Intraoperative aortic dissection in a Turner syndrome patient

Intraoperative aortic dissection in a Turner syndrome patient Aortic dissection is a rare but catastrophic complication of cardiac surgery with a mortality ranging from 24-43% (1). Turner syndrome (TS) patients are known to be at an increased risk of undergoing an insidious aortic dissec-tion, however, little is known about their risk of undergoing iatrogenic dissection. In addition to airway management complications [due to retrognathia, micrognathia, limited neck mobility and short trachea (2)], these patients may also be a high risk population for experiencing an intraoperative aortic dissection particularly during cardiopulmonary bypass (CPB). We experienced an ascending aortic dissection in a 64 year old TS patient with coronary artery disease who was admitted for elective coronary artery bypass grafting. This was noted after aortic unclamping (Figure 1), following successful weaning from CPB, and subsequently required reinstitution of CPB in order to repair the dissected proximal aorta with a prosthetic tube graft. The dissection area was limited and did not require repositioning of aortic can-nulation. The patient was then again weaned from CPB with a 3 µg/min infusion of both epi-nephrine and norephinephrine (0.028 µg/kg/ min of each). Strict blood pressure control was maintained throughout the surgery. The distal ascending aorta was cannulated opposite the in-nominate artery takeoff , as per the surgeon's routine, and hypothermia to 32°F was utilized during the aortic repair. To our knowledge this is the first report of a dissection occurring intra-operatively in a TS patient despite their known higher risk. TS occurs in about 1 in 2,500 females and aortic dissection is 6 times more likely in this population compared to the incidence of dissection in the general population (male and female) of 2.6-3.5 per 100,000 person-years (3). Because of this, echocardiography during diagnosis has become a standard of care screening for these patients and it is recommended that they undergo routine thoracic magnetic resonance imaging (MRI) at 10 years of age (4). Presumably the risk is increased in TS due to the connective tissue pathogenesis and hypertension attributed to the syndrome. This patient had hypertension as well as diabetes mellitus type II and hyper-cholesterolemia, all associated with TS. She also had a history of congestive heart failure secondary to diastolic dysfunction and sleep apnea. She was diagnosed at childhood and did not receive growth hormone therapy or undergo any prior aortic imaging with the exception of cardiac catheterization which noted no significant aortic dilatation. She …