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cardio.pediatric-cardiomyopathy.chronic.v1PRODUCTION
cardio.pediatric-cardiomyopathy.chronic.v1

Pediatric cardiomyopathy (chronic, sub-population)

cardiologychronicpediatricadult
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Care setting:

Encounter flow

12/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

CM type + age + etiology category — pediatric distribution differs sharply from adult

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pediatric CM type + etiology category framed

Patient inputs (10)

Genetic/syndromic/metabolic/neuromuscular/mitochondrial — etiology-specific therapy

Infant/child/adolescent — etiology distribution + weight-based dosing + transition

DCM/HCM/RCM/LVNC/ARVC — phenotype-specific management

Systolic/diastolic function — HF severity + transplant listing

Pediatric functional class (Ross/NYHA) + growth — transplant timing

All pediatric cardiac drug dosing is weight-based

Weight + renal drug dosing

Pompe/FAO/mitochondrial/dystrophinopathy/RASopathy workup

Duchenne/Becker — early ACEi/MRA prophylaxis ± steroids

Pediatric-specific SCD risk (HCM model differs from adult)

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (9)

9 need judgement
  • informationallife_threateningpediatric_rcm_branch
    Pediatric restrictive cardiomyopathy — poor prognosis; early transplant listing (often before severe symptoms) — 2019 AHA Pediatric CM
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningrefractory_pediatric_hf_branch
    Refractory pediatric DCM HF — transplant + Berlin Heart / pediatric VAD bridge (leading pediatric transplant indication) — 2019 AHA Pediatric CM
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveretreatable_metabolic_etiology
    Treatable metabolic etiology (Pompe — ERT; selected FAO/mitochondrial) — disease-modifying therapy materially changes outcome; do not miss — 2019 AHA Pediatric CM
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereduchenne_cardiomyopathy_branch
    Duchenne/Becker muscular dystrophy — EARLY ACEi/MRA prophylaxis before overt cardiomyopathy + glucocorticoid (deflazacort/prednisone) standard of care — 2019 AHA Pediatric CM
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepediatric_hcm_scd_branch
    Pediatric HCM — pediatric-specific SCD risk model (NOT the adult HCM-RISK-SCD); EP referral for risk + ICD (size-aware) — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatesyndromic_rasopathy_branch
    Syndromic CM (Noonan/RASopathy, etc.) — multisystem + genetics; RASopathy HCM has distinct management/prognosis — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatetransition_of_care_branch
    Adolescent transition pediatric→adult CM/ACHD care — structured navigation; loss-to-follow-up is a major preventable risk — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatepediatric_anthracycline_survivor_branch
    Childhood-cancer anthracycline survivor — lifelong cardiomyopathy surveillance (childhood exposure = higher late risk) — ESC Cardio-Oncology
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatefamily_cascade_branch
    Familial pediatric CM — genetic testing + first-degree (and pediatric relative) cascade screening; pediatric-onset has higher genetic yield — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.

Workflow calculators

Run this disease's risk and dosing calculators inline.

TREATMENTrequiredDrives dose adjustment
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Recommended regimen

Pediatric CM — weight-based HF GDMT + etiology-specific + advanced (2019 AHA Pediatric CM; 2023 ESC Cardiomyopathy)
axis: pediatric_cm_weight_based_and_etiology_specificstep 1 - Step 1 — Weight-based HF GDMT (pediatric DCM/systolic dysfunction)
Selected step "Step 1 — Weight-based HF GDMT (pediatric DCM/systolic dysfunction)" — Pediatric DCM / systolic dysfunction
  • enalapril
    first line
    ACEi
    0.05–0.1 mg/kg/day (titrate) • PO • BID
    triggers: pediatric_DCM_systolic_dysfunction
    ACEi is the pediatric HF cornerstone (weight-based); enalapril/lisinopril (2019 AHA Pediatric CM)
    rxcui 203123
  • carvedilol
    first line
    beta_blocker
    0.05 mg/kg BID (titrate to ~0.5 mg/kg BID) • PO • BID
    triggers: pediatric_DCM_stable
    Beta-blocker in pediatric HF (pediatric carvedilol RCT neutral overall but used in practice with selected benefit) (2019 AHA Pediatric CM)
    rxcui 20352
  • spironolactone
    first line
    MRA
    1 mg/kg/day • PO • once–BID
    triggers: pediatric_HF, K<=5.0
    MRA in pediatric HF (2019 AHA Pediatric CM)
    rxcui 9997
  • furosemide
    first line
    loop_diuretic
    0.5–1 mg/kg/dose • PO/IV • once–BID
    triggers: pediatric_congestion
    Weight-based diuretic for pediatric congestion (2019 AHA Pediatric CM)
    rxcui 4603
  • digoxin
    add on
    cardiac_glycoside
    weight/age-based; level-guided • PO • once–BID
    triggers: pediatric_HF_adjunct
    Digoxin retains a role in pediatric HF (level-guided) (2019 AHA Pediatric CM)
    rxcui 3407

outpatient playbook — drug actions (3)

  1. 1. weight-based HF GDMT
    enalapril 0.05–0.1 mg/kg/day + carvedilol + spironolactone + furosemide ± digoxin • PO • per drug
    trigger: Pediatric DCM/systolic dysfunction (2019 AHA Pediatric CM)
    Pediatric HF backbone
  2. 2. etiology-specific (ERT / steroids+early-ACEi-MRA / idebenone)
    per disease protocol • IV/PO • per protocol
    trigger: Treatable etiology (Pompe/Duchenne/Friedreich) (2019 AHA Pediatric CM)
    Disease-modifying
  3. 3. pediatric ICD / transplant-bridge
    device/procedure • device/surgical • n/a
    trigger: High SCD risk / refractory HF (2023 ESC Cardiomyopathy)
    SCD prevention / advanced HF

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Pediatric HF / failure-to-thrive / feeding intolerance; Echo: pediatric DCM/HCM/RCM/LVNC/ARVC; Family history of CM/SCD or syndromic/metabolic features.

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Pediatric cardiomyopathy (chronic, sub-population)** (cardio.pediatric-cardiomyopathy.chronic.v1).
Phenotype framing: CM type + etiology category (genetic vs metabolic vs neuromuscular vs idiopathic vs acquired)
Scope: CM type + age + etiology category — pediatric distribution differs sharply from adult

No severity triggers fired against current inputs.

Plan

Regimen axis: **Pediatric CM — weight-based HF GDMT + etiology-specific + advanced (2019 AHA Pediatric CM; 2023 ESC Cardiomyopathy)** — step "Step 1 — Weight-based HF GDMT (pediatric DCM/systolic dysfunction)".
1. enalapril 0.05–0.1 mg/kg/day (titrate) PO BID (ACEi, first line) — ACEi is the pediatric HF cornerstone (weight-based); enalapril/lisinopril (2019 AHA Pediatric CM)
2. carvedilol 0.05 mg/kg BID (titrate to ~0.5 mg/kg BID) PO BID (beta_blocker, first line) — Beta-blocker in pediatric HF (pediatric carvedilol RCT neutral overall but used in practice with selected benefit) (2019 AHA Pediatric CM)
3. spironolactone 1 mg/kg/day PO once–BID (MRA, first line) — MRA in pediatric HF (2019 AHA Pediatric CM)
4. furosemide 0.5–1 mg/kg/dose PO/IV once–BID (loop_diuretic, first line) — Weight-based diuretic for pediatric congestion (2019 AHA Pediatric CM)
5. digoxin weight/age-based; level-guided PO once–BID (cardiac_glycoside, add on) — Digoxin retains a role in pediatric HF (level-guided) (2019 AHA Pediatric CM)

Setting playbook (outpatient) — Etiology-driven pediatric CM management: weight-based GDMT + disease-specific therapy + pediatric SCD risk + transplant timing + transition (2019 AHA Pediatric CM; 2023 ESC Cardiomyopathy)
6. weight-based HF GDMT enalapril 0.05–0.1 mg/kg/day + carvedilol + spironolactone + furosemide ± digoxin PO per drug — Pediatric DCM/systolic dysfunction (2019 AHA Pediatric CM) (Pediatric HF backbone)
7. etiology-specific (ERT / steroids+early-ACEi-MRA / idebenone) per disease protocol IV/PO per protocol — Treatable etiology (Pompe/Duchenne/Friedreich) (2019 AHA Pediatric CM) (Disease-modifying)
8. pediatric ICD / transplant-bridge device/procedure device/surgical n/a — High SCD risk / refractory HF (2023 ESC Cardiomyopathy) (SCD prevention / advanced HF)

Non-pharmacologic actions:
- Pediatric cardiomyopathy/HF/transplant centre — 2019 AHA Pediatric CM
- Genetics + first-degree family cascade screening — 2023 ESC Cardiomyopathy
- Structured transition to adult CM/ACHD care — 2023 ESC Cardiomyopathy

AVOID / contraindication checks:
- All pediatric cardiac drug dosing is weight based — 2019 AHA Pediatric CM
- Early ACEi MRA prophylaxis in Duchenne before overt cardiomyopathy — 2019 AHA Pediatric CM
- Pediatric HCM SCD risk model differs from adult — 2023 ESC Cardiomyopathy
- RCM pediatric poor prognosis early transplant listing — 2019 AHA Pediatric CM
- Structured transition to adult care prevents loss to follow up — 2023 ESC Cardiomyopathy

Monitoring

Regimen monitoring:
- growth and weight for dose adjustment — 2019 AHA Pediatric CM
- serial echo ventricular function — 2019 AHA Pediatric CM
- etiology specific markers ERT response CK lactate — 2019 AHA Pediatric CM
- Holter and pediatric SCD risk reassessment — 2023 ESC Cardiomyopathy
- family cascade screening — 2023 ESC Cardiomyopathy

Setting (outpatient) monitoring:
- Growth/weight-based dose adjustment; serial echo; etiology markers — 2019 AHA Pediatric CM

Follow-up plan: Structured transition to adult CM/ACHD care; lifelong family cascade
- Close-out criterion: transition + cascade plan documented

Monitoring phase: Growth, ventricular function, etiology-specific markers, arrhythmia surveillance

Disposition

Current setting: outpatient — Etiology-driven pediatric CM management: weight-based GDMT + disease-specific therapy + pediatric SCD risk + transplant timing + transition (2019 AHA Pediatric CM; 2023 ESC Cardiomyopathy)

Disposition criteria:
- Treatable etiology → disease-specific therapy + GDMT
- Refractory DCM/RCM → transplant pathway
- Adolescent → structured adult-care transition

Escalation triggers (move to higher acuity):
- Refractory pediatric HF / RCM → early transplant listing + Berlin Heart bridge — 2019 AHA Pediatric CM
- High pediatric SCD risk → pediatric EP/ICD — 2023 ESC Cardiomyopathy
- Metabolic crisis → pediatric ICU + metabolic team — 2019 AHA Pediatric CM

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Pediatric restrictive cardiomyopathy — poor prognosis; early transplant listing (often before severe symptoms) — 2019 AHA Pediatric CM
- [LIFE_THREATENING] Refractory pediatric DCM HF — transplant + Berlin Heart / pediatric VAD bridge (leading pediatric transplant indication) — 2019 AHA Pediatric CM
- [SEVERE] Treatable metabolic etiology (Pompe — ERT; selected FAO/mitochondrial) — disease-modifying therapy materially changes outcome; do not miss — 2019 AHA Pediatric CM

Citations

- 2019 AHA Pediatric Cardiomyopathy Scientific Statement + 2023 ESC Cardiomyopathy Guideline + 2022 AHA/ACC/HFSA HF Guideline (framework) [PMID:37622657](https://pubmed.ncbi.nlm.nih.gov/37622657/)
- Cited evidence (PMID 35379504) [PMID:35379504](https://pubmed.ncbi.nlm.nih.gov/35379504/)
- Cited evidence (PMID 37622666) [PMID:37622666](https://pubmed.ncbi.nlm.nih.gov/37622666/)
- Cited evidence (PMID 31535829) [PMID:31535829](https://pubmed.ncbi.nlm.nih.gov/31535829/)
- Cited evidence (PMID 25176015) [PMID:25176015](https://pubmed.ncbi.nlm.nih.gov/25176015/)

Last reconciled with current guidelines: 2026-05-16.
References
  • 2019 AHA Pediatric Cardiomyopathy Scientific Statement + 2023 ESC Cardiomyopathy Guideline + 2022 AHA/ACC/HFSA HF Guideline (framework) — PMID:37622657
  • Cited evidence (PMID 35379504) — PMID:35379504
  • Cited evidence (PMID 37622666) — PMID:37622666
  • Cited evidence (PMID 31535829) — PMID:31535829
  • Cited evidence (PMID 25176015) — PMID:25176015