Clinical characteristics and predictors of mortality in patients with acute decompensation of pulmonary arterial hypertension in cardiac intensive care setting

Pulmonary arterial hypertension (PAH) is a very rare disease with an estimated prevalence of 15 to 50 cases per million individuals. Management of patients with acute decompensation of PAH is very challenging as no specific guidelines exist in this scenario. In PAH, pathophysiology and clinical features differ from that of the heart failure due to the left ventricular dysfunction. Hence, there is no data concerning clinical features of patients with acute decompensation of pulmonary arterial hypertension especially in cardiac intensive care setting.

The purpose of our study was to determine clinical characteristics and predictors of in-hospital mortality in patients with acute decompensated PAH specifically in cardiac intensive care setting.

We retrospectively collected data of all patients with PAH (group 1 pulmonary hypertension) who were hospitalized with acute decompensation in our cardiac intensive care unit (CICU) in last 6 years. We included patients with idiopathic PAH, as well as PAH associated with connective tissue disease, congenital heart disease or portal hypertension.

We enrolled 36 patients with PAH who were hospitalized in our CICU. Basic characteristics of patients with acute decompensation of PAH are summarized in Table 1.. 61 % of patients were women, median NT-proBNP concentration level was 8397 pg/ml. 11 patients died during index hospitalization and 25 survived (in-hospital mortality rate of 31 %). Platelet count and total bilirubin level at admission were associated with increased in-hospital mortality in acute decompensated PAH. Non-survivors had lower platelet count 122 [79-214] 1000/µl vs 187 [153-248] 1000/µl (p= 0,03) and higher total bilirubin level 1,3 [0,81-2,03] mg/dl vs 0,94 [0,53-1,28] mg/dl (p = 0,04) as compared to survivors. Interestingely NT-proBNP concentration was not associated with mortality in our study (p = 0,46).

Lower platelet count and higher total bilirubin level at admission, but not NT-proBNP concentration are associated with increased in-hospital mortality in critically ill cardiac patients with acute decompensation of pulmonary arterial hypertension. In this scenario, these intermediate laboratory markers of right ventricular dysfunction may better predict survival than classical markers for left ventricular dysfunction. Further studies are necessary to confirm these findings.