103 research outputs found
Diagnosis of arrhythmogenic cardiomyopathy: The Padua criteria.
The original designation of "Arrhythmogenic right ventricular (dysplasia/) cardiomyopathy"(ARVC) was used by the scientists who first discovered the disease, in the pre-genetic and pre-cardiac magnetic resonance era, to describe a new heart muscle disease predominantly affecting the right ventricle, whose cardinal clinical manifestation was the occurrence of malignant ventricular arrhythmias. Subsequently, autopsy investigations, genotype-phenotype correlations studies and the increasing use of contrast-enhancement cardiac magnetic resonance showed that the fibro-fatty replacement of the myocardium represents the distinctive phenotypic feature of the disease that affects the myocardium of both ventricles, with left ventricular involvement which may parallel or exceed the severity of right ventricular involvement. This has led to the new designation of "Arrhythmogenic Cardiomyopathy" (ACM), that represents the evolution of the original term of ARVC. The present International Expert Consensus document proposes an upgrade of the criteria for diagnosis of the entire spectrum of the phenotypic variants of ACM. The proposed "Padua criteria" derive from the diagnostic approach to ACM, which has been developed over 30Â years by the multidisciplinary team of basic researchers and clinical cardiologists of the Medical School of the University of Padua. The Padua criteria are a working framework to improve the diagnosis of ACM by introducing new diagnostic criteria regarding tissue characterization findings by contrast-enhanced cardiac magnetic resonance, depolarization/repolarization ECG abnormalities and ventricular arrhythmia features for diagnosis of the left ventricular phenotype. The proposed diagnostic criteria need to be further validated by future clinical studies in large cohorts of patients
Focal myocardial fibrosis assessed by late gadolinium enhancement cardiovascular magnetic resonance in children and adolescents with dilated cardiomyopathy
CARDIAC MAGNETIC RESONANCE IMAGING IN DILATED AND ARRHYTHMOGENIC CARDIOMYOPATHIES: AN INSIGHT INTO CLINICAL AND PATHOLOGICAL SIGNIFICANCE OF LATE GADOLINIUM ENHANCEMENT
Background: Cardiomyopathies are an important and heterogeneous group of diseases of the heart muscle in which tissue characterization has been extensively studied only ex-vivo so far. Cardiac magnetic resonance (CMR) can provide in vivo the detection of post-contrast deposition (so called Late Gadolinium Enhancement, LGE). The role of LGE in the differential diagnosis of cardiac diseases has advanced through the years, in particular thanks to its capability to differentiate post-ischemic scar (with subendocardial or transmural coronary artery-related deposition) versus non-ischemic scar of different aetiologies. Among cardiomyopathies, the prognostic significance of LGE pattern is not well established, with the exception of hypertrophic cardiomyopathy. Detection in vivo of LGE in different cardiomyopathies has been used as a surrogate of fibrosis, even if the real basis of contrast deposition is not well understood. To this regard, dilated cardiomyopathy (DCM) is characterized by diffuse interstitial fibrosis with or without replacement-type fibrosis, which are associated with worse prognosis. Another myocardial disease in which CMR offers the capability of a non-invasive tissue characterization is Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC), an inherited heart muscle disease with progressive loss of myocardium and replacement by fibrofatty tissue. The extensively application of CMR to these cardiomyopathies and its comparison with traditional invasive and non-invasive techniques for diagnostic and prognostic purposes has been limited so far.
Aim: in order to assess the clinical significance of LGE in DCM and ARVC, the following lines were pursued:
1) in patients with DCM the diagnostic and the prognostic value of LGE, in particular evaluating the possible different significance of various LGE patterns, total amount of LGE against survival, heart failure and ventricular arrhythmias;
2) in patients with ARVC the comparison between CMR findings and: 1a) traditional electrocardiographic features; 1b) scar detction by endocardial voltage mapping (EVM), and 1c) the prognostic significance of LGE;
3) the significance of CMR tissue abnormalities by comparing LGE and specimen heart and/or endomyocardial biopsy (EMB) findings in DCM and ARVC.
Material and Methods: Between January 2007 and December 2010 we prospectively evaluated two different groups of patients referred to our Tertiary Referral Centre for a complete invasive and non-invasive evaluation for unexplained left ventricle (LV) dilatation (DCM Group, A) and for suspected ARVC (ARVC Group, B). DCM Group (A): we prospectively evaluated 210 patients referred for unexplained LV dilatation with subacute-chronic onset (≥ 1 month), with or without previous history of heart failure, who underwent during the same hospitalization to a complete screening including CMR with LGE, angiography and EMB. ARVC group (B): we prospectively evaluated 52 patients who were referred to our Tertiary Centre for susptected ARVC and in which the diagnosis was reached according to 1994 Task Force Criteria and 2010 Modified Criteria. Data on clinical history, ECG, ECG Holter monitoring, echocardiography, CMR and EMB in selected cases were collected. In a subgroup of patients an electrophysiological study with EVM was performed. For each subject enrolled, a clinical, ECG and echocardiographic follow-up was obtained.
Results: Group A. On the basis of coronary angiography, patients were divided into two groups: ischemic (99 patients) and DCM (111 patients). Ischemic group patients were then excluded from the analysis. Compared to angiography, CMR showed an excellent accuracy (96.5%) for the identification of non-ischemic versus ischemic aetiology for LV dilatation. In the DCM group (111 patients) LGE was present (with a non-ischemic pattern) in 67 cases (60.4%) and absent in 44 (39.6%); no differences between RV and LV volumes and ejection fraction (EF) were found between the two groups. Out of 67 patients with positive LGE, a “gray” pattern was present in 12 patients (17.9%), a midwall/subepicardial stria in 49 (73.1%), a septal junction pattern (anterior and/or posterior) either isolated in 4 (5.9%) or associated with other patterns in 25 patients (37.3%) and finally a spotty (“patchy”) pattern in 2 (2.9%). Among patients with positive LGE, the extent of LGE was 6.3%+/-8.8% of LV mass. Fifty-eight patients underwent to EMB: 33/58 patients (56.9%) showed replacement-type fibrosis, of this subgroup 23 patients (69.7%) showed LGE on CMR. Out of 13/58 patients (22%) without replacement-type fibrosis on EMB, LGE was present in 9/13 (69.2%) patients: in this subgroup the most frequent LGE pattern was midwall/subepicardial stria (n=8/9; 89%) and one patient (11%) showed a “patchy” LGE pattern . Compared to EMB, CMR showed a low accuracy for fibrosis detection. The range of follow-up in LGE group was 8 years-1 month. Kaplan-Meier curves for composite end-point and ventricular arrhythmias showed a significant differences between patients with and without LGE on CMR (Wilcoxon-Breslow: p< 0.05). The amount of LGE%, by univariate analysis, was strongly associated with ventricular arrhythmias (HR 1.05, 95% CI 1.02 to 1.08; p<0.0001). This association was unchanged in multivariate analysis adjusted for a EF<30% (model 5): HR 1.067, 95% CI 1.034 to 1.1; p<0.0001.With an associated HR of 2.5, the presence of LGE was among the strongest multivariate predictors for the combined end point, surpassed only by age < 49 years. ROC curve analysis revealed a LGE percentage of 3.5 as optimal discriminator for the occurrence of ventricular arrhythmic events with an associated HR of 4.11 (95% CI 1.3 to 12.7; p<0.001).
Group B: On the basis of clinical and CMR evaluation, 24 patients (46%) were defined as “classic ARVC phenotype”; 14 (27%) as “left dominant phenotype”, and finally 14 (27%) as “biventricular ARVC phenotype”. Comparison between ECG and CMR showed that ECG indexes of LV dilatation are ST-segment elevation and T-wave inversion beyond V3 (p<0.05); among tissue characterization parameters, only LV LGE has an identifiable ECG abnormality, i.e. ST segment elevation (p<0.05). On EVM, endocardial voltages of RV was abnormal in 21/23 (91%) patients, with a total of 45 electroanatomic scars (EAS). RV LGE was found in 9/23 (39%) patients, with a total of 23 RV LGE scars: there was a mismatch in 24 RV scars, with 22 EAS not confirmed by LGE. In 9/12 (75%) patients with abnormal RV EVM/normal RV LGE, ≥1 LGE were identified in the LV. The patients of ARVC group enrolled for the follow-up were 52 (34 males; mean age 33+/-15 years). The mean LV ejection fraction was 57+/-8%; the mean fractional area change of RV was 39+/-10%. The mean follow-up was 25.6 months (range 38+/-4 months). Considering only the major events (aborted sudden death/ ventricular fibrillation; sudden death; non sudden death/heart transplantation) these occurred in 12 (7 in the LGE group, 5 in the non-LGE group).Kaplan-Meier curves did not show difference between LGE presence/absence for all events and major events. Kaplan-Meier curves did not show difference between three phenotypes detected by CMR both for all events and major events.
Conclusions: CMR can provide a wide range of information in DCM and ARVC, beyond traditional imaging modalities. In the setting of DCM, CMR shows high accuracy in the detection of non-ischemic aetiology compared with angiography. LGE identifies the patients with increased risk of ventricular arrhythmic events. Compared to EMB, CMR with LGE imaging shows low accuracy for fibrosis detection, probably due to resolution power of CMR. However, an integrated approach with CMR and EMB may be useful in cases with negative EMB/positive LGE to identify epicardial lesion, which are not caught by EMB due to its endocardial approach.
In the setting of ARVC, CMR confirms its superiority in assessing the full spectrum of morpho-functional and tissue abnormalities of a disease that should be as a biventricular cardiomyopathy. The ECG indexes able to identify LV dilatation are ST-segment elevation and T-wave inversion beyond V3; as far as tissue characterization parameters are concerned, LV LGE was associated to ST segment elevation. The correlation between EVM and CMR confirms that EVM allows an accurate identification of RV EAS in patients with ARVC and supports its clinical use for substrate-based mapping and catheter ablation of RV tachycardias as well as for imaging-guided EMB. Currently available LGE CMR appears to visualize unsatisfactorily RV scars and this limits its usefulness in ARVC diagnosis and guiding interventional RV procedures. However, the high prevalence of LV involvement in ARVC patients is in keeping with the current perspective of biventricular disease and points out the diagnostic relevance of LV scar detection by LGE CMR.Introduzione: Le cardiomiopatie rappresentano un gruppo eterogeneo di malattie del muscolo cardiaco la cui caratterizzazione tissutale finora è stata eseguita prevalentemente mediante analisi ex vivo. La risonanza magnetica cardiaca (RMC), grazie all’utilizzo di apposite sequenze e all’impiego di un mezzo di contrasto (gadolinio, visibile come Late Gadolinium Enhancement, LGE), rende possibile una caratterizzazione tissutale in vivo. L’analisi della sede ed estensione dell’LGE permette di differenziare la cicatrice miocardica post-infartuale (LGE sub endocardico o trans murale) rispetto ad altre cicatrici di tipo non ischemico. Nelle diverse cardiomiopatie, il significato prognostico dei depositi di LGE non è del tutto chiarito, se non in parte per la cardiomiopatia ipertrofica. In generale la presenza di LGE nel contesto di una cardiomiopatia viene identificato con la presenza di fibrosi miocardica, nonostante il meccanismo di deposito del gadolinio non sia uguale nelle diverse eziologie di cardiomiopatie, non necessariamente associate a fibrosi miocardica. Nell’ambito delle cardiomiopatie, la cardiomiopatia dilatativa (non dovuta ad una eziologia ischemica) (CMD) si caratterizza da un punto di vista istologico per la presenza di fibrosi interstiziale, con o senza fibrosi sostitutiva, entrambe associate ad una prognosi infausta. Una diversa patologia miocardica in cui la RMC offre una eccezionale capacità di caratterizzazione tissutale è la Cardiomiopatia Aritmogena del Ventricolo Destro (CAVD), una miocardiopatia dovuta ad una progressiva atrofia miocardica con successiva sostituzione fibroadiposa. Benché la RMC stia consolidando il suo ruolo nella pratica clinica, non è stata ancora eseguita una applicazione estensiva della RMC in queste due differenti cardiomiopatie a scopo diagnostico e prognostico, ed ancor più manca una analisi sistematica delle correlazioni tra i diversi quadri radiologici ed i tradizionali parametri invasivi e non-invasivi di queste cardiomiopatie.
Scopo dello Studio: al fine di valutare il significato clinico e prognostico dell’LGE nelle CMD e nella CAVD sono state perseguite le seguenti linee di ricerca:
1) nei pazienti affetti da CMD il significato prognostico dell’LGE nelle DCM, con particolare riferimento ad un end-point di eventi combinati ed all’outcome aritmico;
2) nei pazienti affetti da CAVD il confronto tra i diversi aspetti alla RMC ed 1a) i quadri elettrocardiografici; 1b) il confronto con dati ottenuti dal mappaggio endocavitario del ventricolo destro (“Endocardial Voltage Mapping”, EVM); 1c) il significato prognostico dell’LGE;
3) il significato della alterazioni di caratterizzazione tissutale alla RMC confrontati con i dati istologici dei pazienti con biopsia endomiocardica (BEM) o che sono andati incontro a decesso/trapianto cardiaco nei due gruppi.
Materiali e Metodi: tra il Gennaio 2007 e il Dicembre 2010 sono stati arruolati prospetticamente i pazienti riferiti presso il nostro Centro per una valutazione invasiva per il riscontro di una dilatazione del ventricolo sinistro (Gruppo CMD, A) o per sospetta CAVD (Gruppo CAVD, B). Gruppo CMD (A): sono stati valutati 210 riferiti per riscontro di dilatazione ventricolare con esordio subacuto-cronico (≥ 1 mese), con o senza pregressa storia di scompenso cardiaco, che durante la stessa ospedalizzazione sono stati sottoposti a coronarografia, RMC con contrasto e BEM. Gruppo CAVD (B): 52 soggetti riferiti presso il nostro Centro per sospetta CAVD e la cui diagnosi è stata raggiunta in accordo con i correnti criteri clinico-strumentali recentemente modificati. Durante la stessa ospedalizzazione sono stati sottoposti RMC con contrasto e studio elettrofisiologico con EVM e BEM in casi selezionati. Ogni paziente appartenente ad entrambi i gruppi è stato sottoposto ad un follow-up clinico-strumentale.
Resultati: Gruppo A. Sulla base dei risultati della coronarografia i pazienti sono stati suddivisi in due gruppi in base alla presenza o meno di coronaropatia: in 99 è stato definita una eziologia ischemica alla base della disfunzione ventricolare, 111 non mostravano alcuna coronaropatia (gruppo CMD). Il gruppo dei soggetti ischemici è stato escluso dalla successive analisi. Rispetto all’angiografia coronarica, la RMC ha dimostrato un’ottima accuratezza diagnostica (96.5%) nell’escludere una eziologia ischemica. Nel gruppo A la presenza di un LGE di tipo non-ischemico è stata riscontrata in 67 casi (60.4%) mentre era assente in 44 (39.6%). Nessuna differenza nei volumi ventricolari e funzione sistolica è stata riscontrata nei due sottogruppi. Nei 67 pazienti con LGE era presente un pattern di tipo “gray” in 12, tipo stria “midural”/epicardica in 49 (73.1%), alla giunzione settale tra ventricolo destro e sinistro isolatamente in 4 (5.9%), associato ad altri pattern in 25 (37.3%), ed infine tipo “patchy” in 2 casi (2.9%). Nei pazienti con LGE, l’estensione media era pari al 6.3%+/-8.8% della massa del ventricolo sinistro. In 58 casi è stata eseguita la BEM: 33/58 pazienti (56.9%) mostravano aspetti di fibrosi sostitutiva; di questi, 23/33 (69.7%) mostravano anche LGE alla RMC. In 13/58 casi (22%) si riscontrava una BEM negativa: in questo sottogruppo di soggetti era presente un LGE in 9/13 (69.2%): nella maggioranza dei casi (8/9 pari all’89%) l’LGE era tipo stria “midmural”/epicardica, in uno solo (11%) tipo “patchy”. Nel gruppo A il range del follow-up è 8 anni-1 mese. Le curve di sopravvivenza Kaplan-Meier per eventi combinati ed aritmie ventricolari maggiori hanno mostrato una differenza significativa tra i due gruppi di pazienti con una prognosi peggiore nel gruppo con LGE (Wilcoxon-Breslow: p< 0.05). La quantità totale di LGE si è dimostrata associata alle aritmie ventricolari (HR 1.05, 95% CI 1.02-1.08; p<0.0001); tale correlazione rimaneva anche all’analisi multivariata aggiustata per una frazione d’eiezione inferiore al 30% (HR 1.067, 95% CI 1.034-1.1; p<0.0001).I pazienti con LGE mostravano un rischio di 2.5 per la comparsa di aritmie ventricolari e mediante l’analisi della curva ROC la percentuale di LGE pari a 3.5% è risultato il miglior valore predittivo di eventi aritmici (HR 4.11 (95% CI 1.3-12.7; p<0.001).
Gruppo B. Sulla base dei risultati della RMC, 24 soggetti (46%) sono stati definiti affetti da un forma “classica”, 14 (27%) “dominante sinistra”, e 14 (27%) “biventricolare”. Analizzando la correlazione tra ECG e RMC, gli unici predittori della dilatazione del ventricolo sinistro sono risultati la presenza di un sopraslivellamento del tratto ST e le T invertite oltre V3 (p<0.05); nelle sequenze per la caratterizzazione tissutale la presenza di LGE a carico del ventricolo sinistro era associata ad un sopraslivellamento del tratto ST. Dall’analisi di confronto tra mappaggio endocavitario (EVM) e LGE è emerso che in 21/23 (91%) si dimostrava la presenza di un voltaggio ridotto, per un totale di 45 cicatrici elettroanatomiche. La presenza di LGE a carico del ventricolo destro si è riscontrata in 9/23 (39%) casi, per un totale di 23 cicatrici alla RMC: in 24 casi vi è stato un mismatch tra le due metodiche nel riconoscere le cicatrici di sostituzione fibro-adiposa. In 9/12 (75%) dei soggetti con EVM patologico/RMC normale, sono state riscontrate aree di LGE in almeno una regione del ventricolo sinistro. Il follow-up medio dei pazienti con CAVD arruolati nel follow-up (52; 34 maschi; età media 33+/-15 anni) è stato di 25.6 mesi (38+/-4 mesi). Considerando come eventi maggiori la morte cardiaca (abortita o meno), la fibrillazione/tachicardia ventricolare, morte/trapianto cardiaco gli eventi combinati sono stati 12 (7 nel gruppo con LGE e 5 in quelli senza LGE). Non si è dimostrata alcuna differenza in termini di tempo libero da eventi nei tre gruppi.
Conclusioni: La RMC nell’ambito dello studio della CMD e della CAVD offre delle capacità diagnostiche al di là delle tradizionali metodiche di imaging. Nella CMD presenta un’ottima accuratezza diagnostica, rispetto alla ventricolo-coronarografia, nell’escludere una eziologia ischemica alla base della dilatazione e disfunzione ventricolare. La presenza di LGE nella DCM è in grado inoltre di individuare i paziente a maggior rischio aritmico. Tuttavia se confrontata con la BEM, l’accuratezza diagnostica della RMC rimane bassa, probabilmente a causa del suo potere di risoluzione spaziale che limita il riconoscimento di piccole aree di fibrosi miocardica. D’altra parte, la RMC è in grado di vedere lesioni epicardiche che non sono raggiunte dalla BEM supportando così l’importanza di una valutazione combinata delle due metodiche nelle CMD.
Nei soggetti con CAVD, la RMC si conferma tecnica di imaging capace di esplorare l’intero spettro di alterazioni morfo-funzionali e tissutali della CAVD, che spesso è una malattia bi ventricolare e non può essere esclusiva del ventricolo destro. Il confronto tra ECG e RMC indica la presenza di un sopraslivellamento del tratto ST e l’inversione delle onde T come predittori della dilatazione del ventricolo sinistro; la presenza di LGE a carico del ventricolo sinistro è associato ad un sopraslivellamento del tratto ST. Analizzando i dati relativi al mappaggio elettronatomico, emerge come quest’ultimo riconosca più cicatrici di quanto non riesca a fare la RMC, probabilmente per la difficoltà di riconoscere un LGE a carico della parete assai assottigliata del ventricolo destro. Tuttavia la capacità della RMC di riconoscere le lesioni anche a carico del ventricolo sinistro, laddove il mappaggio elettroanatomico non viene applicato, suggerisce un sinergismo diagnostico tra le due metodiche. Probabilmente il breve tempo di follow-up nel quale si è indagato il significato prognostico dell’LGE a carico del ventricolo sinistro ha reso non significativa la differenza in termini di tempo libero da eventi nei tre gruppi. Studi futuri su casistiche più numerose, tipizzate dal punto di vista genetico e con RMC seriate, delucideranno meglio il significato prognostico di un coinvolgimento precoce del ventricolo sinistro
Right ventricular dilatation in arrhythmogenic right ventricular cardiomyopathy: need for a revision of the 2010 International Task Force criteria
Multiple mycotic aneurysms of the aortic root after aortic valve replacement
A 74-year-old woman with severe aortic valve stenosis underwent aortic valve replacement with a pericardial bioprosthesis. Histological analysis of the excised valve showed dystrophic calcification associated with signs of healed infective endocarditis. Two months later, the patient died due to congestive heart failure. Autopsy revealed a bowel infarction and the presence of multiple mycotic aneurysms of the aortic root with mural thrombosis, which were unnoticed during the patient hospital stay and surgical window
Apical ballooning with mid-ventricular obstruction: the many faces of Takotsubo cardiomyopathy.
Takotsubo cardiomyopathy (TTC) is a transient left ventricular dysfunction due to akinesia of the left-ventricular (LV) mid-apical segments (apical ballooning), which can cause severe reduction in LV systolic function. The typical clinical picture of TTC include chest pain, electrocardiographic changes consisting of mild ST-segment elevation followed by diffuse deep T-wave inversion, QTc interval prolongation and mild troponin release in the absence of significant coronary stenoses. The syndrome often affects post-menopausal women and is triggered by sympathetic overstimulation, like intense physical or emotional stress, so that it is called the “broken heart syndrome”. Although left-ventricular systolic dysfunction usually fully recovers within few days, heart failure can still complicate the early phase. We report a case of stress-induced cardiomyopathy that had full recovery after 4 weeks of follow up. The main electrocardiographic, angiographic and imaging features are discussed
Role of Cardiac Magnetic Resonance Imaging in the Evaluation of Athletes with Premature Ventricular Beats
Premature ventricular beats (PVBs) in athletes are not rare. The risk of PVBs depends on the presence of an underlying pathological myocardial substrate predisposing the subject to sudden cardiac death. The standard diagnostic work-up of athletes with PVBs includes an examination of family and personal history, resting electrocardiogram (ECG), 24 h ambulatory ECG (possibly with a 12-lead configuration and including a training session), maximal exercise testing and echocardiography. Despite its fundamental role in the diagnostic assessment of athletes with PVBs, echocardiography has very limited sensitivity in detecting the presence of non-ischemic left ventricular scars, which can be revealed only through more in-depth studies, particularly with the use of contrast-enhanced cardiac magnetic resonance (CMR) imaging. The morphology, complexity and exercise inducibility of PVBs can help estimate the probability of an underlying heart disease. Based on these features, CMR imaging may be indicated even when echocardiography is normal. This review focuses on interpreting PVBs, and on the indication and role of CMR imaging in the diagnostic evaluation of athletes, with a special focus on non-ischemic left ventricular scars that are an emerging substrate of cardiac arrest during sport
Extension and Transmural Entity of Myocardial Necrosis Are Major Determinants of Coronary Flow Reserve Impairment After Acute Myocardial Function Treated With Primary Coronary Angioplasty
Extension and trasmural entity of myocardial necrosis are major determinants of coronary flow reserve impairment after acute myocardial function treated with primary coronary angioplasty
A Case of Occult Myocarditis Causing Fatal Arrhythmia in a Cystic Fibrosis Patient Subjected to Retransplantation
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