Using Step Count to Enhance Daily Physical Activity in Pulmonary Hypertension

This study will collect information about physical activity in patients affected by pulmonary hypertension, through a specific device that can be worn on the wrist, and which measures daily step count. The purpose of the study is to demonstrate whether providing patients with their daily step count can increase their levels of daily activity if they have some basic targets to achieve. Investigators also wish to compare the effects of this approach in increasing daily physical activity, with the benefit gained when a drug therapy for pulmonary hypertension is initiated, in patients that have just been diagnosed.

Pulmonary hypertension (PH) is a rare and progressive disorder and shortness of breath and fatigue, especially during exercise, are very common symptoms, resulting in a reduced capacity to perform daily activities and in an impaired quality of life. Furthermore, reduced exercise capacity in PH is associated with depression and anxiety disorders. Recent literature reveals a strong association of sedentary time with adverse cardiovascular outcomes, so that the development of therapeutic strategies to avoid this event is pivotal in the management of PH, in addition to the beneficial results of modern medical treatment. Recently the role of exercise and rehabilitation in different respiratory and cardiac conditions has been evaluated in the literature, overtaking the concerns raised in the past that exercise could worsen the condition and thus the progression of these kind of diseases, but just a few studies have been done in patients affected by PH.

International guidelines on pulmonary hypertension recommend avoiding strenuous exercise that leads to distressing symptoms, while encourage low level graded aerobic exercise, such as walking, as tolerated. Nonetheless, little is still known about the effects of lifestyle changes, such as the amount of physical activity that patients can or should practice. Pedometers have been used to enhance the daily physical activity and to improve, when assessed, some prognosis related outcomes, both in healthy subjects and those affected by different pathological conditions, in particular cardiac and respiratory diseases. However, a training model based on step count targets has never been evaluated in PH, to our knowledge. Step counting devices have recently experienced a surge in popularity as a tool for motivating and monitoring physical activity in the general population, especially combined with applications for smartphones, that provide feedback, such as an overview of the data recorded. Thus, it seems reasonable to explore the opportunity to develop a step count based training model to enhance daily exercise in PH patients, through these non-invasive, patient friendly and relatively inexpensive devices.

Eligibility Criteria:
Inclusion Criteria:

* Able to give informed consent
* Nice Classification Groups 1 and 4 Pulmonary Hypertension (pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension)
* WHO functional class I-IV
* Stable on current medication for 8/52 for group 1 (feedback and goal-setting group) and group 2 (control group)
* For group 3 (newly diagnosed patients), treatment-naïve patients undergoing investigation who are likely to start therapy (based on clinical judgment of the clinician)
* Patients with handheld devices (such as smartphones or tablets) which can run the Study Admin Mobile application.

Exclusion Criteria:

* Pregnancy: we will screen for this on the basis of the history from the patient, and if she is not sure if she might be pregnant, a pregnancy test will be offered
* Malignant arrhythmias
* Syncope within the last 6 months
* Skeletal or muscle abnormalities prohibiting walking