Dearani, Joseph A.

Background Patients with heterotaxy syndromes (right and left atrial isomerism) are at high risk of poor outcomes after single-ventricle palliation. However, the long-term outcomes and specific parameters associated with poor outcomes are incompletely understood. Methods A retrospective review was performed of all patients with atrial isomerism who had a Fontan at our institution from 1973 to 2020. Standard demographic, as well as pre-, peri-, and postoperative parameters were collected. Features and outcomes of patients with polysplenia were compared to asplenia. Outcomes were analyzed for effect during 4 eras: (1: 1973-1984; n = 27), (2: 1985-1994; n = 93), (3: 1995-2004; n = 28), and (4: 2005-2020; n = 10). Results Of the 1176 patients who had a Fontan operation, 158 (14%) had a heterotaxy syndrome. The median age at the time of Fontan was 8 (9) years. Early mortality was 20% and was greater in patients with asplenia compared to polysplenia (27% vs. 12%, p = .019). But it substantially improved over time (61% in era 1 vs. 7%-10% in the more recent eras ( p < .001)), as did transplant-free survival (22% at 10 years in era 1 vs. 88% in era 4, p < .001). Transplant-free survival was significantly lower in patients with asplenia versus those with polysplenia ( p = .014), and patients with heterotaxy had lower survival than nonheterotaxy ( p = .01). This was largely due to the asplenia group ( p < .001) (hazard ratio = 3.05, p = .007). Conclusions After Fontan operation, patients with heterotaxy, particularly asplenia, continue to demonstrate worse transplant-free survival than nonheterotaxy patients. Early mortality and long-term transplant-free survival have improved in more recent eras.

AbstractObjectivesTo evaluate the outcomes of transcatheter coronary artery fistula (CAF) closure and to identify anatomic/procedural factors that may impact outcomes.BackgroundDue to the rarity of CAF, reported experience with transcatheter closure remains limited and anatomic and procedural factors that may lead to unsuccessful closure, complications, or recanalization of CAF are unclear.MethodsAll patients who underwent transcatheter CAF closure at Mayo Clinic from 1997 to 2018 were retrospectively reviewed. CAF anatomic characteristics, procedural techniques, and clinical/angiographic outcomes were assessed.ResultsA total of 45 patients underwent transcatheter closure of 56 CAFs. The most commonly used devices were embolization coils in 40 (71.4%) CAFs, vascular occluders in 10 (17.8%), or covered stent in 2 (3.6%). Acute procedural success with no or trivial residual flow occurred in 50 (89.3%) CAFs. Residual flow was small in three (5.4%) and large in three (5.4%). Eight (17.8%) patients had complications, including device migration in three, intracranial hemorrhage from anticoagulation in one, and myocardial infarction (MI) in four. MI was a result of covered stent thrombosis or stagnation of flow after closure of large distal CAF. Twenty‐two patients with 27 CAFs had follow‐up angiography after successful index procedure at median time of 423 (IQ 97‐1348) days. Of these, 23 (85.2%) had no/trace flow and 4 had large flow from recanalization.ConclusionsTranscatheter CAF closure is associated with a favorable acute procedural success and complication rate in selected patients. Procedural success and risk for complication are highly dependent on CAF anatomy and closure technique.