Abstract

Inhomogeneity of lung attenuation pattern is observed in high resolution chest computed tomography (HRCT) in some IPAH patients despite lack of interstitial lung disease. Such radiological changes are described either as ill-defined centrilobular nodules (CN) or as focal ground glass opacities (FGGO). There is no consensus in the literature, whether they indicate the distinct type of IPAH, or pulmonary venoocclusive disease (PVOD) with subtle radiological changes. Thus the aim of the present pilot study was to assess the frequency and clinical significance of inhomogenic lung attenuation pattern in IPAH. 52 IPAH patients (38 females, 14 males, mean age 41 years ± 15 years), entered the study. All available chest CT scans were reviewed retrospectively by the experienced radiologist, not aware about the clinical data of the patients. CN were found in 10 patients (19%), FGGO - in 12 patients (23%). No lymphadenopathy or interlobular septal thickening suggestive of PVOD were found. The significant differences between CN and the remaining patients included: lower mean age - 31 and 43.5 years, (p = 0.02), lack of persistent foramen ovale (PFO) - 0% and 43% (p = 0.03), and higher mean right atrial pressure (mRAP) - 12.5 mm Hg and 7.94 mm Hg (p = 0.01). No significant survival differences were observed between the groups of CN, FGGO and the remaining patients. Centrilobular nodules in IPAH were combined with lack of PFO, higher mRAP and younger age of patients.

Highlights

  • Inhomogeneity of lung attenuation pattern is observed in high resolution chest computed tomography (HRCT) in some idiopathic pulmonary arterial hypertension (IPAH) patients despite lack of interstitial lung disease

  • IPAH was recognized in the patients with precapillary pulmonary hyper­ tension (PH) confirmed by right heart catheterization, and defined as mean pulmonary artery pressure ≥ 25 mm Hg, pulmonary artery wedge pressure (PAWP) ≤ 15 mm Hg, and pulmonary vascular resistance (PVR) > 3 WU [12]

  • The standard procedures in IPAH patients consisted of the assessment of functional class (FC) according to WHO, arterialized blood gas analysis, 6-minute walk test (6MWT) performed on the flat ground according to ATS guidelines [13]

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Summary

Introduction

High resolution computed tomography (HRCT) of the lung is an important tool applied to the differential diagnosis of pulmonary hyper­ tension (PH). Significant inter­ stitial lung disease in HRCT excludes idiopathic pulmonary arterial hypertension (IPAH) [1]. 5, pages 265–270 the radiological signs suggestive of pulmonary venoocclusive disease (PVOD) [3−6]. There is no consensus in the literature, wheth­ er such radiological abnormality indicates the distinct type of IPAH or PVOD with subtle radio­ logical changes [8]. The answer to this question is important, as PVOD is combined with worse prognosis, targeted PAH therapy may be harmful and lung transplantation has to be taken into account in the early course of the disease [9−11]. The aim of the present pilot study was to assess the frequency and clinical significance of nonhomogenous lung attenuation pattern in IPAH patients

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