Fontan Outcomes in Oligemia vs Plethora in Univentricular CHD
For patients and families
In plain language
An automatic summary of structured registry data. It is an orientation aid, not a substitute for the official protocol or a physician assessment.
- What is being studied
- The protocol lists: Fontan Procedure.
- Who it may be relevant to
- Registry conditions: Congenital Heart Disease (CHD), Univentricular Heart. Basic parameters: No limits · All.
- What needs checking
- Age, condition and sex are only basic indicators. Prior treatment, laboratory values and other mandatory requirements appear in the eligibility criteria below.
- Where it takes place
- Indonesia
- Next step
- Save the trial, show it to the treating physician, and confirm current recruitment with the study center. Costs, documents and travel →
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Official title
Comparison of Outcomes of Pulmonary Oligemia Versus Pulmonary Plethora in Cyanotic Congenital Heart Disease With Univentricular Heart After Fontan Procedure
Overview
This prospective cohort study evaluates differences in clinical outcomes between pulmonary oligemia and pulmonary plethora in patients with cyanotic congenital heart disease and univentricular heart physiology undergoing staged palliation culminating in the Fontan procedure.Fifty-two patients will be classified into two groups based on pulmonary blood flow characteristics and followed for approximately 1.5 years after the Fontan procedure. Outcomes include mortality, morbidity, pulmonary hemodynamics, functional capacity, neurocognitive status, and quality of life.
Detailed description
Univentricular heart physiology represents a complex form of congenital heart disease requiring staged surgical palliation. The Fontan procedure has improved survival; however, outcomes remain variable and are influenced by pulmonary vascular conditions. Pulmonary blood flow abnormalities-oligemia (reduced flow) and plethora (increased flow)-may affect lung development from fetal life. Oligemia has been associated with pulmonary hypoplasia, impaired alveolar formation, and increased pulmonary vascular resistance, which may adversely affect Fontan circulation.
This prospective cohort study aims to compare outcomes between patients with oligemia and those with plethora. Participants will undergo standard clinical care, including staged procedures (Bidirectional Glenn and Fontan), and comprehensive assessments (clinical, laboratory, imaging, and hemodynamic). Follow-up will be conducted for approximately 1.5 years post-Fontan.
The study will evaluate mortality, morbidity, pulmonary hemodynamics, biomarkers (NT-proBNP, ET-1, Activin-A), exercise capacity, neurocognitive outcomes, and quality of life. Findings are expected to improve risk stratification and management strategies in univentricular CHD.
Interventions
- Procedure Fontan Procedure
Standard staged palliation including Bidirectional Glenn and Fontan procedure. No experimental intervention; observational classification only.
Primary outcome measures
- Mortality [Time frame: From recruitment through study completion (an average of 1 year post-Fontan)]
Secondary outcome measures (12)
- Number of Participants with Postoperative Complications [Time frame: From Fontan procedure through study completion (up to 12 months post-Fontan)]
- Number of Participants with Rehospitalization [Time frame: From hospital discharge post-Fontan through study completion (up to 12 months post-Fontan)]
- Concentration of Activin-A in Blood [Time frame: Baseline (pre-BCPS procedure), Perioperative at Stage II (Bidirectional Cavopulmonary Shunt [BCPS]), Perioperative at Stage III (Fontan procedure)]
- Concentration of Activin-A in Pulmonary Artery Tissue [Time frame: - At time of tissue collection during Stage II (Bidirectional Cavopulmonary Shunt [BCPS]) - At time of tissue collection during Stage III (Fontan procedure) Analysis performed within 1 month after each collection]
- Distance Covered in Six-Minute Walk Test (6MWT) [Time frame: Follow-up (6 months post-Fontan procedure)]
- Neurodevelopmental Score Using Bayley Scales of Infant and Toddler Development, Fourth Edition (BSID-IV) [Time frame: Follow-up (6 months post-Fontan procedure)]
- Cognitive Function Score Using Montreal Cognitive Assessment (MoCA) [Time frame: Follow-up (6 months post-Fontan Procedure)]
- Quality of Life Score Using Child Health Questionnaire (CHQ) [Time frame: Follow-up (6 months post-Fontan procedure)]
- Quality of Life Score Using 36-Item Short Form Survey (SF-36) [Time frame: Follow-up (6 months post-Fontan Procedure)]
- Histopathological Score of Pulmonary Artery Remodeling Using Hematoxylin and Eosin (H&E) Staining [Time frame: At time of tissue collection during BCPS and Fontan procedures; analysis performed within 1 month after each collection]
- Percentage of Collagen Deposition Using Masson's Trichrome Staining [Time frame: At time of tissue collection during BCPS and Fontan procedures; analysis performed within 1 month after each collection]
- Elastin Density Measured by Verhoeff-Van Gieson (VVG) Staining [Time frame: At time of tissue collection during BCPS and Fontan procedures; analysis performed within 1 month after each collection]
Eligibility criteria
Inclusion criteria
- Diagnosed cyanotic CHD
- Univentricular heart physiology
- Planned staged palliation including Fontan procedure
- Informed consent obtained (patient or guardian)
Exclusion criteria
- Refusal to participate
- Incomplete clinical data
- Inability to complete follow-up
Criteria are shown verbatim from the registry (in English). Final eligibility is always assessed by the study center.
Healthy volunteers: No
Study design
- Observational model
- Cohort
Study locations
Indonesia · 1 center
- National Cardiovascular Center Harapan Kita — Jakarta
Identifiers
NCT: NCT07535203 · DP.04.03/D.XIII/6681/2025