I went to the library and tried to find if anything much had been written in the last ten years, there has been such an evolution away from operating on older patients and towards primary repair that I thought there would not be a current series describing older patients. There are a few. This is from the Brompton Hospital, Jane Summerville and her group, and the National Heart and Lung Institute in England. They call is grown up congenital heart patients. In this paper, patients aged 16 to 77 years with a mean age of 31 underwent a variety of operations. If one looks at the primary operations, they chose to break this down into first operations and then reoperations. As you might suspect in this older group of patients, there are a lot of atrial septal defects, a fair number of aortic valve replacements, and just a smattering of other operations including a very few complex repairs which carried a relatively high risk. In the reoperation group, the same sort of operations were seen with conduit changes and the like, and a somewhat higher mortality which, in the paper, is in part related to the group with cyanosis. We looked at our patients in San Diego over the last eight years because I didn't think it was relevant to go back more than that time. There were only 168 patients over the age of 12, and I thought maybe we could include patients eight to twelve, and assume they were precocious partly because some of the patients that had interesting operations were eleven and ten. The age range in this report is from 8 to 75 years. If you look at only the twelve and older patients, the median age is 23, meaning about half the patients are in the quite a bit older group. If one looks at the entire group, the median age was 13. In the noncardiopulmonary bypass group, the median was teenagers, whether it was eight to twelve or twelve on up. We had a lot of ASDs and VSDs, 57 patients, representing 31% of the series, and there is not a lot to be said about ASD and small VSDs except that they can be done through smaller incisions in a relatively cosmetic fashion. I think the factor that we are all going to have to deal with over the next ten years is the use of ASD occluders. This and some of the other interventional slides were provided to me by our interventionalist, Frank Kane, who came to us from Houston. A few of the slides look like Texas slides. This is a four chamber view with a catheter through a small secundum ASD up into the right upper pulmonary vein. The device is barely visible, but the right atrial injection shows no flow. We have been using a device called the amplatser, and it is through the first phase of its clinical trials. It seems to be doing quite well. I suspect that more and more older patients are going to have their atrial septal defects closed by devices in the coming years. An important caveat for the adult surgeon operating on an ASD, particularly if the patients have been worked up by a nonsophisticated cardiologist in terms of congenital heart disease. Patients with partial anomalous pulmonary venous drainage to the right side always have a left-to-right shunt, but that doesn't mean they are operable. In reference three, one of their mortalities was due to failure to recognize pulmonary vascular disease. I am aware of other cases in our community where the same thing happened. I think that is something to watch out for. There are a lot of operations on the left ventricular outflow tract in this group of patients. Some patients with subaortic stenosis present later in life and occasionally a radical subannular cono operation will be appropriate. Some of the patients will come back with disease late. This is a 40 year old man who had a double outlet right ventricle repaired in another institution years ago, and came back with subaortic stenosis and aortic insufficiency. He had a mechanical valve put in, as we have done with the redo cono. In general, we favor the Ross operation for any patient requiring their aortic valve replaced if they are under the age of 30, and I think it is important to pay attention to the size of the aortic root. Some of the kids have a hypoplastic annulus, and they need a resection of subannular tissue of the annulus. Those with a dilated root, such as the patients coming back after coarctation, should have some attempt made to fix the size of the annulus or you are going to see early regurgitation which seems to be preventable with proper management of the aortic annulus. I have mentioned here of one homograft aortic valve that did not include rheumatic heart disease in this series except to make the point that in a 19 year old patient with a history of recent active rheumatic heart disease how the Ross procedure is probably contraindicated. We have seen patients with recurrent stenosis after a previous operation for supravalvar aortic stenosis, and that can easily be retreated. We did not see too many patients presenting over the age of eight with unrepaired tetralogy of Fallot, but there were a group of teenagers and these patients are repaired by standard techniques. One death in the series occurred in a 24 year old patient who had had previous bilateral unifocalization and in retrospect was an inappropriate patient to attempt complete repair on. We were not able to salvage the patient by taking the VSD patch out. I think that those kinds of patients need to be carefully assessed. In the right heart, Ebstein's anomaly is amenable to repair. Some patients come with preexisting rhythms and need a pacemaker implanted at the time of surgery. We have been liberal in applying the bidirectional Glenn technique to younger patients with Ebstein's. This particular patient was 45 years of age. We also did that in a patient that came to us having previously had a porcine valve implanted with failure of a valve. We hope that in appropriate patients, the use of the bidirectional Glenn plus a tricuspid valve prosthesis will increase the time before the next operation. Earlier today, Dr. Irschel was quoted as having said that a pneumonectomy is a new disease. Well, a porcine valve is a new disease, as is a homograft. Some other straightforward lesions will present later in life, and they are not really worth talking about. The right ventricular outflow tract reconstruction is an important part in 20% of the patients. This is a large piece of any adult congenital practice. Patients that have had conduits implanted at the time of primary repair. Patients that have had outflow tract patches, and come back with right ventricular dilatation. What is interesting is that this group of patients may have other abnormalities. For example, ventricular tachycardia in a 21 year old kid about 19 years postrepair had an AICD implanted at the same time. Stenting of the pulmonary arteries, both preoperatively and intraoperatively, is an important component of this. Performing ablation surgery for those patients with flutter is also quite useful. I think that some patients don't do well with homografts in the right ventricular outflow tract. Those patients are those with small distal pulmonary arteries, patients with a very flat chest, patients with pulmonary hypertension or a single pulmonary artery. In those patients, if they have right ventricular dysfunction, you need a competent valve which is going to perform predictably. In those patients, we have used porcine valves, and we have used a bovine pericardial valve in one patient. It is rare that we would do a repair in one out of 36 patients, and leave them without a valve if they have come to us with a problem of the right ventricular outflow tract. The reason for that is that most of the time we can put an oversized homograft in, and unless an unusual immune reaction occurs, the homograft will function reasonably well for a prolonged period of time. Putting a pericardial valve or a porcine valve in guarantees that the patient will require another operation. Pulmonary artery stents are a tremendous adjunct to our practice at the present time. There are very useful for distal and tubular stenoses. They are useful for stenoses behind the aorta or behind the pericardial reflection, areas that tend to restenose with traditional patch techniques. Most of the time these are done prior to operation. Occasionally, we put them in in the operating room when the patient comes to us from an outside hospital where they don't have stent capabilities. Occasionally, the cardiologist is unable to get a stent into a small pulmonary artery in the cath lab. When the stents need to be very close together at the confluence such as after truncus, it is sometimes better to put them in in the operating room. We find this to be a technique that we are becoming more and more comfortable with. We use intraoperative fluoroscopy and we are able to position these very easily. It takes less time than putting bilateral extensive patches in. Here is a right ventricular to pulmonary artery conduit which is stenotic. It has been dilated, and a stent has been implanted. This is a short-term solution because the stents tend to fracture over time due to the motion of the heart. In a small child, this will buy time to allow for implantation of a larger conduit. This is an interesting patient with single ventricle who had had a Fontan procedure, and has a very small left pulmonary artery. A stent was implanted and dilated to about 9.7 mm. The patient came back and was redilated at a later date to 12. mm, and in addition had a stent in this atrial pulmonary connection. This is not a fun group of patients to reoperate on, so they are not going to be appearing in our experience if we can avoid it. You will see patients coming in with incomplete atrioventricular canal defects, patients without a VSD, and I have seen patients with a trileaflet valve that was fully competent in the third and fourth decades of life. Some of these patients also come back with left AV valve regurgitation. This is not a mitral valve. It requires some different approaches to management. We try not to put rings in these patients because annular dilatation is often not the primary problem. The problem is treated by closing the septal cleft which almost invariably elongates in patients that were repaired in early infancy or early childhood, and then using segmental annuloplasty stitches. In selected patients, we have shortened chordae or occasionally sewn the leaflets together a bit in sort of a modified, modified Alfieri approach. There was a death in this series which points out the problem of doing too much too late. A 17 year old female with Down's Syndrome had a complete atrioventricular canal repaired at five months of age. She had a repair of the left valve at six years of age. Came back in with severe stenosis and insufficiency. She had morbid obesity, underwent emergency mitral valve repair, and died postoperatively of respiratory failure. I think that it is not a curative lesion. In the mitral category, there are two kinds of patients that we may be seeing more of. Occasionally, a teenager will come in with a history of having had total anomalous pulmonary venous drainage repaired in infancy. The vein communication may be narrow, and this can be successfully repaired without much ado with improvement. The valves then that we recommend in these patients is in the aortic valve, I personally recommend surgical repair, although I rarely have the opportunity. I think that balloon dilatation of the aortic valve in teenagers often results in dehiscence of a leaflet. Our primary operation for young adults is the Ross procedure. A homograft is the second choice. We avoid mechanical valves, and, in particular, we avoid the cono procedure with a mechanical valve because it has a deleterious effect on ventricular function. Mitral valves we try to repair whenever possible. Mechanical is really your only good choice. Tricuspid valve, repair the tricuspid valve plus a bidirectional Glenn may salvage a situation where valve replacement would have been necessary otherwise. There are some data that suggest that porcine valves are appropriate in the tricuspid position. I am not absolutely convinced. Mechanical valves are unattractive because of the need for Coumadin, and we have used one homograft as a top hat in the tricuspid position. Pulmonary valve, I have already spoken about the need for a porcine valve when a clearly competent valve is needed for the next five to eight years. In the Ross procedure, we think root replacement with appropriate annular modification is the way to go. The pulmonary homograft should be oversized. In our series, two patients came to us who were teenagers who had had a growth spurt. They had had the Ross procedure elsewhere, and both of them had to have the homograft removed. This valve should be at least 4 mm larger than the valve you take out. It is easy to put an oversized homograft in these patients. Operations on the aorta, occasionally patients will present today with primary coarctation. It is unusual for us because of the kind of anatomy that we see to do a primary resection in this group, although it can easily be done. We have tended to use patches or tube grafts, and the same thing is true for redo coarctation where we have used a bypass graft or occasionally the abdominal aorta to ascending aorta kind of graft. Interrupted aortic arch patients are coming back, having outgrown their arch graft. These patients require a treatment by the same techniques that one would use with complex coarctation. Occasionally, these patients are done with bypass, sometimes without bypass. They are reports in the literature of replacing these grafts with deep hypothermia and circulatory arrest performed through the left chest. Here is a picture from Denton Cooley's textbook showing the ascending aorta to supraciliac abdominal aortic graft. This is an attractive final bypass for somebody with a complex aortic problem. However, many of these patients have aortic valve disease, and so I think one has to have some consideration for how many more times you are going to have to come into the aortic root. Patients who have a PDA today now can be treated by a coil. They can have a sack if it is a large one, and video assisted thoracic surgery can be utilized for these patients although we don't perform that particular procedure. Here is a patient with a PDA that has a coil in it completely eliminating flow. Here is a patient with a sack, GG, a Geinterco-Griscus sack, Geingterco was an interventional radiologist here in San Antonio. There is an example of what can be done in larger PDA patients. With complex coarctation patients, Tachy______ arteritis, previous aortic jump graft. Here is the native aorta which is extremely narrow. Here, as a palliative procedure, the cardiologist was able to put a stent in and open that up so that the combined cross-sectional area was adequate. In coarctation of the aorta, it looks like interventional techniques including stenting are being used in many patients with discreet coarctation. This means that the surgical cases are more complex, and less amenable to simple resection. Older patients that come to us seem to have mild stenosis with inadequate collaterals or complex stenoses best treated by bypass grafts. Most of these patients are not normotensive with exercise or stress, and many of them are hypertensive at rest despite the surgery. Usually, they can be made normotensive with much less medication following the surgical procedure. In coarctation surgery in the adult, particularly in these patients with poor collaterals, I think it is important to monitor distal perfusion pressure, consider a shunt or partial bypass if it is a complex lesion, and limit the dissection of the redo aorta. I don't know whether measuring evoked potentials is useful in this group of patients. We typically do it and cerebrospinal fluid pressure manipulation is not something that we have any familiarity with. Older transposition patients are coming in, and when they are reoperated on, they need to have their pulmonary arteries repaired. Occasionally, an arterial switch procedure is useful to convert a patient with a prior atrial switch procedure. These are complex and difficult operations, and often the aortic valve itself needs to be redone after the procedure. In this small series of patients, two of the patients required reoperation in the eight year period for complex anatomic problems, namely aortic insufficiency following conversion to the arterial switch condition. There is a report of a previous atrial switch patient of about 36 years of age that was converted successfully by DeLeon and his group in Chicago. Here is a mustard patient with complete obstruction of the upper baffle treated by a stent. That is another kind of operation that we don't have to do. In our hands, the age of the Fontan has been slowly dropping to below two years of age. It means that we don't see very many older patients who need a Fontan procedure. We have seen two patients during this eight year period. We have certainly seen a lot of patients with difficulties after the Fontan procedure, whether it was performed at our institution or elsewhere. Most of these patients have arrhythmias. They have subaortic stenosis. They have leaky AV valves, and they have VSDs that may need to be enlarged. The only mortality in this group was a 23 year old male fourteen years post-atriopulmonary connection in chronic renal failure on peritoneal dialysis with AV valve regurgitation and a possible hepatic tumor. The purpose of revising his Fontan was to get him into better shape for his hepatic surgery. It was not a successful endeavor. Here is our ablation protocol at the present time. This is the right atrium. Here is the connection to the pulmonary artery. If you are converting this to a total extracardiac conduit, you are going to disconnect it here and you are going to disconnect it there. Then you make a cut from the IVC to the tricuspid valve; a cut from the coronary sinus to the tricuspid annulus; a cut across the cristaterminalis; and then a cut from the superior vena caval orifice over to there which is probably part of your resection. This is all put back together. The right atrium is downsized considerably by this. That leaves room for the conduit, and we typically put a transvenous pacing electrode inside the atrium because of the difficulty with obtaining good, long-term epicardial atrial pacing. Pacemakers aren't worth commenting on. What is worth commenting on are the epi-endocardial atrial lead when you can't use a transvenous electrode as in single ventricle. This can be done without cardiopulmonary bypass by making a little hole in the right atrium, putting the electrode through, and screwing it into the endocardium of the atrium and then fixing it with sutures. We have also found that the subxiphoid approach is useful when going back for a third or fourth time pacemaker. Even when a dual chambered pacer is indicated, we can go up and get at the atrium from that approach. Here is a picture of a Fontan revision with an atrial screw-in electrode implanted at the time of the ablation procedure, and then brought out to the outside of the right atrium and down along the heart border and put to an abdominal battery box. Other palliative operations have not appeared much in our series. There are published reports from UCLA and the Mayo Clinic about late reoperations on patients with Mapcas that have been previously unifocalized. I think there will be a diminution in this type of patient based on the good early results that have been seen with Frank Handley's approach of primary complete repair in infancy. One important caveat regarding the use of the bi-directional caval pulmonary shunt in these older patients is the patient always needs a source of auxiliary pulmonary blood flow. While we have advocated no accessory pulmonary blood flow in infants, in older patients they will be quite cyanotic if they don't have an extra shunt. The operative mortality for this group of 181 patients was 1.7%. I mentioned the three patients. One was an emergency surgery in a very obese patient with a lot of ventricular dysfunction. One was an operation that may have been inappropriate in a patient in chronic renal failure given the low cardiac output state generally obtained after a Fontan revision. Improper preoperative assessment is going to be a source of morbidity and mortality in these older patients. It is really important that we look very carefully at the data lest one jumps in and tries to do something that appears simple, and then has a failure. In follow-up of the entire group of patients, in general all of the patients were improved. I did not attempt to contact them, but we follow most of the local patients. Certainly, those that were cured by operation, all of the Ross procedures, have done well and none of them have required a reoperation. In the Fontan group, this is a subset of patients that need some thought. There were two completions, and one of those patients had poor ventricular function preoperatively. His ventricular function was decreased later, and he died at the time of transplantation. He probably should have been referred for transplantation. These arrows in the slides, that was supposed to be an arrow pointing down. I had revised the slides before I left, and I didn't look at it until yesterday and discovered that the revision was wrong. In the revision patients, all of the patients that survived the operation, seven of the eight are improved to varying degrees. They all have improved exercise tolerance, and decreased arrhythmias on less or no medications. None of them has had flutter since the ablation procedure. In summary, the type of operation utilized to treat congenital heart disease in the adolescent and the young adult is constantly evolving, and I am sure that five years from now the talk will be different with even greater emphasis on interventional techniques. There are fewer untreated patients in North America, and there are an increasing number of long-term survivors of complex repairs in infancy. We are going to be seeing total vein patients, and the right heart bypass patients coming back for repetitive operations. Interventional techniques have replaced surgery very successfully in some areas. For example, patent ductus arteriosus, pulmonary stenosis, recurrent coarctation, and peripheral pulmonary artery stenosis. Interventional techniques show promise in the treatment of secundum ASD. I don't believe that interventional techniques are superior or even equal to surgery for aortic stenosis, and I believe they are contraindicated for congenital mitral stenosis. Balloon dilating an aortic valve, a dysplastic or abnormal aortic valve in infancy or early childhood, has several possible outcomes and only one of them is good. We need better, cheaper, and nonthrombogenic heart valves for use in children, adolescents, and young adults so that we can get away from this constant reoperation and reimplantation of valves in the right heart. Thank you very much. |
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