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DILATED DILATED CARDIOMYOPATHYCARDIOMYOPATHY
EtiologyCauses
Ischemic Dilated CM
Non Ischemic Dilated CM
Other Causes
Pathophysiology
Chamber enlargement
1048708 Abnormal muscle function
1048708 Neurohormonal mechanisms eg SNS
1048708 Neurohormonal antagonists
1
HistorySymptoms
1048708 Asymptomatic for years or suffer from acute pulmonary edema biventricular failure arrhythmias or cardiac
Forward failure
1048708 Backward failure
Most people have BOTH Forward and Backward HF but to differing degrees
(A) Macroscopic short-axis section of the right and left ventricle at a (A) Macroscopic short-axis section of the right and left ventricle at a midventricular level from a patient with dilated cardiomyopathy showing midventricular level from a patient with dilated cardiomyopathy showing midwall fibrosis (straight arrows) mainly in the inferior and lateral walls midwall fibrosis (straight arrows) mainly in the inferior and lateral walls but also in the lower and upper septum (curved arrows) (B) Microscopic but also in the lower and upper septum (curved arrows) (B) Microscopic
section of the heart in which Sirius red staining confirms collagen (arrow) section of the heart in which Sirius red staining confirms collagen (arrow) in areas of fibrosis seen macroscopically Myocytes (stained yellow) are in areas of fibrosis seen macroscopically Myocytes (stained yellow) are admixed with the collagen (red) (C) Premortem cardiovascular magnetic admixed with the collagen (red) (C) Premortem cardiovascular magnetic resonance of the same slice with excellent accord between the areas of resonance of the same slice with excellent accord between the areas of
macroscopic fibrosis and areas of late gadolinium enhancement macroscopic fibrosis and areas of late gadolinium enhancement (matching arrows(matching arrows) )
Criteria for Diagnosis Criteria for Diagnosis PERIPARTUM CMDPERIPARTUM CMD
4 Criteria4 Criteriandash Development of Heart failure in the last month Development of Heart failure in the last month
of pregnancy or within 5 months postpartumof pregnancy or within 5 months postpartumndash Absence of a determinable cause for cardiac Absence of a determinable cause for cardiac
failurefailurendash Absence of heart disease before last month of Absence of heart disease before last month of
pregnancypregnancyndash Left Ventricle impairment demonstrated on Left Ventricle impairment demonstrated on
EchoEcho
EKG ChangesEKG Changes
Sinus TachycardiaSinus Tachycardia
Nonspecific ST Nonspecific ST changeschanges
LV HypertrophyLV Hypertrophy
Chest X-rayChest X-ray
Pulmonary EdemaPulmonary Edema
Venous congestionVenous congestion
Enlarged Cardiac Enlarged Cardiac SilhouetteSilhouette
RO PERO PE
EchocardiogramEchocardiogram
Spherical LVSpherical LV
Mitral and Tricuspid Mitral and Tricuspid regurgitationregurgitation
Left Atrial Left Atrial enlargementenlargement
EF lt55EF lt55
Classification of Types of Restrictive Classification of Types of Restrictive Cardiomyopathy According to CauseCardiomyopathy According to Cause
NoninfiltrativeNoninfiltrative Idiopathic cardiomyopathy[] Idiopathic cardiomyopathy[] Familial cardiomyopathy Familial cardiomyopathy Hypertrophic cardiomyopathy Hypertrophic cardiomyopathy Scleroderma Scleroderma Pseudoxanthoma elasticum Pseudoxanthoma elasticum Diabetic cardiomyopathy Diabetic cardiomyopathy
InfiltrativeInfiltrative Amyloidosis[] Amyloidosis[] Sarcoidosis[] Sarcoidosis[] Gaucher disease Gaucher disease Hurler disease Hurler disease Fatty infiltration Fatty infiltration
From Kushwaha S Fallon JT Fuster V From Kushwaha S Fallon JT Fuster V Restrictive cardiomyopathy N Engl J Med Restrictive cardiomyopathy N Engl J Med 336267 1997 Copyright 1997 336267 1997 Copyright 1997 Massachusetts Medical SocietyMassachusetts Medical Society
These conditions are more likely than the These conditions are more likely than the others to be encountered in clinical practiceothers to be encountered in clinical practice
Storage DiseaseStorage Disease Hemochromatosis Hemochromatosis Fabry disease Fabry disease Glycogen storage disease Glycogen storage disease EndomyocardialEndomyocardial Endomyocardial fibrosis[] Endomyocardial fibrosis[] Hypereosinophilic syndrome Hypereosinophilic syndrome Carcinoid heart disease Carcinoid heart disease Metastatic cancers Metastatic cancers Radiation[] Radiation[] Toxic effects of Toxic effects of anthracycline[] anthracycline[] Drugs causing fibrous Drugs causing fibrous endocarditis (serotonin endocarditis (serotonin methysergide ergotamine methysergide ergotamine mercurial agents busulfan)mercurial agents busulfan)
CMRCMR
EtiologyCauses
Ischemic Dilated CM
Non Ischemic Dilated CM
Other Causes
Pathophysiology
Chamber enlargement
1048708 Abnormal muscle function
1048708 Neurohormonal mechanisms eg SNS
1048708 Neurohormonal antagonists
1
HistorySymptoms
1048708 Asymptomatic for years or suffer from acute pulmonary edema biventricular failure arrhythmias or cardiac
Forward failure
1048708 Backward failure
Most people have BOTH Forward and Backward HF but to differing degrees
(A) Macroscopic short-axis section of the right and left ventricle at a (A) Macroscopic short-axis section of the right and left ventricle at a midventricular level from a patient with dilated cardiomyopathy showing midventricular level from a patient with dilated cardiomyopathy showing midwall fibrosis (straight arrows) mainly in the inferior and lateral walls midwall fibrosis (straight arrows) mainly in the inferior and lateral walls but also in the lower and upper septum (curved arrows) (B) Microscopic but also in the lower and upper septum (curved arrows) (B) Microscopic
section of the heart in which Sirius red staining confirms collagen (arrow) section of the heart in which Sirius red staining confirms collagen (arrow) in areas of fibrosis seen macroscopically Myocytes (stained yellow) are in areas of fibrosis seen macroscopically Myocytes (stained yellow) are admixed with the collagen (red) (C) Premortem cardiovascular magnetic admixed with the collagen (red) (C) Premortem cardiovascular magnetic resonance of the same slice with excellent accord between the areas of resonance of the same slice with excellent accord between the areas of
macroscopic fibrosis and areas of late gadolinium enhancement macroscopic fibrosis and areas of late gadolinium enhancement (matching arrows(matching arrows) )
Criteria for Diagnosis Criteria for Diagnosis PERIPARTUM CMDPERIPARTUM CMD
4 Criteria4 Criteriandash Development of Heart failure in the last month Development of Heart failure in the last month
of pregnancy or within 5 months postpartumof pregnancy or within 5 months postpartumndash Absence of a determinable cause for cardiac Absence of a determinable cause for cardiac
failurefailurendash Absence of heart disease before last month of Absence of heart disease before last month of
pregnancypregnancyndash Left Ventricle impairment demonstrated on Left Ventricle impairment demonstrated on
EchoEcho
EKG ChangesEKG Changes
Sinus TachycardiaSinus Tachycardia
Nonspecific ST Nonspecific ST changeschanges
LV HypertrophyLV Hypertrophy
Chest X-rayChest X-ray
Pulmonary EdemaPulmonary Edema
Venous congestionVenous congestion
Enlarged Cardiac Enlarged Cardiac SilhouetteSilhouette
RO PERO PE
EchocardiogramEchocardiogram
Spherical LVSpherical LV
Mitral and Tricuspid Mitral and Tricuspid regurgitationregurgitation
Left Atrial Left Atrial enlargementenlargement
EF lt55EF lt55
Classification of Types of Restrictive Classification of Types of Restrictive Cardiomyopathy According to CauseCardiomyopathy According to Cause
NoninfiltrativeNoninfiltrative Idiopathic cardiomyopathy[] Idiopathic cardiomyopathy[] Familial cardiomyopathy Familial cardiomyopathy Hypertrophic cardiomyopathy Hypertrophic cardiomyopathy Scleroderma Scleroderma Pseudoxanthoma elasticum Pseudoxanthoma elasticum Diabetic cardiomyopathy Diabetic cardiomyopathy
InfiltrativeInfiltrative Amyloidosis[] Amyloidosis[] Sarcoidosis[] Sarcoidosis[] Gaucher disease Gaucher disease Hurler disease Hurler disease Fatty infiltration Fatty infiltration
From Kushwaha S Fallon JT Fuster V From Kushwaha S Fallon JT Fuster V Restrictive cardiomyopathy N Engl J Med Restrictive cardiomyopathy N Engl J Med 336267 1997 Copyright 1997 336267 1997 Copyright 1997 Massachusetts Medical SocietyMassachusetts Medical Society
These conditions are more likely than the These conditions are more likely than the others to be encountered in clinical practiceothers to be encountered in clinical practice
Storage DiseaseStorage Disease Hemochromatosis Hemochromatosis Fabry disease Fabry disease Glycogen storage disease Glycogen storage disease EndomyocardialEndomyocardial Endomyocardial fibrosis[] Endomyocardial fibrosis[] Hypereosinophilic syndrome Hypereosinophilic syndrome Carcinoid heart disease Carcinoid heart disease Metastatic cancers Metastatic cancers Radiation[] Radiation[] Toxic effects of Toxic effects of anthracycline[] anthracycline[] Drugs causing fibrous Drugs causing fibrous endocarditis (serotonin endocarditis (serotonin methysergide ergotamine methysergide ergotamine mercurial agents busulfan)mercurial agents busulfan)
CMRCMR
Pathophysiology
Chamber enlargement
1048708 Abnormal muscle function
1048708 Neurohormonal mechanisms eg SNS
1048708 Neurohormonal antagonists
1
HistorySymptoms
1048708 Asymptomatic for years or suffer from acute pulmonary edema biventricular failure arrhythmias or cardiac
Forward failure
1048708 Backward failure
Most people have BOTH Forward and Backward HF but to differing degrees
(A) Macroscopic short-axis section of the right and left ventricle at a (A) Macroscopic short-axis section of the right and left ventricle at a midventricular level from a patient with dilated cardiomyopathy showing midventricular level from a patient with dilated cardiomyopathy showing midwall fibrosis (straight arrows) mainly in the inferior and lateral walls midwall fibrosis (straight arrows) mainly in the inferior and lateral walls but also in the lower and upper septum (curved arrows) (B) Microscopic but also in the lower and upper septum (curved arrows) (B) Microscopic
section of the heart in which Sirius red staining confirms collagen (arrow) section of the heart in which Sirius red staining confirms collagen (arrow) in areas of fibrosis seen macroscopically Myocytes (stained yellow) are in areas of fibrosis seen macroscopically Myocytes (stained yellow) are admixed with the collagen (red) (C) Premortem cardiovascular magnetic admixed with the collagen (red) (C) Premortem cardiovascular magnetic resonance of the same slice with excellent accord between the areas of resonance of the same slice with excellent accord between the areas of
macroscopic fibrosis and areas of late gadolinium enhancement macroscopic fibrosis and areas of late gadolinium enhancement (matching arrows(matching arrows) )
Criteria for Diagnosis Criteria for Diagnosis PERIPARTUM CMDPERIPARTUM CMD
4 Criteria4 Criteriandash Development of Heart failure in the last month Development of Heart failure in the last month
of pregnancy or within 5 months postpartumof pregnancy or within 5 months postpartumndash Absence of a determinable cause for cardiac Absence of a determinable cause for cardiac
failurefailurendash Absence of heart disease before last month of Absence of heart disease before last month of
pregnancypregnancyndash Left Ventricle impairment demonstrated on Left Ventricle impairment demonstrated on
EchoEcho
EKG ChangesEKG Changes
Sinus TachycardiaSinus Tachycardia
Nonspecific ST Nonspecific ST changeschanges
LV HypertrophyLV Hypertrophy
Chest X-rayChest X-ray
Pulmonary EdemaPulmonary Edema
Venous congestionVenous congestion
Enlarged Cardiac Enlarged Cardiac SilhouetteSilhouette
RO PERO PE
EchocardiogramEchocardiogram
Spherical LVSpherical LV
Mitral and Tricuspid Mitral and Tricuspid regurgitationregurgitation
Left Atrial Left Atrial enlargementenlargement
EF lt55EF lt55
Classification of Types of Restrictive Classification of Types of Restrictive Cardiomyopathy According to CauseCardiomyopathy According to Cause
NoninfiltrativeNoninfiltrative Idiopathic cardiomyopathy[] Idiopathic cardiomyopathy[] Familial cardiomyopathy Familial cardiomyopathy Hypertrophic cardiomyopathy Hypertrophic cardiomyopathy Scleroderma Scleroderma Pseudoxanthoma elasticum Pseudoxanthoma elasticum Diabetic cardiomyopathy Diabetic cardiomyopathy
InfiltrativeInfiltrative Amyloidosis[] Amyloidosis[] Sarcoidosis[] Sarcoidosis[] Gaucher disease Gaucher disease Hurler disease Hurler disease Fatty infiltration Fatty infiltration
From Kushwaha S Fallon JT Fuster V From Kushwaha S Fallon JT Fuster V Restrictive cardiomyopathy N Engl J Med Restrictive cardiomyopathy N Engl J Med 336267 1997 Copyright 1997 336267 1997 Copyright 1997 Massachusetts Medical SocietyMassachusetts Medical Society
These conditions are more likely than the These conditions are more likely than the others to be encountered in clinical practiceothers to be encountered in clinical practice
Storage DiseaseStorage Disease Hemochromatosis Hemochromatosis Fabry disease Fabry disease Glycogen storage disease Glycogen storage disease EndomyocardialEndomyocardial Endomyocardial fibrosis[] Endomyocardial fibrosis[] Hypereosinophilic syndrome Hypereosinophilic syndrome Carcinoid heart disease Carcinoid heart disease Metastatic cancers Metastatic cancers Radiation[] Radiation[] Toxic effects of Toxic effects of anthracycline[] anthracycline[] Drugs causing fibrous Drugs causing fibrous endocarditis (serotonin endocarditis (serotonin methysergide ergotamine methysergide ergotamine mercurial agents busulfan)mercurial agents busulfan)
CMRCMR
1
HistorySymptoms
1048708 Asymptomatic for years or suffer from acute pulmonary edema biventricular failure arrhythmias or cardiac
Forward failure
1048708 Backward failure
Most people have BOTH Forward and Backward HF but to differing degrees
(A) Macroscopic short-axis section of the right and left ventricle at a (A) Macroscopic short-axis section of the right and left ventricle at a midventricular level from a patient with dilated cardiomyopathy showing midventricular level from a patient with dilated cardiomyopathy showing midwall fibrosis (straight arrows) mainly in the inferior and lateral walls midwall fibrosis (straight arrows) mainly in the inferior and lateral walls but also in the lower and upper septum (curved arrows) (B) Microscopic but also in the lower and upper septum (curved arrows) (B) Microscopic
section of the heart in which Sirius red staining confirms collagen (arrow) section of the heart in which Sirius red staining confirms collagen (arrow) in areas of fibrosis seen macroscopically Myocytes (stained yellow) are in areas of fibrosis seen macroscopically Myocytes (stained yellow) are admixed with the collagen (red) (C) Premortem cardiovascular magnetic admixed with the collagen (red) (C) Premortem cardiovascular magnetic resonance of the same slice with excellent accord between the areas of resonance of the same slice with excellent accord between the areas of
macroscopic fibrosis and areas of late gadolinium enhancement macroscopic fibrosis and areas of late gadolinium enhancement (matching arrows(matching arrows) )
Criteria for Diagnosis Criteria for Diagnosis PERIPARTUM CMDPERIPARTUM CMD
4 Criteria4 Criteriandash Development of Heart failure in the last month Development of Heart failure in the last month
of pregnancy or within 5 months postpartumof pregnancy or within 5 months postpartumndash Absence of a determinable cause for cardiac Absence of a determinable cause for cardiac
failurefailurendash Absence of heart disease before last month of Absence of heart disease before last month of
pregnancypregnancyndash Left Ventricle impairment demonstrated on Left Ventricle impairment demonstrated on
EchoEcho
EKG ChangesEKG Changes
Sinus TachycardiaSinus Tachycardia
Nonspecific ST Nonspecific ST changeschanges
LV HypertrophyLV Hypertrophy
Chest X-rayChest X-ray
Pulmonary EdemaPulmonary Edema
Venous congestionVenous congestion
Enlarged Cardiac Enlarged Cardiac SilhouetteSilhouette
RO PERO PE
EchocardiogramEchocardiogram
Spherical LVSpherical LV
Mitral and Tricuspid Mitral and Tricuspid regurgitationregurgitation
Left Atrial Left Atrial enlargementenlargement
EF lt55EF lt55
Classification of Types of Restrictive Classification of Types of Restrictive Cardiomyopathy According to CauseCardiomyopathy According to Cause
NoninfiltrativeNoninfiltrative Idiopathic cardiomyopathy[] Idiopathic cardiomyopathy[] Familial cardiomyopathy Familial cardiomyopathy Hypertrophic cardiomyopathy Hypertrophic cardiomyopathy Scleroderma Scleroderma Pseudoxanthoma elasticum Pseudoxanthoma elasticum Diabetic cardiomyopathy Diabetic cardiomyopathy
InfiltrativeInfiltrative Amyloidosis[] Amyloidosis[] Sarcoidosis[] Sarcoidosis[] Gaucher disease Gaucher disease Hurler disease Hurler disease Fatty infiltration Fatty infiltration
From Kushwaha S Fallon JT Fuster V From Kushwaha S Fallon JT Fuster V Restrictive cardiomyopathy N Engl J Med Restrictive cardiomyopathy N Engl J Med 336267 1997 Copyright 1997 336267 1997 Copyright 1997 Massachusetts Medical SocietyMassachusetts Medical Society
These conditions are more likely than the These conditions are more likely than the others to be encountered in clinical practiceothers to be encountered in clinical practice
Storage DiseaseStorage Disease Hemochromatosis Hemochromatosis Fabry disease Fabry disease Glycogen storage disease Glycogen storage disease EndomyocardialEndomyocardial Endomyocardial fibrosis[] Endomyocardial fibrosis[] Hypereosinophilic syndrome Hypereosinophilic syndrome Carcinoid heart disease Carcinoid heart disease Metastatic cancers Metastatic cancers Radiation[] Radiation[] Toxic effects of Toxic effects of anthracycline[] anthracycline[] Drugs causing fibrous Drugs causing fibrous endocarditis (serotonin endocarditis (serotonin methysergide ergotamine methysergide ergotamine mercurial agents busulfan)mercurial agents busulfan)
CMRCMR
(A) Macroscopic short-axis section of the right and left ventricle at a (A) Macroscopic short-axis section of the right and left ventricle at a midventricular level from a patient with dilated cardiomyopathy showing midventricular level from a patient with dilated cardiomyopathy showing midwall fibrosis (straight arrows) mainly in the inferior and lateral walls midwall fibrosis (straight arrows) mainly in the inferior and lateral walls but also in the lower and upper septum (curved arrows) (B) Microscopic but also in the lower and upper septum (curved arrows) (B) Microscopic
section of the heart in which Sirius red staining confirms collagen (arrow) section of the heart in which Sirius red staining confirms collagen (arrow) in areas of fibrosis seen macroscopically Myocytes (stained yellow) are in areas of fibrosis seen macroscopically Myocytes (stained yellow) are admixed with the collagen (red) (C) Premortem cardiovascular magnetic admixed with the collagen (red) (C) Premortem cardiovascular magnetic resonance of the same slice with excellent accord between the areas of resonance of the same slice with excellent accord between the areas of
macroscopic fibrosis and areas of late gadolinium enhancement macroscopic fibrosis and areas of late gadolinium enhancement (matching arrows(matching arrows) )
Criteria for Diagnosis Criteria for Diagnosis PERIPARTUM CMDPERIPARTUM CMD
4 Criteria4 Criteriandash Development of Heart failure in the last month Development of Heart failure in the last month
of pregnancy or within 5 months postpartumof pregnancy or within 5 months postpartumndash Absence of a determinable cause for cardiac Absence of a determinable cause for cardiac
failurefailurendash Absence of heart disease before last month of Absence of heart disease before last month of
pregnancypregnancyndash Left Ventricle impairment demonstrated on Left Ventricle impairment demonstrated on
EchoEcho
EKG ChangesEKG Changes
Sinus TachycardiaSinus Tachycardia
Nonspecific ST Nonspecific ST changeschanges
LV HypertrophyLV Hypertrophy
Chest X-rayChest X-ray
Pulmonary EdemaPulmonary Edema
Venous congestionVenous congestion
Enlarged Cardiac Enlarged Cardiac SilhouetteSilhouette
RO PERO PE
EchocardiogramEchocardiogram
Spherical LVSpherical LV
Mitral and Tricuspid Mitral and Tricuspid regurgitationregurgitation
Left Atrial Left Atrial enlargementenlargement
EF lt55EF lt55
Classification of Types of Restrictive Classification of Types of Restrictive Cardiomyopathy According to CauseCardiomyopathy According to Cause
NoninfiltrativeNoninfiltrative Idiopathic cardiomyopathy[] Idiopathic cardiomyopathy[] Familial cardiomyopathy Familial cardiomyopathy Hypertrophic cardiomyopathy Hypertrophic cardiomyopathy Scleroderma Scleroderma Pseudoxanthoma elasticum Pseudoxanthoma elasticum Diabetic cardiomyopathy Diabetic cardiomyopathy
InfiltrativeInfiltrative Amyloidosis[] Amyloidosis[] Sarcoidosis[] Sarcoidosis[] Gaucher disease Gaucher disease Hurler disease Hurler disease Fatty infiltration Fatty infiltration
From Kushwaha S Fallon JT Fuster V From Kushwaha S Fallon JT Fuster V Restrictive cardiomyopathy N Engl J Med Restrictive cardiomyopathy N Engl J Med 336267 1997 Copyright 1997 336267 1997 Copyright 1997 Massachusetts Medical SocietyMassachusetts Medical Society
These conditions are more likely than the These conditions are more likely than the others to be encountered in clinical practiceothers to be encountered in clinical practice
Storage DiseaseStorage Disease Hemochromatosis Hemochromatosis Fabry disease Fabry disease Glycogen storage disease Glycogen storage disease EndomyocardialEndomyocardial Endomyocardial fibrosis[] Endomyocardial fibrosis[] Hypereosinophilic syndrome Hypereosinophilic syndrome Carcinoid heart disease Carcinoid heart disease Metastatic cancers Metastatic cancers Radiation[] Radiation[] Toxic effects of Toxic effects of anthracycline[] anthracycline[] Drugs causing fibrous Drugs causing fibrous endocarditis (serotonin endocarditis (serotonin methysergide ergotamine methysergide ergotamine mercurial agents busulfan)mercurial agents busulfan)
CMRCMR
Criteria for Diagnosis Criteria for Diagnosis PERIPARTUM CMDPERIPARTUM CMD
4 Criteria4 Criteriandash Development of Heart failure in the last month Development of Heart failure in the last month
of pregnancy or within 5 months postpartumof pregnancy or within 5 months postpartumndash Absence of a determinable cause for cardiac Absence of a determinable cause for cardiac
failurefailurendash Absence of heart disease before last month of Absence of heart disease before last month of
pregnancypregnancyndash Left Ventricle impairment demonstrated on Left Ventricle impairment demonstrated on
EchoEcho
EKG ChangesEKG Changes
Sinus TachycardiaSinus Tachycardia
Nonspecific ST Nonspecific ST changeschanges
LV HypertrophyLV Hypertrophy
Chest X-rayChest X-ray
Pulmonary EdemaPulmonary Edema
Venous congestionVenous congestion
Enlarged Cardiac Enlarged Cardiac SilhouetteSilhouette
RO PERO PE
EchocardiogramEchocardiogram
Spherical LVSpherical LV
Mitral and Tricuspid Mitral and Tricuspid regurgitationregurgitation
Left Atrial Left Atrial enlargementenlargement
EF lt55EF lt55
Classification of Types of Restrictive Classification of Types of Restrictive Cardiomyopathy According to CauseCardiomyopathy According to Cause
NoninfiltrativeNoninfiltrative Idiopathic cardiomyopathy[] Idiopathic cardiomyopathy[] Familial cardiomyopathy Familial cardiomyopathy Hypertrophic cardiomyopathy Hypertrophic cardiomyopathy Scleroderma Scleroderma Pseudoxanthoma elasticum Pseudoxanthoma elasticum Diabetic cardiomyopathy Diabetic cardiomyopathy
InfiltrativeInfiltrative Amyloidosis[] Amyloidosis[] Sarcoidosis[] Sarcoidosis[] Gaucher disease Gaucher disease Hurler disease Hurler disease Fatty infiltration Fatty infiltration
From Kushwaha S Fallon JT Fuster V From Kushwaha S Fallon JT Fuster V Restrictive cardiomyopathy N Engl J Med Restrictive cardiomyopathy N Engl J Med 336267 1997 Copyright 1997 336267 1997 Copyright 1997 Massachusetts Medical SocietyMassachusetts Medical Society
These conditions are more likely than the These conditions are more likely than the others to be encountered in clinical practiceothers to be encountered in clinical practice
Storage DiseaseStorage Disease Hemochromatosis Hemochromatosis Fabry disease Fabry disease Glycogen storage disease Glycogen storage disease EndomyocardialEndomyocardial Endomyocardial fibrosis[] Endomyocardial fibrosis[] Hypereosinophilic syndrome Hypereosinophilic syndrome Carcinoid heart disease Carcinoid heart disease Metastatic cancers Metastatic cancers Radiation[] Radiation[] Toxic effects of Toxic effects of anthracycline[] anthracycline[] Drugs causing fibrous Drugs causing fibrous endocarditis (serotonin endocarditis (serotonin methysergide ergotamine methysergide ergotamine mercurial agents busulfan)mercurial agents busulfan)
CMRCMR
EKG ChangesEKG Changes
Sinus TachycardiaSinus Tachycardia
Nonspecific ST Nonspecific ST changeschanges
LV HypertrophyLV Hypertrophy
Chest X-rayChest X-ray
Pulmonary EdemaPulmonary Edema
Venous congestionVenous congestion
Enlarged Cardiac Enlarged Cardiac SilhouetteSilhouette
RO PERO PE
EchocardiogramEchocardiogram
Spherical LVSpherical LV
Mitral and Tricuspid Mitral and Tricuspid regurgitationregurgitation
Left Atrial Left Atrial enlargementenlargement
EF lt55EF lt55
Classification of Types of Restrictive Classification of Types of Restrictive Cardiomyopathy According to CauseCardiomyopathy According to Cause
NoninfiltrativeNoninfiltrative Idiopathic cardiomyopathy[] Idiopathic cardiomyopathy[] Familial cardiomyopathy Familial cardiomyopathy Hypertrophic cardiomyopathy Hypertrophic cardiomyopathy Scleroderma Scleroderma Pseudoxanthoma elasticum Pseudoxanthoma elasticum Diabetic cardiomyopathy Diabetic cardiomyopathy
InfiltrativeInfiltrative Amyloidosis[] Amyloidosis[] Sarcoidosis[] Sarcoidosis[] Gaucher disease Gaucher disease Hurler disease Hurler disease Fatty infiltration Fatty infiltration
From Kushwaha S Fallon JT Fuster V From Kushwaha S Fallon JT Fuster V Restrictive cardiomyopathy N Engl J Med Restrictive cardiomyopathy N Engl J Med 336267 1997 Copyright 1997 336267 1997 Copyright 1997 Massachusetts Medical SocietyMassachusetts Medical Society
These conditions are more likely than the These conditions are more likely than the others to be encountered in clinical practiceothers to be encountered in clinical practice
Storage DiseaseStorage Disease Hemochromatosis Hemochromatosis Fabry disease Fabry disease Glycogen storage disease Glycogen storage disease EndomyocardialEndomyocardial Endomyocardial fibrosis[] Endomyocardial fibrosis[] Hypereosinophilic syndrome Hypereosinophilic syndrome Carcinoid heart disease Carcinoid heart disease Metastatic cancers Metastatic cancers Radiation[] Radiation[] Toxic effects of Toxic effects of anthracycline[] anthracycline[] Drugs causing fibrous Drugs causing fibrous endocarditis (serotonin endocarditis (serotonin methysergide ergotamine methysergide ergotamine mercurial agents busulfan)mercurial agents busulfan)
CMRCMR
Chest X-rayChest X-ray
Pulmonary EdemaPulmonary Edema
Venous congestionVenous congestion
Enlarged Cardiac Enlarged Cardiac SilhouetteSilhouette
RO PERO PE
EchocardiogramEchocardiogram
Spherical LVSpherical LV
Mitral and Tricuspid Mitral and Tricuspid regurgitationregurgitation
Left Atrial Left Atrial enlargementenlargement
EF lt55EF lt55
Classification of Types of Restrictive Classification of Types of Restrictive Cardiomyopathy According to CauseCardiomyopathy According to Cause
NoninfiltrativeNoninfiltrative Idiopathic cardiomyopathy[] Idiopathic cardiomyopathy[] Familial cardiomyopathy Familial cardiomyopathy Hypertrophic cardiomyopathy Hypertrophic cardiomyopathy Scleroderma Scleroderma Pseudoxanthoma elasticum Pseudoxanthoma elasticum Diabetic cardiomyopathy Diabetic cardiomyopathy
InfiltrativeInfiltrative Amyloidosis[] Amyloidosis[] Sarcoidosis[] Sarcoidosis[] Gaucher disease Gaucher disease Hurler disease Hurler disease Fatty infiltration Fatty infiltration
From Kushwaha S Fallon JT Fuster V From Kushwaha S Fallon JT Fuster V Restrictive cardiomyopathy N Engl J Med Restrictive cardiomyopathy N Engl J Med 336267 1997 Copyright 1997 336267 1997 Copyright 1997 Massachusetts Medical SocietyMassachusetts Medical Society
These conditions are more likely than the These conditions are more likely than the others to be encountered in clinical practiceothers to be encountered in clinical practice
Storage DiseaseStorage Disease Hemochromatosis Hemochromatosis Fabry disease Fabry disease Glycogen storage disease Glycogen storage disease EndomyocardialEndomyocardial Endomyocardial fibrosis[] Endomyocardial fibrosis[] Hypereosinophilic syndrome Hypereosinophilic syndrome Carcinoid heart disease Carcinoid heart disease Metastatic cancers Metastatic cancers Radiation[] Radiation[] Toxic effects of Toxic effects of anthracycline[] anthracycline[] Drugs causing fibrous Drugs causing fibrous endocarditis (serotonin endocarditis (serotonin methysergide ergotamine methysergide ergotamine mercurial agents busulfan)mercurial agents busulfan)
CMRCMR
EchocardiogramEchocardiogram
Spherical LVSpherical LV
Mitral and Tricuspid Mitral and Tricuspid regurgitationregurgitation
Left Atrial Left Atrial enlargementenlargement
EF lt55EF lt55
Classification of Types of Restrictive Classification of Types of Restrictive Cardiomyopathy According to CauseCardiomyopathy According to Cause
NoninfiltrativeNoninfiltrative Idiopathic cardiomyopathy[] Idiopathic cardiomyopathy[] Familial cardiomyopathy Familial cardiomyopathy Hypertrophic cardiomyopathy Hypertrophic cardiomyopathy Scleroderma Scleroderma Pseudoxanthoma elasticum Pseudoxanthoma elasticum Diabetic cardiomyopathy Diabetic cardiomyopathy
InfiltrativeInfiltrative Amyloidosis[] Amyloidosis[] Sarcoidosis[] Sarcoidosis[] Gaucher disease Gaucher disease Hurler disease Hurler disease Fatty infiltration Fatty infiltration
From Kushwaha S Fallon JT Fuster V From Kushwaha S Fallon JT Fuster V Restrictive cardiomyopathy N Engl J Med Restrictive cardiomyopathy N Engl J Med 336267 1997 Copyright 1997 336267 1997 Copyright 1997 Massachusetts Medical SocietyMassachusetts Medical Society
These conditions are more likely than the These conditions are more likely than the others to be encountered in clinical practiceothers to be encountered in clinical practice
Storage DiseaseStorage Disease Hemochromatosis Hemochromatosis Fabry disease Fabry disease Glycogen storage disease Glycogen storage disease EndomyocardialEndomyocardial Endomyocardial fibrosis[] Endomyocardial fibrosis[] Hypereosinophilic syndrome Hypereosinophilic syndrome Carcinoid heart disease Carcinoid heart disease Metastatic cancers Metastatic cancers Radiation[] Radiation[] Toxic effects of Toxic effects of anthracycline[] anthracycline[] Drugs causing fibrous Drugs causing fibrous endocarditis (serotonin endocarditis (serotonin methysergide ergotamine methysergide ergotamine mercurial agents busulfan)mercurial agents busulfan)
CMRCMR
Classification of Types of Restrictive Classification of Types of Restrictive Cardiomyopathy According to CauseCardiomyopathy According to Cause
NoninfiltrativeNoninfiltrative Idiopathic cardiomyopathy[] Idiopathic cardiomyopathy[] Familial cardiomyopathy Familial cardiomyopathy Hypertrophic cardiomyopathy Hypertrophic cardiomyopathy Scleroderma Scleroderma Pseudoxanthoma elasticum Pseudoxanthoma elasticum Diabetic cardiomyopathy Diabetic cardiomyopathy
InfiltrativeInfiltrative Amyloidosis[] Amyloidosis[] Sarcoidosis[] Sarcoidosis[] Gaucher disease Gaucher disease Hurler disease Hurler disease Fatty infiltration Fatty infiltration
From Kushwaha S Fallon JT Fuster V From Kushwaha S Fallon JT Fuster V Restrictive cardiomyopathy N Engl J Med Restrictive cardiomyopathy N Engl J Med 336267 1997 Copyright 1997 336267 1997 Copyright 1997 Massachusetts Medical SocietyMassachusetts Medical Society
These conditions are more likely than the These conditions are more likely than the others to be encountered in clinical practiceothers to be encountered in clinical practice
Storage DiseaseStorage Disease Hemochromatosis Hemochromatosis Fabry disease Fabry disease Glycogen storage disease Glycogen storage disease EndomyocardialEndomyocardial Endomyocardial fibrosis[] Endomyocardial fibrosis[] Hypereosinophilic syndrome Hypereosinophilic syndrome Carcinoid heart disease Carcinoid heart disease Metastatic cancers Metastatic cancers Radiation[] Radiation[] Toxic effects of Toxic effects of anthracycline[] anthracycline[] Drugs causing fibrous Drugs causing fibrous endocarditis (serotonin endocarditis (serotonin methysergide ergotamine methysergide ergotamine mercurial agents busulfan)mercurial agents busulfan)
CMRCMR