Citations

AbstractBackground & AimsSmall duct primary sclerosing cholangitis (PSC) is phenotypically a mild version of large duct PSC, but it is unknown whether these phenotypes share aetiology. We aimed to characterize their relationship by investigating genetic associations in the human leucocyte antigen (HLA) complex, which represent the strongest genetic risk factors in large duct PSC.MethodsFour classical HLA loci (HLA‐A, HLA‐B, HLA‐C and HLA‐DRB1) were genotyped in 87 small duct PSC patients, 485 large duct PSC patients and 1117 controls across three geographical regions.ResultsHLA‐DRB1*13:01 (OR = 2.0, 95% CI 1.2–3.4, P = 0.01) and HLA‐B*08 (OR = 1.6, 95% CI 1.1–2.4, P = 0.02) were significantly associated with small duct PSC compared with healthy controls. Based on the observed frequency of HLA‐B*08 in small duct PSC, the strongest risk factor in large duct PSC, an estimated 32% (95% CI 4–65%) of this population can be hypothesized to represent early stages or mild variants of large duct PSC. This subgroup may be constituted by small duct PSC patients with inflammatory bowel disease (IBD), which greatly resembled large duct PSC in its HLA association. In contrast, small duct PSC without IBD was only associated with HLA‐DRB1*13:01(P = 0.03) and was otherwise distinctly dissimilar from large duct PSC.ConclusionsSmall duct PSC with IBD resembles large duct PSC in its HLA association and may represent early stages or mild variants of large duct disease. Different HLA associations in small duct PSC without IBD could indicate that this subgroup is a different entity. HLA‐DRB1*13:01 may represent a specific risk factor for inflammatory bile duct disease.

ObjectiveTotal knee arthroplasty (TKA) is a widely utilized and effective treatment option for end‐stage knee osteoarthritis (OA). Knee OA is more prevalent among women compared to men, but there are limited data on the sex differences in surgical outcomes after primary TKA.MethodsOur sample consisted of all primary TKAs performed in Pennsylvania during the fiscal year 2002. We used International Classification of Diseases, Ninth Revision, Clinical Modification codes to identify major complications and surgical revision. We used mixed‐effects logistic regression models to examine the associations between sex and all‐cause mortality, readmissions, and major surgical complications. We used proportional hazards models to assess the risk of surgical revision after index arthroplasty. We adjusted for race, age, hospital teaching status, hospital procedure volume, insurance status, and risk of mortality.ResultsIn 17,994 primary TKAs, there were 46 and 220 deaths at 30 days and 1 year, respectively. Compared to women, men had higher adjusted odds of 1‐year mortality (odds ratio [OR] 1.48 [95% confidence interval (95% CI) 1.13–1.94]) after primary TKA. The overall odds of most major 30‐day complications did not differ by sex except for surgical wound infections, which were higher in men compared to women (OR 1.31 [95% CI 1.08–1.60]); 30‐day readmission was higher in men (OR 1.25 [95% CI 1.10–1.43]). Men had significantly higher rates of revision of index knee arthroplasty at 5 years (hazard ratio 1.20 [95% CI 1.05–1.36]) compared to women.ConclusionThe higher rates of mortality, hospital readmissions, revision surgery, and wound infections in men undergoing elective primary TKA compared to women indicate there is a sex disparity in these outcomes.

The Fontan procedure is now considered the final common pathway for patients with anatomical or functional single ventricle. These patients initially have their systemic and pulmonary circulations in parallel, supported by one functional ventricular chamber. The ultimate goal with this procedure is to separate the two circulations, to prevent mixing of venous and arterial blood, and to provide adequate tissue oxygenation. The objective of this article is to review the Fontan procedure with its various modifications and refinements since its introduction to clinical practice in 1971, by Fontan and Baudet.

Pulmonary artery translocation has been described as an alternative surgical strategy to treat anomalous aortic origin of the coronary artery from the wrong sinus of Valsalva. We describe another application of this strategy in a patient who underwent complete repair of pulmonary atresia with ventricular septal defect and presented years later with ischemic symptoms due to compression of the left main coronary artery by the right ventricular-to-pulmonary artery conduit. To our knowledge, this is the first application of the pulmonary artery translocation technique to treat such a problem.

In patients with congenitally corrected transposition of the great arteries, the main concern has been the long-term performance of the morphologic right ventricle in association with tricuspid valve regurgitation when it remains as the systemic ventricle. Deterioration in ventricular function can occur slowly over many years, even without associated cardiac anomalies or previous surgical interventions. This review summarizes the authors' experience and provides a thorough review of the literature addressing the management of the failing systemic right ventricle as well as the tricuspid valve regurgitation with congenitally corrected transposition. This includes different surgical options, the authors' preferred management algorithm, and the late outcome.

Background Electron-beam CT (EBCT) quantification of coronary artery calcification (CAC) allows noninvasive assessment of coronary atherosclerosis. We undertook a follow-up study to determine whether CAC extent, measured at the time of angiography by EBCT, predicted future hard cardiac events, comprising cardiac death and nonfatal myocardial infarction (MI). We also assessed the potential of selected coronary artery disease (CAD) risk factors, prior CAD event history (MI or revascularization), and angiographic findings (number of diseased vessels and overall disease burden) to predict subsequent hard events. Methods and Results Two hundred eighty-eight patients who underwent contemporaneous coronary angiography and EBCT scanning were contacted after a mean of 6.9 years. Vital status and history of MI during follow-up were determined. Cox proportional hazards models were used to compare the predictive ability of CAC extent with selected CAD risk factors, CAD event history, and angiographic findings. Median CAC score was 160 (range 0 to 7633). The 22 patients who experienced hard events during follow-up were older and had more extensive CAC and angiographic disease ( P <0.05). Only 1 of 87 patients with CAC score <20 experienced a subsequent hard event during follow-up. Event-free survival was significantly higher for patients with CAC scores <100 than for those with scores ≥100 (relative risk 3.20; 95% CI 1.17 to 8.71). When a stepwise multivariable model was used, only age and CAC extent predicted hard events (risk ratios 1.72 and 1.88, respectively; P <0.05). Conclusions In patients undergoing angiography, CAC extent on EBCT is highly predictive of future hard cardiac events and adds valuable prognostic information.