Pre-surgery Risk Assessments in CTEPH Did Not Foresee Outcomes

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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An illustration of risk, with its dial pointed in the high-risk side.

Among people with chronic thromboembolic pulmonary hypertension (CTEPH), risk assessments and treatment patterns prior to surgical procedures do not predict outcomes after surgery, a new study from Sweden shows.

The results suggest that most CTEPH patients who don’t undergo surgery remain in the same risk category for at least one year after diagnosis — which, researchers say, highlights a need for better treatment for these patients.

Clinicians also should reevaluate their surgical criteria, the team suggested, noting that age, other co-existing medical conditions — called co-morbidities — and the characteristics of lesions were “the primary determining factor for choosing/not choosing [surgery].”

Data showed that patients in the surgery group were younger, had higher mean pulmonary artery pressure, and had fewer co-morbidities than the non-surgical group. “Including these factors in the risk assessment tool might yield information important for outcome after surgery,” the scientists wrote.

The study, “Five year risk assessment and treatment patterns in patients with chronic thromboembolic pulmonary hypertension,” was published in the journal ESC Heart Failure.

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CTEPH is a rare form of pulmonary hypertension (PH) in which blood clots cause increased pressure in the lung’s blood vessels. A surgical treatment to remove the clots, called pulmonary endarterectomy or PEA, can improve blood flow dynamics for people with CTEPH. However, many patients are not eligible for the surgery, and up to a third of individuals who undergo PEA experience persistent or recurring PH.

Investigating treatment outcomes in CTEPH

Now, a team of scientists conducted a retrospective analysis of CTEPH patients in the Swedish pulmonary arterial hypertension registry, known as SPAHR. Launched in 2008, this registry records clinical data for more than 90% of the people with CTEPH in Sweden.

The analysis included 311 people — half women, half men — with CTEPH. The median age at diagnosis was 70, and 98 (32%) underwent PEA. The researchers conducted separate analyses for these patients and for those who did not undergo the surgical procedure.

In the PEA group, the pre-surgery assessment indicated that 20% of the patients had low-risk disease, while 69% were intermediate risk, and 10% were high risk. The assessment of risk was based on standardized measures, taking into account several measures of blood flow dynamics as well as assessments of heart function, motor function, and biomarkers of heart health. Prior to surgery, 60% of the patients were on PH-specific medications.

During five years of follow-up after surgery, 37 patients who received PEA were treated with PH-specific therapies at a long-term visit. Among these patients, 13 were assessed as low-risk after surgery, 23 as intermediate-risk, and one as high-risk.

“In patients who underwent PEA, the pre-PEA risk did not foresee long-term risk after surgical intervention, and pre-surgery treatment with PH-specific drugs did not foresee the treatment patterns after intervention,” the researchers wrote.

Five years after PEA, 62 patients were alive and evaluable — 13 had died; the rest did not have data available. Among the living patients, 34 patients had no PH-specific treatments recorded beyond two years post-PEA.

Among the 213 CTEPH patients who didn’t undergo PEA, 28% were assessed as low risk at diagnosis, while 61% were identified as intermediate risk, and 11% as high risk. About three-quarters of the non-surgical patients were started on a PH-specific treatment within three months of being diagnosed; those at higher risk were generally more likely to start such prompt treatment.

“The highest proportion of untreated patients was assessed as low risk, while most patients at intermediate, and all patients at high risk, were on an upfront PH-specific treatment,” the researchers wrote.

After one year, a majority of the non-PEA patients (57%) remained in the same risk category they had been in at diagnosis. Meanwhile, 14% had improved from intermediate to low risk and 0.5% from high to low risk.

“Patients without PEA tend to remain at the same risk status over time, suggesting room for improved treatment strategies in this group,” the scientists concluded.


A Conversation With Rare Disease Advocates