Integrated echocardiographic right ventricular myocardial work and cardiopulmonary exercise testing in pediatric precapillary pulmonary arterial hypertension: A pilot study - 27/08/26
, Aitor Guitarte, Paul Vignaud, Yves Dulac, Philippe Acar, Khaled Hadeed, Clement KarsentyRésumé |
Introduction |
Right ventricular (RV) adaptation is the main determinant of functional limitation in precapillary pulmonary arterial hypertension (PCPH). Resting hemodynamics may remain preserved despite early impairment of RV mechanical and energetic performance. The relationship between RV myocardial work (MW) and cardiopulmonary exercise testing (CPET) parameters in pediatric PCPH is not well defined. The aim was to investigate the associations between RV MW indices and exercise ventilatory efficiency in children with PCPH.
Methods |
Nine pediatric patients with precapillary PCPH were prospectively evaluated with median age of 15.4 years (range: 7.7–17.9; IQR: 6.3) and median weight of 52.8 kg (range: 21.0–74.6; IQR: 42.3). Etiology included idiopathic PCPH ( n = 6), BMPR2 mutation–associated PCPH ( n = 2), and GDF2 mutation–associated PCPH ( n = 1). Two patients had a Potts shunt. All patients underwent comprehensive 2D and 3D transthoracic echocardiography including speckle-tracking-derived RV strain and MW analysis, together with CPET.
Results |
Median RV ejection fraction was 51.1% (range: 38.7–59.4; IQR: 13.9), demonstrating heterogeneous systolic impairment despite preserved resting hemodynamics. Pulmonary artery systolic pressure was elevated at 66 mmHg (range: 38–122; IQR: 59). Resting cardiac index was preserved in all but one patient (1.7 L/min/m 2 ). CPET revealed impaired ventilatory efficiency with VE/VCO 2 slope 42.9 (range: 38.5–49.8; IQR: 6.2) and reduced minimal PETCO 2 23.3 mmHg (range: 17.1–31.7; IQR: 4.1), with a downward PETCO 2 trajectory during exercise in all patients. Exercise capacity was moderately reduced (VO 2 peak 79% predicted [range: 35–99; IQR: 20.4]).
Minimal PETCO 2 strongly correlated with RV MW parameters: global work index (GWI; r = −0.879, P = 0.003), constructive work (GCW; r = −0.866, P = 0.004), and wasted work (GWW; r = −0.753, P = 0.024). Two clinical events occurred during follow-up (RV failure after viral infection and syncope related to increased pulmonary vascular resistance), both observed in patients with abnormal MW and CPET parameters.
Conclusion |
In pediatric precapillary PCPH, exercise ventilatory inefficiency was associated with RV MW parameters despite largely preserved resting cardiac output. Minimal PETCO 2 may represent an integrative marker of RV functional adaptation during exercise. Larger studies are required to confirm these findings.
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Vol 119 - N° 8-9S
P. S255 - août 2026 Retour au numéro
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