Cardiovascular magnetic resonance detects silent heart disease missed by echocardiography in systemic lupus erythematosus.

Background Accurate diagnosis of cardiovascular involvement in systemic lupus erythematosus (SLE) remains challenging, due to limitations of echocardiography. We hypothesized that cardiovascular magnetic resonance can detect cardiac lesions missed by echocardiography in SLE patients with atypical symptoms. Aim To use cardiovascular magnetic resonance in SLE patients with atypical symptoms and investigate the possibility of silent heart disease, missed by echocardiography. Patients/methods From 2005 to 2015, 80 SLE patients with atypical cardiac symptoms/signs (fatigue, mild shortness of breath, early repolarization and sinus tachycardia) aged 37 ± 6 years (72 women/8 men), with normal echocardiography, were evaluated using a 1.5 T system. Left and right ventricular ejection fractions, T2 ratio (oedema imaging) and late gadolinium enhancement (fibrosis imaging) were assessed. Acute and chronic lesions were defined as late gadolinium enhancement-positive plus T2>2 and T2<2, respectively. Lesions were characterized according to late gadolinium enhancement patterns as: diffuse subendocardial, subepicardial and subendocardial/transmural, due to vasculitis, myocarditis and myocardial infarction, respectively. Results Abnormal cardiovascular magnetic resonance findings were identified in 22/80 (27.5%) of SLE patients with normal echocardiography, including 4/22 with recent silent myocarditis, 5/22 with past myocarditis (subepicardial scar in inferolateral wall), 9/22 with past myocardial infarction (six inferior and three anterior subendocardial infarction) and 4/22 with diffuse subendocardial fibrosis due to vasculitis. No correlation between cardiovascular magnetic resonance findings and inflammatory indices was identified. Conclusions Cardiovascular magnetic resonance in SLE patients with atypical cardiac symptoms/signs and normal echocardiography can assess occult cardiac lesions including myocarditis, myocardial infarction and vasculitis that may influence both rheumatic and cardiac treatment.

Complementary role of cardiovascular imaging and laboratory indices in early detection of cardiovascular disease in systemic lupus erythematosus.

Background Cardiovascular disease (CVD) has been documented in >50% of systemic lupus erythematosus (SLE) patients, due to a complex interplay between traditional risk factors and SLE-related factors. Various processes, such as coronary artery disease, myocarditis, dilated cardiomyopathy, vasculitis, valvular heart disease, pulmonary hypertension and heart failure, account for CVD complications in SLE. Methods Electrocardiogram (ECG), echocardiography (echo), nuclear techniques, cardiac computed tomography (CT), cardiovascular magnetic resonance (CMR) and cardiac catheterization (CCa) can detect CVD in SLE at an early stage. ECG and echo are the cornerstones of CVD evaluation in SLE. The routine use of cardiac CT and nuclear techniques is limited by radiation exposure and use of iodinated contrast agents. Additionally, nuclear techniques are also limited by low spatial resolution that does not allow detection of sub-endocardial and sub-epicardial lesions. CCa gives definitive information about coronary artery anatomy and pulmonary artery pressure and offers the possibility of interventional therapy. However, it carries the risk of invasive instrumentation. Recently, CMR was proved of great value in the evaluation of cardiac function and the detection of myocardial inflammation, stress-rest perfusion defects and fibrosis. Results An algorithm for CVD evaluation in SLE includes clinical, laboratory, ECG and echo assessment as well as CMR evaluation in patients with inconclusive findings, persistent cardiac symptoms despite normal standard evaluation, new onset of life-threatening arrhythmia/heart failure and/or as a tool to select SLE patients for CCa. Conclusions A non-invasive approach including clinical, laboratory and imaging evaluation is key for early CVD detection in SLE.

Cardiovascular magnetic resonance evaluation of paediatric patients with systemic lupus erythematosus and cardiac symptoms.

OBJECTIVES: To evaluate the cardiovascular magnetic resonance (CMR) findings in a paediatric population with systemic lupus erythematosus (SLE) and cardiac symptoms. METHODS: Twenty-five SLE children, aged 10.2 ± 2.6 years, with cardiac symptoms and normal routine non-invasive evaluation were examined by CMR, using a 1.5 T system and compared with sex-matched SLE adults. Left ventricular (LV) volumes, ejection fraction, T2 ratio, early (EGE) and late (LGE) gadolinium enhancement were assessed. Acute and chronic lesions were characterised as LGE-positive plus T2 > 2, EGE > 4 or T2 < 2, EGE < 4, respectively. According to LGE, lesions were characterized as: (a) diffuse subendocardial, (b) subepicardial and (c) subendocardial/transmural, due to vasculitis, myocarditis and myocardial infarction, respectively. RESULTS: LV ejection fraction (LVEF) was normal in all SLEs. T2 > 2, EGE > 4 and positive epicardial LGE wall was identified in 5/25 children. Diffuse subendocardial fibrosis was documented in 1/25. No evidence of myocardial infarction was identified in any children. In contrast, in SLE adults, LGE indicative of myocardial infarction was identified in 6/25, myocarditis in 3/25, Libman-Sacks endocarditis in 1/25 and diffuse subendocardial fibrosis in 2/25. The incidence of heart disease in SLE children was lower compared to SLE adults (p < 0.05), with a predominance of myocarditis in children and myocardial infarction in adults. A significant correlation was documented between disease duration and CMR lesions (p < 0.05). CONCLUSION: CMR identifies a predominance of myocarditis in paediatric SLE with cardiac symptoms and normal routine non-invasive evaluation. However, the incidence of cardiac lesions is lower compared to SLE adults, probably due to shorter disease duration. SIGNIFICANCE AND INNOVATION: CMR identifies heart involvement in a significant percentage of SLE children with cardiac symptoms and normal routine noninvasive evaluation.The incidence of heart disease is lower in SLE children compared with SLE adults.Predominance of myocarditis and myocardial infarction is observed in SLE children and SLE adults, respectively.

The Emerging Role of Cardiovascular Magnetic Resonance Imaging in the Evaluation of Metabolic Cardiomyopathies.

The aim of this review is to discuss the role of Cardiovascular Magnetic Resonance (CMR) in the diagnosis, risk stratification, and follow-up of metabolic cardiomyopathies. The classification of myocardial diseases, proposed by WHO/ISFC task force, distinguished specific cardiomyopathies, caused by metabolic disorders, into 4 types: 1) endocrine disorders, 2) storage or infiltration disorders (amyloidosis, hemochromatosis and familial storage disorders), 3) nutritional disorders (Kwashiorkor, beri-beri, obesity, and alcohol), and 4) diabetic heart. Thyroid disease, pheochromocytoma, and growth hormone excess or deficiency may contribute to usually reversible dilated cardiomyopathy. Glucogen storage diseases can be presented with myopathy, liver, and heart failure. Lysosomal storage diseases can provoke cardiac hypertrophy, mimicking hypertrophic cardiomyopathy and arrhythmias. Hereditary hemochromatosis, an inherited disorder of iron metabolism, leads to tissue iron overload in different organs, including the heart. Cardiac amyloidosis is the result of amyloid deposition in the heart, formed from breakdown of normal or abnormal proteins that leads to increased heart stiffness, restrictive cardiomyopathy, and heart failure. Finally, nutritional disturbances and metabolic diseases, such as Kwashiorkor, beri-beri, obesity, alcohol consumption, and diabetes mellitus may also lead to severe cardiac dysfunction. CMR, through its capability to reliably assess anatomy, function, inflammation, rest-stress myocardial perfusion, myocardial fibrosis, aortic distensibility, iron and/or fat deposition can serve as an excellent tool for early diagnosis of heart involvement, risk stratification, treatment evaluation, and long term follow-up of patients with metabolic cardiomyopathies.

The diagnostic role of cardiac magnetic resonance imaging in detecting myocardial inflammation in systemic lupus erythematosus. Differentiation from viral myocarditis.

OBJECTIVE: The objective of this paper is to evaluate the diagnostic role of cardiac magnetic resonance imaging (CMR) in detecting myocardial inflammation in systemic lupus erythematosus (SLE) and its differentiation from viral myocarditis. PATIENTS AND METHODS: Fifty patients with suspected infective myocarditis (IM), with chest pain, dyspnoea or altered ECG, increase in troponin I and/or NT-pro BNP, with or without a history of flu-like syndrome or gastroenteritis and elevated C-reactive protein (CRP) within three to five (median four) weeks before admission, 25 active SLE patients, aged 38 ± 3 years, and 20 age-matched controls were prospectively evaluated by clinical assessment, ECG, echocardiogram and CMR. All patients underwent coronary angiography, and those with significant coronary artery disease (CAD) were excluded. CMR was performed using STIR T2-W (T2W), early T1-W (EGE) and late T1-W (LGE). Endomyocardial biopsies were performed when clinically indicated by current guidelines. Specimens were examined by immunohistological and polymerase chain reaction (PCR) analysis. RESULTS: Positive coronary angiography for CAD excluded 10/50 suspected IM and 5/25 active SLE. Positive clinical criteria for acute myocarditis were fulfilled by 28/40 suspected IM and only 5/20 active SLE. CMR was positive for myocarditis in 35/40 suspected IM and in 16/20 active SLE. Endomyocardial biopsy (EMB), performed in 25/35 suspected IM and 7/16 active SLE with positive CMR, showed positive immunohistology in 18/25 suspected IM and 3/7 active SLE. Infectious genomes were identified in 24/25 suspected IM and 1/7 active SLE. CONCLUSIONS: CMR-positive IM patients were more symptomatic than active SLE. More than half of CMR-positive patients also had positive EMB. PCR was positive in almost all IM, but unusual in SLE. Due to the subclinical presentation of SLE myocarditis and the limitations of EMB, CMR presents the best alternative for the diagnosis of SLE myocarditis.

Pathophysiology of Q waves in II, III, avF in systemic lupus erythematosus. Evaluation using cardiovascular magnetic resonance imaging.

OBJECTIVES: To investigate the pathophysiology of Q waves in II, III, avF in systemic lupus erythematosus (SLE) by cardiovascular magnetic resonance (CMR). METHODS: Inflammation evaluation by CMR using T2, early (EGE) and late gadolinium enhanced images (LGE) was performed in 20 SLE patients with mild cardiac symptoms and Q in leads II, III, avF of ECG. Their results were compared with 20 SLE patients with the same symptoms and normal ECG. RESULTS: In both groups, T2, EGE and left ventricular ejection fraction were normal. However, in 3/20 with Q in II, III, avF, CMR revealed lesions indicative of acute myocarditis. In the rest of them, CMR documented transmural LGE, due to past inferior myocardial infarction in 4/20 and epicardial LGE due to past myocarditis in 8/20 (4/8 in the inferior and 4/8 in the lateral wall of left ventricle). No LGE was found in 5/20 and the Q was attributed to the position of the heart. In 3/20 with normal ECG, CMR detected past myocarditis in 2/3 and myocardial infarction in 1/3. Coronary angiography assessed coronary artery disease in all SLE with evidence of myocardial infarction and normal coronaries in 9/10 patients with past myocarditis. CONCLUSION: Q in II, III, avF in SLE may indicate myocardial infarction, acute or past inflammation or be a positional finding. The lack of Q does not exclude the possibility of infarction or inflammation. CMR is the best tool to reveal the pathophysiology of Q waves in SLE and guide treatment of heart involvement in these patients.

Cardiovascular magnetic resonance in autoimmune rheumatic diseases: a clinical consensus document by the European Association of Cardiovascular Imaging.

Autoimmune rheumatic diseases (ARDs) involve multiple organs including the heart and vasculature. Despite novel treatments, patients with ARDs still experience a reduced life expectancy, partly caused by the higher prevalence of cardiovascular disease (CVD). This includes CV inflammation, rhythm disturbances, perfusion abnormalities (ischaemia/infarction), dysregulation of vasoreactivity, myocardial fibrosis, coagulation abnormalities, pulmonary hypertension, valvular disease, and side-effects of immunomodulatory therapy. Currently, the evaluation of CV involvement in patients with ARDs is based on the assessment of cardiac symptoms, coupled with electrocardiography, blood testing, and echocardiography. However, CVD may not become overt until late in the course of the disease, thus potentially limiting the therapeutic window for intervention. More recently, cardiovascular magnetic resonance (CMR) has allowed for the early identification of pathophysiologic structural/functional alterations that take place before the onset of clinically overt CVD. CMR allows for detailed evaluation of biventricular function together with tissue characterization of vessels/myocardium in the same examination, yielding a reliable assessment of disease activity that might not be mirrored by blood biomarkers and other imaging modalities. Therefore, CMR provides diagnostic information that enables timely clinical decision-making and facilitates the tailoring of treatment to individual patients. Here we review the role of CMR in the early and accurate diagnosis of CVD in patients with ARDs compared with other non-invasive imaging modalities. Furthermore, we present a consensus-based decision algorithm for when a CMR study could be considered in patients with ARDs, together with a standardized study protocol. Lastly, we discuss the clinical implications of findings from a CMR examination.

Adenosine combined with dynamic exercise for myocardial perfusion imaging.

OBJECTIVES: This study investigated whether combining exercise with adenosine would reduce the adverse effects of adenosine vasodilation. BACKGROUND: Adenosine vasodilation is effective for perfusion imaging but causes frequent unpleasant noncardiac adverse effects, high noncardiac tracer uptake and occasional arrhythmias. METHODS: Of 500 consecutive patients referred for thallium-201 myocardial perfusion imaging, 407 were randomized to three study groups: 6 min of adenosine infusion alone; 6 min of adenosine with submaximal exercise; or symptom-limited exercise with continuous adenosine. Minimal detectable differences are presented; a significance level of 0.05 with a power of 80% is assumed. RESULTS: There was no difference among the three groups in sensitivity and specificity (overall 96% and 78%, minimal detectable differences 5.5% and 11%, respectively) for detection of coronary artery disease or stenosis in individual coronary arteries. There was a trend toward improved sensitivity in the combined exercise groups compared with that in the adenosine-only group (98% vs. 93%, p = 0.07, minimal detectable difference 6%). Noncardiac side effects were reduced by 43% in the exercise groups (p < 0.0001), and major arrhythmias were reduced by 90% (p < 0.0001). There was no effect on minor arrhythmias (25% vs. 22%, p = 0.6, minimal detectable difference 12%). The heart/background ratios were higher in the exercise groups (all p < 0.02). Each ratio was correlated with the exercise level achieved (all p < 0.001). The reversibility score increased with exercise (p = 0.04), as did the number of patients and segments with reversible defects (both p = 0.03). CONCLUSIONS: Combining exercise with adenosine infusion reduced the noncardiac side effects of vasodilation and major arrhythmias while improving redistribution and heart/background ratios. These findings may be clinically important. Although maximal exercise with adenosine infusion produced optimal results, the improvement over the submaximal exercise protocol was minor, and this has the advantage of being simple and achievable within the normal 6-min duration of the adenosine infusion.

Thallium myocardial perfusion tomography using intravenous dipyridamole combined with maximal dynamic exercise.

Exercise thallium imaging is effective and provides information on exercise tolerance and symptoms, but is less sensitive if exercise is submaximal. When dipyridamole alone is used for such patients, the exercise parameters are lost and high background uptake results. This study examines dipyridamole combined with maximal exercise, to capitalize on the strengths of both techniques. Thallium tomography was performed in 122 patients with dipyridamole (group 1), exercise (group 2), or dipyridamole with maximum exercise (group 3). In patients with coronary artery disease, thallium tomography was abnormal in 92, 93 and 95% (P = ns), with reversible ischaemia in 82, 81 and 84% (P = ns) in the three groups, respectively. Among patients in groups 2 and 3 who exercised submaximally, detection of individual arterial stenoses was improved in group 3 (P < 0.05) and larger defects were found (2.4 versus 4.2 segments, P < 0.01). In group 3, no serious complications occurred and fewer vasodilatory side effects were seen than in group 1 (P < 0.01). The heart to splanchnic count ratio in groups 2 and 3 (1.61 versus 1.65, P = ns) was significantly greater than in group 1 (1.28, P < 0.0001). Compared with exercise alone, additional dipyridamole improved diagnostic accuracy in patients with limited exercise potential. Compared with dipyridamole alone, additional exercise provided useful exercise information, reduced vasodilatory side effects and improved scan quality. Combined dipyridamole and exercise stress is safe, and could be used routinely, but a larger study is required to establish if there is an overall diagnostic improvement.

Telecardiology services in the Aegean islands.

Telecardiology services established between the Onassion Cardiac Surgery Centre in Athens and the Primary Health Care Centre (HCC) of Naxos in the Aegean provide 24 h support. During the testing period more than 50 cases were analysed, diagnosed and treated in Naxos HCC, under the supervision of the on-call cardiologists of the Onassion Cardiac Surgery Centre. One patient with an acute myocardial infarction was evacuated to a tertiary hospital in Athens. The services are modelled in a way that they could be applied to five more HCCs in the Aegean. Emphasis is being put on the customization of the electronic medical records for facilitating the work of the primary-care physicians to cope with cardiology emergencies and long-term follow-up of cardiac patients.

Supervision of thrombolysis of acute myocardial infarction using telemedicine.

The treatment of acute myocardial infarction (MI) constitutes a significant problem in remote geographical areas of Greece. Furthermore, thrombolysis, the treatment of choice in the early phase of acute MI, requires the supervision of an expert. We have used thrombolytic treatment, using telemedicine, in remote medical centres. The Onassis Cardiac Surgery Centre was linked to six remote Aegean islands via telemedicine systems which permitted the transmission of 12-lead electrocardiograms (ECGs). The thrombolytic agent anistreplase was administered to patients with acute MI. Supervision, including consultation for treatment of complications, was achieved using the telemedicine system. One hundred and fifty-two ECGs were transmitted during 24 months, of which 108 (71%) indicated specific treatment of a cardiac condition. Ten cases were diagnosed as having acute MI and eight of these were treated with anistreplase. All patients survived acute MI and complications were treated locally. The application of thrombolytic treatment in acute MI is feasible in remote areas, with the use of a telemedicine system.

Transmission of electrocardiograms from a moving ambulance.

Delay is the enemy for patients with acute myocardial infarction. It would be helpful for the hospital cardiologist to interpret the patient's electrocardiogram (ECG) before the arrival of the ambulance. The aim of our study was to determine whether ECG transmission from an ambulance is feasible and to assess the time savings. An ambulance was equipped with an ECG recorder, which was connected to a notebook computer and coupled to a cellular telephone for transmission to a hospital-based station. Paramedics needed 2 min (SD 0.5) to record the ECG on the move and 34 s (SD 14) to transmit it. The ambulance arrived 15.5 min (SD 6.5) after reception. The time between arrival and ECG diagnosis, for a control group patient, was approximately 9.5 min (SD 3.5). Therefore, pre-hospital ECG diagnosis took place 25 min (SD 7.5) before in-hospital diagnosis. We conclude that ECG transmission from a moving ambulance is feasible, reduces in-hospital delays and allows faster triage in critical cardiac cases.

Urgent response telecardiology services and training--collaboration between Onassis Cardiac Surgery Hospital and Aegian Islands' Health Care Centers.

Since 1995, Onassis Cardiac Surgery Center participates in the development of Telecardiology Services in Greece. Existing connections include the islands Skiathos, Naxos, Santorini, Mykonos, Milos, Plomari and Amorgos. First hundred fifty three (153) cases have been already analysed. Eleven of them were acute myocardial infarctions and have been treated by thrombolytic medication (anistreplase). The participation of physicians and nurses was valuable for the success of the program and their further involvement in the new area of Telemedicine. The purpose of the project was to offer high quality cardiac services in remote area patients and to provide specific training in telemedicine issues to health care professionals.

Body mass index, lipid metabolism and estrogens: their impact on coronary heart disease.

Estrogens influence lipid metabolism and body fat distribution in women. Premenopausal women have increased lipoprotein lipase action in abdominal and femoral subcutaneous fat compared with men of the same age. Estrogens may also affect adipose tissue either directly through specific estrogen receptors or indirectly via their effects on other tissues. As adipose tissue produces several cytokines including leptin, adiponectin and interleukin-6, estrogens may alter their levels, thus influencing various biological processes. Lack of estrogens such as in menopause, causes an increase in visceral adiposity, leading to changes in lipid and lipoprotein metabolism. Due to those alterations, postmenopausal women are more prone to coronary heart disease. In this review the influence of estrogens on body mass index, lipid metabolism and some of the therapeutic options will be analyzed.

Sudden cardiac death and diabetes mellitus.

Sudden cardiac death (SCD) affects a significant percentage of diabetic patients. SCD in these patients can be due to several factors, such as diastolic dysfunction, heart failure, altered platelet function, inflammation, sympathetic nervous stimulation and other factors. In the present review, we discuss the association between diabetes mellitus and SCD.

Microscopic polyangiitis and Kawasaki disease without overt clinical cardiovascular manifestations and with abnormal cardiovascular magnetic resonance findings.

Abnormal cardiovascular magnetic resonance findings were found in a patient with microscopic polyangiitis and a patient with Kawasaki disease that presented without overt clinical cardiovascular manifestations.

Detection of myocardial inflammation by contrast-enhanced MRI in a patient with Churg-Strauss syndrome.

This case report emphasizes the application of a combined CMR protocol for the diagnosis of acute myocardial inflammation and fibrosis in CSS.