Stephens, Elizabeth H.

Background Pediatric cardiac surgery is associated with abnormal coagulation, bleeding, and nearly ubiquitous transfusions. With the popularization of patient blood management, attempts are being made to decrease liberal transfusions by administering prothrombin complex concentrates (PCCs). The safety and efficacy of PCCs in adult cardiac surgery has been studied extensively, but only few reports address this in children. We performed an observational study focused on transfusion requirements after off-label use of activated PCC Factor Eight Inhibitor Bypassing Activity (FEIBA) as an adjunct to post-cardiopulmonary bypass (CPB) hemostatic protocol. Methods We reviewed the medical records of children ≤15 kg undergoing cardiac operations with CPB between May 2018 and March 2022. A propensity score (PS) analysis was performed to identify matched pairs of patients who did and did not receive FEIBA. Results Out of 210 patients who met the inclusion criteria, 44 patients received FEIBA. Propensity score-based analysis identified 40 matched pairs of patients with similar baseline characteristics. There was no statistically significant difference in the primary outcome—the volume of transfusion after CPB, which included all allogeneic blood products and salvaged washed red cells administered after protamine. Specifically, FEIBA patients were transfused 28 (22-34) mL/kg and controls were transfused 22 (11-49) mL/kg, P = .989. Upon arrival to ICU, the FEIBA group averaged an 8% lower international normalized ratio, compared with the controls ( P = .009) and a 1.08 g/dL higher hemoglobin ( P = .050). Neither difference remained significant on POD 1. Conclusions In this exploratory study, we found no change in transfusion requirements after CPB despite FEIBA administration.

Background Left (LAAA) and right atrial appendage aneurysms (RAAA) are rare entities, and their natural history, management, and long-term outcomes are not well studied. Methods This retrospective review includes all patients from 2000 to 2021 with atrial appendage aneurysms at our institution identified using an electronic search tool. LAAA and RAAA were confirmed using multimodality imaging and intraoperative findings. Results We identified 13 (87%) patients with LAAA and 2 (13%) with RAAA. At diagnosis, 11 (73%) were female, mean age was 51.4 ± 18.8 years, and ejection fraction 56.5 ± 13.1%. Three (20%) patients had congenital heart disease including 2 (13%) with atrioventricular septal defects and 1 (7%) with congenitally corrected transposition. LAAA/RAAA was diagnosed due to new-onset atrial fibrillation (AF) in 6 (40%) patients and embolic stroke in 2 (13%). Ten patients had preexisting AF diagnosed 2.9 ± 1.4 years earlier at a mean age of 50.2 ± 15.5 years. Thrombus within the aneurysm was identified in 2 (15%) LAAA patients. All patients were on anticoagulation and follow-up of the cohort from diagnosis was 7.1 ± 6.2 years. Eleven (73%) patients were surgically managed with 7 (64%) lesions excised, 1 (9%) stapled, and 3 (27%) ligated. Postoperative complications occurred in 2 (18%) patients with 1 (7%) developing tricuspid regurgitation and another with pericardial effusion and tamponade. Conclusions Atrial appendage aneurysm is a rare disease and nearly half of patients present with AF. Surgical treatment with concomitant AF ablation is a reasonable and safe management option.

The congenital heart surgeon frequently encounters patients with various genetic disorders requiring surgical intervention. Although the specifics of the genetics for these patients and their families lie in the purview of specialists in genetics, the surgeon is well-served to be familiar with aspects of specific syndromes that impact surgical management and perioperative care. This aids in counseling families in expectations for the hospital course and recovery as well as can impact intraoperative and surgical management. This review article summarizes key characteristics for the congenital heart surgeon to be familiar with for common genetic disorders as they help coordinate care.

Background: Optimal management of cardiac implantable electronic devices (CIEDs) in patients with Ebstein anomaly during tricuspid valve (TV) surgery is unknown. Thus, we aimed to characterize CIED management/outcomes in patients with Ebstein anomaly undergoing TV surgery. Methods: Patients at the Mayo Clinic from 1987 to 2020 with Ebstein anomaly and CIED procedure were reviewed for procedural details, complications, echocardiogram, and lead parameters. Five-year cumulative incidence of CIED complications were estimated using the Kaplan-Meier method. Results: Ninety-three patients were included; 51 were female, and mean age was 40.7±17.5 years. A new CIED was implanted in 45 patients at the time of TV surgery with the majority receiving an epicardial (n=37) CIED. Among 34 patients who had preexisting CIED (11 epicardial, 23 transvenous) at time of TV surgery, 20 had a transvenous right ventricular lead managed by externalizing the lead to the TV (n=15) or extracting the transvenous lead with epicardial lead implantation (n=5). Fourteen patients underwent CIED implantation (4 epicardial, 10 transvenous) without concurrent surgery. Placement of lead across the TV was avoided in 85% of patients. The 5-year cumulative incidence of CIED complications was 24% with no significant difference between epicardial and transvenous CIEDs (26% versus 23%, P =0.96). Performance of lead parameters was similar in epicardial and transvenous leads during median (interquartile range) follow-up of 44.5 (61.1) months. Conclusions: In patients with Ebstein anomaly undergoing TV surgery, the use of epicardial leads and externalization of transvenous leads to the TV can avoid lead placement across the valve leaflets. Lead performance and CIED complications was similar between epicardial and transvenous CIEDs.

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.