Anesthetic considerations for laparoscopic surgery in patients with Fontan circulation

A perioperative guide for anesthesia providers: passive pulmonary blood flow, what pneumoperitoneum does to it, ventilation and CO₂, monitoring, and where patients with congenital heart disease should have surgery.

Published October 16, 2026 · Last reviewed October 16, 2026 · Written by Anastasia Jackson, DNAP, CRNA

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What the Fontan circulation is

The Fontan operation is the final stage of palliation for a single functional ventricle. The caval veins are connected to the pulmonary arteries without a pumping ventricle, so blood reaches the lungs only by the pressure difference between the systemic veins and the common atrium.¹ Two features follow from that design, elevated systemic venous pressure and chronically decreased output, and they are the root cause of most long-term problems.¹ The pulmonary vascular bed acts as a bottleneck, with obligatory congestion upstream and decreased flow downstream.²

Today's operation is a total cavopulmonary connection, built either as a lateral tunnel or as an extracardiac conduit; older atriopulmonary connections have been abandoned but are still seen in adults.¹ Many patients have a fenestration, a deliberate small right-to-left shunt from the Fontan pathway to the atrium. It augments ventricular preload and partially offloads systemic venous pressure, at the cost of some desaturation.¹ Without a fenestration, a rise in pulmonary vascular resistance can directly decrease ventricular preload and systemic perfusion.³

What the circulation depends on

• Preload. Pulmonary blood flow, and therefore cardiac output, is exquisitely sensitive to hypovolemia and changes in vascular tone.³ Fontan patients have reduced splanchnic venous capacitance, which makes them sensitive to acute volume shifts.⁴
• Low pulmonary vascular resistance. Resistance is the main determinant of output; increasing contractility does not raise resting output.² Loss of pulsatile flow impairs endothelial nitric oxide release, which promotes pulmonary vascular remodeling and higher resistance over time.⁴
• Low intrathoracic pressure. Without a subpulmonary pump, the skeletal and respiratory muscle pumps matter more for preload, and inspiration (the "thoracic bellows") drives venous return.¹
• Rhythm and ventricular function. The chronically underfilled ventricle develops both systolic and diastolic dysfunction, largely as a secondary effect.² Chronotropic incompetence has been reported in 62% of patients, the highest prevalence among adults with congenital heart disease.¹ Atrioventricular valve regurgitation adds further stress.¹

Any obstruction anywhere in this series circuit reduces cardiac output more than it would in a two-ventricle circulation.¹

Baseline numbers worth knowing

No validated cutoff for an elevated Fontan pressure exists: one adult catheterization laboratory treats 15 mmHg or more as abnormal, a threshold tied to worse outcomes in one study, and others have linked a central venous pressure of 14 mmHg or more to worse outcomes in late survivors.⁵⁸

A central venous catheter in a Fontan patient terminates in the pulmonary artery, so the pressure it reads is the Fontan (pulmonary artery) pressure, not right atrial pressure.⁴

Late complications that change the anesthetic

• Fontan failure. Heart failure symptoms can occur despite reasonable ventricular function, simply as a consequence of venous hypertension.¹ Admission with heart failure carries a poor prognosis.¹
• Arrhythmias. In studies with more than 5 years of follow-up, supraventricular tachycardia was reported in 60% to 80% of patients after atriopulmonary repair and 5% to 15% after an extracardiac conduit.¹ Intra-atrial re-entrant tachycardia is most common, and the ventricular rate may be deceptively normal and mistaken for sinus rhythm.⁵ Arrhythmias that are usually well tolerated can destabilize a Fontan patient.⁵ Pacing is usually epicardial because transvenous leads carry a risk of systemic thromboembolism.⁵ For new-onset atrial flutter or fibrillation, the 2025 guideline recommends timely pharmacological or electrical cardioversion to prevent decompensation (Class 1).⁵⁰
• Fontan-associated liver disease. Liver fibrosis and renal dysfunction are common and may progress.¹ INR may rise and platelet count fall as portal pressure increases.¹
• Protein-losing enteropathy (5% to 12% after Fontan palliation) and plastic bronchitis (under 5%), in which bronchial casts can obstruct the airway and cause severe hypoxemia.¹
• Thrombosis and anticoagulation. Thrombosis is common, driven mainly by stasis; prolonged immobilization is a reported risk factor.¹ The 2025 guideline recommends anticoagulation for adults with a known or suspected thrombus, an atriopulmonary Fontan, prior thromboembolism or sustained atrial flutter or fibrillation, and either aspirin or anticoagulation for the rest (both Class 1); small studies show comparable results for warfarin and non-vitamin K oral anticoagulants.⁵⁰ Rivaroxaban is FDA-labeled for thromboprophylaxis in children aged 2 years and older with congenital heart disease who have undergone the Fontan procedure.⁶¹ Most patients therefore arrive on an antiplatelet or anticoagulant.
• Collaterals. Venovenous collaterals act as a decompressive pathway and cause cyanosis; aortopulmonary collateral flow is increased.^1,5

Preoperative assessment and where to operate

• Review exercise tolerance, existing Fontan complications, medications, and any recent respiratory illness, which can raise airway resistance and pulmonary vascular resistance.⁴
• Prior echocardiograms and cardiac catheterization data are essential to assess cardiac function, pathway patency and fenestration status.⁴
• Obtain baseline hematologic and biochemical tests, even before minor surgery, and assess renal function in older patients.^1,4
• Ask about prior airway complications such as subglottic stenosis or vocal cord paralysis from earlier prolonged intubations.⁴
• Do not abruptly withdraw long-term pulmonary vasodilators.⁴ Plan management of pacemakers and defibrillators, and remember that diuretics can cause dehydration and electrolyte imbalance.⁴
• Any deviation from the patient's baseline is a red flag for worsening Fontan function.⁵

The 2025 ACC/AHA guideline makes three Class 1 recommendations for noncardiac procedures:⁵⁰

• Preprocedural involvement of an adult congenital cardiologist for every adult with congenital heart disease, to risk-stratify, choose the procedure location, plan periprocedural support and optimize the patient (level of evidence B-NR).
• Postprocedural care in collaboration with an adult congenital cardiologist for higher-risk patients, which includes every Fontan patient (B-NR).
• Anesthesia "administered by, or in collaboration or consultation with, an anesthesiologist with ACHD expertise" for higher-risk patients, again including every Fontan patient (C-LD).

Its supporting text adds that patients with complex disease should, when possible, have evaluation and surgery at a center with an adult congenital program, and that careful planning with an adult congenital physician may be the best alternative.⁵⁰ A perioperative review of Fontan patients likewise recommends elective surgery in an adult congenital center when possible.¹⁰ The 2026 perioperative guideline lists single-ventricle and post-Fontan patients as elevated risk and recommends preoperative consultation with an adult congenital specialist before elective surgery (Class 1).⁵¹ Up to 74% of noncardiac surgeries in adults with congenital heart disease take place at non-specialist centers.⁵²

Scale the plan to the patient's reserve. In the outcome series, risk concentrated in patients with baseline cyanosis and poor ventricular function: cyanosis was the only independent risk factor for complications in one large series, and the two Mayo patients whose complications did not resolve had ejection fractions of 22% and 28%.^7,8 The Mayo authors suggested a tertiary institution may be more appropriate, particularly when ejection fraction is below 30%.⁷ Physiologic reserve varies widely, so each plan has to be tailored to the patient.^15,16

Why laparoscopy is the hard case

The 2018 AHA/ACC guideline singles it out: "The application of anesthesia for laparoscopic procedures can be especially challenging in Fontan patients," because "abdominal insufflation may lead to lower preload and hypotension, while at the same time elevating systemic vascular resistance."⁶ Several mechanisms act together:

• Venous return. Insufflation, reverse Trendelenburg positioning and positive-pressure ventilation can each compromise venous return.¹⁸ Raised intra-abdominal pressure can compress the inferior vena cava and splanchnic vasculature.²²
• Pulmonary vascular resistance. Absorbed CO₂ raises it, and in a fenestrated patient that diverts desaturated blood into the systemic circulation.²⁰ Diaphragm elevation and atelectasis add to it.²³ Without a subpulmonary ventricle, a rise in resistance lowers pulmonary blood flow unless venous pressure rises to compensate.¹⁸
• Intrathoracic pressure. Pneumoperitoneum lowers lung compliance and functional residual capacity and raises airway pressure, which reduces pulmonary blood flow.²⁰
• Position. Reverse Trendelenburg can worsen hypotension by reducing venous return, while Trendelenburg may transiently improve it.²² Trendelenburg also raises the diaphragm and increases atelectasis, which can raise pulmonary vascular resistance.²³
• Desufflation. An abrupt fall in intra-abdominal pressure can pool volume in capacitance vessels. In one case, systolic pressure fell from 120 to 70 mmHg after exsufflation.²⁶

In general surgical populations, the hemodynamic effects of laparoscopy are accentuated in patients with pulmonary hypertension and congenital heart disease, and the fall in splanchnic perfusion is proportional to the intra-abdominal pressure and the duration of pneumoperitoneum.^32,33 An AHA scientific statement notes that intra-abdominal pressures below 15 mmHg do not appear to affect cardiac performance in a two-ventricle circulation, but a similar threshold for Fontan physiology is not known.⁵² The 2026 perioperative guideline advises avoiding increased intra-abdominal pressure, hypothermia, hypercapnia, metabolic acidosis and hypovolemia in patients with a prior Fontan to maintain optimal pulmonary vascular resistance.⁵¹

What the published cases show

The evidence is thin. Fontan laparoscopy is described in roughly twenty single case reports and small series, one national database study, and no prospective study measuring Fontan pressures during insufflation. Nearly every report describes success, so publication bias is likely. In the National Inpatient Sample study, 42% of Fontan patients older than five years who had abdominal surgery had it laparoscopically, with no mortality difference between laparoscopic and open approaches (odds ratio 1.4, 95% CI 0.2 to 21.3).³⁰ In children, the outcome advantage of laparoscopy over open surgery was seen with minor congenital heart disease but not with more severe disease.⁴⁹

Earlier reports described one patient through two separate laparoscopic procedures and two pediatric post-Fontan cases.^35,36 In infants with palliated hypoplastic left heart syndrome undergoing laparoscopic fundoplication, insufflation limited to 12 mmHg was well tolerated.³⁴ In single-ventricle infants studied with transesophageal echocardiography during laparoscopic gastrostomy, fractional shortening fell during insufflation and recovered after desufflation.⁴⁸

Intraoperative management

Insufflation

Insufflate slowly and keep intra-abdominal pressure as low as possible, ideally below 10 to 12 mmHg, ready to convert if the circulation does not tolerate it.²² Pans and colleagues advise keeping intra-abdominal pressure below the central venous pressure and monitoring venous pressure continuously.¹⁸ The AHA statement on noncardiac complications notes that case reports show laparoscopy can be performed with acceptable risk at intra-abdominal pressures below 10 mmHg, and other summaries of the case literature describe success below 12 mmHg.^21,24,52 Several teams held pressure at or below 8 mmHg, and low-pressure insufflation systems have been used to keep it low and stable.^21,27,28 No report of gasless laparoscopy in a Fontan patient was found.

Ventilation

The goal is the lowest mean intrathoracic pressure that still prevents hypercarbia, hypoxemia and atelectasis, all of which raise pulmonary vascular resistance.^4,6

• Low airway pressure is desirable because prolonged high intrathoracic pressure decreases cardiac output in a Fontan circulation, and PEEP should be minimized.⁵² Case authors advise low tidal volumes, minimal or no PEEP, and normocapnia.²² One review suggests starting at a tidal volume of 5 to 6 mL/kg with a respiratory rate of no more than 20 breaths per minute, a short inspiratory time, and no excessive PEEP.⁴
• Tidal volume itself may matter less than once thought. In 11 children ventilated at the end of cardiac surgery, 5 versus 10 mL/kg made no significant difference to transpulmonary gradient, pulmonary blood flow or stroke volume, although peak airway pressure was lower at 5 mL/kg.⁵³ In 30 Fontan patients at cardiac catheterization, cardiac index did not differ significantly between 6 and 10 mL/kg (3.19 vs 2.96 L/min/m², p = 0.062); the authors concluded that a lower tidal volume may be favorable for some patients.⁵⁴
• Authors disagree on PEEP. Teams used PEEP between 0 and 6 cmH₂O; one used PEEP 6 in an obese patient because hypoxemia also raises resistance, and another warned that even mild hyperventilation can harm the Fontan circulation by raising airway pressure.^19,21,22,24
• CO₂ keeps accumulating with insufflation time, so minute ventilation needs serial adjustment, and PaCO₂ can keep rising after insufflation ends.^21,24

CO₂ monitoring

Preload and position

• Coordinate fasting to avoid dehydration, but avoid aggressive volume loading because diastolic dysfunction is possible.⁴ Reported cases used prehydration plus intraoperative boluses.^18,20,22,24
• For hypotension, a vasopressor may be preferable to large-volume resuscitation.⁴
• Limit the degree of reverse Trendelenburg and avoid steep tilt combined with high intra-abdominal pressure.^20,24 If hypotension develops during positioning or insufflation, leveling the bed and releasing the pneumoperitoneum is a reported rescue.²⁴
• Anticipate hypotension at desufflation.²⁶

Monitoring

• A pre-induction arterial line helps detect early hemodynamic changes, and intraoperative arterial monitoring is likely appropriate for most cases.^4,7
• A central line reads Fontan (pulmonary artery) pressure; weigh its value against thrombosis risk. One team avoided central access for that reason, together with endocarditis risk.^4,22
• Transesophageal echocardiography is valuable for assessing preload and ventricular function and can show conduit and fenestration flow in real time.^4,20 Cases have also used transpulmonary thermodilution to decide when to convert, and the SpO₂–ScvO₂ gap to track shunting.^19,23

Pulmonary vasodilators and vasoactive drugs

Inhaled nitric oxide lowers pulmonary vascular resistance late after Fontan-type operations.¹³ In 30 adults at catheterization it lowered pulmonary artery pressure, transpulmonary gradient and pulmonary vascular resistance without raising wedge pressure, although cardiac index did not change and pulmonary vasodilation can worsen filling pressures in some patients.¹⁴ Case reports used inhaled nitric oxide during pneumoperitoneum or as a rescue, and inhaled or intravenous milrinone.^19,20,21,25 In patients with Fontan cirrhosis, drugs that lower systemic vascular resistance, such as milrinone or dobutamine, should be used with caution.¹ Intraoperative inotrope use was associated with lower odds of same-day discharge.⁹

Air

Avoid air in intravenous lines, and use air filters in patients with cyanosis or a fenestration.⁴ Transesophageal echocardiography can detect gas embolism, with a risk of paradoxical embolism through a fenestration.²⁵

When to stop

Several teams planned early conversion to open surgery if the circulation deteriorated.^19,20,29 The only explicit criteria found came from the failing-Fontan case: any fall in cerebral oximetry of more than 20%, urine output below 0.5 mL/kg/h, or lactate above 2 mmol/L, while keeping mean arterial pressure at least 40 mmHg above mean pulmonary artery pressure.²⁵ These are one team's criteria, not validated thresholds.

Induction, regional anesthesia and postoperative care

• Induction. No induction agent is preferred; a relatively large, predetermined bolus can precipitate instability. Light anesthesia can trigger a catecholamine surge that raises pulmonary vascular resistance.⁴
• Spontaneous ventilation. For short procedures, Fontan patients typically benefit from spontaneous ventilation, provided hypercarbia and hypoxemia are prevented.⁴ After Glenn or Fontan surgery, resuming pain-free spontaneous breathing lowered pulmonary artery pressure and raised cardiac index.¹¹ In early postoperative and convalescent Fontan patients, a brief period of negative-pressure ventilation raised pulmonary blood flow by a mean of 42%.¹² The effect is smaller in awake adults years after Fontan: negative-pressure ventilation raised pulmonary blood flow by 8%, and biphasic ventilation by 25% with a 16% rise in cardiac output.⁵⁵ The response varies between patients: in 10 spontaneously breathing patients with a failing Fontan, negative extrathoracic pressure lowered Fontan pressure in five but raised it by 2 mmHg in three.⁶² The 2025 guideline describes early extubation as "of particular importance in patients with Fontan physiology" because of the deleterious effects of positive-pressure ventilation on the transpulmonary gradient and ventricular preload.⁵⁰
• Regional anesthesia. Peripheral nerve and abdominal wall blocks avoid hemodynamic disturbance and airway risk and provide opioid-sparing analgesia.^4,22 Neuraxial block can reduce preload through sympathectomy; the AHA statement says neuraxial techniques can be safe in Fontan physiology, and with careful titration, fluid loading and monitoring they have been reported safe, although the cohort data are obstetric.^4,17,52 Most patients take antiplatelet or anticoagulant therapy, so bleeding and thrombotic risk must be weighed for any neuraxial technique, and loss of resistance should use saline rather than air.⁴
• After surgery. Good analgesia limits catecholamine release and the rise in pulmonary vascular resistance; antiemetic prophylaxis supports early oral intake and prevents dehydration; many patients need continuous monitoring for at least the first 24 hours.⁴ Overnight admission is likely appropriate for most adults, though well-selected patients have gone home the same day.^7,9 Postoperative respiratory deterioration occurred in the failing-Fontan child.²⁵

Outcomes of noncardiac surgery after Fontan

• In 39 general anesthetics in 31 adults at Mayo Clinic, complications occurred in 31%, with one postoperative death; the patients whose complications did not resolve had ejection fractions of 22% and 28%.⁷
• In 538 procedures in 154 adults, complications occurred in 15%, most often hypoxia; baseline cyanosis was the only independent risk factor, and complications were more common than in matched biventricular congenital heart disease or no heart disease (18% vs 5% vs 1.4%).⁸
• In a single-center series of 45 interventions in 26 patients there were three bleeding complications and one death.⁴⁷
• In children and young adults, major surgery, intraoperative inotropes, more chronic conditions and preoperative admission each independently lowered the odds of same-day discharge.⁹

Congenital heart disease more broadly

An estimated 2.4 million people were living with congenital heart disease in the United States in 2010, 1.4 million of them adults, and nearly 300,000 had severe disease.³⁷ In Quebec, adults made up 66% of the congenital heart disease population by 2010.³⁸ About 90% of children diagnosed with severe disease are now expected to reach age 18, and repair in childhood is not a cure: almost all adults have sequelae.⁶

A practical framework

Adults with congenital heart disease sit on a continuum: some defects were never corrected, some were palliated (such as cavopulmonary connections), and some had complete anatomic correction.³⁹ In nearly all cases, the condition should be viewed as systemic, with multiorgan involvement.³⁹ The 2018 AHA/ACC guideline classifies each patient by anatomy plus physiological stage.⁶

Physiological stages run from A to D; stage D includes severe hypoxemia, severe pulmonary hypertension, Eisenmenger syndrome and refractory arrhythmias.⁶ The 2025 guideline keeps this system and notes that a Fontan patient can never be in physiological stage A.⁵⁰ The guideline defines hypoxemia as a resting SpO₂ of 90% or less and severe hypoxemia as below 85%.⁶

Shunts, cyanosis and Eisenmenger physiology

• Long-standing left-to-right shunting can cause pulmonary vascular disease with reversed or bidirectional shunting (Eisenmenger syndrome); noncardiac surgery in Eisenmenger physiology should be performed only if absolutely essential.³⁹
• Cyanotic patients are at high risk of both perioperative bleeding and thrombosis, even during minor surgery.³⁹ Secondary erythrocytosis is a compensatory response, routine phlebotomy is not supported, and coagulation sample tubes need adjusted anticoagulant volume when hematocrit exceeds 55%.⁶
• De-air all intravenous lines in patients with intracardiac shunts and consider air filters to prevent paradoxical embolism.^6,39
• High airway pressures can compromise venous return, raise pulmonary vascular resistance and worsen right-to-left shunting.³⁹ In cyanotic patients, avoid or use cautiously drugs that blunt the hypoxic drive to breathe, such as opioids.⁶
• Supraventricular arrhythmias occur in 20% to 45% of patients after atrial surgery and can cause rapid deterioration.³⁹ There are no evidence-based heart failure guidelines for a systemic right ventricle.³⁹
• Patient and family history may be incomplete: obtain old records, and expect abnormal venous or arterial anatomy that affects access.⁶

How risky is noncardiac surgery?

• Adults. In a national sample, adults with congenital heart disease had higher inpatient mortality after major noncardiac surgery than matched controls (4.1% vs 3.6%; adjusted odds ratio 1.29) and more morbidity (21.4% vs 16.0%).⁴⁰ Guideline risk factors include cyanosis, heart failure, poor general health, younger age, pulmonary hypertension, operations on the respiratory and nervous systems, complex disease, and urgent or emergency procedures.⁶ Surgery that is low risk in the general population may carry higher risk here, and general perioperative risk indices may not apply.⁶
• Children. Children with congenital heart disease had higher 30-day mortality after noncardiac surgery (odds ratio 3.5), for minor and major procedures alike, with the greatest excess in neonates and infants.⁴¹ In pediatric NSQIP data, mortality was higher with major (3.9%) and severe (8.2%) disease but not minor disease.⁴² Independent predictors of in-hospital mortality include emergency surgery, severe disease and single-ventricle physiology.⁴³ The 2023 AHA statement summarizes these patients as high risk for mortality, complications and reoperation.⁴⁴

Where surgery should happen

The 2025 ACC/AHA guideline, which replaces the 2018 guideline, recommends preprocedural involvement of an adult congenital cardiologist for every adult with congenital heart disease, postprocedural collaboration with one, and anesthesia by or with an anesthesiologist with adult congenital expertise for higher-risk patients (all Class 1).⁵⁰ Its supporting text advises evaluation and surgery at a center with an adult congenital program for patients with moderate or complex anatomy or significant residual disease, when possible.⁵⁰ The 2018 guideline had already noted that heightened postoperative surveillance may require intensive or other high-acuity care.⁶ For children, the 2023 AHA statement recommends specialized congenital cardiac centers, while noting there is no randomized evidence requiring a pediatric cardiac anesthesiologist for every procedure.⁴⁴

Endocarditis prophylaxis

The 2021 AHA statement made no changes to the 2007 recommendations.⁴⁵ Prophylaxis before qualifying dental procedures is suggested for unrepaired cyanotic congenital heart disease (including palliative shunts and conduits), completely repaired defects with prosthetic material or a device during the first 6 months, repaired defects with residual defects at or adjacent to prosthetic material, pulmonary artery valve or conduit placement, prosthetic valves, and previous endocarditis.⁴⁵ It is not suggested for completely closed septal closure devices or for pacemakers.⁴⁵ Antibiotics given solely to prevent endocarditis are not recommended for gastrointestinal or genitourinary procedures (Class III, level of evidence B).⁴⁶ This is being revisited. The 2023 ESC guideline now says systemic prophylaxis "may be considered" for high-risk patients undergoing invasive gastrointestinal, genitourinary, respiratory, skin or musculoskeletal procedures (Class IIb, level of evidence C, based on observational data), and an AHA science advisory has called for a reappraisal; current AHA guidance is unchanged.^59,60

A practical checklist

1. Before: recent echo and catheterization data, baseline saturation and Fontan pressure, rhythm and device status, anticoagulation plan, liver and renal function, hemoglobin. Involve the adult congenital heart disease team; operate at an experienced center when possible.
2. Fasting and fluids: avoid dehydration; give a measured prehydration; treat hypotension with a vasopressor rather than large volumes.
3. Lines: arterial line before induction; de-air every line and use air filters with a fenestration or cyanosis; decide on central access deliberately.
4. Induction: titrate, avoid large fixed boluses, and avoid light anesthesia.
5. Ventilation: low tidal volume, short inspiratory time, low PEEP, normal CO₂ confirmed by arterial blood gas.
6. Pneumoperitoneum: insufflate slowly to the lowest workable pressure; limit tilt; watch pressure, venous pressure and saturation through each change.
7. Rescue: level the bed, release the pneumoperitoneum, check a blood gas, consider inhaled nitric oxide, and convert early if the circulation does not recover.
8. Desufflation: expect a pressure drop.
9. After: multimodal and regional analgesia, antiemetic prophylaxis, early oral intake, monitored bed for at least the first night in most patients.

Frequently asked questions

Is laparoscopy contraindicated in a Fontan patient?

No. Many case reports describe successful laparoscopy, and a national database study found no mortality difference between laparoscopic and open surgery in Fontan patients.³⁰ The evidence is mostly case reports, so laparoscopy should be planned with an experienced team and a low threshold to convert.

What insufflation pressure is safe?

No pressure has been validated, and the AHA notes that the threshold for Fontan physiology is not known.⁵² Reported pressures ranged from 6 to 15 mmHg, and case authors describe success below 10 to 12 mmHg, ideally below the patient's central venous pressure.^18,22 Use the lowest pressure that gives the surgeon a workable view.

Why does positive-pressure ventilation matter so much?

Blood reaches the lungs only because systemic venous pressure exceeds pressure downstream. Positive intrathoracic pressure narrows that gradient. Positive-pressure ventilation is known to impair pulmonary blood flow in Fontan physiology, and spontaneous or negative-pressure breathing improves it.^11,12

Can I rely on end-tidal CO₂?

Not alone. End-tidal CO₂ underestimated arterial CO₂ substantially in reported cases, so serial arterial blood gases are needed during pneumoperitoneum.^25,31

What does the central venous pressure measure in a Fontan patient?

The catheter tip lies in the Fontan pathway, so it measures pulmonary artery (Fontan) pressure.⁴ Expected values are 10 to 15 mmHg.¹

Does a Fontan patient need endocarditis prophylaxis for abdominal surgery?

Under current AHA guidance, antibiotics given solely to prevent endocarditis are not recommended for gastrointestinal or genitourinary procedures, and the high-risk categories apply to dental procedures.^45,46 The 2023 ESC guideline allows prophylaxis to be considered for high-risk patients having invasive gastrointestinal procedures (Class IIb).⁵⁹ This is separate from routine surgical antibiotic prophylaxis.

Should a Fontan patient have surgery at a specialist center?

When possible, yes. The 2025 ACC/AHA guideline recommends involving an adult congenital cardiologist before the procedure and having anesthesia given by, or with, an anesthesiologist with adult congenital expertise, and its supporting text favors a center with an adult congenital program.⁵⁰ If the patient cannot be moved, careful planning in consultation with an adult congenital physician may be the best alternative.⁵⁰

References

1. Rychik J, Atz AM, Celermajer DS, et al. Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association. Circulation. 2019;140(6):e234-e284. doi:10.1161/CIR.0000000000000696. PMID 31256636.
2. Gewillig M, Brown SC. The Fontan circulation after 45 years: update in physiology. Heart. 2016;102(14):1081-1086. doi:10.1136/heartjnl-2015-307467. PMID 27220691.
3. Greaney D, Honjo O, O'Leary JD. The single ventricle pathway in paediatrics for anaesthetists. BJA Educ. 2019;19(5):144-150. doi:10.1016/j.bjae.2019.01.008. PMID 33456883.
4. Maisat W, Yuki K. The Fontan circulation in pregnancy: hemodynamic challenges and anesthetic considerations. J Cardiothorac Vasc Anesth. 2024;38(11):2770-2782. doi:10.1053/j.jvca.2024.07.021. PMID 39097487.
5. Grewal J, Pula G, Wald RM, et al. Contemporary management and future directions of adults living with a Fontan circulation. JACC Adv. 2026;5(7):102885. doi:10.1016/j.jacadv.2026.102885. PMID 42489389.
6. Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019;139(14):e698-e800. doi:10.1161/CIR.0000000000000603. PMID 30586767.
7. Rabbitts JA, Groenewald CB, Mauermann WJ, et al. Outcomes of general anesthesia for noncardiac surgery in a series of patients with Fontan palliation. Paediatr Anaesth. 2013;23(2):180-187. doi:10.1111/pan.12020. PMID 22998356.
8. Egbe AC, Khan AR, Ammash NM, et al. Predictors of procedural complications in adult Fontan patients undergoing non-cardiac procedures. Heart. 2017;103(22):1813-1820. doi:10.1136/heartjnl-2016-311039. PMID 28501797.
9. Brown ML, Cradeur M, Staffa SJ, Nasr VG, Hernandez MR, DiNardo JA. Anaesthesia for non-cardiac surgery in children and young adults with Fontan physiology. Cardiol Young. 2023;33(10):1896-1901. doi:10.1017/S104795112200333X. PMID 36330834.
10. McNamara JR, McMahon A, Griffin M. Perioperative management of the Fontan patient for cardiac and noncardiac surgery. J Cardiothorac Vasc Anesth. 2022;36(1):275-285. doi:10.1053/j.jvca.2021.04.021. PMID 34023201.
11. Lofland GK. The enhancement of hemodynamic performance in Fontan circulation using pain free spontaneous ventilation. Eur J Cardiothorac Surg. 2001;20(1):114-118. doi:10.1016/s1010-7940(01)00757-6. PMID 11423283.
12. Shekerdemian LS, Bush A, Shore DF, Lincoln C, Redington AN. Cardiopulmonary interactions after Fontan operations: augmentation of cardiac output using negative pressure ventilation. Circulation. 1997;96(11):3934-3942. doi:10.1161/01.cir.96.11.3934. PMID 9403618.
13. Khambadkone S, Li J, de Leval MR, Cullen S, Deanfield JE, Redington AN. Basal pulmonary vascular resistance and nitric oxide responsiveness late after Fontan-type operation. Circulation. 2003;107(25):3204-3208. doi:10.1161/01.CIR.0000074210.49434.40. PMID 12821557.
14. Miranda WR, Jain CC, Frantz RP, et al. Effects of inhaled nitric oxide on Fontan hemodynamics in adults. J Heart Lung Transplant. 2023;42(10):1349-1352. doi:10.1016/j.healun.2023.04.015. PMID 37127071.
15. Neethling E, Heggie JE. Considerations in critical-care and anesthetic management of adult patients living with Fontan circulation. Can J Cardiol. 2022;38(7):1100-1110. doi:10.1016/j.cjca.2022.04.017. PMID 35490923.
16. Adler AC, Nathan AT. Perioperative considerations for the Fontan patient requiring noncardiac surgery. Anesthesiol Clin. 2020;38(3):531-543. doi:10.1016/j.anclin.2020.04.001. PMID 32792182.
17. Miyake T, Masuda S, Tsukinaga A, et al. Perioperative hemodynamics and clinical outcomes of cesarean delivery under neuraxial anesthesia in women with Fontan circulation: a single-center retrospective cohort study. J Cardiothorac Vasc Anesth. 2026;40(10):3260-3269. doi:10.1053/j.jvca.2026.07.004. PMID 42498615.
18. Pans SJ, van Kimmenade RR, Ruurda JP, et al. Haemodynamics in a patient with Fontan physiology undergoing laparoscopic cholecystectomy. Neth Heart J. 2015;23(7-8):383-385. doi:10.1007/s12471-015-0704-7. PMID 26031634.
19. Saito K, Toyama H, Saito M, Yamauchi M. Successive perioperative management of laparoscopic liver resection in the reverse Trendelenburg position for a patient with Fontan physiology: a case report. JA Clin Rep. 2021;7(1):56. doi:10.1186/s40981-021-00456-6. PMID 34258682.
20. Tankul R, Rodrigues B, Duggan LV. Laparoscopic cholecystectomy in a patient with Fontan circulation. Can J Anaesth. 2024;71(10):1417-1422. doi:10.1007/s12630-024-02833-y. PMID 39294432.
21. Takemoto S, Asada M, Maki J, Setoguchi H, Hoka S. Anesthetic management of an adult patient post a Fontan procedure in laparoscopic surgery: a case report. Cureus. 2024;16(5):e59594. doi:10.7759/cureus.59594. PMID 38826999.
22. Catarci S, Zanfini BA, Canistro G, et al. Anesthetic management of a 32-year-old woman undergoing laparoscopic gynecological surgery with prior Fontan repair for a complex congenital heart defect. Am J Case Rep. 2025;26:e950027. doi:10.12659/AJCR.950027. PMID 41472350.
23. Okano S, Kuroki M, Abe S, Matsuura Y, Narisawa A, Toyama H. Anesthesia management for laparoscopic surgery in the Trendelenburg position in a patient with Fontan physiology and severe hypoxemia: a case report. JA Clin Rep. 2025;11(1):53. doi:10.1186/s40981-025-00820-w. PMID 41023349.
24. Hyre N, Greib J, Rashid S, Hilmi I. Perioperative management of a patient with Fontan circulation presenting for laparoscopic cholecystectomy: a case report. Cureus. 2026;18(9):e115741. doi:10.7759/cureus.115741. PMID 42830748.
25. Anagnostopoulos A, Boegli Y, Perez M, de Buys Roessingh A, Di Bernardo S, Mauron S. Laparoscopic surgery in a child with a failing Fontan circulation. Anaesth Rep. 2023;11(1):e12217. doi:10.1002/anr3.12217. PMID 36875790.
26. Lee HC, Nam K, Lee JH, et al. Anesthetic management of laparoscopic pheochromocytoma excision in a patient with a Fontan circulation: a case report. Korean J Anesthesiol. 2014;66(3):252-255. doi:10.4097/kjae.2014.66.3.252. PMID 24729851.
27. Yokota Y, Noda T, Kobayashi S, et al. A case report of Fontan procedure-related hepatocellular carcinoma: pure laparoscopic approach by low and stable pneumoperitoneum. BMC Surg. 2020;20(1):80. doi:10.1186/s12893-020-00741-8. PMID 32316959.
28. Nohara-Shitama Y, Takahashi J, Homma T, Igawa T, Fukumoto Y. Successful laparoscopic pheochromocytoma resection in an adult patient with Fontan physiology: a case report. Eur Heart J Case Rep. 2022;6(11):ytac429. doi:10.1093/ehjcr/ytac429. PMID 36381171.
29. Tanaka Y, Sumie M, Hashimoto T, et al. Anesthetic management of pheochromocytoma and paraganglioma for patients with Fontan circulation: a case series. JA Clin Rep. 2023;9(1):13. doi:10.1186/s40981-023-00605-z. PMID 36897467.
30. Herrick NL, Bickler S, Maus T, et al. Laparoscopic surgery requiring abdominal insufflation in patients with congenital heart disease. J Cardiothorac Vasc Anesth. 2022;36(3):707-712. doi:10.1053/j.jvca.2021.05.005. PMID 34175203.
31. Wulkan ML, Vasudevan SA. Is end-tidal CO2 an accurate measure of arterial CO2 during laparoscopic procedures in children and neonates with cyanotic congenital heart disease? J Pediatr Surg. 2001;36(8):1234-1236. doi:10.1053/jpsu.2001.25775. PMID 11479864.
32. Gutt CN, Oniu T, Mehrabi A, et al. Circulatory and respiratory complications of carbon dioxide insufflation. Dig Surg. 2004;21(2):95-105. doi:10.1159/000077038. PMID 15010588.
33. Atkinson TM, Giraud GD, Togioka BM, Jones DB, Cigarroa JE. Cardiovascular and ventilatory consequences of laparoscopic surgery. Circulation. 2017;135(7):700-710. doi:10.1161/CIRCULATIONAHA.116.023262. PMID 28193800.
34. Mariano ER, Boltz MG, Albanese CT, Abrajano CT, Ramamoorthy C. Anesthetic management of infants with palliated hypoplastic left heart syndrome undergoing laparoscopic Nissen fundoplication. Anesth Analg. 2005;100(6):1631-1633. doi:10.1213/01.ANE.0000149899.03904.3F. PMID 15920186.
35. McClain CD, McGowan FX, Kovatsis PG. Laparoscopic surgery in a patient with Fontan physiology. Anesth Analg. 2006;103(4):856-858. doi:10.1213/01.ane.0000237294.88298.8e. PMID 17000793.
36. Taylor KL, Holtby H, Macpherson B. Laparoscopic surgery in the pediatric patient post Fontan procedure. Paediatr Anaesth. 2006;16(5):591-595. doi:10.1111/j.1460-9592.2005.01826.x. PMID 16677273.
37. Gilboa SM, Devine OJ, Kucik JE, et al. Congenital heart defects in the United States: estimating the magnitude of the affected population in 2010. Circulation. 2016;134(2):101-109. doi:10.1161/CIRCULATIONAHA.115.019307. PMID 27382105.
38. Marelli AJ, Ionescu-Ittu R, Mackie AS, Guo L, Dendukuri N, Kaouache M. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation. 2014;130(9):749-756. doi:10.1161/CIRCULATIONAHA.113.008396. PMID 24944314.
39. Cannesson M, Earing MG, Collange V, Kersten JR. Anesthesia for noncardiac surgery in adults with congenital heart disease. Anesthesiology. 2009;111(2):432-440. doi:10.1097/ALN.0b013e3181ae51a6. PMID 19602959.
40. Maxwell BG, Wong JK, Kin C, Lobato RL. Perioperative outcomes of major noncardiac surgery in adults with congenital heart disease. Anesthesiology. 2013;119(4):762-769. doi:10.1097/ALN.0b013e3182a56de3. PMID 23907357.
41. Baum VC, Barton DM, Gutgesell HP. Influence of congenital heart disease on mortality after noncardiac surgery in hospitalized children. Pediatrics. 2000;105(2):332-335. doi:10.1542/peds.105.2.332. PMID 10654951.
42. Faraoni D, Zurakowski D, Vo D, et al. Post-operative outcomes in children with and without congenital heart disease undergoing noncardiac surgery. J Am Coll Cardiol. 2016;67(7):793-801. doi:10.1016/j.jacc.2015.11.057. PMID 26892415.
43. Faraoni D, Vo D, Nasr VG, DiNardo JA. Development and validation of a risk stratification score for children with congenital heart disease undergoing noncardiac surgery. Anesth Analg. 2016;123(4):824-830. doi:10.1213/ANE.0000000000001500. PMID 27529321.
44. Nasr VG, Markham LW, Clay M, et al. Perioperative considerations for pediatric patients with congenital heart disease presenting for noncardiac procedures: a scientific statement from the American Heart Association. Circ Cardiovasc Qual Outcomes. 2023;16(1):e000113. doi:10.1161/HCQ.0000000000000113. PMID 36519439.
45. Wilson WR, Gewitz M, Lockhart PB, et al. Prevention of viridans group streptococcal infective endocarditis: a scientific statement from the American Heart Association. Circulation. 2021;143(20):e963-e978. doi:10.1161/CIR.0000000000000969. PMID 33853363.
46. Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association. Circulation. 2007;116(15):1736-1754. doi:10.1161/CIRCULATIONAHA.106.183095. PMID 17446442.
47. Palumbo T, Sluysmans T, Rubay JE, Poncelet AJ, Momeni M. Long-term outcome and anaesthetic management for non-cardiac surgery after Fontan palliation: a single-centre retrospective analysis. Cardiol Young. 2015;25(6):1148-1154. doi:10.1017/S1047951114001814. PMID 25245855.
48. Knott EM, Fraser JD, Alemayahu H, et al. Evaluation of cardiac function during laparoscopic gastrostomy in pediatric patients with hypoplastic left heart syndrome using intraoperative transesophageal echocardiography. J Laparoendosc Adv Surg Tech A. 2014;24(10):738-741. doi:10.1089/lap.2014.0287. PMID 25295635.
49. Chu DI, Tan JM, Mattei P, et al. Outcomes of laparoscopic and open surgery in children with and without congenital heart disease. J Pediatr Surg. 2018;53(10):1980-1988. doi:10.1016/j.jpedsurg.2017.10.052. PMID 29157923.
50. Gurvitz M, Krieger EV, Fuller S, et al. 2025 ACC/AHA/HRS/ISACHD/SCAI guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2026;153(8):e115-e251. doi:10.1161/CIR.0000000000001402. PMID 41411375.
51. Thompson A, Fleischmann KE, Smilowitz NR, et al. 2026 AHA/ACC/ACS/ASNC/HRS/SCA/SCCT/SCMR/SVM guideline for perioperative cardiovascular management for noncardiac surgery: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2026;154(13):e413-e504. doi:10.1161/CIR.0000000000001472. PMID 42804570.
52. Lui GK, Saidi A, Bhatt AB, et al. Diagnosis and management of noncardiac complications in adults with congenital heart disease: a scientific statement from the American Heart Association. Circulation. 2017;136(20):e348-e392. doi:10.1161/CIR.0000000000000535. PMID 28993401.
53. Navaratnam M, Schmidt AR, Kaplinski M, et al. Hemodynamic effects of altering tidal volume during positive pressure ventilation in the Fontan circulation: a randomized crossover trial. Paediatr Anaesth. 2025;35(8):607-618. doi:10.1111/pan.15096. PMID 40105302.
54. Adams PS, Stollings LM, Bains S, et al. Comparing cardiac output differences in patients with Fontan physiology between high and low tidal volume strategies: a randomized, crossover trial. Paediatr Anaesth. 2026;36(2):187-193. doi:10.1111/pan.70085. PMID 41277797.
55. Charla P, Karur GR, Yamamura K, et al. Augmentation of pulmonary blood flow and cardiac output by non-invasive external ventilation late after Fontan palliation. Heart. 2021;107(2):142-149. doi:10.1136/heartjnl-2020-316613. PMID 32748799.
56. Short JA, Paris ST, Booker PD, Fletcher R. Arterial to end-tidal carbon dioxide tension difference in children with congenital heart disease. Br J Anaesth. 2001;86(3):349-353. doi:10.1093/bja/86.3.349. PMID 11573523.
57. Fletcher R. The relationship between the arterial to end-tidal PCO2 difference and hemoglobin saturation in patients with congenital heart disease. Anesthesiology. 1991;75(2):210-216. doi:10.1097/00000542-199108000-00007. PMID 1907112.
58. Miranda WR, Aboulhosn JA, Hagler DJ. Catheterization in adults with congenital heart disease: a primer for the noncongenital proceduralist. JACC Cardiovasc Interv. 2022;15(9):907-921. doi:10.1016/j.jcin.2021.12.020. PMID 35512915.
59. Delgado V, Ajmone Marsan N, de Waha S, et al. 2023 ESC guidelines for the management of endocarditis. Eur Heart J. 2023;44(39):3948-4042. doi:10.1093/eurheartj/ehad193. PMID 37622656.
60. Baddour LM, Janszky I, Thornhill MH, et al. Nondental invasive procedures and risk of infective endocarditis: time for a revisit: a science advisory from the American Heart Association. Circulation. 2023;148(19):1529-1541. doi:10.1161/CIR.0000000000001180. PMID 37795631.
61. XARELTO (rivaroxaban) tablet, film coated. Janssen Pharmaceuticals, Inc. US Food and Drug Administration prescribing information, revised June 2025, DailyMed setid 10db92f9-2300-4a80-836b-673e1ae91610. Indications and Usage: Thromboprophylaxis in Pediatric Patients with Congenital Heart Disease after the Fontan Procedure. dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=10db92f9-2300-4a80-836b-673e1ae91610.
62. Peng DM, Zampi JD, Smith SM, et al. Acute hemodynamic effects of negative extrathoracic pressure in Fontan physiology. Pediatr Cardiol. 2019;40(8):1633-1637. doi:10.1007/s00246-019-02197-x. PMID 31468061.