AL amyloidosis: Singapore Myeloma Study Group consensus guidelines on diagnosis, treatment and management.

AL amyloidosis is the most common form of systemic amyloidosis. However, the non-specific nature of presenting symptoms requires the need for a heightened clinical suspicion to detect unexplained manifestations in the appropriate clinical setting. Early detection and treatment are crucial as the degree of cardiac involvement emerges as a primary prognostic predictor of survival in a patient with AL amyloidosis. Following the diagnosis of AL amyloidosis with appropriate tissue biopsies, prompt treatment with a bortezomib, cyclophosphamide and dexamethasone-based first-line induction with or without daratumumab should be initiated. The goal of treatment is to achieve the best haematologic response possible, ideally with involved free light chain <20 mg/L, as it offers the best chance of organ function improvement. Treatment should be changed if patients do not achieve a partial response within 2 cycles of treatment or very good partial response after 4 cycles or after autologous stem cell transplant, as achievement of profound and prolonged clonal responses translates to better organ response and long-term outcomes. Early involvement of multidisciplinary subspecialists such as renal physicians, cardiologists, neurologists, and gastroenterologists for optimal maintenance and support of involved organs is recommended for optimal management of patients with AL amyloidosis.

Death and cardiovascular outcomes in end-stage renal failure patients on different modalities of dialysis.

INTRODUCTION: Cardiovascular morbidity and mortality in end-stage renal failure (ESRF) patients are high. We examined the incidence and predictors of death and acute myocardial infarction (AMI) in ESRF patients on different modalities of dialysis. METHOD: Data were obtained from a population-based database (National Registry Disease Offices) in Singapore. The study cohort comprised all adult patients initiated on dialysis between 2007 and 2012 who were closely followed for the development of death and AMI until September 2014. Cox regression methods were used to identify predictors of death and AMI. RESULTS: Of 5,309 patients, 4,449 were on haemodialysis and 860 on peritoneal dialysis (PD). Mean age of the cohort was 61 (±13) years (44% women), of Chinese (67%), Malay (25%) and Indian (7%) ethnicities. By September 2014, the incidence of all-cause death was 34%; close to a third of the patients died from a cardiovascular cause. Age >60 years and the presence of ischaemic heart disease, diabetes, stroke, peripheral vascular disease and PD were identified as independent predictors of all-cause death. PD patients had lower odds of survival compared to patients on haemodialysis (hazard ratio 1.51, 95% confidence interval 1.35-1.70, P<0.0001). Predictors of AMI in this cohort were older age (>60 years) and the presence of ischaemic heart disease, diabetes, stroke, peripheral vascular disease and current/ex-smokers. There were no significant differences in the incidence of AMI between patients on PD and haemodialysis. CONCLUSION: The short-term incidence of death and AMI remains high in Singapore. Future studies should investigate the benefits of a tighter control of cardiovascular risk factors among ESRF patients on dialysis.

Myocarditis Following COVID-19 Vaccination: A Systematic Review (October 2020-October 2021).

INTRODUCTION: Reports of SARS-CoV-2 coronavirus (COVID-19) vaccine-related myocarditis, particularly after mRNA vaccines, have raised concerns amongst the general public. This review examined the literature regarding myocarditis post COVID-19 vaccination, drawing from vaccine safety surveillance databases and case reports. METHODS: Combinations of search terms were used in PubMed and COVID-19-specific repositories - LitCovid and the Cochrane COVID-19 Study Register - between 1 October 2020 and 31 October 2021. Manual searches of GoogleScholar and screening of article bibliographies were also performed. RESULTS: Information was obtained from five vaccine safety surveillance databases. Fifty-two (52) case reports totalling 200 cases of possible COVID-19 vaccine-related myocarditis were summarised. Vaccine surveillance databases differed in reporting formats and vaccination rates; however, gross estimates suggested low overall incidence rates of 2-5 per million mRNA vaccines. The incidence appeared to be higher in younger male populations, with onset of symptoms within a few days, usually after the second dose. Some with prior COVID-19 infections had onset after the first dose. Cases with prior unrelated myocarditis were also noted. Almost all presented with chest pain (98.0%). Troponin elevation was universally described and cardiac magnetic resonance imaging was commonly reported based on the updated Lake Louise criteria. Clinical course was mild in the majority, with response to anti-inflammatory treatment. CONCLUSION: COVID-19 vaccine-related myocarditis is an important but rare adverse event. More research is needed into its pathogenesis and reasons for its predominance in young males, while gaps in data exist in those aged <16 years, as well as those with prior COVID-19 infections and prior myocarditis.

The role of multimodality imaging in diagnosing acute perimyocarditis secondary to Crohn's disease.

BACKGROUND: Acute perimyocarditis is a rare extra-intestinal manifestation in Crohn's disease which required multimodality imaging to confirm the diagnosis. Here we present a case of acute perimyocarditis as the first presentation of Crohn's disease. To date, this is the first case presentation reporting the use of 18F-FDG PET/CT Scan for diagnosing such condition. CASE PRESENTATION: A 25-year-old male presented to our hospital with severe persistent pleuritic sharp left-sided chest pain. This was his second hospital admission in the past 4 months for chest pain and diarrhea. At the first hospitalization, he was diagnosed with viral perimyocarditis and irritable bowel syndrome. Laboratory findings, electrocardiogram, and cardiac magnetic resonance imaging results confirm the diagnostic of perimyocarditis. Virology, bacteriology, parasitology, and autoimmune evaluations were unremarkable. Colonoscopy, colorectal biopsy, and 18FGD PET findings confirmed manifestation of perimyocarditis, Crohn's disease, and negative for sarcoidosis. CONCLUSIONS: Looking at the overall clinical picture and investigation results of colonoscopy, colorectal biopsy findings, as well as multi-modality imaging with echocardiography, 18FDG PET-scan and CMRI, the patient was diagnosed to have perimyocarditis attending Chron's disease flare up as a rare extra-intestinal manifestation.

Age and diabetes mellitus associated with worse outcomes after percutaneous coronary intervention in a multi-ethnic Asian dialysis patient population.

INTRODUCTION: There is limited literature on clinical outcomes following percutaneous coronary intervention (PCI) in Asian dialysis patients. We evaluated the angiographic characteristics and clinical outcomes of dialysis patients treated with PCI in an Asian society. METHODS: A retrospective analysis was performed of 274 dialysis patients who underwent PCI in a tertiary care institution from January 2007 to December 2012. Data on clinical and angiographic characteristics was collected. The primary endpoint was major adverse cardiac events (MACE), defined as a composite of cardiac death, acute myocardial infarction (AMI) and stroke at two years. RESULTS: 274 patients (65.0% male, median age 62.0 years) with 336 lesions (81.8% Type B2) were treated. 431 stents (35.0% drug-eluting stents) with a mean diameter of 2.96 mm and mean length of 21.30 mm were implanted. The MACE rate was 55.8% (n = 153) at two years, from death (36.5%) and AMI (35.0%). In multivariable analysis, age and diabetes mellitus were significant predictors of both mortality (odds ratio [OR] 1.09, 95% confidence interval [CI] 1.05-1.12, p < 0.001; OR 2.65, 95% CI 1.46-4.82, p = 0.001, respectively) and MACE (OR 1.06, 95% CI 1.03-1.08, p < 0.001; OR 1.84, 95% CI 1.07-3.15, p = 0.027, respectively). Left ventricular ejection fraction (LVEF) (OR 0.97, 95% CI 0.95-0.99, p = 0.006) was a significant predictor of mortality but not MACE. CONCLUSION: Asian dialysis patients who underwent PCI had a two-year MACE rate of 55.8% due to death and AMI. Age, LVEF and diabetes mellitus were significant predictors of mortality at two years.

An unusual case of fulminant leptospiral myocarditis: a case report.

BACKGROUND: Fulminant myocarditis secondary to leptospirosis is rare and associated with poor outcomes. CASE SUMMARY: We describe a 60-year-old gentleman with fulminant leptospiral myocarditis and profound cardiogenic shock requiring veno-arterial extracorporeal membrane oxygenator (VA-ECMO) support. He was given high-dose pulse steroids early on post-VA-ECMO implantation and achieved full recovery. To our knowledge, this is the first reported case of leptospiral myocarditis with multiorgan dysfunction successfully managed by VA-ECMO and high-dose pulse steroids. DISCUSSION: This case report highlights the potential benefits of steroids in the management of leptospiral myocarditis which requires further validation. Early aggressive supportive management with ECMO should be considered in patients with fulminant leptospiral myocarditis.

Mortality outcome and predictive risk factors for death in patients with heart failure and reduced ejection fraction who declined implantable cardioverter defibrillator implantation in Singapore.

BACKGROUND: There have been conflicting data regarding the risk of sudden cardiac death (SCD) in Asian population with reduced left ventricular ejection fraction (LVEF). We aim to study mortality outcome and its risk predictors in patients with reduced LVEF who declined an implantable cardioverter defibrillator (ICD) implantation and assess whether current ICD guidelines for primary prevention are applicable to the population in Singapore. METHODS: This prospective observational study involved 240 consecutive patients who fulfilled the ACC/AHA/HRS criteria for ICD implantation for primary prevention of SCD but declined ICD implantation. Baseline characteristics and mortality outcomes through May 2017 were collected via case-note review after a mean follow-up of 44.8 ± 16.6 months. RESULTS: Majority of our patients were Chinese (71.3%), followed by Malays (16.2%) and Indians (10.8%). Mean age (±SD) was 61 ± 10 years, and 84% were male. Majority were in New York Heart Association (NYHA) functional classes I (46.7%) and II (46.3%). Over a mean follow-up of 44.8 ± 16.6 months, all-cause mortality rate was 34.6%. Diabetes mellitus (HR = 1.57; 95% CI, 1.01-2.44; P = 0.047) and chronic kidney disease (CKD; HR = 1.95; 95% CI, 1.17-3.23; P = 0.010) were independent predictors of mortality. Patients in NYHA classes II (HR = 2.15; 95% CI, 1.32-3.50; P = 0.002) and III (HR = 2.82; 95% CI, 1.34-5.96; P = 0.007) showed higher risk of death. CONCLUSION: The mortality rate was comparable with major primary prevention trials. ICD guideline recommendations for primary prevention may thus be applicable to our local population. Patients with diabetes, CKD, and poorer NYHA status exhibited higher mortality rates.