Pulmonary arterial hypertension (PAH) is a common complication of systemic sclerosis (SSc). Symptoms of coronary artery disease (CAD) and PAH are closely related and cardiac catheterization is needed to confirm their diagnosis.
Objectives: Investigation of the extent of overlap between CAD and PAH in patients with SSc. Methods: Based on non-invasive investigations, 20 patients out of 120 were supposed to have PAH ('suspected PAH' group). Another 10 patients showed the signs of coronary disease ('suspected CAD' Group). In these 30 patients, both right heart catheterization and coronary angiography were performed, and the coronary flow reserve (CFR) was assessed by thermodilution technique.
Results: In the 'suspected PAH' and the 'suspected CAD' groups, PAH was found in 12/20 and 2/10 cases, coronary artery stenosis was in 9/20 and 6/10 cases. Severely reduced CFR was revealed in 7/20 and 3/10 cases, respectively.
Conclusions: PAH, CAD, and reduced CFR show a considerable overlap in symptomatic SSc patients. The current non-invasive investigations are neither sensitive nor specific enough to make an appropriate distinction between these different disease manifestations. A more invasive approach, such as coronary angiography at the initial catheterization, is required to properly characterize and treat the different forms of cardiac involvement in SSc.