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Absence of pulmonary artery growth after fontan operation and its possible impact on late outcome
Stanislav Ovroutski1, Peter Ewert, Vladimir Alexi-Meskishvili
1Department of Congenital Heart Diseases, German Heart Institute Berlin, Berlin, Germany. ovroutski@dhzb.de
Insights
Pulmonary arteries (PAs) in children show reduced growth after Fontan operation, despite normal body growth. This impaired PA development may limit long-term Fontan circulation effectiveness.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Vascular Biology
Background:
- The Fontan operation is a palliative procedure for complex single-ventricle congenital heart defects.
- Long-term outcomes after Fontan palliation are influenced by various factors, including pulmonary artery development.
Purpose of the Study:
- To evaluate the long-term development of pulmonary arteries (PAs) in children following a Fontan operation.
- To assess the relationship between PA growth, somatic growth, and Fontan circulation outcomes.
Main Methods:
- Angiographic measurements of central and lower lobe PA diameters were taken before and after Fontan operation in 35 patients.
- Body surface area-adjusted PA indices were calculated and compared between preoperative and follow-up assessments.
- Correlations between PA indices, follow-up duration, and clinical outcomes were investigated.
Main Results:
- Despite parallel somatic growth, pulmonary arteries showed no significant increase in diameter post-Fontan operation.
- The PA index and lower lobe index significantly decreased during the follow-up period.
- A lower PA index correlated with a longer follow-up duration and unfavorable Fontan outcomes.
Conclusions:
- Pulmonary artery growth is significantly restricted after the Fontan operation, even with normal somatic development.
- Reduced PA growth may increase pulmonary vascular resistance, potentially limiting the long-term efficacy of Fontan circulation.
- This finding highlights a critical factor affecting the long-term management of Fontan patients.
Background:
The purpose of this study was to evaluate the development of the pulmonary arteries (PAs) after Fontan operation in children at long term.
Methods:
Thirty-five patients in whom Fontan operation was performed at median age of 4.2 years (range, 1.5 to 16.1 years) underwent angiographic measurements of the central and lower lobe PA diameter before Fontan operation and during the median follow-up of 4.6 years (range, 1.4 to 15.1 years). The median patient age at follow-up was 8.6 years (range, 3.4 to 27.2 years). Body surface area-dependent PA index and lower lobe index were calculated, and preoperative and follow-up values were compared. A correlation between the PA indices and the duration of the follow-up as well as between PA indices and the outcome was investigated.
Results:
Although percentile parallel somatic development of the children could be documented by body surface area measurements (0.62 to 0.93 m(2) during the follow-up; p < 0.001), the PA showed no gain in diameter at all. The PA index and lower lobe index (preoperative median, 261 and 138 mm(2)/m(2), respectively) decreased significantly during the follow-up period (median, 177 and 109 mm(2)/m(2);p < 0.001). The lowest PA index was noted in patients who had the longest follow-up (R = 0.5; p = 0.009). We found a correlation between a low PA index and an unfavorable Fontan outcome (n = 10, p = 0.002).
Conclusions:
Growth of PAs after Fontan operation is clearly reduced despite somatic growth. This phenomenon may lead to an increase in pulmonary vascular resistance and could be a limit for optimal Fontan circulation in grown children in the long term.
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