Risk assessment in precapillary pulmonary hypertension: a comparative analysis

Sonnweber, Thomas; Schneider, Eva-Maria; Nairz, Manfred; Theurl, Igor; Weiß, Günter; Tymoszuk, Piotr; Löffler‐Ragg, Judith

doi:10.1186/s12931-021-01624-z

Cited by 8 publications

(5 citation statements)

References 25 publications

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“…However, distinguishing high-risk patients who may require more intensive upfront therapies from the beginning is also an important issue. Recently, Sonnweber et al 17 underlined this issue and showed that 7 different risk assessment tools including noninvasive French model and REVEAL 2.0 lack accuracy to differentiate mortality between intermediate- and high-risk groups in a cohort consisted of idiopathic PAH, connective tissue-associated PAH, and chronic thromboembolic pulmonary hypertension. 17 Of note, according to our results, the prognostic separation between intermediate and high-risk groups remains challenging.…”

Section: Discussionmentioning

confidence: 99%

“…Recently, Sonnweber et al 17 underlined this issue and showed that 7 different risk assessment tools including noninvasive French model and REVEAL 2.0 lack accuracy to differentiate mortality between intermediate- and high-risk groups in a cohort consisted of idiopathic PAH, connective tissue-associated PAH, and chronic thromboembolic pulmonary hypertension. 17 Of note, according to our results, the prognostic separation between intermediate and high-risk groups remains challenging. It confirms a limitation of utilizing mainly noninvasive risk parameters to discriminate the prognostic risk between intermediate- and high-risk CHD-PAH patients.…”

Section: Discussionmentioning

confidence: 99%

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Risk Assessment Tool Implementation in Congenital Heart Disease-Associated Pulmonary Arterial Hypertension

et al. 2023

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Background: Risk assessment is recommended for patients with congenital heart disease-associated pulmonary arterial hypertension. This study aims to compare an abbreviated version of the risk assessment strategy, noninvasive French model, and an abridged version of the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management 2.0 risk score calculator, Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2. Methods: We enrolled a mixed prevalent and incident cohort of patients with congenital heart disease-associated pulmonary arterial hypertension (n = 126). Noninvasive French model comprising World Health Organization functional class, 6-minute walk distance, and N-terminal pro-hormone of brain natriuretic peptide or brain natriuretic peptide was used. Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 includes functional class, systolic blood pressure, heart rate, 6-minute walk distance, brain natriuretic peptide/N-terminal pro-hormone of brain natriuretic peptide, and estimated glomerular filtration rate. Results: The mean age was 32.17 ± 16.3 years. The mean follow-up was 99.41 ± 58.2 months. Thirty-two patients died during follow-up period. Most patients were Eisenmenger syndrome (31%) and simple defects (29.4%). Most patients received monotherapy (76.2%). Most patients were World Health Organization functional class I-II (66.6%). Both models effectively identified risk in our cohort ( P = .0001). Patients achieving 2 or 3 noninvasive low-risk criteria or low-risk category by Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 at follow-up had a significantly reduced risk of death. Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 approximates noninvasive French model at discriminating among patients based on c-index. Age, high risk by Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2, and the presence of 2 or 3 low-risk criteria by noninvasive French model emerged as an independent predictors of mortality (multivariate hazard ratio: 1.031, 95% CI: 1.005-1.058, P = .02; hazard ratio: 4.258, CI: 1.143-15.860, P = .031; hazard ratio: 0.095, CI: 0.013-0.672, P = .018, respectively). Conclusions: Both abbreviated risk assessment tools may provide a simplified and robust method of risk assessment for congenital heart disease-associated pulmonary arterial hypertension. Patients not achieving low risk at follow-up may benefit from aggressive use of available therapies.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Risk Assessment Tool Implementation in Congenital Heart Disease-Associated Pulmonary Arterial Hypertension

et al. 2023

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“…Zusammenfassend kann festgestellt werden, dass schon über die derzeitige Risikoeinteilung von PAH-Patienten eine Beurteilung der Prognose gut möglich ist [38]. Erste Daten zu überarbeiteten Prognose-Score-Modellen liegen vor und verbessern die Abschätzung der Prognose weiter [9,[39][40][41]. Die praktische Anwendung solcher Score-Systeme außerhalb von Studien wird durch die Verringerung der Anzahl von genutzten Variablen erleichtert.…”

Section: Schlussfolgerungunclassified

Risikoänderung bei Patienten mit Pulmonaler Arterieller Hypertonie unter medikamentöser Therapie – Ergebnisse aus vier deutschen Zentren

Stubbe

Halank²,

Obst

et al. 2022

Pneumologie

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ZusammenfassungDie Risikobewertung für das abgeschätzte Überleben von PAH-Patienten ist eine wesentliche Grundlage für abgeleitete Therapieentscheidungen. Es existieren verschiedene Modelle für die Einschätzung des Risikos, welche jedoch weitgehend vergleichbar sind.Es wurden 131 PAH-Patienten aus den Jahren 2016–2018 hinsichtlich des Risikos nach dem ESC-/ERS-Schema ausgewertet. Etwa 2 Drittel der Patienten (mittleres Alter 64 ± 14 Jahre) wiesen ≥ 3 Komorbiditäten bzw. kardiovaskuläre Risikofaktoren auf. Zu Beginn der Beobachtung waren 13,8 % der Patienten als „low risk“, 76,3 % als „intermediate risk“ und 9,9 % als „high risk“ einzustufen. Zum Zeitpunkt der zweiten Kontrolle (im Mittel nach 12 ± 3 Monaten, Spanne 9–16 Monate) zeigten von den 111/131 (85 %) lebenden Patienten 20,7 % eine „low risk“-, 64,9 % eine „intermediate risk“- und 14,4 % eine „high risk“-Konstellation. Somit konnte lediglich ein geringer Anteil der Patienten durch die Therapie in eine günstigere Risiko-Kategorie überführt werden.Die von uns beobachteten Patienten mit einem „low risk“-Profil hatten ein Überleben von 100 % über 24 Monate. Die Patienten mit einem „intermediate risk“-Profil zeigten nach 12 Monaten ein Überleben von 96 % und nach 24 Monaten ein Überleben von 82 %. Dagegen lag das Überleben der Patienten mit einer „high risk“-Konstellation nach 12 Monaten bei 89 % und nach 24 Monaten bei nur 51 %.Trotz der aktuell verfügbaren therapeutischen Optionen konnte in unserer Analyse aus deutschen PH-Zentren nur ein kleiner Teil der PAH-Patienten während der Beobachtung in eine Niedrig-Risikogruppe mit guter Prognose überführt werden.

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“…1,7-10 Sonnweber et al published a comparative analysis in which seven different risk assessment methods were compared, and concluded that there is still a need for new tools in intermediate-and high-risk precapillary PH patients, the present methods being inaccurate for predicting mortality. 11 In some clinical circumstances, modified, simplified score calculators that include little accessible data and undependable or invasive hemodynamic parameters can be used to predict 1-year mortality and long-term transplant-free survival in adult PAH or PAH associated with congenital heart disease (PAH-CHD) patients. [12][13][14][15] Over the few past decades, considerable interest in the role of iron status in idiopathic PAH and PAH-CHD has developed due to its potential impact on symptoms, exercise capacity (as assessed by the 6-minute walk distance [6MWD]), prognosis, and mortality.…”

Section: Introductionmentioning

confidence: 99%

“… 1 , 7–10 Sonnweber et al published a comparative analysis in which seven different risk assessment methods were compared, and concluded that there is still a need for new tools in intermediate- and high-risk precapillary PH patients, the present methods being inaccurate for predicting mortality. 11 …”

Section: Introductionmentioning

confidence: 99%

Short-Term Impact of Iron Deficiency in Different Subsets of Patients with Precapillary Pulmonary Hypertension from an Eastern European Pulmonary Hypertension Referral Center

Ţilea¹,

Petra²,

Șerban³

et al. 2021

IJGM

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Background: Over the last few decades, interest in the role of iron status in pulmonary hypertension (PH) has grown considerably due to its potential impact on symptoms, exercise capacity (as assessed by the 6-minute walk distance [6MWD]), prognosis, and mortality. The aim of the present study was to identify iron deficiency (ID) prevalence in specific precapillary PH subgroups of Romanian patients and its short-term impact on 6MWD. Patients and Methods: Complete datasets from 25 precapillary PH adults were examined and included in the analysis. Data were collected at baseline and after continuous follow-up of an average of 13.5 months. Enrolled patients were assigned to group 1 (pulmonary arterial hypertension) or subgroup 4.1 (chronic thromboembolic pulmonary hypertension), and individualized targeted therapy was prescribed. General characteristics, World Health Organization functional class, 6MWD, pulse oximetry, laboratory parameters, and echocardiographic and hemodynamic parameters were recorded. Ferritin values and transferrin saturation were used to assess ID. Results: At baseline, 16 out of 25 patients were iron deficient. The univariate linear regression analysis did not show a statistically significant impact of ID on 6MWD (p=0.428). In multivariate regression analysis, possible predictors of 6MWD, including ID, were not statistically significant at baseline or after an average of 13.5 months follow-up (p=0.438, 0.361, respectively) and ID indicates a negative impact on 6MWD independent of applied corrections. Conclusion:The results of this study demonstrate that 1.4.1 subgroup PAH patients have an increased prevalence of ID compared with other etiologies. ID has a negative impact on the functional status (assessed by 6MWD), in specific groups and subgroups of patients with precapillary PH, albeit not independently nor significant to other known predictors such as age, gender, oxygen saturation, and hemoglobin value. These data can be integrated with global research and are consistent with phenotypes of patients diagnosed with PH of different etiologies.

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Risk assessment in precapillary pulmonary hypertension: a comparative analysis

Cited by 8 publications

References 25 publications

Risk Assessment Tool Implementation in Congenital Heart Disease-Associated Pulmonary Arterial Hypertension

Risk Assessment Tool Implementation in Congenital Heart Disease-Associated Pulmonary Arterial Hypertension

Risikoänderung bei Patienten mit Pulmonaler Arterieller Hypertonie unter medikamentöser Therapie – Ergebnisse aus vier deutschen Zentren

Short-Term Impact of Iron Deficiency in Different Subsets of Patients with Precapillary Pulmonary Hypertension from an Eastern European Pulmonary Hypertension Referral Center

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