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CARDIOMYOPATHY
ANDMYOCARDITIS
Guided by : Dr Sandeep agarwal
Presented by : Dr Abhay Pota
www.dnbpediatrics.com
Table of Contents
[A] CARDIOMYOPATHY
1.HOCM
2.DCM
3.RCM
4.LVNC
5.ARVC
6.EFE
[B] MYOCARDITIS
www.dnbpediatrics.com
[A] CARDIOMYOPATHY
DCM
i) NMD – muscular dystrophies (duchene, becker, emery dreifuss, limb girdle etc)
- myotonic dystrop...
(2) HCM
i) IEM – mitochondrial disorders (freidrich ataxia), storage disorders
( esp. pompe, MPS, fabry, sphingolipidosis,...
(3) RCM
i) NMD- myofibrillar myopathies
ii) metabolic – storage disorders
iii) genetic mutations in cardiomyocyte structur...
iv) Arrythrogenic right ventricular cardiomyopathy
a) genetic mutation in cardiomyocyte structure
v) LV non compaction
b) ...
[B] secondary or acquired myocardial disease
(1) MYOCARDITIS
i) viral
parvo bilateral, adeno, coxsackie A & B, echo, rubel...
(2) systemic inflammatory disease
SLE, scleroderma, churg-strauss RA, RF,
sarcoidosis, dermatomyositis, loffles
(3) Nutrit...
(5) CAD – kawasaki, ALCAPA, familial hypercholesterolemia
(6) Hemato-oncology – anemia, leukemia, SCD
(7) endocrine – neur...
HOCM
It is a genetic disorder
Most common are mutations in genes encoding Cardiac B myosin
heavy chain (MYH7)Myosin bindin...
PATHOGENESIS
Characterized by increased LV thickness in the absence of structural heart disease
or hypertension
Inter-vent...
a large volume of stroke volume (80%) is ejected during the early part of systole
when there is little or no obstruction, ...
Clinical features
H/O : many patients asymptomatic
-50% cases present with heart murmur or detected
during family screenin...
P/E
– over active pre-cordial impulse with a heave
abnormal PP
- Systolic ejection murmur in the aortic region
not associa...
ECG
– LVH, ST-T changes, abnormally deep Q waves
WPW syndrome in Danon and Pompe diasease
X-ray
- mild LVH with globular h...
ECHO
Z Score of 2 or more relative to BSA is diagnostic
LV wall thickness Doppler helps in defining,
localising, quantifyi...
MANAGEMENT
1) sternous activity prohibited and rest
2) B blockers like propranolol or atenolol or CCBs
like verapamil, use...
4) patients with documented ventricular
arrythmias, strong family history or patients
with syncope – ICD
5) dual chamber p...
PROGNOSIS
Children <1 year, with IEM or with some syndromes have poorer prognosis
Risk of sudden death is increased in pat...
DCM
Most common form of cardiomyopathy
Most common cause of DCM is idiopathic
Among familial causes AD is most common
www....
PATHOLOGY AND PATHOPHYSIOLOGY
Weakening of systolic contraction
Dilatation of all 4 chambers
Dilatation of atria is in pro...
CLINICAL FEATURES
history of – fatigue, weakness, dyspnea, orthopnea
P/E – s/o CHF (increased heart rate, crackles,
weak P...
ECG
sinus tachycardia,
LVH & ST-T changes most common LAH,
RAH
Atrial or ventricular arrythmias
AV conduction disturbances...
x ray & echo
Cardiomegaly with or with out pulmonary edema
Echo – most important tool in diagnosis
pulmonary hypertension,...
Additional testing
CPK
KFT
LFT
Troponin
Lactate
plasma amino acids
urine organic acidsacylcarnitiine profile
www.dnbpediat...
MANAGEMENT
[1] medical treatment
Aimed at treating underlying heart failure
Diuretics, digoxin, ACEI, ARB
Bed rest and res...
[2] B adrenergic blockade with carvedilol or metoprolol
Pediatric data not available
[3] ECMO, ventricular assist devices,...
PROGNOSIS
1 and 5 year rates of death or need for transplantation in DCM
patients is 31% and 46%
Independent risk factors ...
RCM
<5 % of cases Incidence increases with age,
M<F Most common etiology is idiopathic
www.dnbpediatrics.com
PATHOGENESIS
Normal ventricular chamber diameter Normal ventricular wall
thickness Preserved systolic function Impaired ve...
Clinical features
H/O : exercise intolerance, weakness, dyspnea, chest pain
P/E : jugular venous distention, gallop rhythm...
ECHO : normal LV systolic function
diastolic dysfunction
bilateral AH with normal LV dimension
Cardiac catheterisation :
P...
MANAGEMENT
1)Treatment of CCF
2) CCB to increase diastolic compliance
3) anti-coagulants & anti-platelets to prevent throm...
www.dnbpediatrics.com
SUMMARY OF CLINICAL CHARACTERISTICS OF CARDIOMYOPATHY
CLINICAL
FEATURES
HYPERTROPHIC DILATED RESTRICTIVE
CAUSE Inherited (...
CLINICAL FEATURES HYPERTROPHIC DILATED RESTRICTIVE
Echo (morphology) Thickened LV (and
occasionally RV) wall
-Small or nor...
4. LVNC
Left ventricular non compaction
affects all ages
Trabeculated or spongy appearing LV,
commonly a/w LVH or LV dilat...
TREATMENT
anticoagulation, anti-arrythmic treatment if needed &
treatment of HF if present
Cardiac transplantation in pati...
5. ARVC
-Arrythmogenic right ventricular cardiomyopathy
-AD from most common
-AR forms a/w skin manifestations
-Characteri...
[6] EFE
endocardial fibroelastosis
Incidence markedly decreased due to abolition of mumps virus inf by immunisation
Charac...
MYOCARDITIS
www.dnbpediatrics.com
Definition and Etiology
Process characterized by inflammatory infiltrate of the
myocardium with necrosis and/or degenerati...
Etiology
www.dnbpediatrics.com
www.dnbpediatrics.com
Time Course of Viral Myocarditis
www.dnbpediatrics.com
Incidence
Often not recognized
Estimated annual incidence of 1/100.000
4 – 5% in young accident victims
12% in adolescents...
Clinical features
H/O : URTI in older children or may have a sudden onset with
anorexia , vomiting, lethargy.
In new borns...
Laboratory Tests
Biomarkers (troponins, creatine kinase MB) occasionally elevated in childhood
(sensitivity 71%, specifici...
ECG
Abnormalities present in 93-100%, but low sensitivity
Sinus tachycardia
Nonspecific T-wave and ST-segment changes
ST-s...
ECG
www.dnbpediatrics.com
Chest X-ray
www.dnbpediatrics.com
Echocardiography
www.dnbpediatrics.com
MRI
www.dnbpediatrics.com
Endomyocardial Biopsy
www.dnbpediatrics.com
Endomyocardial Biopsy
Is a Gold standard for diagnosis
Invasive, potential complications: pneumothorax, hemothorax, arrhyt...
Histology/Dallas Criteria
Acute myocarditis: lymphocytic infiltrate with myocyte necrosis
Borderline myocarditis: inflamma...
Differential Diagnosis
Any disease with impairment of LV function
DCMA
LCAPA
Chronic tachycardia
Arteriovenous malformatio...
Therapy I
Mainly supportive, no trials for specific heart failure therapy in biopsy-proven
myocarditis in adults and child...
Therapy II
In proven viremia: immunoglobulins 2 g/kg for 48 hours (Robinson JL 2005)
No immunosuppresive therapy – risk of...
Acute Congestive Heart Failure/Suspected Acute Myocarditis
www.dnbpediatrics.com
REFERENCES
TEXTBOOK OF PEDIATRIC CARDIOMYOPATHY – S. PARKS 6th edition
NELSON – 19th editionE-medicine.medscape.com
www.dn...
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  1. 1. CARDIOMYOPATHY ANDMYOCARDITIS Guided by : Dr Sandeep agarwal Presented by : Dr Abhay Pota www.dnbpediatrics.com
  2. 2. Table of Contents [A] CARDIOMYOPATHY 1.HOCM 2.DCM 3.RCM 4.LVNC 5.ARVC 6.EFE [B] MYOCARDITIS www.dnbpediatrics.com
  3. 3. [A] CARDIOMYOPATHY DCM i) NMD – muscular dystrophies (duchene, becker, emery dreifuss, limb girdle etc) - myotonic dystrophy - myofibrillar myopathy ii) IEM – fatty acid oxidation disorders - carnitine abnormalities (CPT I, CPT II) - mitochondrial disorders (kearns-sayre) - organic acidemias (propionic acidemia) iii) genetic mutation in cardiomyocyte structure iv) genetic syndromes – alstrom syndrome, barth syndrome v) ischemia – more common in adults vi) chronic tachyarrythmias www.dnbpediatrics.com
  4. 4. (2) HCM i) IEM – mitochondrial disorders (freidrich ataxia), storage disorders ( esp. pompe, MPS, fabry, sphingolipidosis, hemochromatosis) ii) genetic mutation in cardiomyocyte structure iii) genetic syndromes- noonan, costello, cardiofaciocutaneous, beckwith- wiedemann iv) infant of a diabetic mother www.dnbpediatrics.com
  5. 5. (3) RCM i) NMD- myofibrillar myopathies ii) metabolic – storage disorders iii) genetic mutations in cardiomyocyte structure www.dnbpediatrics.com
  6. 6. iv) Arrythrogenic right ventricular cardiomyopathy a) genetic mutation in cardiomyocyte structure v) LV non compaction b) X-linked, AR, AD, mitochondrial, sporadic www.dnbpediatrics.com
  7. 7. [B] secondary or acquired myocardial disease (1) MYOCARDITIS i) viral parvo bilateral, adeno, coxsackie A & B, echo, rubella, varicella, influenza, mumps EBV, cytomegalovirus, mumps, measles, polio, Hepatitis C, HIV, ii) rickettsial – psittacosis, RMSF, typhus, coxiella burnetti iii) bacterial – diptheria, mycoplasma, meningococci, leptospira, lyme, typhoid, TB, streptococci iv) parasites – chagas, toxoplasma, cysticercosis, schistosomia v) fungal – actino, coccidiomyco, histoplasmo www.dnbpediatrics.com
  8. 8. (2) systemic inflammatory disease SLE, scleroderma, churg-strauss RA, RF, sarcoidosis, dermatomyositis, loffles (3) Nutritional deficiency Beri beri, kwashiorkor, keshan (4) drugs doxorubicin, chloroquine, cyclophosphemide, sulphonamides, alcohol, irradiation, herbal remedy www.dnbpediatrics.com
  9. 9. (5) CAD – kawasaki, ALCAPA, familial hypercholesterolemia (6) Hemato-oncology – anemia, leukemia, SCD (7) endocrine – neuroendocrine – hyperthyroidism, carcinoid chromocytoma www.dnbpediatrics.com
  10. 10. HOCM It is a genetic disorder Most common are mutations in genes encoding Cardiac B myosin heavy chain (MYH7)Myosin binding protein C (MYBPC3) AD pattern of inheritance Other are PRKAG2 & LAMP2 α- galactosidase mutations www.dnbpediatrics.com
  11. 11. PATHOGENESIS Characterized by increased LV thickness in the absence of structural heart disease or hypertension Inter-ventricular septum is often disproportionately involved (IHSS) or (ASH). In some patients intra-cavitary gradiant develops during systole. This subaortic obstruction is caused by systolic anterior motion of mitral valve against hypertrophied septum Diastolic ventricular filling is impaired by abnormal stiffness of LV, which leads to LAH and pulmo venous congestion, producing congestive symptoms www.dnbpediatrics.com
  12. 12. a large volume of stroke volume (80%) is ejected during the early part of systole when there is little or no obstruction, producing a sharp upstroke in the arterial pulse- characteristic of HOCM Because the obstruction of LVOT results from SAM of mitral valve against hypertrophied septum, any influence that reduced LV systolic volume (+ve inotropic agents, lowering of SVR. Low blood volume) will increase obstruction and increase the murmur, while any influence that increases the LV systolic volume (-ve inotropic agents, Leg raising, blood transfusion) will decrease obstruction and decrease the murmur www.dnbpediatrics.com
  13. 13. Clinical features H/O : many patients asymptomatic -50% cases present with heart murmur or detected during family screening -palpitation , chest pain, easy fatiguability, -dyspnea, dizziness, syncope -sudden death www.dnbpediatrics.com
  14. 14. P/E – over active pre-cordial impulse with a heave abnormal PP - Systolic ejection murmur in the aortic region not associated with ejection click - A sharp upstroke on arterial pulse www.dnbpediatrics.com
  15. 15. ECG – LVH, ST-T changes, abnormally deep Q waves WPW syndrome in Danon and Pompe diasease X-ray - mild LVH with globular heart - pulmo vascularity normal www.dnbpediatrics.com
  16. 16. ECHO Z Score of 2 or more relative to BSA is diagnostic LV wall thickness Doppler helps in defining, localising, quantifying the degree of myocardial hypertrophy and also demonstrates degree of LVOT and mitral insufficiency Cardiac catheterization Metabolic testing Genetic testing www.dnbpediatrics.com
  17. 17. MANAGEMENT 1) sternous activity prohibited and rest 2) B blockers like propranolol or atenolol or CCBs like verapamil, useful in diminishing LVOT, modifying ventricular hypertrophy and improve ventricular filling. - Though, risk of sudden death not decreased. 3) antiarrythmic treatment in patient with atrial or ventricular arrythmias www.dnbpediatrics.com
  18. 18. 4) patients with documented ventricular arrythmias, strong family history or patients with syncope – ICD 5) dual chamber pacing, alcohol ablation, surgical septal myomectomy, MVR – limited success 6) first degree relatives should be screened every 3-5 years for patients under 12 years of age and then yearly throughout the teenage and young adulthood www.dnbpediatrics.com
  19. 19. PROGNOSIS Children <1 year, with IEM or with some syndromes have poorer prognosis Risk of sudden death is increased in patients with a History of cardiac arrest, History of Vtach, Exercise hypotension, syncope>3 cm wall thickness, F/H/O sudden death www.dnbpediatrics.com
  20. 20. DCM Most common form of cardiomyopathy Most common cause of DCM is idiopathic Among familial causes AD is most common www.dnbpediatrics.com
  21. 21. PATHOLOGY AND PATHOPHYSIOLOGY Weakening of systolic contraction Dilatation of all 4 chambers Dilatation of atria is in proportion to ventricles (unlike RCM) Intracavitary thrombus formation is common in apical part of ventricles Pulmo& systemic emboli On biopsy, myocyte hypertrophy and fibrosis www.dnbpediatrics.com
  22. 22. CLINICAL FEATURES history of – fatigue, weakness, dyspnea, orthopnea P/E – s/o CHF (increased heart rate, crackles, weak PP, distended neck veins, heapatomegaly) Gallop rhythm, murmurs of MR or TR www.dnbpediatrics.com
  23. 23. ECG sinus tachycardia, LVH & ST-T changes most common LAH, RAH Atrial or ventricular arrythmias AV conduction disturbances www.dnbpediatrics.com
  24. 24. x ray & echo Cardiomegaly with or with out pulmonary edema Echo – most important tool in diagnosis pulmonary hypertension, MR or other structural defects. www.dnbpediatrics.com
  25. 25. Additional testing CPK KFT LFT Troponin Lactate plasma amino acids urine organic acidsacylcarnitiine profile www.dnbpediatrics.com
  26. 26. MANAGEMENT [1] medical treatment Aimed at treating underlying heart failure Diuretics, digoxin, ACEI, ARB Bed rest and restriction of activity Inotropic support and mechanical ventilation www.dnbpediatrics.com
  27. 27. [2] B adrenergic blockade with carvedilol or metoprolol Pediatric data not available [3] ECMO, ventricular assist devices, cardiac transplantation [4] specific antiarrythmic therapy and anticoagulation www.dnbpediatrics.com
  28. 28. PROGNOSIS 1 and 5 year rates of death or need for transplantation in DCM patients is 31% and 46% Independent risk factors for death or need for transplant in DCM patients are Older age, CCF Lower left ventricular functional shortening zone www.dnbpediatrics.com
  29. 29. RCM <5 % of cases Incidence increases with age, M<F Most common etiology is idiopathic www.dnbpediatrics.com
  30. 30. PATHOGENESIS Normal ventricular chamber diameter Normal ventricular wall thickness Preserved systolic function Impaired ventricular filling Normal size of ventricles with dilated atrias www.dnbpediatrics.com
  31. 31. Clinical features H/O : exercise intolerance, weakness, dyspnea, chest pain P/E : jugular venous distention, gallop rhythm, systolic murmur of AV valve regurgitation ECG : bilateral AH, AV block X-ray : cardiomegaly, pulmonary venous congestion www.dnbpediatrics.com
  32. 32. ECHO : normal LV systolic function diastolic dysfunction bilateral AH with normal LV dimension Cardiac catheterisation : PApressure & RV & LV EDP Increased Endomyocardial biopsy www.dnbpediatrics.com
  33. 33. MANAGEMENT 1)Treatment of CCF 2) CCB to increase diastolic compliance 3) anti-coagulants & anti-platelets to prevent thrombus 4) pacemaker for CHB5) cardiac transplantation www.dnbpediatrics.com
  34. 34. www.dnbpediatrics.com
  35. 35. SUMMARY OF CLINICAL CHARACTERISTICS OF CARDIOMYOPATHY CLINICAL FEATURES HYPERTROPHIC DILATED RESTRICTIVE CAUSE Inherited (AD in about 50%) Sporadic(new mutation) Pluricausal (e.g.toxic metabolic, infectious, alcohol, doxorubicin Myocardial fibrosis, hypertrophy, or unfiltration (amyloid hemochromatosis) HEMO- DYNAMIC DYSFUNCTIO N Diastolic dysfunction (with normal systolic function) (abnormally stiff LV with impaired ventricular filling Systolic contractile dysfunction (decreased cardiac output, decreased stroke volume, increased LVEDP) Diastolic dysfunction (rigid ventricular walls impede ventricular filling www.dnbpediatrics.com
  36. 36. CLINICAL FEATURES HYPERTROPHIC DILATED RESTRICTIVE Echo (morphology) Thickened LV (and occasionally RV) wall -Small or normal LV chamber dimensionsSupernormal LV contractility HOCM and/or ASH Biventricular dilatation (increased LVDD and LVSD) -atrial enlargement in proportion to ventricular enlargement -Decreased LV contractility Apical thrombus(+/-) Biatrial enlargement -Normal LV and RV volume Normal LV systolic function until advanced stage Atrial thrombus(+/-) Doppler Reduced relaxation pattern Reduced relaxation pattern ‘reestrictive’ pattern Treatment B-adrenoreceptor blockers -calcium antogonists (digitalis/catechols and nitrates contraindicated) - (diuretics may worsen the symptoms) Vasodilator therapy -digitalis plus diuretics B-adrenoreceptor blockers(+/-) -Anticoagulants antiarrythmicsCardiac transplantation Diuretics -Anticoagulants(+/-)- corticosteroids(+/-)- permanent pacemaker for advanced heart blockCardiac transplantation www.dnbpediatrics.com
  37. 37. 4. LVNC Left ventricular non compaction affects all ages Trabeculated or spongy appearing LV, commonly a/w LVH or LV dilatation, and, at times systolic or diastolic dysfunction ECG – LVH, ST-T changes or arrythmias MRI – trabeculated LV myocardium, characteristically within apex of left ventricle Metabolic screen – especially in young patients TA2 gene mutation www.dnbpediatrics.com
  38. 38. TREATMENT anticoagulation, anti-arrythmic treatment if needed & treatment of HF if present Cardiac transplantation in patient refractory to medical treatment www.dnbpediatrics.com
  39. 39. 5. ARVC -Arrythmogenic right ventricular cardiomyopathy -AD from most common -AR forms a/w skin manifestations -Characterized by dilated RV with fibrofatty -infiltration of RV wall -Global and regional right and left ventricular dysfunction and -ventricular tachyarrythmias -Syncope or sudden death can be the presentation -Mx - antiarrythmics, defibrillation and Mx of HF, Cardiac transplantation www.dnbpediatrics.com
  40. 40. [6] EFE endocardial fibroelastosis Incidence markedly decreased due to abolition of mumps virus inf by immunisation Characterized by opaque, white, fibroelastic thickening on the endocardial surface of the ventricle, which leads to systolic or diastolic dysfunction Mx of HF & cardiac transplantation www.dnbpediatrics.com
  41. 41. MYOCARDITIS www.dnbpediatrics.com
  42. 42. Definition and Etiology Process characterized by inflammatory infiltrate of the myocardium with necrosis and/or degeneration of adjacent Myocytes, not typical of the ischemic damage associated with coronary artery disease Most cases from common viral infections and post-viral immune -mediated responseCoxsackievirus A and B, echovirus, poliovirus, PVB 19, HHV6Precursor of dilated cardiomyopathy www.dnbpediatrics.com
  43. 43. Etiology www.dnbpediatrics.com
  44. 44. www.dnbpediatrics.com
  45. 45. Time Course of Viral Myocarditis www.dnbpediatrics.com
  46. 46. Incidence Often not recognized Estimated annual incidence of 1/100.000 4 – 5% in young accident victims 12% in adolescents and young adults with sudden cardiac death www.dnbpediatrics.com
  47. 47. Clinical features H/O : URTI in older children or may have a sudden onset with anorexia , vomiting, lethargy. In new borns and infants signs of CHF like tachycardia, tachyapnea, gallop rhythm, and arrythmia may be present. A soft systolic heart murmur and supra ventricular or ventricular ectopic beats may be present www.dnbpediatrics.com
  48. 48. Laboratory Tests Biomarkers (troponins, creatine kinase MB) occasionally elevated in childhood (sensitivity 71%, specificity 86%) Normal nonspecific markers of inflammation (CRP, leucocytes) do not exclude acute myocardial inflammatory process Utility of virus serology in patients with suspected myocarditis unproven – costly and unreliable • Detection of viral genome in urine or feces www.dnbpediatrics.com
  49. 49. ECG Abnormalities present in 93-100%, but low sensitivity Sinus tachycardia Nonspecific T-wave and ST-segment changes ST-segment elevation AV conduction delays Atrial and ventricular arrhythmias www.dnbpediatrics.com
  50. 50. ECG www.dnbpediatrics.com
  51. 51. Chest X-ray www.dnbpediatrics.com
  52. 52. Echocardiography www.dnbpediatrics.com
  53. 53. MRI www.dnbpediatrics.com
  54. 54. Endomyocardial Biopsy www.dnbpediatrics.com
  55. 55. Endomyocardial Biopsy Is a Gold standard for diagnosis Invasive, potential complications: pneumothorax, hemothorax, arrhythmias, perforation, death Enables identification of lymphocytic invasion and detection of involved virus Poor sensitivity and specificity due to patchy myocardial inflammation Substantial interobserver variation www.dnbpediatrics.com
  56. 56. Histology/Dallas Criteria Acute myocarditis: lymphocytic infiltrate with myocyte necrosis Borderline myocarditis: inflammatory infiltrate without necrosis Chronic myocardits/DCM with inflammation: >14 inflammtory cells/mm2 in the myocardium www.dnbpediatrics.com
  57. 57. Differential Diagnosis Any disease with impairment of LV function DCMA LCAPA Chronic tachycardia Arteriovenous malformation www.dnbpediatrics.com
  58. 58. Therapy I Mainly supportive, no trials for specific heart failure therapy in biopsy-proven myocarditis in adults and children Monitoring of vital parameters Bed rest initially, no physical activities for 3-6 months Trat heart failure therapy with ACE inhibitors, AT1-antagonists ß-blockers, diuretics and aldosterone antagonists according to current guidelines Ventricular assist device <-> HTX Therapy of tachyarrhythmias in adults - Life vest and AED www.dnbpediatrics.com
  59. 59. Therapy II In proven viremia: immunoglobulins 2 g/kg for 48 hours (Robinson JL 2005) No immunosuppresive therapy – risk of enhanced virus replication and blockade of endogenous interferons Steroids only in biopsy-proven virus negative chroniC myocarditis (Frustacci A 2009) Type-1 interferon (IFN-α, IFN-ß) beneficial in animal and human pilot studies (Kühl U 2003, Schmaltz AA 1998) www.dnbpediatrics.com
  60. 60. Acute Congestive Heart Failure/Suspected Acute Myocarditis www.dnbpediatrics.com
  61. 61. REFERENCES TEXTBOOK OF PEDIATRIC CARDIOMYOPATHY – S. PARKS 6th edition NELSON – 19th editionE-medicine.medscape.com www.dnbpediatrics.com www.criticalpediatrics.org

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