Tricuspid annular plane systolic excursion is preserved in young patients with pulmonary hypertension except when associated with repaired congenital heart disease.

Aims: Tricuspid annular plane systolic excursion (TAPSE) is a measure of right ventricular (RV) longitudinal function that correlates with functional status and mortality in adults with pulmonary hypertension (PH). The diagnostic and predictive value of TAPSE in children with PH has not been fully examined. We aimed to define TAPSE across aetiologies of paediatric PH and assess the correlation between TAPSE and measures of disease severity.

Methods And Results: TAPSE measurements were obtained in 84 children and young adults undergoing treatment for PH and 315 healthy children to establish z-scores at moderate altitude for comparison. The relationships between TAPSE and echocardiographic, biomarker, and functional measures of disease severity between aetiologies were assessed. TAPSE z-scores in PH patients with congenital heart disease (CHD) repaired with open cardiac surgery (n = 20, mean -2.73) were significantly decreased compared with normal children and patients with other aetiologies of PH (P < 0.001) but did not reflect poorer clinical status. TAPSE z-scores in children with idiopathic PH (n = 29, -0.41), unrepaired CHD (n = 11, -0.1), and PH related to systemic disease (n = 14, -0.39) were not different from normal. TAPSE correlated modestly with brain natriuretic peptide, echocardiographic function parameters, and functional class except in patients with repaired CHD.

Conclusion: Children with PH maintain normal TAPSE values early except when associated with repaired CHD. Superior RV adaptation to high afterload in children compared with adults may account for this finding. Reduced TAPSE after repair of CHD does not correlate with functional status and may reflect post-operative changes rather than poor function primarily due to PH.