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LONG-TERM RESULTS OF THE BIRMINGHAM HIP RESURFACING IMPLANT IN THE UNITED STATES: AN UPDATED ANALYSIS OF A SINGLE INSTITUTION'S EXPERIENCE
Zuke, William A. , Kromka, Joseph , Clohisy, John C. , Barrack, Robert L. , Hannon, Charles P. , Granger, C.

Orthopaedic Proceedings | August 19, 2024 | Volume: 106-B | Issue: SUPP_16 | Pages: 39-39

10.1302/1358-992x.2024.16.039 Get It

We previously reported the five to ten-year results of the Birmingham Hip Resurfacing (BHR) implant. The purpose of this study was to evaluate the survivorship, radiographic results, and clinical outcomes of the BHR at long-term follow-up.We retrospectively reviewed 250 patients from the original cohort of 324 BHRs performed from 2006 to 2013 who met contemporary BHR indications. Of these, 4 patients died and 4 withdrew. From the 242 patients, 224 patients (93%) were available for analysis. Modified Harris hip score (mHHS) and University of California, Los Angeles (UCLA) scores were collected and compared to a matched total hip arthroplasty (THA) cohort. Mean follow-up was 14 years.Survivorship free of aseptic revision was 97.4% and survivorship free of any revision was 96.0% at 15 years. Revisions included 3 periprosthetic joint infections, 2 for elevated metal ions and symptomatic pseudotumor, 2 for aseptic femoral loosening, and 1 for unexplained pain. The mean mHHS was 93 in BHR patients at final follow-up, similar to the THA cohort (p=0.44). The UCLA score was significantly higher for BHR patients (p=0.02), however there were equal proportions of patients who remained highly active (UCLA 9 or 10) in both groups, 60.5% and 52.2% (p=0.45) for BHR and THA respectively. Metal ion levels at long term follow-up were low (mean serum cobalt 1.8±1.5 ppb and mean serum chromium 2.2±2.0 ppb).BHR demonstrated excellent survivorship in males less than 60 years of age at time of surgery. Clinical outcomes and activity levels were similar to THA patients. Failures related to the metal-on-metal bearing were rare and metal levels were low at long-term follow-up.Level of evidence: IIIKeywords: survivorship; hip arthroplasty; activity; metal-on-metalSurface Replacement Arthroplasty demonstrates low revision rates and similar activity level compared to total hip arthroplasty at long-term follow-up.
Benefit of isolated surgical valve repair or replacement for functional tricuspid regurgitation and long-term outcomes stratified by the TRI-SCORE
Dreyfus, Julien , Juarez-Casso, Fernando M. , Eixerés‐Esteve, Andrea , Bohbot, Yohann, Bazire, Baptiste , Flagiello, Michele , Riant, Elisabeth , Mbaki, Yannick, Tomasi, Jacques , Sénage, Thomas , Eyharts, Damien , Doguet, Fabien , Viau, F , Eggenspieler, Florian , Heuts, Samuel , Galloo, Xavier , Benfari, Giovanni , Badano, Luigi P. , Enriquez-Sarano, Maurice, Bartko, Philipp E. , Lim, Pascal , Radu, Costin , Bernick, Jordan , Wells, George A. , Tribouilloy, Christophe , Iung, Bernard , Bonis, Michele De , Juarez-Casso, Fernando, Bazire, Baptiste , Flagiello, Michele , Riant, Elisabeth , Tomasi, Jacques , Senage, Thomas , Eyharts, Damien , Doguet, Fabien , Viau, Florence , Heuts, Samuel , Sardari Nia, Peyman , Heitzinger, Gregor , Marsan, Nina Ajmone , Maisano, Francesco , Topilsky, Yan , Michelena, Hector, Selton-Suty, Christine , Habib, Gilbert , Lavie-Badie, Yoan , Modine, Thomas , Chan, Vincent , Donal, Erwan , Wells, George , Tribouilloy, Christophe , De Bonis, Michele , Messika-Zeitoun, David , Enriquez-Sarano, Maurice , Benfari, Giovanni , Sala, Alessandra , Carnero, Manuel , Rahmouni, Kenza , Coisne, Augustin , Heitzinger, Gregor , Marsan, Nina Ajmone , Muraru, Denisa , Maisano, Francesco , Topilsky, Yan , Michelena, Héctor I. , Enriquez‐Sarano, Maurice , Bax, Jeroen J. , Selton‐Suty, Christine , Habib, Gilbert , Modine, Thomas , Chan, Vincent , Tourneau, Thierry Le , Donal, Erwan , Obadia, Jean‐François , Crestanello, Juan A. , Messika–Zeitoun, David

European Heart Journal | August 19, 2024 | Volume: 45 | Issue: 42 | Pages: 4512-4522

10.1093/eurheartj/ehae578 | PMID 39212387 Get It

Abstract Background and Aims Severe tricuspid regurgitation is associated with increased mortality rates, but benefit of its correction and ideal timing are not clearly determined. This study aimed to identify patient subsets who might benefit from the surgery. Methods In TRIGISTRY, an international cohort study of consecutive patients with severe isolated functional tricuspid regurgitation (33 centres, 10 countries), survival rates up to 10 years were compared between patients who underwent isolated tricuspid valve surgery (repair or replacement) and those conservatively managed, overall and according to TRI-SCORE category (low: ≤3, intermediate: 4–5, and high: ≥6). Results One thousand and two hundred seventeen were managed conservatively, and 551 underwent isolated tricuspid valve surgery (200 repairs and 351 replacements). TRI-SCORE distribution was 33% low, 32% intermediate, and 35% high. At 10 years, survival rates were similar between surgical and conservative management [41% vs. 36%; hazard ratio (HR) .97; 95% confidence interval (CI) .88–1.08, P = .57]. Surgery improved survival compared with conservative management in the low TRI-SCORE category (72% vs. 44%; HR .27; 95% CI .20–.37, P < .0001), but not in the intermediate (36% vs. 37%; HR 1.17; 95%CI .98–1.40, P = .09) or high categories (20% vs. 24%; HR 1.06; 95% CI .91–1.25, P = .45). Both repair and replacement improved survival in the low TRI-SCORE category (84% and 61% vs. 44%; HR .11; 95% CI .06–.19, P < .0001, and HR .65; 95% CI .47–.90, P = .009). Repair showed benefit in the intermediate category (59% vs. 37%; HR .49; 95% CI .35–.68, P < .0001) while replacement was possibly harmful (25% vs. 37%; HR 1.43; 95% CI 1.18–1.72, P = .0002). Conclusions Higher survival rates were observed with repair than replacement and benefit of intervention declined as TRI-SCORE increased with no benefit of any type of surgery in the high TRI-SCORE category. These results emphasize the importance of timely intervention and patient selection to achieve the best outcomes and the need for randomized controlled trials.
Poster 360: Long-Leg Lateral Monopodal Stress Radiographs for the Assessment of Anterior Tibial Translation with Anterior Cruciate Ligament Injury: A Prospective Study
Otremba, Jace R., Tollefson, Luke V. , Knowlan, Claire J., Kennedy, Nicholas I. , LaPrade, Robert F.

Orthopaedic Journal of Sports Medicine | August 15, 2024 | Volume: 12 | Issue: 7_suppl2 | Pages: No page available

10.1177/2325967124s00326 Get It

Objectives: The purpose of this study is to use standing anterior cruciate ligament (ACL) stress radiographs to evaluate how posterior tibial slope (PTS) compared to anterior tibial translation (ATT) in an injured knee. A secondary purpose was to determine how posterior tibial slope (PTS) affects ATT side-to-side difference. Our hypothesis was that anterior tibial translation would be higher in the injured knee compared to the contralateral side and posterior tibial slope will have a positive effect on anterior tibial translation. Methods: A retrospective study of ACL stress radiographs was assessed from patients with primary ACL reconstructions with no concomitant ligamentous injury between January 2023 and August 2023. Institutional Review Board approval was obtained. The bilateral radiographs were long-leg lateral “stress views” which was accomplished by monopodal weight-bearing in 20° flexion and are a part of the initial patient workup. Stress radiographs were utilized to measure the anterior tibial translation (ATT) and posterior tibial slope (PTS) for both knees. Differences in ATT between knees and the correlation between PTS and ATT was assessed via paired t-tests and regression analysis. Results: A final cohort of 39 patients were analyzed using preoperative ACL stress radiographs. The results reported a significant regression with positive correlation between posterior tibial slope (PTS) and anterior tibial translation for the injured knee using both anatomic (Figure 1, P<0.001, R 2 =0.302) and mechanical (Figure 2, P=0.038, R 2 =0.111) PTS measurements. Anatomic PTS measurements are better at predicting ATT for the injured knee compared to mechanical PTS measurements. Conclusions: The findings of this study report a significant regression with a positive correlation between posterior tibial slope (PTS) and anterior tibial translation (ATT) of the injured knee, with anatomic PTS measurements being a better predictor of ATT than mechanical PTS measurements. This indicates an important relationship between PTS and ATT with higher PTRS predicting higher ATT.
Paper 16: Long-term Outcomes of Primary Hip Arthroscopy: Multicenter Analysis at Minimum 10-Year Follow-Up with Attention to Labral and Capsular Management
Okoroha, Kelechi R. , Ortiguera, Cedric J. , Levy, Bruce A. , Meta, Fabien , Hevesi, Mario , Boos, Alexander M. , Wang, Allen S. , Lamba, Abhinav , Krych, Aaron J. , Pan, Xuankang , Clark, Sean C.

Orthopaedic Journal of Sports Medicine | August 15, 2024 | Volume: 12 | Issue: 7_suppl2 | Pages: No page available

10.1177/2325967124s00029 Get It

Objectives: Hip arthroscopy is rapidly advancing, with literature increasingly reporting positive outcomes at short- and mid-term follow-up; however, available long-term data remains limited. The purpose of this study is to evaluate long-term outcomes of primary hip arthroscopy at minimum 10-year follow-up at two academic centers by describing patient-reported outcomes (PROs) and determining reoperation, revision, and total hip arthroplasty (THA) rates. Methods: Primary hip arthroscopies performed between January 1988 and April 2013 at 2 academic centers were evaluated for postoperative PROs including Visual Analogue Scale (VAS), Tegner Activity Scale score, Hip Outcome Score Activities of Daily Living and Sport Scale (HOS-ADL/HOS-SS), modified Harris Hip Score (mHHS), Nonarthritic Hip Score (NAHS), 12-item International Hip Outcome Tool (iHOT-12), surgery satisfaction, function, and reoperations. Results: Two-hundred ninety-four primary hip arthroscopies with a mean age of 40±14 years; 66% female; and a mean body mass index (BMI) of 27±6 kg/m 2 were followed for mean 12±3 years (range: 10-24). Labral debridement and repair were performed in 41% and 59% of patients, respectively. All patients underwent interportal capsulotomy and 2% underwent T-capsulotomy. Eleven percent of patients underwent capsular repair. At final follow-up, patients reported mean VAS at rest of 2±2, with use of 3±3, iHOT-12 of 68±27, NAHS of 81±18, mHHS of 79±17, HOS-ADL of 82±19, and HOS-SS of 74±25. Mean surgical satisfaction was 8.4±2.4 (Table 1). Sixty-five hips (23%) underwent THA and 33 (11%) underwent revision arthroscopy (Table 2). Risk factors for THA included older age (p<0.001), higher BMI (p<0.001), lower lateral center-edge angle (p=0.039), larger alpha angle (p=0.001), higher Tönnis grade (p=0.011), labral debridement (p=0.004), and capsular nonrepair (p=0.030). Patients undergoing combined labral and capsular repair demonstrated a THA conversion rate of 3% compared to 31% for patients undergoing combined labral debridement and capsular nonrepair (p=0.006) (Table 3). Labral repair trended towards increased 10-year THA-free survival (84% vs. 77%, p=0.085) while capsular repair demonstrated significantly increased 10-year THA-free survival (97% vs 79%, p=0.032) (Figure 1). Conclusions: At minimum 10-year follow-up, patients undergoing primary hip arthroscopy demonstrated high rates of satisfaction and acceptable outcome scores. In total, 11% of patients underwent revision hip arthroscopic surgery with a THA conversion rate of 23%. Conversion to THA was associated with patient factors including older age and higher Tönnis grade as well as potentially modifiable surgical factors including labral debridement and capsular nonrepair.
KCNQ1 suppression-replacement gene therapy in transgenic rabbits with type 1 long QT syndrome
Bains, Sahej , Giammarino, Lucilla , Nimani, Saranda , Alerni, Nicolò , Tester, David J. , Kim, C.S. John, Christoforou, Nicolas , Louradour, Julien , Beslac, Olgica, Barbieri, Miriam , Matas, Lluis , Hof, Thomas , López, Rubén , Perez‐Feliz, Stefanie , Casalta, L.G. Garcia, Jurgensen, Jacqulyn, Bego, Mariana G. , Keyes, Lisa R., Owens, Jane , Pinkstaff, Jason, Koren, Gideon , Zehender, Manfred, Brunner, Michael, Casoni, Daniela , Brooks, Gabriel , Ackerman, Michael J. , Odening, Katja E. , Brunner, Michael , Praz, Fabien , Haeberlin, Andreas , Ackerman, Michael J , Odening, Katja E , Bains, Sahej , Horváth, András , Parodi, Chiara, Barry, Michael A. , Praz, Fabien

European Heart Journal | August 8, 2024 | Volume: 45 | Issue: 36 | Pages: 3751-3763

10.1093/eurheartj/ehae476 | PMID 39115049 Get It

Abstract Background and Aims Type 1 long QT syndrome (LQT1) is caused by pathogenic variants in the KCNQ1-encoded Kv7.1 potassium channels, which pathologically prolong ventricular action potential duration (APD). Herein, the pathologic phenotype in transgenic LQT1 rabbits is rescued using a novel KCNQ1 suppression-replacement (SupRep) gene therapy. Methods KCNQ1-SupRep gene therapy was developed by combining into a single construct a KCNQ1 shRNA (suppression) and an shRNA-immune KCNQ1 cDNA (replacement), packaged into adeno-associated virus serotype 9, and delivered in vivo via an intra-aortic root injection (1E10 vg/kg). To ascertain the efficacy of SupRep, 12-lead electrocardiograms were assessed in adult LQT1 and wild-type (WT) rabbits and patch-clamp experiments were performed on isolated ventricular cardiomyocytes. Results KCNQ1-SupRep treatment of LQT1 rabbits resulted in significant shortening of the pathologically prolonged QT index (QTi) towards WT levels. Ventricular cardiomyocytes isolated from treated LQT1 rabbits demonstrated pronounced shortening of APD compared to LQT1 controls, leading to levels similar to WT (LQT1-UT vs. LQT1-SupRep, P &lt; .0001, LQT1-SupRep vs. WT, P = ns). Under β-adrenergic stimulation with isoproterenol, SupRep-treated rabbits demonstrated a WT-like physiological QTi and APD90 behaviour. Conclusions This study provides the first animal-model, proof-of-concept gene therapy for correction of LQT1. In LQT1 rabbits, treatment with KCNQ1-SupRep gene therapy normalized the clinical QTi and cellular APD90 to near WT levels both at baseline and after isoproterenol. If similar QT/APD correction can be achieved with intravenous administration of KCNQ1-SupRep gene therapy in LQT1 rabbits, these encouraging data should compel continued development of this gene therapy for patients with LQT1.
Long‐term safety and efficacy of filgotinib for the treatment of moderately to severely active ulcerative colitis: Interim analysis from up to 4 years of follow‐up in the SELECTION open‐label long‐term extension study
Feagan, Brian G., Matsuoka, Katsuyoshi , Rogler, Gerhard , Laharie, David , Vermeire, Séverine , Loftus, Edward V. , Beales, Ian , Schreiber, Stefan , Kim, Hyo Jong , Faes, Margaux , Haas, Angela de, Masior, Tomasz , Rudolph, Christine , Danese, Silvio , Loftus, Edward V , Beales, Ian , Schreiber, Stefan , Peyrin-Biroulet, Laurent , Vermeire, Séverine , Danese, Silvio , Peyrin‐Biroulet, Laurent , Matsuoka, K

Alimentary Pharmacology & Therapeutics | August 1, 2024 | Volume: 60 | Issue: 5 | Pages: 563-584

10.1111/apt.18158 | PMID 39086130 Get It

SummaryBackgroundFilgotinib, an oral, once‐daily, Janus kinase 1 preferential inhibitor, is an approved treatment for moderately to severely active ulcerative colitis.AimsThe aim of this study is to assess the safety and efficacy of continued filgotinib therapy over ~4 years in the long‐term extension of the phase 2b/3 SELECTION trial (SELECTIONLTE; NCT02914535).MethodsIn this interim analysis of SELECTIONLTE, SELECTION completers (week 10 responders to filgotinib who completed the maintenance study) continued their assigned treatment (double‐blind filgotinib 200 mg [FIL200] or filgotinib 100 mg) and SELECTION week 10 non‐responders received open‐label FIL200. We assessed safety by adverse events (AEs), and efficacy by partial Mayo Clinic Score (pMCS), inflammatory biomarkers and health‐related quality of life (HRQoL). We compared safety and efficacy between achievers and non‐achievers of a multi‐component endpoint, comprehensive disease control (CDC), comprising symptomatic, endoscopic, inflammatory biomarker and HRQoL improvements.ResultsData for completers (n = 250) and non‐responders (n = 372) were reported for ≤202 weeks. AE occurrences were low and consistent with previous analyses. The as‐observed proportion of FIL200‐treated patients in pMCS, biomarker and HRQoL remission during SELECTIONLTE remained high among completers (week 144: 80.0%, 86.4% and 86.0%, respectively) and increased among non‐responders (week 192: 62.1%, 76.7% and 59.3%, respectively). Significantly higher proportions of CDC achievers at SELECTION week 58 achieved pMCS, IBDQ and corticosteroid‐free pMCS remission than non‐achievers, up to LTE week 96.ConclusionsFilgotinib induced and maintained symptomatic remission and improved HRQoL over 4 years. Safety results showed a proven long‐term benefit–risk profile. FIL200‐treated CDC achievers had better long‐term outcomes than non‐achievers.
Incidence and Long-Term Outcomes of Biliary Tract Cancers in Olmsted County, Minnesota from 1976 to 2018
Nwankwo, Eugene , Guta, Amerti, Cao, Scarlett , Yang, Ju Dong , Abdalla, Abubaker , Taha, Wesam, Larson, Joseph J. , Yin, Jun , Gores, Gregory J. , Roberts, Lewis R. , Gores, Gregory J. , Nwankwo, Eugene C, Cao, Scarlett S , Roberts, Lewis R , Yang, Ju Dong , Cleary, Sean P.

Cancers | July 31, 2024 | Volume: 16 | Issue: 15 | Pages: 2720

10.3390/cancers16152720 | PMID 39123448 Get It

Biliary tract cancers, including cholangiocarcinoma, gallbladder, and ampulla of Vater cancers, rank second among hepatobiliary cancers, known for their poor prognoses. The United States has witnessed a notable increase in intrahepatic cholangiocarcinoma incidence. This study examines the incidence and survival outcomes of biliary tract cancers in Olmsted County, Minnesota from 1976 to 2018. Using data from the Rochester Epidemiology Project (REP), residents aged 20 and above were analyzed across four eras. Incidence rates were calculated and adjusted for age and sex, and temporal trends were assessed using Poisson regression. Intrahepatic cholangiocarcinoma exhibited a significant escalation in incidence rates over time, while gallbladder cancers showed a decline among women. Median survival times for biliary tract cancers notably improved. These findings confirm the rising incidence of intrahepatic cholangiocarcinoma and suggest improving survival rates. Nevertheless, the overall prognosis for biliary tract cancers remains very poor, emphasizing the continual need for enhanced management strategies and further research.
Vigorous Exercise in Patients With Congenital Long QT Syndrome: Results of the Prospective, Observational, Multinational LIVE-LQTS Study
Lampert, Rachel , Day, Sharlene M. , Ainsworth, Barbara E. , Burg, Matthew M. , Marino, Bradley S. , Salberg, Lisa , Tome, Maite , Abrams, Dominic J. , Aziz, Peter F. , Barth, Cheryl , Behr, Elijah R. , Bell, Cheyanne , Berul, Charles I. , Bos, J. Martijn , Cannom, David S. , Cannon, Bryan C. , Concannon, Maryann, Czosek, Richard J. , Dubin, Anne M. , Dziura, James , Erickson, Christopher C. , Etheridge, Susan P. , Gray, Belinda , Haglund-Turnquist, Carla , Johnsrude, Christopher L. , Lara, Alice, Saarel, Elizabeth V. , Sanatani, Shubhayan , Shah, Maully J. , Simone, Laura, Tomaselli, Gordon F., Ware, James S. , Zaręba, Wojciech , Ackerman, Michael J. , Day, Sharlene , Burg, Matthew , Tome Esteban, Maria Teresa , Bos, Johan M , Cannon, Bryan C , Cerrone, Marina , Czosek, Richard J , Dubin, Anne M , Dziura, James , Erickson, Christopher C , Estes, N.A. Mark , Etheridge, Susan P , Goldenberg, Ilan , Gray, Belinda , Haglund-Turnquist, Carla M , Harmon, Kimberly G , James, Cynthia A , Johnsrude, Christopher , Kannankeril, Prince J , Law, Ian H , Link, Mark S. , Molossi, Silvana M , Olshansky, Brian , Noseworthy, Peter A , Saarel, Elizabeth V , Sanatani, Shubhayan , Shah, Maully , Skinner, Jonathan R , Ware, James S , Webster, Gregory , Zareba, Wojciech , Zipes, Douglas P , Ackerman, Michael J , Behr, Elijah Raphael , Abrams, Dominic J , Bradley, David J. , Cerrone, Marina , Goldenberg, Ilan , Harmon, Kimberly G. , James, Cynthia A. , Kannankeril, Prince J. , Law, Ian H. , Li, Fangyong , Olshansky, Brian , Noseworthy, Peter A. , Skinner, Jonathan R. , Webster, Gregory , Zipes, Douglas P.

Circulation | July 25, 2024 | Volume: 150 | Issue: 7 | Pages: 516-530

10.1161/circulationaha.123.067590 | PMID 39051104 Get It

BACKGROUND: Whether vigorous exercise increases risk of ventricular arrhythmias for individuals diagnosed and treated for congenital long QT syndrome (LQTS) remains unknown. METHODS: The National Institutes of Health–funded LIVE-LQTS study (Lifestyle and Exercise in the Long QT Syndrome) prospectively enrolled individuals 8 to 60 years of age with phenotypic and/or genotypic LQTS from 37 sites in 5 countries from May 2015 to February 2019. Participants (or parents) answered physical activity and clinical events surveys every 6 months for 3 years with follow-up completed in February 2022. Vigorous exercise was defined as ≥6 metabolic equivalents for >60 hours per year. A blinded Clinical Events Committee adjudicated the composite end point of sudden death, sudden cardiac arrest, ventricular arrhythmia treated by an implantable cardioverter defibrillator, and likely arrhythmic syncope. A National Death Index search ascertained vital status for those with incomplete follow-up. A noninferiority hypothesis (boundary of 1.5) between vigorous exercisers and others was tested with multivariable Cox regression analysis. RESULTS: Among the 1413 participants (13% <18 years of age, 35% 18–25 years of age, 67% female, 25% with implantable cardioverter defibrillators, 90% genotype positive, 49% with LQT1, 91% were treated with beta-blockers, left cardiac sympathetic denervation, and/or implantable cardioverter defibrillator), 52% participated in vigorous exercise (55% of these competitively). Thirty-seven individuals experienced the composite end point (including one sudden cardiac arrest and one sudden death in the nonvigorous group, one sudden cardiac arrest in the vigorous group) with overall event rates at 3 years of 2.6% in the vigorous and 2.7% in the nonvigorous exercise groups. The unadjusted hazard ratio for experience of events for the vigorous group compared with the nonvigorous group was 0.97 (90% CI, 0.57–1.67), with an adjusted hazard ratio of 1.17 (90% CI, 0.67–2.04). The upper 95% one-sided confidence level extended beyond the 1.5 boundary. Neither vigorous or nonvigorous exercise was found to be superior in any group or subgroup. CONCLUSIONS: Among individuals diagnosed with phenotypic and/or genotypic LQTS who were risk assessed and treated in experienced centers, LQTS-associated cardiac event rates were low and similar between those exercising vigorously and those not exercising vigorously. Consistent with the low event rate, CIs are wide, and noninferiority was not demonstrated. These data further inform shared decision-making discussions between patient and physician about exercise and competitive sports participation. REGISTRATION: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT02549664.
PA-VSD Without MAPCA(s): Review of Long-Term Outcomes and Reinterventions
Stephens, Elizabeth H. , Dearani, Joseph A. , Miranda, William R. , Dearani, Joseph A. , Anderson, Jason H.

World Journal for Pediatric and Congenital Heart Surgery | July 23, 2024 | Volume: 16 | Issue: 2 | Pages: 232-236

10.1177/21501351241254034 | PMID 39043197 Get It

Background Pulmonary atresia with ventricular septal defect without major aortopulmonary collateral arteries (MAPCAs) is an uncommon form of congenital heart disease. As more patients with congenital heart disease live to adulthood, the objective of this article was to review the long-term results of this specific population. Methods A review of the PubMed database was performed using pertinent key words (pulmonary atresia, tetralogy of Fallot, conduit, right ventricle-pulmonary artery) concentrating on studies from 1990-present and published in English. Most studies of pulmonary atresia-ventricular septal defect patients included those with and without MAPCAs. Analysis included examination of the entire cohort, consideration of the proportion of patients with MAPCAs, and any subgroup analysis of the patients without MAPCAs. Results Survival is approximately 80% at ten years and is improved with complete repair and larger pulmonary arteries. Some studies have found genetic syndromes and extracardiac anomalies to impact survival, while others have not. Incomplete repair has been shown to be associated with worse survival. Independent of initial management strategy, patients with pulmonary atresia and ventricular septal defects without MAPCAs require repeat intervention on the right ventricular outflow tract. Hypoplastic pulmonary arteries have been shown to be a risk factor for reintervention, and decreased conduit durability has been shown with younger age at implantation of conduit. Conclusions Long-term outcomes have improved for patients with pulmonary atresia-ventricular septal defect without MAPCAs, with complete repair and adequate pulmonary arteries favorable for survival. Long-term outcomes include reinterventions, both catheter-based and surgical, predominantly on the right ventricular outflow tract.
A Randomized Comparison of Postprandial Glucose Excursion Using Inhaled Insulin Versus Rapid-Acting Analog Insulin in Adults With Type 1 Diabetes Using Multiple Daily Injections of Insulin or Automated Insulin Delivery
Hirsch, Irl B., Beck, Roy W. , Marak, Martin Chase , Calhoun, Peter , Mottalib, Adham , Salhin, Amna, Manessis, Anastasios , Bhargava, Anuj , Thorsell, Ashley, Atakov-Castillo, Astrid , Bode, Bruce W., Levister, Camilla, Donahue, Cassandra, Cordero, Christian, Beatson, Christie , Langel, Christine R., Jacobson, Christopher , Kurek, Corey, Cruse, Dana, Pickering, David, Tamarez, Denisa, Desjardins, Donna, O’Malley, Grenye , Aktürk, Halis Kaan , Diner, Jamie, Baran, Jesica D., Ruedy, Katrina J., Codorniz, Kevin , Klein, Klara R. , Castorino, Kristin , Jordan, L.F., Kipnes, Mark, Church, Mei Mei , Raskin, Philip, Nguyen, Quang T., Weinstock, Ruth S. , Rizvi, Shafaq, Bzdick, Suzan, Rodriguez, Tahereh Ghorbani, Salah, Tareq , Blevins, Thomas , Kudva, Yogish C. , Haider, Zehra, Group, INHALE-3 Study, Bode, Bruce W., Ownby, Jonathan G., Johnson, Joseph H., Ruiz, Adriana, Stephens, Nashia, Maxson, Amanda, Beatson, Christie, Mason, E E , Steenkamp, Devin W., Wolpert, Howard , Sullivan, Asya, Salhin, Amna, Copple, Tina, Beltran, Stephanie, Ryan, Terri, O’Malley, Grenye, Tamarez, Denisa, Ipek, Aslihan, Burke, Christine, Langel, Christine, Bauman, Shannon , Haight, Cristina, Bohr, A., Badri, Marwa Al, Nguyen, Loida, Cordero, Christian, Migliozzi, Brittney, Reid, Corey, Kaur, Ravinder Jeet , Livingood, Daine, Cruse, Dana, Kravarusic, Jelena, Coyne, Kasey, Herrmann, Stefanie, Fronczyk, Evelyn, McKillop, Lorcan, Hyatt, Jamie, Patruno, Maria, Shuirman, Maggie, Cobb, Brandon, Watt, Olivia, Casaubon, Luis, Espinosa, Valerie, Pandit, Keta, Perez, Jennifer, Armstrong, Chloe, Martinez-Arredondo, Maritza, Stewart, Ana, Rodriguez, Tahereh Ghorbani, Young, Laura , Kass, Alexander, Fulghum, Karla, Jordan, Lin, Shao, Baomei, Baran, Jesica, Mandava, Patali, Holod, Rosanna, Kaiserman, Kevin B. , Ulloa, Johanna, Pleitez, Jennifer, Sylvan, Joey, Badgett, Lynn, Borgman, Sarah, Frey, Sarah, Mba-Oduwusi, Nnenna, Miller, Paige , Fults, Ashleigh, Marak, Martin, Pritchard, Rosa, Dolzhenko, E. N. , Gabrielson, Deanna, Brown, Sue A. , Ahmann, Andrew , Bergenstal, Richard M. , Levy, Carol J , Steenkamp, Devin W , Aleppo, Grazia , Akturk, Halis K , Klein, Klara R , Castorino, Kristin , Church, Mei Mei , Hamdy, Osama , Weinstock, Ruth S , Salah, Tareq , Kudva, Yogish C. , Beck, Roy W , Calhoun, Peter , Coviello, Andrea D. , Bode, Bruce W., Levy, Carol J. , Donahue, Cassandra, Aleppo, Grazia , Buse, John B. , Nguyen, Quang T., Lee, Scott Jeffery , Group, INHALE-3 Study, Bode, Bruce W., Beatson, Christie, Mason, Emma, Steenkamp, Devin W., Salhin, Amna, O’Malley, Grenye, Bauman, Shannon, Dhaver, Shilton , Nguyen, Quang T., Cordero, Christian, Cruse, Dana, Johnson, Olivia, Rodriguez, Tahereh Ghorbani, Miller, Paige, McCarthy, Chris

Diabetes Care | July 23, 2024 | Volume: 47 | Issue: 9 | Pages: 1682-1687

10.2337/dc24-0838 | PMID 39042575 Get It

OBJECTIVE To compare postprandial glucose excursions following a bolus with inhaled technosphere insulin (TI) or subcutaneous rapid-acting analog (RAA) insulin. RESEARCH DESIGN AND METHODS A meal challenge was completed by 122 adults with type 1 diabetes who were using multiple daily injections (MDI), a nonautomated pump, or automated insulin delivery (AID) and who were randomized to bolus with their usual RAA insulin (n = 61) or TI (n = 61). RESULTS The primary outcome, the treatment group difference in area under the curve for glucose &gt;180 mg/dL over 2 h, was less with TI versus RAA (adjusted difference −12 mg/dL, 95% CI −22 to −2, P = 0.02). With TI, the glucose excursion was smaller (P = 0.01), peak glucose lower (P = 0.01), and time to peak glucose shorter (P = 0.006). Blood glucose &lt;70 mg/dL occurred in one participant in each group. CONCLUSIONS Postmeal glucose excursion was smaller with TI than with RAA insulin in a cohort that included both AID and MDI users.