Systemic light-chain (AL) amyloidosis is a rare but lethal plasma cell disorder characterized by amyloid deposition from misfolded immunoglobulin light chains, often leading to multi-organ dysfunction. Cardiac involvement is the predominant driver of prognosis and survival. A 66-year-old man presented with an eight-month history of progressive exertional dyspnea, fatigue, and bilateral leg edema. His past medical history included controlled hypertension and diabetes mellitus. Physical examination revealed signs of advanced heart failure, including elevated jugular venous pressure (12 cm H₂O), bibasilar crackles, peripheral edema, and neuropathy. Electrocardiography showed low-voltage QRS complexes in the limb leads, and echocardiography demonstrated concentric left ventricular hypertrophy (septal thickness 16 mm) with preserved systolic function. Strain imaging displayed an apical-sparing “cherry-on-top” pattern, and cardiac MRI revealed markedly increased extracellular volume, suggesting amyloid infiltration. Endomyocardial biopsy confirmed amyloid deposition, exhibiting Congo red positivity with apple-green birefringence and lambda light chain restriction. The patient was diagnosed with systemic AL amyloidosis and advanced cardiac involvement. He was treated with bortezomib, cyclophosphamide, and dexamethasone, and later escalated to daratumumab due to limited hematologic response. Despite aggressive therapy, he developed refractory heart failure and died 29 months after diagnosis. This case highlights the subtle early signs of cardiac amyloidosis and underscores the critical importance of recognizing and treating it promptly before irreversible organ damage develops.

Abstract Background/Aims There is an increased mortality rate associated with RA. Previously, cardiovascular disease (CVD) has been identified as the major cause of mortality in patients with RA. The management of CVD has markedly improved over recent decades, with the recognition of acute coronary syndrome using high sensitive troponin assays and the early use of coronary artery stenting and bypass surgery. Enhanced therapies for cardiac failure have further reduced cardiovascular mortality. Consequently the causes of death in patients with RA may have shifted over the past two decades. Methods The data in this study were collected as part of project IRAS ID 194833, approved by South West regional ethical committee (UK). The cohort consisted of men attending routine rheumatology clinics at the Royal Cornwall Hospital, Cornwall, UK, from February 2015 to August 2016. All patients fulfilled 2010 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) RA criteria at diagnosis. Data were anonymised at source. The men were followed up for 10 years and mortality was recorded; where possible the cause of mortality was ascertained from hospital notes or death certificates. Results A total of 247 deaths were recorded over a 10-year period in a cohort of 667 males with RA. Therefore 37% of the cohort of RA males died over a 10-year period. The annualised death rate was 3.7 %/year. Two hundred deaths were analysed further. The single most common type of death was respiratory in origin. There were 67/200 (33.5%) respiratory deaths. In addition, there were 15 deaths due to lung cancer 15/200 (7.5%). Any other type of cancer accounted for 35/200 (17.5%) deaths. CVD only accounted for 12/200 (6%) deaths. The rest of the deaths (71/200 [35.5%]) were because of frailty, dementia, fracture of the femur and sepsis. Conclusion Respiratory disease and lung cancer account for 41% of all the deaths. CVD only accounted for 6% of deaths. Greater emphasis should be placed on the respiratory system by routinely inquiring about respiratory symptoms at each consultation and performing regular chest auscultation to detect any basal crepitations. The use of hand held spirometers in clinic should be encouraged, along with a strong focus on joint rheumatology and respiratory clinics, smoking cessation programmes and ensuring that Influenza, pneumococcal, and COVID -19 vaccinations are up to date. Disclosure M. Dissanayake: None. D. Hutchinson: None.

BACKGROUND Lipomatous atrial septal hypertrophy (LASH) is a benign but uncommon condition characterized by excessive adipose tissue accumulation within the interatrial septum. Although most cases remain asymptomatic, large lesions may produce superior vena cava (SVC) obstruction. Acute pulmonary embolism as an initial presentation is rare. CASE REPORT A 75-year-old woman with a history of chronic obstructive pulmonary disease and hypertension experienced 7 days of progressively worsening exertional dyspnea, orthopnea, and lower-extremity edema, without chest pain, cough, or fever. Physical examination findings included tachypnea, hypertension, bilateral lower-extremity edema, elevated jugular venous pressure, and bibasilar crackles. Computed tomography pulmonary angiography - prompted by elevated D-dimer - demonstrated bilateral pulmonary emboli and a large LASH causing SVC obstruction with near-complete obliteration of the right atrium. Transthoracic echocardiography confirmed the presence of LASH. The absence of personal or family history of inherited thrombophilia, along with negative deep venous thrombosis findings on lower-extremity Doppler ultrasonography, suggested that pulmonary embolism and right heart failure were related to sluggish right atrial flow secondary to LASH-induced mechanical SVC obstruction. Clinical improvement was achieved with anticoagulation and diuretic therapy, followed by partial surgical resection of the interatrial septum in conjunction with coronary artery bypass grafting. Histopathologic examination confirmed LASH without evidence of inflammation or malignancy. CONCLUSIONS Symptomatic LASH causing SVC obstruction is extremely uncommon and rarely associated with pulmonary embolism. This case underscores the importance of recognizing LASH as a potential contributor to right heart failure and thromboembolism; it highlights echocardiography as an essential diagnostic modality when pulmonary embolism is suspected.

Renal artery stenosis is the major cause of renovascular hypertension, can be found isolated or have association with syndromes or autoimmune conditions. It can be due to atherosclerotic deposition in vessels, autoimmune etiology or fibromuscular dysplasia, the latter is the commonest cause, however, needs biopsy for diagnosis and exclusion of other possible causes. Patients can have diverse presentation, disease may be discovered as incidental finding, with hypertensive crises, Nephropathy or cardiac manifestations. We report a case of 7 years female patient presented with severe respiratory distress, hypertension, Grade 1 BL pitting edema, hepatomegaly, basal crackles in chest and gallop. He had no history of any diagnosed medical condition prior. However, symptoms were evident for last 2 months with Headache, fatigue, cough, exertional dyspnea, edema and decreased urine output. She had no history of arthralgia, frothy urine, hematuria or any skin rashes. Evaluation for Hypertension revealed small sized right kidney with decreased perfusion in segmental arteries. Echocardiography showed Biventricular dilation, dysfunction with Ejection fraction of 40%. She initially managed with iv labetalol infusion later started enalapril and amlodipine then referred to specialty where percutaneous transangioplasty planned abut deferred by pediatrics nephrology as patient responded with medical management and it is considered for resistant hypertension or persistent high renin and aldosterone. The case concluded with diagnosis of Reno cardiac type-iv syndrome, as manifestations are concordant with chronic kidney disease – right SSK due to hypoperfusion that resulted in renovascular hypertension and Cardiac failure.

Acute pulmonary edema following routine fluid administration is an uncommon but potentially life-threatening complication in adults with sickle cell disease (SCD). We report the case of a 42-year-old male with known SCD who presented with severe generalized body pain consistent with vaso-occlusive crisis (VOC). Initial management included intravenous normal saline for mild dehydration. Shortly after receiving approximately 500 mL, he developed acute shortness of breath, hypoxia, and fine basal crackles. Chest imaging demonstrated bilateral alveolar infiltrates consistent with pulmonary edema. Laboratory evaluation revealed baseline anemia, mild leukocytosis, elevated creatinine, and trace proteinuria, suggestive of subclinical sickle nephropathy. Echocardiography showed normal cardiac function, excluding primary cardiogenic causes. The patient received supplemental oxygen, cautious diuretics, and pain control while fluid administration was restricted. Transfusion support was provided to improve oxygen-carrying capacity and reduce sickling risk. Multidisciplinary management included hematology, nephrology, and respiratory care teams. The patient’s respiratory status gradually improved, and pulmonary edema resolved without further complications. This case highlights the risk of flash pulmonary edema triggered by standard intravenous fluids in patients with SCD and previously undiagnosed renal impairment. It underscores the importance of individualized fluid management, careful monitoring, and early recognition of subclinical sickle nephropathy. Clinicians should exercise caution with routine fluid boluses in VOC, even in patients without overt kidney disease, as modest fluid volumes may precipitate life-threatening pulmonary complications. Prompt identification and tailored therapy can prevent morbidity and guide safe management of VOC in adults with SCD.

Background: Heart failure (HF) is a major cause of morbidity and mortality worldwide, with a growing burden in sub-Saharan Africa. Data remain limited in Cameroon, particularly among older adults. This study aimed to describe the epidemiologic, clinical, and paraclinical profile of geriatric patients admitted for HF in two hospitals in Yaoundé. Methods: We conducted a cross-sectional study with a retrospective and prospective phase of data collection over six months (November 2021-April 2022) in the cardiology units of the Yaoundé Central Hospital and the Yaoundé General Hospital which are on our pyramid of our national health care facilities on the top. We aimed then to carry out this study in those highest level of care facilities. Patients aged ≥65 years with a diagnosis of HF based on clinical and echocardiographic criteria were included. Sociodemographic, clinical, geriatric, and paraclinical data were collected after informed patient consent. Analyses were performed using SPSS 23.0, with results expressed as means and proportions. The hospital prevalence was calculated by the ratio of patients above 65 years old admitted for heart failure over all the patients above 65 years old admitted in the selected hospitals during our period of study. We did not include in the study patients aged less than 65 years old, incomplete records and medical files without a clear diagnosis of deep veinous thrombosis (DVT) and or pulmonary embolism (PE). Results: 267 elderly patients have been admitted in those two hospitals among which 92 presented with heart failure. We included 63 elderly patients with HF, giving a hospital prevalence of 34.5% (95% CI: 28.9 - 40.2). The mean age was 75.0 ± 6.4 years. Hypertension (81.0%) and sedentary lifestyle (74.6%) were the most frequent risk factors. Acute decompensated HF was the commonest presentation (38.1%), and therapeutic non-compliance the leading precipitating factor (83.3%) for decompensation. Dyspnea (95.2%) and peripheral edema (82.5%) predominated as symptoms at presentation. On examination, jugular venous distension, hepatomegaly, ascites, basal crackles, and pulmonary congestion were frequently found. Frailty was the most common geriatric syndrome (42.9%). The majority of patients (58.7%) had HF with a preserved LVEF. Conclusion: In our context, studies on heart failure are common but to the best of our knowledge, this is the first study focusing on elderly patients. We have then found out that, the hospital prevalence of heart failure in elderly patients was 34.5% with a confidence interval of 28.9% - 40.2% which was high in our milieu due the high prevalence of uncontrolled hypertension among those group of patients. Dyspnea (95.2%) and peripheral edema (82.5%) was the predominated symptoms at presentation. Frailty was the most common geriatric syndrome (42.9%). The majority of patients (58.7%) had Left Hypertrophy associated with a preserved LVEF.

Toxicity due to calcium channel blocker overdose presents as vasodilatory shock that may be refractory to vasopressors and inotropes. Serious toxicities have also been reported with angiotensin II receptor blocker overdose, which may present as persistent refractory hypotension. The treatment options to reverse vasoplegic shock in such cases include conventional vasopressors, high dose insulin, intravenous calcium, terlipressin, and methylene blue. We report a case of a 60-year-old man who presented with decreased responsiveness following intentional ingestion of 85 mg of amlodipine and 350 mg of losartan. He was hypotensive, dyspneic and had bilateral basal crepitations at presentation. He was anuric for six hours following presentation. He was resuscitated with noradrenaline and vasopressin infusion, intravenous calcium, and high-dose insulin euglycemia therapy, highlighting the possible role of such therapies in such cases.

Dyspnea in adolescents is often underestimated as a simple respiratory infection, yet it may represent the fairst sign of complex autoimmune multiorgan disease. This report highlights the autoimmune mechanisms underlying dyspnea to promote early recognition of autoimmune disease in adolescents. An 18-year-old female presented with acute worsening of chronic dyspnea. She was tachypneic hypertensive, hypoxemic, and severe anemia. Examination revealed pallor, edema, basal fine crackles, and gallop. Laboratory findings showed renal impairment with proteinuria and hematuria. The albumin was normal and blood gas indicated respiratory alkalosis with compensatory metabolic acidosis. Infectious work-up revealed yeast-like fungi and mixed gram-positive cocci/gram-negative bacilli, while GeneXpert MTB was negative. Chest radiograph demonstrated pulmonary edema with pneumonia. Electrocardiography showed sinus tachycardia 119 bpm with right axis deviation and right ventricular hypertrophy. Echocardiography revealed right atria and ventricle enlargement, moderate tricuspid regurgitation, mild pericardial effusion, intermediate pulmonary hypertension, and preserved EF 64%. ANA test was positive, but performed only at discharge before referral for definitive immunological workup. The patient's life-threatening multiorgan dyspnea could be concluded as four major mechanisms, such as : 1) Renal & Volume Overload; 2) Cardiopulmonary Vasculopathy; 3) Autoimmune and Infectious Pulmonary Damage; 4) Severe Anemia. The profound multiorgan involvement and severe systemic inflammation provided a strong clinical rationale for highly active systemic autoimmune disease (SAID), most probable being Systemic Lupus Erythematosus. This case illustrates how adolescents' dyspnea with complicated systemic symptoms deserves an early diagnosis of autoimmune disease without waiting for infectious exclusion, to prevent the devastating consequences associated with diagnostic latency and delayed targeted intervention. As life-threatening dyspnea in an adolescent may be the catastrophic initial presentation of Probable Systemic Lupus Erythematosus.

Description of Case: Giant coronary artery aneurysms (CAAs) are rare and pose diagnostic and therapeutic challenges, particularly when asymptomatic or incidentally discovered. Right atrial (RA) compression due to CAA is extremely uncommon and lacks standardized management guidelines. We report a 59-year-old man with prior inferior ST-elevation myocardial infarction (2009) and known right coronary artery (RCA) ectasia, presenting with dyspnea at rest and new-onset atrial fibrillation. Physical examination revealed hypoxemia, bilateral lower extremity edema, and bibasilar crackles. Transthoracic echocardiography showed mild biventricular dysfunction and biatrial enlargement. Pulmonary computed tomography angiography excluded thromboembolism but incidentally identified a 6.6 × 6.4 cm proximal RCA aneurysm. Coronary computed tomography confirmed RA compression without tamponade or restrictive physiology. Right heart catheterization showed normal pulmonary artery pressures, excluding pulmonary hypertension. The patient remained hemodynamically stable and was initially discharged on optimal medical therapy. Coronary angiography revealed RCA occlusion with retrograde perfusion via left anterior descending artery collaterals. After multidisciplinary Heart Team discussion, conservative management was initially considered. However, due to aneurysm size and risk of complications, an endovascular approach was pursued. Methods: The patient underwent percutaneous intervention under conscious sedation, utilizing dual arterial access (right femoral and left radial). The procedure included selective RCA catheterization, balloon occlusion testing proximal to the aneurysm, and deployment of a vascular plug at the RCA ostium. Discussion: Selective RCA catheterization was achieved, and a 0.014-inch guidewire was advanced into the aneurysmal sac. Balloon occlusion was performed proximal to the aneurysm to test for tolerance, followed by deployment of a vascular plug at the RCA ostium, achieving aneurysm exclusion. The patient remained stable throughout, with no clinical or electrical instability. Post-procedural imaging confirmed cessation of flow into the aneurysmal sac, without new compressive or ischemic complications. This case highlights the importance of individualized, multidisciplinary management in rare giant CAAs. In selected patients with preserved hemodynamics and suitable anatomy, endovascular exclusion with vascular plugs offers a safe and effective alternative to surgery.

The clot thickens: Diagnosing APS in the setting of multifocal thrombosis

Abstract Introduction Scleromyositis is a rare overlap syndrome characterised by features of systemic sclerosis and inflammatory myopathy, often complicated by interstitial lung disease (ILD). It presents a diagnostic challenge due to its variable clinical manifestations and overlap with other systemic and neoplastic conditions. We report the case of a 67-year-old female who initially presented with weight loss, dyspnoea, and mediastinal lymphadenopathy—features mimicking malignancy or sarcoidosis. Extensive investigations and multidisciplinary input ultimately led to a diagnosis of scleromyositis with ILD. This case highlights the importance of diagnostic vigilance and tailored immunosuppressive therapy in managing complex connective tissue disease presentations. Case description A 67-year-old female nurse from South Africa presented with a 5-month history of progressive exertional dyspnoea (NYHA class II), unintentional weight loss (9 kg), and mild anaemia. She had no history of smoking, TB exposure, recent infections, drug use, or environmental triggers (e.g. birds or mould). Initial gastrointestinal investigations (OGD and colonoscopy) were unremarkable. CT thorax and abdomen revealed bilateral ground-glass opacities and mediastinal lymphadenopathy. Pulmonary function tests confirmed a restrictive ventilatory defect (FEV1/FVC ratio 116%), with TLCO 41% and preserved KCO (105%). PET-CT demonstrated metabolically active thoracic and upper abdominal lymphadenopathy, raising suspicion for sarcoidosis or lymphoma. Cervical mediastinoscopy and mediastinal lymph node biopsies showed reactive follicular hyperplasia. Castleman’s disease was excluded following histopathology review. EBUS-guided sampling and bone marrow biopsy were non-diagnostic, showing no granulomas or malignancy. The patient was referred to rheumatology due to systemic symptoms. Clinical examination revealed bibasal crepitations, proximal muscle weakness (MMT8 score 136/150), polyarthralgia, digital vasculopathy, and marked skin thickening (Rodnan skin score 27/51). Serology revealed positive ANA and anti-Scl-70 antibodies with elevated inflammatory markers (ESR >120, CRP 30) and CK (599). MRI (STIR sequences) confirmed inflammatory myopathy affecting the quadratus femoris and adductor muscles bilaterally. Echocardiogram showed no evidence of pulmonary hypertension. Urine studies indicated mild proteinuria. A diagnosis of systemic sclerosis with inflammatory myopathy (scleromyositis) and associated interstitial lung disease (SSc-ILD) was established. She received six intravenous infusions of cyclophosphamide (750 mg each) alongside IV methylprednisolone as remission therapy, followed by tapering oral prednisolone and initiation of mycophenolate mofetil at 2gram/day. While repeat PET-CT showed interval reduction in lymph node activity, her systemic symptoms, shortness of breath and polymyalgia symptoms have shown partial response. She is currently under multidisciplinary review for escalation of immunosuppressive therapy. Notably, she developed right-sided hearing loss, suspected to be an adverse effect of cyclophosphamide. Discussion This case highlights the diagnostic and therapeutic challenges of managing systemic sclerosis with overlapping inflammatory myopathy and interstitial lung disease (SSc-ILD). The patient initially presented with weight loss, dyspnoea, and mediastinal lymphadenopathy—features raising concern for lymphoma or sarcoidosis. An extensive workup, including PET-CT, endoscopy, EBUS, and mediastinoscopy, revealed metabolically active lymph nodes, but histology was non-diagnostic, ruling out malignancy, granulomatous disease, and Castleman’s disease. This emphasises the importance of maintaining diagnostic flexibility and avoiding premature anchoring. The subsequent rheumatological evaluation revealed features of skin tightening, Raynaud’s phenomenon, digital vasculopathy, and proximal muscle weakness. Positive ANA and anti-Scl-70 antibodies, elevated inflammatory markers, and MRI-confirmed myositis established the diagnosis of systemic sclerosis with overlap myopathy. Lung involvement was supported by restrictive spirometry and a severely reduced TLCO (41%), highlighting ILD as a key contributor to her morbidity. She received six cycles of intravenous cyclophosphamide with corticosteroids. While PET-CT showed some reduction in lymph node activity, her symptoms persisted. Mycophenolate mofetil was introduced as a steroid-sparing agent but was limited by gastrointestinal side effects—a common challenge in connective tissue disease management. Notably, she developed right-sided hearing loss, suspected to be an adverse effect of cyclophosphamide, warranting ENT referral. This underscores the need for close monitoring of treatment-related toxicity. This case illustrates the value of multidisciplinary collaboration—including rheumatology, respiratory, radiology, pathology, and ENT—in guiding diagnosis and treatment planning. For refractory cases, biologics or antifibrotic agents may offer further options. Ultimately, this case reinforces the importance of a patient-centred, flexible, and collaborative approach to managing complex autoimmune disease with multi-system involvement. Key learning points • Scleromyositis is a clinically significant overlap syndrome combining features of systemic sclerosis and inflammatory myopathy. It presents with skin fibrosis, proximal muscle weakness, and often interstitial lung disease (ILD), requiring a high index of suspicion for timely diagnosis. • Atypical or non-specific presentations can mimic malignancy or granulomatous disease. In this case, lymphadenopathy and weight loss led to an initial workup for lymphoma or sarcoidosis. Histology was essential in excluding these differentials. • Myositis can present subtly in systemic sclerosis. Proximal muscle weakness, raised creatine kinase, and MRI changes supported the diagnosis, despite a negative myositis-specific antibody panel. MRI remains a valuable tool when serology is inconclusive. • ILD is a major driver of morbidity in scleromyositis. This case showed restrictive spirometry and reduced TLCO (41%), highlighting the need for early respiratory involvement and baseline pulmonary function assessment. • Immunosuppressive therapy must be individualised. Cyclophosphamide is often used as first-line therapy for SSc-ILD with myositis. However, partial response and side effects—including cyclophosphamide-induced hearing loss—demonstrate the need for alternative strategies. • Mycophenolate mofetil, although commonly used, may be limited by tolerability. Gastrointestinal intolerance in this patient emphasised the importance of ongoing evaluation of benefit versus adverse effects. • Multidisciplinary team input is essential, involving rheumatology, respiratory, radiology, pathology, and ENT, especially in complex or refractory cases. • Newer therapies, including rituximab or antifibrotics, may be considered in treatment-resistant disease. • This case reinforces the need for diagnostic flexibility, multidisciplinary care, and early recognition of overlap syndromes, to optimise outcomes in patients with complex autoimmune conditions like scleromyositis.

Abstract Introduction Anti-Melanoma Differentiation-Associated gene 5 dermatomyositis (anti-MDA5 DM) is a distinct subtype of idiopathic inflammatory myopathy, often associated with clinically amyopathic features and rapidly progressive interstitial lung disease (RP-ILD). Early symptoms may resemble seronegative rheumatoid arthritis, complicating early diagnosis. COVID-19 infection adds another layer of complexity, both as a potential trigger for autoimmune phenomena and as a source of overlapping pulmonary pathology. This overlap can result in delayed recognition and treatment of potentially life-threatening autoimmune pathology. We present a case of anti-MDA5 DM with ILD in which an initial misdiagnosis of COVID-19 pneumonitis delayed appropriate intervention. Case description A 38-year-old male from Liberia presented in March 2022 with progressive shortness of breath, fatigue, arthralgia, and generalised weakness. He was initially evaluated by rheumatology for possible undifferentiated inflammatory arthritis and autoimmune ILD, and commenced on oral corticosteroids and methotrexate. In July 2022, he was hospitalised with worsening breathlessness and a non-productive cough. He tested positive for COVID-19. CT pulmonary angiogram (CTPA) revealed scattered airspace opacities and ground-glass changes, interpreted as COVID pneumonitis. Methotrexate was withheld, and he received dexamethasone. One month later, he was re-admitted with chest pain. CT imaging demonstrated diffuse reticular changes, suggestive of fibrotic lung disease. Multidisciplinary discussion in an ILD meeting concluded ongoing post-COVID interstitial changes. Pulmonary function testing revealed a restrictive defect. He remained on steroids. A repeat CT chest in September 2022 continued to show extensive basal fibrotic changes. Autoimmune screening, including rheumatoid factor, anti-CCP, ANCA, and ANA, returned negative. He was referred to an ILD specialist clinic, where he received intravenous methylprednisolone (1 g for 3 days). A myositis panel subsequently revealed anti-MDA5 antibody positivity. By March 2023, on rheumatology review, he described new dermatological symptoms including hyperpigmented, scaling rashes over the face, hands, and feet; tender palmar papules; and ulcerated lesions on the knuckles. He also reported polyarthritis with morning stiffness involving MCPs, PIPs, wrists, elbows, knees, and feet. Examination revealed erythematous, tender nailfolds; synovitis of multiple MCPs; bilateral Gottron’s papules; and healing rashes across the forehead, nasolabial folds, and cheeks. Fine basal crackles were noted on chest auscultation. A final diagnosis of anti-MDA5 positive dermatomyositis with associated ILD was made. He was treated with two cycles of rituximab, commenced on mycophenolate mofetil, and a steroid taper was initiated. Follow-up pulmonary function tests showed gradual improvement, alongside clinical and dermatological symptom resolution. Discussion Anti-MDA5 dermatomyositis is a distinct phenotype of dermatomyositis, typically presenting with minimal muscle involvement but prominent cutaneous features and a high risk of rapidly progressive interstitial lung disease. Without early diagnosis and aggressive treatment, MDA5-associated ILD can have high mortality. During the COVID-19 pandemic, there has been increasing recognition of phenotypic overlap between COVID-19 and autoimmune conditions like dermatomyositis, including respiratory symptoms, ground-glass changes on imaging, and cutaneous manifestations. This diagnostic ambiguity can delay timely immunosuppression in patients with underlying autoimmune disease. In this case, the initial assumption of post-COVID pneumonitis obscured the underlying MDA5-associated ILD. Despite treatment for presumed viral pneumonitis, the patient’s condition failed to improve. The eventual identification of MDA5 antibodies, in conjunction with cutaneous signs and progressive respiratory decline, confirmed the autoimmune diagnosis. This case reinforces the importance of maintaining a broad differential in post-COVID patients, especially those with persistent symptoms and atypical radiological features. Early use of myositis-specific panels, particularly in patients with subtle cutaneous or articular features, may help detect MDA5-positive dermatomyositis before irreversible pulmonary damage occurs. Key learning points 1. Anti-MDA5 dermatomyositis may present with non-specific symptoms, cutaneous findings, and interstitial lung disease, with little or no muscle involvement. 2. COVID-19 infection and anti-MDA5-associated ILD can present with similar respiratory and imaging features, making early differentiation difficult. 3. Delayed diagnosis of MDA5-positive DM due to misattribution of symptoms to COVID-19 pneumonitis can lead to missed treatment windows for aggressive immunosuppression. 4. Myositis-specific antibody testing should be considered in patients with ILD, skin rashes, and joint involvement, especially when standard treatment for viral or inflammatory conditions fails. 5. Early multidisciplinary input and immunosuppressive therapy are key to improving outcomes in MDA5-DM with RP-ILD.

Background Brain natriuretic peptide (BNP) is widely used as a marker of cardiac dysfunction and a good predictor for both total and cardiovascular disease mortality in end-stage kidney disease patients. BNP levels are elevated in hemodialysis (HD) patients not only due to impaired kidney function but also because other variables such as left ventricular mass index, presence of cardiovascular disease, and volume overload are independent risk factors for high BNP in those patients. This study aims to assess the predialysis BNP levels and intradialytic BNP reduction values as markers of clinically assessed volume status and cardiac dysfunction in HD patients. Patients and methods The study was carried out on fifty maintenance HD patients in the Mansoura Nephrology and Dialysis Unit (MNDU). Complete physical examination before and after the mid-week HD session, including blood pressure, body weight, lower limb (LL) edema, lung auscultation, and neck vein evaluation. Laboratory investigations, including routine investigations, predialysis and postdialysis blood urea, with calculation of urea reduction ratio. Measurement of plasma levels of BNP before and after mid-week HD session using specific ELISA kits. Left ventricular mass was measured by echocardiography, and left ventricular mass index was calculated. Results There is a statistically significant decrease in BNP level after the HD session. There is no statistically significant difference between patients with left ventricular hypertrophy (LVH) and those without LVH as regards predialysis, postdialysis, and intradialytic reduction levels of BNP. Patients with low predialysis BNP levels below 100 pg/ml show a statistically significant decrease in postdialysis urea compared with those with high BNP levels above 100 pg/ml. However, there is a nonsignificant decrease in LVH and a nonsignificant increase in ejection fraction in low BNP level patients. Predialysis BNP shows a significant negative correlation with predialysis and postdialysis weight, while it shows a significant positive correlation with heart rate and serum albumin. The predialysis BNP is a good predictor for LL edema with area under the curve (AUC)=0.827 and P =0.006. The intradialytic BNP reduction level is an excellent predictor for LL edema (AUC=0.925, P <0.001), basal lung crepitations (AUC=0.982, P <0.001), and hypertension (AUC=0.965, P <0.001). Conclusion The presence of LVH in maintenance HD patients does not affect the level of predialysis, postdialysis, and intradialytic BNP reduction levels. The predialysis BNP may be a good predictor for LL edema, while the intradialytic BNP reduction level is an excellent predictor for LL edema, basal lung crepitations, and hypertension in maintenance HD patients.

Background: Laubry–Pezzi syndrome perimembranous ventricular septal defect (VSD) complicated by aortic cusp prolapse and aortic regurgitation (AR) creates high-velocity jets that injure aortic leaflets and may predispose to infective endocarditis (IE). Case: A 28-year-old woman with poor dental status presented with acute decompensated heart failure (NYHA IV dyspnea, orthopnea, bibasal crackles, ankle edema) and a high-pitched diastolic murmur with accentuated P2. She was afebrile but had inflammatory anemia and elevated inflammatory markers. Blood cultures yielded Staphylococcus hominis. Transthoracic echocardiography identified a restrictive perimembranous VSD (4 × 2 mm) on the aortic surface of the right anterior cusp findings consistent with Laubry–Pezzi syndrome complicated by IE. Whole-body CT showed homogeneous hepatomegaly without embolic foci. Targeted antimicrobial therapy was initiated (ceftriaxone 2 g/day for 4 weeks plus gentamicin 4 mg/kg once daily for 2 weeks). Management and outcome: After clinical stabilization, a heart-team strategy was planned for surgical closure of the VSD with concomitant aortic valve repair (preferred in young patients) or replacement if repair proved unfeasible, aiming to control infection, treat severe AR, and prevent recurrence. Conclusion: In adults with perimembranous VSD and new or worsening AR, Laubry–Pezzi syndrome should be suspected and actively searched for on echocardiography. Poor oral health warrants a low threshold for IE work-up. Early multidisciplinary referral for combined surgical correction is essential to limit valve damage and reduce the risk of recurrent IE.

Introduction: Dilated cardiomyopathy is the most common cause of congestive heart failure in patients with different cardiac insults. This study aims to explore the signs, symptoms, and outcome of dilated cardiomyopathy in the tertiary level of health care of Nepal. Materials and Methods: It was a prospective observational study in a cardiology department of the National Medical College, Birgunj. This study was conducted on 152 patients admitted to the cardiology department of the medical college. Demographic data was gathered from each patient in the performed sheet at the presentation time. Signs and symptoms, electrocardiographic and echocardiographic findings were also recorded. Patients’ outcomes were recorded in terms of mortality discharge toward, and leave against medical advice. The descriptive data are presented as the number and percentage for categorical data and mean ± standard deviation for continuous data according to their distribution. Results: One hundred and fifty-two patients were included in this study. 84(55.3%) were males and 68(44.7%) were females. Mechanical ventilation was used in 36.8% and inotropes in 73.0% of patients. Chest pain, cough, dyspnea, swelling of limbs, and palpitation were present in 8(5.26%), 27 (17.76%), 64 (42.1%), 29 (19.07%), 9(5.9%) of patients. Bilateral Infiltrates, Cardiomegaly, Normal, and Consolidation in left lower zone were present in 29 (19.07%) 107 (70.39%), 12 (7.89%), 4(2.63%) in our study. 98(64.5%) were discharged to home, 52(34.2%) expired and 2(1.3%) of patients underwent leave against medical advice. The use of inotropes, mechanical ventilation, and presence of cardiomegaly on chest x-rays was associated with mortality. Conclusion: The most common presentation was cough and basal crackles. Cardiomegaly, RS pattern with Left Bundle Branch Block, and global hypokinesia were the most common radiological presentations.

This work investigates the impact of transverse confinement on the separation of binary positive liquid components in thermogravitational columns through theoretical, numerical, and experimental approaches. Using the three-dimensional parallel flow approximation, the theoretical analysis reveals that in the steady-state, the species separation increases both by the decrease in the transverse aspect ratio of the cavity (B) and by the increase in the product of the Rayleigh number (Ra) and the Lewis number (Le). In particular, we determined a specific value (Ra Le)c depending on B, which marks the regime where convection overtakes vertical diffusion. In this state, the separation ratio of species obtained from three-dimensional analysis and that predicted by Furry–Jones–Onsager (FJO) theory coincides, regardless of the thermophysical properties of the mixture and the imposed temperature difference between the vertical walls. Using optical digital interferometry, experiments were carried out in thermogravitational microcolumns with transverse aspect ratios B = 5.88 and B = 1.69 for 1,2,3,4-tetrahydronaphthalene |dodecane (C12) and 1,2,3,4-tetrahydronaphthalene|isobutylbenzene mixtures (mass fraction 0.50|0.50) considering three different Ra Le conditions. Reasonable agreement is observed among the experimental, analytical, and numerical results. Thus, we propose a correlation law for steady-state species separation. This approach offers an alternative to the commonly used FJO theory and provides a reliable method for determining the thermodiffusion coefficient from steady-state species separation measurements.

Haemochromatsis occurs due to iron over load. Thalassaemia major is an inherited disorder that can cause secondary haemochromatosis due to haemolysis and repeated blood transfusion. The secondary haemochromatosis in thalassaemia patient can rarely present with restrictive cardiomyopathy. Here, we report a case, where a 30-year-old woman with thalassaemia major presented with shortness of breath with paroxysmal nocturnal dyspnea, orthopnoea , oedema, skin pigmentation, raised Jugular venous pressure(JVP), cyanosis and bilateral basal crepitation. After through investigations, she was diagnosed as a case of heart failure due to restrictive cardiomyopathy with secondary haemochromatosis as a complication of repeated blood transfusion. She was treated with iron chelation therapy, furosemide, beta-blockers, angiotensin receptor blocker (ARB) and discharged with follow up. Iron chelation therapy should be imitated early to prevent secondary haemochromatsosis and its complications in thalassaemia major patient. University Heart Journal 2024; 20(2): 72-76

Heart failure (HF) and iron deficiency anemia (IDA) are both common in the elderly, especially in India, and the relative paucity of data about the same makes their relationship worthwhile to study. Also, as IDA, especially nutritional, is relatively simple to manage, it can help in reducing disease burden significantly. To examine the clinical and etiological profile of patients with HF in the elderly age-group and correlate HF with serum iron, serum ferritin, and percentage transferrin saturation (%TSAT). The present prospective, observational sectional study was carried out in a tertiary care hospital, for a duration of 1 year. The study population included 138 elderly patients admitted to medicine wards and intensive care unit (ICU) having HF. Among the 138 patients with HF included in the study [41.30% in the age-group of 61-65 years, 70.29% males, 35.51% having ejection fraction <30%, 47.83% belonging to New York Heart Association (NYHA) class III], the most frequent clinical symptom among the study subjects was breathlessness (98.55). The most frequent clinical sign was basal lung crepitations (93.48%). The most common etiological factor was ischemic heart disease (56.52%). There were higher serum iron levels and serum %TSAT in patients with better ejection fraction as well as better functional class. No significant association was found between hemoglobin levels and ejection fraction or between hemoglobin levels and NYHA class. There were lower serum ferritin levels in patients with better ejection fraction. No significant association was found between serum ferritin levels and NYHA class. Prevalence of ID was found to be 73.19% in the present study. Absolute and functional ID was reported in 48.55% and 24.64% of the subjects, respectively. Absolute or functional ID had the highest prevalence in subjects having ejection fraction <30% and least in subjects having ejection fraction 45-50% and >50%, which was statistically significant. Absolute or functional ID was revealed maximum in NYHA class IV while it was found least in class I. ID was associated with severe and more symptomatic HF in the elderly. The results highlight that iron supplementation through diet and medicines will help to decrease mortality, morbidity, and financial burden of the healthcare system.

Background: Ischaemia or infarction occurring due to Coronary Artery Disease (CAD) causes left ventricular systolic and Diastolic dysfunction (DD). DM speeds up the process of coronary atherosclerosis as well as functional and structural cardiac impairments, exhibiting DD at the early stages. Severity of CAD is best assessed by coronary Angiography (CAG). Early diagnosis of this condition is warranted as the mortality risk from it is high. The objective of this study was to detect the CAD in diabetic chronic stable angina with grade IV DD. Material and Methods: 74 Diabetic patients with chronic stable angina with grade-IV diastolic dysfunction were included in this cross-sectional observation study which was done in department of Cardiology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh from July 2023 to June 2024. Results: 32 (43.24%) subjects in rural and 42 (56.75%) subjects in urban areas. Proportion of males was 55%. Mean age of study population was (43 + 4.50) years. Mean age(57+7.0 vs 40+8.21years) in rural was significantly higher than urban subjects. 69 (51.06%) were SOB, 26 (19.24%) were tachycardic, 14 (10.36%) had bradycardia, 59 (43.66%) had hypotension, 21(15.54%) had raised JVP, 19(14.06%) had basal crepitation, 19(14.06%) had dependent oedema, 7(5.18%) had hepatomegaly and only 6(4.44%) had Ascites. Most of the dyspneic patients were in NYHA III stage. Around 30% patients were right dominant coronary artery. Most patients had significant stenosis in both LAD and LCX. About one third patients had insignificant stenosis in RCA. Conclusion: There is a relationship between coronary artery disease and Grade IV DD.

Abstract An 88–year–old woman with a history of atrial fibrillation and amoxicillin allergy presented progressive dyspnea. She had been treated in the previous three months for pneumonia and bronchitis. Physical examination revealed basal crackles and a 4/6 systolic murmur. Echocardiography identified mobile vegetations in the mitral and aortic valves (Figure 1), severe mitral regurgitation and leaflet perforation of the mitral valve (Figure 2-3). Laboratory findings included elevated levels of NT–proBNP and fibrinogen. Blood cultures were negative, but valve tissue culture identified Micrococcus luteus, which plausible entered in the bloodstream through a skin or mucosal injury. Empiric antibiotic therapy with ceftriaxone and vancomycin was started. The patient underwent a successful mitral valve replacement with a bioprosthesis and aortic valve shave through endoscopic right minithoracotomy. The postoperative recovery was uneventful, and the patient completed a 6–week antibiotic course. Follow–up echocardiography showed stable prosthesis function and significant symptomatic improvement. This case highlights the evolving microbiology of infective endocarditis associated with gram–positive catalase–positive cocci, typically considered a skin contaminant. Clinicians should recognize Micrococcus luteus as a potential pathogen in susceptible patients and ensure a timely diagnosis and intervention to optimize results.Figure 1 Figure 2 Figure 3