Right-heart failure after right heart catheterization in a patient with scleroderma and suspected pulmonary hypertension

摘要

The case presented here describes the fatal outcome of a right heart catheterization of a female patient with scleroderma. At autopsy, a massive fibrosis of the cardial muscle, the atrioventricular, and the sinoatrial node was described. Patients with scleroderma are prone to cardiac involvement and have an increased risk of sudden cardiac death. The discussion of this case reflects on identifiable risk factors for cardiac complications in scleroderma. These are the parallel affection of the skeletal musculature, the presence of ventricular ectopies and a dilated right atrium and pericardial effusions. Physicians should be aware of the fact that patients with advanced cardiac fibrosis may be at higher risk of complications in relation with invasive procedures.