Severe Dilatation Research Articles

Management of recurrent end-stage achalasia with robotic-assisted esophagectomy: a case report and literature review

Introduction and Importance: Achalasia, an uncommon esophageal motility disorder, presents therapeutic challenges, especially in refractory cases with a history of multiple surgeries. Here, we present a complex case illustrating the dilemmas and multidisciplinary approach required in managing such patients. This case underscores the relevance of newer techniques like robotic-assisted esophagectomy in refractory achalasia management. Case Presentation: A 53-year-old male with recurrent achalasia endured persistent dysphagia, reflux, and esophageal spasms despite undergoing Heller myotomies, fundoplications, and hiatal hernia repairs. Imaging revealed severe esophageal dilation and anatomical alterations post-surgeries. Opting for a robotic-assisted thoracoabdominal esophagectomy due to relentless symptoms, the patient faced technical hurdles due to adhesions and a dilated esophagus. Post-surgery, complications like thoracic duct injury, milky pleural effusion, atrial fibrillation, and limb ischemia arose, necessitating multidisciplinary intervention. Clinical discussion: Managing refractory achalasia poses significant challenges, particularly in extensively operated patients. Despite aggressive surgeries, debilitating symptoms persisted, emphasizing the need for a multidisciplinary approach. Complications like thoracic duct injury and atrial fibrillation further complicate management, highlighting the intricacies of such cases. Careful consideration of surgical options and the potential of newer techniques like POEM are crucial in navigating such complexities. Conclusion: Managing refractory achalasia, especially in patients with extensive surgical histories, requires a multidisciplinary approach and careful consideration of treatment options. This case underscores the evolving landscape of achalasia management and emphasizes the potential benefits of newer techniques like POEM in select cases.

Echogenic bowel on the second-trimester ultrasound as an indicator of jejunal atresia: a case report

Introduction and importance: Fetal echogenic bowel (FEB) is a finding on prenatal ultrasound characterized by increased echogenicity of the fetal intestine. In this study we present a case of FEB with severe bowel dilatation identified on second-trimester ultrasound that was subsequently diagnosed with jejunal atresia postnatally. Case presentation: A 28-year-old primigravida woman was referred to our perinatology clinic at 18 weeks/3 days gestational age for a fetal anomaly scan. The anomaly scan examination identified grade 3 fetal bowel hyperechogenicity. The results of the fetal echocardiogram and maternal serum screening for TORCH infections were normal, and the NIPT analysis showed low-risk results for chromosomal aneuploidies. Clinical discussion: In our case, the fetal bowel diameter measured 21 mm at 29 weeks/6 days gestational age, which significantly exceeded the established clinical cut-off value for fetal bowel dilatation. This timing is crucial because if a specific problem is detected in the ultrasound that indicates an abortion of the fetus, it can only be performed legally under the 19th week of pregnancy; otherwise, termination of pregnancy is considered illegal according to the policies of this country. Conclusion: Nowadays, ultrasonography is a non-invasive tool that facilitates the early detection of many prenatal problems. In this case, with the early detection of FEB and further investigations, we were able to safely manage the pregnancy until the 39th week of pregnancy and plan the delivery in a tertiary referral center to ensure the availability of intensive care for the baby at birth.

The aortic paradox: a nationwide analysis of 523,994 individual echocardiograms exploring aortic dissection

Abstract Background Increasing aortic dilation increases risk of aortic dissection. Nevertheless, dissection occurs below guideline-directed cut-offs for prophylactic surgery. There is no current large-scale population imaging data assessing aortic dimensions before dissection. Purpose To examine the proportion of aortic dissections occurring in people with aortic diameters below guidelines for prophylactic intervention. Methods Patients within the National Echo Database of Australia (NEDA) were stratified according to absolute, height-indexed and body surface area (BSA)-indexed aortic dimensions. Fatal thoracic aortic dissections (ICD-10-AM code I79) were identified via linkage with the National Death Index. Results 524,994 individuals were assessed, comprising patients with normal aortic dimensions (n=460,992), mild dilation (n=53,402), moderate dilation (n=10,029) and severe dilation (n=572). 274,992 (52.4%) were male, with median age 64 years and median follow-up time 6.9 years. 899 fatal aortic dissections occurred (normal diameter=610, mildly-dilated aorta=215, moderately-dilated =53 and severely-dilated=21). Using normal aortas as the reference population, odds of fatal dissection increased with aortic diameter (mild=OR 3.05, 95% confidence interval (CI) 2.61-3.56; moderate=OR 4.0, 95% CI 3.02-5.30; severe=OR 28.72, 95% CI 18.44-44.72). Due to the much larger number of patients without severe aortic dilation, 97.7% of fatal aortic dissections occurred in non-severely dilated aortas. Following sensitivity analysis, severe aortic dilation was responsible for at most 24.4% of fatal aortic dissections. Results were robust for absolute, height-indexed or BSA-indexed aortic measurements. Conclusion Although severe aortic dilatation is associated with a thirty-fold increase in fatal dissection, severely dilated aortas are implicated in only 2.3-24.4% of fatal dissections. This highlights the ‘aortic paradox’ and limitations of current guidelines. Future studies should seek to refine risk predictors in patients without severe aortic dilation.

Distal ascending aortic dilatation portends a near 2-fold greater risk of cardiovascular mortality than proximal enlargement: results from 120,000 patients in a nationwide echocardiographic database

Abstract Background The management of thoracic aortic dilatation is clinically challenging and acute aortic dissection (AD) is under-recognised as a primary cause of death. Despite the majority of AD occurring in non-severely dilated aortas, absolute aortic diameter remains the most utilised criterion for adjudging preventative surgical correction. In order to improve outcomes and guide management, greater prognostic understanding is needed. Purpose We examined a nationwide echocardiographic dataset linked to mortality outcomes to determine how ascending thoracic aortic dilatation and its site affects cardiovascular disease (CVD) mortality over time. Methods The National Echocardiography Database Australia (NEDA) was examined for aortic dimensions at the sinuses of Valsalva (SoV), sinotubular junction (STJ) and ascending aorta (AscAo) in patients who had undertaken >1 echocardiographic study. Following separation of those who had undergone corrective aortic surgery, patients were stratified according to absolute aortic diameters and grouped as normal (<4cm), mild (4.0-4.5cm), moderate (4.5-5cm) and severely (>5cm) dilated at the prescribed thoracic aortic sites. Mortality data was then linked from the National Death Index (NDI). Results 398,905 echocardiographs from 121,115 patients with a mean age of 62.6±16.2 years were included. At the time of mortality data linkage, 38,681 (32%) had died, of whom 13,292 (34%) died from CVD causes and only 276 (0.007%) had aortic dissection recorded as their primary cause of death. Severe aortic dilatation at any site increased the likelihood of 10-year CVD mortality (29% vs. 15% in those with normal diameters; HR 3.00; CI 1.60-5.63; p<0.0001), with an incremental increase in the probability of CVD death when moving from the proximal to distal ascending aorta; 10-year CVD mortality at the SoV 26% (HR 1.93; CI 1.29-2.87; p<0.001), STJ 39% (HR 2.82; CI 1.64-4.86; p<0.001) and AscAo 44% (HR 4.74; CI 2.46-9.13; p<0.001) respectively (Figure 1). When examining those patients who had undergone corrective aortic surgery, the likelihood of 10-year CVD mortality was comparable to the population with normal aortic diameters (17 vs. 15%, HR 1.1; CI 0.9-1.36; p=0.3) (Figure 2). Conclusion Severe aortic dilatation increases the probability of 10-year cardiovascular mortality. Interestingly, there is an incremental increase in the likelihood of CVD mortality when moving from the proximal to distal ascending aorta, with severe AscAo dilatation conferring a near 2-fold greater risk of CVD mortality compared with severe SoV aneurysms. Additionally, surgical aortic intervention nearly normalises survival probability to the population with healthy aortic diameters. These results identify new considerations for prognostication, risk stratification and surgical guidance of aortic repair.CVD mortality by aortic dilatationCVD mortality after aortic intervention

Proteome profiling in patients with peripartum cardiomyopathy according to ethnicity a biomarker study on the EORP cohort

Abstract Background Peripartum cardiomyopathy (PPCM) is a pregnancy-associated form of heart failure, which occurs in previously healthy women towards the end of pregnancy and in the first months postpartum. Previous reports showed a varying prevalence of PPCM across different regions and potentially a higher incidence in patients with black African ethnicity. The correct and timely diagnosis remains challenging and there is a need for novel disease-specific biomarkers. Accuracy in diagnosing PPCM can be improved by using serum-protein biomarkers such as QSOX1, adiponectin and ITIH3 in addition to NT-proBNP. However, it still needs to be ascertained whether there are ethnic differences in proteomic profiles amongst patients with PPCM with different ethnicities. Aims To delineate possible differences in serum protein biomarker profiles across different ethnic groups within the EURObservation Research Programme (EORP) multi-national cohort of women with PPCM. Methods Demographic and clinical data, as well as serum samples were collected from 84 patients with PPCM from seven EORP participating countries. Serum proteomic profiling was conducted using DIA-based label-free quantitative (LFQ) LC-MS at the time of diagnosis from depleted serum samples. Mass spectrometry data were analyzed by Spectronaut v15 using a study-specific spectral library. From the 84 patients in the EORP PPCM registry on whom we performed proteomic analyses, 82 patients self-declared their ethnicity. These 82 patients were assessed for any ethnical differences in their proteomic expression profile using unsupervised principal component analysis (PCA). Results In this cohort of 82 women with PPCM, the mean age was 30.5±6.7 years (Table 1). Three quarters of this cohort had no hypertension during pregnancy and only 13.8% had a prior diagnosis of PPCM. The mean left ventricular ejection fraction (LVEF) was 35% at time of first diagnosis, with no difference between the ethnic groups. However, women of Middle Eastern decent had more severe LV dilatation (LVEDD: 64.0 (62.0-65.0) mm). Three hundred and twenty-nine (329) proteins were identified in the serum samples. As depicted in Figure 1, PCA did not yield a distinct separation between the different ethnic groups. Principal component 1 (PC1) accounted for 15.2% of the total variability in protein expression among ethnic groups, PC2 explained a comparatively lower percentage at 12%. Conclusion The protein biomarkers identified in PPCM patients showed no discernible racial differences, demonstrating consistent performance across all ethnic groups.

Evaluation of aortic inflammation in marfan syndrome patients with 18-fluorodeoxyglucose positron emission and computed tomography

Abstract Background The role of inflammation in Marfan Syndrome (MS) has been well studied in current research. These studies have investigated the role of serum Interleukin 6 (IL-6), Transforming Growth Factor-b (TGF-b) and Macrophage Colony Stimulating Factor (MCSF) as biomarkers of disease severity and progression. Surgical repair is the recommended treatment of aortic aneurysms when aortic diameter reaches the indication threshold. It has been observed however that dissection may occur even before this threshold is reached. 18-fluorodeoxyglucose (18 F-FDG) positron emission tomography / computed tomography (PET/CT) is the gold standard imaging modality to noninvasively assess vascular inflammation. Purpose Our aim was to investigate whether MS patients have increased aortic wall inflammation as assessed by 18F-FDG PET/CT, and the relationship between aortic wall inflammation with serum levels of IL-6, TGF-b and MCSF. Methods The study population consisted of fifteen MS patients and seventeen sex and age matched controls. In all subjects, serum levels of circulating IL-6, TGF-b and MCSF were measured. In PET/CT, the arterial target-to-background ratio (TBR) was calculated for aortic root (AR), ascending aorta(As), aortic arch (AA) and descending aorta (DA). Also, all patients were assessed with transthoracic echocardiography where the diameters of AR, As, AA, and DA were estimated at baseline and were reexamined at the end of the follow up period. Results TBR for MS vs. controls was 2.17±0.75 vs 2.01±0.36, p=0.75 at AR; 2.15±0.67 vs 1.84±0.35, p=0.040 at As; 2.16±0.64 vs 1.70±0.25, p=0.03 at AA and 1.97±0.52 vs 1.83±0.42, p=0.23 at DA respectively. Additionally, Marfan patients had increased levels of serum biomarkers. However, this difference was statistically significant (SS) only for IL-6 serum levels; 2.17±0.46 vs 1.08±0.12 p=0.011 and IL-6 was SS associated with AR TBR (rho=0.572, p=0.033), As TBR (rho=0.604, p=0.022) and DA TBR (rho=0.714, p=0.004). Two of our patients were operated due to severe dilatation of the aortic root and the surgical specimens showed aortic wall inflammation of moderate severity along with fibrosis. Conclusion MS patients have increased of aortic wall inflammation as assessed by 18F-FDG PET/CT, a finding that highlights the role of inflammation in the pathway of disturbed TGF signaling. Further research is needed to determine its prognostic role and value in identifying patients with an indication for early intervention.

Diversity of heart failure phenotypes in transthyretin amyloid cardiomyopathy. More than just heart failure with preserved ejection fraction

Introduction Current guidelines recommend suspecting transthyretin amyloid cardiomyopathy (ATTR-CM) in patients over 65 years of age with unexplained left ventricular (LV) hypertrophy in a non-dilated LV, heart failure (HF) and preserved ejection fraction (HFpEF), hypertrophic cardiomyopathy or severe aortic stenosis. However, there is evidence indicating a high prevalence of ATTR-CM in other HF phenotypes. As such, this study aimed to characterize the diversity of HF phenotypes of ATTR-CM by examining the LV ejection fraction and LV dilatation using echocardiography. Methods This multicentre, retrospective observational study included patients diagnosed with ATTR-CM between 2015–2023. The diagnosis was based on a positive cardiac biopsy or positive bone scintigraphy without monoclonal gammopathy. Echocardiographic measurements were categorized according to LV ejection fraction (LVEF) into HFpEF (LVEF ≥50%), HF with mildly reduced EF (HFmrEF, LVEF 40–49%), and HF with reduced EF (HFrEF, LVEF <40%). LV cavity size was categorized by LV end-diastolic diameter (LVEDD) and volume index (LVEDVi) as normal, moderately increased and severe dilatation. Results The study included 135 patients with ATTR-CM (mean age, 78 years; 89% male; 89% wild-type ATTR-CM). Most patients were screened for ATTR-CM because of unexplained HF and increased LV wall thickness (57%). Echocardiography showed LVEF <50% in 60% of the patients, with a significant portion presenting with HFrEF. Patients with LVEF <50% had higher NYHA class and elevated N-terminal pro-B-type natriuretic peptide levels than HFpEF patients. LV dilatation was observed in 43% of the patients, with 10% presenting with both LVEF <50% and severe LV dilatation. Conclusion This study revealed significant variability in HF phenotypes among patients with ATTR-CM, from HFpEF without LV dilatation to HFrEF with severe LV dilatation. Relying solely on HFpEF for screening may lead to under-diagnosis. These findings suggest the need for more comprehensive diagnostic criteria beyond echocardiographic measures to improve ATTR-CM detection and management.

Proteomic Signatures of Right Ventricular Outcomes in Pulmonary Arterial Hypertension.

Pulmonary arterial hypertension (PAH) is a disease of progressive right ventricular (RV) failure with high morbidity and mortality. Our goal is to investigate proteomic features and pathways associated with RV-focused outcomes including mortality, RV dilation, and NT-proBNP (N-terminal pro-B-type natriuretic peptide) in PAH. Participants in a single-institution cohort with 3 years of follow-up underwent proteomic profiling of their plasma using 7288 aptamers (targeting 6467 unique human proteins). Partial least squares discriminant analysis was performed to assess global protein variation associated with mortality, RV dilation, and NT-proBNP levels. Differentially abundant proteins and enriched pathways associated with outcomes were identified following baseline adjustments. RV vulnerability models estimated associations for individuals with similar afterload following adjustment for pulmonary vascular resistance. A total of 117 participants with PAH were included. Partial least squares discriminant analysis of the proteome showed clear separation between survivors and nonsurvivors, participants with dilated versus nondilated RVs, and across NT-proBNP levels. Proteins and pathways involving the ECM (extracellular matrix) were upregulated in participants who died during follow-up, those with severe RV dilation, and those with higher levels of NT-proBNP. Pulmonary vascular resistance adjustment reinforced the importance of ECM proteins in the association with RV vulnerability, independent of afterload. These findings were confirmed in independent PAH cohorts with available plasma proteomics and RV tissue gene and protein expression. Distinct plasma proteomic profiles are associated with mortality, RV dilation, and NT-proBNP in PAH. Proteins and pathways governing tissue remodeling are strongly associated with poor outcomes, may mediate RV vulnerability to right heart failure, and represent promising candidates as biomarkers and potential therapeutic targets.

"Hot phase" clinical presentation of biventricular arrhythmogenic cardiomyopathy: when the perfect electrical storm spontaneously stops.

An 18-year-old male presented with syncope during a training break. Post-syncope, he developed effort dyspnea, which he associated with the Pfizer-BioNTech COVID-19 vaccine received a week earlier. Electrocardiogram showed T inversion in V1-V3, III, and aVF, while 24-hour Holter monitoring revealed frequent ventricular premature beats. A transthoracic echocardiogram showed severe biventricular dilation and mild left ventricular (LV) dysfunction. Cardiac magnetic resonance (CMR) imaging confirmed these findings, showing moderate right ventricular (RV) systolic dysfunction with akinesia of the inferior and inferolateral walls. T2 hypersignal in the middle segment of the inferior inferior interventricular septum suggested myocardial edema. Extensive transmural late gadolinium enhancement was noted in the RV and LV walls. An implantable loop recorder was implanted. Three months later, the patient was admitted with palpitations, fever, and a positive SARS-CoV-2 test. Sustained ventricular tachycardia (VT) episodes were documented and managed with amiodarone and β-blockers. Follow-up CMR showed a slight improvement in LV ejection fraction and resolution of edema. A single-chamber implantable cardioverter-defibrillator (ICD) was implanted. Genetic testing for arrhythmogenic RV cardiomyopathy (ARVC) was negative, and family screening was normal. Two years later, pre-syncope episodes occurred, and ICD interrogation revealed nonsustained VT. The patient is awaiting VT ablation. This case highlights the diagnostic and therapeutic challenges of ARVC, particularly in differentiating it from myocarditis. The "hot-phase" presentation, vaccine association, and subsequent SARS-CoV-2 infection added complexity. CMR was crucial for diagnosis, and VT management required a combination of medical therapy and invasive procedures.

Therapeutic results of three-dimensional aortic valve anatomic repair for regurgitant bicuspid aortic valve

Objective: To explore the surgical technique and results of three-dimensional aortic valve anatomic repair for bicuspid aortic valve (BAV) with aortic regurgitation (AR). Methods: This is a retrospective case series study. From August 2021 to December 2023, 130 consecutive patients with BAV-AR underwent aortic valve anatomic repair at the Department of Cardiothoracic Surgery, Zhongshan Hospital, Fudan University,and the data were retrospectively analyzed. There were 115 males and 15 females, aged (38.6±11.7) years (range: 15 to 67 years). All patients received modified aortic root reconstruction, to do three-dimensional root remodeling, including the basal ring, sinus of Valsalva and sino-tubular junction simultaneously. Perioperative and follow-up data were collected and analyzed. Comparisons between groups were performed using independent samples t-test, Wilcoxon paired signed-rank test, or χ² test. Results: No patient transferred to valve replacement during the operation. The cardiopulmonary bypass time (M(IQR)) was 109 (34) minutes (range:67 to 247 minutes), and the aortic cross-clamp time was 76 (26) minutes (range: 32 to 158 minutes). Preoperative transesophageal echocardiography showed 123 patients (94.6%) presented with moderate or severe regurgitation. Immediately postoperative transesophageal echocardiography showed no regurgitation in 22 patients (16.9%), trace regurgitation in 81 patients (62.3%) and mild regurgitation in 27 patients (20.8%). Follow up was complete in all patients, with a follow-up of 5.5 (9.4) months (range: 0.1 to 27.6 months). No mortality was observed during follow-up. Echocardiography was obtained in 112 patients at the latest follow-up, including no regurgitation in 4 patients (3.6%), trace regurgitation in 58 patients (51.8%), mild regurgitation in 45 patients (40.2%), moderate regurgitation in 4 patients (3.6%), and severe regurgitation in 1 patient (0.9%). Conclusion: For patients with BAV-AR who have good valve quality and no severe aortic sinus dilation, the recent outcomes of three-dimensional anatomical repair technique, focusing on overall remodeling of the aortic root, are satisfactory.

7768 Hereditary Phaeochromocytoma/Paraganglioma And Aortopathy In A Patient With Known Macroprolactinoma: A Series Of Incidental Findings

Abstract Disclosure: J.R. Kelly: None. S. Soule: None. P.J. Hunt: None. Background: The three P association (3PAs) describes the combination of pituitary adenoma and phaeochromocytoma/paraganglioma. Recent case series describe a range of underlying genetic causes, including SDH mutations. We describe a patient with a macroprolactinoma and their subsequent incidental diagnoses of phaeochromocytoma and paraganglioma, with a pathogenic SDHA mutation. Clinical Case: A 58-year-old man had previously received a combination of medical therapy with cabergoline and transsphenoidal debulking surgery for a histologically confirmed macroprolactinoma. Due to a small risk of valvulopathy associated with cumulative doses of cabergoline, screening echocardiogram was requested after seven years of treatment. This showed a calcified bicuspid aortic valve and severe dilatation of the ascending aorta at 58mm. Prior to surgical repair, CT aorta was requested, which showed an incidental left adrenal lesion. Formal adrenal imaging and functional assessment confirmed a 50mm phaeochromocytoma, with a right para-aortic paraganglioma subsequently revealed on Ga-68 DOTATATE-PET scan. He described symptoms of catecholamine excess, with episodic palpitations, chest pain, and increasing anxiety over the previous year, on a background of hypertension. Hereditary phaeochromocytoma/paraganglioma gene panel revealed a pathogenic mutation in SDHA (c.1663+1G&amp;gt;T) – a splicing variant, likely resulting in nonsense decay. Screening was recommended for his three children. He initiated alpha blockade with doxazosin, then beta blockade with metoprolol, prior to open adrenalectomy and excision of paraganglioma. Histology confirmed a left phaeochromocytoma (Ki67 0.1%, PASS score 8, no vascular or capsular invasion) and a paraganglioma. Post-operative plasma normetanephrine normalised (406 pmol/L, pre-operative 6776 pmol/L, normal &amp;lt;1200 pmol/L) and the patient described resolution of symptoms. He subsequently underwent tissue aortic valve replacement and Bentall’s procedure. Histology showed severe atherosclerosis with diffuse medial degeneration of the aorta. Repeat Ga-68 DOTATATE-PET seven months later showed no evidence of residual or recurrent disease. Ongoing management includes cabergoline, annual plasma metanephrine measurement, and intermittent MRI of pituitary and base of skull to coccyx. Conclusion: Macroprolactinomas are relatively common, with only a small proportion associated with underlying genetic abnormalities. This case describes a patient with 3PAs, initially presenting with a macroprolactinoma, with incidental detection of phaeochromocytoma and paraganglioma and subsequent identification of a pathogenic SDHA mutation. In the context of a calcified bicuspid aortic valve and severe aortic dilatation, pre-operative alpha blockade allowed successful surgical management of both potentially life-threatening conditions. Presentation: 6/3/2024

5128 Role of Plasmapharesis- A Tale of Two Thyroid Storms

Abstract Disclosure: Z. Zhang: None. S. Joseph: None. A.M. Kodali: None. D. Eagerton: None. Background: Thyroid storm is an acute life threatening endocrinological emergency. The standard treatment for a thyroid storm is antithyroid medication, iodine, beta blockers and corticosteroids. However, when patients are refractory to standard treatment, plasmapheresis may be an effective and safe intervention. Case 1: A 49-year-old male with typical symptoms of hyperthyroidism, presented with a Ventricular tachycardia arrest. After the return of spontaneous circulation, he was febrile, and developed new onset atrial fibrillation (AF) and Amiodarone drip was started. Physical exam showed a thin gentleman with an enlarged thyroid. ECHO showed LVEF 50% with moderate to severe dilatation of left atrium and mild mitral and tricuspid valve regurgitation. CT abdomen/pelvis and CTA chest did not show any pathology. Labs showed TSH of 0.02 (reference range: 0.465-4.68); Free T 4 &amp;gt;6.99 (reference range: 0.78- 2.19). He was started on Propylthiouracil (PTU), Hydrocortisone, Potassium Iodide, and Cholestyramine. His blood pressure was too low to tolerate a beta blocker. He developed increased liver enzymes thought to be due to shocked liver. PTU was switched to Methimazole due to less risk of hepatotoxicity. A Thyroid Stimulating immunoglobulin was 13.40 (reference range 0.00-0.55), confirming Graves’ disease. He developed multiorgan failure and plasmapheresis was initiated. His Free T4 level prior to his first apheresis was still &amp;gt;6.99 but improved to 6.20 after two days. After the second session, free T4 level drastically improved to 2.65. He sustained a level of 2.09 until his hospital discharge. Case 2: A 59-year-old male with untreated hyperthyroidism and AF, who presented with shortness of breath. He was tachycardic, febrile and developed AF with rapid ventricular response. A TSH and free T4 level showed &amp;lt;0.02 (reference range: 0.465-4.68); and &amp;gt;6.99 (reference range: 0.78- 2.19), respectively. Physical exam shows a cachectic man with an enlarged thyroid. ECHO showed reduced ejection fraction of 35% with biventricular failure. He was initially started on PTU, Potassium Iodide, hydrocortisone and esmolol drip and eventually Cholestyramine. Patient developed multisystem organ failure and PTU was switched to methimazole due to continued deterioration of his liver enzymes. Plasmapheresis started with a total of 3 sessions. His free T4 improved from 4.32 to 3.59 by the second session. Round three was performed when his Free T4 increased back to 4.56. Unfortunately, he had a sudden decline in mentation and CT head showed an acute/subacute left anterior parietal stroke and after family discussion, was palliatively extubated and expired. Discussion: This case series shows two cases of thyroid storm treated with plasmapheresis and had two stark contrasts. Further studies should be done to elucidate the usefulness of plasmapheresis for thyroid storm when refractory to standard of treatment. Presentation: 6/2/2024

Características de la insuficiencia tricúspide grave inducida por dispositivos de estimulación intracardiacos

Introduction and aimsTricuspid regurgitation (TR) induced by the implantation of cardiac implantable electronic devices (CIED) is an increasingly common cause of severe TR. Our aim was to describe the echocardiographic phenotypic characteristics of CIED-induced severe TR. MethodsRetrospective cohort study that included patients with severe TR related to CIED diagnosed in the cardiac imaging unit of a Spanish tertiary hospital. Results37 patients with severe TR induced by lead/electrode interference formed our study group. TR was predominantly severe (68%), followed by massive (21%) and torrential (11%). The leaflet most affected by the interference was the septal. 58% of the sample presented severe dilatation of the right atrium (RA) (mean RA area 28cm2). Mean tricuspid annulus measurement was 42mm. The usual parameters for quantifying RV systolic function were on average within the normal range (TAPSE mean 19mm, S’ wave 10mm, FAC 41%), while global RV strain (RVGLS -15%) and free wall strain (RVFWLS -19%) were found reduced. An incipient degree of ventricular/pulmonary arterial uncoupling was evident (mean TAPSE/PSAP 0.34, SGLVD/PSAP 0.27%/mmHg). ConclusionsOur patients with CIED-induced severe TR are characterized by a heterogeneous phenotype with a high prevalence of severe RA and tricuspid annulus dilatation. RVGLS, RVFWLS, and arterial ventricular coupling were the most sensitive parameters for early assessment of RV systolic dysfunction.

Excavation-induced cracking of clastic rock: A true triaxial instantaneous unloading study with varied levels of initial damage

The cracking and squeezing problem in deep engineering significantly constrains project construction. To reveal the cracking mechanism of rock under instantaneous unloading, a self-designed rigid true triaxial experimental apparatus, equipped with groundbreaking electromagnetic unloading and high-speed camera functions, was utilized to conduct instantaneous unloading tests on clastic rock with varied levels of initial damage. The results indicate that samples undergo severe and irreversible lateral dilation during instantaneous unloading, with dilational strain rapidly increasing at higher initial damage levels. Instantaneous unloading induces the generation of numerous tensile microcracks, which propagate and coalesce rapidly. The crack propagation speed, as well as the length and width of the cracks at failure, increase with the rise of the initial damage level. The samples eventually exhibit two distinct macroscopic fracture zones: a tensile fracture zone and a tensile-shear mixed fracture zone. Analysis of acoustic emission hits and energy indicates that higher initial damage levels correspond to greater unloading damage, with a higher overall proportion of tensile cracks throughout the experiment. Under the induction of blasting excavation, radial stress is rapidly unloaded, and dense tensile cracks emerge in the immediate surrounding rock, leading to cracking and squeezing towards the free face. Importantly, higher initial damage levels intensify the squeezing effect. The self-developed equipment serves as a crucial technical tool for researching excavation unloading, and the insights derived from this study provide valuable guidance for understanding cracking mechanisms and informing the construction of deep engineering projects.

Right Ventricular Dysfunction in Patients Undergoing High-Risk PCI with Impella

Right ventricular dysfunction (RVD) is an important prognostic factor in several cardiac conditions, including acute and chronic heart failure. The impact of baseline RVD on clinical outcomes of patients undergoing high-risk percutaneous coronary intervention (HRPCI) supported by Impella is unknown. Patients from the single-arm, multicenter PROTECT III study of Impella-supported HRPCI were stratified based on the presence or absence of RVD. RVD was quantitatively assessed by an echocardiography core laboratory and was defined as fractional area change < 35%, tricuspid annular plane systolic excursion < 17 mm or pulsed-wave Doppler S-wave of the lateral tricuspid annulus < 9.5 cm/s. Procedural outcomes, 90-day major adverse cardiac and cerebrovascular events (MACCE: the composite of all-cause mortality, myocardial infarction, stroke/TIA, and repeat revascularization), and 1-year mortality were assessed. Of the 239 patients who underwent RV function assessment, 124 were found to have RVD. Lower left ventricular ejection fraction, higher blood urea nitrogen levels, and more severe RV dilation were independently associated with RVD. The incidence of hypotensive episodes during PCI, the proportion of patients requiring prolonged Impella support, the completeness of revascularization, and the rate of in-hospital mortality did not differ significantly between patients with vs without RVD. However, 90-day MACCE rates were higher in those with RVD, and RVD was a robust predictor of 1-year mortality in multivariable Cox-regression analyses. In patients undergoing HRPCI with Impella, RVD was associated with more advanced biventricular failure. The use of Impella support during HRPCI facilitated effective revascularization, even in those with concomitant RVD. Nevertheless, RVD was associated with unfavorable long-term prognoses.

Clostridioides difficile infections: Update and therapeutic guidelines

Clostridioides difficile infection (CDI) represents a significant challenge due to its increasing incidence, severity, and treatment difficulty. Effective management requires a multifactorial approach that includes preventive strategies, prudent antibiotic use, and adapted therapeutic options. Ongoing research and innovation offer promising prospects for improving ICD management, making vigilance and informed practices essential among healthcare professionals. Two main complications of ICD are pseudomembranous colitis (PMC) and toxic megacolon. PMC involves severe colonic inflammation due to C.difficile toxins, leading to pseudomembrane formation. Diagnosis relies on clinical criteria, microbiological tests, and endoscopy. Toxic Megacolon is characterized by severe colonic dilation and systemic toxicity, requiring immediate medical intervention. ICD diagnosis combines clinical signs and microbiological tests. These tests include toxin tests, GDH antigen detection, PCR for toxin genes, and stool culture. Imaging techniques assess colonic inflammation and complications. Combined diagnostic criteria from the American Gastroenterological Association (AGA) and European guidelines emphasize integrating clinical and laboratory findings for accurate diagnosis. ICD treatment involves stopping the implicated antibiotics and starting specific antimicrobial therapy. Common treatments include mainly fidaxomicin and oral vancomycin. Fecal microbiota transplantation (TMF) is recommended for recurrent cases unresponsive to standard treatments. Bezlotoxumab, an antibody targeting C.difficile toxin B, is used to prevent recurrence in high-risk adults. ICD poses a major challenge due to its increasing incidence, severity, and difficulty in treatment. A multifactorial approach involving rigorous preventive strategies, prudent antibiotic management, and adapted therapeutic options is essential for controlling the infection. Ongoing research and innovations in treatment offer promising prospects for improving patient management. Healthcare professionals must remain vigilant and informed to ensure effective practices in combating this infection and utilizing available resources optimally.

Grade IV gastric ulceration, pyloric aplasia, and an ectopic duodenal papilla in a 13-year-old Quarter Horse gelding evaluated for recurrent colic.

The objective of this Case Report was to describe the clinical manifestation and postmortem findings of a horse evaluated for recurrent colic diagnosed with pyloric aplasia and an ectopic duodenal papilla. 13-year-old Quarter Horse gelding. The horse was presented to a referral center for signs of colic that were unresponsive to management on the farm. No abnormalities were noted on rectal palpation; however, upon passage of a nasogastric tube, 31 L of net gastric reflux was obtained. Gastroscopy noted grade IV squamous ulceration and moderate glandular ulceration, severe gastric dilatation, an abnormally positioned gastric outflow tract, and an ectopic duodenal papilla leaking bile-like fluid directly into the stomach. The horse was initially treated with gastroprotectants, promotility agents, and dietary modifications. Following treatment failure and repeated episodes of colic with increasing severity, the horse was euthanized and a necropsy performed. At necropsy, it was confirmed that the aboral aspect of the stomach formed a dilated pouch and continued directly into the proximal duodenum with no discernible pyloric sphincter. The area of the pylorus was delineated by a faint indentation. The ectopic duodenal papilla was traced to the liver. Congenital gastric abnormalities, even in adult horses, should be considered as differential diagnoses when investigating horses presenting with a history of chronic colic. Neither pyloric aplasia nor an ectopic duodenal papilla have been described in the equine literature, and this Case Report raises awareness of the presence of such malformations and their manifestations.

Abnormal circadian rhythms exacerbate dilated cardiomyopathy by reducing the ventricular mechanical strength.

Dilated cardiomyopathy (DCM) has etiological and pathophysiological heterogeneity. Abnormal circadian rhythm (ACR) is related to the development of DCM in animal models, but exploration based on clinical samples is lacking. Sleep apnea (SA) is the most common disease related to ACR, and we chose SA as the study object to explore ACR-DCM. We included a derivation cohort (n =105) and a validation cohort (n = 65). DCM patients were divided into SA and without SA group. RT-qPCR was used to determine the change of rhythm gene expression pattern of heart samples from different timepoints. We used single-nucleus RNA sequencing (snRNA-seq) to explore the abnormal transcriptional patterns in the ACR group, and we verified the findings by pathological staining, atomic force microscopy (AFM), and Rev-erbα/β knockout (KO) mice analysis. DCM patients with SA showed decreased amplitude of rhythm gene expression. SA group showed more severe dilation of left heart chambers. From snRNA-seq, ACR-DCM lost the morning transcriptional patterns, detailly, actin cytoskeleton organization of cardiomyocytes (CMs) disrupted and hypertrophy aggravated, and the proportion of activated fibroblasts (Fibs) decreased with the reduction of fibrotic area ratio. The results of pathological staining, mechanical experiments, and transcriptional feature of Rev-erbα/β KO mice supported the above findings. Compared with the non-SA group, left ventricular (LV) wall dilation was more severe and the structural strength was lower in DCM patients with SA, and phenotypic changes in CM and Fib were involved in this process. ACR-DCM was histopathologically characterized by a structurally weak ventricular wall.

Effects of aqueous seed extract of &lt;i&gt;Garcinia kola&lt;/i&gt; on spray paint-induced histological changes in the lungs of Wistar rats

The most common cause of lung diseases among people with spray paint-related occupations is hydrocarbon poisoning which has significant morbidity and mortality if left untreated. This study aimed to investigate the effects of aqueous seed extract of Garcinia kola on spray paint-induced interstitial pneumonitis in the adult Wistar rats. Thirty adult Wistar rats weighing between 240 g and 270 g were divided into five groups of six rats each. Animals in group A were placed on feed and water only. Animals in group B were exposed to 100 ml of spray paintfumes via inhalation. Animals in group C received 500mg/kg body weight per day of Garcinia kola. Animals in group D were exposed to 100 ml of spray paint fumes via inhalation and received 250mg/kg body weight per day of Garcinia kola. Animals in group E were exposed to 100 ml of spray paint fumes via inhalation and received 500mg/kg body weight per day of Garcinia kola. The extract was administered for 30 consecutive days via an orogastric tube. Groups A, C, D and E revealed normal histoarchitecture of the lung; normal alveoli and normal interstitial space. There were observable histological variations in the lung tissues of Group B rats which include severe alveolar dilation, interstitial haemorrhage, interstitial infiltrates of inflammatory cells, severe vascular ulceration and congestion (evidence of interstitial pneumonitis). It is concluded that Garcinia kolahasan ameliorative effect on spray paint-induced histological changes in the lungs of Wistar rats.

Endoscopic balloon dilatation of primary obstructive megaureter: An effective first line management in children

This study evaluates outcomes of endoscopic balloon dilatation (EBD) in the management of primary obstructive megaureter (POM) in children. Retrospective data between 2013 and 2023 from two tertiary paediatric surgical centres in the UK were reviewed. Pre and post-operative clinical and imaging parameters of children managed with EBD were assessed. Failure of procedure was defined as requiring further intervention due to persistent/recurrent symptoms, upper tract dilatation and/or obstruction on MAG3 over the follow up period. 55 children with 61 renal units were evaluated. Median age at treatment was 18 months with a median follow up of 24 months. There was significant reduction in upper tract ultrasound measurements following balloon dilatation but there was no significant difference between the pre and post-operative renal function on MAG3. No significance difference was demonstrated when the outcomes of cutting and non-cutting balloons were compared. No significant difference was shown when outcomes after EBD were compared between infants vs older children as well as ureteric dilatation less than or over 25mm (p=0.841). 87% were successfully treated with a single dilatation and this increased to 95% after second dilatation. The remaining 5% had ureteric re-implantation. Although a retrospective study, the patient population is relatively large. 87% success rate shown after EBD is comparable to similar studies. It has been suggested that children less than 12months and those with severe ureteric dilatation (>25mm) may not be suitable for EBD. No significant difference was demonstrated when the outcomes of these categories of children were compared to other children with POM. All of the patients that had repeat balloon dilatation required no further intervention, a finding that has so far not been well evaluated in available literature. This study demonstrates 87% success rate after single EBD in children with POM and this outcome increased to 95% following a second dilatation. EBD is shown to be an effective definitive surgical management option of POM. It can be safely offered as first line management in all patient groups and repeated if no initial response.