By lesion and procedure, neonate to adolescent: agent, start, target, duration; graded rows by source, adult rows labelled
| Procedure | Post-procedure | Long-term | Before another procedure |
|---|---|---|---|
| Systemic-to-pulmonary shunt (mBTT, central shunt) | Post-procedure
IB-NR Aspirin
IIaB-NR UFH until aspirin is started
IIbC-EO Bivalirudin until aspirin is started |
Long-term
IB-NR Aspirin indefinitely
IIbC-EO Dual antiplatelet therapy for complex shunt scenarios |
Before another procedure
IIbC-EO Avoid holding aspirin; if holding is necessary, bridge with LMWH or UFH |
| Norwood (HLHS) (Sano) | Post-procedure
IB-NR Aspirin
IIaB-NR Consider UFH if aspirin cannot start within 48 h
IIbC-EO Consider bivalirudin if aspirin cannot start within 48 h |
Long-term
IB-NR Aspirin indefinitely |
Before another procedure
IIbC-EO Avoid holding aspirin; if holding, bridge with LMWH or UFH |
| Class | Recommendation | LOE |
|---|---|---|
| I | No increased bleeding risk: long-term low-dose aspirin AHA 2013: the incidence of complications from low-dose aspirin in neonates has not been reported. Dose range in Targets. | B |
| IIa | Continuous low-dose heparin infusion early after surgery, once surgical bleeding concerns have resolved | C |
| IIa | A thrombosis risk factor (suspected or confirmed infection, CVL thrombus, stented shunt, hypercoagulable state): systemic heparinization early after surgery (“probably recommended”) | C |
| IIa | Acute shunt thrombosis: IV heparin bolus 50–100 U/kg and consider an infusion; raise systemic pressure (phenylephrine or epinephrine to effect); intubate, ventilate, paralyse; anticipate cath, shunt revision or ECMO | C |
| IIb | Sustained high risk (stented shunt, hypercoagulable state): anticoagulant plus antiplatelet, e.g. LMWH plus aspirin or clopidogrel | C |
| III | Systemic thrombolysis (e.g. tPA) for shunt thrombosis may be harmful | C |
| Grade | Recommendation | No. |
|---|---|---|
| Grade 2C | Intraoperative UFH | 2.6 |
| Grade 2C | After surgery: aspirin or no antithrombotic therapy, rather than prolonged LMWH or VKA | 2.6 |
Palliated single ventricle at any stage (AHA 2013 §9.2): risk-factor assessment, TTE surveillance, confirming a suspected thrombus, starting or escalating therapy when risk factors are present: rows under .
| Procedure | Post-procedure | Long-term | Before another procedure |
|---|---|---|---|
| Pulmonary atresia, RV–PA conduit | Post-procedure IIbC-EO None |
Long-term IIbC-EO Aspirin 3–6 mo |
Before another procedure IIbC-EO Avoid holding aspirin; if necessary, hold 5–7 d |
| Rastelli or Nikaidoh (RV–PA conduit) | Post-procedure IIbC-EO None; consider UFH if aspirin is needed for the conduit type and cannot start within 48 h |
Long-term IIbC-EO Aspirin 3–6 mo |
Before another procedure IIbC-EO Avoid holding aspirin; if necessary, hold 5–7 d |
| Truncus arteriosus repair (RV–PA conduit) | Post-procedure IIbC-EO None; consider UFH if aspirin is needed for the conduit type and cannot start within 48 h |
Long-term IIbC-EO Aspirin indefinitely |
Before another procedure IIbC-EO Avoid holding aspirin; if necessary, hold 5–7 d |
| CoR | Statement | LoE |
|---|---|---|
| IIb | Low-dose short-acting IV anticoagulation until enteral antiplatelet therapy is feasible, in the immediate (24–72 h) and early (first week) postoperative period (S23) | C-EO |
| IIb | Long term: aspirin monotherapy, duration by procedure and thrombotic risk (S24) | C-EO |
AHA 2013 and CHEST 2012 give no conduit row. AHA 2013: after a bioprosthetic pulmonary valve it is reasonable to forgo anticoagulation ().
Table 7 has a Moderate tier that Table 6 does not grade separately; the text says agents escalate from single antiplatelet (low) to dual antiplatelet (moderate) to antiplatelet plus anticoagulant (high), with the plan set by the team.
| Procedure | Post-procedure | Long-term | Before another procedure |
|---|---|---|---|
| Bidirectional Glenn, hemi-Fontan | Post-procedure IIaB-NR None |
Long-term IB-NR Aspirin |
Before another procedure IIbC-EO Avoid holding aspirin; if holding, bridge with LMWH or UFH |
| Comprehensive stage 2 | Post-procedure IIbC-EO UFH until aspirin is started |
Long-term
IB-NR Aspirin
IIbC-EO Aspirin ± LMWH for 3–6 mo
IIbC-EO Aspirin ± DOAC for 3–6 mo |
Before another procedure IIbC-EO Avoid holding aspirin; if holding, bridge with LMWH or UFH |
| Class | Recommendation | LOE |
|---|---|---|
| IIb | Long-term antiplatelet prophylaxis after a superior cavopulmonary anastomosis | C |
| IIb | Chronically draining pleural effusions of significant volume: closely monitor clotting studies, AT III and anti-FXa levels | C |
| Grade | Recommendation | No. |
|---|---|---|
| Grade 2C | Postoperative UFH | 2.35 |
Palliated single ventricle at any stage (AHA 2013 §9.2): risk-factor assessment, TTE surveillance, confirming a suspected thrombus, starting or escalating therapy when risk factors are present: rows under .
| Class | Recommendation | LOE |
|---|---|---|
| IIa | Long-term antiplatelet therapy after the Fontan | C |
| IIb | Warfarin or LMWH for 3 to 12 months after the Fontan | C |
| IIb | Long-term warfarin after the Fontan with anatomic or hemodynamic risk factors | C |
| IIa | Start or escalate therapy (antiplatelet to anticoagulant, or higher targets) if risk factors are present (become present) at any stage | C |
| IIb | Start or escalate therapy after the Fontan in adolescence or adulthood | C |
| I | Assess anatomic and hemodynamic risk factors; correct or minimize them | B |
| I | Reassess risk factors serially; correct new ones | C |
| I | Periodic TTE with attention to thrombus | C |
| IIa | Suspected thrombus: confirm with TEE, MRI, CT, CT angiography, lung perfusion scan or venography/angiography | C |
| IIb | With risk factors, TEE or MRI for surveillance | C |
| Grade | Recommendation | No. |
|---|---|---|
| Grade 1C | Aspirin, or therapeutic UFH followed by VKA, over no therapy | 2.36 |
| COR | Recommendation | LOE |
|---|---|---|
| 1 | Adult Known or suspected thrombus, atriopulmonary Fontan, history of thromboembolism, or sustained atrial flutter or fibrillation: anticoagulation | B-NR |
| 1 | Adult No high-risk features and no bleeding contraindication: aspirin or anticoagulation | B-NR |
AHA 2019 Fontan statement, Table 18 (no class): risk of thromboembolism is increased; the best prevention is unclear, but some thromboprophylaxis, antiplatelet or anticoagulant, is warranted.
| Procedure | Post-procedure | Long-term | Before another procedure |
|---|---|---|---|
| Mitral valve replacement (mechanical) | Post-procedure
IA UFH until therapeutic on enteral anticoagulation
IIbC-EO Bivalirudin until therapeutic on enteral anticoagulation |
Long-term IA VKA, INR goal 2.5–3.5, + aspirin if there is an indication for antiplatelet therapy |
Before another procedure IIbC-EO If the procedure requires bridging, consider LMWH or UFH if INR <2 during VKA interruption |
| Aortic valve replacement (mechanical) | Post-procedure
IA UFH until therapeutic on enteral anticoagulation
IIbC-EO Bivalirudin until therapeutic on enteral anticoagulation |
Long-term IA VKA, INR goal 2–3, + aspirin if there is an indication for antiplatelet therapy |
Before another procedure IIbC-EO If the procedure requires bridging, consider LMWH or UFH if INR <1.5 during VKA interruption |
| CoR | Statement | LoE |
|---|---|---|
| IIb | Aspirin added to VKA (mechanical mitral or aortic) with an indication for antithrombotic therapy and low bleeding risk (S34) | C-EO |
| Class | Recommendation | LOE |
|---|---|---|
| I | Mitral, any mechanical valve: warfarin, INR 2.5–3.5 | C |
| I | Aortic, bileaflet or Medtronic Hall: warfarin, INR 2.0–3.0; with risk factors 2.5–3.5 | C |
| I | Aortic, Starr-Edwards valves or mechanical disk valves (other than Medtronic Hall), no risk factors: warfarin, INR 2.5–3.5 | C |
| IIa | First 3 months after mechanical aortic valve: warfarin, INR 2.5–3.5 | C |
| I | Unable to take warfarin after mitral or aortic replacement: low-dose aspirin | C |
| I | Adult Add low-dose aspirin to warfarin: mechanical valves, and bioprosthetic valves with risk factors (adult guidelines; data in children limited) | C |
| CoR | Statement | LoE |
|---|---|---|
| IIa | Thromboprophylaxis after any valve replacement based on valve characteristics, location, bleeding and thrombotic risk (S30) | C-EO |
| IIa | Melody (Medtronic) mitral valve: institutional care pathways and multidisciplinary team (S32) | C-EO |
| Class | Recommendation | LOE |
|---|---|---|
| I | Aortic or mitral, no risk factors: low-dose aspirin | C |
| I | Aortic with risk factors: warfarin, INR 2.0–3.0 | C |
| I | Mitral with risk factors: warfarin, INR 2.0–3.0 | C |
| IIa | Aortic or mitral, no risk factors, first 3 months: warfarin, INR 2.0–3.0 | C |
| IIa | Pulmonary: forgo anticoagulation | C |
| IIa | Tricuspid, with or without decreased function: warfarin, INR 2.0–3.0 for 3–6 months | C |
| IIa | Tricuspid: lifelong low-dose aspirin | C |
| IIb | Tricuspid with decreased RV function or risk factors: long-term warfarin, INR 2.0–3.0 | C |
Low-dose aspirin if warfarin cannot be taken after mitral or aortic replacement (AHA 2013, I), and aspirin added to warfarin with risk factors (AHA 2013, I, Adult): rows under . CHEST 2012 (2.40–2.42, no grade printed): follow the adult recommendations for prosthetic valves.
| Low | Moderate | High | |
|---|---|---|---|
| Hardware | Low
|
Moderate
|
High
|
| Location | Low
|
Moderate
|
High
|
| Thrombosis risk | Post-procedure | Long-term | Before another procedure |
|---|---|---|---|
| Lower risk | Post-procedure IIbC-EO None; consider prophylactic UFH or LMWH if aspirin cannot start within 24 h |
Long-term IB-NR Aspirin for 6 mo |
Before another procedure IIbC-EO Avoid holding aspirin; if necessary, hold 5–7 d |
| Higher risk | Post-procedure IIaC-EO Therapeutic UFH or LMWH |
Long-term
IB-NR Aspirin ± clopidogrel for at least 3–6 mo
IIbC-EO Aspirin ± LMWH for at least 3–6 mo
IIbC-EO Aspirin ± DOAC for at least 3–6 mo |
Before another procedure IIbC-EO Avoid holding aspirin; if holding, bridge with LMWH or UFH |
| Class | Recommendation | LOE |
|---|---|---|
| I | Stent placement: UFH bolus 100 U/kg (maximum 5000 U) | C |
| I | Noncoronary stents: low-dose aspirin for at least 6 months | C |
| IIa | Higher-risk lesions (nonpulsatile flow, previous complete occlusion, thrombophilic abnormality), in the absence of contraindications: warfarin or LMWH ± antiplatelet for 3–6 months, then antiplatelet alone | C |
| I | ASD device: at least 100 U/kg UFH (maximum 5000 U) at implantation | C |
| I | ASD device: low-dose aspirin for at least 6 months | C |
| IIb | ASD device, older children and adults: another anticoagulant with aspirin for 3–6 months | C |
| I | VSD device: UFH 100 U/kg (maximum 5000 U) at implantation | C |
| IIa | VSD device: low-dose aspirin for at least 6 months | C |
| Grade | Recommendation | No. |
|---|---|---|
| Grade 2C | Endovascular stents: UFH perioperatively | 2.37 |
| CoR | Statement | LoE |
|---|---|---|
| IIb | Thromboprophylaxis with a CVC, by patient and anatomic risk factors, weighed against bleeding (S29) | C-EO |
| Class | Recommendation | LOE |
|---|---|---|
| I | Remove CVLs as soon as no longer clinically essential; review every line daily | C |
| IIa | Thrombus on imaging: consider removing the CVL, weighing embolization; follow with serial imaging | C |
| IIa | Documented acute CVL thrombus: anticoagulation if no contraindication | B |
| IIb | Future Fontan candidates: low-dose IV heparin until the CVL is removed | C |
| IIb | CVL with bacteremia or a hypercoagulable risk factor (high hematocrit, prior thrombosis, thrombophilia): low-dose IV heparin until removal | C |
| Grade | Recommendation | No. |
|---|---|---|
| Grade 1B | Children, short- or medium-term CVADs: against routine systemic thromboprophylaxis | 2.31 |
| Grade 1A | Neonates: UFH continuous infusion at 0.5 units/kg per h to maintain CVAD patency, over no prophylaxis | 2.4 |
| Grade 1B | Children, first VTE: UFH or LMWH, initially for at least 5 days | 2.22.1 |
| Grade 2C | Children, secondary VTE with the risk factor resolved: anticoagulate for 3 months; with an ongoing but potentially reversible risk factor, continue beyond 3 months (therapeutic or prophylactic doses) until it resolves | 2.22.3 |
| Grade 1B | CVAD no longer required or nonfunctioning: remove | 2.22.6 |
| Grade 2C | At least 3–5 days of anticoagulation before removal | 2.22.6 |
| Grade 2C | CVAD functioning and still required: leave in place and anticoagulate | 2.22.6 |
| Grade 2C | CVAD still needed after 3 months: prophylactic VKA (INR 1.5–1.9) or LMWH (anti-Xa 0.1–0.3 units/mL) until removal; if thrombosis recurs on prophylaxis, therapeutic doses until the CVAD is removed and for a minimum of 3 months following the VTE | 2.22.7 |
| Grade 2C | Neonates, CVAD or UVC with confirmed thrombosis: either initial anticoagulation or supportive care with radiologic monitoring for extension, rather than no follow-up; in previously untreated patients, start anticoagulation if extension occurs | 2.1 |
| Grade 2C | Neonates, CVAD or UVC with thrombosis: remove after 3–5 days of therapeutic anticoagulation | 2.1 |
| Grade 2C | Neonates, if anticoagulated: LMWH, or UFH then LMWH, for 6 weeks to 3 months in total | 2.1 |
| Grade 2C | Neonates, line still in place when treatment ends: prophylactic dose until removal | 2.1 |
| Strength | Recommendation | Certainty |
|---|---|---|
| Strong | Symptomatic DVT or PE: anticoagulation rather than none | very low |
| Conditional | LMWH or VKA | very low |
| Conditional | Provoked DVT or PE: ≤3 months rather than longer | very low |
| Conditional | Functioning CVAD still needed: do not remove | very low |
| Strong | Nonfunctioning or unneeded CVAD: remove | very low |
| Conditional | Remove it some days after anticoagulation starts, not immediately | very low |
| Conditional | Right atrial thrombosis: anticoagulation rather than none | very low |
Antithrombotic management by coronary risk level (AHA 2024 Table 2) is on Kawasaki Coronary Z-Scores.
| Agent | Target or dose | Source |
|---|---|---|
| UFH, therapeutic | Anti-Xa 0.35–0.7 U/mL; if unavailable, PTT 1.5–3× baseline AHA 2013 Table 3: each laboratory sets its therapeutic PTT range (usually 60–100 s); lower PTTs may limit postoperative bleeding; check at least every 24 h. CHEST 2012 1.1 (2C): or an aPTT range matching anti-Xa 0.35–0.7 units/mL or protamine titration 0.2–0.4 units/mL; boluses no greater than 75–100 units/kg. | AHA 2013 Table 3; CHEST 2012 1.1 |
| UFH, low dose | 10–15 U/kg/h AATS 2026 text: the dose most studies report after shunts until aspirin starts, and after conduits; also a prophylaxis option after lower-risk stents when aspirin cannot start within 24 h and thrombosis risk is deemed to outweigh bleeding risk (usually without testing or titration). AHA 2013 Table 3: “low dose is commonly” this range. | AHA 2013 Table 3; AATS 2026 |
| LMWH, therapeutic | Anti-Xa 0.5–1.0 U/mL at 4–6 h after the dose (or 0.5–0.8 at 2–6 h, CHEST) AHA 2013 Table 3: levels at least monthly; INR and PTT are not affected. CHEST 2012 1.2 (2C) applies to once- or twice-daily dosing in neonates and children. | CHEST 2012 1.2; AHA 2013 Table 3 |
| LMWH, prophylactic | Anti-Xa 0.2–0.5 U/mL after a lower-risk stent (AATS) · 0.1–0.3 units/mL with a line kept after VTE (CHEST) AATS 2026 text: if aspirin cannot be started within 24 hours and the risk of thrombosis is deemed to outweigh the risk of bleeding, parenteral anticoagulation at prophylaxis dosing (UFH 10–15 units/kg/h, usually without testing or titration, or LMWH to an anti-Xa of 0.2–0.5 U/mL, or per institutional protocol) may be considered (Table 6, IIb, C-EO). CHEST 2012 2.22.7 (2C): a CVAD still needed after 3 months of treatment for VTE. | AATS 2026 text; CHEST 2012 2.22.7 |
| Warfarin (VKA) | INR 2–3 (CHEST: 2.5, range 2.0–3.0); mechanical mitral 2.5–3.5; mechanical aortic in Mechanical valve AHA 2013 Table 3: INR daily until therapeutic, then at least monthly; retest with illness, medication or diet change. §3.3.2: at least every 4 weeks once the dose is stable (Class I, B). Warfarin is generally not recommended under 1 year of age unless a mechanical valve needs it (AHA 2013). AATS 2026: reaching the target can take 5–7 d in infants; LMWH until the INR is therapeutic. | AHA 2013 Table 3; CHEST 2012 1.3 |
| Warfarin, prophylactic | INR 1.5–1.9 (line kept after VTE) | CHEST 2012 2.22.7 |
| Aspirin | 1–5 mg/kg/day (maximum 81–325 mg/day, AHA) · AATS: 3–5 (unit as printed varies, frequency not stated) AHA 2013 Table 3 and CHEST 2012 1.5 (2C) print 1–5 mg/kg/day. AATS 2026 prints 3–5 as mg/kg/dose (shunt text: “commonly reported”; valve text, S30), as mg/kg/d (conduit text: preferred for early enteral therapy) and as mg/kg (S34 text), with no frequency stated; shunt doses from 1.5 to 12.5 mg/kg/d have been reported. No routine monitoring (AATS 2026 Table 3; AHA 2013: no target studied). Platelet testing: aspirin <550 ARU, clopidogrel about 50–200 PRU on VerifyNow, extrapolated from adult studies (AATS 2026 Table 4). In select neonates and infants with recurrent thrombosis or concern for inadequate antiplatelet effect, platelet responsiveness testing may be considered within experienced centers, recognizing that validation and outcome data are limited (S20, IIb, C-EO). | AHA 2013 Table 3; CHEST 2012 1.5; AATS 2026 |
| Clopidogrel | 0.2–1 mg/kg/day | AHA 2013 Table 3 |