Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry

Hoeper, Marius M.; Pausch, Christine; Grünig, Ekkehard; Klose, Hans; Staehler, G.; Huscher, Dörte; Pittrow, David; Olsson, Karen M.; Vizza, Carmine Dario; Gall, Henning; Benjamin, Nicola; Distler, Oliver; Opitz, Christian; Gibbs, J. Simon R.; Delcroix, Marion; Ghofrani, Hossein‐Ardeschir; Rosenkranz, Stephan; Ewert, Ralf; Kaemmerer, Harald; Lange, Tobias; Kabitz, Hans‐Joachim; Skowasch, Dirk; Skride, Andris; Jurevičienė, Elena; Palevičiūtė, Eglė; Miliauskas, Skaidrius; Claussen, Martin; Behr, Jüergen; Milger, Katrin; Halank, Michael; Wilkens, Heinrike; Wirtz, Hubert; Pfeuffer-Jovic, Elena; Harbaum, Lars; Scholtz, Werner; Dumitrescu, Daniel; Bruch, Leonhard; Coghlan, Gerry; Neurohr, Claus; Tsangaris, Iraklis; Gorenflo, Matthias; Scelsi, Laura; Vonk‐Noordegraaf, Anton; Ulrich, Silvia; Held, Matthias

doi:10.1016/j.healun.2020.09.011

Cited by 113 publications

(98 citation statements)

References 30 publications

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“…Our study (including a large spectrum of PAH patients) indicates a late initiation of combination therapy in patients over 65 years of age. This is comparable to recently published data from the COMPERA registry [ 46 ], in which the proportion of older patients receiving combination PAH therapy also increased over time. In our study, “typical” PAH patients received early combination therapy, as suggested by the guidelines, while older patients with more risk factors and comorbidities received this form of therapy later.…”

Section: Discussionsupporting

confidence: 88%

“…These data suggest that so-called type II pulmonary heart disease, with severe pulmonary vascular involvement and right ventricular dysfunction, is comparable to PAH. A similar approach (cluster analysis) was performed on IPAH patients in the COMPERA registry, linking different phenotypes with survival [ 46 ]. It remains to be seen whether such phenotype classifications will affect therapeutic strategies for PAH patients in the future, as has been proposed for other disease entities, such as heart failure with preserved ejection fraction [ 47 ].…”

Section: Discussionmentioning

confidence: 99%

“…It remains to be seen whether such phenotype classifications will affect therapeutic strategies for PAH patients in the future, as has been proposed for other disease entities, such as heart failure with preserved ejection fraction [ 47 ]. In the recently published COMPERA cluster analysis [ 46 ] of 846 IPAH patients, 38% and 63% of “typical” patients (median age of 45 years old, without so-called “risk factors for left heart disease”) were treated with combined targeted PAH therapy within the first three months and after one year during follow-up, respectively. The other patients were predominantly treated with monotherapy at baseline and during follow-up.…”

Section: Discussionmentioning

confidence: 99%

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Monotherapy in patients with pulmonary arterial hypertension at four German PH centres

Stubbe

Kleymann²,

Halank³

et al. 2021

BMC Pulm Med

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Background Although combination therapy is the gold standard for patients with pulmonary arterial hypertension (PAH), some of these patients are still being treated with monotherapy. Methods We conducted a retrospective analysis at four German PH centres to describe the prevalence and characteristics of patients receiving monotherapy. Results We identified 131 incident PAH patients, with a mean age of 64 ± 13.8 years and a varying prevalence of comorbidities, cardiovascular risk factors and targeted therapy. As in other studies, the extent of prescribed PAH therapy varied with age and coexisting diseases, and younger, so-called “typical” PAH patients were more commonly treated early with combination therapy (48% at 4–8 months). In contrast, patients with multiple comorbidities or cardiovascular risk factors were more often treated with monotherapy (69% at 4–8 months). Survival at 12 months was not significantly associated with the number of PAH drugs used (single, dual, triple therapy) and was not different between “atypical” and “typical” PAH patients (89% vs. 85%). Conclusion Although “atypical” PAH patients with comorbidities or a more advanced age are less aggressively treated with respect to combination therapy, the outcome of monotherapy in these patients appears to be comparable to that of dual or triple therapy in “typical” PAH patients.

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Section: Discussionsupporting

confidence: 88%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Monotherapy in patients with pulmonary arterial hypertension at four German PH centres

Stubbe

Kleymann²,

Halank³

et al. 2021

BMC Pulm Med

Self Cite

View full text Add to dashboard Cite

show abstract

“…The atypical profile with a higher age and comorbidity burden of patients diagnosed with IPAH and CTD-PAH might be another reason for choosing single over combination therapy [ 27 , 28 ]. Data regarding up-front combination therapy vary, with some registries reporting higher [ 28 , 29 ] and another lower [ 30 ] percentages than SPAHR.…”

Section: Discussionmentioning

confidence: 99%

Early risk prediction in idiopathic versus connective tissue disease-associated pulmonary arterial hypertension: call for a refined assessment

et al. 2021

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Despite systematic screening and improved treatment strategies, the prognosis remains worse in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) compared to patients with idiopathic/hereditary pulmonary arterial hypertension (IPAH). We aimed to investigate differences in clinical characteristics, outcome, and performance of the ESC/ERS risk stratification tool in these patient groups.This retrospective analysis included incident patients with CTD-PAH (n=197, of which 64 had interstitial lung disease, ILD) or IPAH (n=305) enrolled in the Swedish PAH Register 2008–2019. Patients were classified as low, intermediate, or high risk at baseline, according to the “SPAHR-equation”. 1-year survival, stratified by type of PAH, was investigated by Cox proportional regression.At baseline, CTD-PAH patients had lower diffusing capacity for carbon monoxide and lower haemoglobin, but, at the same time, lower N-terminal prohormone-brain natriuretic peptide, longer 6 min walking distance, better hemodynamics, and more often a low-risk profile. No difference in age, WHO-FC, or renal function between groups was found. 1-year survival rates were 75, 82 and 83%, in patients with CTD-PAH with ILD, CTD-PAH without ILD, and IPAH, respectively. The 1-year mortality rates for low-, intermediate-, and high-risk groups in the whole cohort were 0, 18 and 34% (p<0.001), respectively. Corresponding percentages for CTD-PAH with ILD, CTD-PAH without ILD, and IPAH patients were: 0, 26, 67% (p=0.008); 0, 19, 39% (p=0.004); and 0, 16, 29% (p=0.001), respectively.The ESC/ERS risk assessment tool accurately identified low-risk patients but underestimated the 1-year mortality rate of CTD-PAH and IPAH patients assessed as having intermediate risk at diagnosis.

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“…Increasing age is known to increase postoperative cardiac events. 50 Previous studies in patients with idiopathic PAH (iPAH) show that comorbidities including lung disease and smoking history worsen survival. 51 In addition, patients with PAH have associated diseases that may adversely affect surgical healing (e.g.…”

Section: Preoperative Risk Assessmentmentioning

confidence: 99%

Perioperative management of patients with pulmonary hypertension undergoing non-cardiothoracic, non-obstetric surgery: a systematic review and expert consensus statement

Price

Martínez

Brame

et al. 2021

British Journal of Anaesthesia

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Background: The risk of complications, including death, is substantially increased in patients with pulmonary hypertension (PH) undergoing anaesthesia for surgical procedures, especially in those with pulmonary arterial hypertension (PAH) and chronic thromboembolic PH (CTEPH). Sedation also poses a risk to patients with PH. Physiological changes including tachycardia, hypotension, fluid shifts, and an increase in pulmonary vascular resistance (PH crisis) can precipitate acute right ventricular decompensation and death. Methods: A systematic literature review was performed of studies in patients with PH undergoing non-cardiac and nonobstetric surgery. The management of patients with PH requiring sedation for endoscopy was also reviewed. Using a

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Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry

Cited by 113 publications

References 30 publications

Monotherapy in patients with pulmonary arterial hypertension at four German PH centres

Monotherapy in patients with pulmonary arterial hypertension at four German PH centres

Early risk prediction in idiopathic versus connective tissue disease-associated pulmonary arterial hypertension: call for a refined assessment

Perioperative management of patients with pulmonary hypertension undergoing non-cardiothoracic, non-obstetric surgery: a systematic review and expert consensus statement

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