PT  - JOURNAL ARTICLE
AU  - Agustin C Martin-Garcia
AU  - Deepa RJ Arachchillage
AU  - Aleksander Kempny
AU  - Rafael Alonso-Gonzalez
AU  - Ana Martin-Garcia
AU  - Anselm Uebing
AU  - Lorna Swan
AU  - Stephen J Wort
AU  - Laura C Price
AU  - Colm McCabe
AU  - Pedro Luis Sanchez
AU  - Konstantinos Dimopoulos
AU  - Michael A Gatzoulis
TI  - Platelet count and mean platelet volume predict outcome in adults with Eisenmenger syndrome
AID  - 10.1136/heartjnl-2016-311144
DP  - 2018 Jan 01
TA  - Heart
PG  - 45--50
VI  - 104
IP  - 1
4099  - http://heart.bmj.com/content/104/1/45.short
4100  - http://heart.bmj.com/content/104/1/45.full
SO  - Heart2018 Jan 01; 104
AB  - Objectives Although a significant proportion of patients with cyanotic congenital heart disease are thrombocytopaenic, its prevalence and clinical significance in adults with Eisenmenger syndrome (ES) is not well studied. Accordingly, we examined the relationship of thrombocytopaenia and mean platelet volume (MPV) to bleeding or thrombotic complications and survival in a contemporary cohort of patients with ES, including patients with Down syndrome.Methods Demographics, laboratory and clinical data were analysed from 226 patients with ES under active follow-up over 11 years.Results Age at baseline was 34.6±11.4 years and 34.1% were men. Mean platelet count and MPV were 152.6±73.3×109/L and 9.6±1.2 fL, respectively. A strong inverse correlation was found between platelet count and haemoglobin concentration and MPV. During the study, there were 39 deaths, and 21 thrombotic and 43 bleeding events. On univariate Cox regression analysis, patients with a platelet count <100×109/L had a twofold increased mortality (HR 2.10, 95% CI 1.10 to 4.01, p=0.024). Platelet count was not associated with an increased risk of thrombosis. However, there was a threefold increased thrombotic risk with MPV >9.5 fL (HR 3.50, 95% CI 1.28 to 9.54, p=0.015). Patients with either severe secondary erythrocytosis (>220g/L) or anaemia (<130g/L) were at higher risk of thrombotic events (HR 3.93, 95% CI 1.60 to 9.67, p=0.003; and HR 4.75, 95% CI 1.03 to 21.84, p=0.045, respectively).Conclusions Thrombocytopaenia significantly increased the risk of mortality in ES. Furthermore, raised MPV, severe secondary erythrocytosis and anaemia, but not platelet count, were associated with an increased risk of thrombotic events in our adult cohort.