Prevalence and incidence of pulmonary arterial hypertension: 10-year follow-up of an unselected systemic sclerosis cohort

2017 ◽  
Vol 2 (3) ◽  
pp. 196-202 ◽  
Author(s):  
Els Vandecasteele ◽  
Karin Melsens ◽  
Kristof Thevissen ◽  
Michel De Pauw ◽  
Ellen Deschepper ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Incidence Rate ◽  
Specific Treatment ◽  
Early Screening ◽  
European Respiratory Society ◽  
Pulmonary Arterial ◽  
Incident Cases

Introduction Early screening for pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) improves outcome. Therefore, we evaluated the screening for PAH during the 10-year follow-up of an unselected prospective SSc cohort by calculating the prevalence and the incidence rate of PAH and we compared the screening before and after implementation of the 2009 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines. Methods Data were evaluated from each SSc-specific visit of 362 consecutive SSc patients included in the SSc Cohort of the Ghent University between May 2006 and December 2015. Results Of the 362 included patients, 23.2% had limited SSc, 59.9% limited cutaneous SSc and 16.9% diffuse cutaneous SSc. At baseline, one patient was already on PAH-specific treatment and eight patients were diagnosed with PAH, implicating a baseline PAH prevalence of 2.5% (9/362). During follow-up (median of 18 months [interquartile range: 0-54 months]), nine patients were diagnosed with incidental PAH, resulting in an incidence rate of 9.3/1000 person-years, 95% confidence intervals (95% CI): 4.3-17.7. Before the ESC/ERS guidelines, five PAH patients, all already diagnosed with prevalent PAH, were included in the cohort. After 2009, 13 patients (4 prevalent cases) were diagnosed with PAH, making the yearly incidence around 1% (0.82%-2.00%). Conclusions During 10-year follow-up in a cohort of 362 unselected SSc patients, the cumulative prevalence of PAH is 5% (18/362) and the incidence rate 9.3/1000 person-years, 95% CI: 4.3-17.7. Before implementation of the 2009 ESC/ERS screening algorithm, there were no incident cases.

scholarly journals Pulmonary arterial hypertension populations of special interest: portopulmonary hypertension and pulmonary arterial hypertension associated with congenital heart disease

2019 ◽  
Vol 21 (Supplement_K) ◽  
pp. K37-K45 ◽  
Author(s):  
Laurent Savale ◽  
Alessandra Manes
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Congenital Heart ◽  
Expert Advice ◽  
Portopulmonary Hypertension ◽  
Early Screening ◽  
Pulmonary Arterial

Abstract Guidelines exist for management of pulmonary arterial hypertension (PAH), but information is limited for certain patient subgroups, including adults with portopulmonary hypertension (PoPH) or with PAH associated with congenital heart disease (PAH-CHD). This article discusses screening, clinical management, and prognosis in PoPH and PAH-CHD and, as such, considers the most recent clinical data and expert advice. A multidisciplinary consultation and follow-up by specialists are crucial for management of both PoPH and PAH-CHD, but each condition presents with unique challenges. Development of PoPH most commonly occurs among patients with liver cirrhosis. Initially, patients may be asymptomatic for PoPH and, if untreated, survival with PoPH is generally worse than with idiopathic PAH (IPAH), so early identification with screening is crucial. PoPH can be managed with PAH-specific pharmacological therapy, and resolution is possible in some patients with liver transplantation. With PAH-CHD, survival rates are typically higher than with IPAH but vary across the four subtypes: Eisenmenger syndrome, systemic-to-pulmonary shunts, small cardiac defects, and corrected defects. Screening is also crucial and, in patients who undergo correction of CHD, the presence of PAH should be assessed immediately after repair and throughout their long-term follow-up, with frequency of assessments determined by the patient’s characteristics at the time of correction. Early screening for PAH in patients with portal hypertension or CHD, and multidisciplinary management of PoPH or PAH-CHD are important for the best patient outcomes.

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 Selexipag treatment in patients with systemic sclerosis–associated pulmonary arterial hypertension in clinical practice, a case series

2020 ◽  
Vol 5 (3) ◽  
pp. NP7-NP11
Author(s):  
Jacqueline MJ Lemmers ◽  
Håvard Fretheim ◽  
Hanneke KA Knaapen ◽  
Frank HJ van den Hoogen ◽  
Jolanda HGM van Haren-Willems ◽  
...  
Keyword(s):  
Risk Assessment ◽  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Side Effects ◽  
Arterial Hypertension ◽  
Interquartile Range ◽  
Walking Distance ◽  
Expert Team ◽  
Pulmonary Arterial

Objective: To describe the efficacy and safety in all patients with systemic sclerosis–associated pulmonary arterial hypertension who started selexipag between 09-2016 and 06-2018 in two pulmonary arterial hypertension expert centers. Methods: All patients with systemic sclerosis–associated pulmonary arterial hypertension diagnosed by right heart catheterization and treated with selexipag were included. Every 12 weeks, treatment effect was assessed by (1) the opinion of the expert team and (2) the abbreviated risk assessment, consisting of functional class, six-minute walking distance, and N-terminal prohormone of brain natriuretic peptide level at baseline and during follow-up. Side effects and adverse events were registered. Results: We included 13 systemic sclerosis–associated pulmonary arterial hypertension patients, 10 patients were female, median age (interquartile range) of 68 (58–75) years, median systemic sclerosis disease duration of 7.4 (4.7–13.5) years, and median pulmonary arterial hypertension duration of 4 (2.5–7.5) years. Two patients discontinued selexipag within 4 weeks due to side effects. The remaining 11 patients had a median follow-up duration of 48 (interquartile range = 24–72) weeks. Two patients died (one pulmonary arterial hypertension–related, the other systemic sclerosis–related). According to the expert team, 8 of 11, 9 of 10, and 5 of 7 patients stabilized or improved at 12, 24, and 48 weeks, respectively. According to the abbreviated risk assessment at study end, 3 of 11 patients had 1 low-risk criterion. No previously unrecorded side effects were reported. Conclusion: Adding selexipag to background therapy in a high-risk cohort of systemic sclerosis–associated pulmonary arterial hypertension patients provided sustained stabilization of symptoms with an acceptable safety profile. Improvement was reached in only two of our patients. Further research should focus on systemic sclerosis–associated pulmonary arterial hypertension patients treated with multiple targeted treatments, preferably these patients should be prospectively followed in international registries.

scholarly journals Haemodynamics and serial risk assessment in systemic sclerosis associated pulmonary arterial hypertension

2018 ◽  
Vol 52 (4) ◽  
pp. 1800678 ◽  
Author(s):  
Jason Weatherald ◽  
Athénaïs Boucly ◽  
David Launay ◽  
Vincent Cottin ◽  
Grégoire Prévot ◽  
...  
Keyword(s):  
Risk Assessment ◽  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Cardiac Index ◽  
Arterial Hypertension ◽  
Arterial Compliance ◽  
Functional Class ◽  
Free Survival ◽  
Pulmonary Arterial

The prognostic importance of follow-up haemodynamics and the validity of multidimensional risk assessment are not well established for systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH).We assessed incident SSc-PAH patients to determine the association between clinical and haemodynamic variables at baseline and first follow-up right heart catheterisation (RHC) with transplant-free survival. RHC variables included cardiac index, stroke volume index (SVI), pulmonary arterial compliance and pulmonary vascular resistance. Risk assessment was performed according to the number of low-risk criteria: functional class I or II, 6-min walking distance (6MWD) >440 m, right atrial pressure <8 mmHg and cardiac index ≥2.5 L·min−1·m−2.Transplant-free survival from diagnosis (n=513) was 87%, 55% and 35% at 1, 3 and 5 years, respectively. At baseline, 6MWD was the only independent predictor. A follow-up RHC was available for 353 patients (median interval 4.6 months, interquartile range 3.9–6.4 months). The 6MWD, functional class, cardiac index, SVI, pulmonary arterial compliance and pulmonary vascular resistance were independently associated with transplant-free survival at follow-up, with SVI performing better than other haemodynamic variables. 1-year outcomes were better with increasing number of low-risk criteria at baseline (area under the curve (AUC) 0.63, 95% CI 0.56–0.69) and at first follow-up (AUC 0.71, 95% CI 0.64–0.78).Follow-up haemodynamics and multidimensional risk assessment had greater prognostic significance than at baseline in SSc-PAH.

scholarly journals P1282 Follow-up evaluation of right chambers mechanics is associated with prognosis in group 1 pulmonary arterial hypertension

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
F Biondi ◽  
S Albani ◽  
F Lo Giudice ◽  
L Howard ◽  
A De Luca ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Longitudinal Strain ◽  
Specific Treatment ◽  
Right Atrial ◽  
Echocardiographic Parameters ◽  
Pulmonary Arterial ◽  
Group 1

Abstract Background Pulmonary arterial hypertension (PAH) is a severe disease that progressively leads to right ventricular (RV) failure and cardiovascular death. Evaluation of right heart mechanics by means of 2-dimensional speckle tracking echocardiography (2D-STE) has displayed to be a promising tool to estimate prognosis in PAH patients. Purpose To evaluate the association between right ventricular free wall longitudinal strain (RVFWLS) and right peak atrial longitudinal strain (RPALS) at follow-up after initiation of specific vasodilator therapy in PAH patients, with outcomes. Methods 83 subjects diagnosed with PAH Group 1 at three University Hospitals (Hammersmith Hospital, London, United Kingdom; Trieste University Hospital, Trieste, Italy; FTGM, Pisa, Italy), who were naive from specific treatment for PAH at the time of diagnosis, were retrospectively enrolled in this study. Standard echocardiographic parameters were collected. Outcomes were defined as the combination of all-cause mortality, hospitalization for PAH and first prostanoid administration. We investigated the correlation between RVFWLS and RPALS with outcomes adjusting for validated echocardiographic parameters strongly associated with prognosis in PAH (right atrial area – RAA and pericardial effusion – PE) and patients’ haemodynamics. Results 30 patients experienced outcomes during a median follow-up time of 33 months. Median RVFWLS at follow-up was -15.8% (IQR: -12.1%/- 21.1%). Median RPALS at follow-up was 25% (IQR: 17.9%/36.6%). In the multivariate analysis, RVFLWS at follow-up was independently associated with outcomes (95% confidence interval [CI]: 1.01 – 1.24, p = 0.04), irrespectively from RAA (95% CI: 0.98 – 1.20, p = 0.12) and pulmonary arterial systolic pressure (PASP, 95% CI: 0.99 – 1.06, p = 0.12). We also observed a trend towards superiority of RPALS at follow-up (95% CI 0.90 – 1.00, p = 0.07) over RAA (95% CI: 0.98 – 1.20, p = 0.14) and PASP (95% CI: 0.99 - 1.05, p= 0.31). Finally, RPALS (95% CI: 0.88 – 1.00, p = 0.05) was independently associated with outcomes over PE (95% CI: 0.14 – 1.89, p = 0.32) and RVFWLS (95% CI: 0.97 – 1.20, p = 0.16). Conclusions In PAH group 1, assessment of both RVFWLS and RPALS at follow-up is associated with outcomes, independently from standard echocardiographic parameters.

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