scholarly journals Survival and Predictors of Mortality in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: Outcomes From the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry

2014 ◽  
Vol 66 (3) ◽  
pp. 489-495 ◽  
Author(s):  
Lorinda Chung ◽  
Robyn T. Domsic ◽  
Bharathi Lingala ◽  
Firas Alkassab ◽  
Marcy Bolster ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Predictors Of Mortality ◽  
Pulmonary Arterial

Screening for pulmonary arterial hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2506-2507
Author(s):  
John Coghlan
Keyword(s):  
Pulmonary Hypertension ◽  
Portal Hypertension ◽  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Diagnostic Tools ◽  
Primary Screening ◽  
Class 1 ◽  
Pulmonary Arterial ◽  
Low Prevalence

Screening programmes for pulmonary hypertension are justifiable in some circumstances but not others. The inherent inaccuracies of the diagnostic tools and the low prevalence of pulmonary hypertension renders screening programmes ineffective unless the population evaluated with the primary screening tool (echocardiography) can be enriched. Nevertheless, significant progress has been made over the past 5 years and screening programmes are now strongly recommended (class 1 recommendation) in asymptomatic systemic sclerosis, BMPR2 mutation carriers, first-degree relatives of patients with hereditable pulmonary hypertension, and patients with portal hypertension referred for transplantation.

Pulmonary arterial hypertension in systemic sclerosis: Diagnosis and treatment according to the European Society of Cardiology and European Respiratory Society 2015 guidelines

2018 ◽  
Vol 4 (1) ◽  
pp. 35-42 ◽  
Author(s):  
Nicola Giordano ◽  
Claudio Corallo ◽  
Chiara Chirico ◽  
Angelica Brazzi ◽  
Adriana Marinetti ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Endothelial Dysfunction ◽  
Arterial Hypertension ◽  
Correct Diagnosis ◽  
European Respiratory Society ◽  
Long Term Outcome ◽  
Screening Programs ◽  
Pulmonary Arterial

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 I2 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.

Abstract 4383: Incidence of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis: A 3-Year Longitudinal Study

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Pascal de Groote ◽  
Eric Hachulla ◽  
Virginie Gressin ◽  
Jean Sibilia ◽  
Elisabeth Diot ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Doppler Echocardiography ◽  
Large Population ◽  
Pulmonary Pressure ◽  
Right Heart Catheterisation ◽  
Normal Mean ◽  
Pulmonary Arterial

We have previously determined a prevalence of 7.85% [95%CI: 5.70–10.00] of pulmonary arterial hypertension (PAH) in a large multicentre systemic sclerosis (SSc) population (the ItinerAIR Scleroderma registry). We used a screening algorithm based on dyspnea, Doppler echocardiographic evaluation of the peak velocity of tricuspid regurgitation (VTR) and right heart catheterisation (RHC). No study has determined the incidence of PAH in a large population at risk. To determine the incidence of PAH over 3 years of follow-up in the ItinerAIR SSc registry. Patients underwent regular screening for PAH with Doppler echocardiography confirmed by RHC in patients with VTR of 2.8–3 m/s and unexplained dyspnea, or VTR >3 m/s. 384 patients were followed for a mean of 41±5.6 months (median 41 months). The baseline characteristics of this population were: 87% female, mean age 53±12 years, mean duration of SSc at study entry 8.7±7.6 years, and 24.0% with diffuse SSc. Doppler echocardiography detected PAH in 18 patients (incidence: 1.37 per 100 patient-years). Among these, RHC identified 8 patients with pre-capillary PAH (incidence: 0.61 per 100 patient-years [95%CI: 0.26–1.20]), 8 with post-capillary pulmonary hypertension (PH) and 2 with PH due to severe interstitial lung disease. The 8 patients with pre-capillary PAH had moderate PAH (mean pulmonary pressure: 36±14.6 mmHg). Among the 8 patients with post-capillary PH, echocardiography was always considered normal by the expert cardiologist, except in 2 cases (moderate left atrial dilatation in one and mild aortic sclerosis in the other). These 8 patients had mild post-capillary PH, 5 of them having normal mean pulmonary pressure at rest but a significant increase during exercise with a pulmonary wedge pressure > 15 mmHg. In this large multicentre SSc cohort we prospectively found an incidence of PAH of 0.61 per 100 patient-years. We also demonstrated that RHC is still mandatory to precisely define the type of pulmonary hypertension since mild post-capillary PH without echocardiographic abnormalities are frequent.

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