Diagnostic tools in pulmonary arterial hypertension associated with congenital heart disease
| Stage of diagnosis | Test | Use and typical findings |
| Detection | ECG | Abnormalities related to the underlying cardiac defect; useful in assessing underlying heart rhythm; may indicate atrial dilatation or right ventricular strain |
| Chest radiography | Dilatation, aneurysm, or calcification of the central pulmonary arteries may be present; highly variable (may appear normal) | |
| Echocardiography | Delineation of underlying cardiac anatomy, defects, levels, and characteristics of shunting, and of ventricular function; estimation of subpulmonary ventricular pressure | |
| Characterisation | Cardiac MRI | Determination of intracardiac anatomy and confirming the location and size of defect; information on right ventricular function and pulmonary vascular bed |
| HRCT | Useful in assessing pulmonary arterial thrombi and to exclude intrapulmonary haemorrhage or infarction; assessment of lung parenchyma | |
| PFT, ventilation-perfusion scintigraphy | May be useful to exclude additional causes of PAH, especially when there are discrepancies between defect and PAH severity | |
| Classification | Exercise test | Reflects disease severity and assists prognostic evaluation; in Eisenmenger syndrome it provides information on change in arterial oxygen saturations during exercise |
| RHC | Establishes the diagnosis of PAH and assesses the severity of pulmonary vascular disease |
MRI: magnetic resonance imaging; HRCT: high-resolution computed tomography; PFT: pulmonary function testing; PAH: pulmonary arterial hypertension; RHC: right heart catheterisation. Reproduced from [10] with permission from the publisher.