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Introduction: The risk stratification in idiopathic pulmonary arterial hypertension (IPAH) patients is currently based on haemodynamic and functional parameters as well as serum biomarker concentrations. Until now the importance of changes appearing in high-resolution computed tomography (HRCT) of the lungs of patients with IPAH has not been investigated. Material and methods: Lung HRCT scans were analysed retrospectively in 48 IPAH patients (patients): 37 women, 11 men, mean age 41 ± 15 years. Results: Focal ground-glass opacifications (FGG) were found in 12 patients (25%), and centrilobular nodules (CN) were found in 8 patients (17%). In the remaining 58% of patients HRCT revealed no changes (N). Significantly lower stroke volume was found in the CN group (41.0 ± 8.5 ml) compared to 60.8 ± 15.1 ml in the FGG group and 58.1 ± 18.0 ml in the N group (p = 0.03). Right atrial pressure was significantly higher in the CN group (12.2 ± 4.86 mm Hg) than in the FGG group (6.9 ± 3.9 mm Hg) and the N group (7.6 ± 5.3 mm Hg), p = 0.047. The presence of nodules was combined with considerably increased risk of death, both in univariate analysis (HR 5.35, 95% CI: 1.16–24.7, p = 0.03) and in multivariate analysis (HR 6.98, 95% CI: 1.41–34.59, p = 0.02). Ground-glass opacifications correlated neither with haemodynamic nor functional indexes, and were of no prognostic significance. Conclusions: The presence of centrilobular nodules in lung HRCT scans of IPAH patients was combined with more severe haemodynamic compromise and was an independent negative prognostic indicator.

The authors describe a case of 21-years old woman with idiopathic pulmonary arterial hypertension with atypical clinical consequences of massive internal bleeding. Despite significant hypovolemia clinical and laboratory presentation was one of RV failure with dilatation of right heart ventricle and increased plasma level of markers of myocardial stretch and injury (NT-proBNP and troponin, respectively). This is attributed to impaired right ventricular coronary perfusion and hypoxia. Intensive treatment restored baseline RV conditions and at 15 months follow-up no persistent right heart impairment was observed. This case demonstrates that bleeding should be also considered in differential diagnosis of exacerbation of right ventricular failure in patients with pulmonary arterial hypertension.