Pulmonary hypertension in congenital heart disease in the UK

  • Research type

    Research Study

  • Full title

    Pulmonary hypertension associated with congenital heart disease: A multicentre UK study



  • Contact name

    Konstantinos Dimopoulos

  • Contact email


  • Sponsor organisation

    Royal Brompton & Harefield NHS Foundation Trust

  • Duration of Study in the UK

    2 years, 2 months, 19 days

  • Research summary

    This is a UK, multicentre cross-sectional study of paediatric and adult patients of all ages with pulmonary arterial hypertension (PAH) and congenital heart disease (CHD). All patients with PAH and/or CHD in the NHS Trusts involved will be eligible. Patients will not require to undergo any additional investigation or treatment as part of this retrospective study.

    The aim of this study is to identify patients with PAH associated with CHD (PAH-CHD) and to investigate the following questions:
    1. How have the demographics of PAH and PAH-CHD changed over time, and has there been an increase in the prevalence of PAH-CHD following CHD repair (group D PAH-CHD);
    2. There is evidence of loss to follow-up and late diagnosis in patients presenting with group D PAH-CHD. At the time of diagnosis, most patients with group D PAH-CHD have intermediate or high risk features in keeping with a late PAH diagnosis;
    3. A simplified, non-invasive algorithm based on international guidelines can be validated for group D PAH-CHD, at presentation and follow-up;
    4. Failure of PAH monotherapy (defined as an addition of a second therapy) is less likely to occur in group D PAH-CHD patients. More group D patients do not appear to reach standard treatment targets than patients with other forms of PAH.
    The study will be retrospective, based on existing hospital databases and review of individual patient records.

    This work will have an impact on future clinical practice by facilitating early diagnosis, risk assessment and effective initiation and escalation of PAH therapies. As a result of improved risk stratification and treatment algorithms in this group of patients, clinicians can target patients who are at greater risk of PAH, and CHD services can ensure pro-active and frequent follow-up in the high-risk groups, allowing better healthcare resource planning.

  • REC name

    South Central - Hampshire A Research Ethics Committee

  • REC reference


  • Date of REC Opinion

    3 Apr 2020

  • REC opinion

    Further Information Favourable Opinion