| Stage | Definition | Examples | Therapy |
At risk of HF
(Stage A) | Patients with increased risk of developing HF but with normal cardiac function and chamber size | - Exposure to cardiotoxic agents (eg, anthracycline exposure)
- Family history of heritable cardiomyopathy
- Repaired or palliated CHD with normal ventricular function
| None |
Presymptomatic HF
(Stage B) | Patients with evidence of ventricular dysfunction on echocardiography (or other cardiac imaging) with no past or present HF symptoms | - History of anthracycline exposure with reduced LVEF
- Repaired or palliated CHD with reduced ventricular function
| - ACE inhibitor
- An ARB can be used if the patient does not tolerate ACE inhibitor therapy
|
Symptomatic HF
(Stage C) | Patients with evidence of ventricular dysfunction on echocardiography (or other cardiac imaging) who have symptoms, including past or present HF symptoms | - Symptomatic cardiomyopathy
- Repaired or palliated CHD with symptomatic ventricular dysfunction
| Our suggested stepwise approach: - Start with an ACE inhibitor plus an MRA (an ARB can be used if the patient does not tolerate ACE inhibitor therapy).
- Provide oral diuretic therapy as needed to address fluid overload.
- After a few weeks of stability, a beta blocker is added in patients with persistent ventricular dilation and dysfunction.
- Low-dose digoxin can be added for additional symptom relief, if needed.
- Once the patient is tolerating full the full HF treatment regimen, they should be reassessed to see if additional or alternative therapy is warranted.
- If heart function is not improving and the patient's blood pressure is in good range, consider transitioning from ACE inhibitor to ARNI.
- A reasonable alternative for patients requiring additional therapy is to add an SGLT2 inhibitor.
- For patients with severe limitation of activity, significant growth failure, intractable arrhythmias, or restrictive cardiomyopathy, early referral should be made to a pediatric transplant center.
|
Advanced HF
(Stage D) | Patients with end-stage HF requiring specialized interventions | - End-stage state of any of the above examples
- There is marked impairment and symptoms at rest despite maximal medical therapy
| - Pharmacologic therapy consists of intravenous diuretics and/or inotropes.
- Other interventions may include positive pressure ventilation, cardiac resynchronization therapy, mechanical circulatory support, and heart transplantation.
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