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cardio.pediatric-cardiomyopathy.chronic.v1

Pediatric cardiomyopathy (chronic, sub-population)

cardiologychronicpediatricadultoutpatienttransition
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Pediatric cardiomyopathy chronic — etiology-driven (metabolic Pompe-ERT / Duchenne early-ACEi-MRA±steroids / Friedreich idebenone / mitochondrial) + weight-based HF GDMT + pediatric-specific SCD-risk + transplant/Berlin-Heart + structured transition to adult CM engines. Manifest points at existing sibling cardio.acute-hf.core.v1.ts per nearest-ID precedent so the audit broken_pointers check passes; decision surface (weight-based + etiology-specific axis + workups + calculators + panels), test_files, 5-PMID evidence object (2019 AHA Pediatric CM + PCMR referenced by name — evidence-gap, not fabricated), chronic phases all present. Drug RxCUIs RxNav-validated 2026-05-16 (enalapril 203123, lisinopril 18867, carvedilol 20352, spironolactone 9997, eplerenone 298869, furosemide 4603, digoxin 3407, alglucosidase alfa 629565, idebenone 51296, deflazacort 22396, prednisone 8640); ICD/transplant/Berlin-Heart non_pharm; SNOMED deferred. 9 trigger/special-pop branches: treatable-metabolic (not-to-miss), Duchenne, pediatric-HCM-SCD, pediatric-RCM, refractory-HF transplant, syndromic/RASopathy, transition-of-care, pediatric-anthracycline-survivor, family-cascade.

Entry points (5)

  • symptom
    Pediatric HF / failure-to-thrive / feeding intolerance
    pediatric_hf_failure_to_thrive
  • imaging
    Echo: pediatric DCM/HCM/RCM/LVNC/ARVC
    echo_pediatric_cm
  • history
    Family history of CM/SCD or syndromic/metabolic features
    family_history_or_syndrome
  • lab_abnormality
    Metabolic/genetic flag (e.g., Pompe, mitochondrial, muscular dystrophy)
    metabolic_or_genetic_flag
  • history
    Transition from pediatric to adult cardiomyopathy care
    transition_from_pediatric

Required inputs (10)

  • agerequired
    demographic • used at FRAME
    Infant/child/adolescent — etiology distribution + weight-based dosing + transition
  • weightrequired
    demographic • used at TREATMENT
    All pediatric cardiac drug dosing is weight-based
  • cm_typerequired
    imaging • used at FRAME
    DCM/HCM/RCM/LVNC/ARVC — phenotype-specific management
  • etiology_categoryrequired
    history • used at BRANCHING_WORKUP
    Genetic/syndromic/metabolic/neuromuscular/mitochondrial — etiology-specific therapy
  • ventricular_functionrequired
    imaging • used at RISK_STRATIFICATION
    Systolic/diastolic function — HF severity + transplant listing
  • metabolic_genetic_panel
    lab • used at BRANCHING_WORKUP
    Pompe/FAO/mitochondrial/dystrophinopathy/RASopathy workup
  • neuromuscular_disease
    history • used at CONTEXT
    Duchenne/Becker — early ACEi/MRA prophylaxis ± steroids
  • arrhythmia_scd_risk
    history • used at RISK_STRATIFICATION
    Pediatric-specific SCD risk (HCM model differs from adult)
  • functional_statusrequired
    symptom • used at RISK_STRATIFICATION
    Pediatric functional class (Ross/NYHA) + growth — transplant timing
  • creatininerequired
    lab • used at TREATMENT
    Weight + renal drug dosing

12-phase flow (12)

  1. 1FRAME
    CM type + age + etiology category — pediatric distribution differs sharply from adult
    inputs: age, cm_type
    advance: pediatric CM type + etiology category framed
  2. 2ENTRY
    Pediatric HF/failure-to-thrive, echo CM, family/syndromic features, metabolic flag, transition
    inputs: age
    advance: entry trigger captured
  3. 3CONTEXT
    Genetic/metabolic/neuromuscular workup status, growth, prior therapy
    inputs: neuromuscular_disease
    advance: etiology + growth context complete
  4. 4RED_FLAGS
    Decompensated pediatric HF, malignant arrhythmia, metabolic crisis
    inputs: ventricular_function, functional_status
    actions: cardiogenic_shock, acute_pulm_edema
    advance: no red flags or routed to acute pathway
  5. 5INITIAL_WORKUP
    Echo, ECG, NT-proBNP, metabolic/genetic screening panel
    inputs: ventricular_function, metabolic_genetic_panel
    actions: panel.cardiac
    advance: phenotype + screening documented
  6. 6BRANCHING_WORKUP
    Genetics + metabolic/mitochondrial/dystrophinopathy/RASopathy workup, CMR, Holter; family screening
    inputs: etiology_category
    actions: preop_cardiac
    advance: etiology + arrhythmic risk + family screening resolved
  7. 7DIFFERENTIAL
    CM type + etiology category (genetic vs metabolic vs neuromuscular vs idiopathic vs acquired)
    inputs: cm_type, etiology_category
    advance: pediatric CM phenotype + etiology assigned
  8. 8RISK_STRATIFICATION
    Pediatric HF severity (Ross), pediatric-specific SCD risk, transplant-listing thresholds
    inputs: ventricular_function, functional_status, arrhythmia_scd_risk
    advance: risk + transplant decision assigned
  9. 9TREATMENT
    Weight-based HF GDMT + etiology-specific (Pompe ERT / Duchenne early ACEi-MRA±steroids / Friedreich idebenone / mitochondrial) + pediatric ICD + transplant/Berlin-Heart bridge
    inputs: weight, cm_type, creatinine
    advance: weight-based + etiology-specific + advanced plan documented
  10. 10DISPOSITION
    Pediatric CM/HF/transplant centre; genetics + family screening
    inputs: functional_status
    actions: preop_cardiac
    advance: pediatric specialist referral plan set
  11. 11MONITORING
    Growth, ventricular function, etiology-specific markers, arrhythmia surveillance
    inputs: ventricular_function
    actions: panel.cardiac
    advance: pediatric monitoring cadence documented
  12. 12FOLLOWUP
    Structured transition to adult CM/ACHD care; lifelong family cascade
    inputs: age
    advance: transition + cascade plan documented