[Initial disorders of pulmonary hemodynamics in patients with systemic sclerosis: the relevance of the new hemodynamic definition of pulmonary arterial hypertension].

AIM: To investigated the impact of the new hemodynamic definition of pulmonary arterial hypertension (PAH) as proposed by the 6th PH World Symposium on the phenotypes, outcome and survival in patients with systemic sclerosis (SSc).

MATERIALS AND

METHODS

We retrospectively analyzed consecutive SSc patients included in our Center for a first right-heart catheterization between 2011 and 2019. The association between hemodynamics' phenotype and outcome and survival was studied.

RESULTS

We included 33 SSc patients, who had a high risk of PAH development with mPAP< 25 mmHg and 7 with early PAH I-II FC. Among them, 6 had a baseline mPAP value between 21 and 24 mmHg and PVR≥2 mmHg without interstitial lung disease and left heart disease, and were reclassified as PAH; only 1 of which raised their mPAP≥25 mmHg during 123 months follow-up. In 18 patients with mPAP< 20 mmHg; 1 of which raised their mPAP≥25 mmHg during 157 months follow-up. In 8 patients with unclassified PH mPAP didn't raise during 123 months of follow-up. The 5-year survival rate was 83% in the group with unconfirmed PH, 100% in the group with unclassified PH, and 88% in those with early PAH. With classical PAH-SSс I-II FC, according to the old hemodynamic criteria, the 5-year survival rate was 100%. No significant differences were found. There were also no differences in survival during the entire follow-up period. We did not observed relation between mPAP, PVR and outcome and mortality.

CONCLUSION

Among patients with the PAH phenotype of SSc who do not have interstitial lung disease or left heart disease, those with early PAH can be found, although the frequency and rate of progression to classical PAH at these stages are very slow.