An observational study in Congenital Heart Disease (CHD) and Univentricular Heart, sponsored by Sisca Natalia Siagian. Recruiting at 1 site in Indonesia. Per ClinicalTrials.gov, last updated 2026-04-17.
Sponsored by Sisca Natalia Siagian · Observational
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.
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.
86 studies on the registry are indexed under Univentricular Heart; 22 are open to participants now.
This study's planned enrollment of 52 is above the median of 42 across 37 observational studies indexed under Univentricular Heart.
Browse Univentricular Heart studies →This is the only study on the registry with Sisca Natalia Siagian as lead sponsor.
Compared within Univentricular Heart, this study's primary condition as ClinicalTrials.gg classifies it; the registry's first condition term is Heart Defects, Congenital. The counts are of registry records indexed under each term, refreshed daily. How we classify →
This study will involve a cohort of clinically diagnosed patients with univentricular heart physiology who are undergoing the Fontan procedure as their final intervention.
Go through the criteria one by one — yes, no or not sure — and take the list to your doctor or the study team. Kept in this browser only.
Exclusion Criteria:
Underlined terms in the criteria open these too. Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
Informed consent
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Patients with cyanotic CHD and univentricular physiology characterized by reduced pulmonary blood flow.
Procedure: Fontan Procedure
Patients with cyanotic CHD and univentricular physiology characterized by increased pulmonary blood flow.
Procedure: Fontan Procedure
Standard staged palliation including Bidirectional Glenn and Fontan procedure. No experimental intervention; observational classification only.
Mortality
All-cause mortality following completion of Fontan procedure.
Time frame: From recruitment through study completion (an average of 1 year post-Fontan)
Number of Participants with Postoperative Complications
Assessment of postoperative morbidity defined as the occurrence of complications (e.g., arrhythmia, infection, thrombosis, or other clinically significant adverse events) following the Fontan procedure. Measurement Tool: Clinical assessment and medical record review Unit of Measure: Number of participants with ≥1 complication
Time frame: From Fontan procedure through study completion (up to 12 months post-Fontan)
Number of Participants with Rehospitalization
Assessment of rehospitalization defined as any unplanned hospital admission after discharge following the Fontan procedure. Measurement Tool: Medical record review Unit of Measure: Number of participants rehospitalized (≥1 admission)
Time frame: From hospital discharge post-Fontan through study completion (up to 12 months post-Fontan)
Concentration of Activin-A in Blood
Measurement of circulating Activin-A concentration as a biomarker of pulmonary vascular remodeling and inflammation in patients with univentricular congenital heart disease undergoing staged palliation. Blood samples will be collected at predefined perioperative time points. Measurement Tool: Enzyme-linked immunosorbent assay (ELISA) or equivalent validated immunoassay Unit of Measure: Picograms per milliliter (pg/mL)
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
Measurement of Activin-A concentration in pulmonary artery tissue obtained during surgical procedures, reflecting local vascular remodeling and inflammatory activity. Measurement Tool: Enzyme-linked immunosorbent assay (ELISA) or equivalent validated immunoassay on homogenized tissue samples Unit of Measure: Picograms per milligram of tissue (pg/mg)
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)
Assessment of functional exercise capacity using the six-minute walk test (6MWT), performed according to standardized protocol. Measurement Tool: Six-minute walk test (6MWT) Unit of Measure: Distance in meters (m) Interpretation: More distance covered means higher exercise capacity
Time frame: Follow-up (6 months post-Fontan procedure)
Neurodevelopmental Score Using Bayley Scales of Infant and Toddler Development, Fourth Edition (BSID-IV)
Assessment of neurocognitive and developmental function in pediatric patients using the Bayley Scales of Infant and Toddler Development, Fourth Edition (BSID-IV). The instrument evaluates multiple domains including cognitive, language (receptive and expressive), motor (fine and gross), social-emotional, and adaptive behavior. Raw scores are converted into standardized composite scores based on age-specific norms. Measurement Tool: Bayley Scales of Infant and Toddler Development, Fourth Edition (BSID-IV) Unit of Measure: Standard score (composite score), typically ranging from 40 to 160, with a mean of 100 and standard deviation of 15 Higher scores indicate better neurodevelopmental performance
Time frame: Follow-up (6 months post-Fontan procedure)
Cognitive Function Score Using Montreal Cognitive Assessment (MoCA)
Assessment of cognitive function in adult patients using the Montreal Cognitive Assessment (MoCA), a screening tool evaluating domains including memory, executive function, attention, language, visuospatial ability, and orientation. Measurement Tool: Montreal Cognitive Assessment (MoCA) Unit of Measure: Score on a scale from 0 to 30, where higher scores indicate better cognitive function; a score of 26 or above is considered normal
Time frame: Follow-up (6 months post-Fontan Procedure)
Quality of Life Score Using Child Health Questionnaire (CHQ)
Assessment of quality of life in pediatric patients using the Child Health Questionnaire (CHQ). The instrument evaluates multiple health domains, which are aggregated into Physical Health Summary (PhS) and Psychosocial Summary (PsS) scores. Measurement Tool: Child Health Questionnaire (CHQ) Unit of Measure: Score on a scale from 0 to 100 (higher scores indicate better health status)
Time frame: Follow-up (6 months post-Fontan procedure)
Quality of Life Score Using 36-Item Short Form Survey (SF-36)
Assessment of quality of life in adult patients using the 36-item Short Form Survey (SF-36). The instrument includes 8 domains aggregated into Physical Component Summary (PCS) and Mental Component Summary (MCS) scores. Measurement Tool: 36-item Short Form Survey (SF-36) Unit of Measure: Score on a scale from 0 to 100 (higher scores indicate better health status)
Time frame: Follow-up (6 months post-Fontan Procedure)
Histopathological Score of Pulmonary Artery Remodeling Using Hematoxylin and Eosin (H&E) Staining
Semi-quantitative assessment of pulmonary artery structural changes (e.g., medial hypertrophy, intimal proliferation, luminal narrowing) using Hematoxylin and Eosin (H\&E) staining. A standardized Pulmonary Artery Remodeling Histopathological Scoring System is used to grade the severity of vascular changes. Measurement Tool: Light microscopy using a semi-quantitative histopathological scoring system Unit of Measure: Score on a scale from 0 to 3, where: 0 = no structural abnormality 1. = mild remodeling 2. = moderate remodeling 3. = severe remodeling Higher scores indicate worse pulmonary artery remodeling
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
Quantification of collagen accumulation in pulmonary artery tissue using Masson's Trichrome staining, analyzed via digital image analysis software. Measurement Tool: Image analysis software (e.g., ImageJ or equivalent) Unit of Measure: Percentage (%) of stained area per total vessel area
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
Evaluation of elastin fiber integrity and distribution in pulmonary artery tissue using Elastica Van Gieson staining. Measurement Tool: Microscopy with image analysis Unit of Measure: Percentage (%) elastin-positive area
Time frame: At time of tissue collection during BCPS and Fontan procedures; analysis performed within 1 month after each collection
Percentage of PAS-Positive Area in Pulmonary Artery Tissue
Assessment of glycoprotein/glycogen and basement membrane components using Periodic Acid-Schiff (PAS) staining. Measurement Tool: Microscopy with quantitative image analysis Unit of Measure: Percentage (%) PAS-positive area
Time frame: At time of tissue collection during BCPS and Fontan procedures; analysis performed within 1 month after each collection
Mean Pulmonary Artery Pressure Measured by Right Heart Catheterization
Measurement of mean pulmonary artery pressure (mPAP) obtained from right heart catheterization to assess pulmonary hemodynamics. Measurement Tool: Right heart catheterization Unit of Measure: Millimeters of mercury (mmHg)
Time frame: Baseline, Post-BCPS preparing for Fontan (average of 3 months post-BCPS procedure), Follow-up (average of 6 months post-Fontan procedure)
Pulmonary Vascular Resistance Measured by Right Heart Catheterization
Calculation of pulmonary vascular resistance (PVR) derived from catheterization data using standard hemodynamic formulas. Measurement Tool: Right heart catheterization with hemodynamic calculation Unit of Measure: Wood units (WU)
Time frame: Baseline, Post-BCPS preparing for Fontan (average of 3 months post-BCPS procedure), Follow-up (average of 6 months post-Fontan procedure)
McGoon Ratio Assessed by Angiography
Evaluation of pulmonary artery size using the McGoon ratio, calculated as the sum of the diameters of the right and left pulmonary arteries divided by the diameter of the descending aorta, measured from angiographic images. Measurement Tool: Angiography with standardized measurement method Unit of Measure: Ratio (unitless)
Time frame: Baseline, Post-BCPS preparing for Fontan (average of 3 months post-BCPS procedure), Follow-up (average of 6 months post-Fontan procedure)
Nakata Index Measured by Angiography
Quantification of pulmonary artery size using the Nakata index, calculated as the sum of cross-sectional areas of the right and left pulmonary arteries normalized to body surface area. Measurement Tool: Angiography with body surface area calculation Unit of Measure: mm²/m²
Time frame: Baseline, Post-BCPS preparing for Fontan (average of 3 months post-BCPS procedure), Follow-up (average of 6 months post-Fontan procedure)
Plan to share: No — Individual participant data will not be publicly shared. Data may be available upon reasonable request to the principal investigator.
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