REVEAL: a contemporary US pulmonary arterial hypertension registry

Inclusion characteristics of a) previously diagnosed and b) newly diagnosed pulmonary arterial hypertension (PAH) patients enrolled in REVEAL. PVR: pulmonary vascular resistance; P̄_pa: mean pulmonary artery pressure; P_pcw: pulmonary capillary wedge pressure; LVEDP: left ventricular end-diastolic pressure.

a) Pulmonary arterial hypertension (PAH) aetiological breakdown of REVEAL patients at enrolment. b) Breakdown of associated PAH subgroup. PPHN: persistent pulmonary hypertension of the newborn; PVOD: pulmonary veno-occlusive disease; PCH: pulmonary capillary haemangiomatosis; FPAH: familial PAH; APAH: associated PAH; IPAH: idiopathic PAH; CHD: congenital heart disease; CVD/CTD: collagen vascular disease/connective tissue disease. Reproduced from [15] with permission from the publisher.

Pulmonary arterial hypertension specific medication use at enrolment among previously diagnosed patients. 184 (7%) of patients were not on a prostaglandin, phosphodiesterase type-5 inhibitor or endothelin receptor antagonist. Of these, 88 were on calcium channel blockers for the treatment of pulmonary arterial hypertension.

1-yr survival estimates of 641 connective tissue disease-associated pulmonary arterial hypertension (CTD-APAH) patients compared with 1,251 idiopathic pulmonary arterial hypertension (IPAH) patients in REVEAL. Log rank p<0.0001. Reproduced from [22] with permission from the publisher.

Cox proportional hazard estimates for multivariate model of survival, limited to terms included in the final stepwise model. Parameters significantly associated with 1-yr survival include the Borg dyspnoea scale, right ventricular dysfunction, pulmonary vascular resistance (PVR) index, pulmonary capillary wedge pressure, cardiac index, mean pulmonary artery pressure and total serum bilirubin. Candidate predictor variables that were not significant at the univariable level included Tei index, vasoreactivity, race, newly diagnosed pulmonary arterial hypertension (PAH) and income. Missing Borg scale and missing PVR index were both associated with lower than average observed survival and were, therefore, considered candidate predictor variables. WHO: World Health Organization; CTD: connective tissue disease; APAH: associated PAH; PoPH: portal hypertension; FPAH: familial PAH; NYHA: New York Heart Association; 6MWD; 6-min walk distance; BNP: brain natriuretic protein; D_L,CO: diffusing capacity of the lung for carbon monoxide; % pred: % predicted; RHC: right heart catheterisation; P̄_ra: mean right atrial pressure. Data are presented as hazard ratio (HR) with 95% confidence level. ^#: reference category was NYHA/WHO functional class II or missing; ^¶: if N-terminal proBNP is available and BNP is not, cut-off points are replaced with <300 pg·mL⁻¹ and >1,500 pg·mL⁻¹; ⁺: restricted to tests performed within 1 yr of enrolment, otherwise, the indicator is set to 0. Reproduced from [27] with permission from the publisher.

Survival estimates of patients in REVEAL using Kaplan–Meier estimates stratified by newly versus previously diagnosed patients and survival estimated by a delayed entry model accounting for truncation. The model includes all REVEAL patients but the number of patients at risk is limited to the time period during which they were followed in the study. Therefore, the first 3 months is estimated exclusively based on newly diagnosed patients and thereafter combined data from previously diagnosed patients and long-term follow-up of newly diagnosed patients. ^#: newly diagnosed; ^¶: previously diagnosed.