Surgical Treatments for Patients with PH


Atrial septostomy surgery

Atrial septostomy is a surgical operation in which a hole is made in the wall (also known as septum) between the left atrium and right atrium. Atrial septostomy allows blood to flow between these 2 chambers of the heart, and allows the heart to pump more blood.

  1. In some PAH patients who do not respond to medical treatments, atrial septostomy may be considered.
    • Quality of evidence: low
    • Recommendation: weak
  2. Atrial septostomy should only be performed at institutions with significant experience and expertise with management of PH patients and with atrial septostomy.
    • Quality of evidence: no evidence
    • Recommendation: strong based on the opinion of PH experts

Pulmonary thromboendarterectomy surgery

In some patients, PH is due to multiple or recurrent blood clots (also known as pulmonary embolism) in the lungs. This is referred to as WHO Group IV PH, or chronic thromboembolic PH (CTEPH). Pulmonary thromboendarterectomy (PTE) is a major surgical operation in which the surgeon opens up the pulmonary arteries, and removes the blood clots and scar tissue.

  1. PH patients with CTEPH should be referred to a PH center with significant experience and expertise in the diagnosis of PH and in the evaluation of PH patients for PTE surgery.
    • Quality of evidence: no evidence
    • Recommendation: strong based on the opinion of PH experts
  2. In PH patients with CTEPH considered to be eligible for PTE surgery, PTE is the first treatment of choice for treating PH. PTE surgery improves pulmonary hemodynamics, clinical and functional status, and survival.
    • Quality of evidence: low
    • Recommendation: moderately strong
  3. Some PH patients with CTEPH are not eligible for PTE surgery. Some CTEPH patients have significant PH which persists after PTE surgery. In these CTEPH patients, medical PH treatments or lung transplantation should be considered. Currently, riociguat (Adempas) is the only approved medication for inoperable CTEPH or persistent PH following PTE surgery in Canada.
    • Quality of evidence: low
    • Recommendation: weak

Lung or heart-lung transplantation of PH patients

In some PH patients, PH may not respond to medical PH treatment. In other PH patients, PH may progress after initially responding to medical PH treatment. In patients with severe, progressive PH and right ventricular (RV) failure, removal of the patient’s lungs and transplantation of new donor lungs is a final treatment option. In most patients with PH, lung transplantation (LT) is adequate. In some patients with PH and heart disease (such as defects of the heart present at birth, also known as congenital heart disease = CHD), both heart & lung transplantation (HLT) may be required.

  1. WHO class III and IV PAH patients should be referred to a transplant medical center for evaluation and listing for LT or HLT.
    • Quality of evidence: low
    • Recommendation: moderately strong
  2. PAH patients accepted and listed for LT or HLT may stabilize on medical PH treatment, and may not actually require LT or HLT.
  3. PAH patients accepted and listed for LT or HLT who do not respond to PH treatment have a poor prognosis and a high risk of death. These patients should undergo LT or HLT.
    • Quality of evidence: fair
    • Recommendation: Strong
  4. In PAH patients who undergo transplantation, the procedure of choice is bilateral or double LT.
    • Quality of evidence: low
    • Recommendation: weak
  5. In children with PAH who are undergoing transplantation, the procedure of choice is also bilateral or double LT.
    • Quality of evidence: low
    • Recommendation: Moderately strong
  6. In adult PAH patients with simple types of CHD (such as atrial septal defect = ASD, ventricular septal defect = VSD), bilateral or double LT with repair of the CHD defect is the procedure of choice.
    • Quality of evidence: low
    • Recommendation: weak
  7. In adult PAH patients with complex types of CHD, HLT is the procedure of choice.
    • Quality of evidence: low
    • Recommendation: Moderately strong