A case of descending aortic dissection in a patient with unrepaired tetralogy of Fallot and pulmonary atresia

Abstract

The prognosis of tetralogy of Fallot with pulmonary atresia (TOF/PA) is mainly determined by the development of major aorto-pulmonary collateral arteries (MAPCAs) that provide pulmonary blood perfusion. TOF/PA can be managed conservatively until adulthood in patients with adequate, but not excessive perfusion via MAPCAs. To the best of our knowledge, this is the first report of a patient with unrepaired TOF/PA who eventually developed descending aortic dissection (AD), and survived with medical treatment. A 46-year-old woman was referred to our hospital by her local cardiologist with exertional dyspnea. A three-dimensional (3-D) computed tomography (CT) performed prior to presentation showed a dilated thoracic aorta, three well-developed MAPCAs, and a patent ductus arteriosus (PDA), whereas the 3-D CT performed at presentation revealed a descending AD with the entry site at the proximal part of the thoracic descending aorta, and neither the MAPCAs nor the PDA originated from the area of the AD. The patient was treated medically and was discharged thereafter. In this case, 3D-CT taken 9 months prior to the dissection showed no involvement of MAPCAs in the dissection area and was useful to make a decision of conservative therapy. Learning objectiveFew systematic studies have addressed patients with tetralogy of Fallot with pulmonary atresia (TOF/PA) who survived more than 20 years due to optimal control of pulmonary blood flow depending on the development of major aorto-pulmonary collateral arteries (MAPCAs). We report a patient with unrepaired TOF/PA who developed descending aortic dissection (AD) in her forties. Three-dimensional computed tomography was useful for diagnosing and choosing a treatment plan by identifying the involvement of MAPCAs within the region of the AD.