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Review
Management of the adult Fontan patient
  1. Fred H Rodriguez1,2,
  2. Wendy M Book3
  1. 1 Internal Medicine and Pediatrics, Emory University, Atlanta, Georgia, USA
  2. 2 Pediatric Cardiology, Children's Healthcare of Atlanta Inc, Atlanta, Georgia, USA
  3. 3 Internal Medicine, Division of Cardiology, Emory University, Atlanta, Georgia, USA
  1. Correspondence to Dr Wendy M Book, Internal Medicine, Division of Cardiology, Emory University, Atlanta, GA 30322, USA; wbook{at}emory.edu

Abstract

Adults with congenital heart disease who underwent the Fontan operation to palliate single ventricle heart defects—by direct connection of caval venous return to the pulmonary circulation—have improved survival due to advancements in surgical and interventional techniques. However, cardiac and non-cardiac comorbidities often coexist, complicating management, and contributing to premature morbidity and mortality. Cardiac issues include heart failure symptoms related to systolic and diastolic dysfunction, atrial and ventricular arrhythmias and systemic atrioventricular valve regurgitation. Structural issues may be related to obstruction of the Fontan pathway, or to branch pulmonary artery stenosis, both of which exacerbate symptoms. Non-cardiac complications in adults involve hepatic congestion, fibrosis and cirrhosis, hepatocellular carcinoma, chronic kidney disease, stroke, venous stasis, lymphatic issues and involvement of other organ systems. ‘Fontan failure’ refers to circulatory dysfunction, either cardiac, non-cardiac, or both, diagnosed after exclusion or treatment of reversible contributors such as structural issues and arrhythmias. Counselling about reproductive health, mental health, perioperative management and overall wellness are paramount for patients’ well-being. Fontan patients are typically managed in highly specialised adult congenital heart centres, but may present to cardiologists or other practitioners with cardiac and non-cardiac emergencies or urgencies, sometimes after being out of care. In this review, we discuss the management of the adult Fontan patient, including surveillance, cardiac and non-cardiac complications, reproductive health, and advanced therapies.

  • Fontan physiology
  • congenital heart disease
  • complex congenital heart disease
  • heart failure with reduced ejection fraction
  • heart failure with preserved ejection fraction

https://doi.org/10.1136/heartjnl-2019-314937

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    Overview of Fontan operation

    Advances in cardiac surgery and critical care over the past several decades have allowed many infants with complex single ventricle heart defects to survive into adulthood. The Fontan operation, first described in 1971,1 results in a unique ‘Fontan circulation’ which is similar in its physiology and late complications across patients, independent of the original anatomy or type of Fontan surgery. The Fontan circulation uses the single ventricle—regardless of left or right ventricular morphology—to pump blood to the systemic circulation. Pulmonary blood flow is passive through direct connection of caval venous return to the pulmonary arteries. Chronically elevated systemic and hepatic venous pressures, a consequence of not having a subpulmonary ventricle, lead to many of the late non-cardiac complications seen in early adulthood.2 Cardiac complications include failure of the single ventricle and atrioventricular valve, arrhythmias, structural problems in the Fontan circuit, development of shunting from collaterals, cyanosis and premature mortality.3 Extra-cardiac complications involve multiple organ systems, particularly the liver and lymphatics.4 Figure 1 illustrates the multiple organ systems that may be affected long-term. When the circulation fails—for cardiac reasons, non-cardiac reasons, or both—and no reversible aetiology is identified, ‘Fontan failure’ should be considered. Due to the complexity of these patients and the broad range of late complications, the adult Fontan patient should be managed in conjunction with an adult congenital heart centre that has expertise in caring for this complex patient population.

    Figure 1
    Figure 1

    Multiorgan dysfunction later after Fontan operation (reproduced with permission from Book et al).2

    Cardiac complications and management

    Cardiac complications can occur immediately after the Fontan operation, including new onset arrhythmias, depressed systolic function, obstruction of the branch pulmonary arteries and other anatomic issues. However, adults with Fontan physiology usually had the Fontan operation during childhood and ‘late’ complications after the Fontan operation relate to issues that may occur more than 10–20 years postoperatively, in early adulthood.3 ‘Fontan Failure’ in adults refers to circulatory dysfunction that may involve multiple organ systems, typically presenting as a diverse clinical picture of any or all of declining exercise intolerance, ascites, oedema, protein losing enteropathy or plastic bronchitis, in the absence of reversible causes. ‘Fontan Failure’ is perhaps best thought of in terms of clinical phenotypes of circulatory dysfunction, which may involve multiple organ systems, with treatment tailored to the specific patient phenotype. Four broad Fontan failure phenotypes (cardiac and non-cardiac) have been described (table 1) with specific treatment suggestions in table 2.2 Phenotypes may coexist and change over time. Structural complications, arrhythmias and functional complications may all present opportunities for improvement, and should be excluded or treated before diagnosing ‘Fontan failure’. Cardiac Fontan failure describes irreversible functional cardiovascular contributors to circulatory dysfunction, whereas non-cardiac Fontan failure is dominated by circulatory failure in the setting of normal cardiac function. A combination of factors may play a role in decompensation in an individual patient, but for the purposes of this review, we will discuss approaching management of the Fontan patient in a systematic way.

    View this table:
    Table 1

    Haemodynamicphenotypes of Fontan failure

    View this table:
    Table 2

    Treatment considerations by failing Fontan type

    Structural complications, surveillance and management

    Obstruction of the systemic venous return to the pulmonary arteries from the Glenn (superior vena cava to pulmonary artery connection) or Fontan (inferior vena cava or right atrium to pulmonary artery connection) can present with signs and symptoms of venous congestion of the upper body (Glenn obstruction), the lower extremities and abdomen (Fontan obstruction), or both. Morphologic liver changes, ascites and/or oedema may be noted in the setting of an obstructed Fontan circuit (figure 2). Structural complications can present at any point after the operation. Recognition of the problem may be during evaluation for exercise intolerance, cyanosis, protein losing enteropathy, ascites, varicose veins, or oedema. After diagnosis of obstruction by imaging, interventions are directed at enlarging or replacing existing conduits and structures,5 and removing thrombi when appropriate. Mechanical thrombectomy may be used for thrombosis, as well as balloons and stenting in the interventional catheterisation lab.6 Some patients may need surgical upsizing of Fontan conduits and/or Fontan revision.

    Figure 2
    Figure 2

    Stenting of Fontan baffle due to obstruction, White arrow: Stent in Fontan baffle.

    Branch pulmonary artery (PA) obstruction is a challenging issue for patients after the Fontan, potentially related to long standing passive, instead of pulsatile, flow in the pulmonary arteries. PA obstruction may present with ascites and peripheral oedema or exercise intolerance. Elevated Fontan pressures may be noted during haemodynamic evaluation with a pressure gradient into the distal PAs. Focal stenosis may be amenable to balloon and/or stent intervention.7 Long segment branch pulmonary artery hypoplasia may not be appropriate for stenting and may require surgical enlargement. However, surgical PA enlargement in adults with Fontan is a major undertaking, and may only be seriously considered in patients undergoing other cardiac operations.

    Valvular disease traditionally was thought to be a contraindication to Fontan operation,8 but not necessarily in the current era. Valvular dysfunction may worsen over time.9 The presence of a heart murmur may be the initial indication of valvular regurgitation or stenosis. Routine echocardiography is indicated in patients with the Fontan operation to detect issues with the atrioventricular and aortic/neoaortic valves. Valve replacement after the Fontan operation occasionally becomes necessary, but often with a higher threshold for surgery due to the potential for complications and worsening ventricular function postoperatively. In some situations, medical therapy for the stable patient, or referral for advanced therapy in the symptomatic patient, is the more appropriate course.

    Table 3 summarises the diagnosis and management of different types of collateral vessels that may develop. Aortopulmonary (AP) collaterals can result in elevated pulmonary arterial and atrial pressures, pulmonary congestion and heart failure through left to right shunting. Haemoptysis may also indicate the presence of AP collaterals in the absence of overt heart failure. A magnetic resonance angiogram or gated cardiac CT angiogram can be helpful for detection of large AP collaterals,10 however, catheter based angiography and intervention are the invasive method for diagnosing and treating AP collaterals at many centres.11 Common sites for symptomatic AP collaterals are the internal mammary arteries, which may be amenable to particle, coil, or device occlusion. The indications for intervention in AP collaterals are controversial, but in general centres would advocate for intervention for significant volume load, ventriculomegaly, heart failure, haemoptysis and if there is an adequate target for intervention.

    View this table:
    Table 3

    Collaterals in Fontan patients

    Venous to pulmonary venous collaterals (VV collaterals) may contribute to worsening cyanosis and exercise intolerance due to right to left shunting. Venous to systemic venous collaterals are usually not intervened on since the blood would return to the pulmonary circulation. An echo with bubble study will be positive in the setting of veno-pulmonary venous collaterals, as it would be with a fenestration, depending on the site of intravenous catheter placement, and anatomic details of the Fontan circulation. After years of chronically elevated Fontan pressures, patients may ‘autofenestrate’ by developing VV collaterals to decompress the Fontan pressures. Prior to the Fontan, paediatric interventional cardiologists will often occlude veno-pulmonary venous collaterals. Embolisation of VV collaterals after the Fontan is associated with decreased survival.12

    Due to the passive flow through the Fontan circuit, elevated vascular resistance may impair systemic venous return through the pulmonary bed. Some patients with Fontan physiology may benefit from pulmonary vasodilators.13 Diagnosis of elevated pulmonary vascular resistance is made in the cardiac catheterisation lab, and patients with high Fontan pressures and low ventricular end diastolic pressure may benefit from starting pulmonary vasodilators.14 However not all benefit, including those with low systemic vascular resistance and hypotension.

    Arrhythmic complications, surveillance and management

    Intra-atrial re-entry tachycardia (IART), other supraventricular tachycardias (SVT) and atrial fibrillation are common years after the Fontan operation.15 Often, the clinical presentation of atrial tachyarrhythmias is a decrease in exercise tolerance, sometimes including palpitations, lightheadedness, or syncope. Patients may recognise the change in symptoms, especially if there is a history of atrial arrhythmias. However, patients with a first episode of SVT or IART may not present to medical care for weeks or months despite reduced exercise tolerance. An Electrocardiogram (EKG) may be misinterpreted as sinus tachycardia; however, having a heart rate of >100 beats per minute and worsened symptoms strongly suggest the possibility of IART in a Fontan patient (figure 3). The standard of care for a Fontan patient in IART, atrial fibrillation, or other SVT is to restore sinus rhythm. Rhythm control is preferred to rate control for atrial fibrillation if possible. Recurrent and intractable arrhythmias are a poor prognostic factor.15 Some pacemakers have antitachycardia pacing capabilities that may be beneficial in terminating IART. The Fontan circuit has a significant risk of spontaneous contrast and thrombus formation when patients have SVT or atrial fibrillation in the setting of an atriopulmonary Fontan, even after cardioversion. (figure 4)16 Anticoagulation with warfarin is indicated for Fontan patients who have a history of arrhythmias or thrombus formation.17 Beta blockers are often first line management for atrial arrhythmias. Antiarrhythmics may be required for refractory arrhythmias. Performing a transesophageal echocardiogram may not always require sedation, but cardioversion in a stable Fontan patient does involve sedation that should be performed in a hospital setting due to the risk of decompensation and need for a higher level of care. Positive pressure ventilation, if required, may decrease cardiac output in Fontan patients due to reduced venous return. Similarly, hypoventilation may increase pulmonary vascular resistance and decrease cardiac output. Intravenous lines should be filtered with 0.22-micron air filters in Fontan patients with saturations<93% due to the risk of paradoxical air emboli. Ablation procedures and pacemaker placement should be performed by an experienced electrophysiologist knowledgeable about variations in Fontan anatomy and physiology, at a comprehensive adult congenital heart centre. Ablation in general is palliative, as eventual recurrence of arrhythmia is common, often at a different focus. For patients with refractory arrhythmias, Fontan conversion with a modified atrial maze procedure may be beneficial.18

    Figure 3
    Figure 3

    (A) Intra-atrial re-entry tachycardia in an adult after the Fontan operation. (B) After cardioversion.

    Figure 4
    Figure 4

    (A)Transesophageal echocardiogram (bicaval view) shows 2:1 intra-atrial re-entrant tachycardia with dense spontaneous echo contrast. online supplemental motion image is available for (A). (Reproduced with permission from Lam et al).16 (B) Transesophageal echocardiogram (bicaval view) after cardioversion to sinus rhythm shows persistent spontaneous echo contrast. Online supplemental motion image is available for (B). (Reproduced with permission from Lam et al).16 IVC, inferior vena cava; LA, left atrium; RA, right atrium/atriopulmonary Fontan circuit to pulmonary arteries

    Supplementary video

    Supplementary video

    Functional complications and management

    Systolic heart failure (type I Fontan failure, tables 1 and 2)2 is treated with typical heart failure therapy, including beta blockers, angiotensin blockade, anticoagulation if appropriate and aldosterone antagonists for severely depressed systemic ventricular systolic function. Angiotensin blockade should be avoided or discontinued for low systemic vascular resistance state from portal hypertension. Diastolic heart failure (type II Fontan failure) is treated with control of hypertension, if present, a low sodium diet and diuretics as needed.

    Non-cardiac complications and management

    Multiple organ systems are impacted by the Fontan circulation (figure 1). Non-cardiac complications, common in adults who have had a Fontan operation, should be managed in conjunction with an adult congenital heart disease centre.19 Initial evaluation should include a thorough evaluation for reversible structural and arrhythmic complications, and an assessment of ventricular function. Once reversible structural and arrhythmic complications have been excluded or treated, and ventricular function is determined to be normal, the diagnosis of non-cardiac Fontan failure can be considered if symptomatic signs of circulatory failure persist.2 20 Non-cardiac Fontan failure phenotypes are characterised by exercise intolerance, oedema, ascites and/or lymphatic abnormalities often in the presence of normal heart function and pressures (table 1).

    Liver

    Chronic elevation of pressures in the liver can lead to hepatic fibrosis, cirrhosis and rarely hepatocellular carcinoma (HCC),21 all of which occur independent of systolic heart failure. Multiple insults to the liver may accelerate the process; thus screening for viral hepatitis, and vaccination for Hepatitis A and B are recommended for all Fontan patients.22 Ascites may be the result of hepatic congestion or fibrosis, lymphatic obstruction, or both. Protein losing enteropathy and plastic bronchitis are rare lymphatic related complications that may occur in the presence of normal cardiac function and normal Fontan pressures, and may mimic the cirrhotic phenotype or coexist with cirrhosis.

    Hepatic fibrosis can predispose to the development of HCC, even in asymptomatic patients, and in the absence of frank cirrhosis, symptoms, or ventricular dysfunction. Hepatic fibrosis is detected in >90% of adults after the Fontan operation by biopsy, often with normal liver enzyme testing.23 24 Once hepatic fibrosis is noted, HCC screening should be conducted every 6 months, in conjunction with a hepatologist familiar with the Fontan circulation.22 Untreated HCC has a poor prognosis; early diagnosis of HCC can thus be life-saving.

    Renal

    Twenty years after the Fontan operation, approximately half of Fontan patients have abnormalities of renal function,25 contributing to approximately 10% of late mortality.26 The onset of renal failure is a poor prognostic sign,27 signifying circulatory failure, with management complicated by anatomic and haemodynamic considerations of renal replacement therapies in the Fontan circulation.

    Venous stasis

    Venous stasis is common related to high systemic venous pressures often exacerbated by multiple prior venous accesses for procedures. Venous stasis contributes to significant late morbidity including venous stasis ulcers, varicose veins, oedema and leg pain.28 Initial therapy with strengthening exercises and graduated thigh or waist high compression stockings at adequate pressure (30–40 mm Hg) may be best initial therapy, although graduated compression has not been studied in the Fontan population. The efficacy and durability of sclerotherapy and venous stripping in the Fontan population is unknown.

    Non-cardiac surgery

    The adult with a Fontan circulation may on occasion present with a need for urgent non-cardiac surgical evaluation. Similar to a young adult population, emergency surgical evaluation in these patients is often for appendicitis, cholecystitis or orthopaedic injuries. When possible, non-emergent surgery should take place in a comprehensive adult congenital heart centre with availability of specialty teams experienced in managing the Fontan circulation in the operating room and intensive care units.19

    Caution is advised when presented with a clinical picture of cholecystitis in the absence of gallstones, as hepatic congestion may cause a similar picture. Development of ascites following cholecystectomy has been described anecdotally, possibly due to disruption of lymphatics or worsening hepatic function. For all surgeries, anaesthesia is best conducted in conjunction with a cardiac anaesthesiologist, as the unique Fontan circulation requires consideration of multiple issues including the decrease in cardiac output from positive pressure ventilation and abdominal insufflation, venous access issues related to prior interventions, the need for air filters on intravenous lines to prevent paradoxical air embolism, knowledge of the patient’s baseline central venous pressure and oxygen saturations, and a solid understanding of the anatomy and physiology of the individual patient. Despite the complexity of these patients, mortality is low when these surgeries are performed by a multi-disciplinary team in a specialty centre.29

    Mental health

    Mental health and executive functioning issues are common in adults with heart defects, negatively impacting quality of life,30 contributing to medication and appointment non-adherence, and difficulties in school or at work.31 The adult Fontan patient may have additional mental health issues related to multiple surgeries, hospitalisations and physical limitations. These young adults live with chronic illness and an uncertain future, which may lead to anxiety, depression and medical non-adherence. Early referral to behavioural health is recommended to reduce morbidity associated with mental health issues. Remaining in congenital heart disease specialty care is important to ensuring the best outcomes for this complex population. Unfortunately, many young adults with chronic illness drop out of care.32 Recognising the need for lifelong care, providers who encounter these patients should refer them back into specialty care.

    Reproductive health

    Our centre discourages pregnancy in women with a Fontan operation in general. Published studies, and anecdotal experience at our centre, have demonstrated successful completion of pregnancy in women with a Fontan operation. However, we discourage pregnancy in Fontan patients due to a high rate of miscarriage, preterm delivery and low birth weight neonates.33 In general, oestrogen containing contraception should be avoided due to the increased risk of thrombus formation. Barrier protection such as condoms are advised for contraception and reducing risk of sexually transmitted diseases, but are not sufficient in isolation to prevent pregnancy. Progesterone-only oral contraceptive pills are not as effective in preventing pregnancy as implantable contraception, so concurrent condom use is advised as a complimentary method. Implantable contraception and intrauterine devices are effective, long lasting and may be a good contraceptive choice.34

    Advanced therapies

    Fontan failure is common among adults and often leads to end-stage circulatory failure. Early referral to an adult congenital heart centre can delineate short and long-term treatment options, before the development of irreversible circulatory failure. Medical management is best guided by Fontan failure type(s) after a thorough evaluation for reversible and/or treatable structural or arrhythmic complications (table 2). Heart transplant alone can be considered for Fontan patients with ventricular failure or preserved ventricular function and circulatory dysfunction, as evidenced by oedema, exercise intolerance and possibly ascites. Evaluation for heart transplant is best done at a centre with expertise in both transplant and adult congenital heart disease, with the availability of congenital heart surgery.35 Expertise in hepatology and liver transplant is essential, as most adult Fontan patients have significant hepatic fibrosis, and some may require combined organ transplant. Mechanical circulatory support is limited by durable device availability to support both pulmonary and systemic circulations.36

    Heart transplantation for Fontan failure is associated with high early mortality and morbidity37 due to need for reconstructions, redo sternotomies, long ischaemic times, bleeding and multi-organ failure. However, conditional outcomes for 1-year survivors are better than for other indications.38 Indications for heart-liver transplant are controversial, vary centre to centre,39 and are beyond the scope of this review. However, most centres would agree the Fontan patient with a history of HCC who needs transplant requires combined heart liver transplant. Not all patients with Fontan failure will benefit from, or be candidates for, transplantation. Referral for palliative care is appropriate for patients with end-stage failing circulations.

    Conclusion

    The Fontan operation has allowed many individuals with single ventricle physiology to not only survive to adulthood but thrive as adults with fulfilling, enriched lives. Both cardiac and non-cardiac long term complications exist and must be closely monitored and addressed. Not all patients with the Fontan operation have similar comorbidities or haemodynamics, thus diagnostics and therapies must be tailored appropriately. Adults who had a Fontan operation are a rapidly expanding patient population with numerous potential complications but exciting prospects for improved long-term outcomes.

    Acknowledgments

    Thank you to Dr. Timothy Slesnick, MD and Dr. Sassan Hashemi, MD for reformatting the image in Figure 2.

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    Footnotes

    • Contributors Both authors, FHR and WMB contributed equally to this manuscript—research and writing.

    • Funding WMB and FHR receive funding/support from the Centers for Disease Control and Prevention, Grant/Award Number: CDC-RFA-DD15-1506.

    • Competing interests None declared.

    • Patient consent for publication Not required.

    • Provenance and peer review Commissioned; externally peer reviewed.