BACKGROUND: The added value of cardiopulmonary exercise testing (CPET) in the follow-up of patients with stable pulmonary arterial hypertension (PAH) remains undefined. METHODS: Idiopathic, heritable, and drug-induced PAH patients free from clinical worsening (CW) after 1 year of treatment were enrolled in derivation (n = 80) and validation (n = 80) cohorts at an interval of 6 years and followed for 3 years. Prognostic models were constructed and validated in low-risk patients in World Health Organization (WHO) Functional Class I or II with cardiac index (CI) ≥2.5 liters/min/m2 and right atrial pressure (RAP) <8 mm Hg. Discrimination and calibration were assessed. RESULTS: Forty-one derivation cohort patients had CW (51.2%) during 722 ± 349 days. Changes (∆) in WHO classification and CI and absolute value of RAP were independent predictors of CW. With addition of CPET variables, peak oxygen uptake (VO2 peak) and ∆CI independently improved the power of the prognostic model. Receiver operating characteristic (ROC)-derived cut-off values for ∆CI and VO2 peak were 0.40 liter/min/m2 and 15.7 ml/kg/min (≥60% predicted value), respectively. Twenty-nine validation cohort patients had CW (36.2%) during 710 ± 282 days. Different combinations of cut-off values of VO2 peak and ∆CI defined 4 groups. The event-free survival rates at 1, 2, and 3 years were 100%, 100%, and 100%, respectively, for the high ∆CI with high VO2 peak combination; 100%, 88%, and 71% for low ∆CI/high VO2 peak; 80%, 54%, and 40% for high ∆CI/low VO2 peak; and 72%, 54%, and 33% for low ∆CI/low VO2 peak. CONCLUSIONS: The combinations of baseline VO2 peak and change in CI during follow-up is important in prognostication of low-risk patients with idiopathic, heritable, and drug-induced PAH.
BACKGROUND: The added value of cardiopulmonary exercise testing (CPET) in the follow-up of patients with stable pulmonary arterial hypertension (PAH) remains undefined. METHODS: Idiopathic, heritable, and drug-induced PAH patients free from clinical worsening (CW) after 1 year of treatment were enrolled in derivation (n = 80) and validation (n = 80) cohorts at an interval of 6 years and followed for 3 years. Prognostic models were constructed and validated in low-risk patients in World Health Organization (WHO) Functional Class I or II with cardiac index (CI) ≥2.5 liters/min/m2 and right atrial pressure (RAP) <8 mm Hg. Discrimination and calibration were assessed. RESULTS: Forty-one derivation cohort patients had CW (51.2%) during 722 ± 349 days. Changes (∆) in WHO classification and CI and absolute value of RAP were independent predictors of CW. With addition of CPET variables, peak oxygen uptake (VO2 peak) and ∆CI independently improved the power of the prognostic model. Receiver operating characteristic (ROC)-derived cut-off values for ∆CI and VO2 peak were 0.40 liter/min/m2 and 15.7 ml/kg/min (≥60% predicted value), respectively. Twenty-nine validation cohort patients had CW (36.2%) during 710 ± 282 days. Different combinations of cut-off values of VO2 peak and ∆CI defined 4 groups. The event-free survival rates at 1, 2, and 3 years were 100%, 100%, and 100%, respectively, for the high ∆CI with high VO2 peak combination; 100%, 88%, and 71% for low ∆CI/high VO2 peak; 80%, 54%, and 40% for high ∆CI/low VO2 peak; and 72%, 54%, and 33% for low ∆CI/low VO2 peak. CONCLUSIONS: The combinations of baseline VO2 peak and change in CI during follow-up is important in prognostication of low-risk patients with idiopathic, heritable, and drug-induced PAH.
Authors: Lorriana E Leard; Are M Holm; Maryam Valapour; Allan R Glanville; Sandeep Attawar; Meghan Aversa; Silvia V Campos; Lillian M Christon; Marcelo Cypel; Göran Dellgren; Matthew G Hartwig; Siddhartha G Kapnadak; Nicholas A Kolaitis; Robert M Kotloff; Caroline M Patterson; Oksana A Shlobin; Patrick J Smith; Amparo Solé; Melinda Solomon; David Weill; Marlies S Wijsenbeek; Brigitte W M Willemse; Selim M Arcasoy; Kathleen J Ramos Journal: J Heart Lung Transplant Date: 2021-07-24 Impact factor: 13.569
Authors: Ahmad Sabbahi; Richard Severin; Cemal Ozemek; Shane A Phillips; Ross Arena Journal: Expert Rev Respir Med Date: 2020-01-03 Impact factor: 3.772
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Authors: B Pezzuto; R Badagliacca; M Muratori; S Farina; M Bussotti; M Correale; A Bonomi; C Vignati; S Sciomer; S Papa; E Palazzo Adriano; P Agostoni Journal: Pulm Circ Date: 2022-03-24 Impact factor: 2.886
Authors: Marius M Hoeper; Christine Pausch; Karen M Olsson; Doerte Huscher; David Pittrow; Ekkehard Grünig; Gerd Staehler; Carmine Dario Vizza; Henning Gall; Oliver Distler; Christian Opitz; J Simon R Gibbs; Marion Delcroix; H Ardeschir Ghofrani; Da-Hee Park; Ralf Ewert; Harald Kaemmerer; Hans-Joachim Kabitz; Dirk Skowasch; Juergen Behr; Katrin Milger; Michael Halank; Heinrike Wilkens; Hans-Jürgen Seyfarth; Matthias Held; Daniel Dumitrescu; Iraklis Tsangaris; Anton Vonk-Noordegraaf; Silvia Ulrich; Hans Klose; Martin Claussen; Tobias J Lange; Stephan Rosenkranz Journal: Eur Respir J Date: 2022-07-07 Impact factor: 33.795