Vascular disease

First Australian snapshot of CTEPH reveals opportunities for better care

Australia’s first major snapshot into chronic thromboembolic pulmonary hypertension (CTEPH) shows long-term survival rates are on par with international outcomes, but greater access to and awareness of treatment options are needed to optimise care.

A review of the Pulmonary Hypertension Society of Australia and New Zealand (PHSANZ) registry explored outcomes for 386 patients diagnosed with CTEPH between 2004 and 2020. It found patients undergoing pulmonary endarterectomy (PEA) had better long-term survival than non-PEA patients (one-year survival: 93% vs 86%, three years: 87% vs 73%, respectively) and that Australian and NZ patients had similar outcomes to those in international registries (one-year survival: 93% vs 86%, three years: 87% vs 73%, respectively).

However, despite PEA’s superior survival rate (P < 0.001), only a third of patients had the surgery. Those remaining received pulmonary hypertension- (PH) specific therapies and/or balloon pulmonary angioplasty (BPA), lead author, St Vincent’s Hospital cardiologist Dr Katherine Kearney and her team wrote in Respirology.

“The rate of patients being operated on in [Australia and NZ] is 37.8%, which is significantly less than overseas reports [60%–63%],” Dr Kearney said.

Even with specialist centres in Brisbane, Sydney, Perth, Melbourne and New Zealand, “the rate of operation has not increased over time”, she and her colleagues reported.

While age, baseline six-minute walking distance (6MWD), surgical experience, comorbidities, disease severity and patient preference can all affect patient operability, the authors believe accessibility issues could have impeded PEA treatment.

PEA remains the “treatment of choice” for technically operable CTEPH patients, but it’s “likely that some patients had limited access or were simply not referred to a PEA centre for multidisciplinary assessment of operability”, the authors wrote.

Think about CTEPH

Dr Kearney told the limbic that cardiologists should “start thinking about CTEPH whenever people have a pulmonary embolism and have ongoing breathlessness afterwards [and] consider it as a cause of newly-diagnosed pulmonary hypertension”.

Patients should be referred to experienced PEA centres earlier in their treatment course while they are younger, not too frail and likely to be better operative candidates, she added.

Dr Kearney said she would also like to see more multidisciplinary PEA expertise and centres develop across Australia and New Zealand.

“Currently, there’s only a couple of centres that offer [PEA]. And, for example, there’s not currently a CTEPH surgeon in New South Wales and this, I think, is a priority to be rectified.”

Non-PEA patients shared in the treatment accessibility problem, with 12% failing to receive initial PH-specific therapy.

“. . . this relatively high proportion of untreated patients relates to the fact that the only PBS-funded agent riociguat was only approved for inoperable CTEPH from 2014”, the authors wrote.

Clinicians apparently tried to fill the gap with off-label use of non-PBS-subsidised agents, with 24% using combination PH-specific therapy, they noted.

Further, BPA, a now “established, minimally invasive procedure” for inoperable or persistent CTEPH, was used in just 6.2% of patients.

The procedure can help “improve haemodynamics, symptoms, exercise capacity and right ventricular function” and its expansion, along with better pharmacotherapy access, could lead to further improvements in inoperable CTEPH survival.

With further research, Dr Kearney hopes to provide some hard evidence for how clinicians can better manage CTEPH and improve access to potentially curative surgeries and specialised treatments.

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