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Background: Heterozygous familial hypercholesterolemia (heFH) is a genetic disorder leading to premature coronary artery disease (CAD). We hypothesized that the subclinical pathophysiologic consequences of hypercholesterolemia may be detected before the occurrence of clinically overt CAD by stress testing and myocardial strain imaging. Patients-methods: We evaluated the treadmill tests (ETTs) of 46 heFH men without known arterial hypertension/diabetes mellitus/vasculopathy like CAD and of 39 healthy men matched for age, baseline systolic/diastolic blood pressure (BP) and heart rate (HR), using Bruce protocol. Global longitudinal strain (GLS) of the left ventricle (LV) additionally to ejection fraction was obtained. Results: heFH men reached a significantly higher peak systolic and diastolic BP compared to controls (p = 0.002 and p

This case reports on an 8-year-old boy with homozygous familial hypercholesterolemia with large tuberous xanthomas over his hands, elbows, buttocks, knees, and feet. Lomitapide 40 mg daily (steadily increased) was added to his classical lipid-lowering therapy. A 50% reduction in the thickness, hardness, size, and color intensity of xanthomas was reported after 2 years of treatment. (Level of Difficulty: Intermediate.)

Although the risk of MRI scanning on patients with conventional devices is lower than initially thought, the patient's safety can only be guaranteed when using MRI-conditional devices. The most important modifications in MRI-conditional devices include a) Reduction in ferromagnetic components to reduce magnetic attraction and susceptibility artifacts; b) Replacement of the reed switch by a Hall sensor in order to avoid unpredictable reed switch behavior; c) Lead coil design to minimize lead heating and electrical current induction; d) Filter circuitry to prevent damage to the internal power supply; and e) Dedicated pacemaker programming to prevent inappropriate pacemaker inhibition and competing rhythms. Although many companies claim to have MRI-conditional devices, adoption in clinical practice is limited because a) Not all companies have MRI-conditional devices approved for both 1.5 and 3T; b) Not all companies offer the option of unlimited MRI scanning (without an exclusion zone in the thorax); c) Certain companies allow only a 30-min MRI scanning and only in afebrile patients; and d) Despite having MRI-conditional pacemakers, certain companies do not have MRI-conditional defibrillators and CRT systems. It is clear that this new technology opens the door for MRI to a growing number of patients; however, the widespread adoption of MRI-conditional devices will depend on real-life issues, such as cost, clinical indications for such a device and the permanent education of health care professionals.

Patients who experience acute coronary syndrome (ACS) are at increased risk of new cardiovascular (CV) events. The main strategies for prevention of recurrence of CV events is the protection from ruptured plaque, thrombus formation, occlusion or downstream embolization in the coronary artery. The percutaneous coronary intervention (PCI) with stenting and anticoagulants, 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase inhibitors (HMGCoAi, commonly called statins) and neurohormonal inhibition, has led to a notable decrease in 1-year mortality events. Today it is well documented that all patients with an ACS should be treated early, intensively and continuously for lowering the LDL-C values to the recommended goals. Regularly interviewing by trained health care personnel and post-discharge follow-up of patients after ACS seems to be more effective concerning adherence to statin for achieving LDL-C treatment goals compared with the standard of care. Keywords: Acute coronary syndrome, Low density lipoprotein cholesterol, Statins, Adherence

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.