Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2025
Pulmonary vascular morphology in cystic fibrosis.
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Xiao Qiwei, Mylavarapu Goutham, Chmiel James F, Fleck Robert, Amoakon Jean-Pierre, Hossain Md Monir, Nakamura Aisaku, DiFrancesco Mark, Paisley Andrew, Schuler Christine L, Bates Alister, Willmering Matthew, Naren Anjaparavanda P, Estépar Raúl San José, Amin Raouf
Аннотация
BACKGROUND: Morphological changes of the pulmonary vasculature in cystic fibrosis (CF) with advancing age, disease progression and modulator therapy are not fully understood.
METHODS: Sixty-five subjects with CF and baseline pulmonary function underwent a high-resolution CT scan, exercise testing, diffusion capacity and lung clearance index. The ratio of small blood vessel volume (vessels< 5mm2 or "BV5") to total blood vessel volume (TBV) was estimated from CT scans (BV5/TBV%). The findings were validated in a second CF cohort with concurrent CT and pulmonary function at baseline. Modulator effects were determined by comparing the changes in BV5/TBV% with age and FEV1 % across groups (subjects who were naïve to modulators, those that received 1st generation modulators and those that received Trikafta). The relationship between BV5/TBV% and age in healthy controls was examined.
RESULTS: BV5/TBV% began to decline in the first decade of life and below FEV1 % of 113; this decline was associated with a decrease in diffusion and exercise parameters. The decline of BV5/TBV% with age was independent from the decline in FEV1 %. There was no significant decline in BV5/TBV% with advancing age in healthy controls or in those who received Trikafta. Early in CF, there was a relative hypervascularity of the small fraction of the pulmonary circulation which reversed to relative hypovascularity with the steady decline of BV5/TBV%.
CONCLUSIONS: The attrition of small blood vessels measured by BV5/TBV% in CF starts during the first decade of life, when lung function is normal, even with 1st generation modulator use, but not with Trikafta.
METHODS: Sixty-five subjects with CF and baseline pulmonary function underwent a high-resolution CT scan, exercise testing, diffusion capacity and lung clearance index. The ratio of small blood vessel volume (vessels< 5mm2 or "BV5") to total blood vessel volume (TBV) was estimated from CT scans (BV5/TBV%). The findings were validated in a second CF cohort with concurrent CT and pulmonary function at baseline. Modulator effects were determined by comparing the changes in BV5/TBV% with age and FEV1 % across groups (subjects who were naïve to modulators, those that received 1st generation modulators and those that received Trikafta). The relationship between BV5/TBV% and age in healthy controls was examined.
RESULTS: BV5/TBV% began to decline in the first decade of life and below FEV1 % of 113; this decline was associated with a decrease in diffusion and exercise parameters. The decline of BV5/TBV% with age was independent from the decline in FEV1 %. There was no significant decline in BV5/TBV% with advancing age in healthy controls or in those who received Trikafta. Early in CF, there was a relative hypervascularity of the small fraction of the pulmonary circulation which reversed to relative hypovascularity with the steady decline of BV5/TBV%.
CONCLUSIONS: The attrition of small blood vessels measured by BV5/TBV% in CF starts during the first decade of life, when lung function is normal, even with 1st generation modulator use, but not with Trikafta.