International journal of cardiology
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Mucopolysaccharidosis type VI (MPS VI, Maroteaux-Lamy syndrome) is an inborn error of metabolism, with incidences at birth ranging from 1 in 1.5 million to 1 in 43,000 live births. This disorder is rarely considered when evaluating patients with common populational cardiovascular diseases. A significant number of MPS VI patients, however, do present cardiovascular disease and MPS VI should be considered as a potential differential diagnosis for other cardiovascular disorders. ⋯ An early diagnosis is difficult due to joint stiffness, respiratory system involvement or skeletal malformations that limit exercise capacity and mask the underlining heart failure. This article is supposed to serve as a very practical reference for cardiologists who may come across MPS VI in their daily practices. A greater awareness of cardiovascular manifestations of MPS VI among cardiologists can help to reduce misdiagnosis and promote early detection of this inborn disorder and aid the implementation of adequate therapy at the earliest stage possible which is crucial for its efficacy.
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The usual diagnostic work-up of chest pain patients includes clinical risk profiling and exercise-ECG, possibly followed by additional tests. Recently cardiac computed tomographic angiography (CCTA) has been employed. We evaluated the prognostic value of the combined use of exercise-ECG and CCTA for the development of cardiovascular endpoints. ⋯ The combined subsequent use of exercise-ECG for functional information and CCTA for anatomical information provides a high diagnostic yield in stable chest pain patients with an intermediate pre-test probability for coronary artery disease.
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Biography Historical Article
Atheroprotector role of the spleen based on the teaching of Avicenna (Ibn Sina).
Many studies have proven atherosclerosis is an inflammatory immune disease. The spleen plays an important immune role in the human body. Splenectomy is often used in several clinical disorders; but recent studies have shown that splenectomy may be effective in the development of atheroma lesions. ⋯ Therefore any disruption in the function of the spleen can lead to various diseases such as atherosclerosis. Based on his description, it is clear that Ibn Sina first described the role of spleen in prevention of atherosclerosis. In this review, we discuss the Avicenna (Ibn Sina) aspect of atheroprotector role of the spleen.
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Extracorporeal membrane oxygenation (ECMO) provides excellent support to circulation. However, ECMO capacity to effectively assist the heart, and in particular the left ventricle, is limited. We herein review the modifications of left ventricular dynamics during peripheral ECMO, highlight the importance of left ventricle venting and describe a method for the conversion of a femoro-femoral ECMO into a paracorporeal left ventricular assist device.