Introduction Pulmonary hypertension (PH) is a rare, life-limiting condition which can aggravate most cardiovascular and pulmonary diseases. PH is debilitating associated with adverse physical, social and emotional aspects of patients’ life. Previous research highlighted the need to gain a greater understanding of patients’ experiences living with PH. The aims of this meta-ethnography were to gain knowledge and understanding of patients’ experiences living with PH to support clinical practice and future research.

Design A systematic review and an interpretative synthesis of eligible primary studies using a meta-ethnography approach.

Methods A systematic literature review was conducted using the following databases: CINAHL, BNI, AMED, MEDLINE, EMBASE, PsycINFO, Cuiden Plus, Scopus, The Cochrane Library, ASSIA, JBI, Campbell Library, and Web of Science. Supplementary search strategies included grey literature, reference list and forward citation searches on seminal studies, and consultation to relevant organisations or experts to maximise sensitivity of the strategy. Meta-ethnography was used to analyse, translate and resemble the primary qualitative research.

Results Of 3691 articles identified, 71 were reviewed in detail and 9 were included, using a total of 132 participants, and 549 online posts, in 8 different countries. Three major third-order constructs were derived from the synthesis: (a) ‘Loss of Self’, (b) ‘Self-Growth’, and (c) ‘Drivers’. Finally, a refined explanatory model of patients’ experiences living with PH was developed, showing a dynamic process between ‘Loss of Self’ and ‘Self-Growth’ depending on five fluctuating ‘Drivers’ (‘coping strategies’, ‘support structures’, ‘management and treatment’, ‘awareness’ and ‘uncertainty’).

Conclusions This meta-ethnography demonstrates a dynamic process of living with chronic and life-threatening condition, constituted by trigger factors which drive patients to positive or negative experiences. It is important to consider the roles of both PH-patient associations and multidisciplinary PH-’experts’ to improve patients’ personal growth, satisfaction and quality of life.