Echocardiography in Pediatric Pulmonary Arterial Hypertension

Abstract

Pulmonary Arterial Hypertension (PAH) is a severe pulmonary vascular disease and often progresses rapidly toward right ventricular (RV) failure and death if left untreated.

Right ventricular base/apex ratio in the assessment of pediatric pulmonary arterial hypertension: Results from the European Pediatric Pulmonary Vascular Disease Network

“…We found dilation of the RV base and RV apex (a low RV b/a ratio) to be an echocardiographic signature of PAH and showed an association of the parameter with echocardiographically determined RV parameters, with the NYHA functional class, and with invasive hemodynamic variables. 25,26 A tool to assess RV dimension, the RV/LV end-systolic dimension ratio, was derived to combine a measure of RV size with septal shift secondary to elevated RV pressure. 5,23 Such an RV/LV dimension ratio had been shown to be significantly higher in children with PAH vs healthy controls.…”

Transthoracic Echocardiography in the Evaluation of Pediatric Pulmonary Hypertension and Ventricular Dysfunction

“…Early evidence for the epidemiology of PH originated from adult registries; however, several newer pediatric international registries have contributed to our understanding of the disease in children as well. [1][2][3][4][5] Pediatric PH can be associated with a variety of underlying conditions, including congenital heart disease (CHD), connective-tissue disorders, pulmonary disease, and endocrine, metabolic, and infectious diseases such as HIV. PH is usually progressive from infancy to adulthood and associated with poor prognosis.…”

Left Ventricular Diastolic Dysfunction in Pediatric Pulmonary Hypertension

“…18 Nevertheless, over-reliance on a single echocardiographic variable and false estimation of RV systolic pressure because of poor CW-Doppler envelopes or severe tricuspid regurgitation can limit the value of the methodology for non-PH experts. 18 More novel, potentially prognostic echocardiographic variables to assess RV/LV geometry/ function and RVP or PAP in pediatric PH include RA size 19,20 ; tricuspid annular plane systolic excursion 21 ; RV outflow tract velocity time integral; trivial tricuspid/RVOT velocity time integral ratio 22 ; pulmonary regurgitation velocity; RV size 20 ; LV size, LV end-systolic eccentricity index, end-systolic and end-diastolic RV/LV ratio 23 ; RV stroke work 24 ; LV strain and strain rate 25 ; RV systolic/diastolic duration ratio 26 ; and pulmonary artery acceleration time (Koestenberger M, MD, unpublished data, 2016). A combination of the above variables can help the clinician avoid some of the pitfalls of the echocardiographic examination at initial diagnosis and serial…”