Multiparametric evaluation of right ventricular function in pulmonary arterial hypertension associated with congenital heart disease

Emmanuelle Fournier, Maëlle Selegny, Myriam Amsallem, Francois Haddad, Sarah Cohen, Estibaliz Valdeolmillos, Jérôme Le Pavec, Marc Humbert, Marc-Antoine Isorni, Arshid Azarine, Olivier Sitbon, Xavier Jais, Laurent Savale, David Montani, Elie Fadel, Joy Zoghbi, Emre Belli, Sebastien Hascoët
Marie Lannelongue Hospital, Groupe Hospitalier Paris Saint Joseph and Paris-Saclay University. Stanford University School of Medicine. Marie Lannelongue Hospital.
France and United States

Revista Espanola de Cardiologia
Rev Esp Cardiol 2022;
DOI: 10.1016/j.rec.2022.07.010

Abstract
Introduction and objectives: Outcome in patients with congenital heart diseases and pulmonary arterial hypertension (PAH) is closely related to right ventricular (RV) function. Two-dimensional echocardiographic parameters, such as strain imaging or RV end-systolic remodeling index (RVESRI) have emerged to quantify RV function.
Methods: We prospectively studied 30 patients aged 48±12 years with pretricuspid shunt and PAH and investigated the accuracy of multiple echocardiographic parameters of RV function (tricuspid annular plane systolic excursion, tricuspid annular peak systolic velocity, RV systolic-to-diastolic duration ratio, right atrial area, RV fractional area change, RV global longitudinal strain and RVESRI) to RV ejection fraction measured by cardiac magnetic resonance.
Results: RV ejection fraction <45% was observed in 13 patients (43.3%). RV global longitudinal strain (ρ [Spearman’s correlation coefficient]=-0.75; P=.001; R2=0.58; P=.001), right atrium area (ρ=-0.74; P <.0001; R2=0.56; P <.0001), RVESRI (ρ=-0.64; P <.0001; R2=0.47; P <.0001), systolic-to-diastolic duration ratio (ρ=-0.62; P=.0004; R2=0.47; P <.0001) and RV fractional area change (ρ=0.48; P=.01; R2=0.37; P <.0001) were correlated with RV ejection fraction. RV global longitudinal strain, RVESRI and right atrium area predicted RV ejection fraction <45% with the greatest area under curve (0.88; 95%CI, 0.71-1.00; 0.88; 95%CI, 0.76-1.00, and 0.89; 95%CI, 0.77-1.00, respectively). RV global longitudinal strain >-16%, RVESRI ≥ 1.7 and right atrial area ≥ 22 cm2 predicted RV ejection fraction <45% with a sensitivity and specificity of 87.5% and 85.7%; 76.9% and 88.3%; 92.3% and 82.4%, respectively.
Conclusions: RVESRI, right atrial area and RV global longitudinal strain are strong markers of RV dysfunction in patients with pretricuspid shunt and PAH.

Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Right Heart Dysfunction Associated with Pulmonary Vascular Disease
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing

Age Focus: Adult Pulmonary Vascular Disease

Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication

Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No

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