Predictive value of chest HRCT for survival in idiopathic pulmonary arterial hypertension

Aneta Kacprzak,Anna Fijałkowska,Monika Szturmowicz,Szymon Darocha,Marcin Kurzyna,Barbara Burakowska,Michał Florczyk,Adam Torbicki,Maria Wieteska-Miłek

doi:10.1186/s12931-021-01893-8

Abstract

BackgroundLittle attention has been paid to chest high resolution computed tomography (HRCT) findings in idiopathic pulmonary arterial hypertension (IPAH) patients so far, while a couple of small studies suggested that presence of centrilobular ground-glass opacifications (GGO) on lung scans could have a significant negative prognostic value. Therefore, the aims of the present study were: to assess frequency and clinical significance of GGO in IPAH, and to verify if it carries an add-on prognostic value in reference to multidimensional risk assessment tool recommended by the 2015 European pulmonary hypertension guidelines.MethodsChest HRCT scans of 110 IPAH patients were retrospectively analysed. Patients were divided into three groups: with panlobular (p)GGO, centrilobular (c)GGO, and normal lung pattern. Association of different GGO patterns with demographic, functional, haemodynamic, and biochemical parameters was tested. Survival analysis was also performed.ResultsGGO were found in 46% of the IPAH patients: pGGO in 24% and cGGO in 22%. Independent predictors of pGGO were: positive history of haemoptysis, higher number of low-risk factors, and lower cardiac output. Independent predictors of cGGO were: positive history of haemoptysis, younger age, higher right atrial pressure, and higher mixed venous blood oxygen saturation. CGGO had a negative prognostic value for outcome in a 2-year perspective. This effect was not seen in the longer term, probably due to short survival of cGGO patients.ConclusionsLung HRCT carries a significant independent prognostic information in IPAH, and in patients with cGGO present on the scans an early referral to lung transplantation centres should be considered.

Highlights

Pulmonary hypertension (PH) is defined as an increase in mean pulmonary artery pressure (PAPm) ≥ 25 mmHg at rest as assessed by right heart catheterisation (RHC) [1]; recently threshold of 20 mmHg has been proposed [2]
The diagnosis may be strongly suspected in pulmonary arterial hypertension (PAH) cases with very low lung diffusion capacity for carbon monoxide (DLCO), resting hypoxemia, severe exertional desaturation, pulmonary oedema in response to PAH therapy, and the characteristic triad of findings on chest high resolution computed tomography (HRCT): centrilobular ground-glass opacities, mediastinal lymphadenopathy, and smooth thickening of the interlobular septa [1, 3,4,5,6]
General characteristics and chest HRCT findings The studied group consisted of 110 idi‐ opathic pulmonary arterial hypertension (IPAH) patients: 102 (93%) incident, 80 (73%) women, age median value was 44.3 years

Summary

Introduction

Pulmonary hypertension (PH) is defined as an increase in mean pulmonary artery pressure (PAPm) ≥ 25 mmHg at rest as assessed by right heart catheterisation (RHC) [1]; recently threshold of 20 mmHg has been proposed [2]. Ultimate distinction between PVOD/PCH and I/HPAH can be made only on histopathologic or genetic basis [1] The former can be applied only either post-mortem or in cases of lung transplantation (LTx) because lung biopsy is contraindicated in PH. The latter requires confirmation of biallelic mutations in the eukaryotic translation initiation factor 2alpha kinase 4 (EIF1AK4) gene — present in all patients with heritable form of PVOD/PCH and in about 25% of sporadic cases [1, 5]. Little attention has been paid to chest high resolution computed tomography (HRCT) findings in idi‐ opathic pulmonary arterial hypertension (IPAH) patients so far, while a couple of small studies suggested that pres‐ ence of centrilobular ground-glass opacifications (GGO) on lung scans could have a significant negative prognostic value. The aims of the present study were: to assess frequency and clinical significance of GGO in IPAH, and to verify if it carries an add-on prognostic value in reference to multidimensional risk assessment tool recommended by the 2015 European pulmonary hypertension guidelines

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Conclusion