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

Treatment of Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Pulmonary endarterectomy (PEA) is the treatment of choice for CTEPH as it is the only potentially curative option and should be considered for all patients with accessible thromboembolic lesions. 1

Diagnostic Approach

Before treatment can begin, proper diagnosis is essential:

1. Initial screening: Ventilation/perfusion (V/Q) lung scan is the preferred screening test

   • A normal V/Q scan effectively rules out CTEPH 1, 2
   • Abnormal perfusion defects warrant further investigation

2. Confirmatory testing:

   • CT pulmonary angiography to assess thromboembolic burden
   • Right heart catheterization to confirm pre-capillary PH (mean PAP ≥25 mmHg, PCWP ≤15 mmHg, PVR >2 Wood units) 1
   • Conventional pulmonary angiography to define the anatomical distribution of lesions

Treatment Algorithm

Step 1: Referral to Expert Center

• All patients with suspected CTEPH should be referred to centers with expertise in PEA 1
• A multidisciplinary team should assess operability based on:
  • Location and accessibility of thrombi
  • Degree of pulmonary hypertension
  • Comorbidities 1

Step 2: Anticoagulation

• Lifelong anticoagulation is mandatory for all CTEPH patients (Class I, Level C) 1
• Vitamin K antagonists (target INR 2.0-3.0) are the standard approach 1

Step 3: Definitive Treatment Options

A. Surgical Treatment

• Pulmonary endarterectomy (PEA) is the first-line treatment (Class I, Level C) 1
  • Mortality rates at experienced centers: 1-2% 3
  • Results in significant hemodynamic improvement and symptom relief
  • Requires cardiopulmonary bypass with deep hypothermia and circulatory arrest 1
  • Post-operative care includes continued anticoagulation 1

B. For Inoperable Patients or Residual PH After PEA

1. Medical therapy:

   • Riociguat (soluble guanylate cyclase stimulator) is the only approved medication (Class I, Level B) 1, 2
   • Improves exercise capacity and reduces pulmonary vascular resistance 1

2. Balloon pulmonary angioplasty (BPA):

   • Consider for patients with technically inoperable disease or unfavorable risk-benefit ratio for surgery (Class IIb, Level C) 2, 4
   • Can improve hemodynamics and functional capacity in selected patients 4

3. Lung transplantation:

   • Last resort for patients who fail other treatment options 1

Special Considerations

• Post-PEA monitoring: Follow-up with echocardiography and right heart catheterization to assess for residual PH
• Comorbid conditions: Inflammatory bowel disease or other bleeding disorders require careful anticoagulation management 5
• Inferior vena cava filters: Not routinely recommended due to lack of evidence 1

Prognosis

• Without treatment, CTEPH has poor prognosis with mortality rates of 70-90% in patients with severe pulmonary hypertension 2
• Successful PEA can normalize pulmonary hemodynamics and provide excellent long-term outcomes 3
• Even with inoperable disease, modern medical therapy and BPA have improved survival compared to historical outcomes 6

Key Pitfalls to Avoid

1. Misdiagnosis: Never rely solely on CT angiography to rule out CTEPH; V/Q scan is essential 1, 2
2. Premature determination of inoperability: Always have cases reviewed by an experienced PEA surgeon 1
3. Inadequate anticoagulation: Lifelong anticoagulation is mandatory, even after successful PEA 1
4. Delayed referral: Early referral to specialized centers improves outcomes 1