Echocardiography for the Assessment of Pulmonary Hypertension and Congenital Heart Disease in the Young

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.

Full recovery of right ventricular systolic function in children undergoing bilateral lung transplantation for severe PAH

“…Our data demonstrate that readily available transthoracic echocardiography (25,28) can sufficiently document the post-LuTx improvements of RVH, RV dilation, and LV filling. In this regard, we unraveled the RV/LV end-systolic diameter ratio (29,30), LV end-systolic eccentricity index (LVES EI) (31,32), and RV end-systolic remodeling index (RVES RI) (33) as the most robust and useful echocardiographic markers (25,28,30,34). We (29) and others (31,35) showed that children with more than mild PH had increased RV/LV endsystolic dimension ratio (>1) that reached a grossly abnormal ratio of 2.6 pre-LuTx in the end-stage PAH children of the current study (Figure 1E,F).…”

Normal Echocardiographic Reference Values of the Right Ventricular to Left Ventricular Endsystolic Diameter Ratio and the Left Ventricular Endsystolic Eccentricity Index in Healthy Children and in Children With Pulmonary Hypertension

“…These data demonstrate that readily available transthoracic echocardiography ( 2 , 37 ) can sufficiently document the post-LuTx improvements of RV-hypertrophy, RV dilation, and LV filling. In this regard, the RV/LVes ratio ( 10 , 38 ), the LVes EI ( 26 , 39 ), and RV endsystolic remodeling index ( 9 ) are robust and useful echocardiographic markers ( 2 , 11 , 37 , 38 ). Moreover, the LV EI and RV/LV dimension ratio were associated with a lower WHO functional class, and worse hemodynamics ( 26 ).…”

“…Taken together, we provide normal reference values and percentiles (of the RV/LVes ratio and LVes EI in healthy children and adolescents (1 day–18 years) that will assist to identify children with abnormal values. The RV/LVes ratio and the LVes EI have been shown to be useful, simple, reliable, and non-invasive markers of right ventricular hypertension in infants with PH ( 11 , 12 , 19 , 36 , 40 ) and may be incorporated in routine protocols when there is a concern for PH in children.…”

A guide to echocardiographic assessment in children and adolescents with pulmonary hypertension

“…While the M-mode measurement and use of the Teichholz formula is based on the assumption of an ellipsoid geometrical structure of the left ventricular cavity, this measurement can be flawed. Particularly in conditions where the geometrical structure of the left ventricle is altered, either due to morphological changes due associated congenital heart disease, previous surgery, abnormal wall motion (abnormal conduction) or in PH with significant septal shift towards the left ventricle, the geometrical shape of the left ventricular cavity is changed (31,(79)(80)(81). For quantitative measure of left ventricular function, the modified Simpson formula can be used, as described elsewhere.…”