Cardiomyopathy

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Basic investigation:

ECG, CXR, ECHO, FBE, clotting, UEC  (incl. Ca++, Mg++, Fe++, PO4), CRP, ESR, albumin, LFT, TFT, BNP, troponin I, troponin T, lactate, ABG, VBG.


Extended investigation:

  • Cardiac MRI.
  • 24 hr Holter monitor.
  • Blood: amino acids, carnitine, acyl-carnitine, ammonia, Cu, caeruloplasmin, transferrin ioforms, pyruvate, selenium, vitamins.
  • Urine: amino acids, organic acids, pligosaccharide screen, MPS screen.
  • Autoimmune: ANA, ENA.
  • Genetic: FISH.
  • Endomyocardial biopsy.

Investigation for myocarditis:

  • Blood & Urine for viral cultures (echo, adeno, parvo, coxsackie, CMV, parainfluenza, influenza, HIV, hepatitis screen) and for bacterial (incl. atypical)/fungal/rickettsial/protozoal and helminithic culture.
  • NPA.
  • ETA.

Identifying the high risk patient:

LVEF < 20% or LVEDD >70 mm or increased C/T ratio >0.7 or MR >3 or complex ventricular arrhythmia (NSVT).

All high risk patients require PICU admission !


Acute management guideline for DCM

Is there low perfusion ? Is there congestion ?
NO YES
NO Ward ManagementACEiβ – Blockeroral diuretics PICU admissioniv Frusemide infusionconsider NIV CPAPobserve for minimum of 48 hrwatch BNP & renal function
YES PICU admissionNIV CPAPiv Adrenaline
(max. 0.05 ≈≥g/kg/min)
observe closelyfor need for ECLS
PICU admissioniv Frusemide infusionconsider NIV CPAPintroduce carefully Milrinoneconsider β agonistsobserve for 4 days

 

1. CPAP or BIPAP. Only intubate when ECLS is on stand-by

2. Fluid and water restriction to 25% maintenance

3. Commence IV frusemide infusion. Aim for euvolaemic state in DCM. Mild hypervolaemia may be needed in HCM. Watch renal function. Avoid hypovolaemia.

4. Commence spironolactone (1 mg/kg BD)

5. Commence milrinone if tolerated

6. Commence β-agonists as indicated. Dopamine, dobutamine or adrenaline up to 0.05 mcg/kg/min

7. Consider levosimendan

8. Consider biventricular pacing

9. Consider antiarrhythmic therapy. Consult cardiology. Do not use β-blocker when on β -agonists. β – Blocker might be useful in HCM

10. Consider anticoagulation therapy

11. Ensure appropriate nutrition. Supplemental therapy with carnitine, co-enzyme Q10, vitamins as indicated.

11. Early use of ECLS if patient is deteriorating or not improving (see ECMO)

12. Specific guided therapy in Pompe disease: myozyme


All Marc’s PICU cardiology FOAM can be found on PICU Doctor and can be downloaded as a handy app for free on iPhone or AndroidA list of contributors can be seen here.

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