Abstract
While invasive assessment of hemodynamics and testing of acute vasoreactivity in the catheterization laboratory is the gold standard for diagnosing pulmonary hypertension (PH) and pulmonary vascular disease (PVD) in children, transthoracic echocardiography (TTE) serves as the initial diagnostic tool. International guidelines suggest several key echocardiographic variables and indices for the screening studies when PH is suspected. However, due to the complex anatomy and special physiological considerations, these may not apply to patients with congenital heart disease (CHD). Misinterpretation of TTE variables can lead to delayed diagnosis and therapy, with fatal consequences, or–on the other hand-unnecessary invasive diagnostic procedures that have relevant risks, especially in the pediatric age group. We herein provide an overview of the echocardiographic workup of children and adolescents with PH with a special focus on children with CHD, such as ventricular/atrial septal defects, tetralogy of Fallot or univentricular physiology. In addition, we address the use of echocardiography as a tool to assess eligibility for exercise and sports, a major determinant of quality of life and outcome in patients with PH associated with CHD.
Highlights
Division of Pediatric Cardiology, Department of Pediatrics, Medical University Graz, 8036 Graz, Austria; European Pediatric Pulmonary Vascular Disease Network, 13125 Berlin, Germany; Department of Pediatric Cardiology, Charité-Universitätsmedizin Berlin, 13353 Berlin, Germany
Ventricular tissue Doppler imaging (TDI) with the pulsed wave (PW) curser placed at the lateral tricuspid annulus in an 8-year-old patient with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD)
While exercise testing such as 6 min walk test is predictive of outcome in children with pulmonary hypertension (PH), its results do not correlate to echocardiographic assessment parameters at rest, hampering the use of resting echocardiography in estimating cardiac function during activity and exercise [167]
Summary
The estimation of sPAP is based on the peak tricuspid regurgitation velocity (TRV). (Figure 1B). Assumes the absence of RVOTO or PS; AVV regurgitation does not reflect sPAP in patients with TCPC; Dependent on RV systolic function. Can be measured in most of PH patients; PAAT < 100 ms in adults—PH is likely; Existing reference values in children. Reflects RV/LV systolic and diastolic function and VVI; Independent of chamber geometry; Existing reference values in children; Decreased in adult/pediatric PH-CHD patients. Reflects LV systolic function; Correlates with invasively measured parameters e.g., PVRi, sPAP/sSAP ratio. The red lines mark the rapid acceleration to peak flow velocity in early systole, followed by a fast deceleration in (PAH-CHD). The red lines thesubjects, rapid acceleration to 55 peak velocity in early systole, followed by patient. Compared to mark healthy the PAAT of msflow measured over 4 circles is reduced in this (B) Apical in mid-systole.
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