OBJECTIVES: Pulmonary arterial hypertension (PAH) is a rare condition, estimated to affect between 15 to 60 people per million in Europe, with between 5 to 10 new cases per million per year. In PAH, the pre-capillary arterioles are affected by an angioproliferative vasculopathy that progressively increases the pulmonary vascular resistance, thus increasing the right ventricular afterload, leading to right heart failure (HF), which is the ultimate cause of death. The evaluation of right heart deterioration parameters is important to detect early development of HF and to allow timely intervention with parenteral prostanoid therapy.

Our purpose was to assess the burden of HF in PAH patients, in terms of hospitalization, readmission rates and length of hospital stay.

METHODS: A systematic literature search was conducted in PubMed. Three search strategies were designed, combining MeSH terms and free terms, i.e. “heart failure”, “economics”, “cost and cost analysis”, “costs”, “pulmonary arterial hypertension”. No language or geographic limits were considered. Results were peer-reviewed, and a third party was consulted in case of discrepancies.

RESULTS: The search strategies resulted in 3,122 references after duplicates removal. Further filtering excluded 2,794 references, resulting in 36 for analysis. Results showed high variability in rates, periods and were mostly driven by the severity of PAH (functional class). PAH-related hospitalization rates due to HF ranged mostly between 10% and 61% (78% maximum). Up to 59% of patients required subsequent rehospitalization due to HF complications. The mean length of hospital stay varied from 6.8-23.8 days in intensive care units, followed by an additional 6.5-33.9 days in general hospital wards.

CONCLUSIONS: PAH is a chronic, life-threatening disease. Hospitalization and readmission rates due to HF in PAH patients involve a significant economic burden for health systems. Prostacyclin therapies improve symptomatology and survival among PAH patients; they are associated with lower rehospitalization rates and longer times to readmission.