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Editorials

Coronary Microvascular Dysfunction in Patients with Cardiomyopathies

Paolo G Camici, MD, FESC, FRCP

From the Medical Research Council Clinical Sciences Centre and National Heart and Lung Institute, Imperial College, London, United Kingdom.

Correspondence to Paolo G. Camici, MD, FESC, FRCP, Medical Research Council Clinical Sciences Centre, Hammersmith Hospital, Du Cane Road, London W12 0NN, United Kingdom. E-mail paolo.camici@csc.mrc.ac.uk

Key Words: heart diseases • microcirculation • myocardium • pathology

An extract of the first 250 words of the full text is provided, because this article has no abstract.

Anginal symptoms and electrocardiographic changes suggestive of myocardial ischemia, despite angiographically normal coronary arteries, are common in patients with cardiomyopathies and those with left ventricular hypertrophy secondary to pressure overload. Studies using positron-emission tomography (PET) to measure regional myocardial blood flow have demonstrated that maximum myocardial blood flow and coronary flow reserve are severely blunted in patients with hypertrophic as well as dilated cardiomyopathy.^1,2 In the absence of epicardial stenoses, blunted maximum myocardial blood flow and coronary flow reserve are suggestive of coronary microvascular dysfunction.^3,4 Furthermore, it has been demonstrated that in both hypertrophic and dilated cardiomyopathy, the severity of coronary microvascular dysfunction, assessed by measuring myocardial blood flow with PET, is an independent predictor of prognosis.^2,5 In patients with cardiomyopathies, coronary microvascular dysfunction can be sustained by a number of different pathogenetic mechanisms. These include structural and functional abnormalities of the intramural arterioles as well as extravascular mechanisms (eg, increased extramural compression).⁴

Article 161

Anderson-Fabry disease (AFD) is an X-linked deficiency of lysosomal -galactosidase A.⁶ This deficiency results in multiorgan damage from glycosphingolipid deposition, leading to renal, cardiac, and cerebrovascular disease and premature death. Patients with AFD complain of angina despite angiographically normal coronary arteries. Recent studies have also shown that there is progressive deterioration in left ventricular systolic function and myocardial scarring in patients with AFD cardiomyopathy. A number of mechanisms may contribute to microvascular dysfunction in these patients. AFD cardiomyopathy is characterized by globotriaosyl-ceramide (Gb₃) and related glycosphingolipid deposition in myocytes, conduction tissue, vascular endothelium, and . . . [Full Text of this Article]