Table 7

Overview of routine laboratory blood tests in paediatric pulmonary hypertension

Diagnostic testAimComment
Full blood count*Anaemia, polycytaemia 
BNP/NT-proBNP*Detect ventricular dysfunction/heart failureMay be useful for longitudinal follow-up/response to treatment
Iron studies (free iron, transferrin, ferritin and iron-binding capacity)*Iron deficiencySupplementation
Nutritional counselling
Regular testing in patients with PH-CHD and cyanosis
Vitamin B12, folic acid*Vitamin B12 or folic acid deficiencySupplementation, malabsorption
Nutritional counselling
Urea and electrolytes including uric acid*  
Liver functional enzymes (GOT, GPT, GGT, LDH)*Exclude liver dysfunctionEvery 4–12 weeks when stable on ERA therapy
Clotting* (PTT, INR, prothrombin, D-dimers)  
Hepatitis serology†  
Thyroid function test†  
CK, CK-MB; troponin†  
HIV†Causative factor for PHELISA
ANAs (anticentromer antibodies, anti-SM, dsDNA, anti-Ro U1-U2 RNP, B23, Th/To)†? connective tissue disease, systemic sclerosis 
Anticardiolipin antibodies†
Lupus anticoagulant†
Lupus erythematodes, peripheral pulmonary embolic disease 
Thrombophilia screening, including anti-phospholipid antibodiesSuspicion of CTEPH
Antiphospholipid syndrome
Immunoassay/PCRSchistosomiasisConsider if appropriate medical history
Hb electrophoresisRule out haemolobinopathy, eg, sickle cell disease/ thalaessaemiaConsider if ethnical risk group
  • *Recommended at presentation in all patients. †May be useful for selected patients, unmarked decision on an individual patient basis.

  • BNP, brain-natriuretic peptide; CHD, congenital heart disease; CTEPH, chronic thromboembolic pulmonary hypertension; ERA, endothelin receptor antagonists; PH, pulmonary hypertension.