Risonanza magnetica cardiaca e scintigrafia con difosfonati nella diagnosi di amiloidosi cardiaca.

Introduzione: L’amiloidosi cardiaca (cardiac amyloidosis, CA) rappresenta una patologia sistemica, che richiede uno sforzo diagnostico multidisciplinare, ancora oggi sottodiagnosticata. L’imaging con la risonanza magnetica cardiaca (cardiac magnetic resonance, CMR) tramite la valutazione del late gadolinium enhancement (LGE) e la scintigrafia con difosfonati hanno dimostrato recentemente di essere due utili strumenti nella sua diagnosi. A tutt’oggi mancano studi relativi alla distribuzione segmentaria del tracciante ed alla valutazione dell’estensione dell’interessamento cardiaco nella CA mediante scintigrafia multiplanare (single photon emission computed tomography, SPECT), che al confronto tra le due metodiche nei sottotipi di CA. Scopo del lavoro: Confrontare i parametri qualitativi e quantitativi di distribuzione del tracciante alla SPECT e la presenza di LGE alla CMR in pazienti con CA da transtiretina (TTR-CA) e da catene leggere (AL-CA). Valutare la feasibility di CMR e scintigrafia con difosfonati in pazienti con CA. Valutare l’accuratezza diagnostica della scintigrafia con idrossi-metilen-difosfonato nella diagnosi di CA. Risultati: Ottantadue pazienti sono stati sottoposti a scintigrafia con difosfonati presso la Fondazione Toscana G. Monasterio di Pisa tra Gennaio 2011 e Maggio 2015 in relazione al sospetto clinico di CA. Il 44% di loro non ha eseguito una CMR e pertanto esclusi dallo studio con il 30% di loro a causa di controindicazioni assolute o relative all’esecuzione dell’esame di CMR. Quarantasei pazienti sono stati sottoposti a entrambi gli esami e hanno rappresentato la nostra popolazione di studio. I gruppi di pazienti con TTR-CA e AL-CA non hanno mostrato differenze statisticamente significative in termini di volumi, masse e spessori biventricolari e presenza di LGE con l’eccezione del gradiente LGE apice-base osservato nel 26% dei pazienti con TTR-CA ed in nessun paziente con AL-CA (p<0.05). Nella diagnosi generica di CA, la scintigrafia ha mostrato una specificità del 100%, sensibilità del 66% e la CMR del 100% sia di specificità che di sensibilità. Nella diagnosi di TTR-CA, la scintigrafia una specificità del 100% e sensibilità del 95%, mentre la CMR una sensibilità del 100% ed una specificità del 67%. Una valutazione quantitativa SPECT mediante il numero dei conti per voxel per minuto (cvm) > 9851 ha permesso di individuare i segmenti verosimilmente interessati da depositi di amiloide con una specificità e sensibilità del 87%. Il confronto dei dati qualitativi per segmento ha evidenziato una concordanza tra SPECT e CMR del 93% (p 12906 individua i segmenti descritti come LGE positivi con una specificità del 92,1% ed una sensibilità del 71,6%. Anche la valutazione quantitativa CMR mediante T1 mapping correla (r: 0,32, p<0.005) con i cvm. Conclusioni: L’uso combinato di CMR e SPECT permette di sfruttare l’elevata accuratezza di CMR nella diagnosi generica di AC e l’elevata accuratezza diagnostica della scintigrafia nell’identificare le forme TTR-CA. Il confronto tra SPECT e CMR nella valutazione della distribuzione segmentaria biventricolare dell’interessamento amiloidotico è altamente concordante tra le due metodiche nella TTR-CA. L’utilizzo della SPECT potrebbe avere in futuro un ruolo importante anche nella valutazione di progressione di malattia e nella valutazione dell’efficacia di nuove terapie farmacologiche specifiche per le forme TTR-CA

Lack of a relationship between circulating gamma-glutamyltransferase levels and carotid intima media thickness in hypertensive and diabetic patients

Marco Nuti, Paolo Spontoni, Chrysanthos Grigoratos, Giulia Dell&amp;#39;Omo, Alberto Balbarini, Roberto PedrinelliDipartimento Cardio Toracico e Vascolare, Universit&amp;agrave; di Pisa, Pisa, ItalyBackground: By increasing the intracellular prooxidant burden, gamma-glutamyltransferase (GGT) may accelerate atherosclerotic vascular disease. That noxious influence may be reflected by circulating enzyme levels, a correlate of cardiovascular risk factors, and a predictor of incident events. To evaluate this hypothesis, we tested the association between circulating GGT and common carotid intima-media thickness (CIMT), a surrogate index of systemic atherosclerotic involvement, in a large and well-characterized group of patients at risk of cardiovascular disease (CVD).Patients: This study analyzed 548 patients with hypertension and/or diabetes and a widely prevalent history of CVD. Subjects with known hepatic disease and abnormal GGT values were excluded.Methods: CIMT (B-mode ultrasonography) values were the mean of four far-wall measurements at both common carotids. Metabolic syndrome (MetS) was diagnosed according to National Cholesterol Education Program-Adult Treatment Panel III criteria. Due to inherent sex-related differences in GGT levels, the data were analyzed separately in males and females in samples dichotomized by the median.Results: The age-adjusted CIMT values did not differ by GGT levels in males or females. In contrast, the carotid wall was consistently thicker in patients with a history of CVD and MetS independent of age and concurrent GGT values. In both sexes, GGT was associated with key components of the MetS such as triglycerides, fasting plasma glucose, and body mass index.Conclusion: The data collected in this mixed group of hypertensive and/or diabetic patients with widely prevalent history of CVD do not support the concept of a direct pathophysiological link between GGT levels within reference limits and atherosclerotic involvement.Keywords: gamma-glutamyltransferase, carotid intima-media thickness, atherosclerosis, metabolic syndrom

An uncommon clinical condition: chronic thrombosis of the inferior vena cava. A case report and review of literature

The lifetime incidence of deep vein thrombosis (DVT) is approximately 0.1% in general population and even more uncommon in subjects below 40 years of age. Thrombosis of the inferior vena cava (IVC) is an exceptionally rare clinical condition, with etiological factors similar to lower limb DVT. We present a case of post-traumatic chronic obstruction of the IVC in 41 years-old man, caused by a prolonged squatted position, while he was working as a bricklayer. We visited the patient fifteen years after the onset of the first clinical setting showing a severe post thrombotic syndrome, as a consequence of the already diagnosticated thrombosis, involving predominantly the right inferior leg. Thrombophilia screening tests showed patient to be a heterozygous carrier of methylenetetrahydrofolate reductase (MTHFR) gene mutation. Computed tomography (CT) scan confirmed the thrombotic obstruction of the infrahepatic IVC, both common iliac veins, right external and internal iliac veins, with multiple collateral pathways. Because of thrombosis extension, inherited prothrombotic condition and the young age of the patient, we decided to continue life-long oral anticoagulant therapy

4-Dimensional Velocity Mapping Cardiac Magnetic Resonance of Extracardiac Bypass for Aortic Coarctation Repair

Abstract This report describes a case of a young lady who, following extracardiac bypass between ascending and descending aorta for severe aortic coarctation, underwent 4-dimensional flow cardiac magnetic resonance, a technique that, by 3-dimensional flow assessment over time (4-dimensional), allows not only quantification of flows but also wall shear stress. In this case, increased wall shear stress was observed in the conduit's acute angle (kinking) as well as at the distal anastomosis level. The authors postulate that increased wall shear stress could help identify and risk stratify adult congenital heart disease who could develop vascular complications in the future. ( Level of Difficulty: Intermediate.

Arrhythmic risk stratification by cardiac magnetic resonance tissue characterization: disclosing the arrhythmic substrate within the heart muscle

Sudden cardiac death (SCD) is a pivotal health problem worldwide. The identification of subjects at increased risk of SCD is crucial for the accurate selection of candidates for implantable cardioverter defibrillator (ICD) therapy. Current strategies for arrhythmic stratification largely rely on left ventricular (LV) ejection fraction (EF), mostly measured by echocardiography, and New York Heart Association functional status for heart failure with reduced EF. For specific diseases, such as hypertrophic and arrhythmogenic cardiomyopathy, some risk scores have been proposed; however, these scores take into account some parameters that are a partial reflection of the global arrhythmic risk and show a suboptimal accuracy. Thanks to a more comprehensive evaluation, cardiac magnetic resonance (CMR) provides insights into the heart muscle (the so-called tissue characterization) identifying cardiac fibrosis as an arrhythmic substrate. Combining sequences before and after administration of contrast media and mapping techniques, CMR is able to characterize the myocardial tissue composition, shedding light on both intracellular and extracellular alterations. Over time, late gadolinium enhancement (LGE) emerged as solid prognostic marker, strongly associated with major arrhythmic events regardless of LVEF, adding incremental value over current strategy in ischemic heart disease and non-ischemic cardiomyopathies. The evidence on a potential prognostic role of mapping imaging is promising. However, mapping techniques require further investigation and standardization. Disclosing the arrhythmic substrate within the myocardium, CMR should be considered as part of a multiparametric approach to personalized arrhythmic stratificatio

Evaluation of a Novel Method Using Computed Tomography to Predict New Onset of Atrial Fibrillation or Embolic Events after Transcatheter Aortic Valve Implantation: the Role of Hounsfield Unit Density Ratio in the Left Atrial Appendage

Backgrounds: Evaluation of left atrial appendage (LAA) with angio-computed tomography (CCTA) in order to predict new onset of atrial fibrillation (AF) or embolic events is a new upcoming topic. No previous reported studies are available in patients undergoing transcatheter aortic valve implantation (TAVI). Methods: We analyzed pre-procedural CCTA scans of 325 patients who underwent TAVI performing a linear coefficient of attenuation analyses with Hounsfield units (HU) in LAA. HU in LAA distal and proximal was calculated, as well as the ratio. A sensibility and specificity analyses was conducted in order to identify the optimal cutoff to predict new onset AF or embolic events after TAVI. Results: Patients were divided into 4 groups according to the presence of AF. Baseline clinical and echocardiographic features were similar except for a significantly higher STS score and mitral regurgitation severity in PRE-TAVI AF group (p=0.003 and p=0.002 respectively). HU analyses showed a statistical difference in measure performed in LAA distal and in the HU LAA distal/Proximal ratio, with the lowest value in patients with pre-TAVI AF (p&lt;0.001 and p&lt;0.001 respectively). The ROC analyses found 0.84 as the cut-off for to predict the composite endpoint of new AF or embolic events, with sensitivity of 51% and specificity of 52% (p=0.008). Conclusion: In patients with aortic stenosis (AS), use of LAA assessment with CCTA to predict embolic events or new onset AF is no efficacy and cannot be substituted clinical indications for prevention and therapy of embolic events

'Image-navigated 3-dimensional late gadolinium enhancement cardiovascular magnetic resonance imaging:feasibility and initial clinical results'

Abstract Background Image-navigated 3-dimensional late gadolinium enhancement (iNAV-3D LGE) is an advanced imaging technique that allows for direct respiratory motion correction of the heart. Its feasibility in a routine clinical setting has not been validated. Methods Twenty-three consecutive patients referred for cardiovascular magnetic resonance (CMR) examination including late gadolinium enhancement (LGE) imaging were prospectively enrolled. Image-navigated free-breathing 3-dimensional (3D) T1-weighted gradient-echo LGE and two-dimensional (2D LGE) images were acquired in random order on a 1.5 T CMR system. Images were assessed for global, segmental LGE detection and transmural extent. Objective image quality including signal-to-noise (SNR), contrast-to-noise (CNR) and myocardial/blood sharpness were performed. Results Interpretable images were obtained in all 2D–LGE and in 22/23 iNAV-3D LGE exams, resulting in a total of 22 datasets and 352 segments. LGE was detected in 5 patients with ischemic pattern, in 7 with non-ischemic pattern, while it was absent in 10 cases. There was an excellent agreement between 2D and 3D data sets with regard to global, segmental LGE detection and transmurality. Blood-myocardium sharpness measurements were also comparable between the two techniques. SNRblood and CNRblood-myo was significantly higher for 2D LGE (P < 0.001, respectively), while SNRmyo was not statistically significant between 2D LGE and iNAV-3D LGE. Conclusion Diagnostic performance of iNAV-3D LGE was comparable to 2D LGE in a prospective clinical setting. SNRblood and CNRblood-myo was significantly lower in the iNAV-3D LGE group