A case of primary amyloidosis located in the larynx in which Beta-2-microglobulin (beta 2m) was the main component of the tissue is presented. Indirect immunofluorescence performed with monoclonal anti-human beta 2 m antibody (BBM1) showed a positive reaction, as did Congo red stain, and both patterns were similar. A significant increase in serum beta 2 m was observed; however, the clinical condition associated with this elevation remains unclear. We suggest that beta 2m is not only the main component of secondary amyloidosis in terminal nephropathy patients undergoing hemodialysis for prolonged periods, but that it could be part of the structure of other types of amyloid--either primary or secondary.

Objective This study aims to review the pharmacology, efficacy, and safety of the soluble guanylate cyclase stimulator, vericiguat, in patients with symptomatic congestive heart failure with ejection fraction less than 45% for the reduction of cardiovascular deaths. Also, to evaluate heart failure–related hospitalization in patients following a hospital discharge secondary to heart failure or those that require outpatient intravenous diuretics. Data source MEDLINE/Pubmed and National Institutes of Health Clinical Trial Registry were searched between January 1989 to February 2021 using the following terms: vericiguat, soluble guanylate cyclase stimulator, heart failure, (was also known as) BAY 1021189. Study Selection and Data Extraction The following study designs were included in the analysis: phase I, II, and III clinical trials; systematic reviews; and meta-analyses. Articles were included if they were published in English and evaluated vericiguat pharmacology, pharmacokinetics, efficacy, and safety. Data Synthesis The Food and Drug Administration approved vericiguat for the reduction of cardiovascular death and hospitalization after having a related hospitalization or the need for outpatient intravenous diuretics, in those with symptomatic chronic heart failure and ejection fraction less than 45%. In the VICTORIA trial, vericiguat demonstrated a 10% reduction in risk of death from cardiovascular causes or first hospitalization for heart failure compared with placebo. Vericiguat was well tolerated overall with hypotension, syncope, and anemia noted as the most common side effects, similar to the other agent in its class. Conclusion Vericiguat may be appropriate as add-on therapy for patients already on guideline-directed medical therapy with recent decompensated HFrEF to reduce hospitalization.

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Primary hyperoxaluria is a rare genetic disorder characterized by oxalate crystal deposition, including in the heart. Hyperoxaluria-associated cardiomyopathy manifests as restrictive infiltrative cardiomyopathy. We present a case of a 52-year-old male with a past medical history of type 2 primary hyperoxaluria, end-stage renal disease on hemodialysis, paroxysmal atrial fibrillation, and hypertension, who presented with dyspnea and lethargy. Transthoracic echocardiogram showed cardiomyopathy with ejection fraction (EF) of 35-40% with severe hypokinesis of apical myocardium. Endomyocardial biopsy revealed interstitial fibrosis and crystal deposition consistent with oxalate. Cardiac MRI showed late gadolinium enhancement with subendocardial, nearly transmural fibrosis of lateral wall along with mid myocardial involvement of anterior and septal wall. To the best of our knowledge, this is the first case of type 2 primary hyperoxaluriaassociated cardiomyopathy utilizing transthoracic echo, endomyocardial biopsy, and cardiac MRI.

Amyloidosis is an underappreciated medical condition with symptoms camouflaging as common medical comorbidities leading to its underdiagnosis due to its systemic involvement. Despite common misconceptions, amyloidosis and its systemic comorbidities are more prevalent and treatable than previously acknowledged by the medical community. There are two major forms of amyloidosis: amyloid light-chain and transthyretin amyloidosis. Each of these have a distinct pathophysiology, diagnostic work-up, treatment, and prognosis. The patient described in this study was diagnosed with transthyretin cardiac amyloidosis months after presenting with heart failure of unknown etiology. Usually, clinicians presume that heart failure results from common comorbidities such as hypertension, diabetes, and hyperlipidemia. Here, the correct etiology was transthyretin cardiac amyloidosis. The patient had five admissions for heart failure symptoms prior to a physician identifying the etiology as cardiac transthyretin amyloidosis. After initiating the transthyretin stabilizer tafamidis, the patient did not experience another heart failure exacerbation. This vignette provides an example of the clinical presentation, diagnostic work-up, and treatment of a patient with cardiac transthyretin amyloidosis. The review of the literature focuses on the epidemiology, and clinical symptoms that should prompt an evaluation for cardiac amyloidosis as well as the diagnostic and therapeutic options are available. Transthyretin cardiac amyloidosis is a rare and underdiagnosed disease, while heart failure is a highly prevalent condition. This clinical vignette seeks to provide education and awareness to an overlooked medical disorder.