National Pulmonary Hypertension Audit, 15th Annual Report

Patients with PH are treated at 1 of 8 specialist PH centres in the UK.

Notes:

1. With the exception of SGC, trust codes are derived from the Organisation Data Service: https://digital.nhs.uk/services/organisation-data-service. SGC is used in the NAPH only.

2. Great Ormond Street provides care for children with PH.

3. Royal Brompton and Harefield NHS Foundation Trust merged with Guy's and St Thomas' NHS Foundation Trust in 2021 and is now reported under Guy's and St Thomas' NHS Foundation Trust. However, as the PH services are still based in Royal Brompton Hospital the PH centre will be referred to as 'Royal Brompton' in this report.

4. Royal Papworth Hospital is the only surgical centre in the UK for pulmonary endarterectomy.

1          Pulmonary arterial hypertension (PAH)

1.1       Idiopathic PAH

1.2       Heritable

1.3       Drugs and toxins induced

1.3.1    BMPR2

1.3.2    ALK1, endoglin (with or without hereditary haemorrhagic telangiectasia)

1.3.3    Unknown

1.4       Associated with (APAH):

1.4.1    Connective tissue diseases

1.4.2    HIV infection

1.4.3    Portal hypertension

1.4.4    Congenital heart disease

1.4.5    Schistosomiasis

1.4.6    Chronic haemolytic anaemia

1.5       Persistent pulmonary hypertension of the newborn

1’ Pulmonary veno-occulusive disease and/or pulmonary capillary haemangiomatosis

2          Pulmonary hypertension due to left heart disease

2.1       Systolic dysfunction

2.2       Diastolic dysfunction

2.3       Valvular disease

3          Pulmonary hypertension due to lung diseases and/or hypoxia

3.1       Chronic obstructive pulmonary disease

3.2       Interstitial lung disease

3.3       Other pulmonary diseases with mixed restrictive and obstructive pattern

3.4       Sleep-disordered breathing

3.5       Alveolar hypoventilation disorders

3.6       Chronic exposure to high altitude

3.7       Developmental abnormalities

4          Chronic thromboembolic pulmonary hypertension

5          Pulmonary hypertension with unclear and/or multifactorial mechanisms

5.1       Haematological disorders: myeloproliferative disorders, splenectomy

5.2       Systemic disorders, sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, neurofibromatosis, vasculitis

5.3       Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders

5.4       Others: tumoural obstruction, fibrosing mediastinitis, chronic renal failure on dialysis

Treatment approved diagnoses

The codes used in the creation of the treatment approved diagnosis group are as follows:

1 Pulmonary arterial hypertension (PAH)

1’ Pulmonary veno-occulusive disease and/or pulmonary capillary haemangiomatosis

4 Chronic thromboembolic pulmonary hypertension

5.2.1 Systemic disorders - Sarcoidosis*

5.4.3 Other - Chronic renal failure on dialysis*

* indicates a code used only in the National Audit of Pulmonary Hypertension. The classification system used in this audit has additional codes to help with clarity and data quality.

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