Figures
- Figure 1.
The pathway to improving long-term outcomes in pulmonary arterial hypertension. WHO-FC: World Health Organization functional class.
- Figure 2.
Proportion of patients in each World Health Organization functional class (WHO-FC) at the time of pulmonary arterial hypertension-associated systemic sclerosis (PAH-SSc) in a) routine practice (n=16) and b) when detected as part of a screening programme (n=16). p=0.036 routine versus detected patients. c) Prognosis for PAH-SSc patients detected in routine practice (n=16) or via a screening programme (n=16). p=0.0037; HR 4.15 (95% CI 1.47–11.71). Reproduced from [7] with permission from the publisher.
- Figure 3.
Results of classification and regression tree analysis in patients with pulmonary hypertension-sickle cell disease. TRV: tricuspid regurgitant velocity; 6MWT: 6-min walk test; NT-proBNP: N-terminal pro-brain natriuretic peptide; RHC: right heart catheterisation; PH: pulmonary hypertension; 6MWD: 6-min walk distance. Reproduced from [18] with permission from the publisher.
- Figure 4.
Effect of placebo (n=88) and bosentan (n=80) on the co-primary end-point pulmonary vascular resistance (PVR) in the EARLY (Endothelial Antagonist Trial in Mildly Symptomatic Pulmonary Arterial Hypertension Patients) study. Treatment effect= -22.6%, p<0.0001. Reproduced from [24] with permission from the publisher.
Tables
- Table 1. Risk factors for pulmonary arterial hypertension (PAH) and the American College of Cardiology Foundation/American Heart Association screening guidelines
Substrate Further assessment BMPR2 mutation Echocardiogram yearly; RHC if echocardiogram demonstrates evidence of PAH (high right ventricular systolic pressure estimates or right heart chambers enlargement) First-degree relative of patient with BMPR2 mutation or within pedigree of two or more patients with a diagnosis of PAH Genetic counselling and recommendation for BMPR2 genotyping; proceed as above if positive Systemic sclerosis Echocardiogram yearly; RHC if echocardiogram demonstrates evidence of PAH (high right ventricular systolic pressure estimates or right heart chambers enlargement) HIV infection Echocardiogram if symptoms or signs suggestive of PAH; RHC if echocardiogram demonstrates evidence of PAH (high right ventricular systolic pressure estimates or right heart chambers enlargement) Portal hypertension Echocardiogram if OLT considered or if symptoms or signs suggestive of PAH; RHC if echocardiogram demonstrates evidence of PAH (high right ventricular systolic pressure estimates or right heart chambers enlargement) Prior appetite suppression use (fenfluramaine) Echocardiogram only if symptomatic Congenital heart disease with shunt Echocardiogram and RHC at time of diagnosis; consider repair of defect Recent acute pulmonary embolism V′/Q′ scintigraphy 3 months after event if symptomatic; consider echocardiogram, RHC and pulmonary angiogram if positive Sickle cell disease Echocardiogram yearly; RHC if echocardiogram demonstrates evidence of PAH (high right ventricular systolic pressure estimates or right heart chambers enlargement) BMPR2: bone morphogenic protein receptor; RHC: right heart catheterisation; OLT: orthotopic liver transplantation; V′/Q′: ventilation/perfusion ratio. Reproduced from [14] with permission from the publisher.
- Table 2. Performance of an optimal screening tool#
Population Prevalence¶ % Accuracy % PPV % Screening tests/diagnosis RHC/100 RHC/diagnosis PAH-SSc 12.5 82 41 8.3 30 2.5 PortoPH 4 81 17 26.3 23 6.1 PAH-SCD 0.5 80 7 71.4 22 15.1 PAH-SLE 0.5 80 2 200 20 41 Idiopathic PAH 80 1 500 0.2 101 PPV: positive predictive value; RHC/100: number of right heart catheterisations required per 100 of population screened; RHC/diagnosis: number of right heart catheterisations performed for every patient diagnosed with pulmonary arterial hypertension (PAH); PAH-SSc: PAH associated with systemic sclerosis; PortoPH: portopulmonary pulmonary hypertension; PAH-SCD: PAH associated with sickle cell disease; PAH-SLE: PAH associated with systemic lupus erythematosus. #: assumptions: 96% sensitivity; 80% specificity. ¶: assumed for each population based on available literature and may be underestimates.
