Arrhythmogenic Mitral Valve Prolapse Revisited: A Not Uncommon Cause of Youthful Sudden Death in Athletes and Women.

BACKGROUND: Sudden deaths (SD) in young people including competitive athletes, albeit uncommon, are usually attributable to genetic, congenital or acquired cardiovascular conditions. However, it is under-appreciated that mitral valve prolapse (MVP), a relatively common valvular heart disease, is associated with SD in this youthful population. METHODS: Forty-three MVP-related SDs are identified from 2 large cardiovascular registries with pathologic, clinical, and demographic findings reported. RESULTS: Events occurred in both genders, but females were unexpectedly common (49%); median age was 22 ± 8 years, and 29 (67%) were engaged in competitive sports, including 17 with preparticipation examination. Of the 43 MVP cases, 21 died suddenly during or just after vigorous exercise including 6 during organized sports. Sixteen (37%) had been evaluated by a cardiologist resulting in confirmed MVP diagnosis in 11.. Pathologic findings characteristic of MVP included: bileaflet myxomatous involvement in all cases; and areas of interstitial or replacement myocardial fibrosis in 79%, most evident in posterolateral left ventricular wall. CONCLUSIONS: Arrhythmogenic myxomatous degeneration (MVP) is an under-recognized cause of SD in young people including competitive athletes, disproportionally affecting females and requiring requires a high index of clinical suspicion. Frequency of left ventricular fibrosis in these young people with MVP suggests a mechanism for ventricular tachyarrhythmias and SD, relevant to future risk stratification.

Acute Aortic Dissection: Observational Lessons Learned From 11 000 Patients.

BACKGROUND: Over the past 25 years, diagnosis and therapy for acute aortic dissection (AAD) have evolved. We aimed to study the effects of these iterative changes in care. METHODS: Patients with nontraumatic AAD enrolled in the International Registry of Acute Aortic Dissection (61 centers; 15 countries) were divided into time-based tertiles (groups) from 1996 to 2022. The impact of changes in diagnostics, therapeutic care, and in-hospital and 3-year mortality was assessed. Cochran-Armitage trend and Jonckheere-Terpstra tests were conducted to test for any temporal trend. RESULTS: Each group consisted of 3785 patients (mean age, ≈62 years old; ≈65.5% males); nearly two-thirds had type A AAD. Over time, the rates of hypertension increased from 77.8% to 80.4% (P=0.002), while smoking (34.1% to 30.6%, P=0.033) and atherosclerosis decreased (25.6%-16.6%; P<0.001). Across groups, the percentage of surgical repair of type A AAD increased from 89.1% to 92.5% (P<0.001) and was associated with decreased hospital mortality (from 24.1% in group 1 to 16.7% in group 3; P<0.001). There was no difference in 3-year survival (P=0.296). For type B AAD, stent graft therapy (thoracic endovascular aortic repair) was used more frequently (22.3%-35.9%; P<0.001), with a corresponding decrease in open surgery. Endovascular in-hospital mortality decreased from 9.9% to 6.2% (P=0.003). As seen with the type A AAD cohort, overall 3-year mortality for patients with type B AAD was consistent over time (P=0.084). CONCLUSIONS: Over 25 years, substantial improvements in-hospital survival were associated with a more aggressive surgical approach for patients with type A AAD. Open surgery has been partially supplanted by thoracic endovascular aortic repair for complicated type B AAD, and in-hospital mortality has decreased over the time period studied. Postdischarge survival for up to 3 years was similar over time.

Can compassionate leadership of senior hospital leaders help retain trainee doctors?

BACKGROUND: High burnout and low retention rates among trainee doctors threaten the future viability of the UK medical workforce. This study empirically examined factors that can sustain trainee doctors. METHOD: A total of 323 trainee doctors from 25 National Health Service (NHS) Trusts in England and Wales completed an online survey on their training and employment experiences. A mixed method approach was employed. RESULTS: Structural equation modelling revealed that perceived compassionate leadership of hospital senior leaders (CLSL) (i.e., doctors in senior clinical and management positions, and senior managers) is directly and negatively associated with trainee doctors' burnout and intention to quit. We propose the associations may be indirectly strengthened through two mediating pathways: increased psychological contract fulfilment (PCF) of training/organisational support and reduced worry about the state of the NHS; however, only the former is supported. The model can explain a substantial 37% of the variance in reported burnout and 28% of intention to quit among trainee doctors. Being a Foundation Year (FY) trainee was significantly associated with poor PCF and burnout. Rich qualitative data further elaborated on their experiences in terms of senior leaders' awareness of their training/working experiences, listening to and acting on. CONCLUSIONS: Active and demonstrable CLSL plays a vital role in trainee doctors' retention. It has both direct (through support) and indirect effects through improving trainee doctors' PCF to reduce burnout and intention to quit. This seems particularly valuable among FY doctors. Implications for the development and management of the medical workforce are discussed.

Examining the impact of median arcuate ligament-induced celiac artery compression on target vessel patency, long-term survival and device integrity in fenestrated and branched endovascular repairs.

OBJECTIVE: To evaluate the impact of celiac artery (CA) compression by median arcuate ligament (MAL) on technical metrics and long-term CA patency in patients with complex aortic aneurysms undergoing fenestrated/branched endograft repairs (F/B-EVARs). METHODS: Single-center, retrospective review of patients undergoing fenestrated/branched endovascular aortic aneurysm repairs and requiring incorporation of the CA between 2013 and 2023. Patients were divided into two groups-those with (MAL+) and without (MAL-) CA compression-based on preoperative computed tomography angiography findings. MAL was classified in three grades (A, B, and C) based on the degree and length of stenosis. Patients with MAL grade A had ≤50% CA stenosis measuring ≤3 mm in length. Those with grade B had 50% to 80% CA stenosis measuring 3 to 8 mm long, whereas those with grade C had >80% stenosis measuring >8 mm in length. End points included device integrity, CA patency and technical success-defined as successful implantation of the fenestrated/branched device with perfusion of CA and no endoleak. RESULTS: One hundred and eighty patients with complex aortic aneurysms (pararenal, 128; thoracoabdominal, 52) required incorporation of the CA during fenestrated/branched endovascular aortic aneurysm repair. Majority (73%) were male, with a median age of 76 years (interquartile range [IQR], 69-81 years) and aneurysm size of 62 mm (IQR, 57-69 mm). Seventy-eight patients (43%) had MAL+ anatomy, including 33 patients with MAL grade A, 32 with grade B, and 13 with grade C compression. The median length of CA stenosis was 7.0 mm (IQR, 5.0-10.0 mm). CA was incorporated using fenestrations in 177 (98%) patients. Increased complexity led to failure in CA bridging stent placement in four MAL+ patients, but completion angiography showed CA perfusion and no endoleak, accounting for a technical success of 100%. MAL+ patients were more likely to require bare metal stenting in addition to covered stents (P = .004). Estimated blood loss, median operating room time, contrast volume, fluoroscopy dose and time were higher (P < .001) in MAL+ group. Thirty-day mortality was 3.3%, higher (5.1%) in MAL+ patients compared with MAL- patients (2.0 %). At a median follow-up of 770 days (IQR, 198-1525 days), endograft integrity was observed in all patients and CA events-kinking (n = 7), thrombosis (n = 1) and endoleak (n = 2) -occurred in 10 patients (5.6%). However, only two patients required reinterventions. MAL+ patients had overall lower long-term survival. CONCLUSIONS: CA compression by MAL is a predictor of increased procedural complexity during fenestrated/branched device implantation. However, technical success, long-term device integrity and CA patency are similar to that of patients with MAL- anatomy.

Advancing Wearable Biosensors for Congenital Heart Disease: Patient and Clinician Perspectives: A Science Advisory From the American Heart Association.

Wearable biosensors (wearables) enable continual, noninvasive physiologic and behavioral monitoring at home for those with pediatric or congenital heart disease. Wearables allow patients to access their personal data and monitor their health. Despite substantial technologic advances in recent years, issues with hardware design, data analysis, and integration into the clinical workflow prevent wearables from reaching their potential in high-risk congenital heart disease populations. This science advisory reviews the use of wearables in patients with congenital heart disease, how to improve these technologies for clinicians and patients, and ethical and regulatory considerations. Challenges related to the use of wearables are common to every clinical setting, but specific topics for consideration in congenital heart disease are highlighted.

Pulmonary Complications of Everolimus in Liver Transplant Patients: A 10-Year Experience.

This retrospective study aims to evaluate the safety of everolimus when used as part of the immunosuppression regimen in patients who underwent liver transplant from 2009 to 2019 at a tertiary liver transplant center. Patients were divided into two groups: those who received everolimus as part of the post-transplant regimen and those who did not. The primary safety outcome measured was the development of new pulmonary complications that had been associated with everolimus use in prior studies. Lung function was determined by pulmonary function tests if available or CT scans of the chest. Secondary outcomes measured included everolimus discontinuation rates and survival rates. During the study period, 450 patients underwent liver transplant; 35% of patients received everolimus (n=156) and 65% of patients did not receive everolimus (n=292). Primary safety outcome of pulmonary complications was seen in 3.9% of patients who received everolimus (n=6) and 6.3% of the control group patients who did not receive everolimus (n=19). The association between everolimus use and new pulmonary complications was not significant with a chi-square statistic of 1.33 (p=0.249). Overall, 51.3% of patients who received everolimus during their post-transplant course discontinued the medication (n=80). Everolimus is safe from a pulmonary toxicity standpoint in liver transplant immunosuppression regimens as there was no significant difference found in pulmonary complications between patients who received the medication and those who did not.

The Health Care Burden of Hepatic Encephalopathy.

Hepatic encephalopathy-a common and debilitating complication of cirrhosis-results in major health care burden on both patients and caregivers through direct and indirect costs. In addition to risk of falls, inability to work and drive, patients with hepatic encephalopathy often require hospital admission (and often readmission), and many require subacute care following hospitalization. The costs and psychological impact of liver transplantation often ensue. As the prevalence of chronic liver disease increases throughout the United States, the health care burden of hepatic encephalopathy will continue to grow.

A challenging case of pancreaticogastric anastomotic stricture in a patient with surgically altered anatomy.

Video 1EUS-guided gastro-jejunostomy using a lumen-apposing metal stent to access the surgical pancreaticogastric anastomotic site and perform EUS-guided de novo pancreatico-gastrostomy for the management of a postsurgical pancreaticogastric anastomotic stricture with pancreatic insufficiency.

Twenty-five years of observations from the International Registry of Acute Aortic Dissection (IRAD) and its impact on the cardiovascular scientific community.

OBJECTIVE: The International Registry of Acute Aortic Dissection (IRAD) celebrated its 25th anniversary in January 2021. This study evaluated IRAD's role in promoting the understanding and management of acute aortic dissection (AD) over these years. METHODS: IRAD studies were identified, analyzed, and ranked according to their citations per year (c/y) to determine the most-cited IRAD studies and topics. A systematic search of the literature identified cardiovascular guidelines on the diagnosis and management of acute AD. Consequently, IRAD's presence and impact were quantified using these documents. RESULTS: Ninety-seven IRAD studies were identified, of which 82 obtained more than 10 cumulative citations. The median c/y index was 7.33 (25th-75th percentile, 4.01-16.65). Forty-two studies had a greater than median c/y index and were considered most impactful. Of these studies, most investigated both type A and type B AD (n = 17, 40.5%) and short-term outcomes (n = 26, 61.9%). Nineteen guideline documents were identified from 26 cardiovascular societies located in Northern America, Europe, and Japan. Sixty-nine IRAD studies were cited by these guidelines, including 38 of the 42 most-impactful IRAD studies. Among them, partial thrombosis of the false lumen as a predictor of postdischarge mortality and aortic diameters as a predictor of type A occurrence were determined as most-impactful specific IRAD topics by their c/y index. CONCLUSIONS: IRAD has had and continues to have an important role in providing observations, credible knowledge, and research questions to improve the outcomes of patients with acute AD.

The State of Diversity, Equity, Inclusion, and Justice in Pediatric Fellowship Programs.

Diversity, equity, inclusion, and justice (DEIJ) lie at the center of excellent patient care and safety. The way we interact with our pediatric patients and families and develop and discuss treatment plans should all be done under the lens of DEIJ. The foundation of DEIJ begins in medical school and is further honed during general pediatric residency training by ensuring residents are exposed to diverse patient populations. During fellowship training, the focus is shifted to research and scholarly activities for that same population. There is a vast catalog of curricula on DEIJ education for medical schools, some exist for general pediatric residency programs, but there is a lack of such curricula for subspecialty pediatric fellowship programs. In this article, we will address the state of DEIJ education in pediatric fellowship education, make a case for the importance of DEIJ education in pediatric fellowship programs, and provide recommendations to address the gap to include a holistic approach that involves holding departments, program leaders, and individuals accountable for continued medical education. [Pediatr Ann. 2023;52(7):e261-e265.].

Attitudes of Gastroenterologists Regarding Delivery of Cancer Diagnoses: a Cross-Sectional Study.

GOALS: To determine the attitudes and practices of gastroenterologists regarding the delivery of cancer diagnoses. BACKGROUND: Gastroenterologists frequently diagnose colorectal cancer. Receiving the news of a cancer diagnosis is difficult, and the delivery of the diagnosis can influence a patient's understanding of their disease. No study to date has reported how gastroenterologists deliver cancer diagnoses to their patients. STUDY: An anonymous questionnaire was sent online to gastroenterologists of the American College of Gastroenterology to assess views regarding the delivery of cancer diagnoses. RESULTS: Of the 280 complete responses (response rate = 1.64%), most respondents were male (n = 205, 73.21%), in practice between 0 and 9 years (n = 133, 47.50%), and at the attending/faculty level (n = 69.53%, 194). Most responded that they would disclose a cancer diagnosis to the patient themselves if they had made the discovery on endoscopy/colonoscopy (n = 255, 94.80%), with the preferred methods being an in person discussion (n = 187, 71.65%). Most respondents were not familiar with any guidelines for delivering cancer diagnoses (n = 202, 75.94%) and would be open to receiving training on cancer diagnosis delivery (n = 207, 78.11%). CONCLUSIONS: Most gastroenterologists take personal responsibility in the delivery of cancer diagnoses. Many gastroenterologists receive no specific training on how to deliver this news and are unaware of any guidelines to follow that may be helpful in their practice. However, most displayed a willingness to learn these guidelines through some form of formal education. Future directions should consider the incorporation of education in cancer diagnosis delivery for gastroenterologists and gastroenterology fellows.

Nutrient Drink Test to Assess Gastric Accommodation in Cyclic Vomiting Syndrome: Single-blinded Parallel Grouped Prospective Study.

Background/Aims: Cyclic vomiting syndrome (CVS) is characterized by episodes of nausea and vomiting, separated by symptom-free intervals. The pathogenesis of CVS is poorly understood. Limited data exist on evaluating impaired gastric accommodation as a mechanistic means for symptoms. We aim to determine if CVS patients demonstrate impaired gastric accommodation applying a nutrient drink test (NDT) protocol. Methods: Through this single-blinded pilot clinical trial, patients with CVS per Rome IV critera and healthy controls were assessed for presence of impaired gastric accommodation by administering an established NDT protocol. Statistical analysis was performed, with data presented as medians and interquartile range. Results: Eleven CVS patients and 15 healthy controls participated in the study between January 2018 and October 2018. Median age was 42.0 years and 37.0 years; majority of subjects were female, 72.7% and 73.3%, respectively. Demographics were similar between CVS and healthy controls. Almost all healthy controls (93.3%) ingested the complete 500 mL protocol, whereas a smaller proportion (72.7%) were able to complete all 4 doses in the CVS group (P = 0.188). Post-prandial visual analogue scale scores of nausea and abdominal pain were found to be significantly higher in CVS patients compared to healthy controls. Conclusions: To our knowledge, this is the first NDT protocol in CVS evaluating the role of impaired gastric accommodation and hypersensitivity as a possible pathophysiologic mechanism. Findings from this study suggest the presence of gastric hypersensitivity in a subset of CVS patients. These results provide the foundational data necessary for future larger testing of NDT and diagnostic accuracy in CVS.

An Unusual Case of a Pharyngeal Fistula to the Cervical Spine Causing Osteomyelitis.

SIGNIFICANCE STATEMENT: Pharyngeal fistulas to the cervical spine resulting in vertebral osteomyelitis are a rare, yet clinically important, complication of total laryngectomy performed in conjunction with chemoradiotherapy or radiation therapy. This complication is likely underdiagnosed and can have a high mortality rate. It is very important that clinicians are aware of this complication as early diagnosis and management may improve patient outcomes.

Variation in paediatric 24-h ambulatory blood pressure monitoring interpretation by Canadian and UK physicians.

Twenty-four-hour ambulatory blood pressure monitoring (ABPM) is widely accepted as a more accurate method for measurement of blood pressure (BP) compared to a single office-based measurement of BP. However, it is unclear how physicians interpret ABPM and make management decisions. This study's goal is to investigate variation in ABPM interpretation among paediatric nephrologists (Canada and UK) and paediatric cardiologists (Canada only) via an online survey. The survey content included baseline demographics, questions on the use and indications for ABPM, interpretation of results, and subsequent management decisions in various clinical scenarios. The survey was sent to 196 Canadian physicians, with 69 (35.2%) total responses. Thirty-five UK clinicians also completed the survey. Most respondents were >44 years old, were in practice for at least 11 years, and were university-based. There were substantial differences among clinicians in ABPM interpretation for isolated systolic, diastolic, and night-time hypertension. For example, only 53.1% of physicians would initiate or modify treatment in those with diastolic HTN in CKD. Further, even for the same abnormal ABPM parameter, the decision to start or alter treatment was influenced by the underlying medical condition. There is significant variation in clinical practice among physicians for interpretation and management of hypertension when using ABPM. Differences in guidelines among various jurisdictions, as well as knowledge gaps in the research on which guidelines are based, create ambiguity regarding ABPM interpretation and management decisions. A more protocolized approach and further insight into the reasoning behind the variation in physicians' interpretation may help to standardise practice.

The ZFP36 family of RNA binding proteins regulates homeostatic and autoreactive T cell responses.

RNA binding proteins are important regulators of T cell activation, proliferation, and cytokine production. The zinc finger protein 36 (ZFP36) family genes (Zfp36, Zfp36l1, and Zfp36l2) encode RNA binding proteins that promote the degradation of transcripts containing AU-rich elements. Numerous studies have demonstrated both individual and shared functions of the ZFP36 family in immune cells, but their collective function in T cells remains unclear. Here, we found a redundant and critical role for the ZFP36 proteins in regulating T cell quiescence. T cell-specific deletion of all three ZFP36 family members in mice resulted in early lethality, immune cell activation, and multiorgan pathology characterized by inflammation of the eyes, central nervous system, kidneys, and liver. Mice with T cell-specific deletion of any two Zfp36 genes were protected from this spontaneous syndrome. Triply deficient T cells overproduced proinflammatory cytokines, including IFN-γ, TNF, and GM-CSF, due to increased mRNA stability of these transcripts. Unexpectedly, T cell-specific deletion of both Zfp36l1 and Zfp36l2 rendered mice resistant to experimental autoimmune encephalomyelitits due to failed priming of antigen-specific CD4+ T cells. ZFP36L1 and ZFP36L2 double-deficient CD4+ T cells had poor proliferation during in vitro T helper cell polarization. Thus, the ZFP36 family redundantly regulates T cell quiescence at homeostasis, but ZFP36L1 and ZFP36L2 are specifically required for antigen-specific T cell clonal expansion.

Iatrogenic aortic dissection of the descending aorta after percutaneous coronary intervention.

Aortic dissection is a rare and potentially fatal complication of coronary angiography. We report a case of a woman in her late 80s who underwent a left femoral approach coronary angiogram for evaluation of a transcatheter aortic valve replacement (TAVR). Following the procedure, she had a cardiac arrest and was found to have a descending aortic dissection on transoesophageal echocardiogram. Autopsy showed an acute intimal tear of the descending aorta, most likely related to catheter manipulation. Patients undergoing evaluation for TAVR, who tend to be elderly with concomitant atherosclerosis, are at risk for complications following cardiac catheterisation including aortic dissection.

Addressing social risk factors in the inpatient setting: Initial findings from a screening and referral pilot at an urban safety-net academic medical center in Virginia, USA.

Social Determinants of Health (SDOH) impact health outcomes; thus, a pilot to screen for important SDOH domains (food, housing, and transportation) and address social needs in hospitalized patients was implemented in an urban safety-net academic medical center. This study describes the pilot implementation and examines patient characteristics associated with SDOH-related needs. An internal medicine unit was designated as a pilot site. Outreach workers approached eligible patients (n = 1,135) to complete the SDOH screening survey at time of admission with 54% (n = 615) completing the survey between May 2019 and July 2020. Data from patient screening survey and electronic health records were linked to allow for examination of associations between SDOH needs for food, housing, and transportation and various demographic and clinical characteristics of patients in multivariate logistic regression models. Of 615 screened patients, 45% screened positive for any need. Of 275 patients with needs, 33% reported needs in 2, and 34% - in 3 domains. Medicaid beneficiaries were more likely than patients with private health insurance to screen positive for 2 and 3 needs; Black patients were more likely than White patients to screen positive for 1 and 3 needs; Patients with no designated primary care physician status screened positive for 1 need; Patients with a history of substance use disorder screened positive for all 3 needs. SDOH screening assisted in addressing social risk factors of inpatients, informed their discharge plans and linkage to community resources. SDOH screening demonstrated significant correlations of positive screens with race/ethnicity, insurance type, and certain clinical characteristics.