A newborn girl with tetralogy of Fallot and coarctation of the aorta underwent 16-channel multidetector-row computed tomography, which demonstrated a right-sided cervical aortic arch, coarctation of the aorta, and aberrant origin of the left subclavian artery (Fig. 1a – anteroposterior view; b – lateral view. RSCA = right subclavian artery; CoA = coarctation of the aorta; RECA = right external carotid artery; RITA = right internal thoracic artery; LCCA = left common carotid artery; LSCA = left subclavian artery). We also noted peculiar, tortuous vessels and repetitive aneurysms and stenoses. Three months later, the aorta was dissected iatrogenically during cardiac catheterization, causing abrupt occlusion of the aortic arch. We carried out an emergency extra-anatomical bypass from the ascending aorta to the descending aorta through a right thoracotomy, using an 8-mm Dacron graft. Computed tomography postoperatively (Fig. 2a – posteroanterior view, b – lateral view. RICA = right internal carotid artery) confirmed the position of the graft, and also visualized the distal portion of the aortic arch. Further catheterization at 10 months revealed severe stenosis (arrows) of the right internal and external carotid arteries, findings confirmed on computed tomography. Patch angioplasty successfully relieved these obstructions. A typical “string of beads” appearance at the time of operation, and histological findings of the arterial tissue, together established the diagnosis of fibromuscular dysplasia. The patient was also known to have Ebstein’s malformation of the tricuspid valve, and this had contributed to gross hypoplasia of the right ventricle, so that a total cavopulmonary connection was needed to replace the previously envisaged biventricular repair, the Fontan circulation being created with a good early outcome.

Figure 1.

Figure 2.

Our patient shows the value of computed tomography in demonstrating coarctation of the aorta and fibromuscular dysplasia, both of which are extremely rare in association with tetralogy of Fallot.Reference Suarez, Kurczynski and Bove¹