on Statement: Society of Echocardiography is accreditedby theAccreditationCouncil for edical Education to provide continuingmedical education for physicians. n Society of Echocardiography designates this educational activity for of 1.0 AMA PRA Category 1 Credits . Physicians should only claim credit te with the extent of their participation in the activity. CCI recognize ASE’s certificates and have agreed to honor the credit hours registry requirements for sonographers. Society of Echocardiography is committed to ensuring that its educational ll sponsored educational programs are not influencedby the special interests ation or individual, and itsmandate is to retain only those authors whose fists canbeeffectively resolved tomaintain thegoals andeducational integrity y. While a monetary or professional affiliation with a corporation does not fluence an author’s presentation, the Essential Areas and policies of the ire that any relationships that could possibly conflict with the educational activity be resolved prior to publication and disclosed to the audience. f faculty and commercial support relationships, if any, have been indicated. ience: is designed for all cardiovascular physicians and cardiac sonographers with erest and knowledge base in the field of echocardiography; in addition, reschers, clinicians, intensivists, and other medical professionals with a spein cardiac ultrasound will find this activity beneficial.

https://www.nationaljewish.org/getattachment/programs/directory/cardiology/pulmonary-hypertension-center/Echo-Guidelines-for-RV-JASE-2010.pdf.aspx

Guidelines for the Echocardiographic Assessment of the Right Heart in Adults: A Report from the American Society of Echocardiography Endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography

The authors summarize the recent developments in speckle-tracking echocardiography (STE), a relatively new technique that can be used in conjunction with two-dimensional or three-dimensional echocardiography for resolving the multidirectional components of left ventricular (LV) deformation. The tracking system is based on grayscale B-mode images and is obtained by automatic measurement of the distance between 2 pixels of an LV segment during the cardiac cycle, independent of the angle of insonation. The integration of STE with real-time cardiac ultrasound imaging overcomes some of the limitations of previous work in the field and has the potential to provide a unified framework to more accurately quantify the regional and global function of the left ventricle. STE holds promise to reduce interobserver and intraobserver variability in assessing regional LV function and to improve patient care while reducing health care costs through the early identification of subclinical disease. Following a brief overview of the approach, the authors pool the initial observations from clinical studies on the development, validation, merits, and limitations of STE.

Assessment of Myocardial Mechanics Using Speckle Tracking Echocardiography: Fundamentals and Clinical Applications

Background The diagnosis of cardiac amyloidosis (CA) is challenging owing to vague symptomatology and non-specific echocardiographic findings. Objective To describe regional patterns in longitudinal strain (LS) using two-dimensional speckle-tracking echocardiography in CA and to test the hypothesis that regional differences would help differentiate CA from other causes of increased left ventricular (LV) wall thickness. Methods and results 55 consecutive patients with CA were compared with 30 control patients with LV hypertrophy (n=15 with hypertrophic cardiomyopathy, n=15 with aortic stenosis). A relative apical LS of 1.0, defined using the equation (average apical LS/(average basal LS + mid-LS)), was sensitive (93%) and specific (82%) in differentiating CA from controls (area under the curve 0.94). In a logistic regression multivariate analysis, relative apical LS was the only parameter predictive of CA (p=0.004). Conclusions CA is characterised by regional variations in LS from base to apex. A relative ‘apical sparing’ pattern of LS is an easily recognisable, accurate and reproducible method of differentiating CA from other causes of LV hypertrophy.

https://heart.bmj.com/content/heartjnl/early/2012/08/02/heartjnl-2012-302353.full.pdf

Relative ‘apical sparing’ of longitudinal strain using two-dimensional speckle-tracking echocardiography is both sensitive and specific for the diagnosis of cardiac amyloidosis

Transthyretin amyloidosis is a progressive and eventually fatal disease primarily characterized by sensory, motor, and autonomic neuropathy and/or cardiomyopathy. Given its phenotypic unpredictability and variability, transthyretin amyloidosis can be difficult to recognize and manage. Misdiagnosis is common, and patients may wait several years before accurate diagnosis, risking additional significant irreversible deterioration. This article aims to help physicians better understand transthyretin amyloidosis—and, specifically, familial amyloidotic polyneuropathy—so they can recognize and manage the disease more easily and discuss it with their patients. We provide guidance on making a definitive diagnosis, explain methods for disease staging and evaluation of disease progression, and discuss symptom mitigation and treatment strategies, including liver transplant and several pharmacotherapies that have shown promise in clinical trials.

/pdf/guideline-of-transthyretin-related-hereditary-amyloidosis-4bztifl0u3.pdf

Guideline of transthyretin-related hereditary amyloidosis for clinicians

The systemic amyloidoses are a family of diseases induced by misfolded or misassembled proteins. Extracellular deposition of these proteins as soluble or insoluble cross β-sheets disrupts vital organ function.1 More than 27 different precursor proteins have the propensity to form amyloid fibrils.2 The particular precursor protein that misfolds to form amyloid fibrils defines the amyloid type and predicts the patient's clinical course. Several types of amyloid can infiltrate the heart, resulting in progressive diastolic and systolic dysfunction, congestive heart failure, and death. Treatment of cardiac amyloidosis is dictated by the amyloid type and degree of involvement. Consequently, early recognition and accurate classification are essential.3

The diagnosis of amyloidosis requires histological identification of amyloid deposits. Congo Red staining renders amyloid deposits salmon pink by light microscopy, with a characteristic apple green birefringence under polarized light conditions (Figure 1). Additional immunohistochemical staining for precursor proteins identifies the type of amyloidosis (Figure 2).4 Ultimately, immunogold electron microscopy and mass spectrometry confer the greatest sensitivity and specificity for amyloid typing.5,6



Figure 1. 
Congo Red staining of myocardial tissue from a patient with amyloid cardiomyopathy. A , Light microscopy; B , polarized light microscopy, 400× magnification.





Figure 2. 
Transthyretin amyloid cardiomyopathy by immunohistochemical staining. Endomyocardial biopsy samples were stained with antibodies to ( A ) kappa light chain, ( B ) lambda light-chain, ( C ) serum amyloid A, and ( D ) transthyretin amyloid. Bright-light micrographs at 400× magnification.



Two types of amyloid commonly infiltrate the heart: (1) Immunoglobulin light-chain (AL or primary systemic) amyloid and (2) transthyretin (TTR) amyloid. Transthyretin-related amyloidoses, in turn, encompass 2 forms of disease: Familial disease arising from misfolding of a mutated or variant TTR (familial amyloid cardiomyopathy or familial amyloidotic polyneuropathy [FAP]) and a sporadic, nongenetic disease caused by misaggregation of wild-type transthyretin (senile systemic amyloidosis [SSA]). Cardiac amyloidosis can also be …

/pdf/transthyretin-ttr-cardiac-amyloidosis-2d6v126inv.pdf

Transthyretin (TTR) Cardiac Amyloidosis

Hypertension is the most common cause of left ventricular (LV) hypertrophy. However, multiple causes can lead to LV hypertrophy, each of which has different histological and mechanical properties. To assess the value of a novel speckle-tracking echocardiographic measurement of myocardial strain and strain rate in defining the mechanical properties of LV hypertrophy, 20 patients with asymmetric hypertrophic cardiomyopathy, 24 patients with secondary LV hypertrophy, 12 patients with biopsy-proved confirmed cardiac amyloidosis, and 22 age-matched healthy asymptomatic volunteers were studied. Patients with amyloidosis had severe diastolic dysfunction, and myocardial deformation was significantly decreased. The new technique allowed cardiac amyloid to be easily differentiated from the other categories. In patients with hypertrophic cardiomyopathy, there was segmental myocardium dysfunction as assessed by strain imaging. LV global systolic velocity and radial displacement were higher, and abnormal relaxation was more frequent, in the group with secondary LV hypertrophy than in normal controls. In conclusion, the observations from strain parameters derived from speckle tracking were consistent with the known underlying pathology of each condition, which speaks to the value of strain imaging. Cardiac amyloid profoundly alters all strain parameters, and analysis of these parameters could aid in the diagnosis.

Differentiation of hypertrophic cardiomyopathy and cardiac amyloidosis from other causes of ventricular wall thickening by two-dimensional strain imaging echocardiography.

Xing Sheng Yang

Papers

Differentiation of hypertrophic cardiomyopathy and cardiac amyloidosis from other causes of ventricular wall thickening by two-dimensional strain imaging echocardiography.

Tetralogy of Fallot with Pulmonary Atresia

Discrete Subaortic Stenosis

Inferior Wall Aneurysm Following Myocardial Infarction

Supravalvular Aortic Stenosis with Williams Syndrome