Heart Failure Management of Patients with Amyloid Cardiomyopathy

Abstract Cardiac amyloidosis (CA) can lead to progressive heart failure (HF) by depositing insoluble amyloid fibrils within the myocardial extracellular space, resulting in an infiltrative and restrictive cardiomyopathy. Although CA was previously perceived as rare and incurable, recent advances in diagnostics and emerging therapies have been changing this outlook. It is crucial to spread awareness about CA to facilitate earlier diagnosis and proper therapeutic interventions, enhancing patient prognosis and survival. Currently, there is an estimated delay of 2 years from symptom onset to diagnosis, typically involving consultation with an average of 5 different professionals. Advances in cardiovascular imaging have facilitated earlier and more accurate diagnosis, reducing the necessity for invasive procedures, such as endomyocardial biopsy. Presently, tafamidis is the only drug that has been shown to offer prognostic benefits in ATTR-CA. Tafamidis is a highly specific medication targeting the circulating TTR protein, stabilizing the TTR tetramer to prevent its dissociation into amyloidogenic monomers that deposit in the myocardium. Alongside specific amyloidosis therapy, supportive HF treatment may be required; however, managing CA with medications typically used for HF with reduced ejection fraction (HFrEF) can be challenging due to potential intolerance. The effectiveness of guideline-directed medical therapy (GDMT) remains undetermined and still requires evaluation through randomized controlled clinical trials (RCCTs). Thus, the treatment cornerstone remains the judicious use of loop diuretics and mineralocorticoid receptor antagonists to control volume overload. Due to the safety profile, not adversely affecting hemodynamics or renal function, sodium-glucose transport protein 2 (SGLT2) inhibitors may be an effective treatment for CA, but they also still require evaluation through RCCTs.

Late atrioventricular groove disruption presenting 7 years after mitral valve replacement: a case report.

BACKGROUND: Left ventricular rupture is the most feared complication in mitral valve surgery. Despite its low incidence, mortality rates can reach up to 75%. It usually presents on the operating room with a dissecting haematoma followed by massive bleeding after discontinuing cardiopulmomary bypass. However, cardiac rupture may be contained by adherent pericardium or scar tissue leading to chronic formation of a pseudoaneurysm (PSA). CASE SUMMARY: A 44-year-old man came to our institution with acute heart failure triggered by community-acquired pneumonia. He underwent mitral valve replacement with a mechanical prosthesis 7 years before and reported suffering from chronic worsening dyspnoea for 18 months. He underwent chest computed tomography scan and cardiac magnetic resonance imaging (CMRI), which showed two extensive left ventricular (LV) multilobulated PSAs. An operative approach was chosen and a tear was found on the posterior atrioventricular groove (AVG), communicating left ventricle with the PSA, which was closed with bovine pericardium patch. After weaning from cardiopulmonary bypass, he presented a diffuse life-threatening bleeding. The surgeons packed his chest with compresses before closing the sternum and he was operatively revised after 48 h. Post-operative CMRI showed that one of the PSAs remained connected with the LV. Despite of all, 1 year after hospital discharge, he remains asymptomatic without signs of heart failure. DISCUSSION: This case illustrates PSAs' potential to grow for a long period before causing symptoms, the complexity and risks of chronic AVG disruption surgery and the importance of careful annular manipulation and debridement as preventive measures in mitral valve surgery.

Increased levels of IL-17A in patients with fibromyalgia.

OBJECTIVES: The aim of this study was to evaluate the plasma levels of IL-17A in fibromyalgia patients, and to look for any correlations between this data and the concentrations of some pro- and anti-inflammatory cytokines. METHODS: We performed a study including 58 fibromyalgia patients and 39 healthy women matched for age and body mass index. The plasma levels of IL-17A and other pro- and anti-inflammatory cytokines were measured by using the technique of cytometric bead array (CBA). The analysis of differences between groups was performed using Mann-Whitney test and the analysis of the correlations by Spearman's correlation test. RESULTS: The analyses showed that fibromyalgia patients present increased levels of IL-17A. They also revealed that plasma concentrations of IL17A positively correlate with levels of IL-2, IL-4 and IL-10, TNF and IFNγ. CONCLUSIONS: As far as we are aware, this is the first study to demonstrate increased levels of IL17A in fibromyalgia patients. The positive correlation between the levels of IL-17A and of other cytokines strengthens the hypothesis of the involvement of inflammatory mechanisms in the development of this syndrome.