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Laks H, Marelli D, Plunkett M, Odim J, Myers J. Adult Congenital Heart Disease.
In: Cohn LH, Edmunds LH Jr, eds. Cardiac Surgery in the Adult. New York: McGraw-Hill, 2003:13291358.

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Chapter 56

Adult Congenital Heart Disease

Hillel Laks/ Daniel Marelli/ Mark Plunkett/ Jonah Odim/ Jeff Myers

GENERAL MANAGEMENT
????Preoperative Evaluation
????Procedures Requiring Reoperation
????Myocardial Protection
????Postoperative Care
????Associated Procedures
SPECIFIC CONGENITAL MALFORMATIONS
????Atrial Septal Defects
????????ANATOMY
????????PHYSIOLOGY AND INDICATIONS FOR SURGERY
????????OPERATIVE PROCEDURE
????????OUTCOMES
????Ventricular Septal Defects
????????ANATOMY
????????PHYSIOLOGY
????????PREOPERATIVE EVALUATION
????????INDICATIONS FOR SURGERY
????????OPERATIVE PROCEDURE
????????OUTCOMES
????Patent Ductus Arteriosus
????????NATURAL HISTORY AND INDICATIONS FOR SURGERY
????????OPERATIVE PROCEDURE
????Coarctation of the Aorta
????????ANATOMY
????????NATURAL HISTORY AND INDICATIONS FOR SURGERY
????????PREOPERATIVE EVALUATION
????????OPERATIVE PROCEDURE
????????OUTCOMES
????Tetralogy of Fallot
????????ANATOMY
????????PHYSIOLOGY
????????INDICATIONS FOR SURGERY
????????PREOPERATIVE EVALUATION
????????OPERATIVE PROCEDURE
????????OUTCOMES
????Pulmonary Atresia with Ventricular Septal Defect and Major Aorta-to-Pulmonary Artery Collaterals
????????ANATOMY
????????PHYSIOLOGY
????????PREOPERATIVE EVALUATION
????????INDICATIONS FOR SURGERY
????????STAGED SURGICAL REPAIR
????????OUTCOMES
????Late Reoperations for Transposition of the Great Arteries
????????ANATOMY AND PHYSIOLOGY
????????PREOPERATIVE EVALUATION
????????OPERATIVE PROCEDURE
????????RESULTS
????Single Ventricle
????????ANATOMY
????????PHYSIOLOGY
????????INDICATIONS FOR SURGERY
????????GLENN SHUNT
????????MODIFIED FONTAN PROCEDURE
????Late Reoperations After Modified and Classic Fontan Procedures
????Ebstein's Anomaly
????????ANATOMY
????????PHYSIOLOGY
????????PREOPERATIVE EVALUATION
????????INDICATIONS FOR SURGERY
????????OPERATIVE PROCEDURE
????????OUTCOMES
????Heart Transplantation
????????PREOPERATIVE EVALUATION
????????OPERATIVE PROCEDURE
????????OUTCOMES
REFERENCES

?? INTRODUCTION
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The steady rise in individuals who have survived congenital heart disease (with or without treatment) into adulthood is expected to create special cardiovascular issues that mandate strategic collaborative care protocols for this swelling subpopulation. If one neglects all patients born before 1990 and those not diagnosed in the first year, assuming stable mortality in early adulthood, nearly 760,000 adults will have congenital heart disease by 2020.1,2

Adults with congenital heart disease who are referred for surgery fall into three general categories: those without previous surgery, those with previous palliation, and those with complete physiological or anatomical repair returning for revision of their repair because of residual defects or sequelae from their repairs.1,2

In the current era, there is a trend toward surgical correction of congenital heart defects in the neonatal period or during infancy. This approach aims at minimizing the long-term consequences of congenital heart defects, such as myocardial dysfunction, endocarditis, and the hematologic and cerebral complications of cyanosis.36

There are, however, some patients who present as adults (particularly from underdeveloped countries) without previous surgery. More common lesions in this category include aortic valve disease, coarctation, pulmonary stenosis, atrial septal defect, and patent ductus arteriosus. Less commonly seen are tetralogy of Fallot, ventricular septal defect, Ebstein's anomaly, and coronary arteriovenous (AV) fistulae. Palliated adults are also unusual and include patients with systemic to pulmonary artery shunts, Glenn cavopulmonary shunts, and pulmonary artery bands. The largest group includes adults who present with residual lesions or sequelae from previous surgeries. These conditions may include patch leaks, recurrent valvular or ouflow tract stenoses, recurrent coarctation, pulmonary valve regurgitation, valve stenosis after tissue valve replacement or homograft insertion, or aneurysm formation in the pulmonary artery or aorta. Both primary repair and redo procedures are frequently complex and at increased risk from long-standing abnormal physiology and hemodynamics. Surgical care of the adult with congenital heart disease requires a multidisciplinary team experienced in pediatric and adult cardiology and cardiac surgery.


?? GENERAL MANAGEMENT
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Preoperative Evaluation

The natural history of the congenital defect, the sequelae of previous surgical interventions, and the development of newly acquired cardiovascular disease mandate thorough evaluation during preoperative surgical planning. Long-standing cyanosis and pressure or volume overload may all result in right or left ventricular dysfunction and secondary valvular regurgitation that may require repair. Additionally, cyanosis and underperfusion of the lungs may cause the development of aortopulmonary collaterals that may require coil embolization prior to reoperation. Pulmonary vascular resistance is usually affected by long-standing excessive flow or by severe underperfusion and, in older patients, by ongoing pulmonary thromboembolism. Older patients may also develop coronary artery disease that requires concomitant revascularization.

Transthoracic or transesophageal echocardiography provides excellent information regarding the segmental and morphologic cardiac anatomy. Additionally, hemodynamic data can assist in evaluating valvular function, stenosis, and direction of shunting. In most complex cases, cardiac catheterization is required and pulmonary vascular resistance is calculated directly. Quantitative perfusion lung scans are useful to assess right- and left-sided blood flow. MR angiograms with three-dimensional reconstruction can provide excellent views of anatomy and are particularly helpful in preparation for redo coarctation procedures to delineate the aortic arch and descending aorta.

Procedures Requiring Reoperation

Redo median sternotomy can be hazardous and result in massive hemorrhage if thin-walled vascular structures are adherent to the posterior sternum. We obtain CT scans in select patients to view the retrosternal structures. In some patients, the femoral artery and vein are exposed prior to sternotomy. If the aorta, right atrium, or a conduit is adherent to the back of the sternum, cardiopulmonary bypass (CPB) may be initiated with femoral artery and vein cannulation using thin-walled cannulae. If the right atrium or right ventricle is entered inadvertently, an intracardiac defect such as ASD or VSD could result in catastrophic systemic air embolism. This complication can be avoided by instituting deep hypothermia with CPB, keeping the heart full, and discontinuing the cardiac dissection until the heart fibrillates. In the presence of aortic regurgitation, decompression of the left ventricle is accomplished by cannulating the left ventricular (LV) apex via a small submammary incision. Bleeding may be a problem during chest opening due to the presence of numerous large thin-walled vessels throughout the mediastinum that are commonly found in chronically cyanotic patients. Chronic cyanosis and polycythemia are associated with a coagulopathy due to platelet dysfunction. Chronic hepatic congestion affects the coagulation factors and exacerbates coagulopathy. The use of antifibrinolytic agents such as aprotinin, aminocaproic acid, or tranexamic acid is considered in high-risk patients except when circulatory arrest is used. Autologous blood donation (when not contraindicated) and the use of cell-saving devices are routine.

A polytetrafluoroethylene (Gore-Tex) pericardial substitute is used at closure for patients requiring later surgery, such as palliative procedures or after use of homografts or tissue valves. This membrane facilitates the dissection of reentry and the risk of injuring a retrosternal structure is markedly reduced.

Myocardial Protection

Right and left ventricular hypertrophy, dilatation, or dysfunction may be present. Noncoronary collateral flow is usually increased and bronchial flow can be torrential. Myocardial protection is therefore critical to the outcome of complex procedures. A left ventricular vent and deeper hypothermia to 24?C are required, and both antegrade and/or retrograde cardioplegia are given every 10 minutes. A purse string is placed around the coronary sinus to improve retrograde distribution.

Postoperative Care

The adult with congenital heart disease may present a far more complex postoperative course than the usual adult with acquired heart disease. Both right and left ventricular function may be compromised and require support. Both pulmonary and systemic vascular resistance may require manipulation. For this reason, right atrial (RA), pulmonary artery (PA), and left atrial (LA) pressure-monitoring lines are used when indicated. Left atrial lines are used rather than attempting to obtain PA wedge pressures because of the danger of PA rupture and possible discrepancy between PA diastolic pressures and LA pressure. Transesophageal echocardiography is required postoperatively to assess left and right ventricular function, to evaluate left- and right-sided valve function, and to look for shunts. Ventilator control to reduce PCO2, inhaled nitric oxide, and milrinone are useful to reduce pulmonary vascular resistance.

Associated Procedures

Many adults with congenital heart disease require corrective surgery after associated procedures. In particular, all patients above the age of 40 years require preoperative coronary angiography. Due to a history of shunts or increased chest wall collaterals from cyanosis, many patients have aortic insufficiency secondary to increased venous return to the systemic ventricle. Such patients should be considered for aortic valve repair.7

Other associated procedures often performed in adults with congenital heart disease include bicuspid aortic valve repair or replacement, mitral or tricuspid valve repair or replacement, and implantation of epicardial pacemaker leads and generator.815

Our preferred approach for epicardial pacemaker implantation is a subxyphoid approach. Atrial tissue is easily identified near the inferior vena cava in most patients. A ventricular site is usually identified on the diaphragmatic surface of the heart. We prefer nonpenetrating steroid-eluding leads that are sutured onto the epicardium. The pacemaker battery is placed in the preperitoneal position beneath the fascia or in the subcutaneous tissues of the left upper quadrant. Transvenous leads are generally contraindicated in the systemic circulation because of the risk of thromboembolism. This is of particular importance in patients with single ventricle physiology.


?? SPECIFIC CONGENITAL MALFORMATIONS
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Atrial Septal Defects

ANATOMY

Atrial septal defects (ASD) of the secundum type are the most common lesions, but sinus venosus defects of both the superior and inferior vena caval types as well as ostium primum atrial septal defects are seen in adults (Fig. 56-1).



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FIGURE 56-1 Diagram showing the locations of the most common types of atrial septal defects. Sinus venosus defects are close to the superior vena cava (SVC) to right atrial (RA) junction and are frequently associated with partial anomalous pulmonary venous drainage (not shown). The right superior pulmonary vein may drain directly into the SVC or at the junction of the SVC and RA.

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PHYSIOLOGY AND INDICATIONS FOR SURGERY

The amount of left-to-right atrial shunting is variable. In older patients, as right ventricular dysfunction and tricuspid regurgitation develop, the degree of left-to-right shunting may decrease. The left-to-right shunt may increase in other patients due to hypertension and reduced LV compliance. Although most patients with an ASD are asymptomatic through the second decade, by the third or fourth decade adults commonly develop atrial fibrillation or a reduction in exercise tolerance, and eventually heart failure.1622 It is preferable to close the defect when the diagnosis is made, before the development of these sequelae. As long as the Qp:Qs ratio (pulmonary flow to systemic flow) is greater than 1.5:1 and the calculated pulmonary vascular resistance (PVR) is less than 6 to 8 units/m2 (or Wood units/m2), depending on systemic vascular resistance at the time of measurement, closure is usually indicated.

About 15% to 20% of children with an ASD eventually develop pulmonary vascular disease.2325 If it does not occur by the end of the second decade, it is very unlikely to occur. Pulmonary vascular disease eventually causes reversal of the shunt and development of hypoxia that may be intermittent and severe depending on right ventricular (RV) function and tricuspid regurgitation. Patients with a patent foramen ovale and systemic embolization are candidates for closure of the defect. In the past, patients with pulmonary vascular disease were considered inoperable and were candidates for eventual lung transplants and ASD closure. The use of long-term prostacyclin (Flolan) has allowed some patients to lower their pulmonary vascular resistance and develop a left-to-right shunt. We have successfully proceeded to ASD closure and continued prostacyclin therapy in some of these patients.

OPERATIVE PROCEDURE

The operation is performed using cardiopulmonary bypass and moderate hypothermia. For cosmetic purposes in young women, a submammary skin incision with median sternotomy is used. For the last 5 years we have used a small right anterolateral thoracotomy (Fig. 56-2). Superior and inferior vena cava cannulation is achieved directly through the thoracotomy incision using right-angled metallic-tipped cannulae. If aortic cannulation is difficult, femoral artery cannulation is used. The aorta is clamped and cold blood cardioplegia alternating with cold blood is run continuously to keep the left atrium and ventricle full to prevent air entry. The chest cavity is filled with CO2 throughout the procedure. The ASD is closed either primarily or with a pericardial patch. The right-sided pulmonary veins must all be identified, and if they are draining anomalously, they are baffled to the left side with a pericardial patch. It is also important to identify the inferior vena caval orifice so that it is not inadvertently baffled into the left atrium by sewing the patch to a well-developed eustachian valve. In adults, the redundant right atrial wall is excised and a regurgitant tricuspid valve (if present) is repaired with an annuloplasty. In patients with chronic atrial fibrillation, a Maze procedure is performed. The cleft in the mitral valve is routinely closed in the primum ASD, often combined with an annuloplasty. The suture line is placed on the tricuspid aspect of the defect and outside the conduction tissue, which is left beneath the patch. After closure of the defect and right atrium, extensive de-airing is performed prior to releasing the cross-clamp. This includes syringe and large-bore needles, de-airing of the left atrium, and venting of the aorta. Rarely, if air has entered the left ventricle during the procedure, a small submammary left-sided thoracotomy is made, and a small left ventricular apical vent is also inserted to assist in de-airing. The left ventricle is inspected with TEE for residual air prior to release of the cross-clamp.



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FIGURE 56-2 Patient position for minimally invasive right thoracotomy approach for repair of an atrial septal defect. A 5- to 7-cm incision is made in the submammary crease. An additional incision may be made in the groin crease for femoral artery cannulation.

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Atrial septal defects are closed via a right minithoracotomy with a submammary skin incision. In most adults the aorta is cannulated in the chest. In some cases the femoral artery is cannulated. Carbon dioxide is infused into the chest cavity to reduce the risk of air embolism.

OUTCOMES

Long-term follow-up after repair of isolated ASDs is well documented.2629 When patients are operated on at or before 25 years of age, normal life expectancy is anticipated, but this may not be the outcome when patients are corrected after 25 years, when both right and left ventricular reserve are diminished following a long-standing ASD. However, patients with ASD closure after 40 years of age may still realize improvement of symptoms. At the University of Alabama, it was found that age and New York Heart Association (NYHA) class were fairly well correlated, thus suggesting that ASD closure is indicated when the defect is hemodynamically significant (Qp:Qs >=1.5:1). Older age per se is not a risk factor for operative mortality.

Ostium primum defects are unusual in adults; however, survival similar to that of a large ASD is expected for partial AV canal defects with minimal valvular incompetence. There is also a group of previously operated patients that may present because of left AV valve malfunction following repair at an earlier age. These patients are often successfully treated with a re-repair of the mitral valve, particularly if a residual defect such as a cleft is present in the anterior leaflet. Some will require valve replacement.

Recently, keen interest in the use of intravascular devices to close ASDs has developed.30,31 The Amplatzer device was recently approved by the FDA for clinical use. Follow-up of 5 to 7 years is now available showing good long-term results. This trend will grow as the Amplatzer and CardioSEAL devices are currently approved by the FDA for atrial septal defect closure. Long-term follow-up is pending. There is growing interest in the use of computer-assisted "robotic" closure of these defects. It is presently unclear what role this modality will play in future.

Ventricular Septal Defects

Unrestrictive ventricular septal defects (VSDs) are mostly associated with congestive heart failure in infancy, and are usually repaired in early childhood. Adult survival with an unrestrictive VSD can occur if there is concurrent pulmonary outflow obstruction that restricts pulmonary blood flow, or if the development of severe pulmonary vascular disease reduces or reverses the left-to-right shunt (Eisenmenger's syndrome). Restrictive VSDs are more commonly found in the adult and may be the result of a persistent small defect, partial spontaneous closure of a larger defect, or a residual patch leak following surgical repair.

ANATOMY

Most ventricular septal defects (VSDs) are categorized into four anatomical types. The perimembranous type is the most common. It is located under the septal leaflet of the tricuspid valve. The subarterial VSD is located in the supracristal area and may lie directly beneath the annulus of the pulmonary valve. Because of the proximity of the aortic annulus to such defects, the right cusp of the aortic valve may prolapse into the defect, reducing the effective orifice and limiting the shunt. Aortic regurgitation is frequently associated with this defect.32 With surgical closure of a subarterial VSD, the aortic valve may require repair with suspension of the prolapsing valve leaflet (Fig. 56-3).



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FIGURE 56-3 Technique of aortic valve repair using pericardial pledgetted sutures to resuspend redundant leaflets. Additional apposition can be achieved by adding sutures below the commissures or at the level of the annulus.

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The endocardial cushion type of VSD is located in the inlet of the right ventricle and beneath the septal leaflet and posterior leaflet of the tricuspid valve. This VSD may have associated mitral valve defects such as cleft anterior leaflet and therefore require mitral valve repair at the time of surgery. The repair usually involves suture closure of the anterior leaflet cleft and reduction of the dilated annulus with annuloplasty. The muscular type of VSD may occur anywhere within the muscular ventricular septum. This type is unusual in adults, as it has a tendency to close spontaneously in the first years of life.33

PHYSIOLOGY

Use of the term restrictive implies a pressure gradient between the ventricles and a restriction to flow across the defect. The amount of left-to right shunting across the defect can be quantified by calculation of the Qp:Qs ratio. VSDs with left-to-right shunting that results in a Qp:Qs of less than 1.5:1 are usually not considered for repair, if there is no other associated pathology (e.g., aortic leaflet prolapse and insufficiency).

Double-chambered right ventricle is an obstruction in the mid-portion of the right ventricle that divides the chamber into two segments: a high-pressure lower chamber and a low-pressure upper chamber. The development of obstructive tissue in the right ventricle is a direct result of a restrictive VSD producing a "jet effect" that strikes adjacent myocardium and produces an area of fibromuscular proliferation. Right ventricular hypertrophy develops secondary to the obstruction and contributes to the progression of right ventricular hypertension. In many patients the VSD will eventually close, as the fibromuscular rim of tissue proliferates around and over the defect. In these patients, the VSD is often identified at the time of surgery with resection of the obstructing tissue in the right ventricle.

In patients with large unrestrictive VSDs, the probability of developing severe pulmonary vascular disease is about 50% by the third decade of life.34,35 Consequently, patients eventually die of complications of Eisenmenger's syndrome if their VSDs remain unrepaired. These patients ultimately will become cyanotic as a result of right-to-left shunting, and the only surgical options available at that point are either heart-lung transplantation or lung transplantation with repair of the VSD. A pulmonary vascular resistance greater than 6 units/m2 is considered a high risk for isolated VSD closure. Preoperative long-term prostacyclin (Flolan) infusion may lower the pulmonary vascular resistance in some patients.

PREOPERATIVE EVALUATION

An adult patient with a VSD should be evaluated by chest radiograph, echocardiography, and possibly cardiac catheterization. A chest radiograph may show an enlarged right heart shadow and right ventricular hypertrophy. It may also reveal chronic changes in the pulmonary vasculature prompting further evaluation by catheterization. Transthoracic and transesophageal echocardiography are both useful in defining VSDs anatomically and identifying associated lesions.36 An estimate of the pulmonary artery pressures may also be obtained. Right and left heart catheterization is useful to confirm the anatomical findings and to accurately measure the pulmonary vascular resistence and estimate the Qp:Qs. In patients more than 40 years old or in those with increased risk factors, coronary artery disease must also be excluded by angiography.

INDICATIONS FOR SURGERY

Adults with small restrictive VSDs who are asymptomatic often require no surgical intervention and should receive endocarditis prophylaxis as necessary. In general, if the Qp:Qs is greater than 1.5:1 and the calculated pulmonary vascular resistance is under 6 units/m2, surgical closure of a VSD can be performed safely and is recommended. The development of a double-chambered right ventricle with outflow obstruction is also an indication for operative intervention. The occurrence of infective endocarditis in an adult with a restrictive VSD is a rare but compelling indication for repair of the defect. In adults with VSD and Eisenmenger's syndrome, heart-lung transplantation or lung transplantation with closure of the defect may be considered.

OPERATIVE PROCEDURE

Operative repair of VSDs is performed on cardiopulmonary bypass with moderate systemic hypothermia and blood cardioplegia for myocardial protection. Most adults with a perimembranous, inlet, and/or muscular VSD can have the defect repaired through the tricuspid valve annulus. If the edges of the defect are difficult to visualize due to multiple attachments of the septal leaflet to the edges of the defect, the septal leaflet may be incised at its base adjacent to the annulus, exposing the VSD under the septal leaflet. Subarterial VSDs may be approached through the pulmonary valve or the right ventricular outflow tract. The patch material for closure of VSDs may be synthetic (e.g., Gore-Tex, Dacron, etc.), or glutaraldehyde-treated autologous pericardium, which we have preferred for 10 years. Postoperatively, elevated pulmonary artery pressures may be treated with inhaled nitric oxide. Currently, transcatheter closure of VSDs remains experimental.37,38

OUTCOMES

Long-term follow-up is recommended to monitor pulmonary artery pressures in those adult patients with large VSDs that are repaired late in life.39 This is achieved echocardiographically if there is a mild tricuspid regurgitation from which to calculate the RV pressure or by measuring the pulmonary-valve opening time. The pulmonary vascular resistance (PVR) may continue to rise after VSD closure, resulting in systemic or suprasystemic right ventricular pressures. This may eventually cause the onset of angina, right heart failure, or even sudden death.

At UCLA, we have repaired 52 VSDs in patients aged 16 to 67 years without mortality. In most patients, the VSD repair was combined with additional procedures such as aortic valve repair, pulmonary valve replacement, and tricuspid valve repair. Except in patients with congenitally corrected transposition, the incidence of complete heart block following VSD closure is approximately 1%. Overall, the long-term outcome for these patients has been excellent.

Patent Ductus Arteriosus

NATURAL HISTORY AND INDICATIONS FOR SURGERY

Isolated patent ductus arteriosus (PDA) may present with congestive heart failure by the third or fourth decade of life.40 Occasionally, the ductus may become aneurysmal secondary to flow characteristics. The aortic end of the PDA is usually calcified in the older adult patient. A long-standing left-to-right shunt may lead to pulmonary vascular disease. Cumulative death rate in childhood is about 0.5% per year. This doubles to 1% by adulthood and increases to 2% to 4% by midlife. There is always a risk of endocarditis (regardless of the PDA size) that is dependent on the presence of abnormal flow. Adults with large PDAs may develop Eisenmenger's syndrome and the classic finding of differential cyanosis in the lower body. It is imperative to determine pulmonary vascular resistance and reactivity preoperatively. If the resistance is greater than 6 to 8 units/m2, the ductus should not be closed, and the patient may be considered for lung or heart-lung transplantation.

OPERATIVE PROCEDURE

Surgical closure of a PDA is usually carried out via a small posterior thoracotomy. Thoracoscopic procedures are probably not appropriate for the adult because of the frequency of calcification and the greater risk of rupture while ligating the ductus.41,42 In patients over 40 years of age, or in the presence of severe ductal calcification, consideration should be given to performing PDA ligation via a median sternotomy using cardiopulmonary bypass.43,44 During cooling, the ductus is occluded by finger pressure on the PA and the branch pulmonary arteries are snared. This prevents steal from the descending aorta. The ductus is exposed via an incision in the PA. Using low flow, the ductus is closed on the PA side with horizontal pledgeted mattress sutures, or the PA defect is patched with glutaraldehyde-treated pericardium or a Gore-Tex patch.

Patients with Eisenmenger's syndrome may be candidates for either single or double lung transplantation combined with PDA closure or heart-lung transplantation if there is significant ventricular dysfunction and tricuspid valve regurgitation. Catheter closure using coils or other devices may be possible depending on the size and length of the ductus.4548

Coarctation of the Aorta

ANATOMY

Aortic coarctation in adults usually presents with upper-body hypertension typically in the second or third decade of life.49 Although these patients comprise a selected group who have survived free of complications beyond childhood, long-term complications include aneurysm formation of the aorta and aneurysmal dilatation of intercostal arteries, which may eventually rupture. This latter anomaly is important because the initial portion of these arteries should be occluded at the time of surgery if they appear disproportionately enlarged. Other complications include premature coronary artery disease, left ventricular hypertrophy, aortic dissection and rupture, endocarditis, and intracranial hemorrhage. The aorta distal to the coarctation site is frequently dilated and thin walled, and is prone to late aneurysm formation after repair. There is usually extensive collateral circulation in adults with coartcation. The source is mainly from branches of the subclavian artery and internal mammary arteries, and intercostal chest-wall circulation. Collateral flow into the descending aorta is dependent on enlarged intercostals at the level of the third and fourth ribs beyond the coarctation.

NATURAL HISTORY AND INDICATIONS FOR SURGERY

In adults with coarctation, congestive heart failure may develop from long-standing hypertension. Up to 40% of patients have an associated bicuspid aortic valve that also may become stenotic and/or incompetent.9 If aortic coarctation is left untreated, 90% of patients eventually die by the age of 50 due to cardiac causes or stroke. The oldest patient in our series was 81 years old. Repair also may be required for patients who have had previous coarctation repairs with recurrence or for patients who have developed recurrence after previous balloon aortoplasty. Residual coarctation following repair in childhood is usually due to failure of growth of the anastomosis or technical factors such as a short subclavian flap aortoplasty. In patients who were initially treated with a patch, aneurysm formation may occur and require reoperation. Surgical repair is indicated when the gradient across the coarctation is greater than or equal to 30 mm Hg at rest.50 If the gradient is less and the anatomical obstruction severe, an exercise test will reveal a more severe gradient, and repair is indicated.

PREOPERATIVE EVALUATION

Echocardiography is performed to evaluate the aortic valve, ventricular function and hypertrophy, and the aorta. In adults, magnetic resonance angiography with three-dimensional computerized reconstruction to assess the transverse arch, isthmus, and descending aorta is often useful (Fig. 56-4).



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FIGURE 56-4 Angiogram of a 27-year-old patient who presented with residual coarctation after initial repair in which a 16-mm interposition graft was inserted. She presented with stenosis at the distal transverse arch and at the interposition graft with proximal hypertension. Repair using a left atrial to aortic bypass circuit involved repairing the distal arch and replacing the graft with a 20-mm Gore-Tex conduit and extended anastomosis.

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OPERATIVE PROCEDURE

The preferred method is resection with extended end-to-end anastomosis (Fig. 56-5), although patch repair is used for reoperations or where the collaterals are particularly enlarged and difficult to mobilize.49,5153 Tube-graft interposition is used when indicated to relieve long segments of obstruction. Special precautions are taken to reduce the major risk of spinal cord ischemia. Arterial lines are placed in the upper and lower extremities for monitoring blood pressure during aortic clamping. The distal pressure should be maintained above 50 mm Hg throughout the procedure. Somatosensory evoked potentials (SSEPs) are monitored intraoperatively to aid in the decision to use extracorporeal circulation to help prevent spinal cord ischemia during aortic cross-clamping. In extensive reoperations or operations for aneurysms, the cerebrospinal fluid (CSF) pressure is monitored by catheter, and the fluid is allowed to drain if pressure exceeds 10 cm H2O (essentially central venous pressure). The CSF pressure is monitored for 24 hours postoperatively. The goal is to optimize perioperative perfusion of the spinal cord by increasing the pressure gradient during and after aortic clamping.



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FIGURE 56-5 Resection of coarctation of the aorta. (A,B) With extended end-to-end anastomosis. (C,D) After excision of the coarctation site and reconstruction of the posterior wall by end-to-end anastomosis, a glutaraldehyde-treated autologous pericardial patch is used to enlarge the isthmus and the site of coarctation repair. The patch is measured to avoid excessive dilatation, which can result in late aneurysm formation.

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The patient is placed on a temperature-regulated blanket and cooled to 33?C to 34?C. Cold saline is used to bathe the left chest cavity to aid in cooling, and the room is cooled. Positioning for the left thoracotomy is important in that one must prep and drape the groins to have access to the femoral arteries if left atrial-to-femoral artery (or descending aorta) bypass becomes necessary during the operation. One must carefully identify chest-wall collaterals, which can bleed massively and which must be ligated individually during the thoracotomy.

The aorta is mobilized extensively, and the ligamentum arteriosum is divided and oversewn. Large intercostal branches are identified and encircled in preparation for snaring. On induction of anesthesia, the patient is given 30 mg/kg of methylprednisolone sodium succinate and lidocaine 2mg/1kg IV, as well as 8 g of mannitol. The patient is anticoagulated with 1 mg/kg of heparin. The aorta is clamped when the rectal temperature is 34?C or below. Once the aorta is clamped proximally and distally, if the distal pressure is below 50 mm Hg, distal aortic bypass is instituted. The distal pressure is maintained above 60 mm Hg. This consists of left atrial-to-descending aortic bypass using a centrifugal pump. In more complex redo or aneurysm procedures, pulmonary artery to descending aortic bypass can be used with an oxygenator. Upper extremity pressure is maintained at about 120 mm Hg systolic. Once the aortic coarctation is resected, reconstruction with a tube graft or end-to-end anastomosis is carried out with 4-0 polypropylene suture mounted on a small needle.

For patients undergoing reoperation for recurrent coarctation, mobilization of the aorta for end-to-end anastomosis may be difficult and cause excessive blood loss. In these patients, the aorta is clamped proximal to the left subclavian artery, and a glutaraldehyde-treated autologous pericardial patch may be used to enlarge the aorta from the base of the subclavian artery to the distal aorta. An excessively large patch should be avoided to prevent late aneurysm development.

There should be no gradient between upper and lower extremities upon release of the clamps. During closure, special care is taken to control intrathoracic bleeding and to check chest tube and pericostal suture sites.

Hypertension is controlled and is treated aggressively in the intensive care unit. Abdominal pain and distension may be present in 5% of patients postoperatively. Management is usually conservative. The patient is given nothing by mouth for at least 24 hours postoperatively until bowel sounds return.

In older patients who have a coarctation and also require coronary revascularization, we prefer a median sternotomy approach with cannulation of both the ascending aorta and the femoral artery. After completing the coronary revascularization, an adequately sized Dacron graft can be placed between the ascending aorta and the proximal abdominal aorta through the diaphragm. Another option to consider is a clamshell-type incision to access the descending thoracic aorta and the mediastinum simultaneously.

OUTCOMES

Outcome following repair is generally good, and follow-up may be assisted with transesophageal echocardiogram, computed tomographic (CT) scan, or magnetic resonance imaging (MRI). The latter is useful to detect aneurysm formation or recoarctation.54,55 Recoarctation is defined as a gradient greater than 20 to 30 mm Hg at rest. The possibility of coronary disease and systemic hypertension requires lifelong monitoring.

Catheter-based techniques are being used for both primary coarctations and recurrences.5658 Residual mild gradients are common after stenting primary coarctations and we therefore prefer surgical therapy.49 For selected recurrent coarctations, stenting may be the preferred method provided that an excellent anatomical relief of the obstruction can be achieved.59

Tetralogy of Fallot

Tetralogy of Fallot (TOF) is the most common cyanotic heart defect in children, constituting approximately 10% of all congenital heart disease. It is, therefore, one of the most common cyanotic congenital heart defects found in adults. Successful repair of TOF in childhood has spanned almost four decades, with many of those patients now returning as adults for reoperation.6069 These patients constitute the majority of adults presenting for surgical intervention for TOF. There are also adults with TOF who underwent palliative procedures in childhood but never underwent complete repair of the defect.70 Occasionally, a patient with a well-balanced TOF defect and adequate pulmonary stenosis to protect their pulmonary vasculature will reach adulthood without any operative intervention.

ANATOMY

TOF is classically defined by Fallot's four original pathologic findings: obstruction of the right ventricular outflow, ventricular septal defect, overriding aorta, and right ventricular hypertrophy. The defect is the result of an anterior displacement of the infundibular septum during development, resulting in obstruction to the right ventricular outflow and a malalignment ventricular septal defect. The aorta is displaced toward the ventricular septum resulting in an overriding position, and hypertrophy of the right ventricle is a direct consequence of the outflow obstruction. The obstruction to pulmonary blood flow is often at multiple levels, which may include subvalvular, valvular, and supravalvular stenosis. The pulmonary valve is frequently malformed or bicuspid and the annulus is often small. Hypoplasia of the pulmonary arteries may be present if the obstruction to pulmonary blood flow is severe, and there is often stenosis of the branch pulmonary arteries either primary or secondary to shunts. Aortopulmonary collaterals arising from the aorta may coexist, as is commonly found in patients having pulmonary atresia with ventricular septal defect. Occasionally, cyanotic adults will present with unrepaired TOF and severe pulmonary obstruction but adequate collateral pulmonary blood flow to allow survival beyond childhood.

PHYSIOLOGY

The pathophysiology of TOF results in restriction of pulmonary blood flow secondary to obstruction of right ventricular outflow and right-to-left shunting across the ventricular septal defect. The age at presentation and the degree of cyanosis vary directly with the degree of obstruction to pulmonary blood flow. Patients undergoing palliative shunt procedures in childhood to increase pulmonary blood flow may do quite well if the resulting oxygenation remains adequate with subsequent growth. These systemic- to-pulmonary shunts are often outgrown at an early age, requiring reintervention for additional palliation or more definitive repair. While currently the modified Blalock-Taussig shunt or central shunt is used for palliation in most patients, the Potts (ascending aorta-to-right pulmonary artery) and Waterston (descending aorta-to-left pulmonary artery) shunts were used in the past, and may be found in some adult patients.

INDICATIONS FOR SURGERY

Residual VSDs, residual or recurrent obstruction to pulmonary blood flow, and severe pulmonary insufficiency with progressive right ventricular dilatation and dysfunction are all indications to consider reoperation in adults who have undergone previous complete repair of TOF. Patients who have previously undergone right ventricle-to-pulmonary artery conduit placement often present later in life with conduit stenosis requiring replacement. Patients who have had TOF repair in late childhood may have other sequelae that may have an impact on late reoperative surgery. The long-term volume load from a large shunt may produce permanent left ventricular dysfunction. The pulmonary vascular resistance may be elevated. Mild or even moderate aortic valve regurgitation is not uncommon due to dilatation of the aorta and may require aortic valve repair or replacement.

Pulmonary valve regurgitation is very common after repair of TOF, since approximately 70% to 80% are repaired with a transannular patch. Even though exercise capacity may be decreased, the vast majority of patients tolerate this well unless they have an additional residual VSD or pulmonary artery stenosis. In addition, some patients, without these associated residual defects, will slowly develop right ventricular dilatation and severe tricuspid regurgitation. This may progress for over 20 years, and patients are currently presenting late as they become symptomatic from combined pulmonary and tricuspid valve regurgitation. In view of the risks of sudden death and the progressive nature of right ventricular dysfunction, surgical intervention is recommended.

Aneurysms of the right ventricular outflow tract may occur following the use of an excessively large transannular pericardial patch with the initial repair (Fig. 56-6). Such aneurysmal dilatation may progress, especially if there is associated right ventricular outflow tract obstruction.



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FIGURE 56-6 A three-dimensional magnetic resonance image documenting aneurysmal dilatation of the right ventricular outflow tract in a patient with previous repair of tetralogy of Fallot.

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Sudden death after TOF repair accounts for a significant number of late deaths. It usually occurs in patients who have had a right ventricular incision and have right ventricular dilatation combined with an elevated right ventricular pressure above 60 mm Hg.71 All else being equal, a QRS duration of greater than 180 milliseconds is associated with an increased risk of sudden death and a progressive increase in QRS duration is considered a factor in deciding on reoperation.72 Any ventricular arrhythmias should be evaluated by electrophysiological studies and focal pathways should be treated with catheter ablation. Pacemakers are required in less than 4% of patients late after TOF repair.73 They may be indicated for sick sinus syndrome, the combination of right bundle branch block with left anterior hemiblock, or the late onset of complete heart block.

PREOPERATIVE EVALUATION

The preoperative evaluation in adults with TOF must take into consideration the patient's previous operative interventions. In addition to chest radiography and electro-cardiography, transthoracic or transesophageal echocardiography has become the fundamental diagnostic tool in most of these patients. Angiography is indicated to define specific hemodynamics such as the pulmonary vascular resistance and to define the pulmonary artery anatomy. Aortic and selective injections are performed to look for aorta-pulmonary collaterals. The coronary anatomy must be known as 4% to 5% of TOF patients have an LAD arising from the right coronary artery, which can be injured by a transannular incision. Adults over age 40 and those with risk factors for early coronary artery disease should undergo coronary angiography to evaluate the need for concomitant coronary bypass. Magnetic resonance imaging with angiography (MRA) or computerized tomography (CT) scans with three-dimensional reconstruction have proven valuable for defining the anatomy. In all patients with TOF, there should be an evaluation of the size and patency of the pulmonary trunk and its branches, the size of the pulmonary annulus, the stenosis and/or competency of the pulmonary valve, the proximal coronary artery location, and the presence of systemic-to-pulmonary artery collaterals. If there is a right ventricle-to-pulmonary artery conduit or an aneurysmal transannular patch present, MRA or CT imaging studies should be used to evaluate the proximity of these structures to the sternum and to the midline site of the redo sternotomy. Preoperative electrophysiological studies may be indicated if significant arrhythmias are identified.

OPERATIVE PROCEDURE

In most adults with TOF, bicaval cannulation is used, and myocardial protection involves both antegrade and retrograde cold-blood cardioplegia followed by warm-blood cardio-plegia and warm-blood reperfusion. Ventricular distension is avoided with venting of the left ventricle. Atrial septal defects should be sutured primarily or closed with a patch of native pericardium. Ventricular septal defects may be approached through the right atrium or through the right ventricular scar or outflow tract patch. A Gore-Tex or glutaraldehyde-treated pericardial patch may be used. The right ventricle is remodeled by resection of scar from the previous ventriculotomy and any aneurysmal tissue in the right ventricular outflow tract. The pulmonary valve is replaced, usually with an oversized porcine bioprosthetic valve seated below the native annulus in the right ventricular outflow tract (Fig. 56-7). A 27- or 29-mm porcine valve can usually be accommodated in all adults. A transannular hood of pericardium or Gore-Tex is used to cover the porcine valve and establish continuity to the pulmonary artery. Homografts are used to replace previously inserted conduits (Fig. 56-8). The pulmonary homograft lasts longer than the aortic homograft, but develops regurgitation earlier. The tricuspid valve can usually be repaired with an annuloplasty and rarely requires replacement. In some cases adherence of the septal leaflet to the VSD patch can be repaired by suturing it to the adjacent anterior and posterior leaflets.



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FIGURE 56-7 Pulmonary valve replacement with patch enlargement of the right ventricular outflow tract. The incision extends across the annulus and beyond the bifurcation to the left pulmonary artery. The outsized porcine valve (27 or 29 mm) is placed within the RV outflow tract to accommodate the larger size valve.

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FIGURE 56-8 Homograft replacement of the right ventricular outflow tract with hood augmentation of the proximal anastomosis and patch enlargement of the branch pulmonary arteries. A reinforced Gore-Tex conduit may be placed behind the aorta to reestablish continuity between right and left pulmonary arteries.

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Definitive procedures in adults may require takedown of previously placed shunts. Systemic-to-pulmonary artery type shunts should be controlled as soon as cardiopulmonary bypass is instituted. Takedown of a Waterston shunt is done from within the pericardium. The right pulmonary artery is mobilized. The aortic cannulation site for cardiopulmonary bypass is placed distally. When cardiopulmonary bypass is initiated, shunt flow is controlled with a clamp flush with the aorta, and the patient is cooled to 20?C. Cardioplegia is administered after aortic clamping. With the heart arrested and at low flow, the shunt clamp is released, and the anastomosis is excised from the aorta. This mobilizes the right pulmonary artery. The aorta is closed primarily. The incision in the right pulmonary artery is extended proximally and distally to relieve any stenoses. The right pulmonary artery is reconstructed with a pericardial or Gore-Tex patch. Takedown of a Potts anastomosis usually requires a period of low flow or circulatory arrest.74 The shunt is occluded by pressure on the left pulmonary artery while blood is cooled to below 20?C. With the head down, the left pulmonary artery is incised and opened under low flow so that the opening to the aorta can be occluded with a Hegar dilator. Under low flow or circulatory arrest, the aortic side is closed primarily, and the pulmonary artery is repaired with a pericardial patch (Fig. 56-9). Takedown of a Blalock-Taussig shunt is usually achieved by dissection, ligation, and division of the shunt at the initiation of cardiopulmonary bypass.



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FIGURE 56-9 Patch repair of a Potts shunt anastomosis. The main and left pulmonary arteries are incised (A) to expose the opening (B) in the posterior proximal left pulmonary artery. The defect is closed with a pericardial or prosthetic patch (C).

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OUTCOMES

Long-term results are well documented in patients who had complete repair of TOF in the late 1950s and early 1960s.7580 Actuarial survival ranges from 77% to 90% at between 20 and 30 years of follow-up. Late mortality from cardiac causes accounts for about two thirds of all late deaths. Between 40% and 60% of these are sudden and presumed to be due to arrhythmias or to heart block. Other causes include right ventricular outflow abnormalities (obstruction, pulmonary incompetence, aneurysm) and congestive heart failure partly related to residual VSDs, which are reported in 1% to 8% of all operated patients in these early series. Currently, residual VSDs are expected in less than 5% of repairs for TOF.

The results of primary repair of tetralogy in adults are very good. Presbitero et al reported an operative mortality of 2.8% in a series of 40 adults with TOF repairs.75 There were two residual VSDs and two patients with residual RVOT obstruction. The results of reoperative surgery in adults with TOF are also generally good. Mortality ranges from 7% to 20%. Pome et al reported an actuarial survival of 87% for 22 patients at 20-year follow-up for this particular cohort.68 Eighty-nine percent of patients were NYHA class I, and only 1 patient (5.5%) was in class III. Two women in this series experienced uncomplicated pregnancy. In the series from the Mayo Clinic reported by Uretzky et al, 5 patients (12%) had a second reoperation.79

At UCLA, we have operated on adults with TOF ranging in age from 16 to 65 years. Most had patch repairs of the ventricular septum, insertion of a right ventricular outflow patch, and pulmonary valve replacement. The oldest TOF patient underwent primary repair and coronary bypass grafting. There was no mortality or significant morbidity in this series.

Pulmonary Atresia with Ventricular Septal Defect and Major Aorta-to-Pulmonary Artery Collaterals

Patients with this complex lesion sometimes survive to adulthood without surgery because of adequate pulmonary blood flow from collaterals. Others have shunts or unifocalization procedures or complete repairs.8183

ANATOMY

The true pulmonary arteries may be absent, hypoplastic, and continuous or discontinuous. The collaterals may be the dominant or only blood supply to the lung or supply only lesser areas of the lung. The VSD is subaortic and usually single. Depending on previous shunts, there may be stenoses in the proximal or distal pulmonary arteries. If repaired there may be a residual VSD, obstruction between RV and PA, and tricuspid valve regurgitation.

PHYSIOLOGY

In order to have arterial saturation of 75% to 84%, these patients with arterial and venous mixing have a left-to-right shunt of between 1:1 and 2:1. Therefore, they all have a variably volume overloaded circulation system. Because of high flow and elevated pressure, they may have developed increased pulmonary vascular resistance in the area of some of the collaterals. The volume overload results in reduced exercise tolerance. The aorta and aortic valve tend to dilate and 50% develop aortic valve regurgitation. Ventricular dilatation and dysfunction can also occur.

PREOPERATIVE EVALUATION

Echocardiography is used to exclude additional VSDs and to evaluate the aortic valve. Angiography is performed to delineate the collaterals and true pulmonary arteries as well as to evaluate transpulmonary gradient, which may be high if there was uncontrolled large collateral flow hypertension. Such elevated gradients may preclude complete repair. MR angiograms with three-dimensional reconstruction give detailed models of the anatomy.

INDICATIONS FOR SURGERY

Patients with inadequate pulmonary blood flow are limited due to cyanosis and may require unifocalization if they have an adequate bed for future repair. If they do not, they may be candidates for a palliative shunt. Patients with excessive pulmonary blood flow and failure can also be treated by unifocalization with reduction of the total flow. The size of the shunt to the unifocalization is crucial to adjust flow to the pulmonary vasculature. After unifocalization on one side (Fig. 56-10), the opposite side is unifocalized 6 months to 1 year later (Fig. 56-11), followed 6 months to 1 year later by a complete repair (Fig. 56-12).



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FIGURE 56-10 Unifocalization of the pulmonary artery blood supply to the right lung using a pericardial tube. Through a lateral thoracotomy a side-to-side anastomosis is created to each major collateral and an adjacent incision made in the autologous pericardium placed behind the lung. The pericardium is turned into a tube by suturing the edges and the collaterals are ligated proximal to the tube. The posteriorly lying tube is extended by a 16-mm Gore-Tex graft to the anterior mediastinum. A 6-mm Gore-Tex shunt is made between the subclavian artery and the 16-mm Gore-Tex extension.

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FIGURE 56-11 A 19 year-old patient who previously underwent left pericardial tube unifocalization to three collaterals at age 18 years. On the right side there was a single collateral to a large pulmonary artery supplying the entire right lung. Right-sided unifocalization was achieved by ligating the collateral and placing a 20-mm Gore-Tex graft from the right pulmonary artery to the ascending aorta creating a central restrictive anastomosis.

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FIGURE 56-12 The complete repair performed 6 months after the right unifocalization shown in Figure 56-10. The ventricular septal defect was closed. An aortic homograft conduit was placed between the right ventricle and the left unifocalization. The right unifocalization was then connected to the homograft by a 16-mm reinforced Gore-Tex tube placed behind the aorta.

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Shunted patients should be repaired if they have an adequate size pulmonary bed, unless they are inoperable because of high pulmonary vascular resistance or poor ventricular function. A residual VSD should be closed in previously repaired patients if the left-to-right shunt is 1.5:1 or above. Conduit or valve obstruction is reoperated when there are symptoms, or if the RV pressure at rest is two thirds to three fourths of systemic pressure. If there is severe pulmonary valve regurgitation with RV dilatation or tricuspid regurgitation, reoperation should be undertaken.

STAGED SURGICAL REPAIR

The goal of surgical management of pulmonary atresia, ventricular septal defect, and multiple aorta-to-pulmonary collateral arteries is closing the ventricular septal defect and establishing continuity between the right ventricle and pulmonary artery. Ultimately, successful definitive repair requires an adequate pulmonary vascular bed, without which VSD closure and RV to PA continuity will lead to RV failure due to a prohibitively high pulmonary vascular resistance. Thus, all efforts during the operative staging are designed to maximize the size, distribution, and normal flow of the pulmonary arteries while preserving myocardial function.

Early palliative procedures in patients with excessive or inadequate pulmonary blood flow are designed to create a balanced pulmonary blood flow and encourage growth of the true pulmonary arteries.

Unifocalization procedures join the multifocal sources of pulmonary flow (true pulmonary arteries and aorta-to-pulmonary artery collaterals) into a single source that can ultimately be accessed in the anterior mediastinum via median sternotomy. The unifocalization procedure is performed through a posterolateral thoracotomy incision. A double-lumen endotracheal tube is employed, when possible, for large children and adults. Single-lung ventilation of the contralateral lung, when tolerated, greatly facilitates exposure. We prefer autologous pericardial tube unifocalization of aortopulmonary collaterals and true pulmonary arteries.

Finally, definitive repair in this disorder entails patch closure of the anterior malaligned ventricular septal defect and establishment of continuity between the right ventricle and the pulmonary arteries. All systemic-to-pulmonary artery shunts, including redundant collaterals and surgically created shunts, have been previously occluded or are readily accessible from the anterior mediastinum for occlusion at the time of definitive biventricular repair. Measurement of the ratio of right ventricle to left ventricle systolic pressure allows intraoperative assessment of the repair. A ratio of 0.75 or less immediately after termination of cardiopulmonary bypass is acceptable, and the ratio can be expected to decrease in the first few days after operation. Higher ratios suggest inadequate pulmonary runoff, and will likely result in right ventricular failure. If the pressure on the right side is near systemic or suprasystemic, perforation of the ventricular septal defect patch may provide survival and reasonable palliation.

OUTCOMES

From 1983 through 2000, 105 children and adults have presented to our institution with pulmonary atresia, ventricular septal defect, and multiple aorta to pulmonary artery collaterals. All patients were subject to a strategy of staged repair. Sixty-four patients in this cohort underwent palliation in the newborn period at a median age of 1 week. Surgical palliation included systemic to pulmonary artery shunts, right ventricular outflow patches, and banding of aorta to pulmonary artery collaterals to reduce high pressure and flow. Interventional cardiac catheterization procedures were performed to promote growth of the pulmonary arteries as necessary.

Ninety-four patients underwent unifocalization at a median of 3.5 years (range, 6 months to 37 years). Fifty-eight (range, 1 to 34 years) of these 94 patients have proceeded to complete repair at a median of 7.2 years. Unifocalization was performed in 19 adults, and of this group, 8 patients have undergone uneventful complete repair. There was neither mortality nor important morbidity in this group. At a median follow-up of 60 months there were a total of 18 deaths for a 17% early and late mortality rate. Survival after initial palliation was 92%, after unifocalization, 91%, and after complete repair, 91%. There were 36 reoperations (12%) and 16 patients required catheter-based interventions after surgery. The mean right ventricle to left ventricle (RV/LV) pressure ratio was 0.46. Nearly all the survivors are asymptomatic and do not exhibit any signs of exercise intolerance.

Although there is some debate about whether a one-stage repair is preferable in neonates and children, patients presenting as adults, with or without prior palliation, may be excellent candidates for the staged approach described above. We prefer a staged approach to one-stage correction in adults whose predominant blood supply to the lungs is from collaterals. In adults with a predominant blood supply from the true pulmonary arteries a one-stage repair may be utilized. The strategy of staged repair for patients with tetralogy of Fallot with MAPCAs (Major Aorto-Pulmonary Collaterals) provides good functional results. The mortality rate and requirements for postoperative interventional cardiac catheterization with this approach are lower than published reports of single-stage repair.

As patients age and mature they will require reoperations to replace right ventricle to pulmonary artery homografts and degenerative bioprostheses in the pulmonary position. Long-standing pressure and volume load on the right ventricle will lead to tricuspid regurgitation and right atrial enlargement that may predispose some patients to atrial arrythmias.

Late Reoperations for Transposition of the Great Arteries

ANATOMY AND PHYSIOLOGY

D-transposition of the great arteries is characterized by atrioventricular concordance and associated ventriculoarterial discordance. Prior to the introduction of the arterial switch procedure in 1982, the Mustard and Senning procedures were the standard operations for d-transposition. In the Mustard procedure, a pericardial baffle was used to redirect the systemic and pulmonary venous return. In the Senning procedure, the atrial septum and wall were used for the baffle. This allowed the deoxygenated blood to be pumped to the pulmonary circulation and the oxygenated blood to be pumped to the systemic circulation. As a consequence of these operations, such patients have the morphologic right ventricle acting as the systemic ventricle, and the natural history of such anatomy is well documented. There is about 70% 80% survival at 20 years; 10% of patients have symptomatic right ventricular dysfunction, and about 60% have dysfunction that becomes evident at exercise testing. Additionally, atrial arrhythmias are common, and many patients are in junctional rhythm at 10 years of follow-up. With either procedure, baffle obstruction can lead to a high incidence of vena caval obstruction or pulmonary venous obstruction.84,85

PREOPERATIVE EVALUATION

Transthoracic or transesophageal echocardiography usually delineates the site of systemic or pulmonary venous obstruction, ventricular function, and valvular regurgitation. In some cases angiography is also required.

OPERATIVE PROCEDURE

Reoperation for obstruction of either systemic veins or pulmonary veins almost always can be accomplished by incision of the site of obstruction and patching using a pericardial patch when it is available. Usually, with repair of the caval part of the baffle, the functional left atrium is also enlarged.

For patients who present with right ventricular (RV) dysfunction and tricuspid valve regurgitation, the choice of therapy is more complex. If the major problem is tricuspid valve regurgitation in the presence of relatively well-preserved RV function, we prefer to repair or replace the tricuspid valve. The results of tricuspid valve repair or replacement in suitable patients are generally good. Care must be taken to avoid conduction tissue that is very vulnerable at the junction of the septal and anterior leaflets. If RV function is significantly depressed, the LV function and the left ventricular outflow tract (LVOT) and pulmonic valve are evaluated. If LV function is good and there is no fixed LVOT obstruction or pulmonic stenosis, the patient may be considered for LV preparation and the arterial switch procedure. Preparation of the LV requires PA banding to a pressure of 60% to 70% of systemic pressures initially and then delayed rebanding to systemic pressures. It should be noted that retraining the left ventricle in the mature heart is a longer process than in the neonate and that there is less margin for error when placing a band in a fully septated heart. Six months to a year may be required to achieve this and to obtain a normal LV wall thickness.

Once this is achieved, the arterial switch operation may be performed. The atrial baffle is removed, and a new atrial septum is constructed in the anatomic position (Fig. 56-13). In adults, LV preparation by successive tightening of a pulmonary artery band can be hazardous and ultimately unsuccessful with the onset of left ventricular failure. The outcomes from this approach have been quite variable and the overall experience is limited. If significant LV dysfunction or fixed LVOT obstruction is identified, the patient may be considered for heart transplantation.



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FIGURE 56-13 Takedown of a Mustard baffle. After opening the anatomic right (functional left) atrium, the four pulmonary veins surrounded on three sides by the Mustard baffle are visible. The illustrated incision enters the atrial chamber that receives systemic venous blood and when completed will expose both caval-atrial junctions and the four pulmonary veins entering a common atrial chamber. For venous return to the perfusion circuit, the superior and inferior vena cavae can be cannulated directly or via peripheral venous cannulas.

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RESULTS

The outcomes from reoperations in patients with previous Mustard and Senning procedures are quite good if the right ventricle is preserved as the systemic ventricle.8498 Generally adults are poor candidates for a staged conversion to an arterial switch procedure.89,94 Results of cardiac transplantation in these patients has also been successful.

Single Ventricle

ANATOMY

This group includes many lesions characterized by the inability to create a two-ventricle repair. Examples include tricuspid atresia, mitral atresia, double-inlet left ventricle, and unbalanced AV canal.

PHYSIOLOGY

These patients have a mixed circulation with oxygenated and deoxygenated blood mixing in the single ventricle. Pulmonary blood flow is supplied either by the pulmonary artery (PA) or by a patent ductus or shunt. The PA may have pulmonary or subpulmonary stenosis or may have been surgically banded. In order to have an adequate arterial oxygen saturation of 80%, the pulmonary blood flow must be approximately 1.5 times the systemic flow, which results in the effects of volume overload on the ventricle and aorta.

INDICATIONS FOR SURGERY

Few patients with single ventricle survive to adulthood without surgical intervention. Patients may require intervention because of too much pulmonary blood flow, causing heart failure, or too little flow, causing cyanosis. Patients are stratified according to their pulmonary artery pressure, pulmonary vascular resistance (PVR), ventricular function, and anatomical complexity into low-, medium-, and high-risk candidates for a Fontan procedure. In medium- and high-risk patients with elevated PA pressure and PVR and impaired ventricular function, a bidirectional Glenn shunt is performed as a first stage to a Fontan procedure or as long-term palliation until a heart transplant may be indicated.

GLENN SHUNT

Physiology By connecting the end of the SVC to the superior aspect of the right PA, about one third of the systemic venous return is diverted to the lungs for oxygenation. This is a more efficient shunt than a systemic to PA shunt and does not cause a volume overload on the ventricle. It is therefore better tolerated in the presence of impaired ventricular function. Provided the SVC pressure is 18 mm Hg or less, the elevation in SVC pressure is well tolerated.

Operative procedure As shown in Figure 56-14, the Glenn shunt is performed without cardiopulmonary bypass in most patients by using an SVC to PA shunt. If additional procedures are required, such as the relief of subaortic obstruction, atrial septectomy, or AV valve repair, an open procedure on cardiopulmonary bypass is required. An additional source of pulmonary blood flow aims for a pulmonary to systemic blood flow ratio of 1 to 1.3 depending on the PVR. Either a banded pulmonary artery or a small systemic to PA shunt is used. The additional source of blood flow improves oxygenation at rest and with exercise and may prevent late arteriovenous fistula development in the lungs.



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FIGURE 56-14 Creation of a bidirectional Glenn shunt (superior vena cava to right pulmonary artery) using an extracorporeal shunt (with systemic heparin) to maintain superior caval flow into the right atrium. A de-airing chamber and port are needed to prevent air entry into the heart.

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Outcomes The early mortality for an isolated Glenn shunt is low (1% to 4%), depending on factors such as the PVR and ventricular function.99102 Additional intracardiac procedures increase the risk. In the long term the Glenn shunt slowly loses its effectiveness due to the development of venous collaterals from the superior vena cava to the inferior vena cava.99,101,102 These can be coil embolized by catheter technique. If there is no additional source of pulmonary blood flow, there is usually severe desaturation on exercise and systemic to PA collaterals develop over time. Intrapulmonary AV fistula can also develop in this situation, resulting in desaturation. In patients with no additional source of pulmonary blood flow and with severely impaired ventricular function, oxygenation can be improved with a controlled shunt by performing an axillary artery-to-vein fistula without thoracotomy.103

MODIFIED FONTAN PROCEDURE

Indications Because of the limited palliation provided by the Glenn shunt, patients who meet hemodynamic criteria are accepted for a Fontan procedure. The criteria include good ventricular function, ejection fraction 50% or higher, normal or close to normal PVR, PA pressure less than 20mm Hg, and no additional severe hemodynamic lesions that require prior attention, such as residual coarctation of the aorta, severe subaortic obstruction, or severe AV valve regurgitation. Generally, severe lesions should be addressed at the time of the Glenn shunt or before the Fontan procedure.

Operative procedure Many different types of connection have evolved to connect both SVC and IVC blood to the pulmonary arteries. The two most commonly performed operations are the lateral tunnel Fontan (Fig. 56-15) and the extracardiac Fontan (Fig. 56-16). The lateral tunnel has the advantage of not requiring warfarin anticoagulation in the majority of cases and a fenestration can be easily included in the procedure. The extracardiac Fontan can be done without arresting the heart and is therefore associated with excellent ventricular function. It requires warfarin anticoagulation for at least 1 year and possibly for life. Because of the extensive atrial suture lines, arrhythmias and sick sinus syndrome may be more common with the lateral tunnel Fontan, although they occur in the extracardiac group as well.



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FIGURE 56-15 A lateral tunnel Fontan operation enlarges the right atrium and directs inferior caval flow into the right pulmonary artery using an end-to-side anastomosis to provide bidirectional flow into both pulmonary arteries. A snared purse-string suture adjusts the size of an atrial septal opening that is used to decompress caval pressures and to control the amount of right-to-left shunting.

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FIGURE 56-16 Extracardiac Fontan with adjustable conduit to atrial connection.

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Outcomes The early mortality of the Fontan procedure in adults depends a great deal on selection of patients and the presence of risk factors. Reported series have an early mortality of 5%.104 Fontan patients may be candidates for pacemakers (10%) and reoperations for valvular (5%) and obstructive problems, and may require transplantation for deteriorating ventricular function.105 Protein-losing enteropathy can occur in 5% to 10% of patients.104,106,107

Late Reoperations After Modified and Classic Fontan Procedures

Patients who have had a right atrial to PA connection may develop massive right atrial enlargement.104,106,107 This can eventually result in atrial arrhythmias, thrombus formation, and thromboembolism. Supraventricular arrhythmias as well as AV node dysfunction are not uncommon, and preoperative evaluation by an electrophysiologist is usually necessary. When the RA is enlarged, warfarin anticoagulation is indicated. Such patients are now considered for reoperation with conversion to either a lateral tunnel Fontan or to an extracardiac conduit with reduction of the enlarged RA and a right-sided Maze procedure.108 If additional surgery is required for Fontan patients with an RA to PA connection, simultaneous conversion to a lateral tunnel is recommended if the RA is enlarged. If they do not meet the criteria, they could be candidates for a heart transplant.

The long-term results after modified Fontan procedures have shown that a significant number of patients require late reoperation. The highest incidence was for patients in whom a valved conduit was used.109,110 Older age at operation remains a risk factor for late death after the Fontan procedure. Revision of the AV valve closure in double-inlet ventricles or repair of the AV valve in tricuspid atresia is needed in more than 5% of patients.

Protein-losing enteropathy (PLE) is sometimes difficult to diagnose. It occurs in about 10% of patients. It is characterized by a low serum albumin level, ascites, and peripheral edema with or without diarrhea. It may be associated with a mortality up to 20%.104,105,107 This seems to occur more frequently in patients with heterotaxia or polysplenic syndromes, as well as in those with elevated pulmonary vascular resistance or abnormal systemic venous drainage. Some of these patients can be helped by transcatheter fenestration of the atrial septum. Conversion to a lateral tunnel or transplantation should be considered for these patients. If they do not meet criteria for a Fontan revision, they should be evaluated for transplantation.111118

Ebstein's Anomaly

Ebstein's malformation is a rare congenital cardiac defect accounting for less than 1% of all congenital heart disease. The primary pathologic finding is an abnormal development of the tricuspid valve marked by a downward displacement of the septal and posterior leaflets into the cavity of the right ventricle. This defect is characterized by a remarkable morphologic variability and a broad spectrum of clinical presentations. Consequently, the diagnosis may be made in symptomatic newborn infants, in children, or in adults.119,120 The degree of tricuspid regurgitation varies depending on the anatomic abnormality. If there is an atrial septal defect, patients may present with cyanosis. If the atrial septum is intact, they may present with cardiomegaly, right-sided heart failure, or arrhythmias. Those patients with atrial or ventricular arrhythmias may present with episodes of syncope, near syncope, or recurrent palpitations.

Paroxysmal supraventricular arrhythmias occur in 25% to 40% of patients and are most often found in teenagers or young adults.121 Aberrant right atrial to RV tracts resulting in tachycardia (Wolff-Parkinson-White syndrome) occur in 10% to 18% of patients.122 Sudden death due to ventricular arrhythmias may occur in as many as 5% to 7% of patients. Likewise, patients with mild manifestations of the Ebstein's malformation may present as late as the third or fourth decade of life with complaints of palpitations or mild exercise intolerance.123,124 While some patients may reach advanced age without serious clinical manifestations, most will eventually develop significant symptoms.125 The most common causes of death are congestive heart failure, severe hypoxia, and cardiac arrhythmias.126

ANATOMY

Ebstein's malformation is defined by a downward displacement of the annular attachments of the septal and posterior leaflets of the tricuspid valve into the inlet portion of the right ventricle.127 This downward displacement of the leaflets reduces the distal chamber of the right ventricle, leaving part of the ventricle above the valve as an extension of the right atrium. The atrialized RV is variable in size and thickness, depending on the extent of downward displacement of the leaflets. The entire wall of the right ventricle, both above and below the tricuspid valve, is often thin, dilated, and dysfunctional. In most patients, annular dilatation and malformation of the leaflets result in moderate to severe insufficiency of the tricuspid valve. It usually occurs in the pulmonary right ventricle, but can occur in the systemic right ventricle in patients with corrected transposition of the great arteries. An atrial septal defect or patent foramen ovale is present in greater than 50% of patients, allowing predominantly right-to-left shunting at the atrial level.

PHYSIOLOGY

In patients with Ebstein's malformation, the tricuspid valve is incompetent, but the degree varies depending on the anatomical features. In addition, there is some degree of functional impairment of the right ventricle. The atrialized right ventricle moves paradoxically with right atrial and right ventricular contractions. The net effect is reduced forward blood flow through the right ventricle and pulmonary arteries. The impaired filling of the functional right ventricle and the incompetence of the tricuspid valve both result in systemic venous hypertension. The right atrium and the atrialized right ventricle become dilated, often to extreme degrees. In patients with atrial septal defects, right-to-left shunting occurs, resulting in cyanosis. Both atrial and ventricular arrhythmias may contribute to impaired right ventricular function.

Although the primary pathology involves the right ventricle, patients with Ebstein's malformation may also demonstrate abnormal left ventricular geometry and function. The severity of left ventricular dysfunction is associated with the degree of displacement of the tricuspid valve, the size and dysfunction of the right ventricle, and the severity of paradoxical motion of the interventricular septum.

PREOPERATIVE EVALUATION

On a chest radiograph, the right border of the heart in the area of the RA is enlarged and there may be massive cardiomegaly. Typically, the shadow of the great vessels is narrow due to a small aorta and main pulmonary artery. Right atrial and right ventricular enlargement produce a globular shape to the heart shadow. The apical region of the left ventricle may be elevated from the diaphragm, as seen in right ventricular enlargement. Pulmonary vascularity may range from normal to significantly decreased in the presence of an ASD. A cardiothoracic ratio greater than 0.65 has been shown to be a predictor of sudden death and is considered by some an indication for surgery.

Echocardiography has evolved as the primary diagnostic tool for patients with Ebstein's malformation. The preoperative echocardiogram is helpful in predicting the ability to repair the valve. Echocardiography can define the morphology of the tricuspid valve and the specific abnormalities of the leaflets. Of the greatest importance are the length and mobility of the anterior leaflet. In addition, the function, thickness, and size of the right and left ventricles can be assessed. Coexisting cardiac lesions can also be identified. Color flow Doppler allows a better assessment of tricuspid valve incompetence and the degree of shunting at the atrial level.128 If echocardiography is inadequate, magnetic resonance angiography imaging with three-dimensional reconstruction is useful for diagnostic purposes. Cardiac catheterization should be avoided as it is usually unnecessary and can result in arrhythmias. Currently, cardiac catheterization is reserved for patients with associated cardiac defects, previous shunt placements, possible pulmonary artery stenosis, or possible coronary artery disease.

INDICATIONS FOR SURGERY

The indications for surgical intervention in patient with Ebstein's malformation include the following: functional New York Heart Association (NYHA) class III or IV symptoms; significant or progressive cyanosis; decline in exercise tolerance; severe cardiomegaly (cardiothoracic ratio greater than 0.65); associated cardiac anomalies (including right ventricular outflow tract obstruction); refractory atrial or ventricular arrhythmias; and a history of paradoxical embolus. With the improved outcomes and a greater ability to repair the valve, there is a trend to perform early repair in the presence of severe tricuspid regurgitation and atrial enlargement.

OPERATIVE PROCEDURE

The goals of surgical intervention in patients with Ebstein's malformation are to increase pulmonary blood flow, minimize tricuspid insufficiency, reduce or eliminate right-to-left shunting, optimize right ventricular function, and reduce or eliminate arrhythmias. Ideally, the tricuspid valve can be repaired, which may avoid valve replacement with a bioprosthetic valve and the need for future valve replacements. Patients with preoperative Wolff-Parkinson-White syndrome are treated by catheter ablation prior to the surgery.

If the anterior leaflet is adequate in size and is not extensively bound down by muscular attachments, repair is almost always possible. Two main techniques of repair have been described. Danielson was the first to demonstrate the ability to repair these valves and avoid replacement.129 Repair includes plication of the atrialized RV back to the true annulus and an annuloplasty (Fig. 56-17).



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FIGURE 56-17 Danielson's repair of the tricuspid valve in Ebstein's anomaly.

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Carpentier et al described a technique in which the atrialized right ventricle is plicated perpendicular to the valve annulus toward the apex of the heart.130 The displaced leaflets are detached from the right ventricle at their base and attached to the true annulus (Fig. 56-18). We perform an annuloplasty using a glutaraldehyde-treated strip of pericardium. The redundant RA wall and appendage are excised, and the ASD is closed. In patients with a large preoperative right-to-left shunt and a thinned-out underdeveloped right ventricle, a snare-controlled adjustable atrial septal defect may be used to allow continued controlled right-to-left shunting until the RV recovers. The ASD can then be closed using the snare, which is exposed under local anesthesia. In addition, electrophysiological mapping for localization of accessory pathways may be performed in patients with arrhythmias.



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FIGURE 56-18 Carpentier's repair of the tricuspid valve in Ebstein's anomaly.

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We prefer to use a porcine bioprosthetic valve. In the absence of atrial arrhythmias or severe atrial wall thickening, this allows anticoagulation with aspirin only. Generally, tissue valves are preferred in the tricuspid position because of the risk of thrombosis of a right-sided mechanical valve in a low-pressure setting.

The right atrial Maze procedure is a modification of the Maze procedure and has been used to treat atrial arrhythmias in patients with Ebstein's malformation. This procedure may reduce or eliminate atrial arrhythmias by preventing reentry conduction at the atrial level (Fig. 56-19).



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FIGURE 56-19 Injury to the conduction system may be avoided during tricuspid valve replacement by suturing a triangular patch of pericardium over the AV node and triangle of Koch.

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OUTCOMES

Results of tricuspid valve repair continue to improve. Danielson and colleagues at the Mayo clinic currently have a surgical experience of more than 400 patients with Ebstein's malformation.131 The data have recently been analyzed for the first 312 patients undergoing surgical intervention from 1972 to 1996. The ages in this series range from 9 months to 71 years, with a mean age of 20.7 years. There were no neonates in this group. In 43% a tricuspid valve repair was successful, and in 53% a bioprosthesis was used to replace the tricuspid valve. Approximately 4% of patients underwent a Fontan reconstruction or other procedures. There were 20 hospital deaths (6.4% early mortality) in this series. Forty-four patients had accessory conduction pathways (Wolff-Parkinson-White syndrome) and underwent successful pathway ablation as part of their repair. Fifteen patients underwent right-sided Maze procedures for control of atrial dysrhythmias and 4 underwent ablation of the atrioventricular node for re-entry tachycardia. There were 24 late deaths (7.3%). Seventeen of the 135 patients (12.6%) who underwent valve repair required reoperation for valve regurgitation 1.5 to 18 years later (mean, 8.7 years). Eight bioprosthetic valves required replacement 1 to 16 years after implantation. Follow-up of those patients evaluated more than a year after operation determined that 93% were in NYHA functional class I or II. The addition of a right atrial Maze procedure to the repair is often successful in reducing or eliminating atrial arrhythmias. Furthermore, the durability of a porcine bioprosthesis for tricuspid valve replacement has been quite favorable.

Heart Transplantation

Adults with congenital heart disease may not be amenable to palliation or repair due to severe ventricular dysfunction or pulmonary vascular disease. They may be candidates for heart, lung, or heart-lung transplantation. In addition, patients previously repaired may deteriorate and may have no other options.132147

In symptomatic patients who are NYHA class III or IV and who have an acceptable pulmonary vascular resistance, heart transplantation is usually feasible. Anomalies of systemic and pulmonary venous return can be corrected. Deformities of the pulmonary artery can be repaired. This may require quite extensive repair, particularly in some patients after the Fontan procedure. Dextrocardia and transposition may be challenging, but with current reconstructive techniques, transplantation is almost always possible.140144,148,149

PREOPERATIVE EVALUATION

General evaluation is as for all heart transplant recipients. Cardiopulmonary exercising testing, pulmonary, dental, and psychosocial evaluations are routine. Pulmonary vascular resistance must be carefully assessed; cut-off is at 4 to 6 Wood units or transpulmonary gradient of 12 to 14 mm Hg. Panel-reactive antibody screening is essential, as most patients have had previous surgery and blood transfusions. If these values are higher than 10%, then prospective cross-matching is preferable. Chest wall collaterals may increase left-sided return particularly after previous surgery, and therefore it is usual to oversize donors by 20%. Some larger aortopulmonary or veno-venous collaterals may be coil-embolized preoperatively.150

OPERATIVE PROCEDURE

It is important to anticipate the recipient's anatomy. For example, in tricuspid atresia d-transposition is common, and the aorta may be immediately behind the sternum. A CT scan is obtained preoperatively to assess the retrosternal structures. It may be necessary to institute bypass before opening the sternum. Aprotonin is used in all cases and it is important to plan correct timing for the arrival of the donor heart, since redo sternotomy in these patients is more complex than usual. Patients are cooled to 22?C to 24?C in order to minimize the pulmonary venous return, which can be torrential. This can warm the donor heart and, after the aortic anastamoses are completed, may wash out the preservation solution if the aortic root is not vented. A vent in the left atrium is therefore used. Anomalies in systemic or pulmonary venous return and the pulmonary arteries are reconstructed prior to bringing the donor heart onto the field. We apply intracardiac cooling to the left ventricle using a catheter passed through the left atrial anastamosis into the left ventricle apex. Generally, we prefer a bicaval anastamosis (Figs. 56-20 and 56-21).



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FIGURE 56-20 Heart transplantation after total cavopulmonary connection in a patient with bilateral superior vena cavae. The innominate vein from the donor is used to reconstruct the left superior vena cava. Alternatively (not shown), if the left superior vena cava is too short, a synthetic graft can be used to route the left superior vena cava blood along the coronary sinus of the donor heart into the recipient native inferior vena cava.

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FIGURE 56-21 Heart transplant in a patient with dextrocardia, right-sided arch, and interrupted inferior vena cava with azygous continuity who previously had undergone a Fontan procedure.

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OUTCOMES

Due to previous surgeries, anatomical complexity, borderline pulmonary vascular resistance, hepatic congestion, and other factors, these patients (particularly the Fontan patients) are at higher risk for transplantation. Over the last 18 years, 30 adults and adolescents with congenital heart disease have undergone transplantation at our institution. Close to 50% of the patients had single-ventricle physiology and on average all had at least two previous sternotomies. Age range was 13 to 49 years old. The early mortality for this high-risk group was 18%.


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