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1.
Eur Radiol ; 33(7): 4621-4636, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-36692598

RESUMO

OBJECTIVES: The prognostic role of left ventricular (LV) papillary muscle abnormalities in patients with preserved LV systolic ejection fraction (LVEF) is unknown. We sought to evaluate the prognosis role of LV papillary muscle abnormalities by CMR in patients with ventricular arrhythmias, preserved LVEF with no cardiac disease. METHODS: A total of 391 patients with > 500/24 h premature ventricular complexes and/or with non-sustained ventricular tachycardia (NSVT), preserved LVEF, and no cardiac disease were enrolled. Different features of LV papillary muscles were considered: supernumerary muscles, papillary thickness, the attachment, late gadolinium enhancement (LGE). Dark-Paps was defined as end-systolic signal hypointensity of both papillary muscles in early post-contrast cine CMR images. Mitral valve prolapse, mitral annular disjunction (MAD), and myocardial LGE were considered. RESULTS: Dark-Paps was found in 79 (20%) patients and was more frequent in females. It was associated with higher prevalence of mitral valve prolapse and MAD. During a median follow-up of 2534 days, 22 hard cardiac events occurred. At Kaplan-Meier curve analysis, patients with Dark-Paps were at higher risk of events than those without (p < 0.0001). Dark-Paps was significantly associated with hard cardiac events in all the multivariate models. Dark-Paps improved prognostic estimation when added to NSVT (p = 0.0006), to LGE (p = 0.005) and to a model including NSVT+LGE (p = 0.014). Dark-Paps allowed a significant net reclassification when added to NSVT (NRI 0.30, p = 0.03), to LGE (NRI 0.25, p = 0.04), and to NSVT + LGE (NRI 0.32, p  = 0.02). CONCLUSIONS: In LV papillary muscles, Dark-Paps is a novel prognostic marker in patients with ventricular arrhythmias and preserved ejection fraction. KEY POINTS: • Papillary muscle abnormalities are seen in patients with ventricular arrhythmias and preserved left ventricular ejection fraction. • Early post-contrast hypointensity of papillary muscles in end-systolic cine images (Dark-Paps) is a novel prognostic marker in patients with ventricular arrhythmias and preserved ejection fraction. • Dark-Paps had an additive prognostic role over late gadolinium enhancement and non-sustained ventricular tachycardia.


Assuntos
Cardiopatias , Prolapso da Valva Mitral , Taquicardia Ventricular , Feminino , Humanos , Músculos Papilares/diagnóstico por imagem , Músculos Papilares/patologia , Meios de Contraste/farmacologia , Volume Sistólico , Prolapso da Valva Mitral/diagnóstico por imagem , Prognóstico , Função Ventricular Esquerda , Gadolínio/farmacologia , Imagem Cinética por Ressonância Magnética/métodos , Arritmias Cardíacas , Taquicardia Ventricular/diagnóstico por imagem , Espectroscopia de Ressonância Magnética , Valor Preditivo dos Testes
2.
Echocardiography ; 40(3): 217-226, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36748264

RESUMO

BACKGROUND: Early diagnosis of Coronary Artery Disease (CAD) plays a key role to prevent adverse cardiac events such as myocardial infarction and Left Ventricular (LV) dysfunction. Myocardial Work (MW) indices derived from echocardiographic speckle tracking data in combination with non-invasive blood pressure recordings seems promising to predict CAD even in the absence of impairments of standard echocardiographic parameters. Our aim was to compare the diagnostic accuracy of MW indices to predict CAD and to assess intra- and inter-observer variability of MW through a meta-analysis. METHODS: Electronic databases were searched for observational studies evaluating the MW indices diagnostic accuracy for predicting CAD and intra- and inter-observer variability of MW indices. Pooled sensitivity, specificity, and Summary Receiver Operating Characteristic (SROC) curves were assessed. RESULTS: Five studies enrolling 501 patients met inclusion criteria. Global Constructive Work (GCW) had the best pooled sensitivity (89%) followed by GLS (84%), Global Work Index (GWI) (82%), Global Work Efficiency (GWE) (80%), and Global Wasted Work (GWW) (75%). GWE had the best pooled specificity (78%) followed by GWI (75%), GCW (70%), GLS (68%), and GWW (61%). GCW had the best accuracy according to SROC curves, with an area under the curve of 0.86 compared to 0.84 for GWI, 0.83 for GWE, 0.79 for GLS, and 0.74 for GWW. All MW indices had an excellent intra- and inter-observer variability. CONCLUSIONS: GCW is the best MW index proving best diagnostic accuracy in the prediction of CAD with an excellent reproducibility.


Assuntos
Doença da Artéria Coronariana , Disfunção Ventricular Esquerda , Humanos , Reprodutibilidade dos Testes , Ecocardiografia , Miocárdio , Função Ventricular Esquerda , Volume Sistólico
3.
Sensors (Basel) ; 23(6)2023 Mar 16.
Artigo em Inglês | MEDLINE | ID: mdl-36991870

RESUMO

A diagnosis of Brugada syndrome (BrS) is based on the presence of a type 1 electrocardiogram (ECG) pattern, either spontaneously or after a Sodium Channel Blocker Provocation Test (SCBPT). Several ECG criteria have been evaluated as predictors of a positive SCBPT, such as the ß-angle, the α-angle, the duration of the base of the triangle at 5 mm from the r'-wave (DBT- 5 mm), the duration of the base of the triangle at the isoelectric line (DBT- iso), and the triangle base/height ratio. The aim of our study was to test all previously proposed ECG criteria in a large cohort study and to evaluate an r'-wave algorithm for predicting a BrS diagnosis after an SCBPT. We enrolled all patients who consecutively underwent SCBPT using flecainide from January 2010 to December 2015 in the test cohort and from January 2016 to December 2021 in the validation cohort. We included the ECG criteria with the best diagnostic accuracy in relation to the test cohort in the development of the r'-wave algorithm (ß-angle, α-angle, DBT- 5 mm, and DBT- iso.) Of the total of 395 patients enrolled, 72.4% were male and the average age was 44.7 ± 13.5 years. Following the SCBPTs, 24.1% of patients (n = 95) were positive and 75.9% (n = 300) were negative. ROC analysis of the validation cohort showed that the AUC of the r'-wave algorithm (AUC: 0.92; CI 0.85-0.99) was significantly better than the AUC of the ß-angle (AUC: 0.82; 95% CI 0.71-0.92), the α-angle (AUC: 0.77; 95% CI 0.66-0.90), the DBT- 5 mm (AUC: 0.75; 95% CI 0.64-0.87), the DBT- iso (AUC: 0.79; 95% CI 0.67-0.91), and the triangle base/height (AUC: 0.61; 95% CI 0.48-0.75) (p < 0.001), making it the best predictor of a BrS diagnosis after an SCBPT. The r'-wave algorithm with a cut-off value of ≥2 showed a sensitivity of 90% and a specificity of 83%. In our study, the r'-wave algorithm was proved to have the best diagnostic accuracy, compared with single electrocardiographic criteria, in predicting the diagnosis of BrS after provocative testing with flecainide.


Assuntos
Síndrome de Brugada , Humanos , Masculino , Adulto , Pessoa de Meia-Idade , Feminino , Síndrome de Brugada/diagnóstico , Bloqueadores dos Canais de Sódio/farmacologia , Bloqueadores dos Canais de Sódio/uso terapêutico , Flecainida , Estudos de Coortes , Eletrocardiografia , Algoritmos
4.
Int J Mol Sci ; 24(24)2023 Dec 16.
Artigo em Inglês | MEDLINE | ID: mdl-38139379

RESUMO

Several studies have demonstrated that, beyond their antithrombotic effects, P2Y12 receptor inhibitors may provide additional off-target effects through different mechanisms. These effects range from the preservation of endothelial barrier function to the modulation of inflammation or stabilization of atherosclerotic plaques, with an impact on different cell types, including endothelial and immune cells. Many P2Y12 inhibitors have been developed, from ticlopidine, the first thienopyridine, to the more potent non-thienopyridine derivatives such as ticagrelor which may promote cardioprotective effects following myocardial infarction (MI) by inhibiting adenosine reuptake through sodium-independent equilibrative nucleoside transporter 1 (ENT1). Adenosine may affect different molecular pathways involved in cardiac fibrosis, such as the Wnt (wingless-type)/beta (ß)-catenin signaling. An early pro-fibrotic response of the epicardium and activation of cardiac fibroblasts with the involvement of Wnt1 (wingless-type family member 1)/ß-catenin, are critically required for preserving cardiac function after acute ischemic cardiac injury. This review discusses molecular signaling pathways involved in cardiac fibrosis post MI, focusing on the Wnt/ß-catenin pathway, and the off-target effect of P2Y12 receptor inhibition. A potential role of ticagrelor was speculated in the early modulation of cardiac fibrosis, thanks to its off-target effect.


Assuntos
Infarto do Miocárdio , Antagonistas do Receptor Purinérgico P2Y , Humanos , Ticagrelor/farmacologia , Antagonistas do Receptor Purinérgico P2Y/farmacologia , Antagonistas do Receptor Purinérgico P2Y/uso terapêutico , beta Catenina , Infarto do Miocárdio/metabolismo , Adenosina , Pericárdio/metabolismo , Fibrose
5.
Eur J Clin Invest ; 52(10): e13815, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35598175

RESUMO

BACKGROUND: Prognostic stratification of acute myocarditis (AM) presenting with normal left ventricular ejection fraction (LVEF) relies mostly on late gadolinium enhancement (LGE) characterization. Left ventricular peak global longitudinal strain (LV-GLS) measured by feature tracking analysis might improve prognostication of AM presenting with normal LVEF. METHODS: Data of patients undergoing cardiac magnetic resonance (CMR) for clinically suspected AM in seven European Centres (2013-2020) were retrospectively analysed. Patients with AM confirmed by CMR and LVEF ≥50% were included. LGE was visually characterized: localized versus. non-localized, subepicardial versus midwall. LV-GLS was measured by dedicated software. The primary outcome was the first occurrence of an adverse cardiovascular event (ACE) including cardiac death, life-threatening arrhythmias, development of heart failure or of LVEF <50%. RESULTS: Of 389 screened patients, 256 (66%) fulfilled inclusion criteria: median age 36 years, 71% males, median LVEF 60%, median LV-GLS -17.3%. CMR was performed at 4 days from hospitalization. At 27 months, 24 (9%) patients experienced ≥1 ACE (71% developed LVEF <50%). Compared to the others, they had lower median LV-GLS values (-13.9% vs. -17.5%, p = .001). At Kaplan-Meier analysis, impaired LV-GLS (both considered as > -20% or quartiles), non-localized and midwall LGE were associated with ACEs. Patients with LV-GLS ≤-20% did not experience ACEs. LV-GLS remained associated with ACEs after adjustment for non-localized and midwall LGE. CONCLUSION: In AM presenting with LVEF ≥50%, LV-GLS provides independent prognostic value over LGE characterization, improving risk stratification and representing a rationale for further studies of therapy in this cohort.


Assuntos
Miocardite , Função Ventricular Esquerda , Adulto , Meios de Contraste , Feminino , Gadolínio , Humanos , Imagem Cinética por Ressonância Magnética , Espectroscopia de Ressonância Magnética , Masculino , Miocardite/diagnóstico por imagem , Valor Preditivo dos Testes , Prognóstico , Estudos Retrospectivos , Volume Sistólico
6.
Eur Radiol ; 32(5): 3035-3044, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-35031838

RESUMO

OBJECTIVES: Although expert consensus recommendations suggest 2-3 h as the time interval between bone-seeking radiotracers injection and acquisition, it has been reported that images obtained early after [99mTc]Tc-HMDP administration are sufficient to diagnose cardiac amyloidosis. We evaluated the diagnostic performance of [99mTc]Tc-DPD early phase whole body scan with respect to late phase imaging. METHODS: We qualitatively and semiquantitatively reviewed [99mTc]Tc-DPD imaging of 53 patients referred for suspect cardiac amyloidosis. Findings of early and late phase images were compared with SPECT results (considered the standard-of-reference) determining sensitivity and specificity for visual analysis of each phase imaging and for each semiquantitative index. RESULTS: SPECT imaging was negative for cardiac accumulation in 25 patients and positive in 28. Visual analysis of early phase whole body scan had an extremely significant capability to predict SPECT results; nevertheless, complete agreement was not reached. Visual analysis of late phase imaging showed slightly better results. Semiquantitative analysis of early phase images, namely heart to mediastinum ratio, performed better than semiquantitative analysis of late phase images. CONCLUSION: Visual analysis of [99mTc]Tc-DPD early phase whole body scan is promising in diagnosing cardiac amyloidosis; further studies are needed to confirm our results in different clinical scenarios. KEY POINTS: • Visual analysis of early phase planar imaging using [99mTc]Tc-DPD is accurate to diagnose cardiac amyloidosis and may be satisfactory at least in frail patients with high cardiac burden of amyloid fibrils.


Assuntos
Amiloidose , Cardiomiopatias , Amiloide , Amiloidose/diagnóstico por imagem , Cardiomiopatias/diagnóstico por imagem , Humanos , Pré-Albumina , Cintilografia , Compostos Radiofarmacêuticos/farmacologia , Imagem Corporal Total
7.
Echocardiography ; 39(2): 294-301, 2022 02.
Artigo em Inglês | MEDLINE | ID: mdl-35066914

RESUMO

BACKGROUND: Myocarditis have variable clinical presentation, evolution and prognosis. Aim of our study was to evaluate the value of speckle tracking echocardiography and cardiac magnetic resonance (CMR) in the short-term prediction of supraventricular arrhythmias (SVA) in patients with acute myocarditis. METHODS: Seventy patients (mean age 31±14 years old) with acute myocarditis and preserved left ventricular ejection fraction (LVEF) were enrolled. Longitudinal systolic strain (LS) of the left ventricle (LV), mechanical dispersion (MD) and CMR with quantitative measurement of delayed enhancement (DE) were performed in a subset of 43 patients. Logistic regression and ROC analysis were used to identify predictors of SVA RESULTS: Only LS measured at sup-epicardial, mid-wall and sub-endocardial level of the apical 4-chamber view was significantly lower in patients with SVA, while MD was marginally prolonged in this setting. A value of LS > - 16.1% measured at LV mid-wall in the apical 4-chamber view (ROC-AUC .75, Sensitivity 63%, Specificity 85%) was the most accurate measure to identify patients with SVA. DE mass was also helpful with a ROC-AUC .76; a DE-Mass > 18.9 gr. had a Sensitivity 63% and a Specificity 77%, to identify patients at risk of SVA. CONCLUSIONS: Both DE mass and LS were associated with higher risk of SVA in patients with acute myocarditis and preserved LVEF. However, LS measured at the mid-wall level and limited to LV segments included in the apical 4-chamber view was the most accurate measure and did not show interaction with DE mass.


Assuntos
Miocardite , Disfunção Ventricular Esquerda , Adolescente , Adulto , Arritmias Cardíacas/complicações , Humanos , Imagem Cinética por Ressonância Magnética , Espectroscopia de Ressonância Magnética , Pessoa de Meia-Idade , Miocardite/complicações , Miocardite/diagnóstico por imagem , Volume Sistólico , Disfunção Ventricular Esquerda/complicações , Disfunção Ventricular Esquerda/diagnóstico por imagem , Função Ventricular Esquerda , Adulto Jovem
8.
J Electrocardiol ; 73: 52-54, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35660341

RESUMO

Atrial fibrillation (AF) is the most common cardiac arrhythmia in adults with substantial morbidity and mortality. The diagnosis of AF is established by electrocardiogram showing heart rhythm without clear P waves with irregular RR intervals. The electrocardiographic diagnosis of AF is not always easy and can still hide pitfalls even for the most experienced cardiologist. We present the case of an 86 years old patient admitted to our hospital for Covid-19 infection affected by dilated cardiomyopathy with an electrocardiogram showing a non-specific intraventricular conduction delay mimicking sinus rhythm in the presence of AF.


Assuntos
Fibrilação Atrial , COVID-19 , Adulto , Idoso de 80 Anos ou mais , Fibrilação Atrial/diagnóstico , Eletrocardiografia , Bloqueio Cardíaco , Ventrículos do Coração , Humanos
9.
J Electrocardiol ; 74: 46-53, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35964522

RESUMO

INTRODUCTION: Atrial fibrillation (AF) is the main cardiac cause of stroke, but it frequently remains undetected. In patients with cryptogenic stroke an Holter electrocardiogram (ECG) monitoring for AF is recommended. OBJECTIVE: To evaluate the prognostic role of Non-Conducted Premature Atrial Complexes (ncPACs) recorded on Holter ECG. METHODS: We prospectively enrolled consecutive patients admitted to the Stroke Unit of our hospital with a diagnosis of cryptogenic stroke between December 2018 and January 2020; all patients underwent 24-h Holter ECG monitoring during hospitalization. Two follow-up visits were scheduled, including a 24-h Holter ECG at 3 and 6 months to detect AF. RESULTS: Among 112 patients, 58% were male with an average age of 72.2 ± 12.2 years. At follow-up, AF was diagnosed in 21.4% of the population. The baseline 24-h Holter ECG burden of ncPACs and Premature Atrial Complexes (PACs) was higher in patients with AF detected on follow-up (13.5 vs 2, p = 0.001; 221.5 vs 52; p = 0.01). ROC analysis showed that ncPACs had the best diagnostic accuracy in predicting AF (AUC:0.80; 95% CI 0.68-0.92). Cut-off value of ≥7 for ncPACs burden showed the highest accuracy with sensitivity of 62.5% and specificity 97.7% to predict AF onset at follow-up. Moreover, at multivariate Cox-proportional hazard analysis ncPACs burden ≥7 was a powerful independent predictor of AF onset (HR 12.4; 95% CI 4.8-32.8; p < 0.0001). CONCLUSIONS: NcPACs burden ≥7 represents a new predictor of AF that could guide the screening of this arrhythmia in cryptogenic stroke patients.


Assuntos
Fibrilação Atrial , AVC Isquêmico , Acidente Vascular Cerebral , Humanos , Masculino , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Feminino , Fibrilação Atrial/complicações , Fibrilação Atrial/diagnóstico , Eletrocardiografia , Acidente Vascular Cerebral/etiologia
10.
Eur J Clin Invest ; 51(11): e13598, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33982288

RESUMO

BACKGROUND: Amyloid transthyretin (ATTR) amyloidosis is caused by the systemic deposition of transthyretin molecules, either normal (wild-type ATTR, ATTRwt) or mutated (variant ATTR, ATTRv). ATTR amyloidosis is a disease with a severe impact on patients' quality of life (QoL). Nonetheless, limited attention has been paid to QoL so far, and no specific tools for QoL assessment in ATTR amyloidosis currently exist. QoL can be evaluated through patient-reported outcome measures (PROMs), which are completed by patients, or through scales, which are compiled by clinicians. The scales investigate QoL either directly or indirectly, i.e., by assessing the degree of functional impairment and limitations imposed by the disease. DESIGN: Search for the measures of QoL evaluated in phase 2 and phase 3 clinical trials on ATTR amyloidosis. RESULTS: Clinical trials on ATTR amyloidosis have used measures of general health status, such as the Short Form 36 Health Survey (SF-36), or tools developed in other disease settings such as the Kansas City Cardiomyopathy Questionnaire (KCCQ) or adaptations of other scales such as the modified Neuropathy Impairment Score +7 (mNIS+7). CONCLUSIONS: Scales or PROMs for ATTR amyloidosis would be useful to better characterize newly diagnosed patients and to assess disease progression and response to treatment. The ongoing ITALY (Impact of Transthyretin Amyloidosis on Life qualitY) study aims to develop and validate 2 PROMs encompassing the whole phenotypic spectrum of ATTRwt and ATTRv amyloidosis, that might be helpful for patient management and may serve as surrogate endpoints for clinical trials.


Assuntos
Neuropatias Amiloides Familiares/fisiopatologia , Neuropatias Amiloides/fisiopatologia , Cardiomiopatias/fisiopatologia , Qualidade de Vida , Humanos , Medidas de Resultados Relatados pelo Paciente
11.
J Cardiovasc Magn Reson ; 23(1): 121, 2021 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-34719402

RESUMO

BACKGROUND: Cardiovascular magnetic resonance permits assessment of irreversible myocardial fibrosis and contractile function in patients with previous myocardial infarction. We aimed to assess the prognostic value of myocardial fibrotic tissue with preserved/restored contractile activity. METHODS: In 730 consecutive myocardial infarction patients (64 ± 11 years), we quantified left ventricular (LV) end-diastolic (EDV) and end-systolic (ESV) volumes, ejection fraction (EF), regional wall motion (WM) (1 normal, 2 hypokinetic, 3 akinetic, 4 dyskinetic), and WM score index (WMSI), and measured the transmural (1-50 and 51-100) and global extent of the infarct scar by late gadolinium enhancement (LGE). Contractile fibrotic (CT-F) segments were identified as those showing WM-1 and WM-2 with LGE ≤ or ≥ 50%. RESULTS: During follow-up (median 2.5, range 1-4.7 years), cardiac events (cardiac death or appropriate implantable defibrillator shocks) occurred in 123 patients (17%). At univariate analysis, age, LVEDV, LVESV, LVEF, WMSI, extent of LGE, segments with transmural extent > 50%, and CT-F segments were associated with cardiac events. At multivariate analysis, age > 65 years, LVEF < 30%, WMSI > 1.7, and dilated LVEDV independently predicted cardiac events, while CT-F tissue was the only independent predictor of better outcome. After adjustment for LVEF < 30% and LVEDV dilatation, the presence of CT-F tissue was associated with good prognosis. CONCLUSIONS: In addition to CMR imaging parameters associated with adverse outcome (severe LV dysfunction, poor WM, and dilated EDV), the presence of fibrotic myocardium showing contractile activity in patients with previous myocardial infarction yields a beneficial effect on patient survival.


Assuntos
Meios de Contraste , Infarto do Miocárdio , Idoso , Gadolínio , Humanos , Infarto do Miocárdio/diagnóstico por imagem , Miocárdio , Valor Preditivo dos Testes
12.
Heart Fail Clin ; 17(2): 179-186, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33673943

RESUMO

Stage A and B heart failure (HF) include asymptomatic patients without and with structural cardiac disorder, respectively. Asymptomatic left ventricular (LV) dysfunction represents an early stage of HF that should be recognized to prevent overt HF development. Echocardiography plays a pivotal role in assessment of cardiac structure and function and represents the ideal imaging technique for screening in the general population, thanks to its availability, feasibility, and low cost. Traditional echocardiography, with LV systolic and diastolic function and cardiac remodeling assessment, is usually performed. Development of new technologies may offer additional information and insights in detection of early LV dysfunction.


Assuntos
Doenças Assintomáticas , Ecocardiografia/métodos , Programas de Rastreamento/métodos , Saúde Pública , Disfunção Ventricular Esquerda/diagnóstico , Humanos , Disfunção Ventricular Esquerda/fisiopatologia
13.
J Peripher Nerv Syst ; 25(3): 273-278, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32395865

RESUMO

V122I is one of more than 130 mutations in transthyretin gene associated with hereditary TTR (ATTRv) amyloidosis. Main clinical expression is an infiltrative pseudohypertrophic cardiomyopathy with mild or no neurological symptoms. It is particularly common among African-Americans (prevalence: 3%-4%). We report 12 subjects from seven unrelated Caucasian families hailing from Sicily and carrying the V122I mutation. One patient was homozygous for V122I and in another family two subjects also carried the E89Q variant in compound heterozygosity. All the subjects underwent neurologic/neurophysiologic evaluation and cardiologic baseline tests; in five of them, cardiac magnetic resonance and/or (99 m) Tc-DPD scintigraphy were performed. Three of 12 subjects were asymptomatic carriers. Of the remaining nine subjects, in four of nine patients, the nerve conduction studies revealed a polyneuropathy; in one of them, this represents the only sign of disease after 5 years of follow-up. In eight of nine subjects, we found a hypertrophic restrictive cardiomyopathy and cardiac failure, associated with a carpal tunnel syndrome. Although in non-Afro-American individuals V122I prevalence is low, subjects carrying this mutation have been identified in the United Kingdom, Italy, and France. Our report describes a large cohort of V122I Caucasian patients from a non-endemic area, confirming the possible underestimation of this mutation in the non-African population. Moreover, it highlights the heterogeneity in the genotype-phenotype correlation of ATTRv mutations, suggesting that the presence of a polyneuropathy has to be identified as soon as possible, since available treatments are, in Europe, so far authorized only for ATTRv amyloid peripheral neuropathy.


Assuntos
Neuropatias Amiloides Familiares , Cardiopatias , Polineuropatias , Pré-Albumina/genética , População Branca , Adulto , Idoso , Neuropatias Amiloides Familiares/complicações , Neuropatias Amiloides Familiares/diagnóstico , Neuropatias Amiloides Familiares/etnologia , Neuropatias Amiloides Familiares/genética , Seguimentos , Cardiopatias/diagnóstico , Cardiopatias/etnologia , Cardiopatias/etiologia , Cardiopatias/genética , Humanos , Masculino , Pessoa de Meia-Idade , Polineuropatias/diagnóstico , Polineuropatias/etnologia , Polineuropatias/etiologia , Polineuropatias/genética , Sicília/etnologia , População Branca/etnologia , População Branca/genética
14.
Heart Vessels ; 35(5): 637-646, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-31712910

RESUMO

We sought to recognize the blood flow velocity (BFV) through the left anterior descending (LAD) coronary artery and its small intramyocardial (IM) branches by transthoracic Doppler-echocardiography in patients with aortic stenosis (AS). Sixty-two patients, aged 74.0 ± 9.6 years, 37 women, with preserved left ventricular (LV) function, apparently free of active ischemic disease, were enrolled and classified into 3 groups according to the mean gradient (MG) across the aortic valve: 13 patients (21%) entered the group A (MG ≤ 20 mmHg), 29 (48%) group B (MG 21-40 mmHg) and 20 (31%) group C (MG > 40 mmHg). Peak and mean coronary BFVs were demonstrated to gradually increase according to AV gradient, especially through the IM arteries. Peak IM-BFV was 58.9 cm/s (95% CI 46.4-71.4) in group A, 73.2 cm/s (95% CI 64.8-81.6) in group B, and 96.4 cm/s (95% CI 86.3-106.5) in group C (p < 0.001), whereas peak LAD-BFV was 38.1 cm/s (95% CI 32.8-43.3), 44.4 cm/s (95% CI 40.9-47.9) and 47.3 cm/s (95% CI 43.1-52.5), respectively (p = 0.03). Also, 34 patients complaining with unspecific symptoms showed much higher IM-BFV than those who were not. High values were also recognized in patients with LV ejection fraction/velocity ratio (EFVR) ≤ 0.90 (IM-BFV 91 ± 26 cm/s vs. 72 ± 24 cm/s in those with EFVR > 0.90, p = 0.001). In conclusion, AS patients in the present study showed gradually higher coronary BFVs according to AS gradient, especially through the IM vessels, and both peak and mean velocities were discriminating specific patient subsets. Pathophysiological mechanisms and potential clinical implications are discussed.


Assuntos
Estenose da Valva Aórtica/diagnóstico por imagem , Circulação Coronária , Vasos Coronários/diagnóstico por imagem , Ecocardiografia Doppler em Cores , Idoso , Idoso de 80 Anos ou mais , Estenose da Valva Aórtica/fisiopatologia , Velocidade do Fluxo Sanguíneo , Vasos Coronários/fisiopatologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Valor Preditivo dos Testes , Prognóstico , Índice de Gravidade de Doença , Volume Sistólico , Função Ventricular Esquerda
15.
Heart Fail Rev ; 24(1): 81-90, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30033478

RESUMO

Acute myocarditis (AM) is commonly found in everyday clinical practice. Differential diagnosis between various causes of myocardial damage with non-obstructive coronary arteries can be cumbersome for clinician. Moreover, AM may be provoked by a number of different causes and clinical presentation can be heterogeneous with potential overlap going from asymptomatic or subclinical to severe heart failure, arrhythmias, and death. Cardiac magnetic resonance (CMR) over the last decades has proven to be the diagnostic technique of choice since it allows identifying AM with excellent diagnostic accuracy. Latest technological advancement with parametric imaging such as T1 and T2 mapping further increases sensitivity and provides additional help towards a correct diagnosis. CMR however is no longer to be considered as a mere diagnostic tool but also as a powerful source of prognostic information. Scientific evidence has corroborated CMR's role beyond diagnosis demonstrating how late gadolinium enhancement (LGE) presence is a powerful predictor of cardiac events and how the presence of septal LGE is to be considered of worst prognosis regardless of LGE extension even in patients with preserved global systolic function. CMR should be routinely performed in all patients with AM suspicion since its diagnostic and prognostic role is of paramount important and could modify therapeutic strategy and subsequent clinical decisions.


Assuntos
Imagem Cinética por Ressonância Magnética/métodos , Miocardite/diagnóstico por imagem , Doença Aguda , Tomada de Decisão Clínica/métodos , Meios de Contraste/metabolismo , Confiabilidade dos Dados , Diagnóstico Diferencial , Feminino , Gadolínio/metabolismo , Humanos , Masculino , Prognóstico , Septo do Cérebro/metabolismo
16.
Eur Radiol ; 29(3): 1555-1564, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30128617

RESUMO

OBJECTIVES: We sought to evaluate the role of cardiac magnetic resonance imaging (CMR) in the evaluation of diastolic function by a combined assessment of left ventricular (LV) and left atrial (LA) function in a cohort of subjects with various degrees of diastolic dysfunction (DD) detected by echocardiography. METHODS: Forty patients with different stages of DD and 18 healthy controls underwent CMR. Short-axis cine steady-state free precession images covering the entire LA and LV were acquired. Parameters of diastolic function were measured by the analysis of the LV and LA volume/time (V/t) curves and the respective derivative dV/dt curves. RESULTS: At receiver operating characteristic (ROC) curve analysis, the peak of emptying rate A indexed by the LV filling volume with a cut-off of 3.8 was able to detect patients with grade I DD from other groups (area under the curve [AUC] 0.975, 95% confidence interval [CI] 0.86-1). ROC analysis showed that LA ejection fraction with a cut-off of ≤36% was able to distinguish controls and grade I DD patients from those with grade II and grade III DD (AUC 0.996, 95% CI 0.92-1, p < 0.001). The isovolumetric pulmonary vein transit ratio with a cut-off of 2.4 allowed class III DD to be distinguished from other groups (AUC 1.0, 95%CI 0.93-1, p < 0.001). CONCLUSIONS: Analysis of LV and LA V/t curves by CMR may be useful for the evaluation of DD. KEY POINTS: • Combined atrial and ventricular volume/time curves allow evaluation of diastolic function. • Atrial emptying fraction allows distinction between impaired relaxation and restrictive/pseudo-normal filling. • Isovolumetric pulmonary vein transit ratio allows distinction between restrictive and pseudo-normal filling.


Assuntos
Função do Átrio Esquerdo , Diástole , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/fisiopatologia , Imageamento por Ressonância Magnética , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/fisiopatologia , Adulto , Idoso , Área Sob a Curva , Cardiomiopatia Hipertrófica/fisiopatologia , Ecocardiografia , Feminino , Insuficiência Cardíaca/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Veias Pulmonares/fisiologia , Curva ROC
17.
Curr Cardiol Rep ; 21(2): 7, 2019 02 12.
Artigo em Inglês | MEDLINE | ID: mdl-30747298

RESUMO

PURPOSE OF REVIEW: The aim is to provide a description of the most important echocardiographic features in systemic amyloidosis. RECENT FINDINGS: Amyloidosis is a heterogeneous group of multisystem disorders, characterized by an extracellular deposition of amyloid fibrils. Several imaging tests are available for the diagnosis; however, echocardiography is the cornerstone of the non-invasive imaging modality for cardiac amyloidosis. So far, little is known about the diagnosis of cardiac amyloidosis through imaging modalities. We summarized the most important echocardiographic findings in cardiac amyloidosis. Hence, we offered a systematic report of the diagnostic performance of cardiac amyloidosis using echocardiography.


Assuntos
Amiloidose/diagnóstico por imagem , Cardiomiopatias/diagnóstico por imagem , Ecocardiografia Doppler/métodos , Ultrassonografia Doppler/métodos , Amiloide , Humanos
18.
Echocardiography ; 35(12): 1966-1973, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30315606

RESUMO

BACKGROUND: The aim of present study was to assess left ventricular (LV) myocardial deformation and changes over time in patients with acute myocarditis (AM) with preserved ejection fraction detected by late gadolinium enhancement (LGE) magnetic resonance imaging. METHODS: Thirty-five male patients with AM diagnoses and preserved systolic function based on cardiac magnetic resonance imaging (MRI) were prospectively enrolled. On admission, echocardiography with measurements of global and segmental longitudinal (LS) strains was performed both at the endocardial (ENDO) and epicardial (EPI) levels. Findings were compared to 25 control subjects. Twenty-six patients were also monitored over a 22-month follow-up (FU group). RESULTS: On admission, global ENDO-LS was poorer in magnitude in AM (-19.2 ± 3.1) than in controls (-24.0 ± 1.05) (P < 0.0001), whereas EPI-LS was not different (-20.6 ± 3.4 vs -19.7 ± 6 P = NS). A functional increase in magnitude in both ENDO-LS (-20.8 ± 5.4, P = NS) and EPI-LS (-22.6 ± 4.6, P = 0.02) was found in FU vs AM patients. CONCLUSIONS: The present study demonstrates a steady ENDO-LS impairment in infarct-like AM during a 2-year follow-up period, despite a preserved LV ejection fraction.


Assuntos
Endocárdio/diagnóstico por imagem , Ventrículos do Coração/diagnóstico por imagem , Miocardite/complicações , Volume Sistólico/fisiologia , Disfunção Ventricular Esquerda/fisiopatologia , Função Ventricular Esquerda/fisiologia , Doença Aguda , Adulto , Progressão da Doença , Ecocardiografia Doppler de Pulso , Seguimentos , Ventrículos do Coração/fisiopatologia , Humanos , Imagem Cinética por Ressonância Magnética , Masculino , Miocardite/diagnóstico , Miocardite/fisiopatologia , Estudos Retrospectivos , Fatores de Tempo , Disfunção Ventricular Esquerda/diagnóstico , Disfunção Ventricular Esquerda/etiologia
19.
Adv Exp Med Biol ; 1067: 313-325, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29532331

RESUMO

Physical activity comprises all muscular activities that require energy expenditure. Regular sequence of structured and organized exercise with the specific purpose of improving wellness and athletic performance is defined as a sports activity.Exercise can be performed at various levels of intensity and duration. According to the social context and pathways, it can be recreational, occupational, and competitive. Therefore, the training burden varies inherently and the heart adaptation is challenging.Although a general agreement on the fact that sports practice leads to metabolic, functional and physical benefits, there is evidence that some athletes may be subjected to adverse outcomes. Sudden cardiac death can occur in apparently healthy individuals with unrecognized cardiovascular disease.Thus, panels of experts in sports medicine have promoted important pre-participation screening programmes aimed at determining sports eligibility and differentiating between physiological remodeling and cardiac disease.In this review, the most important pathophysiological and diagnostic issues are discussed.


Assuntos
Atletas , Cardiopatias/fisiopatologia , Coração/fisiologia , Coração/fisiopatologia , Exercício Físico , Cardiopatias/diagnóstico , Humanos
20.
Adv Exp Med Biol ; 1067: 183-196, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29498024

RESUMO

Cardiomyopathies (Cs) are a heterogeneous group of myocardial diseases with structural and/or functional abnormalities.The aetiology is due to genetic-family substrate in most cases, however, the correct and detailed analysis of morphofunctional abnormalities (severity and distribution of hypertrophy, ventricular dilatation, ventricular dysfunction) and tissue characteristics (myocardial fibrosis, myocardial infiltration) are a crucial element for a definite diagnosis.Among the different diagnostic imaging modalities applied in clinical practice (echocardiography, nuclear medicine), cardiac magnetic resonance (CMR) has emerged as a non-invasive diagnostic tool having high ability to quantify systolic function and tissue abnormalities that represent the substrates of many Cs.The main added value of CMR is the ability to identify cardiomyopathies with respect to ischemic heart disease and, above all, to discriminate the major types of cardiomyopathies based on morpho-functional presentation patterns and the presence and location of myocardial fibrosis.Many CMR elements allow increasing diagnostic accuracy but CMR data should be integrated with an appropriate clinical and instrumental context.Computed Tomographic (CT) scan technology has showed a complementary role in patients having Cs and HF.In this chapter, the diagnostic, pathophysiologic and prognostic value of CMR and CT in heart failure due to the most common cardiomyopathies will be discussed.


Assuntos
Cardiomiopatias/diagnóstico por imagem , Insuficiência Cardíaca/diagnóstico por imagem , Coração/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Tomografia Computadorizada por Raios X/métodos , Cardiomiopatias/patologia , Doença Crônica , Insuficiência Cardíaca/patologia , Humanos
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