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NCT Number: NCT07535203

Fontan Outcomes in Oligemia vs Plethora in Univentricular CHD

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.

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Key information

Conditions

Congenital Heart Disease (CHD) Cardiovascular Abnormalities Cardiovascular Diseases Congenital Abnormalities Congenital, Hereditary, and Neonatal Diseases and Abnormalities Heart Defects, Congenital Heart Diseases Hypovolemia Pathologic Processes Pathological Conditions, Signs and Symptoms Univentricular Heart

Sex eligibility

All sexes

Study type

Observational

Primary location

National Cardiovascular Center Harapan Kita

Jakarta, DKI Jakarta, 11420, Indonesia

Location status: Recruiting

Location contact

Sisca Natalia Siagian, MD

CONTACT

[email protected]

+6281212716332

About this study

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.

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

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

Treatment and study plan

Fontan Procedure

Procedure

Standard staged palliation including Bidirectional Glenn and Fontan procedure. No experimental intervention; observational classification only.

Primary outcomes

  1. Mortality

    Time frame: From recruitment through study completion (an average of 1 year post-Fontan)

    All-cause mortality following completion of Fontan procedure.

Secondary outcomes

  1. Number of Participants with Postoperative Complications

    Time frame: From Fontan procedure through study completion (up to 12 months post-Fontan)

    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

  2. Number of Participants with Rehospitalization

    Time frame: From hospital discharge post-Fontan through study completion (up to 12 months post-Fontan)

    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)

  3. 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)

    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)

  4. 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

    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)

  5. Distance Covered in Six-Minute Walk Test (6MWT)

    Time frame: Follow-up (6 months post-Fontan procedure)

    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

  6. Neurodevelopmental Score Using Bayley Scales of Infant and Toddler Development, Fourth Edition (BSID-IV)

    Time frame: Follow-up (6 months post-Fontan procedure)

    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

  7. Cognitive Function Score Using Montreal Cognitive Assessment (MoCA)

    Time frame: Follow-up (6 months post-Fontan Procedure)

    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

  8. Quality of Life Score Using Child Health Questionnaire (CHQ)

    Time frame: Follow-up (6 months post-Fontan procedure)

    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)

  9. Quality of Life Score Using 36-Item Short Form Survey (SF-36)

    Time frame: Follow-up (6 months post-Fontan Procedure)

    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)

  10. 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

    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

    • = mild remodeling
    • = moderate remodeling
    • = severe remodeling Higher scores indicate worse pulmonary artery remodeling

  11. 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

    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

  12. 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

    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

  13. Percentage of PAS-Positive Area in Pulmonary Artery Tissue

    Time frame: At time of tissue collection during BCPS and Fontan procedures; analysis performed within 1 month after each collection

    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

  14. Mean Pulmonary Artery Pressure Measured by Right Heart Catheterization

    Time frame: Baseline, Post-BCPS preparing for Fontan (average of 3 months post-BCPS procedure), Follow-up (average of 6 months post-Fontan procedure)

    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)

  15. Pulmonary Vascular Resistance Measured by Right Heart Catheterization

    Time frame: Baseline, Post-BCPS preparing for Fontan (average of 3 months post-BCPS procedure), Follow-up (average of 6 months post-Fontan procedure)

    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)

  16. McGoon Ratio Assessed by Angiography

    Time frame: Baseline, Post-BCPS preparing for Fontan (average of 3 months post-BCPS procedure), Follow-up (average of 6 months post-Fontan procedure)

    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)

  17. Nakata Index Measured by Angiography

    Time frame: Baseline, Post-BCPS preparing for Fontan (average of 3 months post-BCPS procedure), Follow-up (average of 6 months post-Fontan procedure)

    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

Study contacts

Contact information is provided by the study sponsor or research team.

Sisca Natalia Siagian, MD

CONTACT

[email protected]

+6281212716332

Sponsors and collaborators

Lead sponsor

Sisca Natalia Siagian

Other

Collaborators

  • Fakultas Kedokteran Universitas Indonesia
  • LMU Klinikum
  • National Cardiovascular Center Harapan Kita Hospital Indonesia

Registry information

Official study title

Comparison of Outcomes of Pulmonary Oligemia Versus Pulmonary Plethora in Cyanotic Congenital Heart Disease With Univentricular Heart After Fontan Procedure

Important dates

Study start
2025
Primary completion
2027
Study completion
2027
First posted
Apr 17, 2026
Registry last updated
Apr 17, 2026

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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