Adults with congenital heart disease now outnumber children with this malady. They constitute a growing population of survivors, with increasingly complex treatment requirements for their heart disease. Over the past two decades, the spectrum of complexity for adult congenital heart disease (ACHD) patients has evolved from late primary repairs of patients with simple lesions—including coarctation, patent ductus arteriosus, septal defects, and tetralogy of Fallot—to nth time reoperations on survivors of complex multistaged palliations. Significant efforts to define the optimal program resources necessary to effectively treat ACHD patients have been made worldwide,1 yet many congenital heart patients continue to suffer from poor continuity of care as they enter adulthood. This chapter will describe several salient treatment strategies for adult patients undergoing congenital heart surgery.
The optimal venue for ACHD treatment is unknown, and it is fair to say that no two programs are the same. The need for a coordinated and comprehensive programmatic strategy for care has been well described, and should encompass not only the lifetime of a generation of patients with congenital heart defects from fetal to adult life, but also the health of pregnant mothers with congenital heart disease who will give birth to a new generation.2 Pregnant mothers with fetal diagnoses of congenital heart disease may be asked to undergo invasive intrauterine procedures to palliate their fetal child’s heart, thus creating a new group of adult patients dealing with the trauma of congenital heart care.3
Our congenital heart program philosophy is to reduce the cumulative trauma of care for each patient with congenital heart disease over their lifetime. To achieve this, we discuss each adult patient referred to our program for surgery or intervention in a combined conference with participation from adult and pediatric interventional cardiologists and surgeons, dedicated cardiac intensivists, cardiac anesthesiologists, cardiac imaging specialists, and the cardiac nursing, pharmacy and social work teams. Treatment options are selected so that the least traumatic form of effective therapy is chosen as the initial approach. Failure to achieve a good result leads to an escalation to the next least traumatic option. As an example, a patient with a secundum atrial septal defect (ASD) would be put forward for device closure, and if this failed, the patient would then be given the option of a minimally invasive surgical repair.
Adult congenital heart disease patients with complex lesions often have dense medical histories, stored in multimedia formats, which expose the weaknesses of current paper and electronic medical record (EMR) systems. Increasing utilization of EMRs by medical teams, and personal health records (PHRs) by patients and families, has been demonstrated to improve outcomes in some patients with chronic disease.4 However, pediatric heart patients and their families often have not been educated about their disease, and what to expect as they transition to adulthood. The PHR could be used to enhance patients’ understanding, and provide roadmaps for follow-up treatments and diagnostic studies. These information systems could also be linked to national and international registries designed to measure clinical outcomes and performance for centers treating adult congenital heart patients.5
The emergence of Web-based hospital EMR systems allows our cardiac team to track patients over their lifetime, and retrieve critical patient information on demand. We have also enabled our patients to access specific data fields from the EMR. We capture intraoperative endoscopic images of each congenital heart lesion, before and after repair,6 and store these images in our EMR. We also capture and store a daily picture of each hospitalized patient. These images are reviewed in morbidity and mortality conferences, to correlate with other clinical information and imaging techniques. The operative images are reviewed prior to reoperations, to regain familiarity with the patient’s anatomy.7 The operative images from this chapter were retrieved from this online image database (Fig. 56-1).
FIGURE 56-1
This is a screen image from our first electronic medical record, showing the operative images for a patient with truncus arteriosus. These images were captured in the operating room, and immediately uploaded to a Web-based EMR. These can be retrieved on demand by searching by procedure, diagnosis, or individual patient.
Congenital heart procedures in adults are frequently reoperations, however, standardized protocols for reoperations in patients with acquired heart disease may not match the challenges presented by ACHD patients. Risk factors for traumatic repeat sternotomy in ACHD patients differ from those identified in patients with acquired heart disease, which often focus on dealing with patent coronary artery bypass grafts.8 Femoral cannulation is often difficult in ACHD patients who have undergone multiple interventional catheterizations during their growth years, resulting in stenotic or occluded femoral vessels. Consequently, we rarely use elective or emergent peripheral cannulation. Key risk factors for ACHD reoperations include retrosternal ventricular to pulmonary artery conduits, particularly if they are calcified, have multiple stents in place, or are clearly embedded in the sternum. Fusion to the sternum should be suspected when the conduit does not move with the cardiac cycle on lateral angiograms. Patients with pulmonary hypertension, right ventricular enlargement, prior sternal infections, aneurysms of the right or left ventricular outflow tracts, pectus excavatum, or aortopulmonary shunts are at increased risk, and qualify for preliminary vascular access dissection and pursestring placement. Patients with multiple risk factors may be placed on bypass to decompress the right ventricle prior to sternal division.
We excise previous sternotomy scars and remove sternal wires. The xiphoid process is removed, and cautery dissection is begun at the inferior sternal edge. The oscillating saw is used to divide the anterior sternal table. Rakes are used to elevate the two sides of the sternum and expose the retrosternal scar. Short segments of retrosternal scar are released, followed by gradual sternal division with the oscillating saw, proceeding up the sternum.
In the event of cardiac or great vessel penetration during sternotomy, when we have not already exposed vessels for cannulation, we release the sternal retractors, and immediately extend the neck incision up and to the right to expose the innominate artery and the internal jugular or high innominate vein. These vessels are cannulated through pursestrings, and bypass is initiated. In patients where venous access cannot be achieved in the suprasternal area, we dissect the inferior vena cava just above the diaphragm. This approach has been described as “simplified aortic cannulation.”9 The sternotomy is completed with the patient in Trendeleberg position. We continuously infuse carbon dioxide into the operative field during every operation to reduce the risk of air embolism, although evidence supporting this technique is not conclusive.10
To reduce adhesion formation, and decrease the risk of future sternotomy, we prevent cardiac dessication by covering the heart with saline-soaked gauze throughout each operation. At the completion of ACHD repairs we occasionally place antiadhesion materials to decrease retrosternal adhesions. In some centers, expanded polytetrafluoroethylene (PTFE) is routinely placed behind the sternum prior to closure.11 Capsule formation may obscure natural tissue planes, making subsequent reoperative dissection more difficult. Bioresorbable films12 and sheets of extracellular matrix13 derived from pig jejunum have also been approved for use as pericardial substitutes.
The advent of transcatheter device closure for ASDs has significantly changed the average complexity level of ACHD surgical case volumes. Patent foramen ovale and most secundum defects are effectively closed with devices, and are rarely referred for surgical closure in programs with effective interventional catheterization teams. Sinus venosus and primum defects, transitional and complete atrioventricular canal, common atrium, and secundum defects with deficient inferior and superior rims are referred for surgery and we repair these on cardiopulmonary bypass with bicaval cannulation and ischemic arrest.
Multiple incisional approaches have been described to improve the cosmetic result, and reduce operative trauma, after open-heart surgery. These include partial upper and lower sternotomy, transxiphoid, anterior thoracotomy, and submammary incisions. Despite the visible scar, median sternotomy may be the least traumatic incision for ACHD patients, allowing the surgeon to avoid vascular trauma from peripheral cannulation, intercostal muscle, vessel, and nerve damage, and mitigate the risk of post-thoracotomy pain syndrome. Median sternotomy allows direct aortic control for safe, effective cannulation, decannulation, deairing, and cardioplegia administration with minimal risk of dissection. Median sternotomy also ensures direct and rapid access to the entire mediastinum, allowing surgeons to deal with unanticipated anatomic variations discovered at surgery, which are not uncommon in ACHD patients.
Our venous cannulation strategy for ASDs is bicaval, via the right atrial appendage into the superior vena cava, and down the inferior venal caval junction into the inferior vena cava. Smaller defects, which in the past might have been closed primarily, are now rarely referred for surgery, and we find that repairs are best performed with patch materials, ideally glutaraldehyde-treated pericardium. Placing the smooth surface on the left atrial side, we use running suture lines of fine polypropylene to create tension-free suture lines (Fig. 56-2A).
For sinus venosus defects, we place a right-angled cannula in the innominate vein, to enable exposure of the partial anomalous pulmonary veins entering the superior vena cava. The atrial incision is made laterally to avoid the sinus node area, and is extended superiorly to a point above the entrance of the highest anomalous vein. A native pericardial patch is used to close the atrial incision to avoid superior vena caval stenosis (Fig. 56-2B).
FIGURE 56-2
(A) Operative image of sinus venosus defect showing the pulmonary venin orifices, the superior vena cava, and the septal defect. (B) Operative image of two patch repair with an internal patch baffling the pulmonary veins to the left atrium, and an external patch to prevent obstruction of the superior venal caval entry into the right atrium.
In patients with primum ASDs, the cleft mitral valve is routinely repaired with fine running polypropylene suture lines, approximating the line of contact between the leaflet segments. Even patients with competent valves are repaired, as late onset of cleft regurgitation is known to occur. Patients at risk for mitral stenosis, particularly those with a single papillary muscle in the left ventricle, may have their clefts left open to avoid valvar stenosis. Results for these repairs are excellent, even in patients at advanced ages.14
Surgery for Ebstein’s anomaly can be performed in older patients at low risk and with good late outcome. The operation is comprised of tricuspid valve repair or replacement, and concomitant procedures such as ASD closure, arrhythmia surgery, and coronary artery bypass grafting.15 Repair techniques for these patients continue to evolve. We believe the presence of an untethered and well-developed anterior tricuspid valve leaflet increases the chance of a successful repair, and have used the Cone technique in adult patients.16 This repair requires dissection of the anterior and posterior tricuspid valve leaflets from their right ventricular attachment. The free edge of the anterior leaflet is then rotated clockwise and sutured to the septal leaflet border. This produces a cone-shaped valve, fixed distally at the right ventricular apex, and proximally at the tricuspid valve annulus. The septal leaflet is incorporated into the cone wall whenever possible, and the ASD is partially closed. Results have been good, with low mortality, significantly less tricuspid regurgitation, and improvement in functional class.
In the modern era, the primary cause of death for adult patients with cyanotic lesions is arrhythmia, followed by heart failure.17 Fontan patients may present with arrhythmia and complications related to systemic ventricular failure, protein-losing enteropathy (PLE), systemic venous pathway obstruction, and semilunar and atrioventricular valve dysfunction. Initial evaluations must focus on ensuring a completely unobstructed vascular pathway to the lungs. The different types of surgical technique historically used to create the Fontan circulation each have characteristic complications. Patients with intracardiac baffles and atriopulmonary connections may present with extreme right atrial enlargement, resulting in stagnant flow, right pulmonary vein compression, and arrhythmia.
Fontan conversion involves takedown of the previously created venous connection, and creation of an extracardiac cavopulmonary connection with a conduit. Because the extracardiac Fontan excludes the systemic veins from the heart, any catheterization procedures requiring atrial level intervention, particularly electrophysiology interventions, must be planned before conversion. We therefore plan Fontan conversions with our electrophysiology team, and frequently combine Fontan conversions with arrhythmia surgery,18 and treatment of atrioventricular valve dysfunction. Valve repairs are often complex in these patients, and replacements are often required to achieve good hemodynamic results.19 Results depend on the patients’ underlying anatomy, right ventricular function, and pulmonary vascular resistance.20
We perform extracardiac Fontan procedures with bicaval cannulation, and leave the heart warm and beating whenever possible. The inferior vena cava is transected at the cavo-atrial junction under a clamp, and the cardiac end is oversewn with a running 4-0 polypropylene suture. We use a ring reinforced expanded PTFE graft from 19 to 23 mm in diameter, and leave enough length to avoid right pulmonary vein compression. The superior anastomosis to the superior vena caval junction with the right pulmonary artery is then constructed with a running 6-0 polypropylene suture. Hybrid stenting procedures, where the interventional catheterization team comes into the cardiac operating room to deploy stents, are used to treat stenoses in the retroaortic pulmonary arteries.
In patients with complex cardiac anatomy, these Fontan revision procedures may best be performed with the participation of the electrophysiology team. This ensures effective interruption or ablation of all reentrant pathways, which may not follow the patterns seen in patients with acquired heart disease and normal cardiac anatomic relationships. A variety of Maze type procedures have been described in an effort to disrupt atrial reentrant pathways. The unpredictable anatomy of the conduction tissue in ACHD patients has resulted in frequent need for pacemaker insertion. In many centers, customized pacemaker therapy has been advocated for management of patients following Fontan conversion. However, based on an experience with 120 Fontan conversion from 1994 to 2008, which began with a flexible approach to each patient’s anticipated pacing needs, Tsao et al now recommend routine placement of a dual-chamber antitachycardia pacemaker with bipolar steroid-eluting leads in patients undergoing Fontan revision.21
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