Myocardial and Pericardial Disease: Difference between revisions
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==Myocardial Disease== | ==Myocardial Disease== | ||
Overall, myocardial diseases can be subdivided into two types: primary and secondary myocardial diseases. Whereas the primary type most commonly has a genetic cause, secondary myocardial diseases are mostly acquired but may be precipitated by a genetic background. Overall, the dilated type is the most prevalent manifestation of cardiomyopathy, and may be induced by a multitude of precipitating factors, such as chronic ischemia or alcohol abuse. The most common primary myocardial disease is hypertrophic cardiomyopathy, which is associated with a wide variety of genetic abnormalities. In the light of their substantially larger prevalence, we will first describe the secondary myocardial diseases, after which the less frequently occurring primary myocardial diseases will be discussed. | Overall, myocardial diseases can be subdivided into two types: primary and secondary myocardial diseases. Whereas the primary type most commonly has a genetic cause, secondary myocardial diseases are mostly acquired but may be precipitated by a genetic background. Overall, the dilated type is the most prevalent manifestation of cardiomyopathy, and may be induced by a multitude of precipitating factors, such as chronic ischemia or alcohol abuse. The most common primary myocardial disease is hypertrophic cardiomyopathy, which is associated with a wide variety of genetic abnormalities. In the light of their substantially larger prevalence, we will first describe the secondary myocardial diseases, after which the less frequently occurring primary myocardial diseases will be discussed. | ||
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!Table 5 -- Classification of Restrictive Cardiomyopathy | !Table 5 -- Classification of Restrictive Cardiomyopathy | ||
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|Myocardial | |'''Myocardial''' | ||
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| | |'''''Noninfiltrative''''' | ||
*Idiopathic cardiomyopathy | *Idiopathic cardiomyopathy | ||
*Familial cardiomyopathy | *Familial cardiomyopathy | ||
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*Diabetic cardiomyopathy | *Diabetic cardiomyopathy | ||
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|Infiltrative | |'''''Infiltrative''''' | ||
*Amyloidosis | *Amyloidosis | ||
*Sarcoidosis | *Sarcoidosis | ||
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*Fatty infiltration | *Fatty infiltration | ||
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|Storage Disease | |'''''Storage Disease''''' | ||
*Hemochromatosis | *Hemochromatosis | ||
*Fabry disease | *Fabry disease | ||
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*Glycogen storage disease | *Glycogen storage disease | ||
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|Endomyocardial | |'''''Endomyocardial''''' | ||
*Endomyocardial fibrosis | *Endomyocardial fibrosis | ||
*Hypereosinophilic syndrome | *Hypereosinophilic syndrome | ||
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! Table 6. The Revised Task Force Criteria for ARVD / ARVC Revised Task Force Criteria | ! Table 6. The Revised Task Force Criteria for ARVD / ARVC Revised Task Force Criteria | ||
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!'''I. Global or regional dysfunction and structural alterations ∗ Major | !'''I. Global or regional dysfunction and structural alterations ∗''' | ||
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|'''Major''' | |||
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|'''By 2D echo:''' | |'''By 2D echo:''' | ||
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|'''By RV angiography:''' | |'''By RV angiography:''' | ||
*Regional RV akinesia, dyskinesia, or aneurysm | *Regional RV akinesia, dyskinesia, or aneurysm | ||
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|'''Minor''' | |||
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|'''By 2D echo:''' | |'''By 2D echo:''' | ||
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**or RV ejection fraction >40% to =45% | **or RV ejection fraction >40% to =45% | ||
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!II. Tissue characterization of wall Major | !II. Tissue characterization of wall | ||
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|'''Major''' | |||
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|Residual myocytes <60% by morphometric analysis (or <50% if estimated), with fibrous replacement of the RV free wall myocardium in =1 sample, with or without fatty replacement of tissue on endomyocardial biopsy | |Residual myocytes <60% by morphometric analysis (or <50% if estimated), with fibrous replacement of the RV free wall myocardium in =1 sample, with or without fatty replacement of tissue on endomyocardial biopsy | ||
|- | |||
|'''Minor''' | |||
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|Residual myocytes 60% to 75% by morphometric analysis (or 50% to 65% if estimated), with fibrous replacement of the RV free wall myocardium in =1 sample, with or without fatty replacement of tissue on endomyocardial biopsy | |Residual myocytes 60% to 75% by morphometric analysis (or 50% to 65% if estimated), with fibrous replacement of the RV free wall myocardium in =1 sample, with or without fatty replacement of tissue on endomyocardial biopsy | ||
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!III. Repolarization abnormalities Major | !III. Repolarization abnormalities | ||
|- | |||
|'''Major''' | |||
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|Inverted T waves in right precordial leads (V<sub>1</sub>, V<sub>2</sub>, and V<sub>3</sub>) or beyond in individuals >14 years of age (in the absence of complete right bundle-branch block QRS =120 ms) | |Inverted T waves in right precordial leads (V<sub>1</sub>, V<sub>2</sub>, and V<sub>3</sub>) or beyond in individuals >14 years of age (in the absence of complete right bundle-branch block QRS =120 ms) | ||
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|'''Minor''' | |||
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|Inverted T waves in leads V<sub>1</sub> and V<sub>2</sub> in individuals >14 years of age (in the absence of complete right bundle-branch block) or in V<sub>4</sup>, V<sub>5</sub>, or V<sub>6</sub> | |Inverted T waves in leads V<sub>1</sub> and V<sub>2</sub> in individuals >14 years of age (in the absence of complete right bundle-branch block) or in V<sub>4</sup>, V<sub>5</sub>, or V<sub>6</sub> | ||
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|Inverted T waves in leads V1, V<sub>2</sub>, V<sub>3</sub>, and V<sub>4</sub> in individuals >14 years of age in the presence of complete right bundle-branch block | |Inverted T waves in leads V1, V<sub>2</sub>, V<sub>3</sub>, and V<sub>4</sub> in individuals >14 years of age in the presence of complete right bundle-branch block | ||
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!IV. Depolarization/conduction abnormalities Major | !IV. Depolarization/conduction abnormalities | ||
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|'''Major''' | |||
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| | | | ||
*Epsilon wave (reproducible low-amplitude signals between end of QRS complex to onset of the T wave) in the right precordial leads (V<sub>1</sub> to V<sub>3</sub>) | *Epsilon wave (reproducible low-amplitude signals between end of QRS complex to onset of the T wave) in the right precordial leads (V<sub>1</sub> to V<sub>3</sub>) | ||
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|'''Minor''' | |||
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| | |||
*Late potentials by SAECG in =1 of 3 parameters in the absence of a QRS duration of =110 ms on the standard ECG | *Late potentials by SAECG in =1 of 3 parameters in the absence of a QRS duration of =110 ms on the standard ECG | ||
*Filtered QRS duration (fQRS) =114 ms | *Filtered QRS duration (fQRS) =114 ms | ||
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*Terminal activation duration of QRS =55 ms measured from the nadir of the S wave to the end of the QRS, including R´, in V<sub>1</sub>, V<sub>2</sub>, or V<sub>3</sub>, in the absence of complete right bundle-branch block | *Terminal activation duration of QRS =55 ms measured from the nadir of the S wave to the end of the QRS, including R´, in V<sub>1</sub>, V<sub>2</sub>, or V<sub>3</sub>, in the absence of complete right bundle-branch block | ||
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!V. Arrhythmias Major | !V. Arrhythmias | ||
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|'''Major''' | |||
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| | | | ||
*Nonsustained or sustained ventricular tachycardia of left bundle-branch morphology with superior axis (negative or indeterminate QRS in leads II, III, and aVF and positive in lead aVL) | *Nonsustained or sustained ventricular tachycardia of left bundle-branch morphology with superior axis (negative or indeterminate QRS in leads II, III, and aVF and positive in lead aVL) | ||
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|'''Minor''' | |||
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| | |||
*Nonsustained or sustained ventricular tachycardia of RV outflow configuration, left bundle-branch block morphology with inferior axis (positive QRS in leads II, III, and aVF and negative in lead aVL) or of unknown axis | *Nonsustained or sustained ventricular tachycardia of RV outflow configuration, left bundle-branch block morphology with inferior axis (positive QRS in leads II, III, and aVF and negative in lead aVL) or of unknown axis | ||
*>500 ventricular extrasystoles per 24 hours (Holter) | *>500 ventricular extrasystoles per 24 hours (Holter) | ||
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!VI. Family history Major | !VI. Family history | ||
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|'''Major''' | |||
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| | | | ||
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*ARVC/D confirmed pathologically at autopsy or surgery in a first-degree relative | *ARVC/D confirmed pathologically at autopsy or surgery in a first-degree relative | ||
*Identification of a pathogenic mutation† categorized as associated or probably associated with ARVC/D in the patient under evaluation | *Identification of a pathogenic mutation† categorized as associated or probably associated with ARVC/D in the patient under evaluation | ||
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|'''Minor''' | |||
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| | |||
*History of ARVC/D in a first-degree relative in whom it is not possible or practical to determine whether the family member meets current Task Force criteria | *History of ARVC/D in a first-degree relative in whom it is not possible or practical to determine whether the family member meets current Task Force criteria | ||
*Premature sudden death (<35 years of age) due to suspected ARVC/D in a first-degree relative | *Premature sudden death (<35 years of age) due to suspected ARVC/D in a first-degree relative | ||
*ARVC/D confirmed pathologically or by current Task Force Criteria in second-degree relative | *ARVC/D confirmed pathologically or by current Task Force Criteria in second-degree relative | ||
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*PLAX indicates parasternal long-axis view; RVOT, RV outflow tract; BSA, body surface area; PSAX, parasternal short-axis view; aVF, augmented voltage unipolar left foot lead; and aVL, augmented voltage unipolar left arm lead. | *PLAX indicates parasternal long-axis view; RVOT, RV outflow tract; BSA, body surface area; PSAX, parasternal short-axis view; aVF, augmented voltage unipolar left foot lead; and aVL, augmented voltage unipolar left arm lead. | ||
*Diagnostic terminology for original criteria: This diagnosis is fulfilled by the presence of 2 major, or 1 major plus 2 minor criteria or 4 minor criteria from different groups. Diagnostic terminology for revised criteria: definite diagnosis: 2 major or 1 major and 2 minor criteria or 4 minor from different categories; borderline: 1 major and 1 minor or 3 minor criteria from different categories; possible: 1 major or 2 minor criteria from different categories. | *Diagnostic terminology for original criteria: This diagnosis is fulfilled by the presence of 2 major, or 1 major plus 2 minor criteria or 4 minor criteria from different groups. Diagnostic terminology for revised criteria: definite diagnosis: 2 major or 1 major and 2 minor criteria or 4 minor from different categories; borderline: 1 major and 1 minor or 3 minor criteria from different categories; possible: 1 major or 2 minor criteria from different categories. | ||
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|∗ Hypokinesis is not included in this or subsequent definitions of RV regional wall motion abnormalities for the proposed modified criteria. | |||
† A pathogenic mutation is a DNA alteration associated with ARVC/D that alters or is expected to alter the encoded protein, is unobserved or rare in a large non–ARVC/D control population, and either alters or is predicted to alter the structure or function of the protein or has demonstrated linkage to the disease phenotype in a conclusive pedigree. E.g.: in TMEM43, DSP, PKP2, DSG2, DSC2, JUP. | |||
|} | |} | ||
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!Restrictive cardiomyopathy | !Restrictive cardiomyopathy | ||
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|Physical examination | |colspan="2"|'''Physical examination''' | ||
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|Early diastolic precordial impulse | |Early diastolic precordial impulse | ||
|Apical impulse may be prominent | |Apical impulse may be prominent | ||
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|Pericardial knock | |colspan="2"|'''Pericardial knock''' | ||
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No murmur | |No murmur | ||
|Third sound may be present | |Third sound may be present | ||
Regurgitant murmur common | Regurgitant murmur common | ||
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|Electrocardiography | |colspan="2"|'''Electrocardiography''' | ||
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|Low voltage | |Low voltage | ||
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Bundle branch block | Bundle branch block | ||
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|Chest radiogram | |colspan="2"|'''Chest radiogram''' | ||
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|Pericardial calcification possible | |Pericardial calcification possible | ||
|Non-specific cardiomegaly | |Non-specific cardiomegaly | ||
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|Echocardiography | |colspan="2"|'''Echocardiography''' | ||
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|Normal wall thickness | |Normal wall thickness | ||
|Pericardial thickening | |Pericardial thickening | ||
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| | |colspan="2"|'''Diastolic notch of interventricular septum''' | ||
|Diastolic notch of interventricular septum | |||
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|Increased wall thickness (amyloidosis) | |Increased wall thickness (amyloidosis) | ||
|Enlarged left and right atria | |Enlarged left and right atria | ||
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|Doppler studies e' septal =8 cm/sec and normal S' mitral annular velocity | |colspan="2"|'''Doppler studies e' septal =8 cm/sec and normal S' mitral annular velocity''' | ||
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|Mitral inflow increase during expiration | |Mitral inflow increase during expiration | ||
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Increased diastolic flow reversal in the hepatic vein with inspiration | Increased diastolic flow reversal in the hepatic vein with inspiration | ||
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|Cardiac catheterization | |colspan="2"|'''Cardiac catheterization''' | ||
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|RVEDP and LVEDP usually equal | |RVEDP and LVEDP usually equal | ||
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LVEDP often >5mm greater than RVEDP | LVEDP often >5mm greater than RVEDP | ||
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|Endomyocardial biopsy | |colspan="2"|'''Endomyocardial biopsy''' | ||
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|Normal or non-specific changes | |Normal or non-specific changes | ||
|May reveal specific causes | |May reveal specific causes | ||
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|CT/MR imaging | |colspan="2"|'''CT/MR imaging''' | ||
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|Pericardium thickened or calcified | |Pericardium thickened or calcified | ||
|Normal pericardium | |Normal pericardium | ||
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|CT, computer tomography; | |colspan="2"|'''CT, computer tomography; e', e wave velocity by tissue velocity imaging;''' | ||
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| | |LVEDP, left ventricular end-diastolic pressure; | ||
LVEDP, left ventricular end-diastolic pressure; | |||
MR, magnetic resonance; | MR, magnetic resonance; |