by Phyllis Hanlon, Contributing Writer
In 1967, doctors performed the first pulmonary endarterectomy (PEA) (also known as pulmonary thromboendarterectomy or PTE) – surgery to remove old blood clots from the pulmonary arteries. This operation continues to be the first recommended treatment for patients with chronic thromboembolic pulmonary hypertension (CTEPH), since it can potentially reduce or even normalize blood pressure in the lungs. However, some patients with CTEPH are not eligible for the procedure. For some patients with inoperable disease, treatment with drugs like riociguat may be an option. Other interventions are available, but are not yet well supported with clinical data. Nick H. Kim, M.D., and Gérald Simonneau, M.D., reviewed the current treatment options for CTEPH and presented their findings in July 2016. (https://www.ncbi.nlm.nih.gov/pubmed/27571007)
The authors looked first at the issue of deciding whether a CTEPH patient is a candidate for PTE, which can be challenging due to variations in evolving guidelines. Whether a patient is considered eligible for surgery is largely still subjective, and hinges upon the experience and abilities of surgical centers to perform PTE. The authors stated that 37 percent of patients included in an international registry did not qualify for PTE surgery for reasons that are not universally considered absolute criteria. (https://www.ncbi.nlm.nih.gov/pubmed/21969018)
Drs. Kim and Simonneau recommended that a multidisciplinary team make the assessment on whether a CTEPH patient should have surgery. A PTE surgeon, CTEPH physician and CTEPH imaging radiologist should collaborate using high-quality imaging to make the determination regarding surgery. They stated that the “…operability definition in CTEPH is, in reality, a moving and evolving concept.” Also, when a clear determination cannot be made, the authors recommend getting a second opinion.
The authors also discuss the use of pulmonary artery specific therapies to medically treat CTEPH, in order to optimize pulmonary artery pressures and right heart function surgery. There is currently no evidence to support that this will improve surgical outcomes. They also pointed out that medication should only be considered for patients physicians feel do not qualify for surgery, or for those who still have pulmonary hypertension (PH) following PTE. For now, Drs. Kim and Simonneau recommend that the use of PH-targeted therapy in potentially operable CTEPH patients be avoided.
The third option, balloon pulmonary angioplasty (BPA), fell out of favor when the procedure resulted in a 61 percent lung injury rate, the authors noted. More recent improvements to the technique have shown promising results; complication rates are lower and outcomes are better, “comparable to those following pulmonary endarterectomy” for some. However, there have been no studies on long term effects and practitioners still have questions related to BPA and the benefits it might have for CTEPH patients.
One of the key questions the authors raise is who should be performing balloon pulmonary angioplasty. Should patients go to centers that perform just a few of these procedures or should they opt for specialty centers that perform many? The authors wrote, “…we need robust balloon pulmonary angioplasty outcomes data to set expectations and best practice guidelines.”
In conclusion, Drs. Kim and Simonneau stressed the need for a better understanding of the connection between blood clots in the lungs and CTEPH, in order to enable better clinical decisions when it comes to CTEPH treatment. They also recommend that treatment should be individualized and that every patient should be assessed for PTE surgery.
Each PH patient is different. It is essential that you talk to your own doctor about what treatment options are best for you. For more information on finding a doctor or an accredited care center, visit https://www.phassociation.org/PHCareCenters/Patients.
