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(Circulation 2017;135:)
1/16/2018
11/16/2017
Anomalies of Inferior Vena Cava
Interrupted IVC with Azygos Vein Continuation
(Right supracardinal vein > Azygos vein)
Bilateral IVC
Both images above are from Netter's Atlas
(From Tal Geva. Anomalies of Systemic Veins chapter in Moss & Adams 8th ed. Page 856)
11/09/2017
11/07/2017
11/04/2017
Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia - Task Force Criteria for Diagnosis
McKenna et al. British Heart J. 1994;71:215-8. (Original criteria)
Marcus, FI. et al. Circulation 2010;121:1533-41. (Revised criteria - listed below (Article is available for free)
Diagnostic levels: (Definite, Borderline and Possible)
Definite diagnosis - 2 majors or 1 major & 2 minor criteria or 4 minor criteria from diff. categories.
Borderline diagnosis - 1 major & 1 minor, or 3 minor criteria from different categories
Possible diagnosis - 1 major or 2 minor criteria from different categories.
The diagnostic criteria fall under 6 different categories:
I) Dysfunction or Structural alterations
II) Tissue characterization
III) Repolarization abnormalities
IV) Depolarization abnormalities
V) Arrhythmias
VI) Family history
By 2D echo:
(i) Regional RV akinesia, dyskinesia or aneurysm & one of the following three
(a) PLAx (end diastole) RVOT ≥ 32 mm (≥19 mm/m2)
(b) PSAx (end diastole) RVOT ≥ 36 mm (≥ 21 mm/m2)
(c) ≤ FAC 33%
By MRI:
(i) Regional RV akinesia or dyskinesia or dyssynchronious RV contraction & one of the following two.
(a) RVEDV ≥ 110 ml/m2 for male, ≥ 100 ml/m2 for female
(b) ≤ RVEF 40%
By RV angiography:
(i) Regional RV akinesia, dyskinesia or aneurysm.
Minor -
By 2D echo:
(i) Regional RV akinesia or dyskinesia & one of the following three
(a) PLAx (end diastole) RVOT 29 - 32 mm (16 - 19 mm/m2)
(b) PSAx (edn diastole) RVOT 32 - 36 mm (18 - 21 mm/m2)
(c) ≤ FAC 33 - 40%
By MRI:
(i) Regional RV akinesia or dyskinesia or dyssynchronious RV contraction & one of the following two.
(a) RVEDV 100 - 110 ml/m2 for male, 90 - 100 ml/m2 for female
(b) RVEF 40 - 45%
(i) Biopsy - Residual myocytes < 60% by morphometric analysis (or < 50% if estimated) with fibrous replacement of RV free wall myocardium (with or without fatty replacement)
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without="">
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without="">Minor -
(i) Biospy - Residual myocytes 60-75% by morphometric analysis or 50-65% if estimated) with fibrous replacement of RV free wall myocardium (with or without fatty replacement).
Minor -
(i) T wave inversion in V1-V2 (in >14 yrs of age, absence of RBBB) or in V4, V5 or V6.
(ii) T wave inversion in V1 - V4 with RBBB.
(i) Epsilon wave in V1 - V3 (Reproducible, low-amplitude signals between QRS and T wave)
Minor -
(i) Late potential in Signal-averaged ECG (in ≥ 1 of 3 parameters in the absence of a QRS ≥ 110 ms in standard ECG)
(ii) Filtered QRS duration (fQRS) ≥ 114 ms.
(iii) Duration of terminal QRS (< 40 µV) ≥ 38 ms.
(iv) Root-Mean-Square voltage of terminal 40 ms ≤ 20 µV
(v) Terminal activation duration of QRS ≥ 55 ms measured from nadir of the S wave to the end of the QRS, including R' in V1, V2 or V3 in the absence of RBBB.
Minor -
(i) V-Tach with RVOT morphology i.e. LBBB morphology with inferior axis (positive in II, III and aVF & negative in aVL) or unknown axis.
(ii) > 500 PVCs in 24 hrs. (Holter)
(ii) First-degree relative with ARVC/D confirmed by surgery or autopsy
(iii) Pathogenic mutation (associated or probably associated with ARVC/D) found in the patient under evaluation (Technically, this is not "family history")
Minor -
(i) First-degree relative with history of ARVC/D that cannot be confirmed with current criteria
(ii) First-degree relative with sudden death < 35 years of age in whom ARVC/D was suspected as cause
(iii) Second-degree relative with confirmed ARVC/D with current criteria or by pathology
✪Treatment of ARVC/D - International Consensus Statement. Circulation 2015;132:441-53. (Article available for free).
Marcus, FI. et al. Circulation 2010;121:1533-41. (Revised criteria - listed below (Article is available for free)
Diagnostic levels: (Definite, Borderline and Possible)
Definite diagnosis - 2 majors or 1 major & 2 minor criteria or 4 minor criteria from diff. categories.
Borderline diagnosis - 1 major & 1 minor, or 3 minor criteria from different categories
Possible diagnosis - 1 major or 2 minor criteria from different categories.
The diagnostic criteria fall under 6 different categories:
I) Dysfunction or Structural alterations
II) Tissue characterization
III) Repolarization abnormalities
IV) Depolarization abnormalities
V) Arrhythmias
VI) Family history
I) Dysfunction or Structural alterations
Major -By 2D echo:
(i) Regional RV akinesia, dyskinesia or aneurysm & one of the following three
(a) PLAx (end diastole) RVOT ≥ 32 mm (≥19 mm/m2)
(b) PSAx (end diastole) RVOT ≥ 36 mm (≥ 21 mm/m2)
(c) ≤ FAC 33%
By MRI:
(i) Regional RV akinesia or dyskinesia or dyssynchronious RV contraction & one of the following two.
(a) RVEDV ≥ 110 ml/m2 for male, ≥ 100 ml/m2 for female
(b) ≤ RVEF 40%
By RV angiography:
(i) Regional RV akinesia, dyskinesia or aneurysm.
Minor -
By 2D echo:
(i) Regional RV akinesia or dyskinesia & one of the following three
(a) PLAx (end diastole) RVOT 29 - 32 mm (16 - 19 mm/m2)
(b) PSAx (edn diastole) RVOT 32 - 36 mm (18 - 21 mm/m2)
(c) ≤ FAC 33 - 40%
By MRI:
(i) Regional RV akinesia or dyskinesia or dyssynchronious RV contraction & one of the following two.
(a) RVEDV 100 - 110 ml/m2 for male, 90 - 100 ml/m2 for female
(b) RVEF 40 - 45%
II) Tissue characterization (Fibrofatty replacement of myocardium)
Major -(i) Biopsy - Residual myocytes < 60% by morphometric analysis (or < 50% if estimated) with fibrous replacement of RV free wall myocardium (with or without fatty replacement)
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without="">
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without="">Minor -
(i) Biospy - Residual myocytes 60-75% by morphometric analysis or 50-65% if estimated) with fibrous replacement of RV free wall myocardium (with or without fatty replacement).
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without=""> III) Repolarization abnormalities:
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without=""> Major - (i) T wave inversion in V1-V3 or beyond (in >14 yrs of age, in the absence of RBBB and QRS ≥120 ms).Minor -
(i) T wave inversion in V1-V2 (in >14 yrs of age, absence of RBBB) or in V4, V5 or V6.
(ii) T wave inversion in V1 - V4 with RBBB.
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without=""> IV) Depolarization abnormalities:
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without=""> Major -(i) Epsilon wave in V1 - V3 (Reproducible, low-amplitude signals between QRS and T wave)
Minor -
(i) Late potential in Signal-averaged ECG (in ≥ 1 of 3 parameters in the absence of a QRS ≥ 110 ms in standard ECG)
(ii) Filtered QRS duration (fQRS) ≥ 114 ms.
(iii) Duration of terminal QRS (< 40 µV) ≥ 38 ms.
(iv) Root-Mean-Square voltage of terminal 40 ms ≤ 20 µV
(v) Terminal activation duration of QRS ≥ 55 ms measured from nadir of the S wave to the end of the QRS, including R' in V1, V2 or V3 in the absence of RBBB.
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without=""> V) Arrhythmias:
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without=""> Major - (i) V-Tach with LBBB morphology with superior axis (negative in II, III and aVF & positive in aVL)Minor -
(i) V-Tach with RVOT morphology i.e. LBBB morphology with inferior axis (positive in II, III and aVF & negative in aVL) or unknown axis.
(ii) > 500 PVCs in 24 hrs. (Holter)
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without=""> VI) Family history:
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without=""> Major - (i) First-degree relative with ARVC/D confirmed by current criteria(ii) First-degree relative with ARVC/D confirmed by surgery or autopsy
(iii) Pathogenic mutation (associated or probably associated with ARVC/D) found in the patient under evaluation (Technically, this is not "family history")
Minor -
(i) First-degree relative with history of ARVC/D that cannot be confirmed with current criteria
(ii) First-degree relative with sudden death < 35 years of age in whom ARVC/D was suspected as cause
(iii) Second-degree relative with confirmed ARVC/D with current criteria or by pathology
✪Treatment of ARVC/D - International Consensus Statement. Circulation 2015;132:441-53. (Article available for free).
11/03/2017
Aortico-Left Ventricular Tunnel
Types:
Type 1 - Slit-like tunnel without valve distortion
Type 2 - Aneurysm - extracardiac
Type 3 - Aneurysm - intracardiac
Type 4 - Aneurysm - both extracardiac and intracardiac.
From Kim & Spray. Sem Thorac Cardiovasc Surg, Pediatr Cardiac Surg Annu 2006:177-9.
Differential diagnosis for Ao-LV tunnel include
1) Sinus of Valsalva Aneurysm (Coronary artery origin will be above the level of aneurysm of SoV, while coronary artery origin will be below the tunnel origin in Ao-LV tunnel).
2) Coronary fistula (Origin of coronary artery will not be identifiable separate from the origin of the tunnel)
Of course, an eccentric jet of AR and paravalvar leaks with prosthetic valves provide similar color signals. But, these two will be obviously different.
Type 1 - Slit-like tunnel without valve distortion
Type 2 - Aneurysm - extracardiac
Type 3 - Aneurysm - intracardiac
Type 4 - Aneurysm - both extracardiac and intracardiac.
Differential diagnosis for Ao-LV tunnel include
1) Sinus of Valsalva Aneurysm (Coronary artery origin will be above the level of aneurysm of SoV, while coronary artery origin will be below the tunnel origin in Ao-LV tunnel).
2) Coronary fistula (Origin of coronary artery will not be identifiable separate from the origin of the tunnel)
Of course, an eccentric jet of AR and paravalvar leaks with prosthetic valves provide similar color signals. But, these two will be obviously different.
7/20/2017
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