A forty-two year old man with cyanotic congenital heart disease returned for clinical follow-up. His cardiac anatomy consisted of right-sided heart with usual atrial arrangement, double outlet right ventricle, and a large ventricular septal defect. The inferior caval vein was interrupted with an azygos continuation to the right superior caval vein. The great vessels both arose from the morphological right ventricle (double outlet right ventricle) with the aorta anterior and to the left of the main pulmonary trunk. There was severe pulmonary stenosis with a hypoplastic pulmonary trunk. The patient had been previously palliated with a Potts shunt from the descending aorta to the left pulmonary artery at seven years of age. At 13 years of age he underwent placement of an interposition graft between the left and right pulmonary arteries after the Potts shunt resulted in discontinuity. Cardiac magnetic resonance imaging confirmed the aforementioned anatomy, in addition to showing the Potts shunt to be widely patent. Axial cine balanced turbo field echo view demonstrated the Potts shunt to be widely patent with marked dilation of the left pulmonary artery (LPA) (Fig. 1a,b). Three-dimensional reconstruction of a gadolinium enhanced magnetic resonance angiogram demonstrated dilation of the left branches with abnormal pruning and tortuosity within the distal left pulmonary artery compared to the normal right pulmonary artery (RPA) (Fig. 2). These studies illustrate the capacity of cardiac magnetic resonance imaging to evaluate specific complications of systemic-pulmonary arterial shunts in adult patients including the development of arteriopathy secondary to pulmonary arterial hypertension.¹

Figure 1.

Figure 2.