IPF patients are limited by mechanical and not pulmonary-vascular factors - Results of a derivation-validation cohort study

Benjamin D. Fox, Yael Shostak, Barak Pertzov, Baruch Vainshelboim, Shimon Itzhakian, Irit Terner, Mordechai R. Kramer

Research output: Contribution to journalArticlepeer-review

Abstract

Background: During cardiopulmonary exercise testing (CPET), Idiopathic Pulmonary Fibrosis (IPF) patients do not reach their direct maximum voluntary ventilation (MVV) and have deranged gas exchange. Their exercise limitation is therefore attributed to a pulmonary vascular mechanism. Methods: We studied two cohorts (derivation and validation) of IPF patients with lung function testing and CPET. Maximal ventilation at exercise (VEpeak) was compared to direct MVV by Bland-Altman analysis. Results: In the derivation cohort (n = 101), direct MVV over-estimated VEpeak by a factor of 1.51, driven by respiratory rate during MVV that was 1.99 times higher at rest as compared to VEpeak at exercise. The formula (FEV1 × 20.1) + 15.4 was shown to predict VEpeak (r2 = 0.56) in the derivation cohort. In the validation cohort of 78 patients, VEpeak was within a factor of 1.27 (6.8 l/min) of predicted according to the novel formula. According to the novel prediction formula the majority of patients (58%) in the entire cohort have VEpeak within 85% of their predicted MVV, which would indicate a mechanical respiratory limitation to exercise. Conclusion: Estimation of direct MVV performed at rest leads to significant over-estimation of the breathing reserve in IPF patients. This may lead to over-diagnosis of pulmonary vascular limitation in these patients. Expected maximal ventilation at exercise may be accurately predicted indirectly by an IPF-specific formula.

Original languageEnglish
Article number244
JournalBMC Pulmonary Medicine
Volume19
Issue number1
DOIs
StatePublished - 11 Dec 2019

Keywords

  • Cardiopulmonary exercise test
  • Exercise
  • Idiopathic pulmonary fibrosis
  • Pulmonary hypertension

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