# Perioperative Management of Congenital Heart Disease

Noncardiac surgery in patients with congenital heart disease requires lesion- and physiology-specific planning. Determine residual obstruction, shunt direction, ventricular function, pulmonary vascular disease, rhythm risk, and procedural stress before selecting surgical setting, monitoring, anesthesia, and postoperative disposition.

**Clinical question:** How should clinicians stratify and manage congenital heart disease physiology before, during, and after noncardiac surgery?

Updated: 2026-08-21T02:23:03.154289+00:00

## What matters in practice
- Do not use the diagnostic label alone for risk assessment; define current anatomy, residual lesion burden, ventricular function, pulmonary vascular status, rhythm history, cyanosis, and functional status before elective noncardiac surgery.[2][16][18]
- Escalate patients with complex congenital heart disease, palliated circulation, pulmonary hypertension, cyanosis, heart failure, or important residual lesions to a congenital heart disease center or coordinated team with congenital cardiology and cardiac anesthesia expertise.[18][19]
- ASA physical status 3 or greater, emergency surgery, major or severe congenital heart disease, single-ventricle physiology, ventricular dysfunction, and selected surgical specialties identify pediatric patients at increased perioperative cardiovascular-event risk.[2][3]
- For right ventricular failure or pulmonary vascular disease, preserve preload and contractility while avoiding increases in pulmonary vascular resistance; systemic hypotension in a right-to-left shunt can worsen shunting and acidosis.[4][9]
- Continue beta-blockers and pulmonary hypertension therapies through surgery when feasible; interruption can destabilize patients whose circulation depends on rhythm control or pulmonary vasodilation.[13]

## Select the care setting from present physiology, not repair history

Classify the current circulation before accepting elective surgery.

Obtain the operative reports, most recent congenital cardiology assessment, ECG, echocardiogram, catheterization data when relevant, baseline oxygen saturation, hemoglobin concentration, medication list, and prior anesthesia record. The preoperative question is whether repair is anatomically and physiologically complete or whether residual obstruction, regurgitation, shunting, ventricular dysfunction, arrhythmia, pulmonary hypertension, cyanosis, or a palliated circulation remains.[5][18]

Patients with complete anatomic repair and no functional deterioration may often undergo conventional anesthetic management. In contrast, complex congenital heart disease, palliative procedures, or interventional repairs require individualized monitoring and hemodynamic planning because the prior operation does not establish normal physiology.[18]

Route patients with complex disease, heart failure, pulmonary hypertension, cyanosis, or a Fontan/single-ventricle circulation to a center with adult or pediatric congenital heart disease expertise when procedure urgency permits. U.S. guidance has supported regionalized care for adults with congenital heart disease undergoing noncardiac surgery.[18][19]
- Defer elective surgery for newly recognized physiologic deterioration until congenital cardiology clarifies the mechanism and perioperative plan; use echocardiography and, when needed, catheterization data to characterize ventricular performance and pulmonary hemodynamics.[18]
- Treat emergency status as an independent risk amplifier; establish lesion-specific hemodynamic goals and a postoperative location before induction rather than attempting to reconstruct anatomy during an intraoperative crisis.[2][3]
- Anticipate difficult peripheral and central access after repeated catheterizations or surgery; review venous patency and prior access history before selecting central venous access.[18]

*Features that should change perioperative location, monitoring, or staffing.[2][3][18][19]*

| Current finding | Why it changes the plan | Immediate planning action |
| --- | --- | --- |
| Repaired simple defect without functional deterioration | Conventional anesthetic techniques may be feasible when repair is complete and physiology remains stable.[18] | Confirm current echocardiographic status and proceed with monitoring proportional to surgical stress.[18] |
| Major or severe congenital heart disease, important residual lesion, or prior complex repair | Complexity and residual physiologic burden are associated with higher perioperative risk.[2][3][16] | Coordinate congenital cardiology, anesthesia, surgery, and postoperative disposition before elective surgery.[18][19] |
| Single-ventricle or Fontan physiology | Pulmonary blood flow is nontraditional and may be vulnerable to increased intrathoracic pressure and impaired venous return.[2][15] | Plan to preserve venous return and pulmonary blood flow; use an experienced congenital anesthesia team and monitored postoperative setting.[15][18] |
| Pulmonary hypertension, cyanosis, or ventricular dysfunction | These conditions increase perioperative complication risk and can produce rapid hemodynamic deterioration.[2][3][18] | Define baseline saturation and ventricular status, avoid triggers for pulmonary vascular constriction, and arrange escalation capability.[4][9][18] |

## Identify the lesion-specific drivers of perioperative events

Assess cardiac physiology and procedural stress together.

In a Boston Children's Hospital analysis of 3,010 patients with congenital heart disease undergoing noncardiac surgery between 2008 and 2013, cardiovascular events occurred in 11.5% and respiratory events in 4.7%. Cardiovascular events were associated with ASA physical status at least 3, emergency surgery, major or severe congenital heart disease, single-ventricle physiology, ventricular dysfunction on preoperative echocardiography, and surgical specialty.[2][3]

Use lesion complexity as a first screen but then identify the dominant active problem: residual shunt, ventricular failure, pulmonary vascular disease, obstructive lesion, rhythm/device dependence, or abnormal preload pathway. Pediatric risk frameworks have classified repaired atrial or ventricular septal defects and mild single-valve stenosis or regurgitation as lower risk; unrepaired simple lesions, repaired complex lesions, prior single-ventricle physiology, transplantation, pacemaker dependence, Wolff-Parkinson-White syndrome, and long-QT syndrome as higher-concern categories requiring individualized planning.[2][3]

Apply the 2024 ACC/AHA perioperative cardiovascular guideline to adult noncardiac-surgery decisions while retaining congenital anatomy and physiology as separate considerations; that guideline supersedes the 2014 ACC/AHA perioperative guideline.[14] Standard adult risk tools do not replace congenital-focused review in patients with residual shunts, pulmonary hypertension, cyanosis, or palliated circulations.[16][18]
- Document baseline oxygen saturation and hemoglobin concentration in cyanotic or shunt physiology, because both inform interpretation of perioperative oxygenation and bleeding tolerance.[5]
- Review the latest echocardiogram for systemic and subpulmonic ventricular function, valve lesions, shunt direction, and estimated pulmonary pressures; ventricular dysfunction was a risk-associated feature in pediatric noncardiac surgery.[2][3]
- Obtain an ECG and review arrhythmia history, implanted device dependence, and antiarrhythmic therapy in patients with prior atrial surgery, channelopathy, pacemaker dependence, or palpitations/syncope.[2][3]
- Match monitoring and postoperative level of care to the highest-risk interaction between patient physiology and procedure, especially emergency, orthopedic, general, neurosurgical, or pulmonary procedures.[2][3]

*Physiology-based preoperative branching for congenital heart disease.[2][4][9][13][18]*

| Dominant problem | Preoperative discriminator | Perioperative priority |
| --- | --- | --- |
| Residual or unrepaired shunt | Define shunt direction, resting saturation, ventricular loading, and susceptibility to systemic hypotension.[4][18] | Avoid hemodynamic changes that increase right-to-left shunting; promptly correct systemic hypotension and acidosis.[4] |
| Pulmonary hypertension or right ventricular dysfunction | Review ventricular function and pulmonary hemodynamics; identify hypoxemia, acidosis, or ventilatory vulnerability.[4][9][18] | Maintain preload and right ventricular contractility while minimizing pulmonary vascular resistance.[9] |
| Single-ventricle or Fontan circulation | Identify prior Fontan-type pathway, baseline saturation, venous congestion, effusions, ascites, and respiratory reserve.[15] | Protect venous return and pulmonary blood flow; avoid circumstances that substantially raise intrathoracic pressure.[15] |
| Arrhythmia or device dependence | Review ECG, device status, prior tachyarrhythmia, and current beta-blocker or antiarrhythmic treatment.[2][3][13] | Maintain continuity of rhythm-directed therapy when feasible and create a device-specific intraoperative plan.[13] |

## Translate congenital physiology into intraoperative hemodynamic goals

Choose techniques and monitoring that preserve the circulation's limiting variable.

There is no single anesthetic technique for congenital heart disease because risk arises from the interaction of defect complexity, residual physiology, and the noncardiac procedure.[8] Before induction, specify the target preload, systemic vascular resistance, pulmonary vascular resistance, ventricular contractility, rhythm, ventilation strategy, vascular-access plan, rescue drugs, and triggers for transesophageal echocardiography or higher-acuity postoperative care.[18]

For pulmonary hypertension or vulnerable right ventricular physiology, maintain adequate preload, minimize pulmonary vascular resistance, and optimize right ventricular contractility.[9] Avoid hypoxia and acidosis, which can worsen pulmonary pressures; in severe pulmonary hypertension with an intracardiac shunt, systemic hypotension can increase right-to-left shunting, promote acidosis, and create a self-reinforcing fall in systemic vascular resistance.[4]

For Fontan-type circulation, pulmonary blood flow depends on systemic venous flow reaching the lungs without passage through a subpulmonic ventricle.[15] Coughing and increased intrathoracic pressure may impair forward flow; plan emergence and ventilation to limit major pressure-related interruption of pulmonary blood flow, particularly when cyanosis, pulmonary disease, effusions, or ascites reduce reserve.[15]
- Continue beta-blockers and pulmonary antihypertensive medications perioperatively when feasible; do not create avoidable withdrawal in patients dependent on rhythm suppression or pulmonary vasodilation.[13]
- Use invasive or advanced monitoring selectively for complex physiology, major surgery, hemodynamic instability, or when beat-to-beat pressure measurement and rapid blood sampling will change management; transesophageal echocardiography may help monitor adults with congenital heart disease during noncardiac surgery.[18]
- Treat central venous access as anatomy-dependent rather than routine: prior vessel catheterization, venous occlusion, shunts, and complex repairs can make line placement difficult or hazardous.[18]
- Use anxiolytic or hypnotic premedication cautiously, particularly when sedation could compromise ventilation or hemodynamics in advanced congenital physiology.[18]

### Pulmonary vascular disease and right-to-left shunting

When pulmonary vascular disease coexists with a cardiac shunt, prioritize immediate correction of systemic hypotension, hypoxemia, and acidosis because each can augment right-to-left shunting or pulmonary vasoconstriction.[4] Persistent instability should prompt reassessment of ventilation, volume status, ventricular function, arrhythmia, bleeding, and the need for congenital-cardiology or cardiac-anesthesia rescue support.[9][18]
- Do not assume a postoperative low oxygen saturation is purely respiratory in a patient with shunt physiology; compare with the documented baseline and assess for a change in shunt fraction or pulmonary vascular tone.[4][5]
- Avoid avoidable interruption of established pulmonary hypertension therapy during transitions between preoperative, intraoperative, and recovery settings.[13]

### Antibiotic prophylaxis

Do not extend infective endocarditis prophylaxis to every congenital lesion or procedure. The AHA approach cited for congenital heart disease limits prophylaxis to cardiac defects or conditions associated with high risk for fatal outcomes; verify the patient-specific indication and planned procedure rather than using diagnosis alone.[18]

*Intraoperative priorities by circulation.[4][9][15][18]*

| Physiology | Avoid | Protect or monitor |
| --- | --- | --- |
| Pulmonary hypertension/right ventricular dysfunction | Hypoxia, acidosis, inadequate preload, and increases in pulmonary vascular resistance.[4][9] | Preload, right ventricular contractility, pulmonary vascular conditions, and rapid identification of hemodynamic compromise.[9][18] |
| Right-to-left intracardiac shunt | Systemic hypotension and acidosis, which can worsen shunting.[4] | Systemic perfusion pressure, baseline and changing oxygen saturation, and acid-base status.[4][5] |
| Fontan circulation | Marked increases in intrathoracic pressure and conditions that impair systemic venous return.[15] | Venous return, pulmonary blood flow, ventilation, and emergence conditions that may provoke coughing or pressure swings.[15] |
| Complex repaired anatomy | Assuming standard vascular access or monitoring is feasible without reviewing prior procedures.[18] | Prior operative and catheterization history; use anatomy-appropriate vascular access and consider transesophageal echocardiography when it will guide management.[18] |

## Plan postoperative surveillance before the procedure begins

The safest recovery location depends on the anticipated failure mode.

Determine whether the patient needs routine recovery, monitored observation, or intensive care before surgery. Cardiovascular events in pediatric congenital heart disease cohorts were frequently identified by inotrope use; 99% of cardiovascular events in one analysis were associated with inotrope administration, emphasizing the need for an environment able to recognize and treat evolving hemodynamic compromise.[2][3]

Use a monitored setting for patients with single-ventricle physiology, ventricular dysfunction, pulmonary hypertension, cyanosis, significant residual lesions, major surgery, or an unstable intraoperative course. Surveillance should target hemodynamics, oxygen saturation relative to baseline, ventilation, rhythm, perfusion, and signs of worsening ventricular or pulmonary vascular failure.[2][3][18]

After an unexpected saturation decline, hypotension, or difficult extubation, reassess congenital physiology rather than treating the event as routine postoperative respiratory disease. In Fontan physiology, increased intrathoracic pressure, cyanosis, pulmonary effusion, chronic lung disease, and ascites may interact to impair ventilation and cardiovascular performance.[15]
- Resume essential chronic cardiovascular therapy promptly when enteral administration is feasible, with special attention to beta-blockers and pulmonary hypertension medications.[13]
- Escalate persistent hypotension, acidosis, rising oxygen requirement above baseline, new arrhythmia, oliguria, congestion, or inability to liberate from positive-pressure ventilation to congenital cardiology and critical care assessment.[4][9][15][18]
- Document postoperative status against preoperative baseline saturation, hemoglobin, ventricular function, and rhythm history rather than applying a generic normal-range interpretation to cyanotic or palliated patients.[5][18]

*Postoperative findings that should prompt physiology-directed reassessment.[4][9][15][18]*

| Finding | Congenital physiology to reconsider | Next action |
| --- | --- | --- |
| Hypotension with desaturation in shunt physiology | Increased right-to-left shunting and reduced systemic vascular resistance.[4] | Correct systemic hypotension, assess acid-base status and ventilation, and obtain expert congenital hemodynamic support.[4][18] |
| Persistent hypoxemia or ventilatory difficulty after Fontan surgery history | Impaired pulmonary blood flow from elevated intrathoracic pressure or reduced venous return; assess concurrent pulmonary disease, effusion, or ascites.[15] | Optimize ventilation and hemodynamics, compare with baseline saturation, and escalate to congenital cardiac critical care when instability persists.[15][18] |
| Hemodynamic deterioration in pulmonary hypertension/right ventricular dysfunction | Acute rise in pulmonary vascular resistance or failing right ventricular compensation.[9] | Correct hypoxemia and acidosis, reassess preload and ventricular function, and continue pulmonary hypertension therapy when feasible.[9][13] |
| New tachyarrhythmia or bradyarrhythmia | Prior atrial surgery, channelopathy, or pacemaker dependence.[2][3] | Obtain ECG/device assessment and restore the patient-specific rhythm-management strategy.[13][18] |

## References
1. Progesterone for Neurodevelopment in Fetuses With ... — jamanetwork.com — https://jamanetwork.com/journals/jamanetworkopen/fullarticle/2819069
2. Perioperative Considerations for Pediatric Patients With Congenital Heart Disease Presenting for Noncardiac Procedures: A Scientific Statement From the American Heart Association | Circulation: Cardiovascular Quality and Outcomes — www.ahajournals.org — https://www.ahajournals.org/doi/abs/10.1161/HCQ.0000000000000113
3. Perioperative Considerations for Pediatric Patients With Congenital Heart Disease Presenting for Noncardiac Procedures: A Scientific Statement From the American Heart Association — www.ahajournals.org — https://www.ahajournals.org/doi/abs/10.1161/HCQ.0000000000000113?af=R
4. ACC/AHA 2007 Guidelines on Perioperative ... — www.ahajournals.org — https://www.ahajournals.org/doi/10.1161/circulationaha.107.185699
5. 2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... — www.ahajournals.org — https://www.ahajournals.org/doi/10.1161/CIR.0000000000001402
6. Essentials of Cardiac Anesthesia for Noncardiac Surgery | ScienceDirect — www.sciencedirect.com — https://www.sciencedirect.com/book/9780323567169/essentials-of-cardiac-anesthesia-for-noncardiac-surgery
7. Preoperative cardiac risk assessment for noncardiac surgery - ScienceDirect — www.sciencedirect.com — https://www.sciencedirect.com/science/article/pii/S0002914999804019
8. Congenital heart diseases and anaesthesia : Indian Journal of Anaesthesia — journals.lww.com — https://journals.lww.com/ijaweb/_layouts/15/oaks.journals/downloadpdf.aspx?an=01762628-201761090-00008
9. Adult congenital heart disease and anesthesia: An ... — onlinelibrary.wiley.com — https://onlinelibrary.wiley.com/doi/10.1111/pan.13982
10. Anesthesia for Congenital Heart Disease: Index — onlinelibrary.wiley.com — https://onlinelibrary.wiley.com/doi/chapter-epub/10.1002/9781119791690.index
11. Pulmonary arterial hypertension in congenital heart disease — onlinelibrary.wiley.com — https://onlinelibrary.wiley.com/doi/full/10.1002/cce2.74
12. Anaesthetic considerations in children with congenital ... — journals.lww.com — https://journals.lww.com/ijaweb/_layouts/15/oaks.journals/downloadpdf.aspx?an=01762628-201256050-00009
13. Error traps in patients with congenital heart disease ... — onlinelibrary.wiley.com — https://onlinelibrary.wiley.com/doi/10.1111/pan.14971
14. 2024 AHA/ACC/ACS/ASNC/HRS/SCA/SCCT/SCMR/SVM... — journals.lww.com — https://journals.lww.com/10.1161/CIR.0000000000001285
15. Abstracts From the 52nd Annual Meeting of the... : Journal of Neurosurgical Anesthesiology — journals.lww.com — https://journals.lww.com/jnsa/fulltext/2025/01000/abstracts_from_the_52nd_annual_meeting_of_the.14.aspx
16. Perioperative Risk in Adults with Congenital Heart Disease Undergoing Non-Cardiac Surgery: Challenges and Tailored Strategies — pmc.ncbi.nlm.nih.gov — https://pmc.ncbi.nlm.nih.gov/articles/PMC12112455
17. Perioperative Outcomes of Noncardiac Surgical and ... - PMC — pmc.ncbi.nlm.nih.gov — https://pmc.ncbi.nlm.nih.gov/articles/PMC13361643
18. Perioperative Management of Patients With Congenital Heart Disease - StatPearls - NCBI Bookshelf — www.ncbi.nlm.nih.gov — https://www.ncbi.nlm.nih.gov/books/NBK585103
19. Decentralization of Care for Adults with Congenital Heart ... — pmc.ncbi.nlm.nih.gov — https://pmc.ncbi.nlm.nih.gov/articles/PMC4172423
20. How Should We Care for Patients with Congenital Heart ... — pmc.ncbi.nlm.nih.gov — https://pmc.ncbi.nlm.nih.gov/articles/PMC8920386
21. Document Type: Study Protocol Official Title — cdn.clinicaltrials.gov — https://cdn.clinicaltrials.gov/large-docs/16/NCT02504216/Prot_001.pdf
22. Index of Suspicion | Pediatrics In Review — pedsinreview.aappublications.org — https://pedsinreview.aappublications.org/content/32/6/257
23. A Scientific Statement From the American Heart Association — pubmed.ncbi.nlm.nih.gov — https://pubmed.ncbi.nlm.nih.gov/36519439
24. Title: Pupillary Unrest as an Indicator of Central Opioid Effect in ... — cdn.clinicaltrials.gov — https://cdn.clinicaltrials.gov/large-docs/55/NCT05391555/Prot_SAP_000.pdf

## Editorial note

Prepared from cited clinical literature using Astra's research workflow. Verify recommendations against current guidance and patient-specific factors.