Early Detection of PH Essential for Optimal Care, 2022 Guidelines Stress

ESC/ERS new guidelines for diagnosing, managing pulmonary hypertension

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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The European Society of Cardiology (ESC) and the European Respiratory Society (ERS) have published a new set of guidelines for the diagnosis and management of pulmonary hypertension (PH).

These guidelines simplify diagnostic criteria for PH, emphasizing the importance of early disease detection and the expedited referral of high-risk patients so that care is started as soon as possible.

“In recent years, substantial progress has been made in detecting and managing PH, and new evidence has been timeously integrated in this fourth edition of the ESC/ERS Guidelines” for pulmonary hypertension, the authors stated in their introduction.

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The paper, “2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension” was published in the European Heart Journal.

PH is characterized by increased pressure in the lungs’ blood vessels, which puts atypical strain on the heart. Common disease symptoms include shortness of breath, fatigue, and a diminished ability to exercise, and because the symptoms are generally nonspecific, diagnostic delays are frequent.

PH guidelines note disease’s complexity, advances realized

“The diagnosis of pulmonary hypertension, and thus initiation of appropriate management, is often substantially delayed,” Stephan Rosenkranz, MD, a co-author of the guidelines from University Hospital Cologne in Germany, said in a press release from the European Society of Cardiology.

“In young patients, frequently women, delays are often due to disbelief of severe illness in an otherwise healthy looking young individual. In older patients, co-existing conditions (such as hypertension, obesity, or cardiopulmonary diseases) may be blamed for the symptoms and pulmonary hypertension is missed,” Rosenkranz said.

The 2022 guidelines define PH as a mean pulmonary arterial pressure (mPAP), a measure of blood pressure in the lungs, that is higher than 20 mmHg at rest.

In order to streamline PH diagnosis, the guidelines outline a general three-step approach: patients who present with PH-like symptoms in an initial clinical evaluation should undergo echocardiography to look for problems on the right side of the heart, a telltale sign of PH. If symptoms and echocardiography both point to PH, the patient should be quickly referred to a specialty center for confirmatory testing.

“Patients with shortness of breath should seek medical help, and if the GP, cardiologist or pulmonologist cannot identify an appropriate reason, referral to an expert pulmonary hypertension centre is indicated,” Rosenkranz said.

The guidelines provide more specific information about diagnosis and treatment for all of the types of PH. But they especially emphasize pulmonary arterial hypertension (PAH), a distinct vascular disorder characterized by PH, and chronic thromboembolic pulmonary hypertension (CTEPH) when PH develops due to blood clots in the lungs.

Over the last decade or two, care for PAH has improved markedly, and early initiation of treatment to prevent disease progression has emerged as a critical goal for care. There are currently three classes of medication approved to treat PAH, and it’s recommended that most patients should be on at least two therapies.

Patients should also be vaccinated against infections that can cause lung problems, including influenza and COVID-19, and psychological support is recommended to help patients cope with the emotional and social impacts of PAH.

Particular attention given to pregnancy with PH

The guidelines also note that pregnancy can substantially increase the risk of complications and death in people with PAH and other PH types, especially when the disease is not well-controlled. They stress that providers should communicate these risks clearly so that patients can make well-informed decisions, and that multidisciplinary care should be given to PH patients who do choose to get pregnant.

“Pregnancy in PH is a very sensitive topic and requires empathic communication. Psychological support should be offered whenever needed,” the guidelines state.

For CETPH, the guidelines note three treatment options that can be used alone or in combination: surgery to remove the disease-driving clots; balloon pulmonary angioplasty, which uses a balloon temporarily inflated in the artery to dislodge clots; and medications.

Broadly, the guidelines stress the importance of multidisciplinary care for people with PH, with specialists across different disciplines working together with patients in order to provide the best possible care.

“The complexity of managing PH requires a multifaceted, holistic, and multidisciplinary approach, with active involvement of patients with PH in partnership with clinicians,” the guidelines state.

Researchers stress that while these guidelines are intended to help guide and optimize care for people with PH, they “are not a substitute for the patient’s relationship with their practitioner.”

“The final decisions concerning an individual patient must be made by the responsible health professional(s), based on what they consider to be the most appropriate in the circumstances. These decisions are made in consultation with the patient and caregiver as appropriate,” the guidelines state.