Outcomes for patients in the RESTORE registry with spinal muscular atrophy and four or more SMN2 gene copies treated with onasemnogene abeparvovec.

OBJECTIVE: We describe outcomes following onasemnogene abeparvovec monotherapy for patients with ≥four survival motor neuron 2 (SMN2) gene copies in RESTORE, a noninterventional spinal muscular atrophy patient registry. METHODS: We evaluated baseline characteristics, motor milestone achievement, post-treatment motor function, use of ventilatory/nutritional support, and adverse events as of December 22, 2022. RESULTS: At data cutoff, 19 patients in RESTORE had ≥four SMN2 copies and were treated with onasemnogene abeparvovec monotherapy (n=12 [63.2%] four copies; n=7 [36.8%] >four copies). All patients were identified by newborn screening and were reported as asymptomatic at diagnosis. Median age at onasemnogene abeparvovec administration was 3.0 months. Median time from treatment to last recorded visit was 15.4 months, with a range of post-treatment follow-up of 0.03-39.4 months. All 12 children who were assessed for motor development achieved new milestones, including standing alone (n=2) and walking alone (n=5). Five children reported one or more treatment-emergent adverse events (one Grade 3 or greater). No deaths or use of ventilatory/nutritional support were reported. CONCLUSIONS: Real-world findings from the RESTORE registry indicate that patients with ≥four SMN2 gene copies treated with onasemnogene abeparvovec monotherapy demonstrated improvements in motor function. Adverse events experienced by these patients were consistent with previously reported findings.

Intravitreal Antibiotics versus Early Vitrectomy plus Intravitreal Antibiotics for Post-Injection Endophthalmitis: an IRIS® Registry (Intelligent Research in Sight).

PURPOSE: To determine if intravitreal injection of antibiotics alone versus early pars plana vitrectomy (PPV) plus injection of intravitreal antibiotics predicted better or worse visual outcomes for patients with endophthalmitis after anti-vascular endothelial growth factor (anti-VEGF) injections. DESIGN: Retrospective cohort study PARTICIPANTS: Patients developing endophthalmitis after receiving an intravitreal anti-VEGF injection from the American Academy of Ophthalmology IRIS® Registry between 2016 and 2020. METHODS: Inclusion criteria were endophthalmitis diagnosis within 1 to 28 days after anti-VEGF injection and a recorded visual acuity (VA) at baseline, on the day of diagnosis, and post-treatment. Patients in the Injection Only group underwent intravitreal injection of antibiotics alone and in the Early Vitrectomy group received PPV with intravitreal antibiotics or intravitreal injection followed by PPV within 2 days of diagnosis. Patients were excluded if they had cataract surgery during the study, intravitreal steroids before endophthalmitis, or intermediate/posterior uveitis or cystoid macular edema. The study created a 1:1 matched cohort using Mahalanobis Distance Matching, accounting for the differences in VA at baseline and diagnosis. MAIN OUTCOME MEASURES: Post-treatment logMAR VA RESULTS: 1,044 patients diagnosed with post-injection endophthalmitis met the inclusion and exclusion criteria. In the unmatched cohort, there were 935 patients in the Injection Only and 109 in the Early Vitrectomy group. In 1:1 matched cohort 218 patients (109 in each group) were included; the median logMAR VAs were 0.32 [20/40-20/50] at baseline, 0.88 [∼20/150] at diagnosis, and 0.57 [20/70-20/80] post-treatment. There were no statistically significant differences in the visual outcomes between the two matched treatment groups (b = 0.05, p = 0.23); including the subgroup of patients with VA worse than 1.0 logMAR (b = 0.05, p = 0.452). CONCLUSIONS: There was no significant difference in final VA outcomes between patients receiving Injection Only and those treated with Early Vitrectomy for post-injection endophthalmitis. The findings support the use of either treatment strategy.

Patient needs in women of childbearing age with psoriasis: retrospective analysis from the German PsoBest registry.

Background: Women of childbearing age (WoCBA) with psoriasis face additional burden related to contraindications of systemic treatments during pregnancy/lactation and to the physical and psychosocial impact of psoriasis on their intimate/sexual relationships. Objective: Within a people-centered health care model, this study aimed to identify specific patient needs and their correlates in WoCBA (18-45 years), in comparison to same-age men and women above 45 years. Methods: Baseline data from the German PsoBest registry, including patients with moderate/severe plaque-type psoriasis with the indication for systemic therapy, were retrospectively analyzed. Psoriasis severity was assessed with the Psoriasis Area and Severity Index and patient-reported outcomes included EuroQoL Visual Analogue Scale, Dermatology Life Quality Index (DLQI), and Patient Needs Questionnaire. Results: The participants were 2308 WoCBA, 3634 men between 18 and 45 years of age, and 3401 women older than 45 years. In comparison to both control groups, WoCBA reported more DLQI impairments and higher needs to reduce social impairments (eg, "to be able to lead a normal working life"; "to be less burdened in your partnership"; "to be able to have a normal sex life"). These patient needs were associated with having psoriasis arthritis, previous systemic therapy, worse general health, and more DLQI impairments. Limitations: Retrospective analysis of an established dataset limited the examination of specific developmental, sexual, and reproductive variables. Conclusion: The specific needs of WoCBA with psoriasis were particularly related to the work context/financial stability and intimate relationships, which are crucial aspects in family planning. These results claim for a sex/age-sensitive approach in the psoriasis health care, by considering these needs as qualifiers in the clinical decision for systemic treatment.

Incidence of atrial fibrillation in patients with atrioventricular nodal re-entrant tachycardia and its association with long-term outcome.

Background: Atrioventricular nodal re-entrant tachycardia (AVNRT) is the most common paroxysmal supraventricular tachycardia. We sought to investigate the incidence of atrial fibrillation in patients with electrophysiologically confirmed/ablated AVNRT and its association with transient ischemic attack (TIA)/stroke as well as mortality during long-term follow-up. Methods: From the Karolinska Ablation Registry, 2855 consecutive patients with a first-time ablation for AVNRT between 2005 and 2018 were analyzed. Results: Patients were 52.1 ± 15.9 years old and 59.3% were women. During follow-up of up to 10 years (median 6.0 years; interquartile range 3.3 to 9.2 years), new onset or recurrence of atrial fibrillation occurred in 317 (11.1%) patients (incidence rate 19 cases per 1000 person-years). Excluding those with history of atrial fibrillation, new onset of atrial fibrillation occurred in 153 (6.1%) patients. In multivariable analysis, history of atrial fibrillation, arterial hypertension, history of TIA/stroke, and heart failure remained independently associated with new onset or recurrence of atrial fibrillation during follow-up. Death of any cause and TIA/stroke occurred in 141 (4.9%) patients and 107 (3.7%) patients, respectively. In multivariable analysis, occurrence of atrial fibrillation during follow-up remained independently associated with both outcomes. The prevalence of atrial fibrillation according to age at the end of follow-up was high among young patients (<60 years of age: 12.7%; 60-69 years of age: 10.6%). Conclusion: In this large cohort of patients with diagnosed AVNRT, the incidence of atrial fibrillation was high (11.1%) during long-term follow-up. Occurrence of atrial fibrillation during follow-up remained independently associated with death for any cause as well as with TIA/stroke. Therefore, a closer monitoring for atrial fibrillation in patients with AVNRT including those at young age is advisable.

Detecting and Dating Early Non-live Pregnancy Outcomes: Generation of a Novel Pregnancy Algorithm From Norwegian Linked Health Registries.

PURPOSE: Pregnancies ending before gestational week 12 are common but not notified to the Medical Birth Registry of Norway. Our goal was to develop an algorithm that more completely detects and dates all possible pregnancy outcomes (i.e., miscarriages, elective terminations, ectopic pregnancies, molar pregnancies, stillbirths, and live births) by using diagnostic codes from primary and secondary care registries to complement information from the birth registry. METHODS: We used nationwide linked registry data between 2008 and 2018 in a hierarchical manner: We developed the UiO pregnancy algorithm to arrive at unique pregnancy outcomes, considering codes within 56 days as the same event. To estimate the gestational age of pregnancy outcomes identified in the primary and secondary care registries, we inferred the median gestational age of pregnancy markers (45 ICD-10 codes and 9 ICPC-2 codes) from pregnancies registered in the medical birth registry. When no pregnancy markers were available, we assigned outcome-specific gestational age estimates. The performance of the algorithm was assessed by blinded clinicians. RESULTS: Using only the medical birth registry, we identified 649 703 pregnancies, including 1369 (0.2%) miscarriages and 3058 (0.5%) elective terminations. With the new algorithm, we detected 859 449 pregnancies, including 642 712 live-births (74.8%), 112 257 miscarriages (13.1%), 94 664 elective terminations (11.0%), 6429 ectopic pregnancies (0.7%), 2564 stillbirths (0.3%), and 823 molar pregnancies (0.1%). The median gestational age was 10+1 weeks (IQR 10+0-12+2) for miscarriages and 8+0 weeks (IQR 8+0-9+6) for elective terminations. Gestational age could be inferred using pregnancy markers for 66.3% of miscarriages and 47.2% of elective terminations. CONCLUSION: The UiO pregnancy algorithm improved the detection and dating of early non-live pregnancy outcomes that would have gone unnoticed if relying solely on the medical birth registry information.

The use of minimum common data set in the development of the Greek Fragility Hip Fracture Registry in the Greek health care setting: the first year of its pilot implementation.

The first Fragility Hip Fracture Registry has been established in Greece. The in-hospital length of stay was 10.8 days and was significantly influenced by the delayed surgical fixation. The increased age, the higher ASA grade, and the male gender influenced negatively the 30-day mortality, which reached 7.5%. BACKGROUND: The increased incidence of fragility hip fractures constitutes a great challenge to the health care professionals and causes a significant burden on national health care systems around the globe. Fragility hip fracture registries have been used in many countries in order to document the cotemporary situation in each country and to identify potential weaknesses of the local health care systems. AIM: The aim of the herein study is to present the results of the pilot implementation of the first fragility hip fracture registry in Greece, which was developed by the Greek Chapter of Fragility Fracture Network (FFN Gr), and use the neural networks in the analysis of the results. MATERIALS AND METHODS: Seven orthopaedic departments from six different hospitals in Greece participated in the present pilot study. All fragility hip fractures from September 2022 until December 2023 were prospectively collected and documented using a central database. For this purpose, the 22 points of minimum common data set, proposed by the Global Fragility Fracture Network, with the addition of the 30-day mortality was used. RESULTS: A total of 1009 patients who sustained a fragility hip fracture were included in the study. The mean age of the cohort was 82.2 ± 8.6 years with the majority of patients being female (72%). Sixty percent (60%) of the patients had an extracapsular hip fracture, with a mean ASA grade 2.6 ± 0.8. Intramedullary nailing and hip hemiarthroplasty were the surgical treatments of choice in the majority of extra- and intra-capsular hip fractures respectively. The mean hospital length of stay of the patients was 10.8 ± 8.5 days, and the 30-day mortality was 7.5%. The multivariant analysis revealed that the age, the ASA grade and the male gender had a significant contribution to the 30-day mortality. The neural network model had a significant under-the-curve predictive value (0.778), with age being the most important predictive factor. The length of stay was significantly influenced only by the delayed surgical fixation (more than 36 h from admission). CONCLUSIONS: The present pilot study provides evidence that establishing a fragility hip fracture registry in Greece is feasible and demonstrates that the minimum common data set can be used as the base of any new registry. In Greece, patients with a fragility hip fracture stay in the hospital for approximately 11 days and have 7.5% 30-day mortality. Unfortunately, due to the logistics of the public healthcare system, they do not receive surgical fixation in a timely manner, which is a factor that negatively affects their length of in-hospital stay.

Characteristic features of late-onset systemic lupus erythematosus: An observational study of data from the Lupus Registry of Nationwide Institutions.

OBJECTIVE: Late-onset systemic lupus erythematosus (LoSLE) is known to possess characteristics different from those of early-onset SLE (EoSLE), thereby making their diagnosis difficult. This study aimed to assess the characteristic features of LoSLE in Japan, a model country with a super-aged society. METHODS: Data were obtained from the Lupus Registry of Nationwide Institutions, which includes a multicenter cohort of patients with SLE in Japan who satisfied the 1997 American College of Rheumatology revised classification criteria for SLE. Data were compared between patients with LoSLE (≥50 years old at onset) and EoSLE (<50 years old at onset). To identify factors associated with LoSLE, binary logistic regression was used for the multivariate analysis, and missing values were complemented by multiple imputations. We also conducted a sub-analysis for patients diagnosed within 5 years of onset. RESULTS: Out of 929 enrolled patients, 34 were excluded owing to a lack of data regarding onset age. Among the 895 remaining patients, 100 had LoSLE, whereas 795 had EoSLE. The male-to-female ratio was significantly higher in the LoSLE group than in the EoSLE group (0.32 vs 0.11, p < 0.001). With respect to SLEDAI components at onset, patients with LoSLE exhibited a higher frequency of myositis (11.9% vs 3.75%, p = 0.031), lower frequency of skin rash (33.3% vs 67.7%, p < 0.001), and lower frequency of alopecia (7.32% vs 24.7%, p = 0.012). No significant differences in overall disease activity at onset were observed between the two groups. Regarding medical history, immunosuppressants were more commonly used in EoSLE. A multivariate analysis revealed that a higher male proportion and a lower proportion of new rash at onset were independent characteristic features of LoSLE. We also identified late onset as an independent risk factor for a high SDI score at enrollment and replicated the result in a sub-analysis for the population with a shorter time since onset. CONCLUSIONS: We clarified that LoSLE was characterized by a higher male proportion, a lower frequency of skin rash and a tendency to organ damage. Now that the world is faced with aging, our results may be helpful at diagnosis of LoSLE.

Longevity in the South Carolina Alzheimer's disease registry.

Background: South Carolina has arguably the most robust Alzheimer's Registry in the United States. For enhanced planning in both clinical practice and research and better utilization of the Registry data, it is important to understand survival after Registry entry. To this end, we conducted exploratory analyses to examine the patterns of longevity/survival in the South Carolina Alzheimer's Disease Registry. Methods: The sample included 42,028 individuals in the South Carolina Alzheimer's Disease Registry (SCADR). Participants were grouped into four cohorts based on their year of diagnosis. Longevity in the Registry (LIR), or the length of survival in the registry, was calculated based on the years of reported diagnosis and death. Results: The median LIR varied between 24 to 36 months depending on the cohort, with 75% of individuals in the three recent cohorts surviving for at least 12 months. Across all cohorts, 25% of the participants survived at least 60 months. The median LIR of females was longer than that of males. Individuals whose race was classified as Asian, American Indian, and other than listed had longer LIR compared to White, African American, and Hispanic individuals. Median LIR was shorter for Registry cases diagnosed at an earlier age (less than 65 years). Conclusion: Our data indicate that significant longevity is to be expected in the SCADR but that there is interesting variability which needs to be explored in subsequent studies. The SCADR is a rich data source prime for use in research studies and analyses.

Open label, interventional, single arm multicentric clinical study to analyze the potency of VELNEZ nasal pack, post-nasal surgery.

Background: Nasal packs are central to nasal surgeries. Primarily, these packs function by controlling bleeding, modulating pain and reducing adhesions postsurgery. However, the major setback of the currently used conventional nasal packs is the unbearable pain the patient undergoes upon removal of these packs. To overcome this shortcoming a variety of biodegradable packs have emerged. This study was aimed at evaluating the safety, efficacy and tolerability of VELNEZ nasal packs. VELNEZ, a patented Datt Mediproducts Pvt. Ltd. nasal pack, is one of its kind biodegradable composite that fragments within a few days of application. Methods: Eighty patients were included in an open label, interventional, single arm clinical study using clinical endpoints to investigate the safety and efficacy of nasal pack VELNEZ. The patients were questioned using a visual analog scale from discharge day to 28th postoperative day (9 follow-up visits) at regular intervals. The standardized questionnaires for hemorrhage control, relief from postoperative pain, moderate obstruction, and pain were used. Results: A total of 76 patients were enrolled in the study and 74 patients completed the study. VELNEZ nasal pack played a significant role in controlling hemorrhage and reducing postoperative pain. The average hemorrhage control time was 7.49 ± 3.90 min with only 34.24% of population complaining of pain on the sixth day of surgery (follow-up 4). Forteen days postsurgery only 10.95% of subject population complained of postoperative pain. This biodegradable composite has an average fragmentation time of 4.7 days in the nasal cavity. In addition, this study did not observe any postoperative adverse events or serious adverse events. Conclusion: VELNEZ, a fragmentable nasal pack, is comfortable, safe, and effective against postsurgery bleeding and pain.

The Impact of Upper Abdominal Surgery Regarding the Outcome of Patients with Advanced Ovarian Cancer.

Objective: Residual tumor after cytoreductive surgery is the most important prognostic parameter for the outcome of patients with advanced ovarian cancer (5-year survival rate FIGO III 39%, FIGO IV 20%). As more than half of the patients suffer from upper abdominal tumor burden, surgery in this area is inevitable in order to achieve adequate cytoreduction. Our analysis focuses on the impact of upper abdominal interventions (UAI) regarding residual tumor and prognosis (OS, PFS). Methods: A total of n = 261 patients with advanced primary ovarian cancer stage FIGO III and IV and radical cytoreductive surgery at the Gynecologic Cancer Center Hamburg-Eppendorf between 2014 and 2019 were analyzed in a retrospective study design and divided into two groups: one with UAI (n = 160) and one without UAI (n = 101). Results: Patients with UAI showed significantly more often a residual tumor of less than 1 cm (R1) than patients without UAI and had a significantly longer OS (59 vs. 45 months [p = 0.041]). Deperitonealization of the diaphragm was the most common (144/160) and prognostically most relevant procedure for UAI. Especially the subgroup with FIGO IIIC stage seemed to benefit most from UAI. However, in multivariate analysis residual tumor burden was the strongest prognostic parameter for survival, followed by FIGO stage and UAI. Mortality was low within in the UAI group (0.6%). Conclusion: UAI is an essential part of cytoreductive surgery in advanced ovarian cancer patients with tumor spread into the upper abdomen as it significantly prolongs survival. The procedure appears to be safe with low mortality. Achieving R1 rather than R2 due to radical surgery combined with UAI should be preferred compared to the early termination of the operation, as this has a significant impact on the prognosis of the patients.

An observational cohort of patients with colorectal peritoneal metastases or pseudomyxoma peritonei treated with CRS-HIPEC: Development and first results of the Dutch CRS-HIPEC quality registry.

INTRODUCTION: To improve care for patients with colorectal peritoneal metastases (CRC-PM) or pseudomyxoma peritonei (PMP), the Dutch CRS-HIPEC quality registry was initiated in 2019. The aims are to describe the development and content of this registry and to give insight into the data collected during the first years. MATERIALS AND METHODS: The registry is an observational cohort in the Netherlands. All patients with CRC-PM or PMP who intend to undergo cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS-HIPEC) from 6 hospitals are included. Clinical data and outcomes (including hospital variation) were analyzed. RESULTS: In 2019-2022, 889 patients were included in the CRS-HIPEC quality registry: 749 (84 %) with CRC-PM and 140 (16 %) with PMP. Peritoneal metastases were diagnosed synchronously in 51 % of CRC-PM patients and in 94 % of PMP patients. In patients undergoing complete CRS, the median peritoneal cancer index was 8 (IQR 4-13) for CRC-PM and 15 (IQR 6-26) for PMP. Complete cytoreduction was achieved in 639 CRC-PM patients (97 %) and 108 PMP patients (82 %). HIPEC was mainly performed with mitomycin C (CRC-PM: 94 %, PMP: 92 %). Major postoperative complications (Clavien-Dindo grade ≥3) occurred in 148 CRC-PM patients (22 %) and 30 PMP patients (23 %) with 90-day mortality rates of 2 %. In CRC-PM, differences between hospitals were observed regarding proportions of diagnostic laparoscopies/laparotomies, (neo)adjuvant treatment, ostomy formations and re-admissions. CONCLUSION: The CRS-HIPEC quality registry provides insight into the outcomes of CRS-HIPEC and enables clinical auditing and observational cohort studies aiming to improve treatment outcomes for patients with CRC-PM and PMP.

The creation of a global neurotrauma registry.

The authors propose the generation of a multi-institutional TBI registry. Barriers to registry implementation include: (1) difficulties in acquiring ethical approval; (2) incomplete clinical data available; (3) lack of information and insufficient technology (IT) support; (4) limited available resources; (5) time constraints involving understaffing yet managing high patient volumes; (6) time constraints associated with entering patient data into the registry tool. The authors detail the current state of affairs on neurotrauma registries worldwide and propose the creation of a multi-institutional, global neurotrauma registries. This private-public partnership will enable appropriate balance among stakeholders while offering care to the largest number of citizens. This initiative will require coordinated efforts involving vetted members of organized neurosurgery. Support from these entities, such as fellowship program creation, provided funding through travel vouchers to LMICs, secured housing and transportation costs in LMI nations, facilitated meetings with global local stakeholders, and promotion of key developments via social media, will accelerate the creation of this global neurotrauma registry. We propose the creation of a global TBI registry, in partnership with large, academic medical centers. Several proposed limitations of registry implementation can be addressed with support from local stakeholders, including government officials and administrative members at key institutions. Several American institutions have well-established global health programs to support this initiative. Further, at Harvard Medical School, the program in Global Surgery and Social Change offers the Paul Farmer Global Surgery Fellowship that trains leaders in policy development and implementation. The fellowship consists of 2 separate tracks: a 2-year research fellow (PGY-5-PGY-6) and 1-year research associate (MD and MBBS, etc.). Funding could be allocated towards creating a year-long fellowship dedicated towards implementing a neurotrauma registry, with this selected scholar granted the resource and connections to network with government officials and healthcare groups in every nation within that jurisdiction. A scholar would be assigned a region of the world with the goal to generate a registry that would later be combined with those generated by peer scholars. In addition, we propose the creation of a fund, controlled by donors, as a funding model.

Statistics of Visceral Sarcoma in Japan: Report From the Population-Based National Cancer Registry (NCR) in Japan.

BACKGROUND AND OBJECTIVES: Sarcomas developing in the visceral organs are extremely rare, with no previous reports to describe their national epidemiology. We analyzed Japanese domestic statistics for visceral sarcoma, using the National Cancer Registry (NCR) in Japan, a population-based database launched in 2016. METHODS: We identified 3245 cases of visceral sarcomas in the NCR dated 2016-2019 to analyze demographic and disease information, initial diagnostic process, volume and type of the hospitals, treatment, and prognosis. RESULTS: Visceral sarcoma shows a higher prevalence in the older generation (60+ years), with a significant male predominance (p = 0.006). Leiomyosarcomas occurred frequently in the gastrointestinal tract (N = 240; 39.5%), and angiosarcomas in the liver, gall bladder, pancreas, and spleen (N = 244; 43.9%). Visceral sarcomas were often treated in facilities of lower volume without specific adjuvant treatments (p < 0.001). The cumulative 3-year overall survival was 44.8%, and several factors such as surgery or absence of chemotherapy positively affected survival. CONCLUSIONS: This is the first nationwide study in Japan to analyze the inclusive epidemiology of visceral sarcomas. Visceral sarcomas are characterized by senior and male predominance with relatively poor prognosis, often managed in nonspecialized facilities and rarely with adjuvant therapies. Several histologic subtypes had the propensity to develop in specific organs.

Heart failure in Europe: Guideline-directed medical therapy use and decision making in chronic and acute, pre-existing and de novo, heart failure with reduced, mildly reduced, and preserved ejection fraction - the ESC EORP Heart Failure III Registry.

AIMS: We analysed baseline characteristics and guideline-directed medical therapy (GDMT) use and decisions in the European Society of Cardiology (ESC) Heart Failure (HF) III Registry. METHODS AND RESULTS: Between 1 November 2018 and 31 December 2020, 10 162 patients with acute HF (AHF, 39%, age 70 [62-79], 36% women) or outpatient visit for HF (61%, age 66 [58-75], 33% women), with HF with reduced (HFrEF, 57%), mildly reduced (HFmrEF, 17%) or preserved (HFpEF, 26%) ejection fraction were enrolled from 220 centres in 41 European or ESC-affiliated countries. With AHF, 97% were hospitalized, 2.2% received intravenous treatment in the emergency department, and 0.9% received intravenous treatment in an outpatient clinic. AHF was seen by most by a general cardiologist (51%) and outpatient HF most by a HF specialist (48%). A majority had been hospitalized for HF before, but 26% of AHF and 6.1% of outpatient HF had de novo HF. Baseline use, initiation and discontinuation of GDMT varied according to AHF versus outpatient HF, de novo versus pre-existing HF, and by ejection fraction. After the AHF event or outpatient HF visit, use of any renin-angiotensin system inhibitor, angiotensin receptor-neprilysin inhibitor, beta-blocker, mineralocorticoid receptor antagonist and loop diuretics was 89%, 29%, 92%, 78%, and 85% in HFrEF; 89%, 9.7%, 90%, 64%, and 81% in HFmrEF; and 77%, 3.1%, 80%, 48%, and 80% in HFpEF. CONCLUSION: Use and initiation of GDMT was high in cardiology centres in Europe, compared to previous reports from cohorts and registries including more primary care and general medicine and regions more local or outside of Europe and ESC-affiliated countries.

Temporal Trends in Acute Adrenal Insufficiency Events in Children With Congenital Adrenal Hyperplasia During 2019-2022.

Background: It is unclear whether targeted monitoring of acute adrenal insufficiency (AI) related adverse events (AE) such as sick day episodes (SDEs) and hospitalization rate in congenital adrenal hyperplasia (CAH) is associated with a change in the occurrence of these events. Aim: Study temporal trends of AI related AE in the I-CAH Registry. Methods: In 2022, data on the occurrence of AI-related AE in children aged <18 years with 21-hydroxylase deficiency CAH were compared to data collected in 2019. Results: In 2022, a total of 513 children from 38 centers in 21 countries with a median of 8 children (range 1-58) per center had 2470 visits evaluated over a 3-year period (2019-2022). The median SDE per patient year in 2022 was 0 (0-2.5) compared to 0.3 (0-6) in 2019 (P = .01). Despite adjustment for age, CAH phenotype and duration of study period, a difference in SDE rate was still apparent between the 2 cohorts. Of the 38 centers in the 2022 cohort, 21 had also participated in 2019 and a reduction in SDE rate was noted in 13 (62%), an increase was noted in 3 (14%), and in 5 (24%) the rate remained the same. Of the 474 SDEs reported in the 2022 cohort, 103 (22%) led to hospitalization compared to 299 of 1099 SDEs (27%) in the 2019 cohort (P = .02). Conclusion: The I-CAH Registry can be used for targeted monitoring of important clinical benchmarks in CAH. However, changes in reported benchmarks need careful interpretation and longer-term monitoring.

Spanish pacemaker registry. 21st official report of Heart Rhythm Association of the Spanish Society of Cardiology (2023).

INTRODUCTION: Data on implants of cardiac pacing systems in Spain in 2023 are presented. METHODS: The registry is based on the information provided by centers to the recording platform of the Heart Rhythm Association after device implantations, through Cardiodispositivos, the online platform of the National Registry. Other information sources include: a) data transfers from the manufacturing and marketing industry; b) the European pacemaker patient card; and c) local databases submitted by the implanting centers. RESULTS: In 2023, 112 hospitals participated in the registry (30 more than in 2022). A total of 24 343 device implantations were reported (48.1% more than in 2022) compared with 45 120 reported by Eucomed (European Confederation of Medical Suppliers Associations). Of these, 1646 were cardiac resynchronization therapy pacemakers. The devices showing the largest increases were leadless pacemakers, with 963 devices implanted, representing an 18.1% increase over 2022. The most frequent indication was atrioventricular block followed, for the first time, by atrial tachyarrhythmia with slow ventricular response. The number of devices included in remote monitoring also increased (cardiac resynchronization therapy defibrillators, 71%; cardiac resynchronization therapy pacemakers, 63%; and conventional pacemakers, 28%), although more moderately. CONCLUSIONS: In 2023, there was an increase in the number of institutions participating in the registry. The reporting of device implantations rose by 48.1%, and the implantation of leadless pacemakers grew by 18.1%. Remote monitoring also experienced modest growth compared with previous years. Full English text available from: www.revespcardiol.org/en.

Improving post-injury follow-up survey response: incorporating automated modalities.

BACKGROUND: Incorporating post-discharge data into trauma registries would allow for better research on patient outcomes, including disparities in outcomes. This pilot study tested a follow-up data collection process to be incorporated into existing trauma care systems, prioritizing low-cost automated response modalities. METHODS: This investigation was part of a larger study that consisted of two protocols with two distinct cohorts of participants who experienced traumatic injury. Participants in both protocols were asked to provide phone, email, text, and mail contact information to complete follow-up surveys assessing patient-reported outcomes six months after injury. To increase follow-up response rates between protocol 1 and protocol 2, the study team modified the contact procedures for the protocol 2 cohort. Frequency distributions were utilized to report the frequency of follow-up response modalities and overall response rates in both protocols. RESULTS: A total of 178 individuals responded to the 6-month follow-up survey: 88 in protocol 1 and 90 in protocol 2. After implementing new follow-up contact procedures in protocol 2 that relied more heavily on the use of automated modalities (e.g., email and text messages), the response rate increased by 17.9 percentage points. The primary response modality shifted from phone (72.7%) in protocol 1 to the combination of email (47.8%) and text (14.4%) in protocol 2. CONCLUSIONS: Results from this investigation suggest that follow-up data can feasibly be collected from trauma patients. Use of automated follow-up methods holds promise to expand longitudinal data in the national trauma registry and broaden the understanding of disparities in patient experiences.

Getting to the HEART of Major Adverse Cardiac Events.

How to cite this article: Samavedam S. Getting to the HEART of Major Adverse Cardiac Events. Indian J Crit Care Med 2024;28(8):724-725.

Geographic Patterns of Ocular Oncologist Supply and Patient Demand for Uveal Melanoma Treatment in the United States: A Supply and Demand Analysis.

Purpose: To study geographic patterns of supply and demand for uveal melanoma and other ocular oncology healthcare by ocular oncology physicians in the United States. Methods: Google search interest data was obtained through trends.google.com. The combined-state density of ocular oncology physicians was calculated by dividing the number of practicing ocular oncologists in each state and its surrounding states by the state population. Relative search volume (RSV) values were divided by ocular oncology physician density to calculate the Google relative demand index (gRDI) for each state. Medicare (mRDI) and IRIS® Registry (iRDI) relative demand indices were calculated using prevalence data obtained through the Vision and Eye Health Surveillance System (VEHSS). Data from the US Census Bureau and Centers for Disease Control (CDC) databases were also utilized to analyze associations with poverty rates, percent living in urban or rural areas, vision screening rates, and ocular neoplasm rates. Results: Alabama showed the highest RSV (100), while the lowest was reported in New Mexico (20). Vermont had the highest density of combined-state ocular oncology ophthalmologists (1.85 per 100,000 residents). New Mexico had the lowest RDI (0.013 gRDI, 0.015 mRDI, 0.018 iRDI) with 32 combined-state ocular oncologists and a population of 2,114,371. Ocular neoplasm prevalence rates ranged between 1.32% and 5.40% and significantly correlated with RSV. Single-state gRDI correlated with rural status and negatively correlated with urban areas (≥50,000 individuals). Single-state ophthalmologist density correlated positively with percent living in urban areas and vision screening rates, and negatively with rural status. Conclusion: This study uncovered significant heterogeneity in the geographical distribution of ocular oncology physicians and RDI throughout the United States, highlighting potential undersupply scenarios. This may guide efforts to increase ocular oncology physician and surgeon availability in areas of need.

Permanent pacemaker implantation after combined mitral- and tricuspid valve surgery-a nationwide multicentre study.

OBJECTIVES: The threshold to perform concomitant TV repair (TVr) during primary mitral valve (MV) surgery has decreased, based on recent randomized evidence. Based on these developments, the indication for TVr during MV surgery is expected to increase further. However, concerns have been raised regarding the risk of permanent pacemaker implantation (PPI) during a concomitant procedure. Therefore, we aim to assess the incidence of PPI in combined MV and TV surgery in a nationwide registry. METHODS: The current study uniquely cross-linked the Cardiothoracic and Pacemaker/ICD registry of the Netherlands Heart Registration. Patients undergoing primary MV and TV surgery (± ASD closure, rhythm surgery, CABG) between January 1st-December 31st 2021 were included. The primary outcome was PPI within 30 days after surgery. Subgroup analyses were performed for isolated MV and TV surgery and MV repair. The association between concomitant TV surgery and PPI was assessed using multivariable binary logistic regression analyses. RESULTS: A total of 1060 patients (n = 833 MV, n = 227 MV+TV) were included. The overall incidence of PPI was 4.3%. No significant difference in PPI between MV and MV+TV surgery were found (3.7% vs 6.6%, P = 0.06). Concomitant TV surgery was not an independent risk factor for PPI after surgery after adjustment for covariates. These results were robust across subgroup and sensitivity analyses. CONCLUSIONS: The current study was not able to find a statistical difference between the PPI rate in MV surgery patients and MV + TV surgery patients. Extension of the waiting period prior to PPI, may result in decreased PPI rates.