Konstantinos Bratis

Cardiovascular magnetic resonance in rheumatology: Current status and recommendations for use.

Targeted therapies in connective tissue diseases (CTDs) have led to improvements of disease-associated outcomes, but life expectancy remains lower compared to general population due to emerging co-morbidities, particularly due to excess cardiovascular risk. Cardiovascular magnetic resonance (CMR) is a noninvasive imaging technique which can provide detailed information about multiple cardiovascular pathologies without using ionizing radiation. CMR is considered the reference standard for quantitative evaluation of left and right ventricular volumes, mass and function, cardiac tissue characterization and assessment of thoracic vessels; it may also be used for the quantitative assessment of myocardial blood flow with high spatial resolution and for the evaluation of the proximal coronary arteries. These applications are of particular interest in CTDs, because of the potential of serious and variable involvement of the cardiovascular system during their course. The International Consensus Group on CMR in Rheumatology was formed in January 2012 aiming to achieve consensus among CMR and rheumatology experts in developing initial recommendations on the current state-of-the-art use of CMR in CTDs. The present report outlines the recommendations of the participating CMR and rheumatology experts with regards to: (a) indications for use of CMR in rheumatoid arthritis, the spondyloarthropathies, systemic lupus erythematosus, vasculitis of small, medium and large vessels, myositis, sarcoidosis (SRC), and scleroderma (SSc); (b) CMR protocols, terminology for reporting CMR and diagnostic CMR criteria for assessment and quantification of cardiovascular involvement in CTDs; and (c) a research agenda for the further development of this evolving field.

Myocardial perfusion-fibrosis pattern in systemic sclerosis assessed by cardiac magnetic resonance.

Systemic sclerosis (SSc) is a connective tissue disease characterized by vascular and fibrotic lesions of skin and internal organs. Focal hypoperfusion contributes to myocardial fibrosis, documented by pathology. SSc heart involvement carries a poor prognosis (1–3). Stress echocardiography and single-photon emission computed tomography (SPECT) detect perfusion defects (PDs) (4–6). Stress cardiac magnetic resonance imaging (CMR) has been also applied to detect PDs in coronary artery disease (7,8). Our aimwas to evaluate myocardial perfusion-fibrosis in SSc using CMR stress perfusion and late gadolinium enhancement (LGE). Seven asymptomatic SSc (2 M/5 F), aged 51±2 yrs, and 2 limited (L)/5 diffuse (D), without pulmonary hypertension were evaluated. Exclusion criteria were: known CAD and CMR contraindications. SSc received 10 mg/day prednisone, without vasodilator or immunosuppressive treatment. Biochemical and inflammatory indexes were normal at examination time. Raynaud phenomenon was present in all, while digital ulcers in 5/7 (71%). SSc were compared with 7 controls and 5 patients with coronary artery disease (CAD). Written consent form was obtained from all.

CMR detects subclinical cardiomyopathy in mother-carriers of Duchenne and Becker muscular dystrophy.

Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder, leading to reduced/abnormal dystrophin. Becker muscular dystrophy (BMD) is a milder form, caused by the same mutation, with a nearly normal life expectancy, but with a 50% chance of cardiac involvement (CI) ([1][1]). Although

CMR evaluation of cardiac involvement during the convalescence of Kawasaki disease.

Kawasaki disease (KD) is a form of vasculitis affecting both the myocardium and coronary arteries. Coronary aneurysms develop in 15% to 25% of untreated patients ([1,2][1]). Myocardial inflammation is nearly universal during the acute phase (100% of cases in both post-mortem analysis and myocardial

Rest perfusion abnormalities in hypertrophic cardiomyopathy: correlation with myocardial fibrosis and risk factors for sudden cardiac death

Aim To measure the prevalence of abnormal rest perfusion in a population of consecutive patients with known hypertrophic cardiomyopathy (HCM) referred for cardiovascular MRI (CMR), and to assess any associations between abnormal rest perfusion and the presence, pattern, and severity of myocardial scar and the presence of risk factors for sudden death. Materials and methods Eighty consecutive patients with known HCM referred for CMR underwent functional imaging, rest first-pass perfusion, and late gadolinium enhancement (LGE). Results Thirty percent of the patients had abnormal rest perfusion, all of them corresponding to areas of mid-myocardial LGE and to a higher degree of segmental hypertrophy. Rest perfusion abnormalities correlated with more extensive and confluent LGE. The subgroup of patients with myocardial fibrosis and rest perfusion abnormalities (fibrosis+/perfusion+) had more than twice the incidence of episodes of non-sustained ventricular tachycardia on Holter monitoring in comparison to patients with myocardial fibrosis and normal rest perfusion (fibrosis+/perfusion–) and patients with no fibrosis and normal rest perfusion (fibrosis–/perfusion–). Conclusions First-pass perfusion CMR identifies abnormal rest perfusion in a significant proportion of patients with HCM. These abnormalities are associated with the presence and distribution of myocardial scar and the degree of hypertrophy. Rest perfusion abnormalities identify patients with increased incidence of episodes of non-sustained ventricular tachycardia on Holter monitoring, independently from the presence of myocardial fibrosis.

Myopericarditis, as the First Sign of Rheumatoid Arthritis Relapse, Evaluated by Cardiac Magnetic Resonance

INTRODUCTION Rheumatoid arthritis (RA) affects many organs, including the heart. Cardiac magnetic resonance (CMR) can assess heart pathophysiology in RA. AIM To evaluate, using CMR, RA patients under remission with recent onset of cardiac symptoms. PATIENTS AND METHODS Twenty RA under remission (15F/5M), aged 60±5 yrs, with recent onset of cardiac symptoms (RAH), were prospectively evaluated by CMR. The CMR included left ventricular ejection fraction (LVEF), T2-weighted (T2-W), early (EGE) and late gadolinium enhanced (LGE) images evaluation. Their results were compared with those of 20 RA under remission without cardiac symptoms (RAC) and 18 with systemic lupus erythematosus (SLE) with clinically overt myocarditis. RESULTS Cardiac enzymes were abnormal in 5/20 RAH. CMR revealed inferior wall myocardial infarction in 2/20 (1M, 1F) and myocarditis in 13/20 (8M/5F) RAH. The T2 ratio of myocardium to skeletal muscle was increased in RAH and SLE compared to RAC (2.5 ± 0.05 and 3.4±0.7 vs 1.8 ± 0.5, p<0.001). EGE was increased in RAH and SLE compared to RAC (15 ± 3 and 12±4.7 vs 2.7±0.8, p<0.001). Epicardial LGEs were identified in 10/13 and pericarditis in 6/13 RAH. Coronary angiography, performed in 5 RAH with increased cardiac enzymes, proved a right coronary artery obstruction in 2/5. In 3/5 with CMR positive for myocarditis, coronary arteries were normal, but endomyocardial biopsy revealed inflammation with normal PCR. An RA relapse was observed after 7-40 days in 10/13 RAH with myopericarditis. The one year follow up showed that a) RAH with myocarditis had more disease relapses and b) CHF was developed in 4 RAH with myocarditis. CONCLUSIONS Myopericarditis with atypical presentation, diagnosed by CMR in RA under remission, may precede the development of RA relapse. In 1 year follow up, RA patients with history of myocarditis have a higher frequency of disease relapse and may develop CHF.

Cardiovascular magnetic resonance imaging pattern at the time of diagnosis of treatment naïve patients with connective tissue diseases

BACKGROUND-AIM Cardiac involvement at diagnosis of connective tissue disease (CTD) has been described by echocardiography. We hypothesized that cardio-vascular magnetic resonance (CMR) detects occult lesions at CTD diagnosis. PATIENTS-METHODS CMR was performed early after diagnosis in 78 treatment-naïve CTDs (aged 43±11, 59F/19M) without cardiac involvement [5 Takayasu arteritis (TA), 4 Churg Strauss syndrome (CSS), 5 Wegener granulomatosis (WG), 16 systemic lupus erythematosus (SLE), 12 rheumatoid arthritis (RA), 8 mixed connective tissue diseases (MCTD), 12 ankylosing spondylitis (AS), 3 polymyalgia rheumatica (PMR), 8 systemic sclerosis (SSc) and 5 dermatomyositis (DM)]. Acute and chronic lesions were assessed by T2>2 with positive LGE and T2<2 with positive LGE, respectively. RESULTS In 3/5 TA, 3/4 CSS, 4/5 WG, 10/16 SLE, 9/12 RA, 6/8 MCTD, 4/12 AS, 1/3 PMR, 2/8 SSc and 2/5 DM, the T2 ratio was higher compared to normal (2.78±0.25 vs 1.5±0.2, p<0.01). Myocarditis was identified in 1 TA, 1 SLE, 1 RA, 1 SSc and 2 DM patients; diffuse, subendocardial fibrosis in 1 CSS and 1 RA patient, while subendocardial myocardial infarction in 3 SLE, 1 MCTD, 1 PMR and 2 RA patients. CMR re-evaluation after 6 and 12months of rheumatic and cardiac treatment, available in 28/52 CTDs with increased T2 ratio, showed significant improvement in T2 ratio (p<0.001), non-significant change in LGE extent and normalisation of those with impaired LV function. CONCLUSIONS Occult CMR lesions, including oedema, myocarditis, diffuse subendocardial fibrosis and myocardial infarction are not unusual in treatment naïve CTDs and may be reversed with appropriate treatment.

Quantitative myocardial perfusion imaging by cardiovascular magnetic resonance and positron emission tomography

Recent studies have demonstrated that a detailed knowledge of the extent of angiographic coronary artery disease (CAD) is not a prerequisite for clinical decision making, and the clinical management of patients with CAD is more and more focused towards the identification of myocardial ischemia and the quantification of ischemic burden. In this view, non-invasive assessment of ischemia and in particular stress imaging techniques are emerging as preferred and non-invasive options. A quantitative assessment of regional myocardial perfusion can provide an objective estimate of the severity of myocardial injury and may help clinicians to discriminate regions of the heart that are at increased risk for myocardial infarction. Positron emission tomography (PET) has established itself as the reference standard for myocardial blood flow (MBF) and myocardial perfusion reserve (MPR) quantification. Cardiac magnetic resonance (CMR) is increasingly used to measure MBF and MPR by means of first-pass signals, with a well-defined diagnostic performance and prognostic value. The aim of this article is to review the currently available evidence on the use of both PET and CMR for quantification of MPR, with particular attention to the studies that directly compared these two diagnostic methods.

Evaluation of myocarditis in a pediatric population using cardiovascular magnetic resonance and endomyocardial biopsy

OBJECTIVES To evaluate myocarditis in a pediatric population using cardiovascular magnetic resonance (CMR) and endomyocardial biopsy. METHODS Twenty suspected for myocarditis patients aged 8-16 years and 20 controls were evaluated. CMR was performed using STIR T2-weighted (T2W), early T1-weighted (EGE) and late gadolinium-enhanced images (LGE). Immunohistologic and polymerase chain reaction (PCR) analysis of myocardial specimens were employed in 8/16, who fulfilled the criteria for myocarditis according to clinical and CMR findings. RESULTS Typical clinical, ECG and echocardiographic presentation were identified in 10/16. Troponine I was positive only in 3/16 patients. T2 and EGE in myocarditis were increased compared to controls (2.35 ± 0.5 vs. 1.57 ± 0.13, p<0.001 and 8.5 ± 3 vs. 3.59 ± 0.08, p<0.001, respectively). LGE was found only in 10/16 patients. Endomyocardial biopsy, performed in 8/16 patients with positive CMR, showed positive immunohistology in 2/8 and presence of viral genomes in 6/8 (Herpes, Parvo B19 and Epstein-Barr). Left ventricular ejection fraction (LVEF) was significantly decreased compared to controls (49.6 ± 14.8 vs. 64 ± 0.2, p<0.001). After 6 months, CMR showed normalization of T2, EGE and decreased/or absent LGE. LVEF was normal in all, except two, who remained with low LVEF but in a stable clinical condition. CONCLUSIONS In a small Greek pediatric population with myocarditis, CMR proved useful for the detection of myocarditis, especially in those with negative troponine and mild clinical presentation.

Cardiac magnetic resonance can early assess the presence and severity of heart involvement in Naxos disease.

A 13 yearold boy, without cardiac symptoms, originating from the island of Naxos, Cyclades, Greece, was referred to our department for cardiac magnetic resonance evaluation. Suspicion about Naxos disease (ND) was raised due to his external appearance (wooly hair and palmoplantar keratoderma) and the history of two sudden deaths at young age in his family that were confirmed genetically as homozygotes for a desmoplakin gene mutation, typical of ND. Although the clinical cardiovascular examination was within normal limits, the boy presented wooly hair and palmoplantar keratoderma, typical phenotype of ND. The only abnormal finding on the ECG was the presence of inverted T waves in leads V1 through V3. The 24-hour Holter ECG did not reveal any arrhythmia. Echocardiographic evaluation showed a normally functioning left ventricle (LV). However, a question was raised about a thinning of the free right ventricular (RV) wall with associated moderate dilatation. The patient was further referred for cardiac magnetic resonance (CMR) evaluation. Cine imaging with steady-state free precession sequences (Fig. 1) showed RV dilatation (EDV: 179 ml, normal values: 110–243 ml), ESV: 134 ml, normal values: 46–112 ml) associated to impaired RV systolic function (RVEF: 25%, normal values: 40–60%) (Fig. 1). The LV had normal function and normal volumes (LVEDV: 148 ml, normal values: 101–236 ml, LVESV: 52 ml, normal values: 28– 93 ml, EF: 65%, normal values: 55–70%). Morphological T1-weighted spin-echo images showed an aneurysmatic RV free wall, but without any fatty replacement of myocardium. On late-enhanced images (LGE), 15 min after contrast agent administration, a transmural enhancement of RV wall was observed, due to the presence of fibrosis