Meet An and Sussie. They both live with pulmonary arterial hypertension (PAH), a rare form of pulmonary hypertension (PH), and share their personal stories in this video.
In this video we hear about their unique paths to their PAH diagnoses, and their advice for newly diagnosed patients, such as finding support from a local patient organisation and talking with other patients about living with PAH.
To hear more personal stories from people living with PAH, visit our PH Human website:
www.janssenwithme.com/en/pulm...
What is PH?
Pulmonary hypertension (PH) is high pressure in the blood vessels of the lungs, as the right side of the heart has to work harder to pump blood through the lungs.1,2 It is a serious, progressive disease and can lead to heart failure and early death.3,4
Pulmonary arterial hypertension (PAH) is a progressive, rare type of PH that affects the blood vessels that carry blood from the heart to the lungs.2,5 These are known as pulmonary arteries.2,5
Over time, the pulmonary arteries become increasingly narrow, as the walls of the blood vessels become thicker and less flexible.2,5
This restricts blood flow to the lungs, which makes it harder for the heart to pump blood through the arteries.6 Trying to maintain the blood flow, the heart has to pump harder, increasing the pressure in the pulmonary arteries.6
Due to the rarity of PAH and the non-specific nature of its symptoms, PAH can be confused with more common conditions such as chronic obstructive pulmonary disease (COPD).3,4
References
1. Galiè N, Humbert M, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2016;37:67-119.
2. Lai Y, Potoka KC, et al. Pulmonary arterial hypertension: The clinical syndrome. Circ Res 2014;115:115-30.
3. Vachiéry JL and Gaine S. Challenges in the diagnosis and treatment of pulmonary arterial hypertension. Eur Respir Rev 2012;21:313-20.
4. Hoeper MM and Gibbs SR. The changing landscape of pulmonary arterial hypertension and implications for patient care. Eur Respir Rev 2014;23:450-7.
5. Schermuly RT, Ghofrani HA, et al. Mechanisms of disease: Pulmonary arterial hypertension. Nat Rev Cardiol 2011;8:443-55.
6. National Organisation of Rare Diseases (2019). Rare Disease Database: Pulmonary Arterial Hypertension. Available at: rarediseases.org/rare-disease...