PurposeTo evaluate if cardiac magnetic resonance elastography (MRE) can measure increased stiffness in patients with cardiac amyloidosis. Myocardial tissue stiffness plays an important role in cardiac function. A noninvasive quantitative imaging technique capable of measuring myocardial stiffness could aid in disease diagnosis, therapy monitoring, and disease prognostic strategies. We recently developed a high‐frequency cardiac MRE technique capable of making noninvasive stiffness measurements.Materials and MethodsIn all, 16 volunteers and 22 patients with cardiac amyloidosis were enrolled in this study after Institutional Review Board approval and obtaining formal written consent. All subjects were imaged head‐first in the supine position in a 1.5T closed‐bore MR imager. 3D MRE was performed using 5 mm isotropic resolution oblique short‐axis slices and a vibration frequency of 140 Hz to obtain global quantitativein vivoleft ventricular stiffness measurements. The median stiffness was compared between the two cohorts. An octahedral shear strain signal‐to‐noise ratio (OSS‐SNR) threshold of 1.17 was used to exclude exams with insufficient motion amplitude.ResultsFive volunteers and six patients had to be excluded from the study because they fell below the 1.17 OSS‐SNR threshold. The myocardial stiffness of cardiac amyloid patients (median: 11.4 kPa, min: 9.2, max: 15.7) was significantly higher (P = 0.0008) than normal controls (median: 8.2 kPa, min: 7.2, max: 11.8).ConclusionThis study demonstrates the feasibility of 3D high‐frequency cardiac MRE as a contrast‐agent‐free diagnostic imaging technique for cardiac amyloidosis.Level of Evidence:2Technical Efficacy:Stage 2J. Magn. Reson. Imaging 2017;46:1361–1367.
Improved outcomes for newly diagnosed AL amyloidosis between 2000 and 2014: cracking the glass ceiling of early death
Key Points Significant improvement in outcome is a reality for newly diagnosed AL amyloidosis in the past decade. Six-month mortality among transplant ineligible patients has declined since 2005.
Diagnosis and Management of Waldenström Macroglobulinemia
Background In adult Fontan patients, ventricular or arterial dysfunction may impact homeostasis of the Fontan circulation and predispose to heart failure. We sought to characterize ventricular‐arterial ( VA ) properties in adult Fontan patients. Methods and Results Adult Fontan patients (n=170), including those with right ( SRV , n=57) and left ( SLV , n=113) dominant ventricular morphology, were compared to age, sex, and body size matched controls (n=170). Arterial function, load‐insensitive measures of contractility, VA coupling, diastolic function, and ventricular efficiency were assessed. Compared to controls, Fontan patients had similar arterial (Ea), but lower end‐systolic ventricular (Ees), elastance, preload recruitable stroke work and peak power index, impaired VA coupling, eccentric remodeling, reduced ventricular efficiency and increased diastolic stiffness ( P <0.05 for all). Ventricular efficiency declined steeply with higher heart rate in Fontan, but not control, patients. Among Fontan patients (n=123) and controls (n=162) with preserved cardiac index ( CI ; ≥2.5 L/min per m 2 ), Fontan patients had worse contractility than controls, but CI was preserved owing to relative tachycardia, lower afterload, and eccentric remodeling. However, 25% of Fontan patients had reduced CI and were distinguished from those with preserved CI by less‐eccentric remodeling and worse diastolic function, rather than more‐impaired contractility. Conclusions Adult Fontan patients have contractile and diastolic dysfunction with normal afterload, impaired VA coupling, and reduced ventricular efficiency with heightened sensitivity to heart rate. Maintenance of CI is dependent on lower afterload, eccentric remodeling, and relative preservation of diastolic function. These data contribute to our understanding of circulatory physiology in adult Fontan patients.
Multicenter Study of Planar Technetium 99m Pyrophosphate Cardiac Imaging
Catheter Ablation for Atrial Arrhythmias in Patients With CABackgroundCardiac amyloidosis (CA) is associated with increased atrial arrhythmias risk. The efficacy/safety of catheter‐based ablation therapy in patients with CA has not been adequately assessed.Methods and ResultsAll diagnosed CA patients who underwent atrial arrhythmia ablation therapy from 1995 to 2015 were reviewed. Arrhythmia recurrence, NYHA symptoms, and mortality were recorded. A total of 26 patients with CA and atrial arrhythmias were included; there were 7 light‐chain (AL), 17 wild‐type transthyretin (ATTRwt), and 2 mutated transthyretin (ATTRm) amyloidosis patients in total. Of which 13 underwent atrial arrhythmia ablation (CA‐A) and 13 underwent AV nodal ablation (CA‐AVN). In the CA‐A group, there were: 3 with atrial fibrillation (AF); 6 with atrial flutter (AFL); 2 with AF/AFL; and 2 with atrial tachycardia (AT). One‐year and 3‐year recurrence‐free survival were 75% and 60%, respectively. NYHA symptom improvement 6 months postablation was observed in both CA‐A and CA‐AVN groups: 7/10 (70%) and 4/8 (50%), respectively. Eleven patients with CA died (8 in CA‐AVN group vs. 3 in CA‐A group).ConclusionsCatheter‐based ablation for patients with CA appears to provide important symptomatic relief. However, mortality from the underlying disease remains a significant issue for the amyloid light‐chain subtype.
Abnormal stress echocardiography findings in cardiac amyloidosis
Right Parasternal Lead Placement Increases Eligibility for Subcutaneous Implantable Cardioverter Defibrillator Therapy in Adults With Congenital Heart Disease
Aims Prognostication and treatment selection for cardiac amyloidosis requires accurate amyloid typing. The aim of this study was to investigate the utility of histomorphology for predicting type. Methods and results Autopsy cases with cardiac amyloidosis (1998–2010) were typed by the use of mass spectrometry‐based proteomics. Deposition patterns were correlated with amyloid type. Among 108 decedents (mean age 75 years; 69% men), 107 had a single type, including transthyretin (ATTR) (60 cases), amyloid light chain (AL) (32 λ; 12 κ), amyloid A (AA) (two), and apolipoprotein AIV (AApoAIV) (one). Interstitial deposition was more extensive in AL amyloidosis cases than in ATTR cases [odds ratio (OR) 6.8, P = 0.0004]. Histomorphological patterns of interstitial deposition were mixed in 61% of AL amyloidosis cases and in 61% of ATTR cases, but diffuse pericellular deposits favoured AL amyloidosis (OR 10.7, P = 0.0001), nodular deposits favoured ATTR (OR 3.1, P = 0.0229), and discrete pericellular deposits tended to partially favour ATTR (OR 1.7, P = 0.1970). Arterial and venous deposits each favoured AL amyloidosis (OR ranging from 9.3 to 192.0, P‐value ranging from 0.0022 to <0.0001), and were severe in AL amyloidosis. Endocardial deposits favoured AL amyloidosis (OR 46.3, P < 0.0001) and were also more severe in AL amyloidosis. Conclusions The extent and distribution of cardiac amyloidosis strongly correlate with amyloid type, suggesting fundamental differences in the pathobiology of deposition. The tendency for mixed patterns to occur limits the practicality and accuracy of using histopathology for amyloid typing.