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Systemic sclerosis involving the heart
  1. jccmoon{at}

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A 39 year old woman with systemic sclerosis (scleroderma) presented with four months ofdyspnoea. Initial investigation including coronary angiography, echocardiography, and cardiovascular magnetic resonance (CMR) without contrast were normal. Over three months she became more breathless and developed arrhythmias. CMR was repeated with contrast. The right ventricle was now dilated with poor function. There was reduced left ventricular function and bilateral pleural effusions were present. The myocardium enhanced greatly with gadolinium at 20 minutes in a heterogeneous pattern, suggesting a patchy process such as fibrosis or infiltration (below left). One month later, despite treatment, the patient entered a terminal low output state with malignant arrhythmias. At postmortem examination the myocardium was grossly abnormal. Large islands of normal myocardium were interspersed with areas of myocardial oedema with myocyte loss, while other areas showed extensive sheets of fibrosis (below right).

Systemic sclerosis is an uncommon cause of cardiomyopathy, although cardiac involvement in systemic sclerosis may be under recognised clinically. Vasospasm and fibrosis occur throughout the body and in the heart this may result in patchy perfusion defects, diastolic dysfunction, arrhythmias, and systolic cardiac failure. In this case, heterogeneous myocardial gadolinium contrast enhancement correlated with postmortem findings of patchy myocardial oedema and fibrosis with interspersed normal myocardium. Late contrast enhancement with gadolinium is known to characterise fibrosis and necrosis in myocardial infarction and ischaemic cardiomyopathy. These images show the potential of the technique in other cardiomyopathies and for the assessment of cardiac systemic sclerosis.