04 February 2019 by Frantzeska Frantzeskaki

Pulmonary arterial hypertension and sepsis: A two-edged sword


Sepsis is a significant cause of morbidity and mortality on patients with pulmonary arterial hypertension (PAH), rendering their management rather challenging.


To retrospectively evaluate the clinical and laboratory characteristics of PAH patients presenting with sepsis and the subsequent mortality.

Patients and Methods:

The study population included prevalent PAH patients, urgently admitted, because of a septic event, from 2008 to 2018. PAH diagnosis was confirmed by right heart catheterization (RHC). Diagnosis of sepsis and septic shock was based on the criteria included in the Third International Consensus definition on Sepsis and Septic shock. Multivariate analysis was used, to evaluate the risk factors associated with mortality.


Fourteen (14) patients with PAH (WHO-FC III and IV) and sepsis were included. Septic shock was present in five patients, in whom a new RHC was performed. Bloodstream infection was present in seven patients (50%). Four among them, were receiving intravenous epoprostenol through a central venous catheter, which was removed. The isolated pathogens were Micrococcus (1 patient), Methicillin Resistant Staphylococcus Aureus (3 patients), Pseudomonas aeruginosa (2 patients) and Klebsiella pneumoniae (1 patient). Pneumonia was present in 3 patients (21%), caused by Streptococcus pneumoniae, Legionella pneumophila and Influenza respectively, while one patient suffered from urosepsis. All patients received broad spectrum antibiotic therapy which was subsequently de-escalated. Specific PAH therapy was continued, while vasopressors were required in five patients, according to the results of RHC. The mean (±SD) hospital stay was 20±17 days, while the observed hospital mortality was 60%. Multivariate analysis showed that hospital mortality was significantly associated with SOFA score and need for vasopressors (p=0.001).


Sepsis is a potentially lethal complication in PAH patients. Antibiotics and specific interventions based on individualized hemodynamic assessment, might improve the prognosis of these critically ill patients.

REFERENCE: Kitterman N et al. Mayo Clinic Proceed. Sept 2012;87(9):825-34

About the author

profile picture of Frantzeska Frantzeskaki

Frantzeska Frantzeskaki

Attending physician, PHD

Attikon University Hospital


Key Contributors

Frantzeskaki F., Orfanos S.E., Konstantonis D., Rizos M., Pappas A., Triantafyllidi H., Armaganidis A., Tsangaris I. : Pulmonary Hypertension Clinic, Attikon University Hospital, National and Kapodistrian University of Athens, Greece

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