New ESC/ERS Pulmonary Hypertension Guidelines Urge Precise Diagnosis, Early Treatment

BARCELONA, Spain—New guidelines for the diagnosis and treatment of pulmonary hypertension (PH), released at the recent European Society of Cardiology (ESC) Congress 2022, include a new hemodynamic definition of PH—incorporating a revised cutoff for pulmonary vascular resistance—and a tweaked diagnostic algorithm emphasizing expedited care and nuanced risk stratification.

Developed jointly by the ESC and the European Respiratory Society (ERS), the document is the fourth such guideline to be released by the ESC/ERS and the first update since 2015. This iteration has been endorsed by the International Society for Heart and Lung Transplantation and the European Reference Network on rare respiratory diseases.

According to the multidisciplinary writing group, co-chaired by Marion Delcroix, MD, PhD (Centre of Pulmonary Vascular Diseases, University Hospitals of Leuven, Belgium), on behalf of the ERS, and Stephan Rosenkranz, MD (Heart Center at the University Hospital of Cologne, Germany), on behalf of the ESC, a key change was adopting the new definitions of PH that had been reviewed and put forward at the 6th World Symposium on Pulmonary Hypertension (WSPH). The 2022 guidelines “endorsed and expanded” on those recommendations, “including a revised cutoff level for pulmonary vascular resistance (PVR) and a definition of exercise PH,” the document notes.

Speaking with TCTMD, Delcroix noted that, indeed, the biggest change in the new guidance was lowering the cutoff for diagnosis from 25 to 20 mm Hg. “We looked more in depth at the data and now see that normal values are clearly under 20 mm Hg but also that when you are above 20 mm Hg, regardless of the cause of your pulmonary hypertension, this has a negative prognosis,” she said.

Delcroix also emphasized the more “collaborative” and interdisciplinary pathway for the diagnosis and escalation of treatment in patients who first present to their general practitioners with shortness of breath. From here, she said, the guidelines recommend the “very important interaction between the cardiologist and the respiratory physician, who do their respective tests and then come together to note that yes, there is really pulmonary hypertension and something needs to be done.”

The third step is referral to a pulmonary hypertension center where the diagnosis can be refined and treatment initiated. “This approach is much more collaborative and much more what happens in real life,” Delcroix said. Previously, “we had an algorithm but it was very theoretical and now it really specifies at which level of care these steps have to happen.”

This latest update to the guidelines retains the five main subcategories of PH set forth in earlier iterations:

1. Pulmonary arterial hypertension as a distinct entity
2. PH stemming from left heart disease
3. PH linked with chronic lung disease
4. Chronic thromboembolism pulmonary hypertension (CTEPH)
5. PH of unclear or multifactorial causes

What cardiologists need to keep in mind, said Delcroix, is that while left heart failure is the leading cause of PH (linked to approximately 70% of cases), that doesn’t mean that they shouldn’t be on the lookout for category-1 cases, which have very specific, unique, and effective treatments distinct from those that have proved helpful for PH of other etiologies.

These guidelines will be especially helpful for cardiologists—and pulmonologists—to be able to distinguish the category-1 PH patients, who are very rare, from the much more common PH patients whose disease stems from the heart or the lungs.

Other Key Updates

All the additions and changes to the new guidelines are summarized in a comprehensive table spanning nine pages of the 144-page document. Some key new recommendations graded as Class I in the guidelines:

• An emphasis on right heart catheterization with a “complete set” of hemodynamics, following a standardized protocol
• New considerations for evaluating severity and risk of death, which is very important, said Delcroix, because recent registries have made it clear that high-risk patients face a 20% risk of death per year, not 10% as earlier guidelines specified
• Multiple dos and don’ts for diagnosing PH, including the recommendation that an “echocardiographic probability” be assigned based on an abnormal tricuspid regurgitation velocity, according to updated definitions
• New recommendations for women of childbearing age; for pediatric patients; and for patients with systemic sclerosis, connective tissue disorders, HIV, and adult congenital heart disease
• Advice for triaging patients for lung transplantation
• New advice around PH associated with drugs and toxins
• Recommendations for the use of different imaging techniques, including CT, cardiac MRI, and echocardiography, to refine diagnosis, track disease progression, and monitor right heart structure and function

An important emphasis of the 2022 guidelines is on aggressive monitoring of patients, Delcroix stressed. “We encourage the doctors to follow up the patients very rapidly after stratification of treatment,” she said. In the past, the approach was to wait for patients to deteriorate before intensifying treatment. “Now we very proactively ask that the patient is followed up in 3 to 6 months, [and that] a new risk stratification has to be done.”

The ERS hosts its own international congress later this week in Barcelona—these new guidelines will be getting their second debut at that meeting, Delcroix noted.