Pulmonary Vascular Disease |

Survival in PAH Patients in the Era of Targeted Treatment: A Single Center Experience FREE TO VIEW

Milan Luknar, MD; Branislav Liska, MD; Peter Lesny, MD; Ivan Varga, MD; Peter Solik, MD; Eva Goncalvesova, MD
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National Cardiovascular Institute, Bratislava, Slovakia

Chest. 2014;145(3_MeetingAbstracts):514A. doi:10.1378/chest.1835244
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SESSION TITLE: Pulmonary Hypertension Posters II

SESSION TYPE: Poster Presentations

PRESENTED ON: Saturday, March 22, 2014 at 01:15 PM - 02:15 PM

PURPOSE: Despite the availability of specific treatment, pulmonary arterial hypertension (PAH) remains a devastating and fatal disease. Slovakia has a systemic program of PAH management. Mortality data, however, have been lacking. The aim was to describe the survival in pts with newly diagnosed PAH at a referral centre in Slovakia in the era of targeted treatment and analyse causes of death.

METHODS: We analyzed characteristics and survival of 68 pts (17 men) newly diagnosed with PAH at the National Cardiovascular Institute in Bratislava from October 2005 to June 2012. The diagnosis was based on right heart catheterization and a comprehensive diagnostic algorithm. Median age at diagnosis was 58 (18-79) yrs. Mean WHO functional class (FC) was 2.9±0.61. Thirty-seven pts had idiopathic PAH, 18 PAH associated with connective tissue disease, 8 with congenital heart disease, 3 portopulmonary hypertension, and 2 other PAH. Forty-one pts received endothelin receptor antagonists, 32 sildenafil, 11 treprostinil, and 10 no specific treatment. Epoprostenol has not been available. Combination treatment was used in 26 pts. Four pts were long-term responders to calcium channel blockers. Non-responders (n=64) were included in Kaplan-Meier survival analysis from the time of diagnosis. Survival in pts who underwent lung transplant was censored at the time of surgery.

RESULTS: During the follow-up period, 12 pts died. The cause of death was progressive right heart failure in 11 pts and sudden death in 1 patient. One patient underwent urgent lung transplantation. One-, 2-, 3-, and 4-year survival rates were 88% (95% CI, 84-92), 85% (95% CI, 80-90), 76% (95% CI, 69-83), and 67% (95% CI, 59-75), resp.

CONCLUSIONS: Survival in patients with incident PAH in a single centre in Slovakia is comparable to major international registries. Mortality remains high and most patients die from progressive right heart failure.

CLINICAL IMPLICATIONS: Despite the availability of targeted treatments, the prognosis of PAH patients remains poor. Novel treatments are needed, right heart failure being an important target.

DISCLOSURE: The following authors have nothing to disclose: Milan Luknar, Branislav Liska, Peter Lesny, Ivan Varga, Peter Solik, Eva Goncalvesova

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