Screening for PAH

The key to early diagnosis is introducing screening for high risk patient populations if they are asymptomatic.

High risk patient populations include:
• Family members of a patient with familial Pulmonary Arterial Hypertension (PAH)
• Patients with systemic sclerosis (SSc)
• Patients with HIV
• Patients with portopulmonary hypertension (PoPH)

The results of a disease registry in France indicated that without screening, the majority of patients were diagnosed in FC III or FC IV, and only 24% of patients were in FC II at diagnosis.¹ (See Fig. 1, left panel.) Furthermore, the results of a national screening programme in a high-risk population indicated that it is possible to detect Pulmonary Arterial Hypertension (PAH) in an earlier stage in a high-risk population (see Fig. 1, right panel).²

Figure 1. WHO FC at diagnosis, with or without screening
Humbert et al 2006 Am.J.Respir.Crit Care Med. 173, Hachulla et al 2005 Arthritis Rheum. 52

International guidelines now recommend annual screening high-risk groups with Doppler echocardiography.^3-5 Doppler echocardiography is currently the most effective method for screening, however, for a definitive diagnosis right heart catheterisation has to be performed (see ‘How is PAH diagnosed?' ).

References
1. Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am.J.Respir.Crit Care Med. 2006;173:1023-1030.
2. Hachulla E, Gressin V, Guillevin L, et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum. 2005;52:3792-3800.
3. Hachulla E and Coghlan JG. A new era in the management of pulmonary arterial hypertension related to scleroderma: endothelin receptor antagonism. Ann.Rheum.Dis. 2004;63:1009-1014.
4. Galiè N, Torbicki A, Barst RJ, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension: The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur Heart J 2004;25:2243-2278.
5. McGoon M, Gutterman D, Steen V, et al. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004;126:14S-34S.