Gadolinium-enhanced three-dimensional magnetic resonance angiographic assessment of the pulmonary artery anatomy in cyanotic congenital heart disease with pulmonary stenosis or atresia: comparison with cineangiography.

Pulmonary stenosis (PS) or pulmonary atresia (PA) is an important component of complex cyanotic congenital heart disease, especially in tetralogy of Fallot or lesions with ventricular septal defect (VSD)-PS physiology. Management strategy in these patients depends on accurate assessment of PAs and identification of all sources of pulmonary blood flow. X-ray cineangiography is the "gold standard" for this purpose, but it has the inherent risks of an invasive procedure. Gadolinium-enhanced three-dimensional magnetic resonance angiography (Gd-MRA) has been shown to noninvasively and accurately evaluate various lesions of the vascular system. This study was undertaken to evaluate the accuracy of Gd-MRA compared with cineangiography in the evaluation of pulmonary anatomy. Nineteen patients having complex cyanotic heart disease with PS or PA were included in the study. All patients underwent Gd-MRA and cineangiography. Catheterisation and MRA findings regarding the anatomic variable of interest were analysed for agreement by Bland-Altman analysis. There was total agreement between the two modalities in the delineation of confluent PAs. McGoon's ratio and the Nakata index, which are standard measures of the adequacy of PA size, also showed excellent agreement between the two modalities. MRA was able to delineate all aorto-pulmonary collaterals in the setting of PA. MRA can delineate all sources of pulmonary blood supply in cyanotic congenital heart disease with PS and/or PA as well as provide accurate assessment of PA size for planning corrective surgery.