1. Atrial septostomy

The creation of this right-left shunt is indicated in cases of right heart failure and syncope despite maximum conservative therapy. The aim is to reduce the right ventricular end-diastolic pressure. This procedure may also be used as a bridging measure before other forms of treatment.

2. Pulmonary thrombendarterectomy (PEA)

PEA is the treatment of choice for patients with CTEPH. In this operation, the blood clot and some of the vascular media are dissected out of the pulmonary vessel. The indication is based on functional restriction, haemodynamics and location of the blood clots (more central or more peripheral). Whether thromboembolic lesions can be reached depends very much on the experience of the surgical team. In the ideal case, thrombotic material can be removed even from subsegmental vascular sections. The prerequisite for PEA is oral anticoagulation for at least 3 months. Perioperative mortality is reported as between 5 and 24% depending on the centre. With a 5-year survival rate of 75–80%, PEA is clearly superior to drug therapy and also to lung transplant and should therefore be undertaken in all patients who meet the criteria for surgery.

3. Lung transplant

If the condition of a patient does not improve despite the maximum drug therapy, lung transplant constitutes a further option. The 5-year survival rate for this procedure is about 45%.

Because of the complexity of both the disease and the treatment, patients with PAH should be very closely monitored. For patients in the early stages of the disease, being given oral therapy, six-monthly check-ups are recommended. Patients at an advanced stage of the disease, particularly those receiving parenteral or combined therapy, should be observed in specialist centres at three-monthly intervals.