Abstract 4135534: Total and Regional Lung Volumes And Pulmonary Blood Flow In Repaired Tetralogy Of Fallot - Smaller Lungs And A Maldistribution of Flow

Introduction: Many pts with repaired tetralogy of Fallot (rTOF) are left with residual pulmonary regurgitation (PR) and poor exercise performance. Small studies in young children suggest that the total lung volume of rTOF pts are smaller than normal and may contribute to these sequelae. As exercise and cardiac performance are intimately related to lung function, it’s surprising that few rTOF studies have investigated cardiopulmonary interactions. Aims: To determine if regional and total lung volumes in rTOF pts are the same size as normals and if the larger amount of forward blood flow courses to the bigger lung. We hypothesized that this may not be the case. Methods: rTOF patients undergoing clinically indicated cardiac magnetic resonance (CMR) were evaluated for lung volumes using dark blood imaging (figure) and blood flow to the branch pulmonary arteries (bPA) using velocity mapping. Significance < P=0.05. Results: Eleven rTOF patients and 25 normal controls were studied. Total control lung volume was significantly larger than those of rTOF. Regional rTOF right and left lung volumes were smaller than their respective control lung volumes, barely missed statistical significance (table). In 6 rTOF pts with CMR prior to and after pulmonary valve replacement (PVR), lung volumes did not differ between the 2 timepoints (1034 vs 998 cc/m 2 respectively). Only 3/11 (27%) rTOF pts had the higher bPA flow coursing to the higher regional lung. The ratio of right bPA forward flow/right lung volume to left bPA forward flow/left lung volume ranged from 0.72-1.51 (ideal balanced flow to each lung would be 1), implying a maldistribution of flow. Conclusion: rTOF patients have smaller total lung volumes than controls with no change after PVR in our study. In addition, only a small proportion of rTOF patients have the larger amount of flow coursing to the larger lung. The ratio of individual bPA flow to individual lung volume implies a maldistribution of pulmonary flow relative to the respective lung volume. Our findings may have implications for timing of PVR, partially explaining PR and poor exercise performance. Flow limiters placed by cath or surgery may be able to redistribute the appropriate flow to regional lung fields.