Figures
- Figure 1.
Survival of newly diagnosed (incident) patients with idiopathic, heritable or anorexigen-associated pulmonary arterial hypertension by New York Heart Association/World Health Organization functional class (NYHA/WHO FC) assessed at the time of diagnosis. Reproduced from [4] with permission from the publisher.
Tables
- Table 1. Characteristics of pulmonary arterial hypertension according to New York Heart Association/World Health Organization functional class (NYHA/WHO FC) at diagnosis
Parameter NYHA/WHO FC p-value I/II III IV 6MWD n 134 359 55 Distance m 415±86 319±92 192±96 <0.0001 Haemodynamic variables n 162 405 77 Pra mmHg 6±4 9±5 11±7 <0.0001 P̄pa mmHg 50±17 56±15 56±13 0.0002 Ppcw mmHg 8±3 8±3 8±4 0.75 Cardiac index L·min−1·m−2 2.9±0.9 2.4±0.7 2.1±0.8 <0.0001 Sv,O2 67±8 62±8 54±9 <0.0001 PVRI mmHg·L·min·m−2 15.8±9.7 21.5±9.8 25.5±10.1 <0.0001 Data are presented as mean±sd, unless otherwise stated. 6MWD: 6-min walk distance; Pra: right atrial pressure; P̄pa: mean pulmonary arterial pressure; Ppcw: pulmonary capillary wedge pressure; Sv,O2: venous oxygen saturation; PVRI: pulmonary vascular resistance index. Reproduced from [10] with permission from the publisher.
- Table 2. Parameters with established importance for assessing disease severity, stability and prognosis in pulmonary arterial hypertension
Better prognosis Determinants of prognosis Worse prognosis No Clinical evidence of right ventricle failure Yes Slow Rate of progression of symptoms Rapid No Syncope Yes I, II NYHA/WHO FC IV Longer (>500 m)# 6MWT Shorter (<300 m) Peak O2 consumption >15 mL·min−1·kg−1 Cardio-pulmonary exercise testing Peak O2 consumption <12 mL·min−1·kg−1 Normal or near normal BNP/NT-proBNP plasma levels Very elevated and rising No pericardial effusion TAPSE¶ >2.0 cm Echocardiographic findings¶ Pericardial effusion TAPSE¶ <1.5 cm Pra <8 mmHg and CI ≥2.5 L·min−1·m−2 Haemodynamics Pra >15 mmHg or CI ≤2.0 L·min−1·m−2 NYHA/WHO FC: New York Heart Association/World Health Organization functional class; 6MWT: 6-min walk test; BNP: brain natriuretic peptide; NT-proBNP: N-terminal pro-B-type natriuretic peptide; TAPSE: tricuspid annular plane systolic excursion; Pra: right atrial pressure; CI: cardiac index. #: depending on age; ¶: TAPSE and pericardial effusion have been selected as they can be measured in the majority of patients. Reproduced from [13] with permission from the publisher.
- Table 3. Percentage of New York Heart Association/World Health Organization functional class (NYHA/WHO FC) patients enrolled in randomised controlled trials (RCTs) with monotherapies
Study drug RCT acronym or study group Patients Subjects n NYHA/WHO FC IV Duration weeks Primary end-point Epoprostenol Rubin et al. [22] IPAH 23 NA 8 NA Barst et al. [23] IPAH NYHA/WHO FC III/IV 81 26% 12 6MWD Badesch et al. [24] SSc 111 17% 12 6MWD Treprostinil Simonneau et al. [25] IPAH, CTD, CHD 469 7% 12 6MWD Beraprost ALPHABET [26] PAH 130 0% 12 6MWD Barst et al. [27] IPAH, CTD, CHD 116 0% 52 TTCW Iloprost AIR [28] IPAH, CTD, CTEPH 203 41% 12 Composite Bosentan BREATHE-1 [29] IPAH, CTD 213 8.5% 16 6MWD Ambrisentan ARIES 1 [30] PAH 202 7% 12 6MWD ARIES 2 [30] PAH 192 2.3% 12 6MWD Sildenafil SUPER 1 [31] IPAH, CTD, CHD 277 3% 12 6MWD Tadalafil PHIRST [32] PAH 405 1.7% 16 6MWD PAH: pulmonary arterial hypertension; IPAH: idiopathic PAH; SSc: systemic sclerosis-associated PAH; CTD: connective tissue diseases-associated PAH; CHD: congenital heart disease-associated PAH; CTEPH: chronic thromboembolic pulmonary hypertension; NA: not applicable; 6MWD: 6-min walk distance; TTCW: time to clinical worsening.
