Jan Kuchař, David Ambrož, Regina Votavová, Radim Bečvář, Michael Aschermann, Pavel Jansa, Aleš Linhart
Introduction and aim: Systemic scleroderma is the most common cause of pulmonary arterial hypertension (PAH)
among systemic diseases. Therefore, the diagnostic strategy is oriented to the effort to detect the disease at its early
stage, which is unambiguously more promising therapeutically. The aim of this work is to describe the real clinical practice
in the PAH diagnosis associated with systemic scleroderma in the Czech Republic (CR), where systematic echocardiographic
screening was introduced in 2007.
Methodology: In 2007, annual systematic echocardiographic PAH screening was introduced in the Czech Republic for
systemic scleroderma. Until then, patients used to be checked unsystematically. In the presence of echocardiographic
signs of pulmonary hypertension patients without significant impairment of pulmonary interstitium and left heart were
examined using catheterisation. Definitive evaluation of diagnosed PAH also included BNP (brain natriuretic peptide)
examination and six-minute walking test (6MWT). Alternatively, the PAH diagnosis was assessed in patients presenting
with symptoms, particularly shortness of breath, or within the new diagnosis of systemic scleroderma.
Results: By 2015, a total of 582 systemic scleroderma patients were examined using transthoracic echocardiography in
order to rule out or confirm pulmonary hypertension. A total of 66 of these patients were indicated for right heart catheterisation.
In 10 cases, pulmonary hypertension detected during screening led to PAH diagnosis. At the time of diagnosis,
these patients had lower pulmonary vascular resistance, lower BNP concentration, and a longer distance achieved
at 6MWT than patients diagnosed for symptoms.
Conclusion: In the case of detecting pulmonary hypertension during screening, the diagnosis of PAH associated with
systemic scleroderma is characterised by more favourable hemodynamics, lower BNP concentration, and longer distance
achieved at 6MWT. However, the above does not involve for sure the disease diagnosis at an early stage. This
can apparently be approached by combining echocardiography with other methods, in particular by determining BNP.