Bringing Pulmonary Hypertension to the Forefront

Diagnosis Impacts and Challenges

Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are rare, progressive and severe diseases that involve permanent changes to blood flow patterns and increased blood pressure in the blood vessels of your lungs.^6-8 Patients with either of these types of pulmonary hypertension (PH) face a poor prognosis. Data from U.S. registries showed that PAH has a 3-year survival rate of 72% at the time of diagnosis while CTEPH has a 3-year survival rate of 87%.^9,10 Early identification and treatment can improve clinical outcomes,^4,11 yet the majority of patients with PAH or CTEPH are in the advanced stages of the disease when they are finally diagnosed.¹²

PAH and CTEPH ultimately lead to right-sided heart failure, as the pulmonary arteries constrict, and blood becomes harder to move through the lungs.^5,7 Treatments for PAH focus on improving some symptoms and slowing progression.^5,11 CTEPH may be treated with an operation called pulmonary endarterectomy (PEA). During this highly specialized operation, clots and other blockages of the pulmonary arteries are removed. CTEPH can potentially be cured after the surgical procedure.^7,13 
 

Pulmonary Arterial Hypertension

PAH causes gradual, progressive stiffening and narrowing of the pulmonary arteries, raising the pulmonary arterial pressure and eventually leading to heart failure.^5,6 Some forms of PAH don’t have a known cause, adding another layer of complexity to a timely, accurate diagnosis.^6,17

While there is no cure for PAH, with a diagnosis in hand, a variety of treatment options are available, including lifestyle modifications (such as physical activity), supportive therapies (oxygen, diuretics, anticoagulants) and specific PAH drug therapy.^12,14 In severe cases, a lung transplant may be considered.¹² 

Chronic Thromboembolic Pulmonary Hypertension 

CTEPH may be caused by blood clots obstructing blood flow in the lungs, which increase resistance to blood flow.⁶ CTEPH may occur after a pulmonary embolism (PE), which is a blood clot blockage in one of the arteries in the lungs.^13,18 Depending on the location and number of blood clots in the lungs, some cases of CTEPH can be cured with a procedure called pulmonary endarterectomy (PEA).¹³ When surgical intervention is not an option, other therapies may be.¹² Without treatment, the blood vessels adapt to the embolic obstructions, leading to irreversible changes that further and permanently worsen blood flow.^7,8,16

Enhancing Care for PAH and CTEPH Patients

Early identification, diagnosis and treatment are critical for slowing disease progression and improving a patient’s daily life.^5,7,19-21 Despite available PAH-specific therapies and interventions for CTEPH, diagnosis of PH remains at >2 years from symptom onset, therefore, it's crucial to improve screening and early detection.¹² 

Bayer is committed to raising awareness of PAH and CTEPH and offers accompanying services and resources to support those currently living with and managing PAH and CTEPH. Through a unique support program and commitment to research and innovation, Bayer is taking actionable steps to support and treat those who are and will be living with these rare diseases.