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 detection of disease and prompt referral of high-risk patients so that care can begin as soon as possible.
“In recent years, substantial progress has been made in the detection and management of PH, and new evidence has been incorporated in time into this fourth edition of the ESC/ERS guidelines” for pulmonary hypertension, said stated the authors in their introduction.
The paper, “ESC/ERS 2022 guidelines for the diagnosis and treatment of pulmonary hypertensionwas published in the European journal of the heart.
PH is characterized by an increase in pressure in the blood vessels of the lungs, which puts an atypical pressure on the heart. Common symptoms of the disease include shortness of breath, fatigue, and reduced ability to exercise, and since symptoms are usually nonspecific, diagnostic delays are common.
PH guidelines note complexity of disease, progress made
“The diagnosis of pulmonary hypertension, and therefore the initiation of appropriate management, is often significantly delayed,” said Stephan Rosenkranz, MD, co-author of the guidelines from Cologne University Hospital in Germany, in a press release from the European Society of Cardiology. .
“In young patients, often women, delays are often due to disbelief of serious illness in an otherwise healthy young individual. In older patients, coexisting conditions (such as hypertension, obesity, or cardiopulmonary disease) may be responsible for the symptoms, and pulmonary hypertension is ignored,” Rosenkranz said.
The 2022 guidelines define PH as mean pulmonary arterial pressure (PAPm), a measure of blood pressure in the lungs, greater than 20 mmHg at rest.
In order to streamline the diagnosis of PH, the guidelines describe a general three-step approach: patients who present with PH-like symptoms on an initial clinical evaluation should undergo echocardiography to look for problems on the right side of the heart , a telltale sign of PH. . If both symptoms and echocardiography indicate PH, the patient should be promptly referred to a specialist center for confirmatory testing.
“Patients with shortness of breath should seek medical attention, and if the GP, cardiologist or pulmonologist cannot identify an appropriate reason, referral to a center with expertise in pulmonary hypertension is indicated,” Rosenkranz said.
The guidelines provide more specific information on the diagnosis and treatment of all types of PH. But they particularly focus on 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 past two decades, care for PAH has improved dramatically, and early initiation of treatment to prevent disease progression has become an essential goal of care. There are currently three classes of drugs approved to treat PAH, and it is recommended that most patients receive at least two treatments.
Patients should also be immunized 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.
Special attention paid to pregnancy with PH
The guidelines also note that pregnancy can significantly increase the risk of complications and death in people with PAH and other types of PH, especially when the disease is not well controlled. They emphasize that providers must clearly communicate these risks so that patients can make informed decisions, and that multidisciplinary care must be provided to patients with PH who choose to become pregnant.
“Pregnancy in PH is a very sensitive subject and requires empathic communication. Psychological support should be offered whenever needed,” the guidelines state.
For the CETPH, the guidelines note three treatment options that can be used alone or in combination: surgery to remove the clots causing the disease; balloon pulmonary angioplasty, which uses a temporarily inflated balloon in the artery to dislodge clots; and medications.
Generally, the guidelines emphasize the importance of multidisciplinary care for people with PH, with specialists from different disciplines working with patients to provide the best possible care.
“The complexity of PH management requires a multi-faceted, holistic and multidisciplinary approach, with active involvement of PH patients in partnership with clinicians,” the guidelines state.
The researchers emphasize that while these guidelines are intended to guide and optimize care for people with PH, they “do not replace the patient’s relationship with their practitioner.”
“Final decisions regarding an individual patient should be made by the responsible healthcare professional(s), based on what they consider to be most appropriate in the circumstances. These decisions are made in consultation with the patient and caregiver, as appropriate,” the guidelines state.