Agostino Meduri

Recognizing and treating myocarditis in recent-onset systemic sclerosis heart disease: Potential utility of immunosuppressive therapy in cardiac damage progression

OBJECTIVES Scleroderma heart disease is a major risk of death in systemic sclerosis (SSc). Mechanisms underlying myocardial damage are still unclear. We performed an extensive study of SSc patients with recent-onset symptoms for heart disease and examined the efficacy of immunosuppressive therapy. METHODS A cohort of 181 SSc patients was enrolled. Of these, 7 patients newly developed clinical symptoms of heart disease (heart failure, chest pain, and palpitation); all of them showed mild but persistent increase in cardiac enzymes. These patients underwent Holter ECG, 2D-echocardiography, perfusional scintigraphy, delayed-enhancement-cardiac magnetic resonance (DE-CMR), coronary angiography, and endomyocardial biopsy. Patients were treated for at least 12 months and followed-up for 5 years. RESULTS Ventricular ectopic beats (VEBs) were found in 4 patients, wall motion abnormalities in 3, pericardial effusion in 6, and DE in CMR in 6 with T2-hyperintensity in 2. In all patients, histology showed upregulation of endothelium adhesion molecules and infiltration of activated T lymphocytes, with (acute/active myocarditis in 6) or without (chronic/borderline myocarditis in 1) myocyte necrosis. Parvovirus B19 genome was detected in 3. None showed occlusion of coronary arteries or microvessels. Compared with SSc controls, these patients more often had early disease, skeletal myositis, c-ANCA/anti-PR3 positivity, VEBs, pericardial effusion, and systolic and/or diastolic dysfunction. Immunosuppressive therapy improved symptoms and led to cardiac enzyme negativization; however, 2 patients died of sudden death during follow-up. CONCLUSIONS Myocarditis is a common finding in SSc patients with recent-onset cardiac involvement. Its early detection allowed to timely start an immunosuppressive treatment, preventing cardiac damage progression in most cases.

Contrast transoesophageal echocardiography remains superior to contrast-enhanced cardiac magnetic resonance imaging for the diagnosis of patent foramen ovale

AIMS In 30-40% of patients with acute ischaemic stroke, the cause remains undefined (cryptogenic stroke). Contrast transoesophageal echocardiography (TEE) is considered the gold standard for patent foramen ovale (PFO) detection. Recently, however, cardiac magnetic resonance (CMR) has also been applied to detect PFO. In this study, we compared the diagnostic value of CMR and TEE in detecting PFO in a group of patients with apparently cryptogenic stroke. METHODS AND RESULTS Twenty-five patients (age 50 ± 13 years, 16 males) with apparently cryptogenic ischaemic stroke underwent contrast-enhanced TEE and contrast CMR for detection of possible PFO. Both imaging studies were performed during Valsalva manoeuvre. PFO grading results were assessed visually both for TEE and for CMR, according to the entity of contrast passage in the left atrium (grade 0 = no PFO; grades 1, 2, and 3 = mild, medium, and wide PFO, respectively). TEE detected PFO in 16 patients (64%). Contrast-enhanced CMR identified a PFO in 7 (44%) of these patients. TEE showed a grade 1 PFO in five patients, a grade 2 PFO in eight patients, and a grade 3 PFO in three patients. Of these patients, CMR failed to identify PFO in all five patients with a grade 1 PFO, in one patient with a grade 2 PFO, and one patient with grade 3 PFO according to TEE. None of the nine patients without PFO at TEE was shown to have a PFO at CMR. When compared with TEE, the present methodology of CMR had a sensitivity of 50%, specificity of 100%, negative predictive value of 31%, and a positive predictive value of 100%. CONCLUSION Our data suggest that TEE is the cornerstone imaging diagnostic test to detect and characterize PFO in patients with ischaemic stroke, and is shown to be better compared with the current CMR sequences.

Characterization of the electroanatomic substrate in a case of noncompaction left ventricle

An apparently healthy 43-year-old man was submitted to cardiological evaluation for sport activity. Two-dimensional echocardiography led to suspicion of noncompaction deformity, later confirmed by magnetic resonance imaging (MRI), left ventricular catheterization and endomyocardial biopsies. To exclude life-threatening arrhythmias, the patient was submitted to an electrophysiological study and to a real-time three-dimensional electroanatomic reconstruction of left ventricle. The electroanatomic map revealed extensive area of electrical abnormalities. Extent and localization of scar areas mostly corresponded to the areas of enhancement observed at MRI. The present report is the first on electroanatomic substrate evaluation in a noncompaction left ventricle. Our findings show that ventricular noncompaction is characterized by electrical abnormalities including low voltage and scar areas, mainly related to the presence and extent of myocardial fibrosis rather than noncompacted myocardium. Electroanatomic mapping may contribute to detect and quantify fibrotic areas in patients presenting this rare cardiomyopathy.

Role of first pass and delayed enhancement in assessment of segmental functional recovery after acute myocardial infarction

PurposeAssessing myocardial viability is crucial in decision making and prognostic restratification after acute myocardial infarction (MI). A number of noninvasive imaging modalities have been employed in viability identification, but contrast-enhanced magnetic resonance (MR) imaging has been shown to be extremely accurate because of its transmural resolution and precise definition of microvascular obstruction. Our purpose was to assess functional recovery after acute MI, with special focus on the role of infarct transmurality and microvascular obstruction.Materials and methodsForty-six consecutive patients with first acute MI, reperfused by primary percutaneous transluminal coronary angioplasty (PTCA) (n=40) or fibrinolysis (n=6), underwent MR imaging within the first week to assess oedema, microvascular obstruction, function and viability and then again after 4–6 months to assess functional recovery and scar.ResultsAt first MR examination, postcontrast images were analysed according to three patterns, based on a combination of first-pass and delayed-enhancement data: pattern 1 (normal first pass and late hyperenhancement <50% thickness) identified viable myocardium, whereas pattern 2 (late hyperenhancement >50% thickness, with or without first-pass perfusion defect) and pattern 3 (perfusion defect at first pass and late hypoenhancement) recognised nonviable myocardium, with 93% sensitivity, 75% specificity, 92% positive predictive value and 78% negative predictive value for identifying viable tissue. Furthermore, by dividing pattern 2 into two subpatterns, 2A and 2B, based on absence or presence of microvascular obstruction in >50% transmural infarcts, we were able to better identify the segments without recovery or that were nonviable with a 1.39 relative risk of failed recovery.ConclusionsAfter acute MI, not all infarcts with transmurality >50% can be considered nonviable; microvascular obstruction detected at first pass can help to better stratify these cases.RiassuntoObiettivoL’identificazione del miocardio vitale dopo infarto del miocardio è di fondamentale importanza sia per l’impostazione terapeutica che per la ristratificazione prognostica. Numerose metodiche di imaging non invasivo sono state utilizzate per lo studio della vitalità, ma tra queste la risonanza magnetica (RM) si è dimostrata particolarmente accurata, grazie alla sua risoluzione transmurale e alla identificazione dell’ostruzione del microcircolo. Nel nostro studio abbiamo valutato il recupero contrattile dopo infarto del miocardio, con particolare attenzione al ruolo della transmuralità dell’infarto e alla presenza dell’ostruzione del microcircolo.Materiali e metodiQuarantasei pazienti consecutivi con primo infarto acuto del miocardio e trattati con angioplastica coronarica percutanea (PTCA) (n=40) o fibrinolisi (n=6) sono stati studiati con RM con mezzo di contrasto (MdC), per definire edema, funzione, ostruzione del microcircolo e vitalità. Le immagini post-contrasto sono state valutate sulla base di 3 pattern di combinazione tra dati del primo passaggio e del late enhancement.RisultatiConsiderando il pattern 1 (iper-enhancement tardivo<50% dello spessore, con primo passaggio normale) come miocardio vitale, ed i pattern 2 (iperenhancement tardivo>50% dello spessore, con o senza difetto di perfusione al primo passaggio) e 3 (ipo-enhancement al primo passaggio e tardivo) come miocardio non vitale, si è ottenuta una sensibilità del 92%, una specificità del 75%, un valore predittivo positivo del 93% ed un valore predittivo negativo del 78% nella identificazione della vitalità. Tuttavia suddividendo il pattern 2 in due sottogruppi, A e B, in base alla assenza o alla presenza di ostruzione del microcircolo negli infarti con transmuraità>50%, si è ottenuta una migliore identificazione dei segmenti privi di recupero contrattile, o non vitali, con un rischio relativo di non recupero pari a 1,39.ConclusioniNegli infarti acuti, non tutti i segmenti con transmuralità maggiore del 50% mostrano mancato recupero contrattile, indice di non vitalità; l’analisi del danno microcircolatorio al primo passaggio può aiutare la corretta identificazione e stratificazione di questi casi.

MDCT coronary angiography – postprocessing, reading, and reporting: last but not least

Significant literature on MDCT coronary angiography (MDCT-CA) has emerged in the last decade concerning patients selection, technical aspects of different generations of CT equipment, ECG gating, contrast material and beta-blockade administration, acquisition parameters, and radiation dose. However, the literature regarding postprocessing, reading, and reporting is not so extensive. This review highlights the main elements of MDCT-CA data analysis, thereby allowing the radiologist to take full advantage of this technology and enable a structured report to be generated, promoting best practice with high-quality results.

Correlation between clinical presentation and delayed-enhancement MRI pattern in myocarditis

PurposeThe exact incidence of myocarditis is unknown, as the diagnosis is frequently delayed or missed. Clinical presentation and disease course are extremely variable, as there may be acute onset with acute coronary syndrome, or cardiogenic shock, or progressive heart failure or arrhythmias. The purpose of this study was to identify prognostic factors on magnetic resonance imaging (MRI) performed in patients with bioptically proven myocarditis at presentation and after 6 months.Materials and methodsFifty-six consecutive patients with different presentations of myocarditis (20 with acute coronary syndrome, 20 with heart failure, 16 with arrhythmias) were enrolled. All patients underwent B-mode echocardiography (echo) and tissue Doppler imaging, coronarography, ventriculography, endomyocardial biopsy and contrast-enhanced MRI examination, as well as clinical and echo follow-up at 6 months.ResultsAt 6-month follow-up, patients were divided in two groups according to values of end-systolic volume and ejection fraction: patients with negative remodelling and those with positive remodelling. Late enhancement was found to be an independent predictor of negative remodelling.ConclusionsContrast-enhanced MRI is useful both in the diagnosis and as a prognostic indicator in the clinical suspicion of myocarditis.RiassuntoObiettivoL’esatta incidenza delle miocarditi non è ben conosciuta e spesso la diagnosi viene raggiunta tardivamente o non viene raggiunta affatto; anche la presentazione ed il decorso clinico sono estremamente variabili, potendo manifestarsi con un esordio acuto come una sindrome simil-coronarica acuta o shock cardiogeno, oppure con una insufficienza cardiaca spesso progressiva, o ancora con l’improvvisa comparsa di aritmie. Scopo del nostro studio è stato il tentativo di identificare eventuali fattori prognostici alla risonanza magnetica (RM) eseguita all’esordio della sintomatologia e dopo sei mesi in pazienti con biopsia endomiocardica positiva per miocardite.Materiali e metodiDa gennaio a dicembre 2010 sono stati studiati 56 pazienti consecutivi in base alle possibili presentazioni cliniche suddette (20 con sindrome similcoronarica acuta, 20 con scompenso cardiaco e 16 con aritmie), tutti sottoposti ad ecocardiografia b-mode e doppler tissutale, coronarografia, ventricolografia e biopsia endomiocardica e RM con mezzo di contrasto (MdC).RisultatiA 6 mesi è stato eseguito follow-up clinico e mediante ecocardiografia. I pazienti al follow-up sono stati divisi in due gruppi in base alla variazione di volume telesistolico (VTS) e frazione di eiezione (FE): pazienti con rimodellamento negativo e pazienti con rimodellamento positivo. Il late enhancement (LE) si è dimostrato predittore indipendente di rimodellamento negativo.ConclusioniLa RM con MdC è estremamente utile non solo per la diagnosi ma anche come indicatore prognostico nel sospetto clinico di miocardite.

Studio post-infarto acuto e cronico

L’infarto e la causa principale di morte in tutto il mondo [1] e l’incidenza della malattia ischemica e in continuo aumento: appare quindi evidente la necessita di implementare strategie diagnostiche e terapeutiche per ridurre, da un lato, la mortalita e per migliorare, dall’altro, la gestione dei pazienti infartuati che hanno maggiori probabilita di un successivo evento cardiaco maggiore.

Le sequenze a sangue nero

La risonanza magnetica fornisce, con i suoi diversi approcci, una grande varieta di informazioni anatomiche, angiografiche e funzionali. Le sequenze morfologiche a sangue nero, pur non fornendo informazioni funzionali, hanno elevato contrasto, alta risoluzione spaziale e consentono una dettagliata visualizzazione delle strutture cardiache, del mediastino e delle pareti vascolari.

Come strutturare un esame RM completo

La risonanza magnetica cardiaca (RMC) trova indicazione in quasi tutti gli ambiti della patologia cardiaca e la tecnica di esame puo variare considerevolmente a seconda dell’indicazione all’esame stesso. Per questa ragione e opportuno cercare di organizzare la conduzione dell’esame in moduli che possano essere applicati in differenti condizioni e che consentano un approccio organico all’esame.

Reduced time of arrival on brain perfusion CT in a patient with recurrent cryptogenic stroke: an indirect sign of a patent foramen ovale

Sir, We would like to bring your readers’ attention to an interesting observation we recently made on a patient presenting with acute stroke, where an abnormality in the timing of contrast agent bolus arrival on brain perfusion CT (pCT) raised the suspicion about a right-to-left shunt and prompted targeted diagnostic investigations to diagnose a patent foramen ovale (PFO). A 49-year-old female patient, with history of a cryptogenic right sylvian ischemic stroke during adolescence, was admitted for sudden onset of right hemiparesis and Broca’s aphasia. Upon admission, 6 h after symptoms’ onset, the patient underwent a multimodal stroke CT protocol composed of non-contrast CT, CT angiography, and dynamic bolus first pass pCT of the brain. The non-contrast CT (Fig. 1a) showed a widened right sylvian fissure related to a remote right sylvian infarct as well as early signs of ischemia in the left insula and lentiform nucleus. CT angiography (Fig. 1b) demonstrated a left distal M1 occlusion, with collateral filling of the sylvian branches and normal carotid bifurcation bilaterally. The pCT (Fig. 1c) showed a perfusion deficit in the left sylvian region. Assessment of the arterial time– density curves, reflecting the contrast agent first pass through the cerebral vasculature, revealed abnormal, early arrival of the bolus, within 7–8 s, as opposed to the 15– 20 s normally required for the bolus injected, to reach the intracranial arteries (Fig. 1d,e). The early bolus arrival raised the concern for a right-to-left shunt. Further investigation with cardiac MR confirmed our suspicion of a PFO. No additional cardiovascular or coagulation risk factors were identified. Because of the recurrent nature of her cerebrovascular accidents, the patient was placed on life-term oral anticoagulation. It is worthy to discuss the finding of a short time of bolus arrival on the pCT performed upon admission. The pCT scan is performed applying a delay time of 7 s from the start of the power-injector-aided administration of 50 ml of non-ionic iodinated contrast material in an antecubital vein via a 20-gauge cannula at a flow rate of 4 ml/s. The short scan delay is aimed to obtain an initial baseline density measurement before arrival of the contrast bolus [1]. In our patient, the arterial curve rise was coincident with the scan start, indicating that the bolus arrived 7–8 s after the injection, which is unusually short compared to the typical bolus arrival after 15–20 s from the injection [2]. There are multiple conditions that can result in the bolus arrival being delayed, including poor intravenous access and impaired cardiac function [2]. However, it is unusual to observe an abnormally short time of arrival. These sometimes happen in very young patients with a hyperdynamic heart. Another possible cause is an abnormal intracardiac communication. The presence of such a communication, in normal conditions, given the pressure gradients, leads to a spontaneous left-to-right shunt. Neuroradiology (2008) 50:613–615 DOI 10.1007/s00234-008-0414-6