The increased incidence of pulmonary hypertension and its association with decreased survival is well-recognised in patients with systemic sclerosis. This association is not widely appreciated in patients with polymyositis-dermatomyositis. We report clinical and hemodynamic characteristics and response to vasoactive therapy in three patients with polymyositis-dermatomyositis and pulmonary hypertension and discuss them in light of the available literature.

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Objectives: Utility is a health state value that is influenced by disease, treatments or policy. The use of the EuroQOL five dimension questionnaire (EQ-5D) has been frequently implemented to measure utility in cardiovascular (CV) disease. The objective of the analysis was to report baseline EQ-5D estimates in high CV risk patients with hypercholesterolemia not optimally treated with maximally tolerated statins ± other lipid-lowering treatments. MethOds: Baseline EQ-5D was calculated via pooled analysis of the ODYSSEY FH I, FH II, HIGH FH, COMBO I, COMBO II and LONG TERM registered clinical trials to assess the efficacy and safety of alirocumab for lowering low-density lipoprotein cholesterol (LDL-C) in high CV risk patients. High CV risk patients included: history of recent acute coronary syndrome (ACS); coronary heart disease (CHD); ischemic stroke (IS); peripheral artery disease (PAD); and heterozygous familial hypercholesterolemia (HeFH). The five EQ-5D item scores were estimated and the UK tariff was applied. Results were calculated for each patient segment and were not mutually exclusive, i.e. patients may have had a history of > 1 of the above-mentioned CV conditions. Results: 4,203 patients with baseline EQ-5D were included. Mean age was 59 years and 63% were male. Results by patient segment were: ACS 0-12 months, age 56 years, utility 0.844; ACS 12-24 months, age 59 years, utility 0.858; CHD, age 61 years, utility 0.851; IS, age 64 years, utility 0.797; PAD, age 63 years, utility 0.771; HeFH, age 53 years, utility 0.905. cOnclusiOns: Unadjusted results suggest that the mean baseline EQ-5D for all high CV risk patients ranges from 0.771 for PAD patients to 0.905 for HeFH patients. These results are important for informing the utilities of high CV risk patients.

Similar to patients with chronic lung diseases, PH in the setting of OSA generally appears to be mild to moderate, 24,25 with severe PH being less common. 7,24,25 Largely as a result of concern over the invasive nature of RHC, the true prevalence of PH in OSA is not known due to a lack of large, population-based studies. Prevalence data on resting, awake PH in OSA is based primarily on retrospective case series or prospective cohort studies with poorly defined entry criteria (Table 1). 7,12,13,18,[22][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37] This literature is further confounded by several limitations-the most important among which are a lack of consistency in defining PH, the varying methods used to detect its presence, and the lack of exclusion of other secondary causes with proper evaluation. It is generally accepted that measurement of PH in obese patients with OSA is especially prone to error when performed by Doppler echocardiography (DE), 5,10,38,39 and although DE estimates of right ventricular systolic pressure (RVSP) correlate strongly with systolic PAP measured by RHC, there is large variation among individual pa-Key Words-obstructive sleep apnea, pulmonary hypertension, right heart failure, right ventricular hypertrophy Address for reprints and other correspondence: minaio@ccf.org AP Chua, MD OA Minai, MD Downloaded from