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 Android. A list of contributors can be seen here.
