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

Abstract

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.

Keywords: congenital heart disease; pulmonary hypertension; right ventricular function; tricuspid annular plane systolic excursion.

Figure 1

TAPSE values for BSA in normal population at altitude. Relationship between BSA (m²) and TAPSE (cm) in normal children is shown (adjusted-R² = 0.60). The mean is marked by the solid line. Z-scores of ± 2 are represented by the dashed lines.

Figure 2

Comparison of TAPSE z-scores by aetiology of PH. TAPSE z-scores are significantly reduced only in patients with PH and repaired CHD. Z-scores for patients with idiopathic PH, PH-unrepaired CHD, and other aetiologies did not differ significantly from the normal population. P-values reflect Tukey multiple comparisons of aetiological groups with the repaired CHD group.

Figure 3

Comparison of TAPSE z-scores in patients undergoing surgical repair of CHD. Comparison of TAPSE z-score in patients who underwent surgical repair with and without CPB. Mean z-score for those with surgical repairs on bypass was −2.71 ± 1.29. Those repaired off bypass had mean TAPSE z-score of 0.13 ± 2.02, P < 0.001.

Figure 4

Differences in the relationship between TAPSE Z-score and duration of disease for different aetiologies of PH. TAPSE z-score is reduced but not dependent on duration of disease in patients with repaired CHD. TAPSE is preserved in patients with unrepaired CHD or Eisenmenger syndrome. TAPSE remains normal in most patients up to 15 years after initial diagnosis in patients with other aetiologies of PH, but longer duration of illness is associated with a fall in TAPSE z-score. Dotted lines mark the range of normal z-scores.

Figure 5

Relationship between TAPSE z-scores and qualitative assessment of right ventricular effects of PH by echocardiogram. Box plots illustrating the decreased TAPSE z-scores in patients with (A) RV dysfunction compared with those with qualitatively normal RV systolic function (P = 0.059) and (B) septal flattening compared with normal septal configuration (P = 0.027).

Figure 6

TAPSE Z-score by WHO class in patients with and without exposure to bypass. TAPSE z-score is similar in patients with PH exposed to cardiopulmonary bypass regardless of functional class. TAPSE is significantly lower in patients with worse functional status in those without bypass exposure (I vs. II, P = 0.07, I vs. III/IV P = 0.01, II vs. III/IV ns). *P = 0.01.