Tracking Multiorgan Treatment Response in Systemic AL-Amyloidosis With Cardiac Magnetic Resonance Derived Extracellular Volume Mapping.

BACKGROUND: Systemic light chain amyloidosis is a multisystem disorder that commonly involves the heart, liver, and spleen. Cardiac magnetic resonance with extracellular volume (ECV) mapping provides a surrogate measure of the myocardial, liver, and spleen amyloid burden. OBJECTIVES: The purpose of this study was to assess multiorgan response to treatment using ECV mapping, and assess the association between multiorgan treatment response and prognosis. METHODS: The authors identified 351 patients who underwent baseline serum amyloid-P-component (SAP) scintigraphy and cardiac magnetic resonance at diagnosis, of which 171 had follow-up imaging. RESULTS: At diagnosis, ECV mapping demonstrated that 304 (87%) had cardiac involvement, 114 (33%) significant hepatic involvement, and 147 (42%) significant splenic involvement. Baseline myocardial and liver ECV independently predict mortality (myocardial HR: 1.03 [95% CI: 1.01-1.06]; P = 0.009; liver HR: 1.03; [95% CI: 1.01-1.05]; P = 0.001). Liver and spleen ECV correlated with amyloid load assessed by SAP scintigraphy (R = 0.751; P < 0.001; R = 0.765; P < 0.001, respectively). Serial measurements demonstrated ECV correctly identified changes in liver and spleen amyloid load derived from SAP scintigraphy in 85% and 82% of cases, respectively. At 6 months, more patients with a good hematologic response had liver (30%) and spleen (36%) ECV regression than myocardial regression (5%). By 12 months, more patients with a good response demonstrated myocardial regression (heart 32%, liver 30%, spleen 36%). Myocardial regression was associated with reduced median N-terminal pro-brain natriuretic peptide (P < 0.001), and liver regression with reduced median alkaline phosphatase (P = 0.001). Changes in myocardial and liver ECV, 6 months after initiating chemotherapy, independently predict mortality (myocardial HR: 1.11 [95% CI: 1.02-1.20]; P = 0.011; liver HR: 1.07 [95% CI: 1.01-1.13]; P = 0.014). CONCLUSIONS: Multiorgan ECV quantification accurately tracks treatment response and demonstrates different rates of organ regression, with the liver and spleen regressing more rapidly than the heart. Baseline myocardial and liver ECV and changes at 6 months independently predict mortality, even after adjusting for traditional predictors of prognosis.

Infective Native Extracranial Carotid Artery Aneurysms: A Systematic Review.

BACKGROUND: Infective native extracranial carotid artery aneurysms are rare, and their management is variable due to a lack of evidence assessing outcomes. METHODS: We performed a systematic literature review following PRISMA guidelines to identify all reported cases of infective native extracranial carotid artery aneurysms between January 1970 and March 2021. RESULTS: This study identified 193 infective native aneurysms of the extracranial carotid artery from 154 sources. Patients were predominantly male (71.4%), and age ranged from 6 months to 89 years old. The most common presenting features were a neck mass and fever, but also included hemorrhage, respiratory distress, and neurological symptoms. Most aneurysms were located in the internal carotid artery (47.4%). Staphylococcus (23.3%) was the most commonly identified causative pathogen, followed by Mycobacterium tuberculosis (20.9%). Most appeared to become infected by direct local spread. Treatment strategies involved open surgical methods in 101 cases and an endovascular approach in 41 cases. In 4 cases, a hybrid method involving concurrent endovascular and open surgical management was undertaken. In 5 cases, there was antibiotic treatment alone. In the open surgery-treated group, the complication rate was 20.8% compared to 13.2% in the endovascular group. Mortality rate was 5.6%. CONCLUSIONS: Our review identified 193 cases of infective native extracranial carotid artery aneurysms. Direct local spread of a staphylococcus infection was the commonest cause. Endovascular management was associated with fewer early complications than open surgical management.