What are the treatment options for Chronic Thromboembolic Pulmonary Hypertension (CTEPH)?

Treatment Options for Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Pulmonary endarterectomy (PEA) is the recommended first-line treatment for CTEPH as it is potentially curative, with nearly normalized pulmonary hemodynamics and substantial clinical improvement in most patients. 1

First-Line Treatment: Pulmonary Endarterectomy

• PEA should be considered in all patients with CTEPH who have evidence of hemodynamic or ventilatory impairment at rest or with exercise 1
• Patients should be referred for surgical evaluation at an experienced center as soon as possible, even if symptoms are mild 1
• In Europe, in-hospital mortality for PEA is currently as low as 4.7%, and even lower in high-volume centers 1
• The majority of patients experience substantial symptom relief and near normalization of hemodynamics after PEA 1, 2
• Long-term outcomes after PEA are excellent, with survival rates of 75-92.3% at 6 years, compared to 51.9% for lung transplantation 1
• Quality of life improvements are significant, with 93% of patients in NYHA class I or II after surgery, compared to 95% in class III or IV before surgery 1

Patient Selection for PEA

• Operability assessment should be performed by a multidisciplinary CTEPH team at an experienced center 1
• Key factors in determining operability include:
  • Surgical accessibility of thrombi in the main, lobar, or segmental pulmonary arteries 1
  • Patient comorbidities and overall risk-benefit ratio 1
  • Degree of microvascular disease versus macroscopic disease in surgically accessible vessels 1
• Important considerations:
  • There is no upper limit of pulmonary vascular resistance (PVR) or degree of right ventricular dysfunction that absolutely excludes a patient from surgery 1
  • Advanced age alone is not a contraindication for surgery 1
  • Severe hemodynamic abnormalities should not automatically deem a patient "inoperable" 1

Alternative Treatments for Inoperable CTEPH

1. Medical Therapy

• Lifelong therapeutic anticoagulation is indicated for all CTEPH patients, regardless of whether they undergo surgery 1
• Warfarin targeted to an INR of 2-3 is the standard anticoagulant therapy 1
• For inoperable patients or those with persistent/recurrent PH after PEA:
  • Riociguat (a soluble guanylate cyclase stimulator) is recommended as it has demonstrated improvements in exercise capacity and pulmonary vascular resistance 1
  • Other pulmonary arterial hypertension (PAH)-specific medications may be considered off-label in selected patients 1

2. Balloon Pulmonary Angioplasty (BPA)

• BPA should be considered for patients who are:
  • Technically inoperable due to distal disease 1
  • Have an unacceptable risk-benefit ratio for PEA 1
• BPA has shown hemodynamic improvement in randomized controlled trials 3
• Perioperative mortality risk is low (<1%) in expert centers 2

3. Lung Transplantation

• Bilateral lung transplantation may be considered for advanced cases that are not suitable for PEA or other interventions 1
• This option is typically reserved for patients with severe persistent symptomatic PH despite other treatments 1

Multimodal Treatment Approach

• Many patients may benefit from a combination of treatments 2, 3
• Treatment decisions should be made by a multidisciplinary team at an experienced CTEPH center 1
• The treatment algorithm typically follows:
  1. Assessment for PEA as first-line therapy
  2. For inoperable patients: medical therapy and/or BPA
  3. For persistent/recurrent PH after PEA: medical therapy and/or BPA
  4. Lung transplantation for severe cases unresponsive to other treatments 1, 2

Supportive Care

• Diuretics and oxygen therapy should be provided in cases of heart failure or hypoxemia 1
• Regular follow-up with pulmonary hemodynamic assessment is essential to monitor treatment response 1

Common Pitfalls and Caveats

• Delay in diagnosis and referral to CTEPH centers is associated with poor outcomes 4
• Not all patients with CTEPH have a history of prior pulmonary embolism or deep vein thrombosis 5
• The preoperative differentiation of operable from inoperable CTEPH remains challenging and requires experienced assessment 1
• Patients should be referred to centers with expertise in CTEPH management, as the disease remains significantly underdiagnosed and undertreated 5