Overcoming Challenges in Pulmonary Hypertension
June 3rd, 2025
Pulmonary hypertension (PH) is a rare, progressive, debilitating condition characterised by elevated pulmonary artery pressure [1]. This elevated pressure over time causes the walls of the pulmonary artery to become thick and stiffened, and can lead to symptoms including shortness of breath, chest pains, dizziness, and weakness. Eventually, PH can also lead to serious complications, including heart failure, blood clots and cardiac arrhythmias.
Although pharmacological drugs and lifestyle changes can help manage PH symptoms and slow disease progression, symptoms can still significantly impact the individual’s physical mobility and emotional well-being, and adversely affect their quality of life [2]. PH can also have a significant effect on an individual's social life and their relationships with others, and can lead to feelings of isolation and depression [2].
Without doubt, for those living with PH, the disease can have a devastating effect and adverse impact on all aspects of their life. This blog will discuss some key challenges PH patients face in managing their condition and tips on how to overcome these challenges.
Coping With the Emotional Toll
PH can have a significant impact on emotional well-being, as patients are confronted each day with the physical limitations imposed by the condition. Additionally, patients must also cope with the daily uncertainties and changes that occur as the disease progresses. A recent survey found that 92% of PH sufferers reported the condition impacted their emotional well-being, and a further 53% reported experiencing or being diagnosed with anxiety or depression [3].
To help cope with the condition, PH patients must learn to adapt to their new circumstances and to prepare for future health challenges. This means that patients adjust their expectations and focus on more manageable and realistic goals [4]. It is also advisable that PH patients create a list of potential future health challenges as well as a plan of action for managing each of these challenges if they do occur [4].
It can also help PH patients to connect with others who are living with the condition through online support groups and communities, where they can exchange information and experiences [4]. Additionally, PH patients could consider using smartphone-based apps that offer mindfulness exercises, guided meditation, or other stress management techniques.
Adapting to Lifestyle Changes
The effective management of PH often requires lifestyle changes, including changes in diet, physical activity, and habits. When carrying out their daily routine, PH patients must also ensure that they pace themselves and get adequate rest [5]. However, PH patients may find making and maintaining lifestyle changes a challenge as it may mean changing deeply ingrained routines and habits. They may also no longer be able to participate in activities they previously enjoyed, including hobbies and social events.
It is important that PH patients work with their healthcare team to create a plan to implement these changes that is both achievable and aligns with their lifestyle and personal preferences. It can also help to break changes into smaller, more achievable goals, as this can help them seem less overwhelming. Additionally, celebrating any achievements can help PH patients to stay focused and motivated.
Accessing online educational materials can help empower PH patients to better understand their condition and how to implement lifestyle changes. The use of wearable devices and apps can enable tracking of diet and physical activity levels and can provide feedback and positive reinforcement that can help PH patients to maintain these changes.
Effective Long-Term Management
The effective long-term management of PH involves close adherence to prescribed medications and regular monitoring and follow-ups. Medication adherence varies considerably among PH patients, with studies reporting rates of 50-80% [6–9]. Poor medication adherence in PH has been linked to increased hospitalisations and a poorer prognosis [10,11]. Regular monitoring and follow-ups are also important for assessing treatment responses and enabling treatment adjustments. However, the burden imposed by PH symptoms means it can be a challenge for PH patients to travel to and attend clinic appointments.
To improve adherence, PH patients should make taking their medications a key part of their daily routine, and to organise their medications using pillboxes. It may also be helpful for patients to discuss with their healthcare provider any problems they are having relating to attending follow-up appointments and ensure they reschedule any missed appointments.
Those technologies that can aid in the long-term management of PH include the use of medication reminder apps, which can help alert patients when they need to take their medications. Additionally, remote patient monitoring and telehealth platforms can enable improved communication between patients and their care team and provide real-time monitoring of patients outside the clinic setting.
Undoubtedly, PH is a challenging condition, and there may be moments when patients feel emotionally overwhelmed, struggle to maintain lifestyle changes and to effectively manage the condition long term. An improved awareness of these challenges will undoubtedly lead to the increased implementation of technology within PH patient care.
References:
[1] Hoeper MM, Humbert M, Souza R, et al. A global view of pulmonary hypertension. Lancet Respir Med [Internet]. 2016;4(4):306–322.
[2] Delcroix M, Howard L. Pulmonary arterial hypertension: the burden of disease and impact on quality of life. European Respiratory Review. 2015;24(138):621–629.
[3] PHA UK. Beneath the surface: The true emotional impact of pulmonary hypertension. 2019.
[4] Pulmonary Hypertension Association. Coping as a long-term survivor [Internet]. [cited 2025 Apr 28]. Available from: https://phassociation.org/patients/living-with-ph/coping-with-ph/coping-as-a-long-term-survivor/.
[5] Pulmonary Hypertension Association. Day-to-day Living [Internet]. [cited 2025 Apr 29]. Available from: https://phassociation.org/patients/living-with-ph/day-to-day-living-for-ph-patients/.
[6] Kjellström B, Sandqvist A, Hjalmarsson C, et al. Adherence to disease-specific drug treatment among patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. ERJ Open Res. 2020;6(4):00299–02020.
[7] Reimann L, Preiss H, Müller J, et al. Medication adherence and clinical outcome in patients with pulmonary arterial hypertension or distal chronic thromboembolic pulmonary hypertension. BMJ Open Respir Res. 2025;12(1):e003023.
[8] Grady D, Weiss M, Hernandez‐Sanchez J, et al. Medication and patient factors associated with adherence to pulmonary hypertension targeted therapies. Pulm Circ. 2018;8(1):1–9.
[9] Blyth R, Zarifis I, Moriarty E, et al. Medication adherence in patients with pulmonary hypertension. Pulmonary hypertension. European Respiratory Society; 2024. p. PA3432.
[10] Frantz RP, Hill JW, Lickert CA, et al. Medication adherence, hospitalization, and healthcare resource utilization and costs in patients with pulmonary arterial hypertension treated with endothelin receptor antagonists or phosphodiesterase type‐5 inhibitors. Pulm Circ. 2020;10(1):1–11.
[11] Le Bozec A, Korb-Savoldelli V, Boiteau C, et al. Medication adherence, related factors and outcomes among patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension: a systematic review. European Respiratory Review. 2024;33(173):240006.