A 65 year-old woman with an echodense mitral annular mass.

Caseous calcification of the mitral annulus (CCMA) is a rare variant of mitral annular calcification which usually represents an incidental finding during cardiac imaging. Differential diagnosis from significant lesions such as myocardial abscesses or tumours may be problematic. Herein, we present the case of a 65 year-old woman with CCMA who was managed conservatively. Besides providing useful diagnostic clues, we briefly discuss management issues for this under-recognised clinical entity.

From cell to heart: the impact of the cell organelles dysfunction on heart disease.

: Cellular morphology reflects biologic behavior and activity of the tissue and of the organ also reflects the genetic and molecular biology of the cells themselves. This intermediary position places examination of the cell in a key role to our understanding of the innumerable processes that affect this closely knit chain, from molecules to host. A large volume of the cell is occupied by organelles that come in a variety of shapes and sizes. Organelles are dynamic to maintain homeostasis and adjust to the various functions of the cell. The cardiovascular system is metabolically very active and is therefore particularly vulnerable to defects of the cellular substructures, such as the mitochondrial respiratory chain. Given the functional complexity of the cardiovascular system, it is not surprising that defects in cell organelles produce diverse clinical manifestations. Organelle dysfunction is being recognized as the basis of a wide variety of heart diseases. In this review, the authors discuss the relationship between organelle structure and function in myocardial cells and how these organelles have been linked to the cardiovascular diseases.

Percutaneous transcatheter closure of aorto-right ventricular fistula using the Amplatzer duct occluder.

The occurrence of aorto-right ventricular fistula after an aortic valve replacement is rare. If it remains untreated, this condition can result in heart failure and could thus significantly compromise patient survival. Surgical closure is the treatment of choice; however, transcatheter closure has been attempted with relatively acceptable results. Here, we report on a patient who presented with heart failure with an aorto-right ventricular fistula that was present for nine years following aortic valve replacement. Successful transcatheter closure of the fistula with the use of the Amplatzer duct occluder was performed, suggesting that the percutaneous approach is an efficient technique for the treatment of such fistulae.

Life-threatening Rupture of a Femoral Pseudoaneurysm after Cardiac Catheterization.

A pseudoaneurysm refers to a defect in the arterial wall, allowing communication of arterial blood with the adjacent extra-luminal space. Pseudoaneurysms result from traumatic arterial injury. With the increasing utilization of percutaneous arterial interventions, iatrogenic arterial injury has become the predominant cause of pseudoaneurysm formation. Rupture of the pseudoaneurysm comprises a vascular emergency. Clinical suspicion and imaging techniques are the cornerstones of timely diagnosis and appropriate management of the condition. Herein, we report the case of a 69 year-old woman who suffered a life-threatening profunda femoral artery pseudoaneurysm rupture after a routine cardiac catheterization, that was treated surgically.

Cardiac Dual-source Computed Tomography for the Detection of Left Main Compression Syndrome in Patients with Pulmonary Hyper-tension.

INTRODUCTION: Left Main Compression Syndrome (LMCS) represents an entity described as the extrinsic compression of the left main coronary artery (LMCA) by a dilated pulmonary artery (PA) trunk. We examined the presence of LMCS in patients with pulmonary hypertension (PH) using dual-source computed tomography (DSCT), as a non-invasive diagnostic tool. METHODS: The following parameters were measured: PA trunk diameter (PAD), the distance between PAD and LMCA (LMPA) and the distance between PA and aorta (AoPA). These measurements were related with demographic, echocardiographic, hemodynamic and clinical parameters. Angiography was performed in two patients with LMCS suspected by cardiac computed tomographic angiography. Patients without PH but with angina were examined as controls, using DSCT cardiac angiography to assess the same measurements and to detect the prevalence of coronary artery disease. RESULTS: PA diameter value over 40.00 mm has been associated with PH and LMCS. Furthermore, LMCS did not occur at a distance smaller than 0.50 mm between the PA and the LMCA, and did not correlate with the distance between the PA and the aorta or with cardiac index and NT-proBNP. CONCLUSION: DSCT may represent the initial testing modality in PH patients with dilated PA trunk to exclude LMCS. A periodical rule-out of this rare entity, as assessed by DSCT, in patients with a severely dilated PA seems to be mandatory for PH patients contributing to survival improvement.

First application of coronary flow reserve measurement for the assessment of left main compression syndrome in pulmonary hypertension.

Left main compression syndrome (LMCS) refers to extrinsic compression of the left main coronary artery because of a dilated pulmonary artery trunk. The condition represents an unusual cause of angina, left ventricular dysfunction, and sudden cardiac death in patients with pulmonary hypertension. We present 2 patients with the syndrome who were followed with serial assessments of coronary flow reserve by transthoracic echocardiography to screen for LMCS-related ischemia.

The role of magnetic resonance imaging in the evaluation of transfusional iron overload in myelodysplastic syndromes.

Myelodysplastic syndromes represent a group of heterogeneous hematopoietic neoplasms derived from an abnormal multipotent progenitor cell, characterized by a hyperproliferative bone marrow, dysplasia of the cellular hemopoietic elements and ineffective erythropoiesis. Anemia is a common finding in myelodysplastic syndrome patients, and blood transfusions are the only therapeutic option in approximately 40% of cases. The most serious side effect of regular blood transfusion is iron overload. Currently, cardiovascular magnetic resonance using T2 is routinely used to identify patients with myocardial iron overload and to guide chelation therapy, tailored to prevent iron toxicity in the heart. This is a major validated non-invasive measure of myocardial iron overloading and is superior to surrogates such as serum ferritin, liver iron, ventricular ejection fraction and tissue Doppler parameters. The indication for iron chelation therapy in myelodysplastic syndrome patients is currently controversial. However, cardiovascular magnetic resonance may offer an excellent non-invasive, diagnostic tool for iron overload assessment in myelodysplastic syndromes. Further studies are needed to establish the precise indications of chelation therapy and the clinical implications of this treatment on survival in myelodysplastic syndromes.

Acute coronary syndromes in patients with atrial fibrillation and heart failure. Could novel oral anticoagulants be the solution of the optimal antithrombotic therapy puzzle?

The patients experiencing an acute coronary event are exposed to increased risk of thromboembolic events. That risk becomes substantially greater when AF fibrillation and heart failure are present as well. Dual antiplatelet therapy remains the gold standard in the treatment of patients with ACS. The combination of an oral anticoagulant agent with dual antiplatelet therapy is proven to be more effective in prevention of further antithrombotic events but is followed by increased risks of clinically significant bleeding thus it is not suggested in the treatment of ACS. However, it has been proven beneficial in patients with AF who present with an acute coronary episode. NOACs have proved to be at least as effective as vitamin K antagonists in protecting patients with atrial fibrillation from thromboembolic events without increased risk of major bleeding. However, only data on the effectiveness of NOACS in patients with ACS and AF have been quite contradictory. Even more, the data on the effect of NOACS in patients with concomitant HF and AF who present with an acute coronary event is almost lacking from current bibliography. In this review, we attempt to describe the available data of the use of NOACS in patients with AF and HF who experience an ACS and to address the need for further studies in this area.

Safety and efficacy of novel oral anticoagulants: a comparison to vitamin K antagonists.

Novel oral anticoagulants (NOACs) have been developed as alternatives for vitamin K antagonists (VKAs) for the prevention of thromboembolic events in patients with a variety of medical conditions. In this review, we summarize the current data on NOACs safety and efficacy compared to VKAs and in specific patients' groups including heart valve replacement, venous thromboembolism, advanced renal failure and the elderly.

Novel oral anticoagulants in peripheral arterial and coronary artery disease.

Atherosclerosis comprises of a chronic disease of the vessels which mainly targets the arterial system. The disease's main characteristic is the accumulation of inflammatory cells, lipids, smooth muscle cells and connective tissue within the vascular intima layer. The atherosclerotic lesion can be more accurately defined as a fibro-inflammatory lipid plaque. The pathogenesis of the atherosclerotic plaque is a progressive and additive process that usually occurs over decades. Antiplatelet and anticoagulant agents have been the major elements of large trials since decades, in an attempt to promote the primary and secondary prevention of atherothrombosis. The atherosclerotic plaque rupture and the following thrombosis involve, among others, activation of both platelets and coagulation factors, therefore a potential combination of antiplatelet and anticoagulant therapy, particularly in the setting of secondary prevention has been reconsidered in the light of the newly developed oral anticoagulants.

Acute epigastric and low back pain during amiodarone infusion; is it the drug or the vehicle to blame?

Amiodarone is a Class III antiarrhythmic agent used for cardioversion and prevention of recurrences of atrial fibrillation. However, its use is limited due to its side-effects resulting from the drug's long-term administration. We have described acute epigastric pain following treatment with intravenous amiodarone for atrial fibrillation in a previous report. Hereby, we describe a second patient who suffered acute epigastric pain, as well as one who suffered acute low back pain. Intravenous amiodarone has been related to a series of minor and major adverse reactions, indicating other constituents of the intravenous solution as the possible cause, possibly polysorbate-80. A possible correlation between acute epigastric and low back pain after intravenous amiodarone loading is unproven; however it is of crucial importance for clinicians to be aware of this phenomenon, and especially since an acute epigastric pain is implicated in the differential diagnosis of cardiac ischemia.

Left ventricular pseudoaneurysm formation: Two cases and review of the literature.

Left ventricular wall rupture (LVWR) comprises a complication of acute myocardial infarction (AMI). Acute LVWR is a fatal condition, unless the formation of a pseudoaneurysm occurs. Several risk factors have been described, predisposing to LVWR. High index of suspicion and imaging techniques, namely echocardiography and computed tomography, are the cornerstones of timely diagnosis of the condition. As LVWR usually leads to death, emergency surgery is the treatment of choice, resulting in significant reduction in mortality and providing favorable short-term outcomes and adequate prognosis during late follow-up. Herein, we present two patients who were diagnosed with LVWR following AMI, and subsequent pseudoaneurysm formation. In parallel, we review the aforementioned condition.

Prediction of ventricular arrhythmias using cardiovascular magnetic resonance.

Ventricular tachycardia (VT) is the commonest cause of sudden cardiac death (SCD) in developed countries. Coronary artery disease (CAD) is the most frequent cause of VT in individuals over the age of 30, while hypertrophic cardiomyopathy (HCM), myocarditis and congenital heart disease in those below 30 years of age. Cardiac magnetic resonance (CMR), a non-invasive, non-radiating technique, can reliably detect the changes in ventricular volumes and the ejection fraction that can be predictive of VT/SCD. Furthermore, the capability of CMR to perform tissue characterization and detect oedema, fat and fibrotic substrate, using late gadolinium enhanced images (LGE), can predict VT/SCD in both ischaemic and non-ischaemic cardiomyopathy. The extent of LGE in HCM is correlated with risk factors of SCD and the likelihood of inducible VT. In idiopathic-dilated cardiomyopathy, the presence of midwall fibrosis, assessed by CMR, also predicts SCD/VT. Additionally, in arrhythmogenic right ventricle (RV) dysplasia/cardiomyopathy, CMR has an excellent correlation with histopathology and predicted inducible VT on programmed electrical stimulation, suggesting a possible role in evaluation and diagnosis of these patients. A direct correlation between LGE and VT prediction has been identified only in chronic Chagas' heart disease, but not in viral myocarditis. In CAD, infarct size is the strongest predictor of VT inducibility. The peri-infarct zone may also play a role; however, further studies are needed for definite conclusions. Left ventricle, RV, right ventricular outflow tract (RVOT) function, pulmonary regurgitation and LGE around the infundibular patch and RV anterior wall play an important role in the VT prediction in repaired Tetralogy of Fallot. Finally, in treated transposition of great arteries, the extent of LGE in the systemic RV correlates with age, ventricular dysfunction, electrophysiological parameters and adverse clinical events, suggesting prognostic importance.

The role of magnetic resonance imaging in the evaluation of transfusional iron overload in myelodysplastic syndromes

Myelodysplastic syndromes represent a group of heterogeneous hematopoietic neoplasms derived from an abnormal multipotent progenitor cell, characterized by a hyperproliferative bone marrow, dysplasia of the cellular hemopoietic elements and ineffective erythropoiesis. Anemia is a common finding in myelodysplastic syndrome patients, and blood transfusions are the only therapeutic option in approximately 40% of cases. The most serious side effect of regular blood transfusion is iron overload. Currently, cardiovascular magnetic resonance using T2 is routinely used to identify patients with myocardial iron overload and to guide chelation therapy, tailored to prevent iron toxicity in the heart. This is a major validated non-invasive measure of myocardial iron overloading and is superior to surrogates such as serum ferritin, liver iron, ventricular ejection fraction and tissue Doppler parameters. The indication for iron chelation therapy in myelodysplastic syndrome patients is currently controversial. However, cardiovascular magnetic resonance may offer an excellent non-invasive, diagnostic tool for iron overload assessment in myelodysplastic syndromes. Further studies are needed to establish the precise indications of chelation therapy and the clinical implications of this treatment on survival in myelodysplastic syndromes...