Sudden Onset Cardiomyopathies in Children

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Sudden Onset Cardiomyopathies in Children

Transcript Of Sudden Onset Cardiomyopathies in Children

Sudden Onset Cardiomyopathies
in Children
Annette Schure, MD, DEAA, FAAP Boston Children’s Hospital Harvard Medical School

Sudden Onset Cardiomyopathies in Children
Annette Schure, MD

No disclosures

You are on call ….
Call from GI:
9yo girl with epigastric pain and N/V x 2 weeks Add on EGD for tomorrow
Previously healthy Returned from summer camp Fever, malaise and GI symptoms Work up by Primary Care Doc:
=> mildly elevated LFT’s

You go and see her….
Vital signs:
Temp 37.6 BP 80/40 HR 146 Sat 100% on RA
WBC 11 Hct 41 Plts 266 AST 910 ALT 1595 Bili 3.3 Crea 1.1
Physical exam:
• Tired appearing, NAD • Lungs clear • Abdomen soft and non-distended, mild epigastric tenderness • Tachycardic, gallop, capillary refill  2 s

You ask for a Cardiology Consult
Severe biventricular dysfunction: EF 17%

Cardiomyopathy in Children
WHO Definition of Cardiomyopathy (CM):
“Disease of the myocardium associated with cardiac dysfunction”

- Idiopathic - Inflammatory - Infectious



Arrhythmogenic RV


Williams GD, Hammer GB. Cardiomyopathy in childhood. Curr Opin Anaesthesiol. 2011;24(3):289-300

What about …..
It’s a primary
Clearly associated with his secondary CM

Forget about the Phenotype

Primary CM
Hypertrophic CM (HCM) Dilated CM (DCM) Restrictive CM (RCM) Left ventricle noncompaction CM Arrhythmogenic Right Ventricle

Acquired CM

Multisystem Disease with CM

Acute myocarditis (DCM) Tachycardia-induced (DCM) Pacing-induced (DCM) Antineoplastic drugs (DCM) Nutritional (DCM) Takosubo CM Infant of diabetic mother (HCM)

Muscular dystrophies (DCM) (Becker, Duchenne, Barth Syndrome., Emery Dreifuss, Limb girdle, fascioscapularhumeral) Mitochondrial and respiratory chain disorders(DCM, HCM) Fatty acid oxidation disorders (DCM, HCM) Glycogen storage disorders (HCM) (Pompe, Danon) Mucopolysaccharidosis Type 1,2,5 (HCM) Glycolipid lipidosis (Fabry disease) (HCM) Hereditary hematochromatosis (DCM) Carvajal Syndrome (DCM) Noonan’s Syndrome (HCM) Beckwith-Wiedemann Syndrome (HCM) Cardio-facial-cutaneous Syndrome (HCM) Costello Syndrome, LEOPARD syndrome (HCM)

Maron BJ, Towbin JA, Thiene G, Antzelevitch C, Corrado D, Arnett D, et al. Circulation. 2006;113(14):1807-16. Elliott P, Andersson B, Arbustini E, Bilinska Z, Cecchi F, Charron P, et al. Eur Heart J. 2008;29(2):270-6.
DcmHcmOnset CardiomyopathiesChildren Annette SchureChildren