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<p>The Fontan circulation is characterized by chronically elevated central venous pressure, and patients with this circulation are at risk for Fontan-associated liver disease (FALD). Progressive hepatic fibrosis contributes substantially to long-term morbidity. Echocardiographic strain imaging enables non-invasive assessment of atrial and ventricular function, and Fontan patients commonly exhibit impaired atrial strain and diastolic dysfunction. The study investigated whether alterations in atrial function and diastolic filling in patients with single ventricle anatomy and Fontan circulation are associated with FALD. This IRB-approved retrospective cohort study included 98 children with Fontan circulation. Atrial and ventricular longitudinal strain were assessed using two-dimensional speckle-tracking echocardiography and Doppler-derived diastolic indices. These metrics were compared between Fontan patients and age- and sex-matched healthy controls. Cardiac catheterization reports were retrospectively reviewed for hemodynamic parameters, including ventricular end-diastolic and mean Fontan pressures. Liver status was determined by histopathology, with ≥Stage 3 fibrosis as the primary outcome for advanced liver fibrosis. Associations between echocardiographic parameters, ventricular morphology, and advanced liver fibrosis were analyzed using logistic regression models. Among 98 Fontan patients (median age 13y.o., IQR = 9.8–17), 20% demonstrated advanced liver fibrosis. These patients showed reduced atrial conduit strain (–14.3 vs − 19.5%, <i>p</i> = 0.033) and reduced ventricular compliance including shorter mitral deceleration time (median 136 vs 175ms, <i>p</i> = 0.002), steeper deceleration slope (median 7.74 vs 3.1m/s<sup>2</sup>, <i>p</i> &lt; 0.0001), higher E/E’ ratios (median 10.9 vs 9.2, <i>p</i> = 0.033) and higher end-diastolic pressure (median 11 vs. 9mmHg, <i>p</i> = 0.006) compared with those without advanced liver fibrosis. Impaired SA-LAScd and steeper mitral deceleration slope remained independently associated with advanced liver fibrosis. Reduced atrial conduit strain and restrictive diastolic filling patterns in Fontan patients are associated with advanced liver fibrosis, linking atrial dysfunction and impaired ventricular filling to Fontan-associated liver disease. These findings support atrial and ventricular diastolic function as non-invasive markers in Fontan circulation, underscoring the need for a prospective study of both Fontan and ventricular hemodynamics in their respective contributions to FALD.</p>

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Reduced atrial conduit strain and abnormal diastolic filling are associated with fontan-associated liver disease

  • 期刊论文

  • 发表日期：

    2026年4月17日

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  • Melina Weller,
  • Miriam S. Burmeister,
  • Shailee Mitra,
  • Susana Gaviria,
  • Roland Axt-Fliedner,
  • Sarah Kollar,
  • Brett A. Meyers,
  • Yue-Hin Loke

摘要

The Fontan circulation is characterized by chronically elevated central venous pressure, and patients with this circulation are at risk for Fontan-associated liver disease (FALD). Progressive hepatic fibrosis contributes substantially to long-term morbidity. Echocardiographic strain imaging enables non-invasive assessment of atrial and ventricular function, and Fontan patients commonly exhibit impaired atrial strain and diastolic dysfunction. The study investigated whether alterations in atrial function and diastolic filling in patients with single ventricle anatomy and Fontan circulation are associated with FALD. This IRB-approved retrospective cohort study included 98 children with Fontan circulation. Atrial and ventricular longitudinal strain were assessed using two-dimensional speckle-tracking echocardiography and Doppler-derived diastolic indices. These metrics were compared between Fontan patients and age- and sex-matched healthy controls. Cardiac catheterization reports were retrospectively reviewed for hemodynamic parameters, including ventricular end-diastolic and mean Fontan pressures. Liver status was determined by histopathology, with ≥Stage 3 fibrosis as the primary outcome for advanced liver fibrosis. Associations between echocardiographic parameters, ventricular morphology, and advanced liver fibrosis were analyzed using logistic regression models. Among 98 Fontan patients (median age 13y.o., IQR = 9.8–17), 20% demonstrated advanced liver fibrosis. These patients showed reduced atrial conduit strain (–14.3 vs − 19.5%, p = 0.033) and reduced ventricular compliance including shorter mitral deceleration time (median 136 vs 175ms, p = 0.002), steeper deceleration slope (median 7.74 vs 3.1m/s2, p < 0.0001), higher E/E’ ratios (median 10.9 vs 9.2, p = 0.033) and higher end-diastolic pressure (median 11 vs. 9mmHg, p = 0.006) compared with those without advanced liver fibrosis. Impaired SA-LAScd and steeper mitral deceleration slope remained independently associated with advanced liver fibrosis. Reduced atrial conduit strain and restrictive diastolic filling patterns in Fontan patients are associated with advanced liver fibrosis, linking atrial dysfunction and impaired ventricular filling to Fontan-associated liver disease. These findings support atrial and ventricular diastolic function as non-invasive markers in Fontan circulation, underscoring the need for a prospective study of both Fontan and ventricular hemodynamics in their respective contributions to FALD.