Predictors of right atrial dilatation and long-term function after right ventricular outflow tract surgical repair: Quantification of restrictive physiology matters

Right diastolic dysfunction; Right atrium function; Restrictive physiologyDisfunción diastólica derecha; Función de la aurícula derecha; Fisiología restrictivaDisfunció diastòlica dreta; Funció de l'aurícula dreta; Fisiologia restrictivaRight ventricular (RV) diastolic dysfunction in patients with a surgically-repaired RV outflow tract (RVOT) obstruction merits further studies. Right atrial (RA) dilation and function may be related to (RV) diastolic dysfunction in this setting. The end-diastolic forward flow (EDFF) in the pulmonary artery (PA) has been suggested as a non-invasive marker of poor RV compliance, however, there is controversy regarding its true significance; EDFF quantification may help elucidate this controversy. Objective to study predictors of RA enlargement and dysfunction in patients with a surgically-repaired RVOT obstruction and its relationship with quantitative EDFF. Methods In 81 consecutive patients (mean age: 37.5 (±7) years), transthoracic echocardiography (Echo) and cardiac magnetic resonance (CMR) were performed. Echo parameters: RA size (indexed RA area (iRAA)), RA function (RA global strain (RAGS)) and maximum EDFF velocity-time integral (VTI-EDFF) obtained during a whole respiratory cycle. CMR-indexed RA area (imRAA) was also obtained. Patients were divided into three groups according to iRAA, imRAA and RAGS; bivariate analysis was performed. A multivariate model was then applied using variables that were found to be statistically significant in the bivariate analysis. Results Upon multivariate analysis, higher VTI-EDFF values and the presence of significant tricuspid regurgitation proved to be independent factors associated with increased iRAA and imRAA and lower RAGS, whereas RV volumes, function and pulmonary regurgitant fraction were not. Conclusion VTI-EDFF linearly correlated with the degree of RA dilation and deformation; EDFF quantification as against qualitative assessment may be considered a non-invasive tool for diastolic RV dysfunction

Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension

To assess severity markers and outcomes of patients with systemic sclerosis (SSc) with or without pulmonary arterial hypertension (PAH-SSc/non-PAH-SSc), and the impact of interstitial lung disease (ILD) on PAH-SSc. Non-PAH-SSc patients from the Spanish SSc registry and PAH-SSc patients from the Spanish PAH registry were included. A total of 364 PAH-SSc and 1589 non-PAH-SSc patients were included. PAH-SSc patients had worse NYHA-functional class (NYHA-FC), worse forced vital capacity (FVC) (81.2 +/- 20.6% vs 93.6 +/- 20.6%, P < 0.001), worse tricuspid annular plane systolic excursion (TAPSE) (17.4 +/- 5.2 mm vs 19.9 +/- 6.7 mm, P < 0.001), higher incidence of pericardial effusion (30% vs 5.2%, P < 0.001) and similar prevalence of ILD (41.8% vs. 44.9%). In individuals with PAH-SSc, ILD was associated with worse hemodynamics and pulmonary function tests (PFT). Up-front combination therapy was used in 59.8% and 61.7% of patients with and without ILD, respectively. Five-year transplant-free survival rate was 41.1% in PAH-SSc patients and 93.9% in non-PAH-SSc patients (P < 0.001). Global survival of PAH-SSc patients was not affected by ILD regardless its severity. The multivariate survival analysis in PAH-SSc patients confirmed age at diagnosis, worse NYHA-FC, increased PVR, reduced DLCO, and lower management with up-front combination therapy as major risk factors. In conclusion, in PAH-SSc cohort risk of death was greatly increased by clinical, PFT, and hemodynamic factors, whereas it was decreased by up-front combination therapy. Concomitant ILD worsened hemodynamics and PFT in PAH-SSc but not survival regardless of FVC impairment

Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension

To assess severity markers and outcomes of patients with systemic sclerosis (SSc) with or without pulmonary arterial hypertension (PAH-SSc/non-PAH-SSc), and the impact of interstitial lung disease (ILD) on PAH-SSc. Non-PAH-SSc patients from the Spanish SSc registry and PAH-SSc patients from the Spanish PAH registry were included. A total of 364 PAH-SSc and 1589 non-PAH-SSc patients were included. PAH-SSc patients had worse NYHA-functional class (NYHA-FC), worse forced vital capacity (FVC) (81.2 ± 20.6% vs 93.6 ± 20.6%, P &lt; 0.001), worse tricuspid annular plane systolic excursion (TAPSE) (17.4 ± 5.2 mm vs 19.9 ± 6.7 mm, P &lt; 0.001), higher incidence of pericardial effusion (30% vs 5.2%, P &lt; 0.001) and similar prevalence of ILD (41.8% vs. 44.9%). In individuals with PAH-SSc, ILD was associated with worse hemodynamics and pulmonary function tests (PFT). Up-front combination therapy was used in 59.8% and 61.7% of patients with and without ILD, respectively. Five-year transplant-free survival rate was 41.1% in PAH-SSc patients and 93.9% in non-PAH-SSc patients (P &lt; 0.001). Global survival of PAH-SSc patients was not affected by ILD regardless its severity. The multivariate survival analysis in PAH-SSc patients confirmed age at diagnosis, worse NYHA-FC, increased PVR, reduced DLCO, and lower management with up-front combination therapy as major risk factors. In conclusion, in PAH-SSc cohort risk of death was greatly increased by clinical, PFT, and hemodynamic factors, whereas it was decreased by up-front combination therapy. Concomitant ILD worsened hemodynamics and PFT in PAH-SSc but not survival regardless of FVC impairment