Paediatric pulmonary hypertension: aetiology, pathophysiology and treatment

Paediatric pulmonary hypertension (PH) is associated with great morbidity and mortality. Various classifications have been formulated according to the aetiology or pathophysiology. The aetiology of pulmonary hypertension is very diverse and trying to pinpoint a cause can help us in optimizing the treatment as much as possible. Nonetheless, in a large number of children, the aetiology remains unclear. There is a large spectrum of the disease presentation and the diagnosis is still mainly based on echocardiographic and cardiac catheter findings with many supportive investigations. Once a diagnosis of PH has been established a thorough diagnostic workup is helpful as the treatment options depend critically upon the underlying cause which may be treatable. This article describes the known causes in children, the important diagnostic tests required and the currently available treatment options.