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Left ventricular Non Compaction Cardiomyopathy in ACHD
Dr CHARALAMPOS KAVVOURAS MD, Msc
ACHD Consultant Liverpool Heart and Chest Hospital, UK.
Multimodality Imaging in ACHD and PH with implementation of ISACHD protocol RBHT September 2019
Disclosures
• No conflict of interest regarding this talk
Definition
• Noncompaction cardiomyopathy (NCCM) is a cardiomyopathy with excessive trabeculations of the LV.
• Initially referred to as “spongy” heart
• Spongiform cardiomyopathy • Myocardial sinusoids • Embryonic myocardium • Ventricular hypertrabeculation • Anomalous ventricular myocardial pattern • Ventricular dysplasia • Honey-combed ventricle • Isolated ventricular abnormal trabeculation
Nomenclature for LVNC
Classification of Cardiomyopathies
MaronB J et al. Circulation 2006;113:1807
Classification of Cardiomyopathies
Elliott P et al. EurHeart J 2008;29:270
Historical background
•Spongy appearance of myocardium 1926 by Grant •Persistent isolated myocardial sinusoids 1967 by Bender •Echocardiography 1984 by Engberding •Detailed description 1986 by Jenni •Term non-compaction 1990 by Chin
First time recognized/reported
Jenni R. Cardiovasc Intervent Radiol 1986;9:127
Persisting Sinusoids •Primitive vessels (occurring earlier than coronary arteries) •Remnants in the normal heart: venae cordis minimae •When persisting: associated with pulmonary atresia, congenital pulmonary stenosis, congenital aortic stenosis •When persisting: endothelium-lined and wide communications between ventricles and coronary arteries
Krishnan U et al. Circulation 2008;117:e315
Arrows: arteriosinusoidal fistulae filling with contrast agent on injection, delineating the endocardial border
Morphology of Non-compaction
Normal
Non-compaction
Left: half of the heart of a 21-year-old man. There are both numerous trabeculations and deep recesses. fibroelastosis of the LV. Right: transmural, histologic section There are both an epicardial (compacted) layer (arrows) and an endocardial (noncompacted) layer. The necrosis within the trabeculations (asterisks) .
Diagnosis of Non-compaction
Diagnostic Criteria for Non-compaction
• Absence of coexisting cardiac abnormalities by definition
• Typical two-layered structure of the myocardium with a thin, compacted outer (epicardial) layer and a much thicker, non-compacted inner (endocardial) layer consisting of trabecular meshwork with deep endocardial spaces
• Maximal end systolic ratio of non-compacted to compacted layer >2
• Segmental location with non-compacted myocardium predominantly (80%) found in the apical and mid-ventricular areas of the inferior and lateral wall
• Color Doppler: deeply perfused intertrabecular recesses; intertrabecular spaces do communicate with the ventricular lumen, but not the coronary circulation
Jenni et al. EurHeart J 2011;32:1446-1456
Non-compaction Normal
Contrast for Diagnosis of Non-compaction
• Contrast does not facilitate the diagnosis of non-compaction
• Contrast improves the identification of the compacted myocardium
• Contrast improves the measurement of LV end diastolic and end systolic volume
• Contrast improves the measurement of LV ejection fraction
Diagnostic criteria (MRI)
HCM & Non-compaction
Occurrence of Non-compaction
• Isolated
• Congenital heart disease:
» Ebstein anomaly » Pulmonary atresia » Aortic stenosis » Ventricular septal defect
• Genetic syndromes: • Noonan syndrome(cardiac anomalies: PS, ASD, VSD, HOCM) • Barth syndrome(cardiac anomalies: ventricular arrhythmias, DCM)
• Neuromuscular disorders
• Duchenne • Becker
LVNC in ACHD
• A total of 110 (1.38%) patients with an echocardiographic diagnosis of VH were identified out of 7995 .
• The most common underlying CHD diagnosis
• Aortic coarctation (n=25, 23%),
• Bicuspid aortic valve (n=23, 20.9%),
• Tetralogy of Fallot (n=20, 18.2%)
• Ebstein anomaly (n=18, 16.3%).
• The prevalence of VH was highest in patients with
• Ebstein anomaly (11.2% )
• aortic coarctation (3.8%)
• Tetralogy of Fallot (2.7%).
• Bicuspid aortic valve (2.4%),
• RBHT DATA BASE MAY 2018
LVNC in ACHD
The LV apex (n=79, 71.8%), Lateral Wall (n=53, 48.2%) Inferior Wall (n=17, 15.5%) 37 patients had more than 1 segment of the LV were involved (36%). RV involvement was observed in 27 (24.5%) patients, more frequently in ToF: 48.0% Kavvouras Et.al under review by JACC
Baseline demographic and clinical characteristics of patients with congenital heart disease and ventricular hypertrabeculation
Parameters
Patients (n) 110
Males 61%
Age, years
Diagnosis
TOF
BAV
Aortic coarctation
Ebstein anomaly
Clinical events
Death
Hospitalization for heart failure
Arrhythmias
SVT
New onset Afib
NSVT
VT
ICD implantation
Pulmonary embolism
33.5 ± 17.9
20(18.2%)
23(20.9%)
25(23%)
18(16.3%)
14 (12%)
50 (45%)
83 (75%)
55
5
20
8
22(20%)
6 (5%)
Kavvouras Et.al under review by JACC 07/2019
Clinical Course of Non-compaction
GreutmannM et al. Am J Cardiol2012;109:276
Kavvouras Et.al under review by JACC
Kavvouras Et.al under review by JACC
Survival in CHD patients with VH according to LVEF and LV strain
Kavvouras Et.al under review by JACC
Clinical outcome of LVNC
Therapy of Patients with Non-compaction
• Similar to patients with dilated cardiomyopathy
• ICD: OMT, NYHA II-III, LV-EF <=35%
• Appropriate shocks in ≈1/3 of patients • Medical therapy for heart failure
– Anticoagulation when LV-EF <=40%
• CRT: OMT, QRS >=130ms, LV-EF <=35%
Genetics & Non-compaction
• Beta-myosin heavy chain
• Alpha cardiac actin
• Cardiac myosin binding proteinC
• Cardiac troponinT
• Cardiac troponinI
• Alpha tropomyosin
• Lamin A/C
Klaassen S et al. Circulation 2008;117:2893
Summary
• Phenotype: • -Thickened myocardial wall • -Thin compacted myocardial layer • -Thick non-compacted myocardial layer • -Recesses perfused from ventricular lumen • -Apical, mid-lateral, mid-inferior segments • -Isolated or combined(congenital heart defects, genetic syndromes) • Genotype: • -Different mutations(sarcomere proteins, others) • -Familial occurrence(autosomal dominant, X-linked) • Clinic: • -Wide clinical spectrum • -Heart failure, ventricular arrhythmias, thrombo-embolic events • Treatment: • -Similar to dilated cardiomyopathy • -Exception: anticoagulation
Prevalence, frequent overestimation/underestimation. Definitive echocardiographic and CMR criteria for diagnosis. Prevalence of RV involvement and diagnostic criteria. Whether prognosis can be improved by early diagnosis-treatment. .
Unresolved issues
Thank you