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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.

The treatment landscape of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) has significantly evolved in recent years. Targeted therapy with Bruton’s tyrosine kinase (BTK) inhibitors and BCL-2 inhibitors has emerged as an effective chemotherapy-free option for patients with previously untreated or relapsed/refractory CLL/SLL. Undetectable minimal residual disease after the end of treatment is emerging as an important predictor of progression-free and overall survival for patients treated with fixed-duration BCL-2 inhibitor-based treatment. These NCCN Guidelines Insights discuss the updates to the NCCN Guidelines for CLL/SLL specific to the use of chemotherapy-free treatment options for patients with treatment-naïve and relapsed/refractory disease.

Background:Research on the utilization and effectiveness of antitumor necrosis factor (TNF) biologics in children with very early onset inflammatory bowel disease (VEOIBD) is urgently needed. Here we describe anti‐TNF use and durability in a multicenter cohort.Methods:We performed a retrospective cohort study of patients diagnosed with VEOIBD (<6 years) between 2008 and 2013 at 25 North American centers. We performed chart abstraction at diagnosis and 1, 3, and 5 years after diagnosis. We examined the rate of initiation and durability of infliximab and adalimumab and evaluated associations between treatment durability and the following covariates with multivariate Cox proportional hazard regression: age at diagnosis, sex, disease duration, disease classification, and presence of combined immunomodulatory treatment versus monotherapy.Results:Of 294 children with VEOIBD, 120 initiated treatment with anti‐TNF therapy and 101 had follow‐up data recorded [50% Crohn disease (CD), 31% ulcerative colitis (UC), and 19% IBD unclassified (IBD‐U)]. The cumulative probability of anti‐TNF treatment was 15% at 1 year, 30% at 3 years, and 45% at 5 years from diagnosis; 56 (55%) were treated between 0 and 6 years old. Anti‐TNF durability was 90% at 1 year, 75% at 3 years, and 55% at 5 years. The most common reason for discontinuation of anti‐TNF were loss of response in 24 (57%) children. Children with UC/IBD‐U had lower durability than those with CD (hazard ratio [HR] 0.17; 95% confidence interval [CI], 0.06–0.51; P = 0.001).Conclusions:Utilization and durability of anti‐TNF in VEOIBD is relatively high and comparable with older children. Having Crohn disease (compared with UC/IBD‐U) is associated with greater durability.

Study Design: This was an observational study. Objectives: This study aims to determine the correlation between patient-reported outcomes (PROs) pulled from a national spine registry and physician ratings from physician review websites (PRWs). Summary of Background Data: PRWs are frequently utilized by patients to make health care decisions; however, many PRWs appear to incorporate subjective experiences unrelated to a surgeon’s clinical performance into ratings. As such, their utility as a health care decision-making tool remains unclear. Materials and Methods: This study evaluated 8834 patients from the Quality Outcomes Database (QOD) who underwent 1-level elective lumbar spine surgery. The lumbar module of QOD was queried to rank 124 surgeons using PROs (Oswestry Disability Index, EuroQOL, Numerical Rating Scale—back/leg pain, and patient satisfaction). The QOD PRO-ranking system was compared against PRWs including Healthgrades, Vitals, WebMD, and Google. The Spearman correlation coefficients, Kruskal-Wallis tests, and multiple linear regression models were used for statistical comparison. The primary outcome was the correlation between PRW scores and PROs. Results: Surgeon PRO-derived ranking showed high intercorrelational congruence with coefficients between the 3 PROs (Oswestry Disability Index, EuroQOL, Numerical Rating Scale back/leg) ranging between 0.70 and 0.88. Low correlations were observed between PRO-derived rankings and PRWs, ranging from 0.23 to 0.37. Healthgrades performed most similarly to PRO-derived rankings, correlating best with patient satisfaction, though the correlation was low (ρ=0.37). Conclusions: While PRWs are often used to evaluate surgeon competency, these results demonstrate they poorly correlate with a surgeon’s clinical ability measured by PROs. PRWs should be used with caution when making health care decisions by patients, payers, and administrators. Level of Evidence: Level III.

Understanding the morphology of atrioventricular septal defects and learning the operative strategies for their repair is one of the more difficult tasks for congenital cardiac surgery residents to master. The modified single-patch technique for several reasons lends itself to being a strategy that is relatively easy to teach residents. It has shorter cross-clamp and bypass times than the standard two-patch technique, which allows more time for the instructor to safely take the resident through the case. The steps of the operation are quite standard and broadly applicable to the wide variety of cardiac morphologies found in children with atrioventricular septal defects. The purpose of this review is to carefully point out the technical details of each step of the modified single-patch technique focusing on teaching the resident surgeon. The ease of teaching this excellent technique is just another reason to use the modified single-patch technique.