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Serial assessment of somatic and cardiovascular development in patients with single ventricle undergoing Fontan
M Granegger1, S Küng2, O Bollhalder2
1Pediatric Cardiovascular Surgery, Department of Surgery, Pediatric Heart Center, University Children's Hospital Zurich, Zurich, Switzerland; Children's Research Center, University Children's Hospital Zurich, Zurich, Switzerland; Biofluid Mechanics Laboratory, Institute for Imaging Science and Computational Modelling in Cardiovascular Medicine, Charité-Universitätsmedizin Berlin, Berlin, Germany.
Insights
Fontan procedure patients show catch-up growth in somatic and cardiac parameters, but diastolic function remains altered. Factors like procedure timing and ventricular morphology influence this development in single ventricle (SV) patients.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Research
- Cardiovascular Development
Background:
- The Fontan procedure improves survival for single ventricle (SV) patients but has limitations due to cardiovascular complications.
- Understanding long-term somatic and cardiovascular development post-Fontan is crucial for managing these patients.
Purpose of the Study:
- To describe somatic and cardiovascular development in Fontan patients until adolescence.
- To identify factors influencing this development, including age at procedure, gender, and ventricular morphology.
Main Methods:
- Retrospective analysis of 134 patients from birth to 16 years.
- Transthoracic echocardiography used to assess somatic growth, pulmonary artery and SV dimensions, and semilunar valve function.
- Doppler inflow patterns and z-scores analyzed with linear mixed effect models.
Main Results:
- Somatic growth catch-up observed post-Fontan, with lower height/weight z-scores in males and systemic right ventricles.
- Early Fontan procedure timing favored somatic growth but not vascular development.
- Single ventricle end-diastolic diameter z-scores decreased, and diastolic function (E/A ratio) remained diminished.
Conclusions:
- Post-Fontan catch-up growth is influenced by procedure timing, gender, and ventricular morphology.
- Diastolic function of the single ventricle remains persistently altered despite other parameter improvements.
Background:
The palliation of patients with single ventricle (SV) undergoing Fontan procedure led to improved long-term survival but is still limited due to cardiovascular complications. The aim of this study was to describe the somatic and cardiovascular development of Fontan patients until adolescence and to identify determining factors.
Methods:
We retrospectively assessed somatic growth, vascular growth of pulmonary arteries, and cardiac growth of the SV and systemic semilunar valve from 0 to 16 years of age using transthoracic echocardiography. The Doppler inflow pattern of the atrioventricular valve was quantified by E-, A-wave and E/A ratio. All data were converted to z-scores and analyzed using linear mixed effect models to identify associations with age at Fontan procedure, gender, and ventricular morphology.
Results:
134 patients undergoing Fontan procedure at a median age of 2.4 (IQR 2.12 to 2.8) years were analyzed. A catch-up of somatic growth after Fontan procedure until school age was found, with lower body height and weight z-scores in male patients and patients with systemic right ventricles. An early time of Fontan procedure was favorable for somatic growth, but not for vascular growth. Cardiac development indicated a decrease of SV end-diastolic diameter z-score until adolescence. Despite a trend towards normalization, E-wave and E/A ratio z-scores were diminished over the entire period.
Conclusions:
There is a catch-up growth of somatic, vascular and cardiac parameters after Fontan procedure, which in our cohort depends on the time of Fontan procedure, ventricular morphology, and gender. Beside other factors, diastolic function of the SV remains altered.
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