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The following electronic only articles are published in conjunction with this issue of Heart.
Williams syndrome associated with complete atrioventricular septal defect
S.Nakamoto, T Saga, T Shinohara
Williams syndrome is a genetic disorder associated with characteristic facies, supravalvar aortic stenosis, peripheral pulmonary stenosis, mental retardation, hypertension, premature aging of skin, and congenital cardiac defects. Many cardiac defects such as bicuspid aortic valve, mitral valve regurgitation, coarctation of the aorta, and ventricular or atrial septal defects are linked to the syndrome. Complete atrioventricular septal defect has rarely been associated with Williams syndrome and only one necropsy case has been reported in the literature. The long term follow up of Williams syndrome associated with complete atrioventricular septal defect is reported. During a 10 year follow up period, the pressure gradient in the ascending aorta did not increase despite narrowing of the ascending aorta as identified on an aortogram.
Innocent victim of a localised outbreak: legionella endocarditis
R Massey, P Kumar, J R Pepper
Legionella pneumophila endocarditis is extremely rare. The case of a fit 26 year old man who had previously undergone homograft aortic root replacement is reported. He was admitted with legionella pneumonia during the recent localised outbreak but went on to develop endocarditis. His aortic valve was replaced with a mechanical valve and he made an uneventful recovery. Public health issues and diagnosis in susceptible patients during localised outbreaks are discussed.
Trash feet after coronary angiography
A M Khan, S Jacobs
Cholesterol crystal embolisation is a frequently underdiagnosed condition. While coronary catheterisation is safe and commonly performed, the reported patient developed very painful trash feet after undergoing this routine procedure. Ulceration and gangrene occurred after catheter manipulation during cardiac angiography, which caused occlusion of the small arteries in his feet. The triad of pain, livedo reticularis, and intact peripheral pulses is pathognomonic for cholesterol embolisation. The prognosis depends on the extent of the systemic disease and a high rate of mortality (75–80%) is observed. Prognosis is poor and the treatment is only supportive. It is suggested that while cardiac catheterisation is largely safe and a very commonly performed procedure, it can still lead to complications with serious side effects and can even prove fatal.
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