Table 1 Management of the consequences of PAH-CHD
From: General management of pulmonary arterial hypertension associated with adult congenital heart disease
| Problems | Causes | Solutions |
|---|---|---|
| Chronic hypoxaemia | Right to left shunting through cardiac defects |
Consider supplemental oxygen only if results in an increase in arterial oxygen saturation and improvement in symptoms Screen for sleep apnoea and nocturnal hypoventilation |
| Hyperviscosity syndrome | Erythrocytosis due to chronic hypoxaemia |
Maintain adequate hydration Use therapeutic phlebotomy only in presence of moderate/severe symptoms |
| Haemoptysis | Bronchial collateral vessels | Consider bronchial artery embolization for large volume bleeds |
| Pulmonary artery thrombosis/stroke | Sluggish pulmonary artery flow | Avoid iron deficiency and dehydration |
| Gout/cholelithiasis | Hyperuricaemia due to erythrocytosis |
Maintain adequate hydration Treat acute gout with colchicine, avoid non-steroidal anti-inflammatory drugs Allopurinol to maintain serum urate levels < 300 μmol/L |
| Chest pain | Right ventricular ischaemia, coronary artery compression, gastro-oesophageal reflux | Investigate cause and treat as appropriate |
| Arrythmias | Related to heart defect +/− intervention | Cardioversion, ablation, antiarrhythmic medication |
| Hypothyroidism | Increased incidence with chronic cyanosis |
Annual thyroid function screening and treat as required Care with iodine-based contrast media and amiodarone |
| Renal dysfunction | Erythrocytosis and hypoxaemia |
Maintain adequate hydration Care with nephrotoxic drugs and contrast agents |