Transthoracic echocardiography for the evaluation of children and adolescents with suspected or confirmed pulmonary hypertension. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and D6PK

Abstract

Transthoracic echocardiography (TTE) is a useful method for non-invasive screening of patients at risk of pulmonary hypertension (PH). Since TTE often serves as the initial study before invasive cardiac catheterisation, misinterpretation of TTE variables may lead to missed or delayed diagnosis with devastating consequences for the patients, or unnecessary invasive diagnostics that have inheriting risks. Due to the heterogeneous anatomy in congenital heart disease, particularly the assessment of myocardial function in children with PH is challenging. Here, we present recommendations on the use of TTE in the screening, diagnosis and follow-up of patients with PH, and discuss the limitations of this non-invasive imaging technique. This expert consensus statement focuses on key TTE variables used to determine the pressure in the pulmonary artery, myocardial contractility and systolic and diastolic function of the RV and LV. A particular focus is on the TTE assessment of RV function and geometry. According to the published data on the application of TTE in PH in childhood, we suggest a structured approach for non-invasive assessment of pulmonary artery pressure and myocardial function that may help to identify patients with early ventricular deterioration and their response to advanced pharmacotherapy. In addition to clinical and biochemical markers, serial examination of patients with PH using a standardised TTE approach, determining conventional and several more novel echocardiographic variables may allow early diagnosis and treatment, better recognition of disease progression and guide tailored therapy.