The investigation and diagnosis of pulmonary hypertension in adults with congenital heart disease

Pulmonary hypertension is not uncommon in adult patients with congenital heart disease and can significantly affect their exercise capacity, quality of life and prognosis. Timely identification and management of pulmonary hypertension in these patients is important. Patients must be allocated to the correct diagnostic group and treatment decided upon, including offering pulmonary arterial hypertension therapies to those who are most likely to benefit. In this paper, we provide an overview of the diagnostic modalities that are used to investigate and diagnose pulmonary hypertension in modern adult congenital heart disease practice. Emphasis is placed on echocardiography, which is the modality of choice for raising the suspicion of PH, and cardiac catheterisation, which is fundamental in establishing the diagnosis. Other modalities, such as cardiac magnetic resonance imaging and computed tomography can provide complementary information on anatomy and physiology. Combining all this information into a clinical diagnosis and management plan requires clinical expertise and a multidisciplinary approach, especially when managing this rare and complex group of patients.