sudden onset cardiomyopathies in children
TRANSCRIPT
Sudden Onset Cardiomyopathies
in Children
Annette Schure, MD, DEAA, FAAP
Boston Children’s Hospital
Harvard Medical School
Sudden Onset Cardiomyopathies in ChildrenAnnette 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
Labs: 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”
Cardiomyopathy
Dilated ArrhythmogenicRV
Hypertrophic Restrictive Unclassified
- Idiopathic
- Inflammatory
- Infectious Williams GD, Hammer GB. Cardiomyopathy in childhood. Curr Opin Anaesthesiol. 2011;24(3):289-300
What about …..
It’s a primary
CM
Clearly associated with his secondary CM
Forget about the Phenotype
Primary
CM
Acquired
CM
Multisystem
Disease with CMHypertrophic CM (HCM)
Dilated CM (DCM)
Restrictive CM (RCM)
Left ventricle noncompaction CM
Arrhythmogenic Right Ventricle
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.
How common is CM in children ?
Annual incidence:
1.13 per 100 000 in children < 18y
Distribution:
DCM: 50% HCM: 42% RCM 2.5%
Prognosis: relatively poor
40% death or heart transplant within 2y
=> But 8.4 per 100 000 in infants
Category
Phenotype
Pathophysiology
Echo findings
Anesthetic
ConsiderationsDilated CM 4 chamber dilation and poor EF
Predominantly LV dilation
Mitral or tricuspid insufficiency
Neuromuscular co-morbidities?
Maintain adequate preload, normal HR, avoid
increases in SVR.
Invasive monitoring
Inotropes may be required
Hypertrophic CM Dynamic LVOTO
Ventricular cavity obliteration
Poor diastolic relaxation
Possibly normal EF, but poor function
Increased LV mass (g/m2)
Maintain relatively slow HR
Prevent hypovolemia
Aim for normal to increased SVR
Phenylephrine bolus if ST changes on ECG
Avoid Propofol as sole agent
Associated metabolic, genetic diseases state?
Restrictive CM Near-normal ventricles
Dilated atria and pulmonary veins
Poor diastolic relaxation
Intra-ventricular thrombi?
Maintain preload, contractility
Avoid bradycardia
Avoid any further increases in PVR
Arrhythmogenic
RV dysplasia
Fibro-fatty infiltration of RV
Arrhythmias/abnormal ECG
RV failure
Avoid exogenous catecholamine/drugs with
sympathomimetic effects
External defibrillator pads
LV non-
compaction
Deep myocardial trabeculations
Varying degrees of hypokinesis
Possible intraventricular thrombi
Associated neuromuscular disorders?
Ing RJ, Ames WA, Chambers NA. Paediatric cardiomyopathy and anaesthesia. Br J Anaesth. 2012;108(1):4-12.
Sudden Onset Cardiomyopathies • Most often Dilated Cardiomyopathy
• Few unspecific symptoms
=> heart failure, arrhythmias, shock
• Etiology: – 66% idiopathic or “unknown” => genetic testing
– 19% Myocarditis
– 9% metabolic or neuromuscular
• Prognosis: – 5 year mortality 35-70%
Myocarditis: 50-80% full recovery within 2 yearsbut worse survival rates after transplant
• Viral Infections most common causeEnterovirus, coxsackie, adenovirus, parvovirus B19, herpes virus type 6, CMV, EBV, parcheovirus, parainfluenca virus
• Outcomes:
18.9% ECMO 4.3% VAD 4.1% OHT 7.2% death
Pat on ECMO or VAD:
56.9% full recovery 16.3% OHT 24% death
Circ Cardiovasc Qual Outcomes. 2012;5(5):622-7.
Age Distribution for Acute Myocarditis
Ghelani SJ, Spaeder MC, Pastor W, Spurney CF, Klugman D. Demographics, trends, and outcomes in pediatric acute myocarditis in the United States, 2006 to 2011. Circ Cardiovasc Qual Outcomes. 2012;5(5):622-7.
Pathophysiology Inflammatory disease with 3 phases:
1. Viral invasioninflammatory cell invasion => Myocardial necrosis and apoptosis
2. T-cell attack: Virus + infected myocytes1-2 weeks later
3. Healing => fibrosis
Blauwet L, et al: Progress in Cardiovascular Disease 52 (2010) 274-288
Presentation
Mild flulike symptoms – heart failure – cardiac arrest
Non-specific signs:
• Neonates: Irritability, poor feeding, fever, diaphoresis, cyanosis, apnea
• Older children and Adolescents: GI symptoms, dyspnea, vomiting
Typical cardiac signs often absent:
Tachycardia 30%, abnormal lung auscultation 34%, hepatomegaly 50%
Arrhythmias 20% (VES, VT > CHB > SVT )
=> In 83% diagnosis missed by first medical providerSuspect Myocarditis if- h/o recent viral illness- unexplained dyspnea or fatigue- new onset arrhythmias or acute cardiac failure
Call from the PICU
Cardiac Cath and biopsy scheduled for tomorrow
They would like to add on a cardiac MRI.
Could this be done under one anesthesia?
Really ?Why do we need the MRI ?
Diagnostic Tests• ECG abnormalities: Tachycardia, ST changes, etc
• Chest XR: Cardiomegaly
• Laboratory: elevated AST, ESR, CPR, Troponin, BNP
• Viral test: Viral PCR, cultures, serology
– Positive blood PCR and positive IgM = new infection
– Serology and cardiac biopsy correlate in only 9% !
• Echo: regional wall motion abnormalities , EF ↓↓
• Endomyocardial biopsy: “the gold standard”
– Patchy infiltrates, only 35-50% positive
– higher complication rates: 9.1% in myocarditis group vs 1.9% overall Pophal et al, JACC, 1999
Kuehl U, et al. “Myocarditis in Children.” Heart Failure Clinic 6 (2010) 483-496
So what about the MRI?
Lake Louise Consensus Criteria: • Regional or global myocardial SI increase
in T2 weighted images
• Increased global myocardial early gadolinium enhancement
• Late gadolinium enhancement:At least 1 focal lesion with non-ischemic distribution
CMR Study Report: ✓ LV volume and function
✓ Presence or absence of markers for inflammatory activity and injury
✓ Conclusion
✓ Recommendation for follow up
> 4 wks after onset of symptoms => prognosis
Advantages of CMRI• Detection of subtle myocardial involvement
• Assessment and quantification of myocardial damage
• Acute inflammation versus later remodeling
• Guide for Endomyocardial Biopsy: Optimal locations
• Non-invasive for older children and adolescents
• Prognostic Value for pediatric patients?
– Poor outcome if: EF < 30%, peak BNP > 10 000 pg/ml and
late enhancement of CMRI
But: Anesthesia for infants and small children=> Careful Risk/Benefit analysis
Friedrich et al: J Am Coll Cardiol. 2009;53(17):1475-87.
Sachdeva S et al: Am J Cardiol. 2015;115(4):499-504.
Recent Trend
Ghelani SJ, Spaeder MC, Pastor W, Spurney CF, Klugman D. Circ Cardiovasc Qual Outcomes. 2012;5(5):622-7.
Pediatric Health Information System Database
42 tertiary care pediatric hospitalsApril 2006-March 2011
514 patients
Another call from the PICU!• They want to start Milrinone and
need a PICC line.
• The PICC team tried several times – and failed!
• Now the child is really upset
• IR is ready
• Could you help sedate the child?
You go up to the PICU
Park MK: How to read Pediatric ECGs. 4th ed., Mosby Elsevier, 2006
Arrhythmias and Myocarditis
• 50% of patients: acute and subacute => worse outcomes
• Ventricular arrhythmias most common
• ECG changes for early identification: low voltage, ST changes
Miyake CY et al.: Am J Cardiol. 2014;113(3):535-40
The PICU calls again ……
• Really “uncomfortable”
=> Transfer to specialized center
• Transport team is busy
• Could Anesthesia help ?
Transport Issues
• Specialty Critical Care Transport teams preferred
• Choice of transport: ground vs. air (helicopter – fixed wing)
• Intubation and sedation prior to transport ?? ECMO standby?
• Inotropic support
• Rate and rhythm control: transcutaneous pacer, defibrillator
Pediatric Emergency Care 2010; 26(7)
High Risk of Sudden Deterioration
Back to our 9 yo girl• Transferred to tertiary center
• Wide complex rhythm and hypotension, but conscious and responsive
• Started on BIPAP, ECMO stand-by organized
• Lines placed: A-line, PIVs, femoral CVL
• Sudden loss of consciousness, rhythm change
=> Defibrillation & CPR started
=> no ROSC after one round => ECMO
Treatment • Supportive therapy
– Inotropes, afterload reduction, diuretics, ventilation etc.
• Treatment of post-infectious inflammation
– IgG, Corticosteroids ??? Azathioprine ???
• Antiviral Therapy
– Interferon and Ganciclovir for viral persistence ???
• Antiarrhythmic therapy
– Temporary pacing, antiarrhythmics, cardioversion
• ECMO and VAD: Bridge to Recovery or Transplant
• Heart Transplant
Anesthesia
Specific Considerations
•Rapid disease progression
•Malignant Arrhythmias
•Cardiovascular collapse
=> Potential need for E-CPR extra-corporeal CPR (= ECMO)
Preoperative Evaluation
• Discussion with Cardiologist
• History & Current Status
• Maturity and anxiety level
• Current medications
• Current intravenous access
• Most recent Echo & ECG/Monitor tracing
Preparation • Type of procedure?
• Off –site location: Adequate resources and equipment?
• ECMO Candidate? Team discussion, access site, cannula size
• Equipment and medications to treat arrhythmias
• Medications and equipment for inotropic support
• Need for invasive monitoring?
• Additional monitoring? TEE, NIRS?
• Postoperative Recovery? PACU or ICU?
Management• Transport = critical period
Risk for sudden deterioration, arrhythmias, flow changes
• Anesthetic Agents: Any sedation = decrease in endogenous catecholamines
Prolonged circulation time
Etomidate and Ketamine most often used
– Ketamine: can be cardiodepressant (if catecholamine stores depleted)
– Propofol: dose dependent hypotension (vasodilation & cardiodepression)
– Volatile agents: myocardial depressants
– Opioids: decreased sympathetic tone and bradycardia
What happened to our girl?
• Cath lab later that dayEndomyocardial biopsies and atrial septostomy for LA decompression
• Portable Head CT: negative
• Decannulated on Day # 5
• Extubated on Day # 6
• Transferred to floor on Day # 12, EF 56%
• Discharged after 20 days. EF 59%
• Follow up 3 month later: back in school, nl EF
Take Home Message
• Recognition of unusual presentation
• Thorough preoperative evaluation
• Good communication with care team
• Adequate preparation of location & logistics
• Emergency Back-up plan – ECMO?
References 1.Ing RJ, Ames WA, Chambers NA. Paediatric cardiomyopathy and anaesthesia. Br J Anaesth. 2012;108(1):4-12.
2.Rosenthal DN, Hammer GB. Cardiomyopathy and heart failure in children: anesthetic implications. Paediatr Anaesth. 2011;21(5):577-84.
3.Williams GD, Hammer GB. Cardiomyopathy in childhood. Curr Opin Anaesthesiol. 2011;24(3):289-300.
4.Richardson P, McKenna W, Bristow M, Maisch B, Mautner B, O'Connell J, et al. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies. Circulation. 1996;93(5):841-2.
5.Maron BJ, Towbin JA, Thiene G, Antzelevitch C, Corrado D, Arnett D, et al. Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation. 2006;113(14):1807-16.
6.Elliott P, Andersson B, Arbustini E, Bilinska Z, Cecchi F, Charron P, et al. Classification of the cardiomyopathies: a position statement from the European Society Of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J. 2008;29(2):270-6.
7.Konta L, Franklin RC, Kaski JP. Nomenclature and systems of classification for cardiomyopathy in children. Cardiol Young. 2015;25 Suppl 2:31-42.
8.Ghelani SJ, Spaeder MC, Pastor W, Spurney CF, Klugman D. Demographics, trends, and outcomes in pediatric acute myocarditis in the United States, 2006 to 2011. Circ CardiovascQual Outcomes. 2012;5(5):622-7.
9.Blauwet LA, Cooper LT. Myocarditis. Prog Cardiovasc Dis. 2010;52(4):274-88.
10.Kuhl U, Schultheiss HP. Myocarditis in children. Heart Fail Clin. 2010;6(4):483-96, viii-ix.
11.Durani Y, Egan M, Baffa J, Selbst SM, Nager AL. Pediatric myocarditis: presenting clinical characteristics. Am J Emerg Med. 2009;27(8):942-7.
12.Miyake CY, Teele SA, Chen L, Motonaga KS, Dubin AM, Balasubramanian S, et al. In-hospital arrhythmia development and outcomes in pediatric patients with acute myocarditis. Am J Cardiol. 2014;113(3):535-40.
13.Mahfoud F, Gartner B, Kindermann M, Ukena C, Gadomski K, Klingel K, et al. Virus serology in patients with suspected myocarditis: utility or futility? Eur Heart J. 2011;32(7):897-903.
14.den Boer SL, Meijer RP, van Iperen GG, Ten Harkel AD, du Marchie Sarvaas GJ, Straver B, et al. Evaluation of the diagnostic work-up in children with myocarditis and idiopathic dilated cardiomyopathy. Pediatr Cardiol. 2015;36(2):409-16.
15.Aretz HT. Myocarditis: the Dallas criteria. Hum Pathol. 1987;18(6):619-24.
16.Pophal SG, Sigfusson G, Booth KL, Bacanu SA, Webber SA, Ettedgui JA, et al. Complications of endomyocardial biopsy in children. J Am Coll Cardiol. 1999;34(7):2105-10.
17.Friedrich MG, Sechtem U, Schulz-Menger J, Holmvang G, Alakija P, Cooper LT, et al. Cardiovascular magnetic resonance in myocarditis: A JACC White Paper. J Am Coll Cardiol. 2009;53(17):1475-87.
18.Sachdeva S, Song X, Dham N, Heath DM, DeBiasi RL. Analysis of clinical parameters and cardiac magnetic resonance imaging as predictors of outcome in pediatric myocarditis. Am J Cardiol. 2015;115(4):499-504.
19.Hsu DT, Canter CE. Dilated cardiomyopathy and heart failure in children. Heart Fail Clin. 2010;6(4):415-32, vii.
20.Levine MC, Klugman D, Teach SJ. Update on myocarditis in children. Curr Opin Pediatr. 2010;22(3):278-83.
21.Robinson J HL, Vandermeer B, KlassenTP. . Intravenous immunoglobulin for presumed viralmyocarditis in children
and adults. Cochrane Database of Systematic Reviews 2015. 2015(5).
22.Chen HS W, Wu SN, Liu JP. Corticosteroids for viral myocarditis. Cochrane Database of Systematic Reviews 2013. 2013(10).
23.Zafar F, Castleberry C, Khan MS, Mehta V, Bryant R, 3rd, Lorts A, et al. Pediatric heart transplant waiting list mortality in the era of ventricular assist devices. J Heart Lung Transplant. 2015;34(1):82-8.
24.Thrush PT, Hoffman TM. Pediatric heart transplantation-indications and outcomes in the current era. J Thorac Dis. 2014;6(8):1080-96.
25.Dipchand AI, Edwards LB, Kucheryavaya AY, Benden C, Dobbels F, Levvey BJ, et al. The registry of the International Society for Heart and Lung Transplantation: seventeenth official pediatric heart transplantation report--2014; focus theme: retransplantation. J Heart Lung Transplant. 2014;33(10):985-95.