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cardio.achd-vsd.chronic.v1PRODUCTION
cardio.achd-vsd.chronic.v1

Adult ventricular septal defect (chronic ACHD)

cardiologychronicadult
Hard-required inputs
0 / 7
Care setting:

Encounter flow

12/12 authored

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

Current phase

Frame

Detailed

Define VSD type/size + shunt + PVR + AR involvement

Inputs
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Actions
0
Advance rule
Set
Advance when

VSD type + physiology framed

Patient inputs (10)

Adult surveillance + intervention timing

Perimembranous/muscular/inlet/outlet + restrictive vs non-restrictive — modality + AR risk

LV dilatation supports hemodynamic significance

Qp:Qs ≥1.5 + LV overload = closure indication (if PVR acceptable)

Severe PAH/Eisenmenger = closure CONTRAINDICATED

Symptomatic significant VSD strengthens closure indication

Contrast for TEE/RHC; drug dosing

Progressive AR from cusp prolapse = surgery trigger (even small shunt)

Prior IE = closure indication + prophylaxis

Well-tolerated if small/no PAH; Eisenmenger contraindicated

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

Severity triggers (9)

9 need judgement
  • informationallife_threateningeisenmenger_no_closure
    Severe PAH / Eisenmenger physiology (high PVR, bidirectional/R→L shunt) — VSD closure CONTRAINDICATED; PAH-targeted therapy + Eisenmenger precautions — 2020 ESC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveresignificant_shunt_closure
    Qp:Qs ≥1.5 + LV volume overload + acceptable PVR, or prior VSD-related IE — closure indicated — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverevsd_associated_ar_branch
    Outlet/perimembranous VSD with progressive AR from aortic cusp prolapse — surgery to halt AR (timing AR-driven, not shunt-size-driven) — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereie_history_branch
    Prior VSD-related infective endocarditis — closure indication + IE prophylaxis + low fever-workup threshold — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveredeveloping_pah_branch
    Rising PA pressure / borderline PVR on serial assessment — RHC + reassessment of closure window before Eisenmenger develops — 2022 ESC/ERS PH
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepregnancy_special_pop
    Pregnancy with VSD — well-tolerated if small/no PAH (mWHO I–II); Eisenmenger = very high maternal mortality (pregnancy contraindicated); cardio-obstetric — ESC 2018 Pregnancy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateresidual_vsd_post_closure
    Residual VSD / device or patch complication post-closure — imaging + ACHD re-evaluation; residual jet = IE risk — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateckd_special_pop
    CKD — minimise contrast (TEE/RHC); renal-adjust PAH/AC drugs — KDIGO 2024
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmildsmall_restrictive_branch
    Small restrictive VSD, normal PA pressure, no LV overload/AR/IE — no closure; IE-awareness + periodic surveillance — 2020 ESC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

VSD — closure / AR-surgery / PAH decision (2018 AHA/ACC ACHD; 2020 ESC ACHD; 2022 ESC/ERS PH)
axis: vsd_closure_ar_or_pahstep 1 - Step 1 — Assess shunt + PVR + AR (gatekeeper)
Selected step "Step 1 — Assess shunt + PVR + AR (gatekeeper)" — Any VSD

outpatient playbook — drug actions (3)

  1. 1. closure if significant + acceptable PVR
    procedure • surgical/transcatheter • n/a
    trigger: Qp:Qs ≥1.5 + LV overload + acceptable PVR (2018 AHA/ACC ACHD)
    Reverse LV overload, reduce PAH/IE risk
  2. 2. surgery for VSD-associated progressive AR
    procedure • surgical • n/a
    trigger: Cusp-prolapse progressive AR (2018 AHA/ACC ACHD)
    Halt AR progression
  3. 3. PAH therapy + NO closure if Eisenmenger
    macitentan 10 mg ± tadalafil 40 mg • PO • daily
    trigger: Severe PAH/Eisenmenger (2022 ESC/ERS PH)
    Closure contraindicated

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Harsh holosystolic murmur (LLSB) ± thrill; Echo: VSD with LV volume overload / left-to-right shunt; New/progressive AR (aortic cusp prolapse — outlet/perimembranous).

Objective

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

Assessment

**Adult ventricular septal defect (chronic ACHD)** (cardio.achd-vsd.chronic.v1).
Phenotype framing: Restrictive vs moderate-large; AR-associated; Eisenmenger; double-chambered RV
Scope: Define VSD type/size + shunt + PVR + AR involvement

No severity triggers fired against current inputs.

Plan

Regimen axis: **VSD — closure / AR-surgery / PAH decision (2018 AHA/ACC ACHD; 2020 ESC ACHD; 2022 ESC/ERS PH)** — step "Step 1 — Assess shunt + PVR + AR (gatekeeper)".

Setting playbook (outpatient) — Quantify shunt/PVR/AR; close significant or AR-associated VSD if PVR acceptable; PAH-treat (no closure) if Eisenmenger; IE awareness (2018 AHA/ACC ACHD; 2020 ESC ACHD)
1. closure if significant + acceptable PVR procedure surgical/transcatheter n/a — Qp:Qs ≥1.5 + LV overload + acceptable PVR (2018 AHA/ACC ACHD) (Reverse LV overload, reduce PAH/IE risk)
2. surgery for VSD-associated progressive AR procedure surgical n/a — Cusp-prolapse progressive AR (2018 AHA/ACC ACHD) (Halt AR progression)
3. PAH therapy + NO closure if Eisenmenger macitentan 10 mg ± tadalafil 40 mg PO daily — Severe PAH/Eisenmenger (2022 ESC/ERS PH) (Closure contraindicated)

Non-pharmacologic actions:
- ACHD centre + structural/PAH referral — 2018 AHA/ACC ACHD
- IE-awareness education + prophylaxis where indicated — 2018 AHA/ACC ACHD
- Pregnancy + transition-of-care counseling — 2018 AHA/ACC ACHD

AVOID / contraindication checks:
- VSD closure CONTRAINDICATED in severe PAH Eisenmenger — 2020 ESC ACHD
- Close for progressive AR from cusp prolapse before AR severe not shunt size driven — 2018 AHA/ACC ACHD
- Small restrictive VSD no PAH no AR no IE no closure IE awareness only — 2020 ESC ACHD
- Assess PVR before any closure — 2018 AHA/ACC ACHD

Monitoring

Regimen monitoring:
- post closure echo residual VSD and AR — 2018 AHA/ACC ACHD
- AR progression surveillance outlet perimembranous — 2018 AHA/ACC ACHD
- PAH reassessment if borderline — 2022 ESC/ERS PH
- IE awareness and fever evaluation — 2018 AHA/ACC ACHD

Setting (outpatient) monitoring:
- Post-closure residual VSD + AR progression + PAH — 2018 AHA/ACC ACHD

Follow-up plan: Lifelong ACHD; pregnancy + transition planning; IE education
- Close-out criterion: lifelong ACHD plan documented

Monitoring phase: Residual VSD/device, AR progression, PAH

Disposition

Current setting: outpatient — Quantify shunt/PVR/AR; close significant or AR-associated VSD if PVR acceptable; PAH-treat (no closure) if Eisenmenger; IE awareness (2018 AHA/ACC ACHD; 2020 ESC ACHD)

Disposition criteria:
- Significant or AR-associated VSD, PVR acceptable → surgery/closure
- Eisenmenger → PAH therapy, no closure, ACHD-PAH centre
- Small restrictive, no PAH/AR/IE → IE-awareness + surveillance

Escalation triggers (move to higher acuity):
- Severe PAH/Eisenmenger → STOP closure pathway, PAH therapy + Eisenmenger route — 2020 ESC ACHD
- Progressive AR → expedite surgery — 2018 AHA/ACC ACHD
- Active IE → IV antibiotics + surgical evaluation — 2018 AHA/ACC ACHD

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Severe PAH / Eisenmenger physiology (high PVR, bidirectional/R→L shunt) — VSD closure CONTRAINDICATED; PAH-targeted therapy + Eisenmenger precautions — 2020 ESC ACHD
- [SEVERE] Qp:Qs ≥1.5 + LV volume overload + acceptable PVR, or prior VSD-related IE — closure indicated — 2018 AHA/ACC ACHD
- [SEVERE] Outlet/perimembranous VSD with progressive AR from aortic cusp prolapse — surgery to halt AR (timing AR-driven, not shunt-size-driven) — 2018 AHA/ACC ACHD

Citations

- 2018 AHA/ACC Adult Congenital Heart Disease Guideline (Stout) + 2020 ESC Adult Congenital Heart Disease Guideline (Baumgartner) + 2022 ESC/ERS Pulmonary Hypertension Guideline [PMID:30121239](https://pubmed.ncbi.nlm.nih.gov/30121239/)
- Cited evidence (PMID 32860028) [PMID:32860028](https://pubmed.ncbi.nlm.nih.gov/32860028/)
- Cited evidence (PMID 36017572) [PMID:36017572](https://pubmed.ncbi.nlm.nih.gov/36017572/)
- Cited evidence (PMID 32673028) [PMID:32673028](https://pubmed.ncbi.nlm.nih.gov/32673028/)

Last reconciled with current guidelines: 2026-05-16.
References
  • 2018 AHA/ACC Adult Congenital Heart Disease Guideline (Stout) + 2020 ESC Adult Congenital Heart Disease Guideline (Baumgartner) + 2022 ESC/ERS Pulmonary Hypertension Guideline — PMID:30121239
  • Cited evidence (PMID 32860028) — PMID:32860028
  • Cited evidence (PMID 36017572) — PMID:36017572
  • Cited evidence (PMID 32673028) — PMID:32673028