Outcome measures for heart involvement in systemic sclerosis

Primary myocardial involvement is common in SSc. Increasing evidence strongly suggests that this involvement is related to repeat focal ischaemic injury causing irreversible myocardial fibrosis. Clinically evident cardiac involvement is recognized to be a poor prognostic factor, thus pre-clinical identification is highly encouraged. Echocardiography, if possible coupled to pulsed tissue Doppler, is the cornerstone of heart assessment even if radionuclide ventriculography remains the gold standard for evaluation of ventricular function. Myocardial perfusion may be assessed by single photon emission CT, but cardiac MRI will probably supplant this technique; it, furthermore, offers the possibility to concomitantly determine, ventricular function, myocardial perfusion and tissular parameters (i.e. myocarditis or burden of fibrosis). Conduction system abnormalities are common but not serious, while arrhythmias may be life-threatening, necessitating 24-h ambulatory Holter ECG. Natriuretic peptides have been used mainly when the heart has been involved secondary to pulmonary arterial hypertension, but may also be useful for the identification of early heart dysfunction. Their predictive value should also be investigated when there is primary heart involvement.