Treatment Options for Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

First-Line Treatment: Pulmonary Endarterectomy

The American Heart Association recommends pulmonary endarterectomy (PEA) as the first-line treatment for CTEPH, as it is potentially curative, with nearly normalized pulmonary hemodynamics and substantial clinical improvement in most patients 1, 2
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, 3
In-hospital mortality for PEA is currently as low as 4.7%, and even lower in high-volume centers 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

Operability assessment should be performed by a multidisciplinary CTEPH team at an experienced center 2, 4
Key factors in determining operability include surgical accessibility of thrombi, patient comorbidities, and degree of microvascular disease versus macroscopic disease in surgically accessible vessels 2, 1
There is no upper limit of pulmonary vascular resistance (PVR) or degree of right ventricular dysfunction that absolutely excludes a patient from surgery 1, 2
Advanced age alone is not a contraindication for surgery 2

Lifelong therapeutic anticoagulation is indicated for all CTEPH patients, regardless of whether they undergo surgery, with warfarin targeted to an INR of 2-3 as the standard anticoagulant therapy 1, 5, 2
Riociguat is recommended for inoperable patients or those with persistent/recurrent PH after PEA, as it has demonstrated improvements in exercise capacity and pulmonary vascular resistance 2, 4
Balloon pulmonary angioplasty (BPA) should be considered for patients who are technically inoperable due to distal disease or have an unacceptable risk-benefit ratio for PEA 2, 4
Bilateral lung transplantation may be considered for advanced cases that are not suitable for PEA or other interventions 6, 2

Treatment decisions should be made by a multidisciplinary team at an experienced CTEPH center 5, 2
The treatment algorithm typically follows a step-wise approach, starting with assessment for PEA as first-line therapy, followed by medical therapy and/or BPA for inoperable patients, and lung transplantation for severe cases unresponsive to other treatments 2

Diuretics and oxygen therapy should be provided in cases of heart failure or hypoxemia 2
Regular follow-up with pulmonary hemodynamic assessment is essential to monitor treatment response 2

The preoperative differentiation of operable from inoperable CTEPH remains challenging and requires experienced assessment 1