Survey
* Your assessment is very important for improving the work of artificial intelligence, which forms the content of this project
Download Infiltrative Myocardial Diseases - sha
Document related concepts
no text concepts found
Transcript
5/11/2014 Echocardiographic Assessment of infiltrative Myocardial Diseases April 24, 2014 Bahaa M. Fadel, MD, FACC Director, Adult Echocardiography Laboratory Director, Adult Cardiology Fellowship Program King Faisal Specialist Hospital & Research Center KFSHRC Infiltrative Myocardial Diseases Heterogeneous group of disorders Myocardial involvement Diffuse Restrictive Cardiomyopathy Amyloidosis Hypertrophic Cardiomyopathy Patchy Dilated Cardiomyopathy Ischemic Cardiomyopathy Sarcoidosis Fabry disease Wegener Granulomatosis Danon disease Mucopolysaccharidosis Hemochromatosis KFSHRC Friedreich ataxia 1 5/11/2014 Infiltrative Myocardial Diseases Extracellular deposition Intracellular deposition with cell death Intracellular deposition without cell death Amyloidosis Hemochromatosis Fabry disease Inflammatory infiltration Myocyte hypertrophy Sarcoidosis Friedreich’s ataxia KFSHRC KFSHRC F. Ruberg, MD 2 5/11/2014 Hypertrophic Myopathy • 68 year old male • Hypertension • DM • Renal impairment • COPD • Atrial fibrillation • NYHA class IV • Lower limb edema KFSHRC Hypertrophic Myopathy MV Septum MV-DT = 200 msec IVRT = 105 msec PV KFSHRC TDI- Septum 3 5/11/2014 Deformation Imaging Longitudinal Strain Amyloidosis • Multisystem disease • Extracellular deposition of proteinaceous material: amyloid fibers • Due to misfolding of a precursor protein • Presence and severity of cardiac involvement depends on the precursor protein: – Light chain Ig – Transthyretin – Serum Amyloid A 4 5/11/2014 Amyloidosis • The most common infiltrative heart disease • Restrictive cardiomyopathy • Stiff ventricles: diastolic impairment precedes systolic dysfunction • “Amyloidosis does not discriminate”: Amyloid fibers deposit everywhere in the heart – Myocardium, endocardium, pericardium – Interatrial septum – Conduction system – Coronary arteries • Endomyocardial biopsy almost always positive Cardiac Amyloidosis 5 5/11/2014 Cardiac Amyloidosis Inferior vena cava Mitral valve Deformation Imaging Longitudinal Strain Septum Lateral wall Destruction of the longitudinal function of the left ventricle 6 5/11/2014 Deformation Imaging Longitudinal Strain Longitudinal motion Lateral wall ~ 5 mm Cardiac MRI “Not a Luxury in suspected infiltrative cardiomyopathies but a Need” Guadolinium RV LV RV LV Circumferential enhancement Subendocardial Both ventricles 7 5/11/2014 Cardiac Amyloidosis • Classical features only present in late stages of disease: already poor prognosis • Echo cannot distinguish various types of amyloidosis: AL vs. TTR • Increased LV wall thickness + history of hypertension does not necessarily imply hypertensive heart disease • Increased LV wall thickness + low ECG voltage: infiltrative myocardial disease till proven otherwise • Do not expect granular or sparkling appearance of myocardium in all patients • Strain imaging should be done in all patients with LVH Cardiac Amyloidosis Too late for diagnosis MV IVC Optimal timing 8 5/11/2014 Hypertrophic Myopathy How to pursue a diagnosis? 9 5/11/2014 Longitudinal Strain Fadel, BM. Cardiogenetics. 2013 10 5/11/2014 Longitudinal Strain HTN Friedreich ataxia Amyloidosis Fabry Fadel, BM. Cardiogenetics. 2013 Longitudinal Strain 11 5/11/2014 Longitudinal Strain HCM Apical HCM 12 5/11/2014 Cardiac Sarcoidosis • • • • Great masquerader Patchy granulomatous infiltration of heart Clinical cardiac involvement in 5% of patients Difficult diagnosis: – Various manifestations of the disease – Lack of gold standard diagnostic modality – Imaging modalities: poor sensitivity and specificity – Endomyocardial biopsy positive in < 20% of cases • Risk of sudden cardiac death: – Ventricular arrhythmias – Conduction abnormalities Cardiac Sarcoidosis • No distinctive findings: – Wall motion abnormalities: thinning and aneurysms – Systolic / diastolic dysfunction – Pulmonary hypertension – Pericardial effusion FDG-PET CMR Shah BN; Echocardiography 2013 Greulich; JACC Img 2013 13 5/11/2014 Algorithm for diagnostic evaluation of infiltrative cardiomyopathies Srinivasan; Heart 2013 Algorithm for diagnostic evaluation of infiltrative cardiomyopathies Strain Imaging Srinivasan; Heart 2013 14 5/11/2014 15
Related documents