No abstract

Scleroderma (systemic sclerosis) is an autoimmune connective tissue disease which presents endothelial dysfunction and fibroblast dysregulation, resulting in vascular and fibrotic disorders. Pulmonary hypertension is frequent in patients with systemic sclerosis: the natural evolution of the disease can induce the development of different forms of pulmonary hypertension, representing one of the main causes of death. Among the different forms of pulmonary hypertension in systemic sclerosis, pulmonary arterial hypertension is the most frequent one (rate of occurrence is estimated between 7% and 12%). This pulmonary vascular complication should be treated with a combination of drugs that is able to counteract endothelial dysfunction, antagonizing the endothelin-1 system and replacing prostaglandin I 2 and nitric oxide activity. A correct diagnosis is mandatory, because it is possible only for pulmonary arterial hypertension to use specific drugs that are able to control the symptomatic condition and the evolution of the disease. According to the most recent guidelines, for the patients with systemic sclerosis, also without pulmonary hypertension symptoms, echocardiography screening for the detection of pulmonary hypertension is recommended. Pulmonary arterial hypertension screening programs in systemic sclerosis patients is able to identify milder forms of the disease, allowing earlier management and better long-term outcome.

Background Although loop diuretic is the cornerstone of treatment in acute heart failure (AHF) there is no consensus about the best modality and amount to be used during acute phase. Current Guidelines do not provide specific insights regarding timing course and target dose. Usually physicians double the oral domestic amount when they start intravenous infusion, but a precise algorithm does not exist. Aims To compare admission and pre discharge clinical congestion and BNP trend in relation to furosemide amount and modality administration; 2- to evaluate diuretic efficiency and renal function in the four arms and the potential effects on outcome. Methods This is a multicentre prospective Trial (DIUR-AHF) designed in order to clarify the correct loop diuretic target avoiding potential side effects. The study enrolled patients with AHF BNP level >100 pg/ml and congestion signs. Patients were divided in four arms in accordance with modality administration: Continuous (Ci) vs Bolus (Bi) and dose administered Low (LD) vs High (HD) considering a cutoff 125 mg/die. All patients executed a clinical congestion evaluation and Chest radiography at admission and pre discharge, BNP sample and renal function were measured during the first 12 hours and before discharge. Diuretic efficiency (DE) defined as weight change per 40 mg of furosemide during infusional period. DE was estimated as the net fluid output produced per 40 mg of furosemide equivalents, Follow up were obtained by direct visit or phone contact at 30 and 60 days after discharge Results We included 268 hospitalized patients with a mean BNP level 987±440 pg /ml, mean congestion score (3.5±2) creatinine and GFR value were 1.6±0.7 mg/dl; and 48±20 ml/min/m2 respectively. At admission BNP and Creatinine were modestly increased in HD (P<0.01) compared with all other groups. Pre discharge Congestion score were increased in Bi and HD groups (2.5±1 vs 1±1), similarly BNP levels were increased in Bi and HD with respect to Ci and LD (454±215 and 413±223 vs 288±170 and 312±248 p<0.05). Whereas DE were significantly increased in Ci compared with the other arms (−1.23 vs −0.55 p<0.01). In all groups, low DE, residual congestion and BNP reduction <30% resulted in escalation of diuretic strategies and impaired outcome (HR 1.88 [1.16–204]; 2.1 [1.4–2.8]; 1.3 [0.88–2.1]). A significant correlation between poor DE and residual congestion was recruited (r=0.76). Worsening Renal function (WRF) occurred much more in HD and Ci compared to LD and Bi (HD 44%, Ci 35% vs LD 33% and Bi 23% p<0.01) without significant effects on outcome. Conclusions HD and Bi of furosemide are both related with reduced congestion and invreased BNP level before discharge. In all groups low DE residual congestion and poor BNP reduction appear associated with higher rate of adverse events. Current data provide additional features for AHF patients during intravenous loop diuretic administration Funding Acknowledgement Type of funding source: None

Aims The role of worsening renal function during acute heart failure (AHF) hospitalization is still debated. Very few studies have extensively evaluated the renal function (RF) trend during hospitalization by repetitive measurements. The aim of this study is to investigate the prognostic relevance of different RF trajectories together with the congestion status in hospitalized patients. Methods and results This is a post hoc analysis of a multi-centres study including 467 patients admitted with AHF who were screened for the Diur-AHF Trial. We recognized five main RF trajectories based on serum creatinine and estimated glomerular filtration rate (eGFR) behaviour: according to the RF curve our sample was divided into 1-stable (S), 2-transient improvement (TI), 3-permanent improvement (PI), 4-transient worsening (TW), and 5-persistent worsening (PW). The primary outcome was the combined endpoint of 180 days including all causes of mortality and re-hospitalization. We recruited 467 subjects with a mean congestion score of 3.5 ± 1.08 and a median creatinine value of 1.28 (1.00–1.70) mg/dl, eGFR 50 (37–65) ml/min/m2, and NTpro BNP 7000 (4200–11 700) pg/ml. A univariate analysis of the RF pattern demonstrated that TI and PW patterns were significantly related to poor prognosis [HR: 2.71 (1.81–4.05); P < 0.001; HR: 1.68 (1.15–2.45); P = 0.007, respectively]. Conversely, the TW pattern showed a significantly protective effect on outcome [HR: 0.34 (0.19–0.60); P < 0.001]. Persistence of congestion and BNP reduction ≥ 30% were significantly related to clinical outcome at univariate analysis [HR: 2.41 (1.81–3.21); P < 0.001 and HR: 0.47 (0.35–0.67); P < 0.001]. A multivariable analysis confirmed the independently prognostic role of TI, PW patterns, persistence of congestion, and reduced BNP decrease at discharge. Conclusions Various RF patterns during AHF hospitalization are associated with different risk(s). PW and TI appear to be the two trajectories related to worse outcome. Current findings confirm the importance of RF evaluation during and after hospitalization.

Aims The role of worsening renal function during acute heart failure (AHF) hospitalization is still debated. Very few studies have extensively evaluated the renal function (RF) trend during hospitalization by repetitive measurements. The aim of this study is to investigate the prognostic relevance of different RF trajectories together with the congestion status in hospitalized patients. Methods and results This is a post hoc analysis of a multi-centres study including 467 patients admitted with AHF who were screened for the Diur-AHF Trial. We recognized five main RF trajectories based on serum creatinine and estimated glomerular filtration rate (eGFR) behaviour: according to the RF curve our sample was divided into 1-stable (S), 2-transient improvement (TI), 3-permanent improvement (PI), 4-transient worsening (TW), and 5-persistent worsening (PW). The primary outcome was the combined endpoint of 180 days including all causes of mortality and re-hospitalization. We recruited 467 subjects with a mean congestion score of 3.5 ± 1.08 and a median creatinine value of 1.28 (1.00–1.70) mg/dl, eGFR 50 (37–65) ml/min/m2, and NTpro BNP 7000 (4200–11 700) pg/ml. A univariate analysis of the RF pattern demonstrated that TI and PW patterns were significantly related to poor prognosis [HR: 2.71 (1.81–4.05); P < 0.001; HR: 1.68 (1.15–2.45); P = 0.007, respectively]. Conversely, the TW pattern showed a significantly protective effect on outcome [HR: 0.34 (0.19–0.60); P < 0.001]. Persistence of congestion and BNP reduction ≥ 30% were significantly related to clinical outcome at univariate analysis [HR: 2.41 (1.81–3.21); P < 0.001 and HR: 0.47 (0.35–0.67); P < 0.001]. A multivariable analysis confirmed the independently prognostic role of TI, PW patterns, persistence of congestion and reduced BNP decrease at discharge. Conclusions Various RF patterns during AHF hospitalization are associated with different risk(s). PW and TI appear to be the two trajectories related to worse outcome. Current findings confirm the importance of RF evaluation during and after hospitalization.