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Univentricular repair in children under 2 years of age: early and midterm results
U K Chowdhury1, B Airan, R Sharma
1Department of Cardiothoracic and Vascular Surgery, Cardiothoracic Centre, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.
Insights
Younger children under two years old can undergo successful univentricular repair, showing similar survival rates to older patients. Careful patient selection and routine fenestration of the intra-atrial baffle can improve outcomes in univentricular repair.
Area of Science:
- Pediatric Cardiac Surgery
- Congenital Heart Disease Repair
- Single Ventricle Physiology Management
Background:
- Early univentricular repair has shown promising results in treating complex congenital heart defects.
- The impact of young age, specifically under two years, on the outcomes of univentricular repair requires further assessment.
Purpose of the Study:
- To evaluate the outcomes of univentricular repair in patients younger than two years compared to older patients.
- To identify risk factors associated with Fontan failure and pleural effusion in this pediatric population.
Main Methods:
- A retrospective comparison of 65 patients under two years (group 1) versus 246 patients over two years (group 2) undergoing univentricular repair.
- All patients from 1994 onwards received intra-atrial baffle fenestration.
- Outcomes analyzed included early mortality, Fontan failure, pleural effusion, and long-term survival.
Main Results:
- Early mortality rates were comparable (9.2% in group 1 vs. 7.7% in group 2).
- Overall Fontan failure rates were similar (12.3% in group 1 vs. 12.2% in group 2).
- Significant risk factors for Fontan failure included aortic cross-clamp time > 60 minutes (P < 0.01). Significant risk factors for pleural effusion included low weight (<10 kg), absence of fenestration, ventricular dysfunction, and elevated pressures.
Conclusions:
- Carefully selected patients under two years are suitable for one-stage univentricular repair, achieving survival rates comparable to older patients.
- Routine fenestration of the intra-atrial baffle is a valuable strategy to reduce morbidity and mortality.
- Non-compliance with more than two Choussat criteria may negatively impact outcomes.
Background:
Encouraging results have been obtained from early univentricular repair. Our study was designed to assess the impact of young age (less than 2 years) on the outcome of univentricular repair.
Patients And Methods:
Between January 1992 and December 1998, 65 out of 311 patients undergoing univentricular repair at the All India Institute of Medical Sciences, New Delhi, were less than 2 years of age. We compared these 65 carefully selected patients (group 1) with the 246 patients who were more than 2 years of age (group 2). Since 1994, all patients of both groups had a fenestration of the intra-atrial baffle.
Results:
The early mortality rate was 9.2% in group 1 and 7.7% in group 2. The overall Fontan failure rate was 12.3% in group 1 and 12.2% in group 2. In group 1, a higher incidence of Fontan failure was noted in patients with suboptimal weight, non-tricuspid atresia morphology, nonfenestrated Fontan and those who did not comply with more than two of Choussat's criteria, but the values were not statistically significant. Aortic cross-clamp time of more than 60 min was the only and highly significant predictor of Fontan failure (P < 0.01). The overall effusion rate was 24.05% in group 1 and 27.98% in group 2. In group 1, patients weighing less than 10 kg (P = 0.0007), without fenestration of the atrial baffle (P < 0.05) and with systemic ventricular dysfunction (P < 0.001), systemic ventricular end-diastolic pressure of more than 12 mmHg (P < 0.001), mean pulmonary artery pressure of more than 15 mmHg (P < 0.001) and aortic crossclamp time of more than 60 min (P < 0.01), were all found to be significant risk factors of pleural effusion. Pulmonary artery distortion needing reconstruction did not increase the Fontan failure or effusion rates. Oxygen saturation ranged from 85 to 94% (mean 89%) in patients having a functioning fenestration at a mean follow-up period of 30 months. The actuarial survival at 84 months was 90 +/- 0.04% in group 1 and 88 +/- 0.02% in group 2.
Conclusions:
Our results suggest that carefully selected patients under 2 years of age are suitable candidates for one-stage univentricular repair and that survival is not significantly different from that of older patients. Routine fenestration of the intra-atrial baffle is an option available to ameliorate morbidity and obviate mortality. Non-compliance with more than two of Choussat's criteria appears to be additive in unsatisfactory outcome.
