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DILATED CARDIOMYOPATHY DILATED CARDIOMYOPATHY

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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

CMRCMR