Clinical implications of idiopathic pulmonary arterial hypertension phenotypes defined by cluster analysis

Badagliacca, Roberto; Rischard, Franz; Papa, Silvia; Kubba, Saad; Vanderpool, Rebecca; Yuan, Jason X.‐J.; Garcia, Joe G.N.; Airhart, Sophia; Poscia, Roberto; Pezzuto, Beatrice; Manzi, Giovanna; Miotti, Cristiano; Luongo, Federico; Scoccia, Gianmarco; Sciomer, Susanna; Torre, R. La; Fedele, Francesco; Vizza, Carmine Dario

doi:10.1016/j.healun.2019.12.012

Cited by 37 publications

(23 citation statements)

References 43 publications

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“…On the other hand, previous studies have suggested that the clinical response to targeted PAH drugs can be comparable, irrespective of the number of comorbidities [ 9 , 10 , 43 ]. Recent studies using cluster analyses have described different IPAH phenotypes based on age, the presence of cardiovascular risk factors and comorbidities and selected echocardiographic, spiroergometric and haemodynamic findings [ 44 ], as done previously in patients with pulmonary heart disease [ 45 ]. These data suggest that so-called type II pulmonary heart disease, with severe pulmonary vascular involvement and right ventricular dysfunction, is comparable to PAH.…”

Section: Discussionmentioning

confidence: 99%

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: Discussionmentioning

confidence: 99%

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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show abstract

“…However, only 13 patients were included between 2012 and 2013, when the access to anti-fibrotic treatment was limited and the awareness of disease lower. Cluster analysis should ideally be performed on large multinational cohorts of more than 1,000 patients for identifying as many patient profiles as possible [12,13]. However, the highlighted disease and patient features at diagnosis associated with disease outcome by using this methodology that integrates all potential risk factors have revealed at least two major points: diagnostic delay and cardiovascular-metabolic comorbidities.…”

Section: Discussionmentioning

confidence: 99%

“…However, some biological disorders may be a common trigger of different comorbidities in the same patient, such as metabolic syndrome or telomeric disorders [3,9]. Cluster analysis has become a useful resource to identify homogeneous patients with similar clinical characteristics, prognosis and healthcare requirements [12,13]. Additionally, the integrated study of respiratory diseases through clusters [14] helps identify hidden and unsuspected associations between different diseases and patient features, which could generate new hypotheses to be later explored in controlled studies.…”

Section: Introductionmentioning

confidence: 99%

Idiopathic pulmonary fibrosis cluster analysis highlights diagnostic delay and cardiovascular comorbidity association with outcome

et al. 2021

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IntroductionIdiopathic pulmonary fibrosis (IPF) prognosis is heterogeneous despite antifibrotic treatment. Cluster analysis has proven to be a useful tool in identifying interstitial lung disease phenotypes, which has yet to be performed in IPF. The aim of this study is to identify phenotypes of IPF with different prognoses and requirements.MethodsObservational retrospective study including 136 IPF patients receiving antifibrotic treatment between 2012–2018. Six patients were excluded due to follow-up in other centers. Cluster analysis of 30 variables was performed using approximate singular value-based tensor decomposition method and comparative statistical analysis.ResultsThe cluster analysis identified 3 different groups of patients according to disease behavior and clinical features, including mortality, lung transplant and progression-free survival time after 3-year follow-up. Cluster 1 (n=60) was significantly associated (p=0.02) with higher mortality. Diagnostic delay was the most relevant characteristic of this cluster, as 48% of patients had ≥2 years from first respiratory symptoms to antifibrotic treatment initiation. Cluster 2 (n=22) had the longest progression-free survival time and was correlated to subclinical patients evaluated in the context of incidental findings or familial screening. Cluster 3 (n=48) showed the highest percentage of disease progression without cluster 1 mortality, with metabolic syndrome and cardiovascular comorbidities as the main characteristics.ConclusionThis cluster analysis of IPF patients suggests that diagnostic and treatment delay are the most significant factors associated with mortality, while IPF progression was more related to metabolic syndrome and cardiovascular comorbidities.

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“…Moreover, most patients end up in the intermediate-risk category, characterized by a heterogeneous population with different outcomes, representing a missed opportunity for treatment optimization and personalized medicine [8].…”

Section: Strengths and Weaknesses Of Current Risk Scoresmentioning

confidence: 99%

The Growing Role of Echocardiography in Pulmonary Arterial Hypertension Risk Stratification: The Missing Piece

Miotti

Papa

Manzi

et al. 2021

JCM

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Pulmonary arterial hypertension (PAH) is a rare, progressive disease with a poor prognosis. The pathophysiologic model is mainly characterized by an afterload mismatch in which an increased right ventricle afterload, driven by increased pulmonary vascular resistance (PVR), leads to right heart failure. International guidelines recommend optimization of treatment based on regular risk assessments to achieve or maintain a low-risk status. Current risk scores are based on a multi-modality approach, including demographic, clinical, functional, exercise, laboratory, and hemodynamic parameters, which lack significant echocardiographic parameters. The originality of echocardiography relies on the opportunity to assess in a non-invasive way a physiologically meaningful combination of easy to measure variables tightly related to right ventricle adaptation/maladaptation to increased afterload, the main determinant of a patient’s prognosis. Echo-derived morphological and functional parameters have been investigated in PAH, proving to have prognostic relevance. Different therapeutic strategies proved to have different effects in reducing PVR. An upfront combination of drugs, including a parenteral prostacyclin, has shown to be associated with right heart reverse remodeling in a greater proportion of patients than other treatment strategies as a function of PVR reduction. Adding echocardiographic data to current risk scores would allow better identification of right ventricle (RV) adaptation in PAH patients’ follow-up. This additional information would allow better stratification of the patient, leading to optimized and personalized therapeutic management.

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Clinical implications of idiopathic pulmonary arterial hypertension phenotypes defined by cluster analysis

Cited by 37 publications

References 43 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

Idiopathic pulmonary fibrosis cluster analysis highlights diagnostic delay and cardiovascular comorbidity association with outcome

The Growing Role of Echocardiography in Pulmonary Arterial Hypertension Risk Stratification: The Missing Piece

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