Comparison of Outcomes of Pulmonary Oligemia Versus Pulmonary Plethora in Cyanotic Congenital Heart Disease With Univentricular Heart After Fontan Procedure
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 发起方
- 入组人数
- 52
- 试验地点
- 1
- 主要终点
- Mortality
研究概览
简要总结
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.
详细描述
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.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Diagnosed cyanotic CHD
- •Univentricular heart physiology
- •Planned staged palliation including Fontan procedure
- •Informed consent obtained (patient or guardian)
排除标准
- •Refusal to participate
- •Incomplete clinical data
- •Inability to complete follow-up
研究组 & 干预措施
Pulmonary Oligemia
Patients with cyanotic CHD and univentricular physiology characterized by reduced pulmonary blood flow.
干预措施: Fontan Procedure (Procedure)
Pulmonary Plethora
Patients with cyanotic CHD and univentricular physiology characterized by increased pulmonary blood flow.
干预措施: Fontan Procedure (Procedure)
结局指标
主要结局
Mortality
时间窗: From recruitment through study completion (an average of 1 year post-Fontan)
All-cause mortality following completion of Fontan procedure.
次要结局
- Number of Participants with Postoperative Complications(From Fontan procedure through study completion (up to 12 months post-Fontan))
- Number of Participants with Rehospitalization(From hospital discharge post-Fontan through study completion (up to 12 months post-Fontan))
- Concentration of Activin-A in Blood(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(- 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)(Follow-up (6 months post-Fontan procedure))
- Neurodevelopmental Score Using Bayley Scales of Infant and Toddler Development, Fourth Edition (BSID-IV)(Follow-up (6 months post-Fontan procedure))
- Cognitive Function Score Using Montreal Cognitive Assessment (MoCA)(Follow-up (6 months post-Fontan Procedure))
- Quality of Life Score Using Child Health Questionnaire (CHQ)(Follow-up (6 months post-Fontan procedure))
- Quality of Life Score Using 36-Item Short Form Survey (SF-36)(Follow-up (6 months post-Fontan Procedure))
- Histopathological Score of Pulmonary Artery Remodeling Using Hematoxylin and Eosin (H&E) Staining(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(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(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(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(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(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(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(Baseline, Post-BCPS preparing for Fontan (average of 3 months post-BCPS procedure), Follow-up (average of 6 months post-Fontan procedure))
研究者
Sisca Natalia Siagian
Principal Investigator and Sponsor
National Cardiovascular Center Harapan Kita Hospital Indonesia