Decision making for patients with<unk>  can be complex and needs to be managed by<unk>  teams in expert centres.<unk>  teams comprise cardiologists and pulmonologists with specialist PH training, radiologists, experienced PEA surgeons with a significant caseload of<unk>  patients per year and physicians with percutaneous interventional expertise. Currently, there are three recognised targeted treatment options available including the standard treatment of pulmonary endarterectomy (PEA). Balloon pulmonary angioplasty (BPA) and pulmonary vasodilator drug treatment may be considered for those people that are not suitable for surgery. Specialist imaging using either magnetic resonance or invasive PA is necessary to determine risks and benefits of interventional treatment with PEA or BPA. Medical therapy Standard medical treatment consists of anticoagulants (blood thinners), diuretics, and oxygen. Lifelong anticoagulation is recommended, even after PEA. Routine inferior vena cava filter placement is not recommended. In patients with non-operable<unk>  or persistent/recurrent PH after PEA, there is evidence for benefit from pulmonary vasodilator drug treatment. The microvascular disease component in<unk>  has provided the rationale for off-label use of drugs approved for PAH. Currently, only riociguat (a stimulator of soluble guanylate cyclase) is approved for treatment of adults with inoperable<unk>  or persistent or recurrent<unk>  after surgical treatment. Other drug trials are ongoing in patients with inoperable<unk> , with macitentan recently proving efficacy and safety in MERIT Pulmonary endarterectomy pulmonary endarterectomy in a patient with<unk> . Pulmonary endarterectomy (PEA) is the gold standard treatment for suitable<unk>  patients. Operability of patients with<unk>  is determined by multiple factors among which surgical accessibility of thrombi and the patient-determined risk-benefit ratio are most important. There is no haemodynamic (e.g., considering pulmonary pressure) or age threshold that should exclude patients from surgery, and concomitant cardiac procedures can be included if necessary. However, only 60% of patients are classified as operable across Europe and Canada. In contrast to surgical embolectomy for acute PE, treatment of<unk>  necessitates a true bilateral endarterectomy (removal of blockage from the blood vessels) through the medial layer of the pulmonary arteries, which is performed under deep hypothermia (lowering of body temperature) and circulatory arrest (temporary stoppage of blood flow), which is not complicated by cognitive dysfunction. The majority of patients experience substantial relief from symptoms and improvement in haemodynamics after PEA. In Europe, in-hospital mortality during PEA is currently 4.7% or lower in high volume single centres. Up to 35% of patients may have persistent/recurrent<unk>  following surgery. The definition of post-PEA PH is still not clear, but some data suggest that 500–590 dynes·s·cm−5 may represent a pulmonary vascular resistance (PVR) threshold for poor long-term outcome. Recent data from National UK PEA cohort suggests residual PH post PEA only impacts on longer term survival when mPAP is >38 mmHg or PVR >425 dynes·s·cm−5. Bridging therapy with PAH-targeted drugs, complications and additional procedures during PEA, and residual PH after PEA are associated with worse outcomes. Immediate postoperative PVR is a long-term predictor of prognosis. Balloon pulmonary angioplasty Although BPA technique is still evolving, it may currently be considered for<unk>  patients who are technically non-operable or carry an unfavourable risk-benefit ratio for PEA. The technique has been refined in Japan and initial reports have confirmed the safety and efficacy of the technique, based on data showing haemodynamic improvement and recovery of right ventricular function. Research is ongoing.
Chronic thromboembolic pulmonary hypertension
Pulmonary hypertension
Portopulmonary hypertension
Pulmonary venoocclusive disease
Absent pulmonary valve syndrome
Bilharzial cor pulmonale
Asthma
Lettuce big-vein disease