Clinical Suspicion of Pulmonary Arterial Hypertension

1. Clinical Suspicion of Pulmonary Arterial Hypertension Echocardiogram Right Ventricular Systolic Pressure (RVSP) ≤35 Investigate for other causes and/or review as appropriate Right Ventricular Systolic Pressure (RVSP) > 35 Investigate for Pulmonary and connective tissue disease. Consider PFT’s HRCT Chest, V/Q scan or CTPA, Sleep study, autoantibody screen (ANA, ENA profile) and anti-topoisomerase. (See note 1) No Pulmonary or Connective Tissue Disease Refer To Pulmonary Arterial Hypertension Services for further investigation and decision on management including right heart catheterisation. Dr C Wilson, Belfast HSCT Linked to Papworth Congenital Heart Disease Refer To Pulmonary Arterial Hypertension Services for further investigation and decision on management including right heart catheterisation. Dr C Wilson, Dr A Sands, Dr M Spence, Belfast HSCT Linked to Great Ormond Street Connective Tissue Disease identified Refer to CTD / PAH Service at Ulster Hosp for further investigation and decision on management including right heart catheterisation. Dr Anita Smyth, Dr S McMechan SET. Dr Marshall Riley, Belfast HSCT and Dr G Coghlan (Visiting Cardiologist, Royal Free Hospital, London) Underlying lung disease identified Treat Lung Disease Note 1 HRCT Chest, V/Q Scan and CTPA may be carried out locally, although not considered essential to referral centre.. 08 Sept 08