Step 1


FVC % pred./DLCO % pred.
no Alternatively, enter FVC and DLCO separately
info
FVC % predicted/DLCO % predicted
The forced vital capacity/pulmonary capacity for carbon monoxide ratio. A reduced and decreasing DLCO is a predictor of the development of PAH in patients with SSc. In PAH, the DLCO falls significantly and disproportionately to FVC; therefore, a high or increased FVC/DLCO ratio is associated with an increased risk of PAH.
Telangiectasias
yes yes no no
info
Telangiectasias
Dilated capillaries or venules that can manifest as vascular lesions on the fingers, hands, face and mucous membranes. These lesions may be a sign of ongoing vascular insult that could serve as a clinical biomarker for systemic vascular defects. There is an association between increased numbers of telangiectasias and an increased risk of PAH.
Anti-centromere antibody (ACA)
yes pos. no neg.
info
Anti-centromere antibody (ACA)
Belongs to the anti-endothelial cell antibody family, which is associated with a higher incidence of vascular manifestations. Anti-centromere antibodies may trigger the inflammation of endothelial cells and activate fibroblasts, which are some of the mechanisms underlying PAH-SSc. The detection of anti-centromere antibodies is more common in SSc patients who develop PAH.
NTproBNP
info
NTproBNP
N-terminal pro-brain natriuretic peptide. NTproBNP is a useful biomarker to detect PAH. Levels of NTproBNP reflect the severity of right ventricular dysfunction and baseline values are prognostic of patient outcome.
Serum urate
info
Serum urate
Final product of purine degradation and proposed biomarker of PAH. Elevated levels of serum urate have been found in idiopathic PAH patients. Levels of serum urate have also been found to be useful in assessing the prognosis of patients with PAH.
Right axis deviation on ECG
yes yes no no
info
Right axis deviation on ECG
One of the ECG findings that is suggestive of PAH. It is important to note that right axis deviation on the ECG is a sign of right ventricular hypertrophy and not an indication of PAH itself.


Calculate


Step 1 total risk score


No Echo
recommended
Echo recommended
220 300 370 440




Continue to Step 2
The PAH risk calculator cannot replace your specialist clinical judgment. Actelion Pharmaceuticals Ltd does not assume any responsibility for use of the PAH risk calculator.

How to use

Enter the values for each variable in the boxes provided.

Click calculate to generate the Step 1 total risk score.

Step 1 total risk score is needed for Step 2 and must be saved if Step 2 will be performed in a different session (should Step 1 and 2 be performed in the same session, the Step 1 score will be automatically entered in Step 2). Step 1 results can be documented and filed as a pdf print-out, a pdf file saved on your computer, or manually in the hospital chart.

Missing variables

If one of the six variables in Step 1 was not obtained, the calculator can still be used and provides reliable results. The missing value is automatically imputed based on the DETECT data pool. If more than one variable is missing, the total risk score cannot be calculated. If TR velocity cannot be determined because no TR is detectable upon echocardiography (see tickbox "No measurable TR"), this does not count as a missing variable.

Nomogram

The paper nomogram of the PAH risk calculator provides an alternative to the electronic version available on this website. It may be particularly helpful to demonstrate and explain the tool to colleagues, physicians in training, and students. The paper nomogram can be downloaded here. For guidance on how the nomogram works, please refer to the dedicated page How it works in the WHAT IS DETECT section of this website.