3/8/2018 1 Left Ventricular Non-Compaction Case Studies Left Ventricular Non-Compaction Case Studies Matt Umland, ACS, RDCS, FASE Aurora Health Care Milwaukee, WI Matt Umland, ACS, RDCS, FASE Aurora Health Care Milwaukee, WI Left Ventricular Noncompaction Cardiomyopathy Left Ventricular Noncompaction Cardiomyopathy • 1926 Grant - Malformed heart of a child • 1975 Dusek - Spongy Myocardium • 1984 Englberding – Echo Diagnosis of Myocardial Sinusoids • 1986 Jenni – Biventricular Sinusoids • 1990 Chiu Isolated noncompaction • 1926 Grant - Malformed heart of a child • 1975 Dusek - Spongy Myocardium • 1984 Englberding – Echo Diagnosis of Myocardial Sinusoids • 1986 Jenni – Biventricular Sinusoids • 1990 Chiu Isolated noncompaction Historical Perspective LVNC LVNC • Although heterogeneous it has genetic underpinnings – TAZ gene • Autosomal dominant • Sarcomeric genes • Our knowledge and understanding regarding prevalence and mortality is similar to HCM 50 years ago • Although heterogeneous it has genetic underpinnings – TAZ gene • Autosomal dominant • Sarcomeric genes • Our knowledge and understanding regarding prevalence and mortality is similar to HCM 50 years ago Left Ventricular Noncompaction Cardiomyopathy
Transcript
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Left Ventricular Non-Compaction Case Studies
Left Ventricular Non-Compaction Case Studies
Matt Umland, ACS, RDCS, FASEAurora Health Care
Milwaukee, WI
Matt Umland, ACS, RDCS, FASEAurora Health Care
Milwaukee, WI
Left Ventricular Noncompaction Cardiomyopathy
Left Ventricular Noncompaction Cardiomyopathy
• 1926 Grant - Malformed heart of a child
• 1975 Dusek - Spongy Myocardium
• 1984 Englberding – Echo Diagnosis of Myocardial Sinusoids
• 1986 Jenni – Biventricular Sinusoids
• 1990 Chiu Isolated noncompaction
• 1926 Grant - Malformed heart of a child
• 1975 Dusek - Spongy Myocardium
• 1984 Englberding – Echo Diagnosis of Myocardial Sinusoids
• 1986 Jenni – Biventricular Sinusoids
• 1990 Chiu Isolated noncompaction
Historical Perspective
LVNCLVNC
• Although heterogeneous it has genetic underpinnings – TAZ gene
• Autosomal dominant• Sarcomeric genes •Our knowledge and understanding regarding prevalence and mortality is similar to HCM 50 years ago
• Although heterogeneous it has genetic underpinnings – TAZ gene
• Autosomal dominant• Sarcomeric genes •Our knowledge and understanding regarding prevalence and mortality is similar to HCM 50 years ago
Left Ventricular Noncompaction
Cardiomyopathy
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J Am Soc Echocardiogr. 2012 Apr;25(4):363-75
Left Ventricular NoncompactionCardiomyopathy
Left Ventricular NoncompactionCardiomyopathy
• Persistent Sinusoids
• Spongy myocardium
• Embo myocardium
• Honeycomb LV
• Hypertrabeculations
• Isolated LVNC
• Persistent Sinusoids
• Spongy myocardium
• Embo myocardium
• Honeycomb LV
• Hypertrabeculations
• Isolated LVNC
Nomenclature
Normal Noncompacted
Myocardium
Non-Compaction Fetal Heart DevelopmentFetal Heart Development
• Week 3-4 of fetal life
• No coronary circulation
•Compaction
•Coronary arteries
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HistologyHistology The SPECTRUM of LVNCThe SPECTRUM of LVNC
• Heterogeneity
• From 12-18 weeks of gestation until the 90s
• Heterogeneity
• From 12-18 weeks of gestation until the 90s
0
10
20
30
40
50
60
70
Presenting SymptomsPresenting Symptoms
66%
15%
SOB Chest Pain Palpitations
13%
5%
Embolism
3%
Syncope
Left Ventricular NoncompactionCardiomyopathy
Left Ventricular NoncompactionCardiomyopathy
• Bilayered myocardium (C+NC)
• Ratio of NC/C ≥ 2.0
• Large trabecular
• Prominent intertrabecular recesser
• Apical location
• Bilayered myocardium (C+NC)
• Ratio of NC/C ≥ 2.0
• Large trabecular
• Prominent intertrabecular recesser
• Apical location
Diagnostic Criteria
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Left Ventricular Noncompaction Cardiomyopathy
Left Ventricular Noncompaction Cardiomyopathy
Left Ventricular Noncompaction Cardiomyopathy
Left Ventricular Noncompaction Cardiomyopathy
Left Ventricular Noncompaction Cardiomyopathy
Left Ventricular Noncompaction Cardiomyopathy
Left Ventricular Noncompaction Cardiomyopathy
Left Ventricular Noncompaction Cardiomyopathy
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Left Ventricular Noncompaction Cardiomyopathy
Left Ventricular Noncompaction Cardiomyopathy
Panel A
Panel DPanel C
Panel B
LV
RV
LV
RV
LV
LV
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clot
clot
LV
RV
MRI
• This is the major and primary diagnostic imaging criterion
• It is critical to obtain images that are not foreshortened and are perpendicular to the ventricular long-axis view
• This is the major and primary diagnostic imaging criterion
• It is critical to obtain images that are not foreshortened and are perpendicular to the ventricular long-axis view
Tips & Tricks in the Echo Lab
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• Meticulous attention must be paid to the short-axis images, and a circular cavity image should be obtained and maintained in all short-axis views.
• Oblique and tangential cuts (noncircular LV cavity appearance) should not be used to measuring noncompacted and compacted layers as this will lead to false diagnosis of LVNC.
• Meticulous attention must be paid to the short-axis images, and a circular cavity image should be obtained and maintained in all short-axis views.
• Oblique and tangential cuts (noncircular LV cavity appearance) should not be used to measuring noncompacted and compacted layers as this will lead to false diagnosis of LVNC.
Tips & Tricks
• The use of contrast for opacification of the LV apex and demonstration of the intertrabecular recesses has been beneficial in selected cases as discussed in the literature.
• The use of contrast for opacification of the LV apex and demonstration of the intertrabecular recesses has been beneficial in selected cases as discussed in the literature.
