, a significant improvement in the MoCA score was found (20.4 ± 5 vs. 24.7 ± 3.7; p < 0.0001) as a result of significant amelioration in the following domains: attention task (p = 0.014), abstract reasoning (p = 0.003), language repetition (p = 0.002), memory recall (p < 0.0001), orientation (p < 0.0001), and visuospatial abilities (p < 0.0001). Moreover, the improvement remained significant after multivariate analysis adjusted for several confounding factors. Finally, at discharge, 43% of the patients with cognitive impairment normalized their cognitive function, while 4.7% were discharged with residual moderate cognitive impairment. In conclusion, our study provides evidence of the effects of multidisciplinary rehabilitation enhanced with neuropsychological treatment on improvement in the cognitive function of post-acute COVID-19 patients.

11.
J Pers Med ; 13(5)2023 May 22.
Article in English | MEDLINE | ID: mdl-37241047

ABSTRACT

Sudden cardiac death represents the leading cause of death worldwide; although the majority of sudden deaths occur in an elderly population with coronary artery disease, some occur in young and otherwise healthy individuals, as is the case of cardiomyopathies. The aim of the present review is to provide a stepwise hierarchical approach for the global sudden death risk estimation in primary cardiomyopathies. Each individual risk factor is analyzed for its contribution to the overall risk of sudden death for each specific cardiomyopathy as well as across all primary myocardial diseases. This stepwise hierarchical and personalized approach starts from the clinical evaluation, subsequently passes through the role of electrocardiographic monitoring and multimodality imaging, and finally concludes with genetic evaluation and electro-anatomical mapping. In fact, the sudden cardiac death risk assessment in cardiomyopathies depends on a multiparametric approach. Moreover, current indications for ventricular arrhythmia ablation and defibrillator implantation are discussed.

12.
Biomolecules ; 13(4)2023 04 04.
Article in English | MEDLINE | ID: mdl-37189393

ABSTRACT

Over recent years, preclinical and clinical evidence has implicated myocardial inflammation (M-Infl) in the pathophysiology and phenotypes of traditionally genetic cardiomyopathies. M-Infl resembling myocarditis on imaging and histology occurs frequently as a clinical manifestation of classically genetic cardiac diseases, including dilated and arrhythmogenic cardiomyopathy. The emerging role of M-Infl in disease pathophysiology is leading to the identification of druggable targets for molecular treatment of the inflammatory process and a new paradigm in the field of cardiomyopathies. Cardiomyopathies constitute a leading cause of heart failure and arrhythmic sudden death in the young population. The aim of this review is to present, from bedside to bench, the current state of the art about the genetic basis of M-Infl in nonischemic cardiomyopathies of the dilated and arrhythmogenic spectrum in order to prompt future research towards the identification of novel mechanisms and treatment targets, with the ultimate goal of lowering disease morbidity and mortality.


Subject(s)
Cardiomyopathies , Heart Failure , Myocarditis , Humans , Myocarditis/genetics , Cardiomyopathies/genetics , Cardiomyopathies/pathology , Heart Failure/complications , Arrhythmias, Cardiac/genetics , Inflammation/genetics , Inflammation/complications
13.
Int J Mol Sci ; 23(24)2022 Dec 16.
Article in English | MEDLINE | ID: mdl-36555671

ABSTRACT

Aging is associated with an increasing burden of morbidity, especially for cardiovascular diseases (CVDs). General cardiovascular risk factors, ischemic heart diseases, heart failure, arrhythmias, and cardiomyopathies present a significant prevalence in older people, and are characterized by peculiar clinical manifestations that have distinct features compared with the same conditions in a younger population. Remarkably, the aging heart phenotype in both healthy individuals and patients with CVD reflects modifications at the cellular level. An improvement in the knowledge of the physiological and pathological molecular mechanisms underlying cardiac aging could improve clinical management of older patients and offer new therapeutic targets.


Subject(s)
Cardiomyopathies , Cardiovascular Diseases , Heart Failure , Humans , Heart , Heart Failure/therapy , Cardiomyopathies/epidemiology , Cardiomyopathies/genetics , Cardiovascular Diseases/epidemiology
14.
Cereb Circ Cogn Behav ; 3: 100136, 2022.
Article in English | MEDLINE | ID: mdl-36324405

ABSTRACT

Background: Heart rate variability (HRV), a measure of autonomic function, has been associated with both cardiovascular disease and cognitive dysfunction. In turn, cardiovascular risk has been linked to an increased risk of dementia onset. However, whether autonomic dysfunction may represent an early marker of cognitive decline in individuals with high cardiovascular risk is still an open issue. Methods: We performed a complete 24-hour HRV analysis in 50 middle-aged and elderly subjects with cardiovascular risk as assessed with the European Society of Cardiology Systematic Coronary Risk Evaluation (ESC SCORE). Cognitive performance was evaluated by Montreal Cognitive Assessment (MoCA), Free and Cued Selective Reminding Test (FCSRT) and Stroop Color and Word Test. Stepwise regression was used to identify significant associations between 24-hour ambulatory ECGs parameters and cognitive performances. Results: There were 30 women and 20 men with mean age of 64.9 years (range 51-77) and the mean ESC SCORE was 6%. Four subjects were diagnosed with mild cognitive impairment. Associations were found between measures of HRV and measures of cognition. Ultra-low frequency (ULF) band power of HRV significantly correlated with MoCA (r = 0.424, p = 0.003), also after adjustment for demographics and education. A significant association was also found between the ESC SCORE and ULF band power (r = -0.470, p = 0.0009). Conclusions: Ultra-low frequency band power of HRV is associated with cognitive performance of middle-aged and elderly subjects with cardiovascular risk. This finding may indicate that autonomic nervous system dysregulation plays a role in developing cardiovascular risk and cognitive decline.

15.
Microcirculation ; 29(8): e12790, 2022 11.
Article in English | MEDLINE | ID: mdl-36198058

ABSTRACT

OBJECTIVE: Coronary microvascular dysfunction (CMD) is a key pathophysiological feature of hypertrophic cardiomyopathy (HCM), contributing to myocardial ischemia and representing a critical determinant of patients' adverse outcome. The molecular mechanisms underlying the morphological and functional changes of CMD are still unknown. Aim of this study was to obtain insights on the molecular pathways associated with microvessel remodeling in HCM. METHODS: Interventricular septum myectomies from patients with obstructive HCM (n = 20) and donors' hearts (CTRL, discarded for technical reasons, n = 7) were collected. Remodeled intramyocardial arterioles and cardiomyocytes were microdissected by laser capture and next-generation sequencing was used to delineate the transcriptome profile. RESULTS: We identified 720 exclusive differentially expressed genes (DEGs) in cardiomyocytes and 1315 exclusive DEGs in remodeled arterioles of HCM. Performing gene ontology and pathway enrichment analyses, we identified selectively altered pathways between remodeled arterioles and cardiomyocytes in HCM patients and controls. CONCLUSIONS: We demonstrate the existence of distinctive pathways between remodeled arterioles and cardiomyocytes in HCM patients and controls at the transcriptome level.


Subject(s)
Cardiomyopathy, Hypertrophic , Myocardial Ischemia , Humans , RNA-Seq , Cardiomyopathy, Hypertrophic/genetics , Myocardium/metabolism , Microvessels
16.
Biomedicines ; 10(9)2022 Sep 03.
Article in English | MEDLINE | ID: mdl-36140281

ABSTRACT

Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiomyopathy. The molecular mechanisms determining HCM phenotypes are incompletely understood. Myocardial biopsies were obtained from a group of patients with obstructive HCM (n = 23) selected for surgical myectomy and from 9 unused donor hearts (controls). A subset of tissue-abundant myectomy samples from HCM (n = 10) and controls (n = 6) was submitted to laser-capture microdissection to isolate cardiomyocytes. We investigated the relationship among clinical phenotype, cardiac myosin proteins (MyHC6, MyHC7, and MyHC7b) measured by optimized label-free mass spectrometry, the relative genes (MYH7, MYH7B and MYLC2), and the MyomiR network (myosin-encoded microRNA (miRs) and long-noncoding RNAs (Mhrt)) measured using RNA sequencing and RT-qPCR. MyHC6 was lower in HCM vs. controls, whilst MyHC7, MyHC7b, and MyLC2 were comparable. MYH7, MYH7B, and MYLC2 were higher in HCM whilst MYH6, miR-208a, miR-208b, miR-499 were comparable in HCM and controls. These results are compatible with defective transcription by active genes in HCM. Mhrt and two miR-499-target genes, SOX6 and PTBP3, were upregulated in HCM. The presence of HCM-associated mutations correlated with PTBP3 in myectomies and with SOX6 in cardiomyocytes. Additionally, iPSC-derived cardiomyocytes, transiently transfected with either miR-208a or miR-499, demonstrated a time-dependent relationship between MyomiRs and myosin genes. The transfection end-stage pattern was at least in part similar to findings in HCM myectomies. These data support uncoupling between myosin protein/genes and a modulatory role for the myosin/MyomiR network in the HCM myocardium, possibly contributing to phenotypic diversity and providing putative therapeutic targets.

17.
Life (Basel) ; 12(9)2022 Sep 03.
Article in English | MEDLINE | ID: mdl-36143412

ABSTRACT

BACKGROUND: Anxiety and depressive disorders represent predisposing factors for the autonomic dysfunctions that characterize the acute phase of Takotsubo syndrome (TS). However, there is insufficient data on this relationship after the acute event. The present study aimed at evaluating the psychological and autonomic status of patients with a history of TS. METHODS: Ten TS patients whose acute event occurred at least 1 year prior to the evaluation and nine healthy age- and sex-matched subjects were evaluated. The cardiovascular assessment included a clinical examination, beat-to-beat heart rate monitoring to assess heart rate variability, and a psychological examination using the 16 Personality Factors-C Form (16PF), the Acceptance and Action Questionnaire-II, the Coping Orientations to Problems Experienced (COPE), the Beck Depression Inventory-II, and the State-Trait Anxiety Inventory (STAI). RESULTS: TS patients scored significantly higher on the STAI (i.e., Anxiety Trait), 16PF (i.e., Tension), and COPE (i.e., Transcendental Orientation). TS patients also showed lower heart rate variability. Moreover, a significant inverse correlation was found between sympathetic tone (LF/HF ratio) and coping orientation. CONCLUSIONS: Long after the acute event, TS patients are characterized by elevated anxiety, high tension, and a specific religious coping strategy.

18.
Clin Neuropsychiatry ; 19(3): 163-173, 2022 Jun.
Article in English | MEDLINE | ID: mdl-35821868

ABSTRACT

Objective: Psychopathology (and depression in particular) is a cardiovascular risk factor independent from any co-occurring pathology. This link is traced back to the mind-heart-body connection, whose underlying mechanisms are still not completely known. To study psychopathology in relation to the heart, it is necessary to observe the autonomic nervous system, which mediates among the parts of that connection. Its gold standard of evaluation is the study of heart rate variability (HRV). To investigate whether any association exists between the HRV parameters and sub-threshold depressive symptoms in a sample of healthy subjects. Method: In this cross-sectional study, two short-term HRV recordings (5 min - supine and sitting) have been analyzed in 77 healthy subjects. Here we adopted a three-fold approach to evaluate HRV: a set of scores belonging to the time domain; to the frequency domain (high, low, and very low frequencies) and a set of 'nonlinear' parameters. The PHQ-9 (Patient Health Questionnaire-9) scale was used to detect depressive symptoms. Results: Depressive symptoms were associated only with a parameter from the non-linear approach and specifically the long-term fluctuations of fractal dimensions (DFA-α2). This association remained significant even after controlling for age, gender, BMI (Body-Mass-Index), arterial hypertension, anti-hypertensive drugs, dyslipidemia, and smoking habits. Moreover, the DFA-α2 was not affected by the baroreflex (postural change), unlike other autonomic markers. Conclusions: Fractal analysis of HRV (DFA-α2) allows then to predict depressive symptoms below the diagnostic threshold in healthy subjects regardless of their health status. DFA-α2 may be considered as an imprint of subclinical depression on the heart rhythm.

20.
Eur Heart J Cardiovasc Imaging ; 23(2): 200-208, 2022 01 24.
Article in English | MEDLINE | ID: mdl-33486507

ABSTRACT

AIMS: Fabry cardiomyopathy is characterized by glycosphingolipid storage and increased myocardial trabeculation has also been demonstrated. This study aimed to explore by cardiac magnetic resonance whether myocardial trabecular complexity, quantified by endocardial border fractal analysis, tracks phenotype evolution in Fabry cardiomyopathy. METHODS AND RESULTS: Study population included 20 healthy controls (12 males, age 32±9) and 45 Fabry patients divided into three groups: 15 left ventricular hypertrophy (LVH)-negative patients with normal T1 (5 males, age 28±13; Group 1); 15 LVH-negative patients with low T1 (9 males, age 33±9.6; Group 2); 15 LVH-positive patients (11 males, age 53.5±9.6; Group 3). Trabecular fractal dimensions (Dfs) (total, basal, mid-ventricular, and apical) were evaluated on cine images. Total Df was higher in all Fabry groups compared to controls, gradually increasing from controls to Group 3 (1.27±0.02 controls vs. 1.29±0.02 Group 1 vs. 1.30±0.02 Group 2 vs. 1.34±0.02 Group 3; P<0.001). Group 3 showed significantly higher values of all Dfs compared to the other Groups. Both basal and total Dfs were significantly higher in Group 1 compared with controls (basal: 1.30±0.03 vs. 1.26±0.04, P =0.010; total: 1.29±0.02 vs. 1.27±0.02, P=0.044). Total Df showed significant correlations with: (i) T1 value (r=-0.569; P<0.001); (ii) LV mass (r=0.664, P<0.001); (iii) trabecular mass (r=0.676; P <0.001); (iv) Mainz Severity Score Index (r=0.638; P<0.001). CONCLUSION: Fabry cardiomyopathy is characterized by a progressive increase in Df of endocardial trabeculae together with shortening of T1 values. Myocardial trabeculation is increased before the presence of detectable sphingolipid storage, thus representing an early sign of cardiac involvement.


Subject(s)
Cardiomyopathies , Fabry Disease , Fabry Disease/complications , Fabry Disease/diagnostic imaging , Humans , Hypertrophy, Left Ventricular/diagnostic imaging , Hypertrophy, Left Ventricular/etiology , Male , Prospective Studies , Ventricular Function, Left
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