Pediatric Tricuspid Atresia Treatment & Management

Updated: Jan 17, 2017
  • Author: Syamasundar Rao Patnana, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
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Treatment

Medical Care

The prognosis for patients with tricuspid atresia and other complex congenital cardiac defects with one functioning ventricle has improved because of the advent of physiologically corrective surgery for tricuspid atresia and its modifications. However, such procedures are usually restricted to patients older than 1 year, though patients with tricuspid atresia are symptomatic in the neonatal period or early infancy. Palliation should be performed to allow infants to reach the age and weight requirements for correction.

As a consequence, the objective of any management plan is not only to provide symptomatic relief but also to preserve, protect, and restore the anatomy (with good-sized and undistorted pulmonary arteries) and physiology (normal pulmonary artery pressure and preserved left ventricular function) to normal so that a corrective procedure can be safely performed when the patient reaches an optimal age and weight. [29]

Management at presentation

Medical management during the process of identification, transfer to a pediatric cardiology center, initial workup, and cardiac catheterization (if needed) and during and after palliative surgery or procedures includes maintenance of a neutral thermal environment, normal acid-base balance, normoglycemia, and normocalcemia with appropriate monitoring and correction, if necessary. [29, 57, 74] Unless associated pulmonary parenchymal pathology is present, the fraction of inspired oxygen (FIO2) administered should be no more than 0.4.

Neonates who have low arterial PO2 and O2 saturation and ductal-dependent pulmonary blood flow should receive an intravenous infusion of PGE1 0.03-0.1 mcg/kg/min to open the ductus arteriosus or to maintain its patency. [29, 74, 75] This is followed by an aortopulmonary shunt (see Palliative surgery).

In the infant who presents with signs of congestive heart failure (type Ic or IIc), anticongestive therapy with digoxin, diuretics, and afterload reduction should be promptly given. [29, 74] Considerations pertaining to pulmonary artery banding are reviewed in Surgical Care.

In patients with severe aortic coarctation, which is particularly observed in those with type II disease, ductal dilation with an infusion of PGE1 may improve systemic perfusion. [29] Surgical repair of the coarctation should follow. Some cardiologists use balloon angioplasty to relieve the aortic obstruction.

If interatrial obstruction is present, it should be relieved by means of balloon atrial septostomy. On occasion, blade or surgical septostomy is necessary. [76, 77]

For patients presenting after infancy, the treatment approach is similar to that described above, except that PGE1 is not effective in opening the ductus.

Medical management after palliation

The management issues in tricuspid atresia are similar to those in other cyanotic congenital heart defects and are discussed in Tricuspid Atresia. [29]

Intercurrent illnesses cause significant interstage mortality. [78] This is much higher between Stage I and II than between Stage II and III (see below). Intercurrent illness should be promptly assessed and treated appropriately. [79] In addition, the aortopulmonary shunts may become occluded with a thrombus within the Gore-Tex graft. [80] Consequently, even minor illnesses in patients with single-ventricle physiology such as tricuspid atresia must be treated aggressively.

Hemoglobin should be periodically measured, and anemia and polycythemia, when present, should be treated.

Patients should receive antibiotic prophylaxis before undergoing any bacteremia-producing surgery or procedures.

The risks of stroke and brain abscess are similar to those in other cyanotic heart defects. When such a problem develops, appropriate neurologic or neurosurgical consultation and treatment is indicated. [29]

Routine well-child care, including immunizations, by the primary care physician is suggested. Administration of polyvalent pneumococcal vaccine and influenza vaccine and immunization against respiratory syncytial virus should be considered. [29]

Issues such as physical and emotional development, genetic counseling, vocational training and rehabilitation, [81] pregnancy, and contraception are addressed similarly to those in other cyanotic heart defects. [82]

The development of hyperuricemia, gout, and uric acid nephropathy in adolescents and adults with long-standing cyanosis and polycythemia is similar to that in other cyanotic heart defects. [29] Timely palliative and corrective surgery may prevent such complications.

Catheter intervention

In the neonate, obstruction at the level of the atrial septum may be treated with conventional Rashkind balloon atrial septostomy. [83] In infants and children, the interatrial septum may be too thick to be torn with balloon septostomy; therefore, Park blade septostomy should precede the Rashkind procedure. [76]

In most patients, obstruction to pulmonary blood flow is at the ventricular septal defect (VSD) level or in the subpulmonary region. In some patients, though rare, the predominant obstruction is at the pulmonary valve. In such patients, balloon pulmonary valvuloplasty may be useful in improving pulmonary blood flow and oxygen saturation. [84]

If progressive cyanosis develops after a previous Blalock-Taussig shunt and if the hypoxemia is due to a stenotic shunt, balloon dilatation may be used to improve oxygen saturation. [85] Rarely, stents may be required to keep the shunt open. [80] However, if the patient is of sufficient size and age to undergo a bidirectional Glenn procedure, this procedure should be performed instead of catheter intervention to open up the narrowed Blalock-Taussig shunt.

If severe aortic coarctation is present, particularly in patients with tricuspid atresia type II, balloon angioplasty may be useful in relieving aortic obstruction and may help achieve better control of congestive heart failure. [86, 87] However, it should be mentioned that some caregivers prefer surgery to relieve the aortic obstruction.

If clinically significant branch pulmonary artery stenosis is present before bidirectional Glenn or Fontan conversion or after a Fontan procedure is performed, balloon angioplasty or placement of intravascular stents is recommended. [88, 89]

Development of aortopulmonary collateral vessels has been increasingly observed in recent studies. Before the final Fontan conversion, occlusion of these vessels in the catheterization laboratory, usually by means of coil embolization, is recommended [90, 91] to reduce left ventricular volume overloading and, probably, the duration of chest-tube drainage.

After a Fontan procedure, some patients may have recurrent pleural effusion, liver dysfunction, plastic bronchitis or protein-losing enteropathy. In these patients, rule out obstructive lesions in the Fontan circuit, then puncture of the atrial septum by using a Brockenbrough technique followed by static balloon atrial septal dilatation or stent implantation may be beneficial.

Patients who undergo a fenestrated Fontan operation or who have a residual atrial defect despite correction may have clinically significant right-to-left shunting that causes severe hypoxemia. These residual atrial defects may be closed by using transcatheter techniques. [92, 93, 94, 95]

Some patients may develop systemic venous–to–pulmonary venous collateral vessels following Fontan operation, causing arterial desaturation. These vessels should be defined and closed by coils, plugs, or devices, depending on the size, location, and accessibility. [91, 96, 97]

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Surgical Care

Surgical management may be broadly grouped into palliative and corrective therapies.

Palliative surgery

Palliative therapy depends on the hemodynamic disturbance the associated cardiac anomalies produce and may be discussed in terms of decreased pulmonary flow, increased pulmonary flow, or intracardiac obstruction. [98]

Decreased pulmonary blood flow

Pulmonary blood flow may be increased by surgical creation of an aortopulmonary shunt. After Blalock and Taussig (1945) initially described subclavian artery–to–ipsilateral pulmonary artery anastomosis in 1945, [99] other procedures have been described, including Potts shunt (descending aorta–to–left pulmonary artery anastomosis), Waterston-Cooley shunt (ascending aorta–to–right pulmonary artery anastomosis), central aortopulmonary fenestration or expanded polytetrafluoroethylene (Gore-Tex; W. L. Gore & Associates, Inc, Newark, Delaware) shunt, modified Blalock-Taussig shunt (Gore-Tex interposition graft between the subclavian artery and the ipsilateral pulmonary artery), Glenn shunt (superior vena cava–to–right pulmonary artery anastomosis, end-to-end), formalin infiltration of the wall of ductus arteriosus, enlargement of the VSDs, and stent implantation into the ductus.

Modified Blalock-Taussig shunt with an interposition Gore-Tex tube graft between the subclavian artery and the ipsilateral pulmonary artery, which de Leval et al described, has stood the test of time and is currently the procedure of choice for palliation in pulmonary oligemia. [100] Some surgeons prefer central aorta to pulmonary Gore-Tex tube grafts.

Because the site of obstruction to pulmonary flow in most patients with tricuspid atresia is at the VSD, resection of the septal muscle to enlarge the VSD, which Annechino et al advocate, appears to be a logical choice because it addresses rather than bypasses the site of obstruction. [101] However, this resection is an open-heart surgical procedure and more cumbersome than the modified Blalock-Taussig shunt in augmenting the pulmonary blood flow; it has not been routinely used. In the rare patient with predominant obstruction at the pulmonary valve, balloon pulmonary valvuloplasty may be used to increase the pulmonary blood flow. [84] Stenting the arterial duct is an attractive nonsurgical option; however, because of limited experience, it is not currently a first-line therapeutic option. [102, 103]

In summary, a number of palliative procedures are available to augment pulmonary blood flow, but the modified Blalock-Taussig shunt is the recommended procedure of choice in most, if not in all, patients with tricuspid atresia with pulmonary oligemia. [29]

Increased pulmonary blood flow

Patients with increased pulmonary blood flow are likely to have type Ic or type IIc defects without associated pulmonary stenosis. Congestive heart failure is likely to occur in these patients.

In patients with tricuspid atresia type II (transposition of the great arteries), pulmonary artery banding should be performed after stabilization with anticongestive measures. Banding not only improves congestive heart failure but also helps achieve normal pulmonary artery pressure so that bidirectional Glenn and Fontan procedures can safely be performed later. [29] If associated aortic coarctation is present, it must be relieved. Pulmonary artery banding stimulates more ventricular hypertrophy, which may further reduce the size of the VSD, thus increasing subaortic obstruction. [104]

In patients with tricuspid atresia type I (normally related great arteries), aggressive anticongestive measures should promptly be undertaken. Because natural history studies suggest that the VSD spontaneously closes or becomes smaller with time and the patients with pulmonary plethora develop pulmonary oligemia, banding of the pulmonary artery is generally not recommended in this group of patients. However, if symptoms are not relieved after optimal anticongestive therapy and some delay, pulmonary-artery banding should be performed. Patients with no pulmonary-artery banding should receive careful follow-up and monitoring of pulmonary artery pressure. [29]

Absorbable pulmonary artery bands have been used for palliation in such infants. [105] By restricting the pulmonary blood flow, the absorbable polydioxanone band decreases pulmonary artery pressure and initially helps abate symptoms of heart failure. As the VSD spontaneously closes, the pulmonary artery band is resorbed and does not produce the severe pulmonary oligemia that might have been associated with a conventional nonabsorbable band. This is an ingenious approach, although it is likely to be helpful in a limited number of patients. [106]

Intracardiac obstruction

Intracardiac obstruction may occur at the level of patent foramen ovale and VSD. It can be subdivided into interatrial and interventricular obstruction.

Interatrial obstruction

Because the entire systemic venous return must pass through the patent foramen ovale, it should be large enough to allow unimpeded egress of systemic venous blood. Because of the compliant right atrium and proximal systemic veins, evaluating interatrial obstruction is difficult. Clinical signs of systemic venous congestion and presystolic hepatic and jugular venous pulsations suggest obstructed atrial septum. A mean atrial pressure difference of more than 5 mm Hg and prominent a waves in the right atrial pressure wave are generally considered diagnostic of clinically significant obstruction. [29, 33, 41]

Balloon atrial septostomy usually promptly improves presystolic hepatic and jugular pulsations and decreases interatrial pressure difference. Blade atrial septostomy is occasionally necessary, especially in older infants and children. [82, 107] Surgical atrial septostomy is needed even less frequently. However, surgical septectomy to allow unrestricted flow across the atrial system should be performed concurrently with bidirectional Glenn procedure.

Interventricular obstruction

Spontaneous closure of the VSD can occur, causing interventricular obstruction. [26, 27, 47, 49, 50, 51]

Functional VSD closure in tricuspid atresia type I results in cyanotic spells, similar to those in tetralogy of Fallot. [48] Initial management is similar to that of tetralogy of Fallot and includes knee-chest positioning, humidified oxygenation, and the administration of morphine sulfate 0.1 mg/kg. If the patient's condition is unresponsive, beta-blockers (propranolol, esmolol) or intravenous pressors (methoxamine, phenylephrine) are administered to increase systolic blood pressure by 10-20%. Concurrent correction of anemia or metabolic acidosis should also be undertaken.

If no improvement occurs, emergency surgical palliation with a Blalock-Taussig type of shunt may be necessary. If the infant's condition improves clinically, elective surgery, Blalock-Taussig shunting, or a bidirectional Glenn or Fontan procedure may be performed, depending on the patient's age and weight and the status of pulmonary arteries and left ventricle. [48]

Partial or complete anatomic closure of the VSD can also occur in patients with type I, causing pulmonary oligemia. The management is as described in the decreased pulmonary blood flow section above.

In patients with tricuspid atresia type II, spontaneous closure of the VSD produces subaortic obstruction. This obstruction should be relieved or bypassed as soon as it is detected because it produces left ventricular hypertrophy, which, in turn, increases the risk at the time of Fontan operation. [108] The VSD, right ventricle, and aortic valve may be bypassed with an anastomosis of the proximal stump of the divided pulmonary artery to the ascending aorta (Damus-Kaye-Stansel procedure) directly or by means of a prosthetic conduct at the time of bidirectional Glenn or Fontan conversion. [23, 50]

Alternatively, the conal septal muscle may be resected to enlarge the VSD [109] ; this is a direct approach in relieving the subaortic obstruction. However, development of heart block, inadequate relief of obstruction, and spontaneous closure of the surgically enlarged or created VSD remain major concerns.

Corrective surgery

After Fontan and Kreutzer's initial description of the physiologically corrective operation for tricuspid atresia, [110, 111] corrective surgery was widely adapted by most workers in the field. The concept was even extended to treat other cardiac defects with a functionally single ventricle.

The originally described Fontan operation consisted of the following [110] :

  • Superior vena cava–to–right pulmonary artery end-to-end anastomosis (Glenn procedure) [112]

  • Anastomosis of the proximal end of the divided right pulmonary artery to the right atrium directly or by means of an aortic homograft

  • Closure of the atrial septal defect (ASD)

  • Insertion of a pulmonary valve homograft into the inferior vena caval orifice

  • Ligation of the main pulmonary artery to completely bypass the right ventricle

Kreutzer performed anastomosis of the right atrial appendage and pulmonary artery directly or by using a pulmonary homograft and closed the ASD. [111] A Glenn procedure was not performed, and a prosthetic valve was not inserted into the inferior vena cava.

Fontan's concept was to use the right atrium as a pumping chamber; therefore, he inserted a prosthetic valve into the inferior vena cava and right atrial–pulmonary artery junction. Kreutzer's view was that the right atrium may not function as a pump and that the left ventricle functions as a suction pump in the system.

Numerous modifications to the aforementioned procedures were made by these and other workers in the field, as Chopra and Rao reviewed in the American Heart Journal (1992). [113] When their review was published, the 4 major types of Fontan-Kreutzer procedures used were right atrium–to–pulmonary artery anastomosis with or without a valved conduit and right atrium–to–right ventricular connection with and without a valved conduit.

On the basis of immediate-term and intermediate-term results, direct atriopulmonary anastomoses (ie, without a conduit) appears to be the best procedure for patients with type I defects who have a small (< 30% of normal) right ventricle and for all patients with type II (transposition) defects. Right atrial–to–right ventricular valved conduit (preferably homograft) anastomosis appears to be most suitable for patients with type I defects who have good-sized (>30% of normal) right ventricles and a trabecular component. [113, 114]

Subsequent to Chopra and Rao's review, several other concepts emerged. These include bidirectional cavopulmonary anastomosis, fenestrated Fontan, total cavopulmonary connection, and staged cavopulmonary connection.

Bidirectional cavopulmonary anastomosis

Bidirectional cavopulmonary anastomosis is a modified Glenn procedure in which the upper end of the divided superior vena cava is anastomosed end-to-side to the right pulmonary artery without disconnecting the latter from the main pulmonary artery. Thus, the superior vena cava blood is diverted into both the right and left pulmonary arteries, justifying the name "bidirectional."

Haller et al (1996) studied experimental bidirectional cavopulmonary connection in animal models, [115] and Azzolina et al (1972) first described its clinical use. [116] Others later applied this technique to palliate complex heart defects with decreased pulmonary blood flow. Hemodynamic advantages of the bidirectional Glenn procedure are improved effective pulmonary blood flow, decreased total pulmonary blood flow, and reduced left ventricular volume overloading. Preserved continuity of the pulmonary artery is another advantage and may help enable a low-risk Fontan procedure.

When right and left superior vena cavae are present, bilateral bidirectional Glenn shunting should be performed, especially if the bridging innominate vein is absent or small.

Fenestrated Fontan operation

Fenestrated Fontan operation is another procedure used in tricuspid atresia.

Many cardiologists and surgeons have modified the criteria Choussat et al outlined. [117] Patients not meeting these criteria are at higher risk for a poor prognosis after a Fontan operation than patients who do. For the high-risk group, several workers have advanced the concept of leaving a small ASD open to facilitate decompression of the right atrium. [118, 119, 120] Laks et al advocated closure of the atrial defect by constricting the preplaced suture in the postoperative period, [120] whereas Bridges et al used a transcatheter closure technique later. [119]

Clinically significant decreases in the postoperative pleural effusions and systemic venous congestion and higher cardiac output have been noted after a fenestrated Fontan procedure. The duration of hospitalization appears to be decreased. However, these beneficial effects are at the expense of mild arterial hypoxemia.

Although the fenestrated Fontan procedure was initially conceived for patients at high risk, it has since been used in patients with modest or even low risk. Although rare, reports of cerebrovascular or other systemic arterial embolic events occurring after a fenestrated Fontan operation tend to contraindicate the use of fenestrations in patients with low or usual risk. Some data indicate that routine fenestration is not necessary. [121]

Total cavopulmonary connection

On the basis of their hemodynamic studies, de Leval et al (1988) concluded that the right atrium is not an efficient pump. [122] Flow in nonvalved circulation generates turbulence with consequent net decrease in flow, and energy losses are significant in the nonpulsatile chambers, corners, and obstructions. Using these principles, they designed and performed total cavopulmonary diversion in which the upper end of the divided superior vena cava is anastomosed end-to-side to the top of the undivided right pulmonary artery (bidirectional Glenn), and the inferior vena caval blood is routed via an intra-atrial tunnel into the cardiac end of the superior vena cava, which, in turn, is connected end-to-side to the undersurface of the right pulmonary artery.

Technical simplicity, maintenance of low right atrial and coronary sinus pressures, and reduction in right atrial thrombi are advantages of the procedure. Subsequent experimental work by Sharma et al indicated that complete or minimal offset between the orifices of the superior and inferior vena cavae into the right pulmonary artery decreases energy losses. [123]

Although the total cavopulmonary connection was initially devised for patients with complex atrial anatomy and/or systemic venous anomalies, it has since been used extensively for all types of cardiac anatomy with one functioning ventricle and irrespective of venous anomalies.

Staged Fontan

Performing the Fontan procedure in stages appears to decrease overall mortality, most likely related to improving the ventricular function by correction of the afterload mismatch that is associated with one-stage Fontan procedure. At the current time, most centers prefer staged Fontan with bidirectional Glenn initially, followed later by extracardiac conduit diversion of the inferior vena caval blood into the pulmonary artery.

Relatively recent developments

Since the author's review more than 2 decades ago, [113, 114] several observations have been made that tended to support that total cavopulmonary connection is the Fontan procedure of choice, staged Fontan (ie, a bidirectional Glenn followed later by final conversion to Fontan) is preferred, and extracardiac conduit is preferable to lateral tunnel for diversion of inferior vena caval blood into the pulmonary artery.

However, some studies indicated equal efficacy with these 2 methods of Fontan conversion. [124]  The author originally preferred lateral tunnel Fontan conversion because creation of fenestration, if necessary, is easier to perform and the lack of growth potential for the extracardiac conduit. However, it has been observed that the creation fenestration is equally easy with extracardiac conduits.

Current surgical approaches

The patient's age, weight, and anatomic and physiologic status determine the types of surgery recommended. The overall objective is to achieve a staged total cavopulmonary connection.

Stage I

In neonates and young infants with pulmonary oligemia, modified Blalock-Taussig shunting is undertaken to improve the pulmonary oligemia.

Stage II

In patients aged 6 months to 1 year, Blalock-Taussig shunting and bidirectional Glenn procedure are the choices. The author prefers the bidirectional Glenn operation at this age. Some clinicians perform bidirectional Glenn procedures in patients as young as 3 months; however, the probability of failure is increased at this young age presumably because of pulmonary vascular reactivity.

For children aged 1-2 years, initial bidirectional Glenn procedure is preferable, with intent to attempt a Fontan conversion later.

Stage III

For patients older than 2 years, total cavopulmonary connection may be performed, but most authorities suggest staging by using an initial bidirectional Glenn operation followed by Fontan conversion in 6-12 months.

At the time of bidirectional Glenn surgery, any narrowing of the pulmonary artery should be repaired. Issues related to subaortic obstruction and mitral valve regurgitation should also be addressed.

Before Fontan conversion, cardiac catheterization should be undertaken to ensure normal anatomy and pressure of the pulmonary artery as well as normal left ventricular end-diastolic pressure (LVEDP). At the same time, aortopulmonary collaterals should be evaluated by means of selective subclavian artery and descending thoracic aortic angiography. If collateral vessels are present, they should be occluded with coils. Some authors question routine use of pre-Fontan catheterization and suggest prospective evaluation of this issue. [125]

At the time of Fontan conversion, most surgeons currently prefer extracardiac conduit diversion of inferior vena caval blood into the right pulmonary artery. To address the growth issue related to extracardiac Fontan surgery, some surgeons use autologous [126, 127] or bovine pericardial roll grafts. [128]

In patients with associated transposition of the great arteries, early banding of the pulmonary artery, relief of aortic coarctation (if present), and bypassing (by means of a Damus-Kaye-Stansel procedure) or resecting the subaortic obstruction should be incorporated into the management plan. [29]

Some patients with tricuspid atresia have rare associated abnormalities such as truncus arteriosus, [129, 130] aortopulmonary window, [131] absent pulmonary valve syndrome, [132] vascular ring, [133] or anomalous pulmonary venous drainage. [130] Initial successful palliation and eventual Fontan conversion have been undertaken in such patients. [129, 130, 131, 132, 132, 133] Also, patients with left ventricular noncompaction [134, 135] have had successful Fontan palliation. Consequently, the author recommends that all patient with tricuspid atresia, irrespective of associated abnormalities, should have initial palliation while addressing the associated cardiac defect and subsequent staged Fontan conversion.

Emerging therapies

Staged cavopulmonary connection is currently recommended for achieving Fontan circulation. Konertz et al proposed a staged surgical-catheter approach. [136] They initially perform a modified hemi-Fontan that is later completed by transcatheter method. [136, 137] This approach reduces the total number of operations required.

The modified hemi-Fontan operation involves the usual bidirectional Glenn technique. The lower end of the divided superior vena cava is anastomosed to the undersurface of the right pulmonary artery. The superior vena cava is then banded around a 16-gauge catheter with 6-0 Prolene suture slightly above the cavoatrial junction. A lateral tunnel with a Gore-Tex baffle is created to divert the inferior vena caval blood toward the superior vena cava. The baffle is then fenestrated with 3-5 holes 5 mm wide. Thus, the first stage achieves a physiologic bidirectional Glenn condition.

At the time of the second stage, the transcatheter stage, the superior vena caval constriction is dilated with a balloon, and fenestrations are closed with devices or by placement of covered stent.

The original physicians [136, 137] and other workers [138, 139] have performed these procedures in a limited number of patients, and preliminary data suggest that the usual post-Fontan complications, such as pleural effusion and ascites, have not occurred with this approach. Scrutiny of results of larger experience and longer-term follow-up and ready availability of covered stents are necessary for routine application of this innovative approach.

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Consultations

When neurologic complications such as strokes or brain abscesses develop, consultation with neurologist or neurosurgeon is advisable for appropriate guidance of therapy.

At some time, patients may require catheter-directed therapy. Examples include balloon and/or blade atrial septostomy, static dilation of the atrial septum, balloon pulmonary valvuloplasty, balloon angioplasty of aortic coarctation, implantation of stents, coil, plug or device occlusion of collateral vessels, transcatheter closure of ASDs and/or fenestrations and creation of fenestration. Consultation with an interventional pediatric cardiologist is needed to determine the feasibility and timing of the procedures and to perform them.

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Diet

In most patients with tricuspid atresia, no dietary restrictions are necessary. In patients with heart failure, fluid and salt restriction may be appropriate.

When fluid is administered by means of an intravenous and/or nasogastric route, only maintenance quantities of 100 mL/kg/day should be given. When the infant feeds independently, ad lib feedings are suggested; thirst and hunger mechanisms control intake, and the infant is unlikely to become overloaded.

Salt restriction is advised for patients with heart failure. Because food without salt is unpalatable, patients may be allowed a diet with regular salt but with a slight increase in diuretic therapy. This approach is taken in the interest of encouraging adequate caloric intake; however, adding salt to foods with a high salt content is not advised.

In infants with heart failure and failure to thrive, a high-calorie formula (24, 27, or even 30 cal/oz.) may be needed to ensure adequate weight gain. Lowered amounts of diluent (water) combined with glucose polymers (Polycose) and medium-chain triglycerides may be used.

A diet rich in medium-chain triglycerides may be useful in postoperative patients with chylothorax.

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Activity

Exercise

No specific exercise restrictions are recommended. Allow patients to set their own pace of activity. Participation in scholastic physical education is not discouraged, but patients may set their limits of tolerance.

Patients with tricuspid atresia, with or without corrective surgery, are not able to participate in competitive sports.

Lifestyle implications

Even after effective palliation or successful Fontan surgery, patients are left with single-ventricle physiology (ie, one functioning ventricle). These patients tolerate normal activity, but they may not be able to participate in highly exertional activities. Changes in lifestyle are necessary to avoid highly exertional activities, such as professional sports or work that requires substantial physical activity. [140]

Traveling or living at altitudes higher than 5000 feet should be undertaken carefully and with consultation and approval from the cardiologist.

Pregnancy

Significant hemodynamic and hematologic changes occur during normal pregnancy. Cardiac output increases, initially due to an increase in stroke volume and later due to an increase in heart rate. Most of the increase takes place in the first 30 weeks' gestation, and the change is gradual.

Hematologic changes include increases in blood volume of up to 150% of pregestational values. Plasma volume is increased higher than the red cell volume with a consequent decrease in the hematocrit. Changes in regional blood flows are well documented. Uterine (and placental) blood flow increases 10-fold, and renal flow increases by 30%. Although peripheral vasodilatation occurs, the blood pressure remains constant because of increased cardiac index.

Maternal hyperventilation also occurs to meet increasing oxygen demands by the fetus. During the third trimester, the gravid uterus may compress the inferior vena cava, especially in the supine position, and decrease cardiac output.

Patients with significant cyanotic heart disease poorly tolerate the pronounced cardiovascular changes associated with pregnancy. Complications include hemoptysis, pulmonary embolism, stroke, and death.

Data from patients with tricuspid atresia not operated on or palliated are sparse. The maternal mortality rate is 14%, and miscarriages occur in 54% of patients. A relatively high incidence of prematurity, small-for-gestational-age babies, and perinatal mortality have been reported.

Data are even more limited after a Fontan procedure. The limited data suggest that acceptable outcomes may result if the left ventricular function is excellent and if the functional New York Heart Association (NYHA) class is I or II at the time of conception. Further data must be obtained to make any definitive recommendations for patients who have undergone a Fontan procedure. [82]

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Long-Term Monitoring

Most follow-up care of tricuspid atresia is outpatient-based. Inpatient care may become necessary to treat postoperative complications.

Hospitalization and inpatient care may be required for medical, transcatheter, or surgical treatment of arrhythmias; for relief of obstructed Fontan pathways by means of transcatheter or surgical methods; for transcatheter or surgical closure of residual shunts; and/or for treatment of issues related to protein-losing enteropathy.

Periodic follow-up after surgical correction is necessary. Some children need continued support with inotropes and/or diuretics. Angiotensin-converting enzyme inhibitors to effect afterload reduction are generally prescribed to augment left ventricular output with consequent improvement of forward pulmonary flow. Although this concept has theoretical advantages, no data from control studies are available to substantiate this thesis.

Anticoagulation with warfarin is recommended because of risk of thrombi development in the right atrium in the conventional Fontan procedure. Whether such recommendation is valid for total cavopulmonary connection is not clear. As an alternative, platelet-inhibiting doses of aspirin 5-10 mg/kg/d in children or clopidogrel 75 mg/d particularly in adults may be used.

Post-Fontan surgery complications

Whereas most patients do well after Fontan surgery, some develop problems, including arrhythmia, obstruction at the anastomotic sites or in the pulmonary artery, residual shunts, and/or systemic venous congestion (eg, protein-losing enteropathy). [141, 142, 143, 144, 145, 146]

Arrhythmias

Atrial flutter and/or fibrillation and supraventricular tachycardia should be treated with appropriate pharmacologic therapy. If arrhythmias are not adequately controlled, electrophysiologic study and transcatheter or surgical ablation (Maze procedure) may be indicated. Revision of the classic Fontan procedure to total cavopulmonary connection with elimination of the enlarged right atrium should also be considered; some reports indicate success with this approach. [147] Atrioventricular block and sick sinus syndrome may be observed in some children; in such patients, pacemaker insertion may be necessary. Ventricular arrhythmias are infrequent.

Obstructed Fontan pathways

Any signs and symptoms suggestive of obstruction in the Fontan circuit should be promptly investigated. Poor echo windows may make this evaluation difficult. MRI and magnetic resonance angiography (MRA) may be helpful in defining these obstructions. Cardiac catheterization and angiography may be needed to confirm or exclude such obstruction. If notable obstruction is present, balloon angioplasty, stent placement, and, if necessary, surgery should be undertaken to provide prompt relief. [40]

Residual shunts

Residual atrial defects and/or intentional Fontan fenestration may cause substantial arterial hypoxemia. Cerebrovascular accidents and systemic emboli are reported, presumably due to paradoxical embolism. In these situations, transcatheter occlusion of the fenestration is recommended. Test occlusion of the defect should be performed to ensure adequate cardiac output. Most such defects are amenable to transcatheter occlusion. Regular interatrial fenestrations and residual atrial septal defects (ASDs) can be transcatheter occluded by the conventional atrial septal closing devices. Tubular fenestrations such as those associated with an extracardiac Fontan operation may require coils or other devices (eg, Amplatzer duct occluder or Amplatzer vascular plug; AGA Medical, Golden Valley, Minnesota). [91, 96, 97]

Residual systemic to pulmonary or systemic venous collaterals

Residual systemic to pulmonary or systemic venous collaterals that might result in additional left ventricular volume load or residual right-to-left shunting, respectively. These are also treatable with coil or vascular plug occlusion. Late follow-up results after fenestration closure appear encouraging. [93]

Chronic systemic venous congestion

Protein-losing enteropathy, recurrent pleural effusion, and liver dysfunction are noted in a small percentage of patients after Fontan surgery. [148, 149, 150] Protein-losing enteropathy results in a high mortality rate. Its etiology is unknown.

Although protein-losing enteropathy has been reported in total cavo-pulmonary connection type of Fontan, prevalence in the total cavo-pulmonary group may be lower (1.2%) than that seen in atriopulmonary connection type (11.1%) of Fontan. [150, 151, 152]

Loss of protein in the bowel appears to be secondary to lymphatic distension, which may be due to elevated systemic venous pressure. However, this phenomenon is also observed in patients with normal pressures for the Fontan circuit. Symptoms usually manifest 6 months or later after a Fontan procedure. Diarrhea, edema, ascites, and/or pleural effusions are the usual findings at presentation. Hypoalbuminemia and elevated alpha-1-antitrypsin levels in the stool are present. Confirmation of the syndrome with technetium 99m-labeled human serum albumin scintigraphy may be useful. [153]

Obstruction in the Fontan circuit must be evaluated for and, if present, relieved by means of transcatheter or surgical therapy. Likewise, aortopulmonary connections [151, 154] and naturally occurring or previous surgical shunts should be sought out and closed with transcatheter methods or surgery. A diet of medium-chain triglycerides and the administration of parental albumin are supportive. Replacement of immunoglobulins may also be considered. Prednisone, [155, 156] an elementary diet, [157] calcium replacement, [158] regular high-molecular-weight heparin and low-molecular-weight heparin, [159, 160, 161] somatostatin, high-dose spironolactone, [162] sildenafil, [163] and resection of localized intestinal lymphangiectasia (if demonstrated) [164] have been used with variable success.

Reduction of right atrial pressure by creating an ASD has been helpful in some patients. Numerous reports show success with this method. [165, 166, 167]

Protein-losing enteropathy is a potentially fatal complication and should be aggressively treated. In patients with the atriopulmonary type of Fontan, conversion to total cavopulmonary connection may be helpful, [58, 168] although such converting operations are likely to have high mortality rates. Cardiac transplantation should also be considered. Numerous centers have reported improvement after transplantation. [169]

In patients with so called "failed Fontan," after excluding and addressing obstructions and residual shunts apart from other conventional treatment, consideration for right atrial and left ventricular (atrioventricular sequential) pacing, [170, 171] conversion of atrioventricular Fontan to total cavopulmonary anastomosis, [58, 147] and/or cardiac transplantation [172, 173] should be considered.

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