Khan, Arooj R.

Background Aortic dilation and aortic valve disease are known long-term complication of tetralogy of Fallot (TOF), but the risk of aortic dissection and the indications for prophylactic aortic surgery are unknown in this population. The purpose of this study was to: (1) determine the prevalence of significant aortic valve disease and/or significant aortic aneurysm (AVD-AA); (2) determine the incidence of progressive aortic dilation and aortic dissection in patients with TOF. Methods Retrospective review of adults with repaired TOF, and no prior aortic valve/aorta surgery, who had ≥2 measurements of the thoracic aorta >12 months apart, 1990–2017. The aortic root and mid-ascending aorta were measured at the onset of QRS complex from leading edge to leading edge. Significant aortic valve disease was defined as the presence of ≥moderate aortic stenosis and/or ≥moderate aortic regurgitation. Significant aortic aneurysm was defined as aortic root or mid-ascending aorta dimension ≥50 mm. Progressive aortic dilation was defined as increase in aortic dimension ≥2 mm. Results Of the 453 consecutive patients (37±13 years, men 216 (49%)) in the study, aortic aneurysm was present in 312 (69%) based on normative data; progressive aortic dilation occurred in 40 (9%), and there was no case of aortic dissection. Significant AVD-AA occurred in 52 (12%) patients; and 15 of them (29%) underwent aortic surgery without any surgical mortality. Conclusions Although aortic aneurysm was common, progressive aortic dilation was uncommon and aortic dissection did not occur in our patients with TOF with significant aortic aneurysms who did not undergo aortic surgery. This has important clinical implication in deciding the frequency of imaging follow-up and timing of surgical intervention in this population.

Objective To determine the prevalence and outcomes of intracranial aneurysm (IA) in patients with bicuspid aortic valve (BAV). Methods Retrospective review of patients with BAV who underwent brain MR angiography at the Mayo Clinic from 1994 to 2013. Results There were 678 patients included in this study—mean age 57±13 years, men 480 (71%), mean follow-up 10±3 years (5913 patient-years). Coarctation of aorta (COA) was present in 154 (23%) patients. There were 59 IAs identified in 52 of 678 patients (7.7%). IA was present in 20/154 patients (12.9%) with COA and 32/524 patients (5.7%) without COA (p<0.001). For the patients without COA, female gender and right–left cusp fusion were risks factors for IA in women after adjustment for all potential variables (HR 1.76, CI 1.31 to 2.68, p=0.03). There was no significant trend in the risk for IA across age tertiles: age ≤40 years versus 41–60 years (HR 1.19, p=0.34), and age 41–60 years versus 61–80 years (HR 1.06, p=0.56). Among the 52 patients with IA, enlargement occurred in three patients (6%), rupture in two patients (4%) and four patients (8%) underwent coil embolisation. For the 626 patients without IA at baseline, no patient developed IA over 7±2 years of imaging follow-up. Conclusions BAV is associated with a higher prevalence of IA compared to the general population, and this risk is higher in patients with COA, right–left cusp fusion and female gender.

Objective Limited data exist regarding the outcomes of non-cardiac procedures (NCPs) in adult patients after Fontan operations (Fontan patients). Methods To compare procedural outcomes after NCPs in Fontan patients with outcomes for two matched control groups: patients with repaired congenital heart disease and biventricular circulation (CHD-BiV) and patients with no heart disease (NHD). We defined cyanosis as oxygen saturation <90% and procedural hypoxia as saturation <80% or a decrease in saturation >10% from baseline. Results There were 538 NCPs in 154 Fontan patients (mean age, 30 years) performed in 1990–2015. Sedation and anaesthesia types were monitored anaesthesia care (256,48%), general anaesthesia (51,9%), minimal sedation (105,20%), local anaesthesia (75,14%) and regional anaesthesia (51,9%). Ninety-three complications occurred in 79 procedures (15%) and included arrhythmia requiring intervention (9), hypotension (14), bradycardia (8), hypoxia (38), heart failure requiring intravenous diuretics (2), acute kidney injury (3), bleeding requiring blood transfusion (1), unplanned procedures for dialysis catheter placement (2), readmission (2), unplanned hospitalisation for hypoxia (8) and unplanned transfer to intensive care unit (1). Baseline cyanosis was the only multivariable risk factor for complications (HR, 1.87 (95% CI 1.14 to 3.67), p=0.04). Procedural complications were more common in the Fontan group (18%) than in the CHD-BiV (5%) and NHD groups (1.4%) (p=0.001). Conclusions Complications after NCPs were more common in Fontan patients, and baseline cyanosis was a risk factor for complications. All-cause mortality was low and may be related to the multidisciplinary care approach used for Fontan patients at our centre.