Summary of Task Force comments.

  • 1. Presence of SAPH is associated with significant symptomatology and morbidity, as evidenced by increased WHO FC, decreased 6MWD or desaturation and increased oxygen use.

  • 2. Pulmonary hypertension is an independent predictor of mortality in patients with sarcoidosis.

  • 3. Since the cause of SAPH is multifactorial, the committee commented that the current recommendation to keep SAPH in Group 5 was reasonable. The committee considered that patients with left ventricular disease either due to sarcoidosis or other conditions should be treated as WHO Group 2. The committee felt it was important to identify a dominant cause for SAPH on an individual basis, as it is likely to have treatment implications.

  • 4. The suggestions of who should undergo transthoracic echocardiogram are summarised in figure 3.

  • 5. The committee's suggestions regarding who should undergo RHC are summarised in figure 5.

  • 6. The committee suggested that the right-heart catheterisation results should be interpreted by a multidisciplinary team with at least a sarcoidosis and PH expert and decisions should be made as summarised in figure 6.

  • 7. Evaluate for pulmonary artery stenosis or mediastinal compression by chest imaging.

  • 8. In SAPH, treatment decision and follow-up should be made by a multidisciplinary team with a sarcoidosis and a PH expert. Off-label use of PAH medical therapy may be considered for symptomatic patients on a case-by-case basis (figure 7).

  • 9. Patients who have failed to respond to treatment for PH should be referred for lung transplant evaluation, if they are deemed otherwise to be appropriate candidates.

SAPH: sarcoidosis-associated pulmonary hypertension; WHO FC: World Health Organization Functional Class; 6MWD: 6-min walk distance; RHC: right-heart catheterisation; PH: pulmonary hypertension; PAH: pulmonary arterial hypertension.