Worse Quality of Life, More Hospitalizations in CTEPH vs. IPAH

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by Marta Figueiredo PhD |

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People with chronic thromboembolic pulmonary hypertension (CTEPH) have significantly worse quality of life at their initial assessment, and higher hospitalization rates over time, relative to idiopathic pulmonary arterial hypertension (IPAH) patients, according to a multicenter study in the U.S.

Notably, these quality-of-life differences disappeared over time, and CTEPH patients who underwent pulmonary thromboendarterectomy or PTE — CTEPH’s mainstay treatment, a surgery to remove blood clots from major blood vessels in the lungs — experienced significant gains in quality of life, the data showed.

These findings not only support PTE’s benefits in people with CTEPH, but also highlight room for improvement in the clinical management of this patient population, the researchers noted.

The study, “Health-related quality of life and hospitalizations in chronic thromboembolic pulmonary hypertension versus idiopathic pulmonary arterial hypertension: an analysis from the Pulmonary Hypertension Association Registry (PHAR),” was published in the journal Pulmonary Circulation.

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CTEPH is a rare type of pulmonary hypertension (PH) caused by blood clots in the lungs that obstruct and increase blood pressure in the arteries. It impacts the arteries that go from the heart to the lungs, forcing the heart to work harder to pump blood.

PTE, a surgery to remove chronic blood clots from lung arteries, “remains the mainstay of treatment for patients eligible for this procedure; however, 20 to 40% of CTEPH patients have inoperable disease or are not surgical candidates for other reasons,” the researchers wrote.

In addition, one-third of those who undergo PTE “still have residual disease,” they added.

When such a surgery is not possible, CTEPH treatment may include Bayer’s Adempas (riociguat), which helps widen blood vessels, and balloon pulmonary angioplasty (BPA). BPA involves placing and inflating a balloon in lung arteries to unblock them and restore healthy blood flow.

While CTEPH has been associated with functional impairment and poor health-related quality of life, few studies have compared patient-reported outcomes between CTEPH and IPAH patients.

IPAH is a type of pulmonary hypertension of unknown cause characterized by the narrowing of the pulmonary arteries, restricting blood and oxygen flow, and raising blood pressure.

To address this knowledge gap, a team of researchers in the U.S. evaluated demographic and clinical data, as well as information on quality of life and hospitalization rates, among 169 adults with CTEPH and 461 adults with IPAH. These patients were participating in the Pulmonary Hypertension Association Registry (PHAR), established in 2015.

PHARs is collecting data over time from people with pulmonary arterial hypertension and CTEPH who are starting evaluation and/or treatment at one of the more than 50 pulmonary hypertension care centers in the U.S. accredited by the association.

Included patients had enrolled in the registry up through September 2019, a date chosen to allow at least six months of follow-up before COVID-19-related shutdowns. CTEPH patients who had already undergone PTE before registry enrollment were not included.

The patients’ quality of life was assessed with two questionnaires: the pulmonary hypertension-specific emPHasis-10 (E10) and the medical outcome short form-12 (SF-12) that includes physical and mental component scores. Lower E10 scores and higher SF-12 scores indicate better quality of life.

The data showed, that at the time of recruitment (baseline), CTEPH patients were older (mean age 58 vs. 55 years), were more often men (50% vs. 24%), and had a higher proportion of African Americans (24% vs. 11%), in comparison with IPAH patients.

People living with CTEPH also showed better blood flow parameters and similar functionality to those with IPAH. Adempas and anti-clotting medication were more commonly used in the CTEPH group.

Nearly two-thirds (63%) of CTEPH patients were referred for surgical evaluation at enrollment, and 22% subsequently underwent PTE. Most (78%) received pharmacological treatment, or medications for disease management.

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The researchers noted that, after adjusting for potential influencing factors, including age, sex, body mass index (a measure of body fat), and functional capacity, CTEPH patients had significantly worse quality of life at baseline, based on E10 scores (26 vs. 24), compared with IPAH patients.

However, this two-point difference was still smaller than the nearly six-point change considered clinically meaningful for E10 scores — “so the clinical implications of the difference in scores remain to be determined” — the researchers wrote.

In addition, these quality of life differences between the groups disappeared over time, even in CTEPH patients who did not undergo PTE, suggesting that improvements in quality of life were not associated only with this surgery.

No group differences were detected in SF-12 scores at baseline and over time.

Among CTEPH patients, those who underwent PTE were younger and more often male. Compared with medication-based treatment, the PTE group had worse E10 scores, but better cardiac function and exercise capacity. After surgery, PTE was associated with improved health-related quality of life.

Moreover, during 1,585 person-years of follow-up, people with CTEPH had significantly higher rates of all-cause hospitalizations (excluding those related to PTE) than IPAH patients, after adjusting for potential influencing factors. Person-years consider the number of people in a study as well as the amount of time they spent in the study.

This result may reflect the higher rate of simultaneous health conditions (comorbidities) and bleeding-related complications among CTEPH patients, or may reflect admissions for BPA, according to the investigators. The reported worse baseline quality of life also may be linked to older age and higher rates of comorbidities.

However, data on simultaneous conditions and BPA were not collected in PHAR at the time of the analysis, nor were the patients’ reasons for not undergoing PTE surgery (eligibility issues and refusal), limiting the study’s conclusions, the team emphasized.

Notably, to address some of these limitations, BPA data is being recorded from June 2020.

“The worse baseline quality of life and higher hospitalization rate of CTEPH patients present an opportunity for improvement in clinical management of these patients,” the researchers concluded.