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cardio.achd-asd.chronic.v1PRODUCTION
cardio.achd-asd.chronic.v1

Adult atrial septal defect (chronic ACHD)

cardiologychronicadult
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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

Define ASD type + shunt direction/magnitude + pulmonary vascular status

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ASD type + shunt physiology framed

Patient inputs (10)

Elderly late-presentation needs LV diastolic assessment before closure

Secundum (transcatheter) vs primum/sinus-venosus/coronary-sinus (surgical)

RV dilatation/overload supports hemodynamic significance

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

Severe PAH/Eisenmenger (high PVR, R→L) = closure CONTRAINDICATED

Symptomatic significant ASD strengthens closure indication

Contrast for TEE/RHC/CMR; drug dosing

Adequate rims → transcatheter device feasibility (secundum)

AF/atrial flutter — rate/rhythm + anticoagulation

Well-tolerated if no PAH; PAH = very high maternal risk

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Severity triggers (9)

9 need judgement
  • informationallife_threateningeisenmenger_no_closure
    Severe PAH / Eisenmenger physiology (high PVR, bidirectional/R→L shunt, cyanosis) — ASD 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 + RV volume overload + acceptable PVR — closure indicated (transcatheter secundum / surgical otherwise) — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereparadoxical_embolism_branch
    Cryptogenic stroke / paradoxical embolism via ASD/PFO — closure + anticoagulation consideration — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepregnancy_special_pop
    Pregnancy with ASD — well-tolerated if no PAH (mWHO I–II); severe PAH/Eisenmenger = very high maternal mortality (pregnancy contraindicated); cardio-obstetric — ESC 2018 Pregnancy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateasd_type_branch
    Sinus venosus / primum / coronary-sinus ASD — surgical (not transcatheter); sinus venosus assess PAPVR by CMR — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateaf_atrial_flutter_branch
    AF/atrial flutter (common, increases with age/late closure) — rate/rhythm + anticoagulation; concomitant maze at surgical closure — 2020 ESC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateelderly_late_presentation_branch
    Elderly late-presenting ASD — assess LV diastolic function/filling pressures before closure (risk of post-closure LV failure) — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateresidual_shunt_post_closure
    Residual shunt / device complication (erosion, thrombus, malposition) post-closure — imaging + ACHD re-evaluation — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateckd_special_pop
    CKD — minimise contrast (TEE/RHC/CMR); renal-adjust AC/PAH drugs — KDIGO 2024
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

ASD — closure-vs-PAH decision + arrhythmia/AC (2018 AHA/ACC ACHD; 2020 ESC ACHD; 2022 ESC/ERS PH)
axis: asd_closure_or_pahstep 1 - Step 1 — Assess shunt + pulmonary vascular resistance (gatekeeper)
Selected step "Step 1 — Assess shunt + pulmonary vascular resistance (gatekeeper)" — Any hemodynamically relevant ASD

outpatient playbook — drug actions (3)

  1. 1. closure (transcatheter/surgical) if PVR acceptable
    procedure • transcatheter/surgical • n/a
    trigger: Qp:Qs ≥1.5 + RV overload + acceptable PVR (2018 AHA/ACC ACHD)
    Reverse RV overload, reduce arrhythmia/PAH risk
  2. 2. PAH therapy + NO closure if Eisenmenger
    macitentan 10 mg ± tadalafil 40 mg ± selexipag • PO • daily/BID
    trigger: Severe PAH/Eisenmenger (2022 ESC/ERS PH)
    Closure contraindicated; treat pulmonary vascular disease
  3. 3. AF anticoagulation/rate control
    apixaban 5 mg BID ± metoprolol • PO • BID
    trigger: AF / paradoxical embolism (ESC 2024 AF)
    Thromboembolic prevention

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Fixed split S2 / systolic flow murmur with RV heave; Echo: ASD with RV volume overload / left-to-right shunt; Exertional dyspnea / new atrial fibrillation in adult.

Objective

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

Assessment

**Adult atrial septal defect (chronic ACHD)** (cardio.achd-asd.chronic.v1).
Phenotype framing: Secundum vs primum vs sinus venosus vs PFO; shuntable vs Eisenmenger
Scope: Define ASD type + shunt direction/magnitude + pulmonary vascular status

No severity triggers fired against current inputs.

Plan

Regimen axis: **ASD — closure-vs-PAH decision + arrhythmia/AC (2018 AHA/ACC ACHD; 2020 ESC ACHD; 2022 ESC/ERS PH)** — step "Step 1 — Assess shunt + pulmonary vascular resistance (gatekeeper)".

Setting playbook (outpatient) — Quantify shunt + PVR, close if PVR-acceptable significant ASD, PAH-treat (no closure) if Eisenmenger, manage arrhythmia/AC (2018 AHA/ACC ACHD; 2020 ESC ACHD)
1. closure (transcatheter/surgical) if PVR acceptable procedure transcatheter/surgical n/a — Qp:Qs ≥1.5 + RV overload + acceptable PVR (2018 AHA/ACC ACHD) (Reverse RV overload, reduce arrhythmia/PAH risk)
2. PAH therapy + NO closure if Eisenmenger macitentan 10 mg ± tadalafil 40 mg ± selexipag PO daily/BID — Severe PAH/Eisenmenger (2022 ESC/ERS PH) (Closure contraindicated; treat pulmonary vascular disease)
3. AF anticoagulation/rate control apixaban 5 mg BID ± metoprolol PO BID — AF / paradoxical embolism (ESC 2024 AF) (Thromboembolic prevention)

Non-pharmacologic actions:
- ACHD centre + structural/PAH referral — 2018 AHA/ACC ACHD
- Eisenmenger precautions (air filters, avoid dehydration/high altitude, IE prophylaxis) — 2020 ESC ACHD
- Pregnancy + transition-of-care counseling — 2018 AHA/ACC ACHD

AVOID / contraindication checks:
- ASD closure CONTRAINDICATED in severe PAH Eisenmenger high PVR R to L — 2020 ESC ACHD
- Assess PVR before closure especially elderly or PAH suspected — 2018 AHA/ACC ACHD
- Secundum transcatheter non secundum surgical — 2018 AHA/ACC ACHD
- Meticulous air filter and IE considerations with R to L shunt — 2020 ESC ACHD

Monitoring

Regimen monitoring:
- post closure echo residual shunt and device position — 2018 AHA/ACC ACHD
- arrhythmia surveillance AF flutter — 2020 ESC ACHD
- PAH reassessment RHC if borderline — 2022 ESC/ERS PH
- RV remodeling serial echo — 2018 AHA/ACC ACHD

Setting (outpatient) monitoring:
- Post-closure residual shunt + arrhythmia + RV remodeling — 2018 AHA/ACC ACHD
- PAH reassessment if borderline — 2022 ESC/ERS PH

Follow-up plan: Lifelong ACHD; pregnancy + transition planning; persistent-AF AC review
- Close-out criterion: lifelong ACHD plan documented

Monitoring phase: Post-closure residual shunt/device surveillance, arrhythmia, PAH progression

Disposition

Current setting: outpatient — Quantify shunt + PVR, close if PVR-acceptable significant ASD, PAH-treat (no closure) if Eisenmenger, manage arrhythmia/AC (2018 AHA/ACC ACHD; 2020 ESC ACHD)

Disposition criteria:
- PVR-acceptable significant ASD → closure
- Eisenmenger → PAH therapy, no closure, ACHD-PAH centre
- Small/non-significant ASD → surveillance

Escalation triggers (move to higher acuity):
- Severe PAH/Eisenmenger identified → STOP closure pathway, PAH therapy + route Eisenmenger — 2020 ESC ACHD
- Decompensation/stroke → acute pathway — 2018 AHA/ACC ACHD
- Persistent AF → AC + rhythm strategy — ESC 2024 AF

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Severe PAH / Eisenmenger physiology (high PVR, bidirectional/R→L shunt, cyanosis) — ASD closure CONTRAINDICATED; PAH-targeted therapy + Eisenmenger precautions — 2020 ESC ACHD
- [SEVERE] Qp:Qs ≥1.5 + RV volume overload + acceptable PVR — closure indicated (transcatheter secundum / surgical otherwise) — 2018 AHA/ACC ACHD
- [SEVERE] Cryptogenic stroke / paradoxical embolism via ASD/PFO — closure + anticoagulation consideration — 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