Objective: Congenital heart diseases in pediatric patients are associated with a wide variety of extracardiac thoracic vascular abnormalities. This study analyzed the value of gadolinium-enhanced magnetic resonance angiography during quiet free breathing for assessing extracardiac thoracic vascular abnormalities in young children with congenital heart diseases.
Methods: Fifty-three children with congenital heart diseases (age range, 1 day-40 months; mean, 10.9 months) associated with extracardiac thoracic vascular abnormalities who had undergone both free-breathing, gadolinium-enhanced magnetic resonance angiography and cardiac catheterization, surgical intervention, or both within 2 weeks were reviewed. Diagnostic findings on gadolinium-enhanced magnetic resonance angiography among patients grouped according to 3 major conditions of conotruncal abnormalities (n = 33), aortic or venous abnormalities (n = 11), and pulmonary vascular abnormalities (n = 9), as well as associated extracardiac thoracic vascular abnormality findings, were compared with findings made by using cardiac catheterization, surgical intervention, or both.
Results: Extracardiac thoracic vascular abnormality findings on gadolinium-enhanced magnetic resonance angiography were similar to those on catheterization, surgical intervention, or both in patients with conotruncal abnormalities (124 vs 127, P = .083), aortic or venous abnormalities (36 vs 33, P = .083), and pulmonary vascular abnormalities (24 vs 25, P = .317). The overall sensitivity of gadolinium-enhanced magnetic resonance angiography for detection of these lesions identified by means of catheterization, surgical intervention, or both was 97.9%. However, gadolinium-enhanced magnetic resonance angiography revealed 11 additional extracardiac thoracic vascular abnormalities that were not found on cardiac catheterization. The kappa coefficient for the correlation of image quality and diagnostic value of gadolinium-enhanced magnetic resonance angiography by using a 4-point-scale (1 = nondiagnostic to 4 = excellent) assessed by 2 independent reviewers was excellent (mean score = 3.66), with superb interobserver agreement (kappa = 0.727-0.874).
Conclusions: Free-breathing, gadolinium-enhanced magnetic resonance angiography is clinically feasible for detailed anatomic delineation and treatment planning of various extracardiac thoracic vascular abnormalities in young children with congenital heart diseases.