Table 2

Echocardiographic investigations in suspected or confirmed paediatric PH

Echocardiographic variablesInterpretationAt diagnosisFollow-upRef.
PASP (mm Hg)Bernoulli equation (dP=4×v2): TR velocity used to estimate RVSP (≈PASP)YesYes10 11
PASP >50 mm Hg at rest, makes PAH highly likely in adults
Mean PAP (mm Hg)Estimated mPAP=maximum PR velocity plus mean RAPYesYes11
Def. paediatric PH: mean PAP >25 mm Hg and PVRi >3.0 WU m2
Diastolic PAP (mm Hg)Estimated PADP=minimal (end diastolic) PR velocity plus mean RAPYesYes12
PADP independent of RV stroke volume
TR peak velocity (m/s)RVSP=maximum instantaneous TR gradient across tricuspid valve plus theYesYes11
right atrial v wave. If TR velocity >3.4 m/s, PH is likely (see PASP)
TAPSE (mm)Decreased in advanced PH with systolic RVD in children and adultsYesYes17 18
Tricuspid Em (cm/s)—TV inflowImpaired <8 cm/s indicates higher mortality risk in children with PHYesYes35
S/D ratioIncreased S/D duration ratio >1.4 inversely correlates with survival in children with PH; indicator of PH severityYesProbably31 32
Strain (%)Patients with PH may have lower end-systolic longitudinal strain (RV free wall)If available20 23
Strain rate (s–1)RV strain rate at the RV free wall may be impaired in patients with PHIf available21
RV strain and strain rate are frequently altered in severe PH with RVD
3D echocardiographyUseful for determination of RV and LV volumesIf available28
S′ in RV TDI (cm/s)Decreased in advanced PH with systolic RV dysfunction; an impaired S′YesYes34
(measured in TDI, lateral RV wall) predicts adverse outcome
RV/LV ratioRV/LV ratio >1 indicates increased risk for adverse events in paediatric PHYesYes39
PA acceleration time (ms)PAAT of <100 ms—high probability of PH in adults (HR dependent)YesYes41
RV FAC (%)Decreased RV FAC indicated decreased systolic RV function in adult PHProbably17
E/A ratio (mitral inflow)Reversed E/A (E>A) indicates a high LAP and a high LVEDPYesYes36 37
S′ TDI (cm/s; LV lateral, septal)Nagueh formula may be used to estimate the PCWP (≈ LVEDP)Probably36
LVEF (%; LV systolic function)LVEF (Simpson’s method) may be decreased in severe RV–LV interactionYesYes37
  • Em, early diastolic myocardial relaxation velocity; FAC, fractional area change; LAP, left atrial pressure; LVEDP, LV end-diastolic pressure; PAAT, pulmonary artery acceleration time; PCWP, pulmonary capillary wedge pressure; PADP, pulmonary artery end-diastolic pressure; PAH, pulmonary arterial hypertension; PAP, pulmonary artery pressure; PASP, pulmonary artery systolic pressure; PVR, pulmonary vascular resistance; PR, pulmonary regurgitation; RAP, right atrial pressure; RVD, RV dysfunction; RVSP, RV systolic pressure; S′, peak systolic velocity; S/D, systolic/diastolic ratio; TAPSE, tricuspid annular plane systolic excursion; TDI, tissue Doppler imaging; TR, tricuspid regurgitation; TV, tricuspid valve.