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.

scholarly journals Contemporary treatment of pulmonary arterial hypertension: the North-West Registry data analysis

2012 ◽  
Vol 11 (4) ◽  
pp. 79-84
Author(s):  
N. S. Goncharova ◽  
A. V. Kazymly ◽  
A. V. Naimushin ◽  
O. M. Moiseeva
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
National Registry ◽  
Registry Data ◽  
Eisenmenger Syndrome ◽  
Specific Therapy ◽  
Right Heart Catheterisation ◽  
Pulmonary Arterial

Aim. Using the prospective Registry data, to assess the effects of conventional and specific therapy on the clinical course and survival of the patients with pulmonary arterial hypertension (PAH). Material and methods. The study included 124 patients (mean age 38,2±13,7 years; 34 men and 78 women): 31 with idiopathic PAH (IPAH), 52 with Eisenmenger syndrome, 17 with inoperable chronic thromboembolic pulmonary hypertension, 9 with PAH and corrected congenital heart disease, 6 with PAH and systemic scleroderma, and 6 with PAH and HIV infection. Results. The cumulative one-year and three-year survival rates were 94% and 75%, respectively. Irrespective of the absence of right heart catheterisation and vasoreactive testing, 42,7% of the patients were treated with calcium antagonists. PAH-specific therapy was administered to 40,3% of the participants (64,5% and 21% of those with IPAH and Eisenmenger syndrome, respectively). PAH-specific therapy was associated with an increase in survival time. Conclusion. In PAH patients, the prognosis is linked to early administration of specific monotherapy and possible combination therapy. Developing a national registry of pulmonary hypertension will facilitate the assessment of the real-world demand for specific therapy and the related costs.

scholarly journals Pathophysiology and treatment of pulmonary hypertension in sickle cell disease

Blood ◽  
2016 ◽  
Vol 127 (7) ◽  
pp. 820-828 ◽  
Author(s):  
Victor R. Gordeuk ◽  
Oswaldo L. Castro ◽  
Roberto F. Machado
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Sickle Cell Disease ◽  
Pulmonary Artery ◽  
Arterial Hypertension ◽  
Sickle Cell ◽  
Left Ventricular ◽  
Cell Disease ◽  
Pulmonary Pressure ◽  
Pulmonary Arterial

Abstract Pulmonary hypertension affects ∼10% of adult patients with sickle cell disease (SCD), particularly those with the homozygous genotype. An increase in pulmonary artery systolic pressure, estimated noninvasively by echocardiography, helps identify SCD patients at risk for pulmonary hypertension, but definitive diagnosis requires right-heart catheterization. About half of SCD-related pulmonary hypertension patients have precapillary pulmonary hypertension with potential etiologies of (1) a nitric oxide deficiency state and vasculopathy consequent to intravascular hemolysis, (2) chronic pulmonary thromboembolism, or (3) upregulated hypoxic responses secondary to anemia, low O2 saturation, and microvascular obstruction. The remainder have postcapillary pulmonary hypertension secondary to left ventricular dysfunction. Although the pulmonary artery pressure in SCD patients with pulmonary hypertension is only moderately elevated, they have a markedly higher risk of death than patients without pulmonary hypertension. Guidelines for diagnosis and management of SCD-related pulmonary hypertension were published recently by the American Thoracic Society. Management of adults with sickle-related pulmonary hypertension is based on anticoagulation for those with thromboembolism; oxygen therapy for those with low oxygen saturation; treatment of left ventricular failure in those with postcapillary pulmonary hypertension; and hydroxyurea or transfusions to raise the hemoglobin concentration, reduce hemolysis, and prevent vaso-occlusive events that cause additional increases in pulmonary pressure. Randomized trials have not identified drugs to lower pulmonary pressure in SCD patients with precapillary pulmonary hypertension. Patients with hemodynamics of pulmonary arterial hypertension should be referred to specialized centers and considered for treatments known to be effective in other forms of pulmonary arterial hypertension. There have been reports that some of these treatments improve SCD-related pulmonary hypertension.

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.

scholarly journals Screening for pulmonary arterial hypertension in an unselected prospective systemic sclerosis cohort

2017 ◽  
Vol 49 (5) ◽  
pp. 1602275 ◽  
Author(s):  
Els Vandecasteele ◽  
Benny Drieghe ◽  
Karin Melsens ◽  
Kristof Thevissen ◽  
Michel De Pauw ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
High Risk ◽  
Positive Predictive Value ◽  
Arterial Hypertension ◽  
Predictive Value ◽  
Right Heart ◽  
Heart Catheterisation ◽  
Right Heart Catheterisation ◽  
Pulmonary Arterial

Screening for pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) improves outcomes. The DETECT screening algorithm is recommended in a high-risk SSc subgroup. This study aims to compare prospectively the positive predictive value of screening using the DETECT algorithm and the 2009 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines, and to compare their cost-effectiveness in an unselected, day-to-day SSc population. Post hoc, screening according to the 2015 ESC/ERS guidelines using echocardiographic parameters alone (“2015 echo screening”) or combined with the DETECT algorithm (“2015 combined screening”) in high-risk subjects was analysed.195 consecutive SSc patients included in the Ghent University Hospital SSc cohort were screened using different algorithms.The referral rate for right heart catheterisation was 32% (63 out of 195 patients) (46/4/13/34/40 patients using the DETECT algorithm/2009 guidelines/both/2015 echo screening/2015 combined screening). Right heart catheterisation was performed in 53 patients (84%) (36 (78%)/four (100%)/13 (100%)/28 (82%)/32 (80%) patients recommended by the DETECT algorithm/2009 guidelines/both/2015 echo screening/2015 combined screening). PAH was diagnosed in three patients (incidence 1.5%·year–1, 95% CI 0.5–4.4), in whom all algorithms recommended a right heart catheterisation. The positive predictive value was 6% (95% CI 2–17%; three out of 49 patients) for the DETECT algorithm, 18% (95% CI 6–41%; three out of 17 patients) for the 2009 guidelines, 23% (95% CI 8–50%; three out of 13 patients) for both, 11% (95% CI 4–27%; three out of 28 patients) for the 2015 echo screening and 9% (95% CI 3–24%; three out of 32 patients) for the 2015 combined screening. The cost was EUR224/80/90/112 per patient using the DETECT algorithm/2009 guidelines/2015 echo screening/2015 combined screening.Echocardiography may remain a candidate first step for PAH screening in SSc.

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