Catheter ablation of atrial arrhythmias in cardiac amyloidosis: Impact on heart failure and mortality.

BACKGROUND: Atrial arrhythmias (AA) commonly affect patients with cardiac amyloidosis (CA) and are a contributing risk factor for the development of heart failure (HF). This study sought to investigate the long-term efficacy and impact of catheter ablation on HF progression in patients with CA and AA. METHODS: Thirty-one patients with CA and AA undergoing catheter ablation were retrospectively included (transthyretin-ATTR CA 61% and light chain-AL CA 39%). AA subtypes included atrial fibrillation (AFib) in 22 (paroxysmal in 10 and persistent in 12), atrial flutter (AFl) in 17 and atrial tachycardia (AT) in 11 patients. Long-term AA recurrence rates were evaluated along with the impact of sinus rhythm (SR) maintenance on HF and mortality. RESULTS: AA recurrence was observed in 14 patients (45%) at a median of 3.5 months (AFib n = 8, AT n = 6, AFl = 0). Post-cardioversion, medical therapy or catheter ablation, 10 patients (32%) remained in permanent AA. Over a median follow-up of 19 months, all-cause mortality was 39% (n = 12): 3 with end-stage HF, 5 due to late complications of CA, 1 sudden cardiac death, 1 stroke, 1 COVID 19 (and one unknown). With maintenance of SR following catheter ablation, significant reductions in serum creatinine and natriuretic peptide levels were observed with improvements in NYHA class. Two patients required hospitalization for HF in the SR maintenance cohort compared to 5 patients in the AA recurrence cohort (p = 0.1). All 3 patients with deaths secondary to HF had AA recurrence compared to 11 out of the 28 patients whom were long-term survivors or deaths not related to HF (p = 0.04). All-cause mortality was not associated with AA recurrence. CONCLUSION: This study demonstrates moderate long-term efficacy of SR maintenance with catheter ablation for AA in patients with CA. Improvements in clinical and biological status with positive trends in HF mortality are observed if SR can be maintained.

Being a Better Starfish Thrower, Carpal Tunnel Syndrome, and Cardiac Amyloidosis: The Hand Surgeon's Opportunity to Make a Profound Difference.

In recent years, there is increasing literature in cardiac and hand surgery journals demonstrating a stronger association between seemingly idiopathic carpal tunnel and amyloidosis. Despite this, it can be difficult for hand surgeons to identify who need biopsies, and this is further complicated by the cost of a biopsy and the low likelihood that a patient has cardiac amyloidosis. In patients with cardiac amyloidosis and carpal tunnel syndrome (CTS), CTS is typically diagnosed 5-10 years prior. Early diagnosis of cardiac amyloidosis is crucial, as current medications work to slow disease progression, but do not treat existing amyloid deposits. Hand surgeons can play an essential role in early diagnosis. The patient case discussed describes a man who had a carpal tunnel biopsy because of his bilateral CTS, recurrent trigger fingers, and his age. After confirmation of amyloidosis, he was referred for cardiac amyloidosis evaluation. Testing confirmed this diagnosis, and he was started on tafamidis, which studies show provide patients an opportunity for increased survival and quality of life. The responsibility falls on cardiologists and hand surgeons to continue refining the indications for carpal tunnel biopsy and spreading awareness of carpal tunnel biopsy and amyloid testing, as much work is still needed.

[A Case of Transverse Colon Cancer with Gastrointestinal Amyloidosis Prevented from Anastomotic Leakage by Multidimensional Approach].

The patient was a 68-year-old woman who was on hemodialysis due to systemic amyloidosis and nephrotic syndrome. Biopsy revealed amyloid deposition in the stomach, duodenum, and colon. A transverse colon tumor was found on a follow- up CT after the aortic dissection surgery. We performed lower gastrointestinal endoscopy and contrast-enhanced CT and diagnosed transverse colon cancer with gastric wall infiltration(cStage Ⅲc). We considered that transverse colon resection was oncologically sufficient. However, due to concurrent gastrointestinal amyloidosis, which increased the risk of anastomotic leakage we performed laparoscopic extended right hemicolectomy to avoid colon-colon anastomosis with partial gastrectomy. Additionally intraoperative indocyanine green(ICG)fluorescence imaging showed that the fluorescence signal in the small intestinal wall was satisfactory, while it was weak in the colon wall. As a result, we suspected of impaired blood flow of colon wall due to an amyloidosis, so we additionally created a loop ileostomy. It is said that gastrointestinal amyloidosis raises the risk of anastomotic leakage. A case of transverse colon cancer complicated by gastrointestinal amyloidosis in which we successfully prevented anastomotic leakage through a multidimensional evaluation and approach is reported, along with a literature review.

Kidney Transplantation in Patients With AA Amyloidosis: Outcomes in a French Multicenter Cohort.

RATIONALE & OBJECTIVE: Outcomes of kidney transplantation for patients with renal AA amyloidosis are uncertain, with reports of poor survival and high rates of disease recurrence. However, the data are inconclusive and mostly based on studies from the early 2000s and earlier. STUDY DESIGN: Retrospective multicenter cohort study. SETTING & PARTICIPANTS: We searched the French national transplant database to identify all patients with renal AA amyloidosis who underwent kidney transplantation between 2008 and 2018. EXPOSURES: Age, cause of amyloidosis, use of biotherapies, and C-reactive protein levels. OUTCOMES: Outcomes were all-cause mortality and allograft loss. We also reported amyloidosis allograft recurrence, occurrence of acute rejection episodes, as well as infectious, cardiovascular, and neoplastic disease events. ANALYTICAL APPROACH: Kaplan-Meier estimator for mortality and cumulative incidence function method for allograft loss. Factors associated with patient and allograft survival were investigated using a Cox proportional hazards model and a cause-specific hazards model, respectively. RESULTS: 86 patients who received kidney transplants for AA amyloidosis at 26 French centers were included. The median age was 49.4 years (IQR, 39.7-61.1). The main cause of amyloidosis was familial Mediterranean fever (37 cases; 43%). 16 (18.6%) patients received biotherapy after transplantation. Patient survival rates were 94.0% (95% CI, 89.1-99.2) at 1 year and 85.5% (77.8-94.0) at 5 years after transplantation. Cumulative incidences of allograft loss were 10.5% (4.0-17.0) at 1 year and 13.0% (5.8-20.1) at 5 years after transplantation. Histologically proven AA amyloidosis recurrence occurred in 5 transplants (5.8%). An infection requiring hospitalization developed in 55.8% of cases, and there was a 27.9% incidence of acute allograft rejection. Multivariable analysis showed that C-reactive protein concentration at the time of transplantation was associated with patient survival (HR, 1.01; 95% CI, 1.00-1.02; P=0.01) and allograft survival (HR, 1.68; 95% CI, 1.10-2.57; P=0.02). LIMITATIONS: The study lacked a control group, and the effect of biotherapies on transplantation outcomes could not be explored. CONCLUSIONS: This relatively contemporary cohort of patients who received a kidney transplant for AA amyloidosis experienced favorable rates of survival and lower recurrence rates than previously reported. These data support the practice of treating these patients with kidney transplantation for end-stage kidney disease. PLAIN-LANGUAGE SUMMARY: AA amyloidosis is a severe and rare disease. Kidney involvement is frequent and leads to end-stage kidney disease. Because of the involvement of other organs, these patients are often frail, which has raised concerns about their suitability for kidney transplantation. We reviewed all patients with AA amyloidosis nephropathy who underwent kidney transplantation in France in the recent era (2008-2018) and found that the outcomes after kidney transplantation were favorable, with 85.5% of patients still alive 5 years after transplantation, a survival rate that is comparable to the outcomes of patients receiving a transplant for other forms of kidney diseases. Recurrence of amyloidosis in the transplanted kidney was infrequent (5.8%). These data support the practice of kidney transplantation for patients with AA amyloidosis who experience kidney failure.

Localized amyloidosis of the ureter: a case report.

BACKGROUND: Amyloidosis is a collection of disorders characterized by the extracellular deposition of amyloid, a specialized fibrous protein, in diverse tissues, leading to functional impairments. CASE PRESENTATION: A 70-year old Asian-Japanese female was referred to our department for further examination of her left hydronephrosis come from lower ureteral obstruction. Contrast enhanced CT and retrograde pyelo-nephrography revealed left ureteral tumor. Though ureteroscropic biopsy did not show malignant pathological findings, ureteroscopic image suspected malignant disease, thus nephroureterectomy was performed. Pathological findings revealed localized ureteral amyloidosis. Whole body examination including gastro endoscopy and cardio ultrasonography could not reveal amyloidosis except ureter. She was free from recurrence 9 months postoperatively. CONCLUSION: We herein report a rare case of localized ureteral amyloidosis.

Coronary artery bypass grafting for triple vessel disease in cardiac amyloidosis.

Cardiac amyloidosis is a rare condition with an estimated incidence of 18-55 per 100 000 person-years. It is associated with either immunoglobulin light chain (AL) or transthyretin amyloid (ATTR), both of which result in a restrictive cardiomyopathy complicated initially by diastolic dysfunction and subsequently followed by biventricular systolic heart failure. Untreated cardiac amyloidosis carries an extremely poor prognosis with an estimated median survival time of less than 1 year in AL and 4 years in ATTR amyloidosis. This is the sixth described report of coronary artery bypass grafting in patients with underlying cardiac amyloidosis.

Surgical Management of Breast Amyloidosis.

Amyloidosis is characterized by extracellular deposition of insoluble misfolded beta-pleated proteins. Amyloid disease involving the breast is rare and there is a paucity of literature guiding surgical management in caring for these patients. In this article we review medical and surgical management with an emphasis on post mastectomy breast reconstruction. We propose an algorithm for breast reconstructive options based on unique considerations in this patient population. An institutional database at the Medical College of Wisconsin was used to identify patients diagnosed with breast amyloidosis from 2011 to 2021. We utilized the electronic medical record to present patient demographics, diagnostic and treatment data regarding the medical and surgical management of these patients. Five women were identified with a median age of 70 years and a median follow up of 19 months (range, 9-80 months). All patients were diagnosed with light chain (AL) type of amyloidosis. Systemic amyloidosis was identified in 3 patients and localized disease was identified in 2 patients. Concurrent breast malignancy was identified in 2 patients who underwent skin-sparing mastectomies followed by breast reconstruction with both prosthetic and autologous techniques. Both prosthetic and autologous reconstructive techniques are safe in patients with amyloidosis, however careful consideration and preoperative work-up are warranted to avoid complications in this vulnerable population. Further studies are warranted to improve surgical outcomes in patients with amyloidosis involving the breast.

Surgical treatment of laryngeal amyloidosis: a systematic review.

PURPOSE: The aim of this review was to study the surgical management of laryngeal amyloidosis and estimate the rate of recurrence after surgery. METHODS: A systematic review searching PubMed and EMBASE was performed. A qualitative synthesis of data regarding the surgical management of LA and a quantitative analysis of the recurrence rate after surgery was conducted. RESULTS: This systematic review included 14 retrospective studies, one of whom is retrospective controlled. A total of 515 subjects were included, the mean age ranged from 43.3 to 58 years with a male-to-female ratio of 1:1.3. All cases had a localized laryngeal amyloidosis. The supraglottic region was the most affected laryngeal site and multiple sites were commonly involved. Surgical treatment consists of endoscopic excision using laser, cold or powered instruments. Open surgery is required for severe primary case or revision surgery. Surgical complications such as granulomatosis scar tissue formation, tracheostomy, laryngotracheal stenosis, pneumothorax and concomitant malignancy were developed in 17.5% of patients. The time onset to diagnosis varied from 1 months to 15 years and the duration of follow-up from 3 months to 25 years. The rate of recurrence was 28.4% (95% CI 24.5-32.6) and the timing of recurrences ranged from 3 months to 10 years. CONCLUSION: The recurrence rate after primary surgery for laryngeal amyloidosis is high. A tailored surgical treatment based on the disease extension and a long-term follow up are recommended.

Outcomes in patients with cardiac amyloidosis undergoing heart transplantation: the eurotransplant experience.

BACKGROUND: When advanced heart failure occurs in cardiac amyloidosis, prognosis is poor. In this setting heart transplantation (HTX) is a treatment option for selected patients. We here present the results of post-transplantation outcomes in cardiac amyloidosis within the Eurotransplant area, investigating possible predictors of survival. METHODS: Of 115 patients undergoing HTX due to cardiac amyloidosis in the Eurotransplant region between November 1987 and May 2020, detailed assessment prior to transplantation was available in 85 patients. The present study was conducted in a retrospective approach. Primary endpoint was mortality after HTX. Baseline variables were entered in a Cox proportional hazards model with the primary endpoint as a dependent variable. RESULTS: Median overall survival following HTX was 6.3 years in the overall collective and the subgroup. Univariate Cox proportional hazards model revealed a significant relationship between overall survival and the transplantation period (2008 to 2020 vs 1987 to 2007; median survival 9.7 years vs 1.8 years, hazard ratio 0.45, p = 0.01). Further predictors were albumin concentration (hazard ratio 0.92, p < 0.001), and systolic blood pressure (hazard ratio 0.96, p < 0.001). The transplant period as well as albumin concentration remained significant independent predictors in the AL sub cohort in a multivariate Cox proportional hazards model. CONCLUSIONS: HTX is a viable treatment option for patients at an advanced stage of cardiac amyloidosis as overall survival after transplantation has improved in the modern age. Patients at a very advanced stage of the disease, indicated by low serum albumin and blood pressure, show worse outcomes following HTX. Optimal timing and careful patient selection may therefore be particularly important to further improve post-HTX survival in amyloidosis patients.

Systemic amyloidosis as a cause of chronic diarrhea after gastric surgery. Literature review.

Amyloidosis with gastrointestinal involvement is a rare cause of chronic diarrhoea, and should be considered especially in adult patients with intestinal malabsorption and extra GI manifestations. We present the case of a male patient who, after an oncological gastrectomy, presented with chronic diarrhea that did not respond to treatment and, after study, the cause of the diarrhea was finally found. Primary systemic light chain amyloidosis (AL) initially presents as chronic diarrhea and weight loss after gastrectomy. Immunohistochemistry was completely negative for amyloid A, which virtually rules out AA amyloidosis. With regard to the immunoglobulin chains, an amyloid signal was observed for both light chains, with a predominance of lambda light chain but not entirely conclusive. This situation is not uncommon since amyloid, whatever its chemical nature, can annex serum proteins, including immunoglobulin chains. In the case of chronic diarrhea, the possibility of amyloidosis should be kept in mind, especially in the case of weight loss.

Long-term results of kidney transplantation in patients with familial mediterranean fever.

INTRODUCTION: Long-term kidney transplantation (KT) results in patients with familial Mediterranean fever (FMF)-related amyloidosis are not well studied. This study reviewed the long-term survival outcomes of FMF patients who underwent KT. METHODS: We compared the outcomes of 31 patients who underwent (KT) for biopsy-proven amyloidosis secondary to FMF with 31 control patients (five with diabetes mellitus and 26 with nondiabetic kidney disease) undergoing KT between 1994 and 2021 at Baskent University Hospital. All data were recorded retrospectively from patients' files. RESULTS: THE MEDIAN AGE (QUARTILE DEVIATION: QD) at the time of KT in the FMF and control group were 31 (6.7) and 33 (11), respectively. The median follow-up period (QD) after KT was 108 (57) months in the FMF and 132 (72) months in the control group. In the FMF group, graft and patient survivals were 71% and 84% at 5 years and 45% and 48% at 10 years, respectively. In the control group, graft and patient survivals were 79% and 100% at 5 years and 63% and 71% at 10 years, respectively. Patient survival in the FMF group at 5 years was significantly lower than in the control group (p = .045). There was no statistically significant difference between the FMF and control groups in terms of graft and patient survival, and serum creatinine levels at 10 years. All patients were given triple immunosuppressive treatment with cyclosporine, mycophenolate mofetil, and prednisolone. Three patients received anakinra and one received canakinumab in addition to colchicine treatment. One FMF patient also underwent heart transplantation due to AA amyloidosis. Of the FMF patients, 11 died during follow-up. CONCLUSION: We have found that the long-term outcome of KT in patients with FMF amyloidosis is numerically worse but not statistically different from the control group. However, short- and long-term complications still need to be resolved.

Initial experience with transcatheter tricuspid valve repair in patients with cardiac amyloidosis.

AIMS: Wildtype transthyretin amyloid cardiomyopathy is an under-recognized cause of heart failure in elderly patients. Transcatheter tricuspid valve repair is a newly emerging therapeutic option for severe tricuspid regurgitation (TR). We present first insights into safety and possible benefits of this procedure in patients with cardiac amyloidosis. METHODS AND RESULTS: Eight patients with cardiac non-hereditary (wildtype) transthyretin (ATTRwt) amyloidosis and severe to torrential TR, undergoing successful transcatheter tricuspid valve repair, were included in the analysis and compared to a control group of 21 patients without cardiac amyloidosis. All patients presented with an advanced stage of amyloid cardiomyopathy. Primary endpoint was reduction in TR at 3 months follow-up. Secondary endpoints were feasibility, safety, hospitalization or death, clinical improvement, cardiac biomarkers, and structural and functional right heart parameter obtained by echocardiography. Transcatheter tricuspid valve repair resulted in a significant reduction of TR (IV to II, P = 0.008) in all eight patients with cardiac amyloidosis (100%). Device success (amyloidosis 75% vs. control group 86%, P = 0.597) and overall probability of hospitalization or death (amyloidosis 13% vs. control group 25%, P = 0.646) were similar compared with those in the control group at 3 months follow-up. Transcatheter tricuspid valve repair led to an improvement of New York Heart Association functional class (P = 0.031) and 6 min walking distance (from 313 ± 118 to 337 ± 106, P = 0.012). TR reduction in amyloidosis patients was less extensive compared with that in control group (TR-reduction 1.6 ± 0.3, P = 0.008 vs. control group 2.3 ± 0.3, P < 0.0001). Furthermore, these patients showed no significant improvement of structural right heart parameters. CONCLUSIONS: Transcatheter tricuspid valve repair is a safe and feasible new treatment option in patients with amyloid cardiomyopathy and has the potential to improve TR-grade and clinical status. However, the benefit appears to be less pronounced compared with patients without cardiac amyloidosis.

Amyloidosis in the nasopharynx and larynx: a case report.

Amyloidosis is a disease caused by amyloid deposition in tissues or organs. According to the extent of the lesion, it can be divided into systemic amyloidosis and localized amyloidosis. Amyloidosis originating in the larynx accounts for approximately 0.5% to 1.0% of benign lesions of the larynx; such lesions are relatively rare and mostly localized. Nasopharyngeal amyloidosis combined with laryngeal amyloidosis is even rarer. We herein present a case involving a patient with amyloidosis in the nasopharynx and larynx who presented with a foreign body sensation and hoarseness in the pharynx. Electronic fiber laryngoscopy revealed a smooth neoplasm in the left nasopharynx and left vocal cord. The patient underwent surgical treatment, and the postoperative pathologic examination results suggested amyloidosis. Special staining performed using Congo red and crystal violet was positive, confirming amyloidosis. The patient recovered after surgery, and no recurrence was present at the 3- and 6-month follow-ups.

Cervical amyloidoma of transthyretin type: a case report and review of literature.

BACKGROUND: Amyloidoma is a rare clinical entity characterized by the focal aggregation of amyloid protein within the body, void of systemic involvement. To our knowledge, there have only been 26 reports of cervical amyloidoma to date. Amyloid light chain and beta-2-microglobulin are the most common types, with only three previous reports of transthyretin (ATTR) Amyloidoma. CASE PRESENTATION: We report a case of a 71-year-old male who presented with worsening strength and coordination of his upper extremities, right upper-leg pain, unsteady gait, and a reduced range of motion of his neck in all planes. Magnetic resonance imaging revealed a solitary mass compressing the spinal cord at C1-C2. Treatment consisted of cervical decompression and stabilization. Pathological examination confirmed solitary amyloid deposition of ATTR. Postoperative neurological assessment revealed improved balance, gait, hand function, and grip strength. Investigational imaging was ordered 8 months postoperatively revealing no evidence of systemic involvement, confirming the diagnosis of cervical ATTR amyloidoma. A discussion is provided surrounding the published literature of ATTR amyloidoma with description of the typical presentation, management, and outcomes of this rare pathology. CONCLUSION: Previous cases and studies indicate clinical signs such as ligamentum of flavum hypertrophy and carpal tunnel syndrome may precede focal ATTR spinal disposition. Outcomes for amyloidoma are generally favourable, as tumour resection prevents irreversible deficits. Patients have a low rate of recurrence with an overall excellent prognosis following resection and stabilization.

Recovery from AA amyloidosis-cardiomyopathy complexed with unicentric Castleman disease.

We report a case of cardiac amyloid A (AA) amyloidosis due to unicentric Castleman disease (UCD) in a patient whose cardiac function was restored 15 years after surgical resection of the mesenteric lymph node lesion. A man in his 40s had recurrent palpitations and fainting spells. ECG revealed torsades de pointes Increased C-reactive protein, interleukin-6 and serum AA levels, and marked concentric thickening of the left ventricular (LV) wall with diastolic restrictive filling pattern were observed. Duodenal biopsy revealed AA amyloid deposits. He had a mesenteric tumour, comprising many plasma cells. He was diagnosed with plasma cell-type UCD associated with secondary AA amyloidosis. C-reactive protein, interleukin-6 and serum AA levels were normalised 2 months postresection. Episodes of lethal ventricular arrhythmias decreased. LV wall thickness was gradually reduced. Approximately 15 years postresection, the LV wall thickness nearly normalised and ventricular arrhythmias disappeared. Better outcomes are expected following surgical tumour resection.