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Do we really correct congenital heart defects?
Volume 97,
Number 1
January 1989
THORACIC AND CARDIOVASCULAR SURGERY The Journal
of
J THORAC CARDIOVASC SURG 1989;97: 1-9
Honored Guest's Address
Do we really correct congenital heart defects? J. Stark, FRCS, FACS, FACC, London, England
I
t is a great honor for me to address your Association. I should like to share this honor with my colleagues from the Cardiothoracic Unit at The Hospital for Sick Children, Great Ormond Street. I should also like to express my gratitude to my teachers: in Czechoslovakia, Dr. Kudr and Professor Kafka (Fig. 1); and in London, David Waterston and Eoin Aberdeen (Fig. 2). Our department has had close links with American cardiac surgery. Many bright young men and women from your country have spent an extra year of training with us, and I am delighted that they are doing a superb job in pediatric cardiac surgery all over North America. When your president asked me to deliver this address I was delighted, but I was also concerned about the choice of topic. After some hesitation I have decided to discuss the question: "Do we really correct congenital heart defects?" As you know, many operations are called "corrective." The word "correction" is used liberally in the surgical literature, but I am not aware of
From The Hospital for Sick Children, Great Ormond Street, London, England. Read at the Sixty-eighth Annual Meeting of The American Association for Thoracic Surgery, Los Angeles, Calif., April 18-20, 1988. Address for reprints: Mr. J. Stark, Consultant Cardiothoracic Surgeon, The Hospital for Sick Children, Great Ormond Street, London, WClN 3JN, England.
any accepted definition. Ideally, an operation for congenital heart defects should be considered corrective if (1) normal function is achieved and maintained, (2) life expectancy is normal, and (3) further medical or surgical treatment is not necessary. Would any operation qualify for the term "correction" according to these criteria? I believe not, because we do not know yet whether our patients will reach normal life expectancy or ultimately die of causes unrelated to the congenital heart defects. The length of follow-up is therefore crucial to our definition. I have reviewed the articles evaluating long-term results that have been were published in THE JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY during the past 2 years. Sixteen (or 39%) discussed follow-up times of less than 4 years, 21 (51%),4 to 8 years, and only four (10%) had follow-ups of more than 8 years. I believe it is important to know what has happened to patients at 4 and 8 years. However, with increasing survival rates after most operations, we should try to obtain long-term follow-up after 20 or 25 years. Perhaps I should replace "normal life expectancy" in my definition with "normal health during the long-term follow-up." Let us now examine the list of various congenital heart defects and see which could be considered correctable (Table I). According to the aforementioned definition, probably only two lesions are truly correctable: persistent ductus arteriosus and atrial septal defect. Even these defects are truly corrected only if the
2
The Journal of Thorac ic and Cardiovascular Surgery
Stark
Fig. 1. Professor V. Kafka (left) and Dr. J. Kudr (right).
Fig. 2. Mr. E. Aberdeen (lef t) and Mr. D. J. Waterston (right).
operation is performed early, that is, before the development of pulmonary vascular changes. All the operations for lesions listed in the middle column in Table I are generally called corrective. They may achieve a complete repair for individual patients.
However, early or late complications will develop in some of these patients. Some will necessitate further medical or even surgical treatment. Some complications will lead to disability or death . You may be surprised that I have put a question mark by correction of
Volume 97 Number 1 January 1989
Congenital heart defects
3
Table I. Congenital heart defects Correction
Correction?
Patent ductus arteriosus Atrial septal defect
Coarctation of aorta Transposition of great arteries Ventricular septal defect Fallot's tetralogy Pulmonary valve stenosis Aortic stenosis
Palliation?
Conduits Transplantation Definitive shunts Operations in pulmonary vascular obstructive disease Pulmonary atresia + ventricular septal defect + collaterals
Table II. Cardiac operations for transposition of the great arteries (1965-1987) Type
No.
Simple (Mustard, Senning, switch) Complex (plus closure of ventricular septal defect, left ventricle- pulmonary artery conduit, Rastelli)
467
Total
990
523
Fig. 3. Aortic homograft extended with Dacron graft. The conduit is placed between the apex of the left ventricleand the junction of the main and left pulmonary arteries. AHo, aortic homograft; DE. Dacron extension. Table
m. Extracardiac valved conduits
(1971-1985)
Type
coarctation of the aorta. Since the first repair of coarctation by Crafoord in 1941, I many have considered this operation corrective. The recent long-term follow-up from Green Lane Hospital' concluded that only about 20% of patients operated on for coarctation of the aorta after 1 year of age will be free of residual or recurrent cardiovascular disease 25 years later. In the same review the authors pointed out that aneurysm of the aorta at the site of aortoplasty repair may develop as late as 20 years after operation. Another lesion I should like to discuss is transposition of the great arteries. Since the first Mustard operation performed in our hospital in 1965, we have performed 990 corrections for transposition and associated lesions (Table II). Originally we were pleased with the results of the Mustard operation. In 1974 we' reported a 96% survival rate in a group of 52 infants younger than 1 year. At that time we used to tell the parents that, although the operation provided only physiologic rather than anatomic correction, the anomaly could be considered corrected. Today, 14 years later, we know better. Late complications developed in some of these patients. Only 31 of the 50 survivors (62%) are free of symptoms and live what we would call a normal life. To illustrate the change from our early optimism to current realism, I should like to present the case history of a child with transposition of the great arteries treated in 1975.
No.
Truncus arteriosus Transposition of great arteries + ventricular septal defect + left ventricular outflow tract obstruction Corrected transposition of great arteries Pulmonary atresia and ventricular septal defect Other
104 82
Total
395
66 51 92
C A SE I. The infant was admitted at the age of IO hours. Cardiac catheterization established the diagnosis of transposition with ventricular septal defect (YSD) and a small ductus arteriosus. Balloon septostomy was performed and her condition improved. However, on day 2 her status deteriorated and she became hypoxic and oliguric despite repeat balloon septostomy. She required peritoneal dialysis. On day 3 a Mustard operation was performed. She recovered well and was discharged from the hospital in excellent condition. At that time we did not know the long-term outlook of a neonate after the Mustard operation. Since her development was normal throughout her infancy, it was tempting to consider her condition corrected. However, at routine cardiac catheterizationat the age of 2 years, a highleftventricularpressure was measured. As several of our patients had tolerated a high left ventricular pressureafter the Mustard operation, we decided to wait. At the age of IO years, repeat cardiac catheterization showed a left ventricular pressure of 200 mm Hg. We electedto inserta left ventricle-pulmonary artery conduit, an operation that we' first performed in 1974. Through a left thoracotomy, bypass was established by cannulating the descending aorta and the left atrium which, after the Mustard operation, becomes the systemic venous atrium. An aortic homograft, extended with a Dacron tube,
4
Stark
The Journal of Thoracic and Cardiovascular Surgery
Fig. 4. A, The obstructed conduit is transected and removed. Note a thick neointima inside the Dacron tube. B, A new aortic homograft has been sutured in place. The right ventricular outflow tract is completed with a homograft anterior leaflet of the mitral valve. (Reproduced, with the Publisher's permission, from Stark and Pacifico (Eds), Re-Operations in Cardiac Surgery, Springer-Verlag, London, Berlin, Heidelberg, New York, Paris and Tokyo, 1989.)
was placed between the apex of the left ventricle and the pulmonary artery (Fig. 3). The left ventricular pressure was reduced from 200 to 45 nun Hg, with a pulmonary artery pressure of 30 nun Hg. Today, 3Jh years later, at the age of 14 years, she is fully active and requires no medication. However, from our own late follow-up of children with transposition and from the data published in the literature, we know that she remains at risk for arrhythmias, right ventricular dysfunction, and possibly conduit obstruction.
Fig. 5. Patient 2 with her daughter 26 months after conduit replacement. (Published with permission of The Hospital for Sick Children.)
It is clear that the Mustard and Senning operations do not fulfill my criteria for correction. Some of the complications observed after the Mustard and Senning operations do not occur after the Jatene arterial switch operation. Therefore, today the switch operation is considered corrective. But is it? Some of the recent reports describe complications and reoperations even after this operation. These include right and left ventricular outflow tract obstruction, pulmonary branch stenosis, aortic valve incompetence, and coronary artery problems." In the group called "palliation" in Table I, I have included patients who will almost certainly require at least one reoperation, such as those with extracardiac valved conduits or transplantation, or patients who are either permanently disabled or who will die prematurely, such as those with definitive shunts and those who had pulmonary vascular disease at operation. I shall not discuss the Fontan operation. I shall merely mention that Fontan's concept opened new horizons and
Volume 97 Number 1
Congenital heart defects 5
January 1989
100~-_
118
%
21
survival of homografls
80
<21 days
60
40
20 >21 days
2345678
9
10
11
12 years
Fig. 6. Length of homograft storage related to risk of obstruction (Kaplan-Meier).
enabled us to operate on many complex congenital heart defects.
Extracardiac valved conduits are another milestone in the treatment of complex congenital heart defects (Table III). They were introduced into clinical practice by Ross and Somerville" in 1966. Repair of truncus arteriosus may be a rewarding operation. To my knowledge, nobody has been able to come close to the outstanding results of your president, Dr. Ebert. As my colleagues and I have more experience with the treatment of transposition of the great arteries, I have selected my next case from this group. It is a classic example of the use of a valved conduit in the Rastelli operation. CASE 2. The child had transposition of the great arteries, multiple VSDs, and left ventricular outflow tract obstruction. Because of increasing cyanosis she underwent a left BlalockTaussigshunt at the age of 8 years. When she was 12 years old an elective Rastelli operation was performed, with an aortic homograft being placed between the right ventricle and the pulmonary artery. At the end of the operation the intracardiac pressures were normal. She was discharged after an uncomplicated recovery and subsequently did very well. Eight years after the operation, at the age of 20, she became more tired and complained of palpitations. Cardiac catheterization and angiocardiography showed obstruction of the conduit and stenosis of the conduit valve. At an elective reoperation the conduit was removed. A thick neointima was present, and the homograft valve itself was calcified and stenosed (Fig. 4, A). The conduit was replaced with a new aortic homograft (Fig. 4, B). After I year she sent me a picture
of her 2-month-old daughter. Two years after operation both she and her daughter are well (Fig. 5).
The aortic homograft conduit first enabled us to repair her heart. Eight years later it became a cause for reoperation. The new conduit functions well, but will it last for her entire life span? Probably not. A variety of conduits have been.developed and used. They have included aortic homografts and heterografts, such as porcine, bovine, or fascia lata valves, prosthetic valves, and pulmonary homografts. We 9 studied the performance of valved conduits in 249 patients. Both homografts and heterografts have deteriorated with time. Ten years after operation only a small proportion of patients had a well-functioning original conduit. The results of aortic liomograft conduits presented by Professor Fontan and his associates'? were better. There were no reoperations among their 25 patients who received a homograft for repair of tetralogy of Fallot of pulmonary atresia with a mean follow-up of 12 years. To understand this difference, my colleague, Dr. Almeida, reviewed our data on 176 patients who also received aortic homograft conduits. He found that two factors were associated with obstruction: the length of its storage and the use of a Dacron extension. The study demonstrated that homografts used within 3 weeks of dissection performed better than homografts used between 3 and 6 weeks. The difference was statistically significant (Fig. 6). Ninety-three percent of homografts used alone were free of obstruction 8 years after
The Journal of Thoracic and Cardiovascular Surgery
6 Stark
T
Fig. 7. Diagram of an operation in patient 4. A preclotted woven Dacron conduit was placed between the ascendingand descendingaorta. The aortic arch was transected between the left carotid and left subclavian arteries to relieve the tracheal compression. AA, Ascendingaorta; DA, descending aorta; CO, conduit; LPA, left pulmonary arter; SA, subclavian artery; T, trachea. (Modified from Szamicki R, Maurseth K, de Leval MR, Stark J. Tracheal compression by the aortic arch following right pneumonectomy in infancy. Ann Thorac Surg 1978;25:231-5.) insertion, compared with only 40% of homografts that were extended with a Dacron tube. We now hope that by using fresher homografts and avoiding the Dacron extension, we will prolong the durability of aortic homograft conduits. I would like to stress that, despite the problems with the durability of valved conduits, they have enabled us to return many patients with complex congenital heart defects to a normal life. Replacement or rereplacements of the conduit will obviously be unpleasant events for the patients and their families. However, as in the second case that I reported, we can hope that new conduits will assure a normal life for another period of time. Thus the distinction between correction and palliation becomes unclear. Pulmonary vascular obstructive disease is generally considered to be inoperable. However, there are situations in which even such patients can be helped by surgical treatment.
CASE 3. The child was initiallyobserved in another hospital and was not referred to us until she was 5 years old. At catheterization, we found that she had transposition with intact ventricular septum, but pulmonary arteriolar resistance was increased to 14 units· m'. We were convinced that with this degree of pulmonary vascular obstructive disease she would not survive the Mustard operation. As we had a good experience with the palliative Mustard operation for transposition of the great arteries associated with YSD," I suggested to the family that we perform a Mustard operation and, in addition, make a YSD. The YSD was created in the apical portion of the ventricular septum." Although the Mustard operation was slightly complicated by left juxtaposition of the atrial appendages, her postoperative course was uneventful. One year after operation the YSD was open, as expected, but the pulmonary resistance remained high. She was fully active, receiving no medication, and later started participating in sports. Today, at the age of 18 years, she enjoys a normal life.
This operation was clearly a palliative one right from the beginning. Neither the parents nor the patient was ever in doubt about the nature of this operation. They understand the prognosis of the Eisenmenger condition, with consequently reduced life expectancy. Nevertheless, they are delighted with the result. The progress in heart-lung transplantation may offer further hope for her in the future. Other congenital, noncardiac defects may also cause cardiovascular problems. CASE 4. The infant was born with an esophageal bronchus. At the age of 8 months the abnormal hypoplastic right lung was removed, and severe stridor developed. The chest x-ray film showed that the heart was rotated to the right and the angiogram demonstrated that the aortic arch was compressing the trachea and producing an unusual form of vascular ring. We 13 reported the treatment of this child in 1978. A conduit was placed between the ascending and descending aorta and the aortic arch was transected between the left carotid and subclavianarteries to relieve the tracheal compression (Fig. 7). The stridor disappeared and the child was able to lead a normal life. Today, at the age of 18 years, she participates in cross-country skiing,gymnastics,and dancing, despitethe fact that she has only one lung and has some rather strange plumbing inside her chest. This is another example of a palliative operation after which the patient performs as if she has had a corrective procedure.
Patients with pulmonary atresia and VSDs are usually grouped under the heading of Fallot's tetralogy. Yet this condition represents a very wide spectrum of lesions. Patients with Fallot's tetralogy may have congenital or acquired pulmonary valve atresia. An alternative blood supply must be established either through an aortopulmonary shunt (Fig. 8, A) or with an outflow tract patch or a conduit. Other patients with pulmonary atresia and VSD may have no main pulmonary artery, the pulmonary blood supply being via a large aortopulmonary collateral (Fig. 8, B). Patients from both these sub-
Volume 97 Number 1 January 1989
Congenital heart defects
7
Fig. 8. A, Pulmonary angiogram in a patient with pulmonary valve atresia and VSD. The pulmonary artery opacifies through the left modified Blalock-Taussig shunt. B, Pulmonary angiogram in a patient with pulmonary atresia and VSD. Injection is into a large aortopulmonary collateral that originated from the inominate artery in the neck. (From Stark J, Huhta JC , Macartney FJ . Palliative surgery for pulmonary atresia with ventricular septal defect. In: Anderson RH , Macartney FJ, Shineboume EA, Tynan M, 008. Paediatric cardiology. Vol. 5. Edinburgh: Churchill Livingstone, 1983:126-36.) C, Selective injection into the aortopulmonary collateral demonstrates grossly hypoplastic central pulmonary arteries and abnormal distribution of pulmonary blood flow to the periphery of the lungs.
groups can usually be treated as if they have tetralogy of Fallot. Their outlook is generally satisfactory, as for other patients with Fallot's tetralogy. However, there are others with pulmonary atresia and VSD whose
central pulmonary arteries are severely hypoplastic and whose pulmonary blood supply is via multiple aortopulmonary collaterals. An example of this extreme end of the spectrum is shown in Fig. 8, C.
8
Stark
The history of the next patient demonstrates some of the problems that we face during treatment of such patients. CASE 5. The patient had been cyanotic since birth, and the diagnosis of pulmonary atresia with VSD and multiple collaterals was made after catheterization in another hospital. At exploratory thoracotomy at the age of 3 years, the pulmonary arteries were considered to be too small for a shunt. She was later referred to us. The angiocardiogram showed hypoplastic central pulmonary arteries, only 3 mm at the bifurcations, with several major collaterals. We decided to place a right ventricle-pulmonary artery conduit. Her condition improved after this operation. Two years later, a biopsy specimen was taken from the lung, and the two major collaterals were ligated. At the end of the operation the pulmonary artery pressure was 50 mm Hg and the aortic pressure, 100 mm Hg. She was restudied at the age of 12 years. Although the appearance of the pulmonary arteries was grossly abnormal on angiocardiogram, in view of the histologic report and the catheterization data we decided to proceed with the intracardiac repair. The VSD was closed, the remaining collateral was ligated, the bifurcation was reconstructed, and an aortic homograft was placed between the right ventricle and the pulmonary artery. Right ventricular pressure after repair was 70 mm Hg, pulmonary artery pressure 50 mm Hg, and left ventricular pressure 85 mm Hg. After initial improvement, the child was readmitted in severe heart failure. At restudy biventricular failure with the right ventricular pressure at systemic level was found. Pulmonary arteriolar resistance was calculated to be 20 units. m', She was discharged and 2 months later died at home. This was a very sad end to a long saga starting first with very little hope, progressing through ever-increasing hope, and then, after what could be called a successful repair, ending in eventual deterioration and death.
Patients like this child represent a small proportion of patients with pulmonary atresia and VSD. Between 1979 and 1986 we operated on 173 patients with pulmonary atresia and VSD. Twenty-six of them were at this difficult end of the spectrum. In the past we" have treated such patients with a combination of several palliative procedures, including shunts, right ventricular patches or conduits, and anastomoses between collaterals and pulmonary arteries, to prepare them for eventual repair. In 1987 we" reviewed the results of our surgical efforts. Four of 26 patients undergoing complex palliation died. In 13 the anatomy and physiology at restudy were considered incompatible with correction. Only three had a corrective operation, and two of them died. Four others are considered candidates for correction and two are awaiting restudy. These results are disappointing and suggest that some patients with pulmonary atresia, VSD, and multiple collaterals should not be subjected to conventional palliation, but perhaps should be considered as candidates for heart-lung transplantation.
The Journal of Thoracic and Cardiovascular Surgery
After this review I realize that it is difficult to answer my original question: "Do we really correct congenital heart defects?" Using my initial criteria, I can say yes, we do correct, but not very often. However, should we use such strict criteria? I have looked up the definition of correction in the Oxford Dictionary: "To correct is to set right, to substitute right for wrong or to bring into accordance with a good standard." Translating this definition into our surgical practice, we could call an operation corrective if the patient is likely to be well and lead a normal life. Subsequent medication, or perhaps even reoperation, may be acceptable. On the other hand, if it is likely that the patient will be disabled or his life span much shortened, perhaps it would be appropriate to call such an operation palliative. As I have shown, there are situations in which the distinction between palliation and correction remains unclear. We must observe our patients during their entire life. The information thus obtained will confirm that some defects are truly corrected but that others are only palliated. If the results achieved by techniques currently available are clearly unsatisfactory, as I have demonstrated in the subgroup of patients with pulmonary atresia, hypoplastic pulmonary arteries, and multiple collaterals, we may decide not to operate on some patients at all. This will avoid considerable suffering and grief for the families of such children. I hope that further progress will follow in years to come. The major discoveries will probably not be in the surgical knitting and stitching but in finding the causative mechanisms of congenital heart defects. It may be that one day congenital heart defects will be preventable. We will then not have to worry whether we correct or palliate. We may all be out of a job by then. REFERENCES 1. Crafoord C, Nylin G. Congenital coarctation of the aorta and its surgical treatment. J THORAC SURG 1945;14:34761. 2. Clarkson PM, Nicholson MR, Barratt-Boyes, BG, Neutze JM, Whitlock RM. Results after repair of coarctation of the aorta beyond infancy: a 10 to 28 year follow-up with particular reference to late systemic hypertension. Am J CardioI1983;51:1481-8. 3. Stark J, de Leval M, Waterston DJ, Graham GR, Bonham-Carter RE. Corrective surgery for transposition of the great arteries in the first year of life. J THORAe CARDIOVASC SURG 1974;67:673-81. 4. Singh AK, Stark J, Tylor JFN. Left ventricle to pulmonary artery conduit in treatment of transposition of the great arteries, restrictive ventricular septal defect and acquired pulmonary atresia. Br Heart J 1976;38:1213-6. 5. Muster AJ, Berry TE, Ilbawi MN, et al. Development of neo-coarctation in patients with transposed great arteries
Volume 97
Congenital heart defects 9
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January 1989
6.
7.
8. 9.
10.
11.
and hypoplastic aortic arch after Lecompte modification of anatomical correction. J THORAC CARDIOVASC SURG 1987;93:276-80. Martin RP, Ladusans EJ, Parsons JM, Keck E, RadleySmith R, Yacoub MH. Incidence and site of pulmonary stenosisafter anatomical correction of transposition of the great arteries [Abstract]. Br Heart J 1988;59:122-3. Sidi D, Planche C, Kachaner J, et al. Anatomic correction of simple transposition of the great arteries in 50 neonates. Circulation 1987;75:429-35. Ross DN, Somerville J. Correction of pulmonary atresia with a homograft aortic valve. Lancet 1966;2:1446-7. Bull C, Macartney FJ, Horvath P, et al. Evaluation of long-term results of homograft and heterograft valves in extracardiac conduits. J THORAC CARDIOVASC SURG 1987; 94:12-9. Fontan F, Choussat A, Deville C, Doutremepuich C, Coupillaud J, Vosa C. Aortic valve homografts in the surgical treatment of complex cardiac malformations. J THORAC CARDIOVASC SURG 1984;87:649-57. Lindesmith GG, Stiles QR, Tucker BL, Gallaher ME,
12.
13.
14.
15.
Stanton RE, Meyer BW. The Mustard operation as a palliative procedure. J THORAC CARDIOVASC SURG 1972; 63:75-80. Stark J, de Leval MR, Taylor JFN. Mustard operation and creation of ventricular septal defect in two patients with transposition of the great arteries, intact ventricular septum and pulmonary vascular disease. Am J Cardiol 1976;38:524-7. Szamicki R, Maurseth K, de Leval MR, Stark J. Tracheal compression by the aortic arch following right pneumonectomy in infancy. Ann Thorac Surg 1978; 25:231-5. Stark J, Huhta JC, Macartney FJ. Palliative surgery for pulmonary atresia with ventricular septal defect. In: Anderson RH, Macartney FJ, Shineboume EA, Tynan M, eds. Paediatric cardiology. Vol 5. London: Churchill Livingstone, 1983:126-36. Sullivan 10, Wren C, Stark J, de Leval MR, Macartney FJ, Deanfield JE. Surgical unifocalization in pulmonary atresia with ventricular septal defect. A realistic goal? Circulation [In press].
Number 1
January 1989
THORACIC AND CARDIOVASCULAR SURGERY The Journal
of
J THORAC CARDIOVASC SURG 1989;97: 1-9
Honored Guest's Address
Do we really correct congenital heart defects? J. Stark, FRCS, FACS, FACC, London, England
I
t is a great honor for me to address your Association. I should like to share this honor with my colleagues from the Cardiothoracic Unit at The Hospital for Sick Children, Great Ormond Street. I should also like to express my gratitude to my teachers: in Czechoslovakia, Dr. Kudr and Professor Kafka (Fig. 1); and in London, David Waterston and Eoin Aberdeen (Fig. 2). Our department has had close links with American cardiac surgery. Many bright young men and women from your country have spent an extra year of training with us, and I am delighted that they are doing a superb job in pediatric cardiac surgery all over North America. When your president asked me to deliver this address I was delighted, but I was also concerned about the choice of topic. After some hesitation I have decided to discuss the question: "Do we really correct congenital heart defects?" As you know, many operations are called "corrective." The word "correction" is used liberally in the surgical literature, but I am not aware of
From The Hospital for Sick Children, Great Ormond Street, London, England. Read at the Sixty-eighth Annual Meeting of The American Association for Thoracic Surgery, Los Angeles, Calif., April 18-20, 1988. Address for reprints: Mr. J. Stark, Consultant Cardiothoracic Surgeon, The Hospital for Sick Children, Great Ormond Street, London, WClN 3JN, England.
any accepted definition. Ideally, an operation for congenital heart defects should be considered corrective if (1) normal function is achieved and maintained, (2) life expectancy is normal, and (3) further medical or surgical treatment is not necessary. Would any operation qualify for the term "correction" according to these criteria? I believe not, because we do not know yet whether our patients will reach normal life expectancy or ultimately die of causes unrelated to the congenital heart defects. The length of follow-up is therefore crucial to our definition. I have reviewed the articles evaluating long-term results that have been were published in THE JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY during the past 2 years. Sixteen (or 39%) discussed follow-up times of less than 4 years, 21 (51%),4 to 8 years, and only four (10%) had follow-ups of more than 8 years. I believe it is important to know what has happened to patients at 4 and 8 years. However, with increasing survival rates after most operations, we should try to obtain long-term follow-up after 20 or 25 years. Perhaps I should replace "normal life expectancy" in my definition with "normal health during the long-term follow-up." Let us now examine the list of various congenital heart defects and see which could be considered correctable (Table I). According to the aforementioned definition, probably only two lesions are truly correctable: persistent ductus arteriosus and atrial septal defect. Even these defects are truly corrected only if the
2
The Journal of Thorac ic and Cardiovascular Surgery
Stark
Fig. 1. Professor V. Kafka (left) and Dr. J. Kudr (right).
Fig. 2. Mr. E. Aberdeen (lef t) and Mr. D. J. Waterston (right).
operation is performed early, that is, before the development of pulmonary vascular changes. All the operations for lesions listed in the middle column in Table I are generally called corrective. They may achieve a complete repair for individual patients.
However, early or late complications will develop in some of these patients. Some will necessitate further medical or even surgical treatment. Some complications will lead to disability or death . You may be surprised that I have put a question mark by correction of
Volume 97 Number 1 January 1989
Congenital heart defects
3
Table I. Congenital heart defects Correction
Correction?
Patent ductus arteriosus Atrial septal defect
Coarctation of aorta Transposition of great arteries Ventricular septal defect Fallot's tetralogy Pulmonary valve stenosis Aortic stenosis
Palliation?
Conduits Transplantation Definitive shunts Operations in pulmonary vascular obstructive disease Pulmonary atresia + ventricular septal defect + collaterals
Table II. Cardiac operations for transposition of the great arteries (1965-1987) Type
No.
Simple (Mustard, Senning, switch) Complex (plus closure of ventricular septal defect, left ventricle- pulmonary artery conduit, Rastelli)
467
Total
990
523
Fig. 3. Aortic homograft extended with Dacron graft. The conduit is placed between the apex of the left ventricleand the junction of the main and left pulmonary arteries. AHo, aortic homograft; DE. Dacron extension. Table
m. Extracardiac valved conduits
(1971-1985)
Type
coarctation of the aorta. Since the first repair of coarctation by Crafoord in 1941, I many have considered this operation corrective. The recent long-term follow-up from Green Lane Hospital' concluded that only about 20% of patients operated on for coarctation of the aorta after 1 year of age will be free of residual or recurrent cardiovascular disease 25 years later. In the same review the authors pointed out that aneurysm of the aorta at the site of aortoplasty repair may develop as late as 20 years after operation. Another lesion I should like to discuss is transposition of the great arteries. Since the first Mustard operation performed in our hospital in 1965, we have performed 990 corrections for transposition and associated lesions (Table II). Originally we were pleased with the results of the Mustard operation. In 1974 we' reported a 96% survival rate in a group of 52 infants younger than 1 year. At that time we used to tell the parents that, although the operation provided only physiologic rather than anatomic correction, the anomaly could be considered corrected. Today, 14 years later, we know better. Late complications developed in some of these patients. Only 31 of the 50 survivors (62%) are free of symptoms and live what we would call a normal life. To illustrate the change from our early optimism to current realism, I should like to present the case history of a child with transposition of the great arteries treated in 1975.
No.
Truncus arteriosus Transposition of great arteries + ventricular septal defect + left ventricular outflow tract obstruction Corrected transposition of great arteries Pulmonary atresia and ventricular septal defect Other
104 82
Total
395
66 51 92
C A SE I. The infant was admitted at the age of IO hours. Cardiac catheterization established the diagnosis of transposition with ventricular septal defect (YSD) and a small ductus arteriosus. Balloon septostomy was performed and her condition improved. However, on day 2 her status deteriorated and she became hypoxic and oliguric despite repeat balloon septostomy. She required peritoneal dialysis. On day 3 a Mustard operation was performed. She recovered well and was discharged from the hospital in excellent condition. At that time we did not know the long-term outlook of a neonate after the Mustard operation. Since her development was normal throughout her infancy, it was tempting to consider her condition corrected. However, at routine cardiac catheterizationat the age of 2 years, a highleftventricularpressure was measured. As several of our patients had tolerated a high left ventricular pressureafter the Mustard operation, we decided to wait. At the age of IO years, repeat cardiac catheterization showed a left ventricular pressure of 200 mm Hg. We electedto inserta left ventricle-pulmonary artery conduit, an operation that we' first performed in 1974. Through a left thoracotomy, bypass was established by cannulating the descending aorta and the left atrium which, after the Mustard operation, becomes the systemic venous atrium. An aortic homograft, extended with a Dacron tube,
4
Stark
The Journal of Thoracic and Cardiovascular Surgery
Fig. 4. A, The obstructed conduit is transected and removed. Note a thick neointima inside the Dacron tube. B, A new aortic homograft has been sutured in place. The right ventricular outflow tract is completed with a homograft anterior leaflet of the mitral valve. (Reproduced, with the Publisher's permission, from Stark and Pacifico (Eds), Re-Operations in Cardiac Surgery, Springer-Verlag, London, Berlin, Heidelberg, New York, Paris and Tokyo, 1989.)
was placed between the apex of the left ventricle and the pulmonary artery (Fig. 3). The left ventricular pressure was reduced from 200 to 45 nun Hg, with a pulmonary artery pressure of 30 nun Hg. Today, 3Jh years later, at the age of 14 years, she is fully active and requires no medication. However, from our own late follow-up of children with transposition and from the data published in the literature, we know that she remains at risk for arrhythmias, right ventricular dysfunction, and possibly conduit obstruction.
Fig. 5. Patient 2 with her daughter 26 months after conduit replacement. (Published with permission of The Hospital for Sick Children.)
It is clear that the Mustard and Senning operations do not fulfill my criteria for correction. Some of the complications observed after the Mustard and Senning operations do not occur after the Jatene arterial switch operation. Therefore, today the switch operation is considered corrective. But is it? Some of the recent reports describe complications and reoperations even after this operation. These include right and left ventricular outflow tract obstruction, pulmonary branch stenosis, aortic valve incompetence, and coronary artery problems." In the group called "palliation" in Table I, I have included patients who will almost certainly require at least one reoperation, such as those with extracardiac valved conduits or transplantation, or patients who are either permanently disabled or who will die prematurely, such as those with definitive shunts and those who had pulmonary vascular disease at operation. I shall not discuss the Fontan operation. I shall merely mention that Fontan's concept opened new horizons and
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Congenital heart defects 5
January 1989
100~-_
118
%
21
survival of homografls
80
<21 days
60
40
20 >21 days
2345678
9
10
11
12 years
Fig. 6. Length of homograft storage related to risk of obstruction (Kaplan-Meier).
enabled us to operate on many complex congenital heart defects.
Extracardiac valved conduits are another milestone in the treatment of complex congenital heart defects (Table III). They were introduced into clinical practice by Ross and Somerville" in 1966. Repair of truncus arteriosus may be a rewarding operation. To my knowledge, nobody has been able to come close to the outstanding results of your president, Dr. Ebert. As my colleagues and I have more experience with the treatment of transposition of the great arteries, I have selected my next case from this group. It is a classic example of the use of a valved conduit in the Rastelli operation. CASE 2. The child had transposition of the great arteries, multiple VSDs, and left ventricular outflow tract obstruction. Because of increasing cyanosis she underwent a left BlalockTaussigshunt at the age of 8 years. When she was 12 years old an elective Rastelli operation was performed, with an aortic homograft being placed between the right ventricle and the pulmonary artery. At the end of the operation the intracardiac pressures were normal. She was discharged after an uncomplicated recovery and subsequently did very well. Eight years after the operation, at the age of 20, she became more tired and complained of palpitations. Cardiac catheterization and angiocardiography showed obstruction of the conduit and stenosis of the conduit valve. At an elective reoperation the conduit was removed. A thick neointima was present, and the homograft valve itself was calcified and stenosed (Fig. 4, A). The conduit was replaced with a new aortic homograft (Fig. 4, B). After I year she sent me a picture
of her 2-month-old daughter. Two years after operation both she and her daughter are well (Fig. 5).
The aortic homograft conduit first enabled us to repair her heart. Eight years later it became a cause for reoperation. The new conduit functions well, but will it last for her entire life span? Probably not. A variety of conduits have been.developed and used. They have included aortic homografts and heterografts, such as porcine, bovine, or fascia lata valves, prosthetic valves, and pulmonary homografts. We 9 studied the performance of valved conduits in 249 patients. Both homografts and heterografts have deteriorated with time. Ten years after operation only a small proportion of patients had a well-functioning original conduit. The results of aortic liomograft conduits presented by Professor Fontan and his associates'? were better. There were no reoperations among their 25 patients who received a homograft for repair of tetralogy of Fallot of pulmonary atresia with a mean follow-up of 12 years. To understand this difference, my colleague, Dr. Almeida, reviewed our data on 176 patients who also received aortic homograft conduits. He found that two factors were associated with obstruction: the length of its storage and the use of a Dacron extension. The study demonstrated that homografts used within 3 weeks of dissection performed better than homografts used between 3 and 6 weeks. The difference was statistically significant (Fig. 6). Ninety-three percent of homografts used alone were free of obstruction 8 years after
The Journal of Thoracic and Cardiovascular Surgery
6 Stark
T
Fig. 7. Diagram of an operation in patient 4. A preclotted woven Dacron conduit was placed between the ascendingand descendingaorta. The aortic arch was transected between the left carotid and left subclavian arteries to relieve the tracheal compression. AA, Ascendingaorta; DA, descending aorta; CO, conduit; LPA, left pulmonary arter; SA, subclavian artery; T, trachea. (Modified from Szamicki R, Maurseth K, de Leval MR, Stark J. Tracheal compression by the aortic arch following right pneumonectomy in infancy. Ann Thorac Surg 1978;25:231-5.) insertion, compared with only 40% of homografts that were extended with a Dacron tube. We now hope that by using fresher homografts and avoiding the Dacron extension, we will prolong the durability of aortic homograft conduits. I would like to stress that, despite the problems with the durability of valved conduits, they have enabled us to return many patients with complex congenital heart defects to a normal life. Replacement or rereplacements of the conduit will obviously be unpleasant events for the patients and their families. However, as in the second case that I reported, we can hope that new conduits will assure a normal life for another period of time. Thus the distinction between correction and palliation becomes unclear. Pulmonary vascular obstructive disease is generally considered to be inoperable. However, there are situations in which even such patients can be helped by surgical treatment.
CASE 3. The child was initiallyobserved in another hospital and was not referred to us until she was 5 years old. At catheterization, we found that she had transposition with intact ventricular septum, but pulmonary arteriolar resistance was increased to 14 units· m'. We were convinced that with this degree of pulmonary vascular obstructive disease she would not survive the Mustard operation. As we had a good experience with the palliative Mustard operation for transposition of the great arteries associated with YSD," I suggested to the family that we perform a Mustard operation and, in addition, make a YSD. The YSD was created in the apical portion of the ventricular septum." Although the Mustard operation was slightly complicated by left juxtaposition of the atrial appendages, her postoperative course was uneventful. One year after operation the YSD was open, as expected, but the pulmonary resistance remained high. She was fully active, receiving no medication, and later started participating in sports. Today, at the age of 18 years, she enjoys a normal life.
This operation was clearly a palliative one right from the beginning. Neither the parents nor the patient was ever in doubt about the nature of this operation. They understand the prognosis of the Eisenmenger condition, with consequently reduced life expectancy. Nevertheless, they are delighted with the result. The progress in heart-lung transplantation may offer further hope for her in the future. Other congenital, noncardiac defects may also cause cardiovascular problems. CASE 4. The infant was born with an esophageal bronchus. At the age of 8 months the abnormal hypoplastic right lung was removed, and severe stridor developed. The chest x-ray film showed that the heart was rotated to the right and the angiogram demonstrated that the aortic arch was compressing the trachea and producing an unusual form of vascular ring. We 13 reported the treatment of this child in 1978. A conduit was placed between the ascending and descending aorta and the aortic arch was transected between the left carotid and subclavianarteries to relieve the tracheal compression (Fig. 7). The stridor disappeared and the child was able to lead a normal life. Today, at the age of 18 years, she participates in cross-country skiing,gymnastics,and dancing, despitethe fact that she has only one lung and has some rather strange plumbing inside her chest. This is another example of a palliative operation after which the patient performs as if she has had a corrective procedure.
Patients with pulmonary atresia and VSDs are usually grouped under the heading of Fallot's tetralogy. Yet this condition represents a very wide spectrum of lesions. Patients with Fallot's tetralogy may have congenital or acquired pulmonary valve atresia. An alternative blood supply must be established either through an aortopulmonary shunt (Fig. 8, A) or with an outflow tract patch or a conduit. Other patients with pulmonary atresia and VSD may have no main pulmonary artery, the pulmonary blood supply being via a large aortopulmonary collateral (Fig. 8, B). Patients from both these sub-
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Congenital heart defects
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Fig. 8. A, Pulmonary angiogram in a patient with pulmonary valve atresia and VSD. The pulmonary artery opacifies through the left modified Blalock-Taussig shunt. B, Pulmonary angiogram in a patient with pulmonary atresia and VSD. Injection is into a large aortopulmonary collateral that originated from the inominate artery in the neck. (From Stark J, Huhta JC , Macartney FJ . Palliative surgery for pulmonary atresia with ventricular septal defect. In: Anderson RH , Macartney FJ, Shineboume EA, Tynan M, 008. Paediatric cardiology. Vol. 5. Edinburgh: Churchill Livingstone, 1983:126-36.) C, Selective injection into the aortopulmonary collateral demonstrates grossly hypoplastic central pulmonary arteries and abnormal distribution of pulmonary blood flow to the periphery of the lungs.
groups can usually be treated as if they have tetralogy of Fallot. Their outlook is generally satisfactory, as for other patients with Fallot's tetralogy. However, there are others with pulmonary atresia and VSD whose
central pulmonary arteries are severely hypoplastic and whose pulmonary blood supply is via multiple aortopulmonary collaterals. An example of this extreme end of the spectrum is shown in Fig. 8, C.
8
Stark
The history of the next patient demonstrates some of the problems that we face during treatment of such patients. CASE 5. The patient had been cyanotic since birth, and the diagnosis of pulmonary atresia with VSD and multiple collaterals was made after catheterization in another hospital. At exploratory thoracotomy at the age of 3 years, the pulmonary arteries were considered to be too small for a shunt. She was later referred to us. The angiocardiogram showed hypoplastic central pulmonary arteries, only 3 mm at the bifurcations, with several major collaterals. We decided to place a right ventricle-pulmonary artery conduit. Her condition improved after this operation. Two years later, a biopsy specimen was taken from the lung, and the two major collaterals were ligated. At the end of the operation the pulmonary artery pressure was 50 mm Hg and the aortic pressure, 100 mm Hg. She was restudied at the age of 12 years. Although the appearance of the pulmonary arteries was grossly abnormal on angiocardiogram, in view of the histologic report and the catheterization data we decided to proceed with the intracardiac repair. The VSD was closed, the remaining collateral was ligated, the bifurcation was reconstructed, and an aortic homograft was placed between the right ventricle and the pulmonary artery. Right ventricular pressure after repair was 70 mm Hg, pulmonary artery pressure 50 mm Hg, and left ventricular pressure 85 mm Hg. After initial improvement, the child was readmitted in severe heart failure. At restudy biventricular failure with the right ventricular pressure at systemic level was found. Pulmonary arteriolar resistance was calculated to be 20 units. m', She was discharged and 2 months later died at home. This was a very sad end to a long saga starting first with very little hope, progressing through ever-increasing hope, and then, after what could be called a successful repair, ending in eventual deterioration and death.
Patients like this child represent a small proportion of patients with pulmonary atresia and VSD. Between 1979 and 1986 we operated on 173 patients with pulmonary atresia and VSD. Twenty-six of them were at this difficult end of the spectrum. In the past we" have treated such patients with a combination of several palliative procedures, including shunts, right ventricular patches or conduits, and anastomoses between collaterals and pulmonary arteries, to prepare them for eventual repair. In 1987 we" reviewed the results of our surgical efforts. Four of 26 patients undergoing complex palliation died. In 13 the anatomy and physiology at restudy were considered incompatible with correction. Only three had a corrective operation, and two of them died. Four others are considered candidates for correction and two are awaiting restudy. These results are disappointing and suggest that some patients with pulmonary atresia, VSD, and multiple collaterals should not be subjected to conventional palliation, but perhaps should be considered as candidates for heart-lung transplantation.
The Journal of Thoracic and Cardiovascular Surgery
After this review I realize that it is difficult to answer my original question: "Do we really correct congenital heart defects?" Using my initial criteria, I can say yes, we do correct, but not very often. However, should we use such strict criteria? I have looked up the definition of correction in the Oxford Dictionary: "To correct is to set right, to substitute right for wrong or to bring into accordance with a good standard." Translating this definition into our surgical practice, we could call an operation corrective if the patient is likely to be well and lead a normal life. Subsequent medication, or perhaps even reoperation, may be acceptable. On the other hand, if it is likely that the patient will be disabled or his life span much shortened, perhaps it would be appropriate to call such an operation palliative. As I have shown, there are situations in which the distinction between palliation and correction remains unclear. We must observe our patients during their entire life. The information thus obtained will confirm that some defects are truly corrected but that others are only palliated. If the results achieved by techniques currently available are clearly unsatisfactory, as I have demonstrated in the subgroup of patients with pulmonary atresia, hypoplastic pulmonary arteries, and multiple collaterals, we may decide not to operate on some patients at all. This will avoid considerable suffering and grief for the families of such children. I hope that further progress will follow in years to come. The major discoveries will probably not be in the surgical knitting and stitching but in finding the causative mechanisms of congenital heart defects. It may be that one day congenital heart defects will be preventable. We will then not have to worry whether we correct or palliate. We may all be out of a job by then. REFERENCES 1. Crafoord C, Nylin G. Congenital coarctation of the aorta and its surgical treatment. J THORAC SURG 1945;14:34761. 2. Clarkson PM, Nicholson MR, Barratt-Boyes, BG, Neutze JM, Whitlock RM. Results after repair of coarctation of the aorta beyond infancy: a 10 to 28 year follow-up with particular reference to late systemic hypertension. Am J CardioI1983;51:1481-8. 3. Stark J, de Leval M, Waterston DJ, Graham GR, Bonham-Carter RE. Corrective surgery for transposition of the great arteries in the first year of life. J THORAe CARDIOVASC SURG 1974;67:673-81. 4. Singh AK, Stark J, Tylor JFN. Left ventricle to pulmonary artery conduit in treatment of transposition of the great arteries, restrictive ventricular septal defect and acquired pulmonary atresia. Br Heart J 1976;38:1213-6. 5. Muster AJ, Berry TE, Ilbawi MN, et al. Development of neo-coarctation in patients with transposed great arteries
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and hypoplastic aortic arch after Lecompte modification of anatomical correction. J THORAC CARDIOVASC SURG 1987;93:276-80. Martin RP, Ladusans EJ, Parsons JM, Keck E, RadleySmith R, Yacoub MH. Incidence and site of pulmonary stenosisafter anatomical correction of transposition of the great arteries [Abstract]. Br Heart J 1988;59:122-3. Sidi D, Planche C, Kachaner J, et al. Anatomic correction of simple transposition of the great arteries in 50 neonates. Circulation 1987;75:429-35. Ross DN, Somerville J. Correction of pulmonary atresia with a homograft aortic valve. Lancet 1966;2:1446-7. Bull C, Macartney FJ, Horvath P, et al. Evaluation of long-term results of homograft and heterograft valves in extracardiac conduits. J THORAC CARDIOVASC SURG 1987; 94:12-9. Fontan F, Choussat A, Deville C, Doutremepuich C, Coupillaud J, Vosa C. Aortic valve homografts in the surgical treatment of complex cardiac malformations. J THORAC CARDIOVASC SURG 1984;87:649-57. Lindesmith GG, Stiles QR, Tucker BL, Gallaher ME,
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Stanton RE, Meyer BW. The Mustard operation as a palliative procedure. J THORAC CARDIOVASC SURG 1972; 63:75-80. Stark J, de Leval MR, Taylor JFN. Mustard operation and creation of ventricular septal defect in two patients with transposition of the great arteries, intact ventricular septum and pulmonary vascular disease. Am J Cardiol 1976;38:524-7. Szamicki R, Maurseth K, de Leval MR, Stark J. Tracheal compression by the aortic arch following right pneumonectomy in infancy. Ann Thorac Surg 1978; 25:231-5. Stark J, Huhta JC, Macartney FJ. Palliative surgery for pulmonary atresia with ventricular septal defect. In: Anderson RH, Macartney FJ, Shineboume EA, Tynan M, eds. Paediatric cardiology. Vol 5. London: Churchill Livingstone, 1983:126-36. Sullivan 10, Wren C, Stark J, de Leval MR, Macartney FJ, Deanfield JE. Surgical unifocalization in pulmonary atresia with ventricular septal defect. A realistic goal? Circulation [In press].