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

Abstract

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.

Pulmonary hypertension in the primary care setting

“…Other useful TTE parameters include evidence of left heart dysfunction including left atrial dilatation, which often point to significant left heart diseases or significant congenital heart diseases. 9 It should be noted that the accuracy of these results can be operator dependent and it can be difficult to get good echo windows in certain patient groups (eg COPD, obese), and therefore over-and underestimation of the pulmonary artery pressure can occur. The findings of an abnormal echocardiogram in primary care should prompt urgent referral to respiratory or cardiology services for further evaluation.…”

“…Therefore other surrogate markers of elevated pulmonary hypertension can be looked for, which include right ventricular and atrial dilatation. Other useful TTE parameters include evidence of left heart dysfunction including left atrial dilatation, which often point to significant left heart diseases or significant congenital heart diseases 9 …”

Quality of life in adults with congenital heart disease with and without pulmonary hypertension: a comparative study

“…The clinical classification of PH in CHD (PH-CHD) comprises the Eisenmenger Syndrome (ES), PH associated with a predominant systemic-to-pulmonary shunt, PH associated with a small defect, and PH associated with a repaired defect, as well as special forms such as pulmonary vascular disease in Fontan circulation ( 6 ). Only recent advances in research and treatment of PH have led to increasing survival rates ( 7 ). Although it is well known that patients with idiopathic forms of PH are at risk for a severely impaired quality of life (QOL) ( 8 ), reports on impaired QOL due to PH-CHD are still limited and not routinely employed in general care of this particular patient population.…”