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Recent results of cardiovascular surgery in infants in the first year of life
Recent Results of Cardiovascular First Year of Life
Surgery in Infants in the
William F. Bernhard, MD, Boston, Massachusetts S. Bert Litwin, MD, Boston, Massachusetts Willis W. Williams, MD, Boston, Massachusetts Jimmy E. Jones, MD, Boston, Massachusetts Robert E. Gross, MD, Boston, Massachusetts
The natural history of congenital heart disease indicates a high mortality during the first year of life. Thirty-four per cent of such babies die within the first month after birth and 60 per cent during the first year [I]. Cardiovascular surgery in this age group is usually not performed on an elective basis. It does, however, offer the best, and often the only, chance for survival when there is congestive heart failure, hypoxemia, or respiratory distress secondary to cardiac anomalies [2-41. During a period of three decades, we have performed 1,003 cardiovascular operations on babies under twelve months of age. To summarize the current results of therapy, we present here only material of the last seven years, 1964 through 1970, during which 581 consecutive infants under one year of age were brought to surgery for various palliative or corrective procedures. Ages ranged from a few hours to one year; 62 per cent were under three months of age and 84 per cent were under six months. Many babies had multiple cardiac defects, and some required more than one surgical procedure. Each patient is listed only once, under the initial operative procedure. Except where indicated, all mortality figures represent hospital deaths. Principal diagnoses are listed in Table I. Transposition of the great arteries was the From the Department of Cardiovascular Surgery, Children’s Hospital, and the Department of Surgery of the Harvard Medical School, Boston, Massachusetts. This work was supported in part by Grant 260, Children’s Bureau, Maternal and Child Health Service, Department of Health, Education, and Welfare, Washington, DC: and grants from the National Heart and LUng InStitUte of the National Institutes of Health and the John A. Hartford Foundation, Inc. Reprint requests should be addressed to Dr Litwin, Department of Surgery. Children’s Hospital, Boston, Massachusetts 02115. Presented at the Fifty-Second Annual Meeting of the New England Surgical Society, Portsmouth, New Hampshire, September 30, October 1 and 2, 1971.
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most common lesion (22 per cent), followed by tetralogy of Fallot (16 per cent), patent ductus arteriosus (13 per cent), and isolated ventricular septal defect (13 per cent). Considerations of the various cardiovascular abnormalities are given briefly in the following sections. Transposition of the Great Arteries Transposition was the most common cardiac abnormality. A variety of hemodynamic problems were requiring different therapeutic apencountered, proaches. They included (1) inadequate mixing between the systemic and pulmonary circulations; (2) excessive flow to the lungs through a ventricular septal defect or patent ductus arteriosus; or (3) decreased pulmonary blood flow because of associated pulmonary stenosis. Severe hypoxemia secondary to inadequate mixing at the atria1 level occurred in eighty-four infants, and was treated by surgical septectomy alone or in combination with another palliative procedure. (Table II.) Fifty-one (61 per cent) of this group were operated upon within the first month of life. We now believe that the atria1 septum should be opened by balloon septostomy at the time of cardiac catheterization [5]; when this approach proves inadequate, surgical septectomy should be undertaken. Of sixty-one infants subjected to attempts at balloon septostomy, thirty-eight subsequently came to surgical septectomy (which are included in the eighty-four previously listed). Pulmonary artery hypertension from excessive flow (through an associated ventricular septal defect or patent ductus arteriosus) was the main consideration in twenty-two babies and was treated by pul-
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TABLE I
Cardiovascular Surgery in Infants in the First Year of Life
Diagnosis Transposition of the great arteries Tetralogy of Fallot Tricuspid atresia Ventricular septal defect Atrioventricularis communis Truncus arteriosus Patent ductus arteriosus, isolated Coarctation Pulmonary atresia and intact ventricular septum Pulmonic stenosis Aortic stenosis Hypoplastic left heart Vascular anomalies producing tracheal or esophagea! compression Total anomalous pulmonary venous drainage Miscellaneous Total
Number of Patients
Percentage of Total 22.6
131 96 20 76 11 9 78 30
16.5 3.4 13.1 1.9 1.5 13.4 5.2
30 15 13 11
5.2 2.6 2.2 1.9
25
4.3
16 20 581
2.8 3.4 100
monary artery banding (Table III); in addition, three of these had division of a patent ductus arteriosus. Two other babies had only a ductal division without pulmonary artery banding. A critical reduction in pulmonary blood flow with feature in severe cyanosis was the outstanding twenty-three patients; in twenty-one of these some form of a systemic-pulmonary artery shunt alone was established and for two pulmonic valvotomy was performed when the anatomy proved unsuitable for creation of a shunt. (Table IV.) Technical Considerations. For performing atria1 septectomy, operation was carried out while working in a compression chamber. Hyperbaria at a pressure of 3.0 atmospheres (absolute) was always employed TABLE II
Transposition of great arteries with intact ventricular septum Transposition of great arteries plus ventricular septal defect Transposition of great arteries plus pulmonary stenosis Miscellaneous (errors in diagnosis)
452
Tetralogy of Fallot or Pulmonary Atresia with Ventricular Septal Defect
In ninety-six babies with tetralogy of Fallot, there was a wide range of anatomic deformities with the es-
Transposition of the Great Arteries with Insufficient Mixing between Pulmonary and Systemic Circulations Diagnoses
Total
to increase the patient’s arterial oxygen tension sufficiently to minimize central nervous system hypoxic damage [6]. The procedure was performed through a right lateral thoracotomy in the fifth intercostal space, with temporary occlusion of the inferior and superior venae cavae and cross clamping of the ascending aorta and main pulmonary artery. Excision of the septum primum was accomplished through a right atriotomy, and all of the tissue overlying the right pulmonary veins was also removed. Results. Of eighty-four infants treated by septectomy alone, in combination with a shunt procedure, or in combination with pulmonary banding, sixty-two survived (74 per cent). One-third of the deaths resulted from associated cardiovascular malformations (coarctation, pulmonary stenosis, aortic stenosis, patent ductus arteriosus); one-third were due to complications which occurred preoperatively (septicemia, cardiac arrest, myocardial perforation); and one-third were related to technical problems of surgery. Of the twenty-four babies whose outstanding troubles were greatly increased pulmonary flow and who were treated by pulmonary banding alone, banding plus ductal closure, or ductal closure alone, nineteen survived (79 per cent). Of the twenty-three babies whose main problem was related to greatly diminished pulmonary blood flow, twenty-one were treated with some form of a shunt and two by pulmonary valvotomy. Survival was 70 per cent. Of the entire 135 patients with transposition, treated by some form of palliative surgery, 72 per cent survived.
Number of Patients
Operative
Procedure
Alive
61
Atrial septal defect creation alone
46 (77%)
17
Atrial septal defect creation and pulmonary banding Atrial septal defect creation and systemicpulmonary artery shunt Atrial septal defect creation and pulmonary artery banding
13 (76%)
4
2
a4
3
0
62 (74%)
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TABLE
III
Transposition
of the Great
Diagnoses Transposition of great arteries plus ventricular septal defect Transposition of great arteries, ventricular septal defect, and patent ductus arteriosus Transposition of great arteries and patent ductus arteriosus Total
Arteries
with Greatly
Number of Cases 19
3
2
Pulmonary
in Infants
Flow
Procedure
Alive
artery banding
16
Pulmonary artery banding plus division of patent ductus arteriosus Division of patent ductus arteriosus
2
Operative Pulmonary
1
24
sential pathology of right ventricular outflow obstruction (atresia in thirty-nine) in combination with a ventricular septal defect. Severe cyanosis was present because of greatly reduced pulmonary blood flow and also right to left shunting at the ventricular level. Infants with the more severe forms of this abnormality (pulmonary atresia) were in the early weeks of life. Others with the more typical forms of tetralogy were in the first months of life, but many cases were found later in the first year. Urgent surgical intervention was necessitated whenever there was profound continued hypoxemia and acidosis or whenever there were hypoxic spells. Progressively increasing, generalized cyanosis was a less urgent but definite indication for operation. Operations for infants with tetralogy of Fallot (or pulmonary atresia with ventricular septal defect) were palliative and designed to increase pulmonary blood flow. Procedures consisted primarily of establishing some kind of shunt between the aortic and pulmonary artery systems; pulmonic valvotomy was practical in only a few. Choice of a particular operative procedure must take into account which type of shunt is most likely to remain open, can give sufficient blood flow to the lungs, will not flood the lungs, and can be most easily closed at the time of subsequent total correction of the cardiac abnormality. In about 40 per cent of cases wherein some kind of shunt was being established, hyperbaric oxygenation (3 atmospheres absolute) was used to increase arterial oxygen tension and maintain the baby in the best possible state of oxygenation while the operative step was being performed. The subclavian artery to pulmonary artery (Blalock-Taussig) shunt is performed in the hemithorax ipsilateral to the innominate artery. If this is done, there is no kinking at the base of the subclavian artery when it is turned down in a caudad direction to permit end to side anastomosis with the pulmonary artery. The Blalock shunt is apt to carry a high failure rate in small infants, but for those beyond six months of
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Increased
Surgery
19 (79%)
age the shunt is quite satisfactory. It seldom causes lung flooding; it is not difficult to close off at later intracardiac repair. We much prefer to join the ascending aorta to the right pulmenary artery (Waterston shunt) [7,8] for infants under six months of age because of the diminutive vascular structures. Early shunt thrombosis has been rare. The anastomosis must be limited to 3 mm in diameter in the neonate and 4 mm in older babies. Adequacy of shunt flow can be estimated by detection of a soft thrill in the right pulmonary artery and by measurement of the arterial oxygen saturation while the patient is breathing 50 per cent oxygen. With creation of a satisfactory shunt, the saturation should rise to the range of 70 to 80 per cent. A value over 85 per cent indicates excessive pulmonary flow and demands immediate narrowing of the shunt by placement of sutures at the margins of the anastomosis. Although this operation necessitates opening the pericardium and possibly causing pericardial adhesions, its central location facilitates shunt closure at the time of total cardiac repair. The descending aorta to left pulmonary artery anastomosis (Potts shunt) has fallen into disuse because of the potential difficulties in closing it at the time of subsequent complete cardiac repair [9]. Shunts between the ascending aorta and left pulmonary artery or main pulmonary artery were utilized
TABLE
IV
Transposition of the Great Arteries Diminished Pulmonary Blood Flow
with
_
Operative
Procedure
Number of Patients
Alive ~_
Waterston shunt Blalock-Taussig shunt Ascending aorta-main pulmonary artery shunt Potts shunt Brock pulmonic valvotomy Total
11 5 4 1 2 23
6 4 3 0 1 16 (70%)
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in rare cases when there was very unusual anatomy (such as pulmonary atresia with dextrocardia and situs inversus totalis or severe tetralogy of Fallot with diminutive pulmonary artery branches).
Results. Three infants were treated by a Brock procedure, with very unsatisfactory results and only one survivor. There were six who were found to have extraordinary anatomic situations, and attempts to palliate these all ended in failure. Of eighty-seven for whom some shunting procedures were performed, there was over-all survival of 82 per cent. (Table V.) A second operation was necessary in two babies to narrow a Waterston shunt in the immediate postoperative period. For the total group of ninety-six patients, a second palliative shunt was required in the late follow-up period in fourteen (after an initial Waterston shunt in six and after a Blalock-Taussig shunt in eight others). Tricuspid Atresia Atresia of the tricuspid valve was found in twenty babies. The anomaly was always associated with a patent foramen ovale or an atria1 septal defect which allowed systemic venous blood to reach the left heart. In addition, a ventricular septal defect was usually present, but pulmonary blood flow was restricted due to hypoplasia of the right ventricle. Severe hypoxia often developed in these infants at the time of spontaneous ductal closure. The operation required in all was a procedure to increase the pulmonary blood flow. In older children with this anomaly we prefer to join the superior vena cava to the right pulmonary artery. However, in infants this has seldom been satisfactory and it is better to make an arterial anastomosis from the aortic system to the pulmonary artery; we prefer to accomplish this with a Waterston shunt. Results. In fifteen babies with a Waterston shunt, nine survived. In five with a Blalock union, four have survived.
Congestive heart failure and pulmonary artery hypertension (pulmonary artery pressure two-thirds or more than aortic pressure) were present in all babies. When possible, operation was delayed until a regimen of sodium restriction as well as digitalis and diuretic administration could be undertaken. Failure to thrive or to improve on this decongestive regimen prompted surgical intervention. Operations were performed through a left thoracotomy incision, employing a 6.0 mm wide cotton tape as the banding material. Since cotton stimulates local fibrosis, the problem of a band cutting through the vessel was extremely uncommon and caused no deaths. Serial intraoperative measurements of mean pulmonary artery pressure distal to the band were recorded to help decide the degree of constriction required. The vessel was narrowed until this pressure fell to approximately 50 per cent of the original level. Measurements of arterial oxygen saturation were valuable; a lowered saturation indicated a right to left intracardiac shunt from too severe a banding.
Results. Of the seventy-six patients with a ventricular septal defect (with or without an associated patent ductus arteriosus) seventy-one (93 per cent) are alive after this palliative step. This is particularly significant since totally corrective intracardiac surgery can be offered subsequently. Of the entire ninetysix patients we have put together into this group (consisting of three categories) eighty-three (86 per cent) have survived. (Table VI.) Patent Ductus Arteriosus Because of congestive heart failure from a large left to right ductal shunt, operation was undertaken in seventy-eight infants and the ductus closed off. Ductal division was carried out in most infants, but in a few critically ill neonates double ligation was performed to reduce the length of operative time. The results of this surgery were most gratifying, with seventy-five babies surviving (96 per cent). TABLE V
Tetralogy of Fallot (Including Pulmonary Atresia
Ventricular Septal Defect, Atrioventricularis Communis, and Truncus Arteriosus These three abnormalities are grouped together because of the common method of treating each of them (surgical banding of the pulmonary artery to diminish a very large pulmonary flow and bring under control cardiac failure). Not infrequently a patent ductus had to be closed surgically in conjunction with the pulmonary banding, a combination in twentyeight of ninety-six cases in this group.
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plus Ventricular
Septal Defect)
Number of Patients
Alive
Waterston shunt Blalock-Taussig shunt Potts shunt
51 30 3
41 (80%) 25 (83%) 2
Ascending aorta to main pulmonary or left pulmonary artery shunt Miscellaneous Total
3 9 96
2 1 71 (74%)
Operative
Procedure
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VI
isolated
Ventricular septal defect with or without patent ductus arteriosus Atrioventricularis canal with or without patent ductus arteriosus Truncus arteriosus with or without patent ductus arteriosus Total
Pulmonary Artery Banding Alone
Pulmonary Artery Banding plus Ductal Division
Alive
50
26
71 (93%)
9
2
8 (73%)
9 68
0 28
83 (86%)
of the Aorta
Although the vast majority of patients with coarctation of the aorta are best treated in later childhood when there are more suitable conditions for operation and there is a higher likelihood of attaining a larger anastomotic lumen, there are some babies with coarctation who are severely ill and cannot be maintained on medical management. They should be operated upon regardless of the high risk. Unfortunately, most such babies also have other intra- or extracardiac anomalies which greatly complicate the aortic obstruction problem and indeed might far overshadow it. We have found it desirable to classify the thirty infants brought to surgery into two somewhat different groups. Group I. This consisted of twenty-one babies, who had obstruction demonstrated at the aortic isthmus, but who, in about three-quarters of cases, also had other conditions such as patent ductus arteriosus, atria1 septal defect, ventricular septal defect, or considerable mitral valve deformity. In addition, twothirds of them had some degree of aortic arch hypoplasia. Included in this entire group of twenty-one, were eight babies who had preductal coarctation; a large ductus was in direct continuity with the descending aorta, which it supplied by a right to left flow. Looking at this group of twenty-one as a whole, we thought that the coarctation was the dominant lesion and there was a reasonable outlook for surgical attack on the coarctation. Group II. This consisted of nine desperately ill infants, each having a variety of other defects as hypoplastic left ventricle, aortic stenosis, and/or severe hypoplasia of the transverse arch. We recognized that in all of these children the coarctation was distinctly
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in Infants
Ventricular Septal Defect, Atrioventricular Canal, Truncus Arteriosus
Diagnosis
Coarctation
Surgery
4
the lesser part of the over-all picture, but decided to operate upon each hoping that correction of one part of a complex problem might save the baby. At operation, the attempt was always to remove the highly obstructed (or atretic) segment and bring remaining aortic ends together for a reasonable primary anastomosis [IO]. After aortic reconstruction, and in the known presence of ventricular septal defect, if the pulmonary artery pressure remained higher than two-thirds of the systemic pressure, the pulmonary artery was banded. Results. Of the twenty-one babies in group I, twelve had resection alone, and nine are alive. Eight had aortic resection plus pulmonary banding; four of these survived. Thus in group I, 62 per cent survived (four had preductal coarctations). Of the nine infants in group II, resection was accompanied by pulmonary artery banding in three. Of the group of nine, there was only one survivor. Some persisting mild aortic obstruction (or restenosis) is not uncommon. We have not yet had to perform secondary resections of residual obstructions although such may be necessary after longer periods of observation in some patients. Pulmonary
Atresia with Intact Ventricular
Septum
Thirty infants comprising this group had a combination of problems including right ventricular hypertension (systemic or suprasystemic pressures), hypoxemia (related to inadequate pulmonary blood flow), occasionally with tricuspid stenosis (with or without regurgitation), and rarely with obstruction at the atria1 septum. The right ventricular chamber was underdeveloped in all, varying in size from 50 per cent of normal to extreme hypoplasia. In twenty-
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eight infants, precipitous onset of symptoms occurred at the time of spontaneous ductal closure, and operative relief was necessary in the first few weeks of life; two babies were operated upon at several months of age. Choice of Operation. When the right ventricular chamber was of reasonable size (25 to 50 per cent of normal), wide excision of the atretic pulmonary valve established continuity between the small ventricle and the main pulmonary artery (working through a main pulmonary arteriotomy with cardiac inflowocclusion). This was carried out in fourteen infants; four survived and are alive one to six years later. Deaths occurred in most of these babies within a few days after operation and were often the result of inadequate relief of the right ventricular outflow tract obstruction. Militating against a successful result was unsuspected tricuspid stenosis in some. When the ventricular chamber was poorly developed (less than 25 per cent of normal size), initially any interatrial pressure gradient was eliminated by balloon septostomy to allow free passage of blood from the right to left side of the heart. Then a systemic-pulmonary artery shunt (ascending aorta to right pulmonary artery) was performed. This relieved hypoxia by increasing pulmonary blood flow, but did not affect right ventricular hypertension. Even though thirteen of sixteen babies survived a shunt operation, ten of them succumbed within three years after surgery. Most late deaths were related to shunt failure, but three patients died of intractable arrhythmias and congestive failure. Our current policy is to recatheterize these infants with shunts at four to six months of age and visualize the right ventricle. If the right ventricular body and outflow tract are now of suitable size, resection of the atretic valve can be carried out and simultaneously the existing Waterston shunt can be reduced in size, or even completely eliminated, as the circumstances require. Valvar Pulmonic Stenosis In infants with pulmonic stenosis and an intact ventricular septum, the right ventricular pressures were greater than two-thirds of systemic pressure in all. Severe cyanosis due to right to left shunting at the atria1 level was present in most, and congestive heart failure was seen in a few. In fifteen patients, valvotomy was performed either as a closed transventricular (Brock) procedure or under direct vision down through a main pulmonary arteriotomy. The latter approach is now preferred since the fibrous commissures of the valve may be carefully incised; the use of temporary cardiac inflow-occlusion provides a dry field.
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All of these infants with critical valvar pulmonic stenosis and an intact ventricular septum survived. Critical Aortic Stenosis Thirteen infants suffered from extreme degrees of valvular aortic stenosis. Ten were under three months of age and all were less than seven months at the time of operation. Additional congenital anomalies were present in half, including preductal coarctation, patent ductus arteriosus, endocardial fibroelastosis, mitral valve anomalies, and hypoplasia of the left ventricle. Acute left ventricular failure was present in all. For all thirteen babies, unresponsive to strict medical management, and in spite of a most forbidding outlook, operation was undertaken. Under normothermic hyperbaric conditions, open valvotomy was performed during temporary circulatory interruption (for about two minutes). The valve was exposed through a vertical aortotomy, and the tiny, irregular, verrucous-looking structures .incised at one side. Suprisingly, eleven of the thirteen survived; associated cardiovascular anomalies certainly contributed to the demise of two patients. Vascular Anomalies Producing Tracheal or Esophageal Compression Twenty-five babies had serious difficulties from vascular abnormalities which produced obstruction of either the trachea or esophagus, or both of these structures. Symptoms generally included stridor or respiratory distress or dysphagia, symptoms referable to disturbed respiratory airway far exceeding in number and severity those arising from a compressed esophagus. Diagnoses included: double aortic arch (twelve), right arch with a left ligamenturn arteriosum (six), an aberrant innominate artery pressing on the trachea (one), an aberrant subclavian artery (two), and a normally positioned aorta pushing on an abnormal trachea (trachea-malacia) (four). In all instances, the surgical approach was through a left arterolateral thoracotomy. In patients with a double aortic arch, the smaller arch (usually the anterior one) was divided. Whenever there was a right arch and left ligamenturn, the ligament was divided, as was the left subclavian artery. In both types of anomalies, even though a constricting ring had been broken by surgical section of some part of it, there generally continued to be some pressure of the aorta on the trachea. We therefore always pulled the aortic arch forward by placing three or four silk sutures through the adventitia of anterior vascular structures (anterior arch, or base of the in-
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nominate or carotid arteries), passing these sutures forward through the sternum (after removing most of the thymus), and then tying with appropriate tension to pull the vessels (and thus the anterior wall of the trachea) forward. In those cases in which an aberrant right subclavian artery was the sole abnormality, this vessel was doubly ligated and divided in the posterior mediastinum to relieve esophageal compression. All twenty-five infants survived. Most babies were markedly improved immediately after operation; in a few some symptoms persisted for a period of months.
Surgery in Infants
pulmonary artery with a tubular Teflon graft. Two infants with congenital heart block had pacemakers implanted successfully. One baby with a hypoplastic left heart plus a patent ductus arteriosus and two others with double outlet right ventricles survived palliative surgery. One baby underwent resection of a large intrapericardial teratoma and is well two years later. Extracorporeal Circulation and Cardiopulmonary Bypass
Extracorporeal circulation with cardiopulmonary bypass (in a standard nonpressurized operating room) 9 has been employed for twenty patients. Sixteen Aortic Arch Interruption or Aortic Atresia babies had total anomalous pulmonary venous drainage (four supracardiac, five intracardiac, and seven Four newborn infants with type B aortic arch intersubdiaphragmatic). There were six survivors (one ruption (total block between the left common carotid with supracardiac, three with cardiac, and two with artery and the left subclavian artery) were opersubdiaphragmatic drainage). The remaining four ated upon [II]. Since attempts at bridging the gap babies had various defects including incomplete endobetween segments have usually failed in patients cardial cushion defect (two), secundum atria1 septal in this age group [12], an artificial ductus (main puldefect (one), and aorticopulmonary fenestration monary artery to descending aorta) was created using (one); all of these survived. a 6 mm tubular Teflon@ prosthesis to allow perfusion Hypothermia (by surface cooling), followed by a of the lower torso. Simultaneously, the pulmonary arshort period of cold extracorporeal circulation, and tery was banded beyond the take-off of the shunt to elective circulatory arrest during the surgical proceprevent flooding of the lungs. Two infants survived, dure, provides a quiet and dry operative field. Some exand at twenty and forty months, respectively, are citing progress has been reported in which this methquite well. od was employed [11]. We have used it only on a few After initial success in infants with aortic arch inoccasions, but have been so impressed by its merits terruption, the same operation was performed withthat doubtless we will employ it more widely in the out success in seven newborn babies with aortic future. atresia (atresia of the aortic valve and marked hypoplasia of the ascending aorta and transverse arch, Comments plus a ventricular septal defect). We no longer beToday cardiovascular surgery in patients less than lieve that this surgical procedure is applicable to paone year of age can be carried out with a remarkable tients with aortic valve atresia. degree of success. Two large surgical series [4,14] have indicated over-all survival rates of 64 per cent Surgery for Unusual Problems and 72 per cent. In our own material there has been a surgical survival rate of 78 per cent. Improvements in To illustrate what can at times be accomplished in diagnostic methods, anesthetic management, postfacing what might seem to be most discouraging proboperative ventilatory support, and surgical technics lems, the following list briefly presents some isolated have all contributed to increasing success in recent but encouraging cases. years. Although totally corrective operative proFour infants had a left coronary artery arising from cedures have met with some good results recently in the main pulmonary artery; ligation of this vessel was these small patients, palliative surgery continues to successfully accomplished in all of them. Four babies be the more widely used approach for some babies had large anomalous systemic arteries feeding se[15]. It is well to point out that fully 90 per cent of questered lobes of lungs; such aberrant systemic these infants who have come through palliative therarteries were divided into two infants, and sequesapy have a good promise of having total repair in trated lung tissue was resected in two others; all surlater childhood years by technics which are now genvived. One child had an anomalous right pulmonary erally available. artery originating from the ascending aorta; the It is not possible here to elaborate upon detailed flooded lung was dramatically relieved by severing technics for carrying out the various procedures sumits artery from the aorta and attaching it to the main
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marized in this paper. However, a few remarks are in order, which have some value because of somewhat differing approaches to these matters on other surgical services. Hyperbaric conditions in the operating room have played a part in the management of babies requiring surgery for cardiovascular abnormalities. It has been our custom to perform such surgery with the entire surgical team and set-up in a hyperbaric chamber, but in only 40 per cent of the cases (severe hypoxemia or the use of circulatory interruption) was it necessary to proceed with pressurization. Whenever this was done, environmental pressure was raised to 3.0 atmospheres (absolute), with the patient breathing 100 per cent oxygen, and the surgical team breathing room air. There can be no doubt that the temporary raising of the arterial oxygen tension in the infant by such means gave a better subject to operate upon and contributed considerably to survival and avoidance of hypoxic brain damage.
Summary During a seven year period (1964 through 1970), 581 infants underwent palliative or corrective operations for congenital cardiovascular defects. Sixty-two per cent were under three months and 84 per cent under six months of age. Most babies had multiple cardiovascular anomalies and were limited by severe congestive heart failure and hypoxemia prior to operation; others had compression of the esophagus or trachea by an abnormal arterial vessel. Some had fair circulatory and pulmonary function, but failed to thrive. There was a wide variety of cardiovascular malformations, and indeed in many instances there were combinations of several defects. Although the group consisted of severely ill infants, who are rather forbidding subjects for surgery, 78 per cent of them nevertheless survived palliative corrective surgery.
References 1. MacMahon
B, McKeown T, Record RG: The incidence and life expectation of children with congenital heart disease. Brit Heart J 15: 121, 1953. 2. Coran AG, Bernhard WF: The surgical management of valvular aortic stenosis during infancy. J Thorac Cardiovasc Surg 58: 401) 1969. 3. Mustard WT, Bedard P, Trusler GA: Cardiovascular surgery in the first year of life. J Thorac Cardiovasc Surg 56: 761, 1970. 4. Stark J, Hucin B, Aberdeen E, Waterston DJ: Cardiac surgery in the first year of life: experience with 1,049 operations. Surgery 69: 463, 1971. 5. Rashkind WJ, Miller WW: Creation of an atrial septal defect without thoracotomy: a palliative approach to complete transposition of the great arteries. JAMA 196: 991, 1966.
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6. Bernhard WF, Norman J: Surgical treatment of cardiovascular defects in infants younger than one year of age, Chap 15, p 205. Cardiac Surgery. New York, AppletonCentury-Crofts, 1969. 7. Bernhard WF, Jones JE. Friedberg DZ, Litwin SB: Ascending aorta-right pulmonary shunt in infants and older children with certain types of cyanotic congenital heart disease. Circulation 43: 560, 1971. 8. Waterston DJ: The treatment of Fallot’s tetralogy in infants under the age of one year. Rozhl Chir 41: 161, 1962. 9. Gross RE, Bernhard WF. Litwin SB: Closure of Potts anastomosis in the total repair of tetralogy of Fallot. J. Thorac Cardiovasc Surg 57: 72, 1969. 10. Litwin SB, Bernhard WF, Rosenthal A, Gross RE: Surgical resection of coarctation of the aorta in infancy. J Pediat Surg 6: 307, 1971. 11. Litwin SB, Van Praagh R, Bernhard WF: A palliative operation for certain infants with aortic arch interruption. Ann Thorac Surg, in press. 12. Van Praagh R, Bernhard WF, Rosenthal A, Parisi LF, Fyler DC: Qterrupted aortic arch: surgical treatment. Amer J Cardiol27: 200, 1971. 13. Barrett-Boyes BG, Simpson M, Neutz JM: Intercardiac surgery in neonates and infants using deep hypothermia with surface cooling and limited cardiopulmonary bypass. Circulation 43: 1, 1971. 14. Hallman GL, Cooley DA: Cardiovascular suroerv in newborn infants: results in 1,050 patients less than one year old. Ann Surg 173: 1007, 1971. 15. Chung E. buShane JW, McGoon DC, Danielson GK: Total correction of cardiac anomalies using extracorporeal circulation. J Thorac Cardiovasc Surg 62: 117, 1971.
Discussion Horace C. Stansel (New Haven, Conn): A clinical series of this magnitude certainly qualifies Boston to be designated as the Houston of the East! This paper reflects a tremendous experience in the management of a most difficult group of lesions, that being congenital heart disease in the infant. This type of surgery requires the most exacting preoperative diagnostic tools, the most intensive postoperative management, and an excellence in surgical technic to achieve the results presented by Dr Litwin and his group. These are extremely difficult problems, many of which have to be faced two or more times before the infant has a reasonable lease on life. I agree with the authors’ approach to most of the lesions presented here today, but I should like to raise a couple of questions. We have had particular problems with infants with pulmonary atresia and an intact ventricular septum, especially in those with a diminutive right ventricular chamber. In our experience, simple pulmonary valvulotomy has not been sufficient to pull these children out of their immediate neonatal problems, apparently because the small volume of the right ventricular cavity does not permit sufficient pulmonary blood flow for survival, in spite of an adequate pulmonary valvulotomy. In these children, to achieve survival, we have also had to combine the pulmonary valvulotomy with a Waterston-type shunting procedure. I should like to ask the authors how often they have been able to achieve satisfactory results with only simple pulmonary valvulotomy in the management of this very difficult lesion.
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In reference to the Waterston shunt, the authors have described a rise in oxygen saturation to a level of 70 to 80 per cent as indicative of the desired degree of shunting achieved. A value of over 85 per cent saturation was interpreted by them as excessive pulmonary flow, and attempts were made to decrease the size of the shunt by placing sutures at that time. Our experience with this type of management has not been so rewarding, at least in the operating theater itself. We agree that if the child has very high oxygen saturation levels after a day or so, then certainly the possibility of excessive pulmonary blood flow exists, but our experience has frequently yielded very high saturation levels at the termination of a shunt, while the patient is under anesthesia with very high inspired oxygen levels and frequently a low cardiac output. This shunt, the technic of which must be carried out meticulously, can be easily made too large or too small, and our experience has shown that the best indication of the desirable size of the anastomosis has been by measuring the anastomosis to approximately a 4 mm length, as determined by the aortotomy. It is only with a shunt greater than this in terms of diameter that we have experienced excessive pulmonary blood flow. It is of interest that the authors carried out atria1 septectomy in two patients, presumably with normal relationship of the great vessels, because of incorrect preoperative diagnosis. This had sounded preposterous in this day of sophisticated radiographic technics or so I thought until recently when I performed this unique operation myself. However, I guess it now gives us the opportunity to follow the natural history of uncomplicated atria1 septal defects. We agree with the authors, however, that certainly balloon atria1 septostomy is the procedure of choice when it works, but unfortunately, it does not work too often. When it does, it certainly makes the subsequent Mustard operation simple when compared to surgical septectomy which creates dense adhesions between the pericardium and the heart. The excellent results that can be achieved by pulmonary artery banding in the infant with ventricular septal defect are borne out by the 93 per cent survival rate achieved by the authors. What material was used for banding? Until about six years ago, we routinely used a wide nylon tape, but experience has shown that this produces a marked deformity in the pulmonary artery, such that reconstruction of the pulmonary artery is necessary at the time of definitive repair. We now use simply a heavy braided silk suture, and have found that after removal of this suture, the constricted pulmonary artery will easily dilate to almost its normal configuration, eliminating an often difficult reconstructive procedure and a heavily scarred field. We certainly agree with the aggressive approach to the infant in failure with patent ductus arteriosus, and believe that surgical interruption of the ductus is indicated in the critically ill baby. Mortimer J. Buckley (Boston, Mass): I am greatly impressed with the results presented. Certainly, today we have
Volume
23, April
1972
Surgery in Infants
heard a review of an effort that has produced some of the most outstanding successes in the area of surgery for congenital heart disease in infants. There are two areas of special interest to me in this account. All of us have attempted pulmonary artery banding for ventricular septal defects and have to be greatly impressed by the very low mortality seen in this group of patients. Similarly, the results obtained for palliation of transposition of the great vessels are also excellent. The effort put into this is certainly great because of the many hours required to perform this surgery on an emergency basis. We would like to ask the authors if there is any place for a wider application of the concept of total correction in early life in these children. Certainly, with the mortality that has been achieved it is hard to imagine a better result. However, because of the progression of pulmonary vascular disease, particularly in the infants with transposition, we would appreciate the authors’ comments concerning total correction as opposed to palliative procedures. S. B. Litwin (closing): Infants with pulmonary atresia and an intact ventricular septum are difficult clinical problems. We have four of thirteen operated upon by valvotomy alone who are long-term survivors. We base our choice of operation on the right ventricular chamber size as seen on the preoperative angiogram. In practically all of these infants, the right ventricle will be less than 50 per cent normal in size. If it is roughly 25 per cent of normal or larger and if the outflow tract is at least moderately well developed, we carry out pulmonic valvotomy alone as the primary procedure. This increases pulmonary blood flow and allows decompression of the hypertensive right ventricle. Unfortunately, a number of these infants will have ventricular chambers smaller than 25 per cent of normal. In such cases, an ascending aorta to right pulmonary artery anastomosis is performed initially, after balloon septostomy has been carried out at the time of cardiac catheterization (in order to open the atria1 septum widely). These babies then return at four to six months of age for a repeat cardiac catheterization. If the ventricular chamber is at all suitable at this time, a second operation is performed. The pulmonary valve is opened and the systemic-pulmonary artery anastomosis is narrowed or closed appropriately. We have recently performed staged procedures in this fashion in two infants. As to the banding material, we use a 6 mm cotton tape. This does cause a moderate amount of scarring which requires a small pericardial patch in the wall of the main pulmonary artery in order to open that vessel properly at the time of total correction. On the other hand, this local fibrosis seems to prevent the band from eroding through the vessel wall after the banding operation. In our entire series of banded patients, we are aware of only two instances of this complication. Neither child had untoward effects from such. Contrary to this, there are reports in the literature of death occurring after erosion of the band into the pulmonary artery when Teflon tape was used at the initial operation. Doctor Buckley’s comments are certainly timely. Re-
459
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cently, there have been reports of total correction of various malformations in early life. I think this philosophy is particularly applicable to patients with transposition. We have, in recent months, become aware of the fact that even though a successful palliative operation is performed in infants with this malformation, many develop
460
pulmonary artery hypertension progressing to pulmonary vascular obstruction in early life. This militates against a successful corrective operation. Therefore I believe that, particularly in this group of infants, cardiac surgeons will have to perform totally corrective surgery in the first year of life.
The American Journal of Surgery
Surgery in Infants in the
William F. Bernhard, MD, Boston, Massachusetts S. Bert Litwin, MD, Boston, Massachusetts Willis W. Williams, MD, Boston, Massachusetts Jimmy E. Jones, MD, Boston, Massachusetts Robert E. Gross, MD, Boston, Massachusetts
The natural history of congenital heart disease indicates a high mortality during the first year of life. Thirty-four per cent of such babies die within the first month after birth and 60 per cent during the first year [I]. Cardiovascular surgery in this age group is usually not performed on an elective basis. It does, however, offer the best, and often the only, chance for survival when there is congestive heart failure, hypoxemia, or respiratory distress secondary to cardiac anomalies [2-41. During a period of three decades, we have performed 1,003 cardiovascular operations on babies under twelve months of age. To summarize the current results of therapy, we present here only material of the last seven years, 1964 through 1970, during which 581 consecutive infants under one year of age were brought to surgery for various palliative or corrective procedures. Ages ranged from a few hours to one year; 62 per cent were under three months of age and 84 per cent were under six months. Many babies had multiple cardiac defects, and some required more than one surgical procedure. Each patient is listed only once, under the initial operative procedure. Except where indicated, all mortality figures represent hospital deaths. Principal diagnoses are listed in Table I. Transposition of the great arteries was the From the Department of Cardiovascular Surgery, Children’s Hospital, and the Department of Surgery of the Harvard Medical School, Boston, Massachusetts. This work was supported in part by Grant 260, Children’s Bureau, Maternal and Child Health Service, Department of Health, Education, and Welfare, Washington, DC: and grants from the National Heart and LUng InStitUte of the National Institutes of Health and the John A. Hartford Foundation, Inc. Reprint requests should be addressed to Dr Litwin, Department of Surgery. Children’s Hospital, Boston, Massachusetts 02115. Presented at the Fifty-Second Annual Meeting of the New England Surgical Society, Portsmouth, New Hampshire, September 30, October 1 and 2, 1971.
Volume 123, April 1972
most common lesion (22 per cent), followed by tetralogy of Fallot (16 per cent), patent ductus arteriosus (13 per cent), and isolated ventricular septal defect (13 per cent). Considerations of the various cardiovascular abnormalities are given briefly in the following sections. Transposition of the Great Arteries Transposition was the most common cardiac abnormality. A variety of hemodynamic problems were requiring different therapeutic apencountered, proaches. They included (1) inadequate mixing between the systemic and pulmonary circulations; (2) excessive flow to the lungs through a ventricular septal defect or patent ductus arteriosus; or (3) decreased pulmonary blood flow because of associated pulmonary stenosis. Severe hypoxemia secondary to inadequate mixing at the atria1 level occurred in eighty-four infants, and was treated by surgical septectomy alone or in combination with another palliative procedure. (Table II.) Fifty-one (61 per cent) of this group were operated upon within the first month of life. We now believe that the atria1 septum should be opened by balloon septostomy at the time of cardiac catheterization [5]; when this approach proves inadequate, surgical septectomy should be undertaken. Of sixty-one infants subjected to attempts at balloon septostomy, thirty-eight subsequently came to surgical septectomy (which are included in the eighty-four previously listed). Pulmonary artery hypertension from excessive flow (through an associated ventricular septal defect or patent ductus arteriosus) was the main consideration in twenty-two babies and was treated by pul-
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TABLE I
Cardiovascular Surgery in Infants in the First Year of Life
Diagnosis Transposition of the great arteries Tetralogy of Fallot Tricuspid atresia Ventricular septal defect Atrioventricularis communis Truncus arteriosus Patent ductus arteriosus, isolated Coarctation Pulmonary atresia and intact ventricular septum Pulmonic stenosis Aortic stenosis Hypoplastic left heart Vascular anomalies producing tracheal or esophagea! compression Total anomalous pulmonary venous drainage Miscellaneous Total
Number of Patients
Percentage of Total 22.6
131 96 20 76 11 9 78 30
16.5 3.4 13.1 1.9 1.5 13.4 5.2
30 15 13 11
5.2 2.6 2.2 1.9
25
4.3
16 20 581
2.8 3.4 100
monary artery banding (Table III); in addition, three of these had division of a patent ductus arteriosus. Two other babies had only a ductal division without pulmonary artery banding. A critical reduction in pulmonary blood flow with feature in severe cyanosis was the outstanding twenty-three patients; in twenty-one of these some form of a systemic-pulmonary artery shunt alone was established and for two pulmonic valvotomy was performed when the anatomy proved unsuitable for creation of a shunt. (Table IV.) Technical Considerations. For performing atria1 septectomy, operation was carried out while working in a compression chamber. Hyperbaria at a pressure of 3.0 atmospheres (absolute) was always employed TABLE II
Transposition of great arteries with intact ventricular septum Transposition of great arteries plus ventricular septal defect Transposition of great arteries plus pulmonary stenosis Miscellaneous (errors in diagnosis)
452
Tetralogy of Fallot or Pulmonary Atresia with Ventricular Septal Defect
In ninety-six babies with tetralogy of Fallot, there was a wide range of anatomic deformities with the es-
Transposition of the Great Arteries with Insufficient Mixing between Pulmonary and Systemic Circulations Diagnoses
Total
to increase the patient’s arterial oxygen tension sufficiently to minimize central nervous system hypoxic damage [6]. The procedure was performed through a right lateral thoracotomy in the fifth intercostal space, with temporary occlusion of the inferior and superior venae cavae and cross clamping of the ascending aorta and main pulmonary artery. Excision of the septum primum was accomplished through a right atriotomy, and all of the tissue overlying the right pulmonary veins was also removed. Results. Of eighty-four infants treated by septectomy alone, in combination with a shunt procedure, or in combination with pulmonary banding, sixty-two survived (74 per cent). One-third of the deaths resulted from associated cardiovascular malformations (coarctation, pulmonary stenosis, aortic stenosis, patent ductus arteriosus); one-third were due to complications which occurred preoperatively (septicemia, cardiac arrest, myocardial perforation); and one-third were related to technical problems of surgery. Of the twenty-four babies whose outstanding troubles were greatly increased pulmonary flow and who were treated by pulmonary banding alone, banding plus ductal closure, or ductal closure alone, nineteen survived (79 per cent). Of the twenty-three babies whose main problem was related to greatly diminished pulmonary blood flow, twenty-one were treated with some form of a shunt and two by pulmonary valvotomy. Survival was 70 per cent. Of the entire 135 patients with transposition, treated by some form of palliative surgery, 72 per cent survived.
Number of Patients
Operative
Procedure
Alive
61
Atrial septal defect creation alone
46 (77%)
17
Atrial septal defect creation and pulmonary banding Atrial septal defect creation and systemicpulmonary artery shunt Atrial septal defect creation and pulmonary artery banding
13 (76%)
4
2
a4
3
0
62 (74%)
The American Journal of Surgery
Cardiovascular
TABLE
III
Transposition
of the Great
Diagnoses Transposition of great arteries plus ventricular septal defect Transposition of great arteries, ventricular septal defect, and patent ductus arteriosus Transposition of great arteries and patent ductus arteriosus Total
Arteries
with Greatly
Number of Cases 19
3
2
Pulmonary
in Infants
Flow
Procedure
Alive
artery banding
16
Pulmonary artery banding plus division of patent ductus arteriosus Division of patent ductus arteriosus
2
Operative Pulmonary
1
24
sential pathology of right ventricular outflow obstruction (atresia in thirty-nine) in combination with a ventricular septal defect. Severe cyanosis was present because of greatly reduced pulmonary blood flow and also right to left shunting at the ventricular level. Infants with the more severe forms of this abnormality (pulmonary atresia) were in the early weeks of life. Others with the more typical forms of tetralogy were in the first months of life, but many cases were found later in the first year. Urgent surgical intervention was necessitated whenever there was profound continued hypoxemia and acidosis or whenever there were hypoxic spells. Progressively increasing, generalized cyanosis was a less urgent but definite indication for operation. Operations for infants with tetralogy of Fallot (or pulmonary atresia with ventricular septal defect) were palliative and designed to increase pulmonary blood flow. Procedures consisted primarily of establishing some kind of shunt between the aortic and pulmonary artery systems; pulmonic valvotomy was practical in only a few. Choice of a particular operative procedure must take into account which type of shunt is most likely to remain open, can give sufficient blood flow to the lungs, will not flood the lungs, and can be most easily closed at the time of subsequent total correction of the cardiac abnormality. In about 40 per cent of cases wherein some kind of shunt was being established, hyperbaric oxygenation (3 atmospheres absolute) was used to increase arterial oxygen tension and maintain the baby in the best possible state of oxygenation while the operative step was being performed. The subclavian artery to pulmonary artery (Blalock-Taussig) shunt is performed in the hemithorax ipsilateral to the innominate artery. If this is done, there is no kinking at the base of the subclavian artery when it is turned down in a caudad direction to permit end to side anastomosis with the pulmonary artery. The Blalock shunt is apt to carry a high failure rate in small infants, but for those beyond six months of
Volume 123, April 1972
Increased
Surgery
19 (79%)
age the shunt is quite satisfactory. It seldom causes lung flooding; it is not difficult to close off at later intracardiac repair. We much prefer to join the ascending aorta to the right pulmenary artery (Waterston shunt) [7,8] for infants under six months of age because of the diminutive vascular structures. Early shunt thrombosis has been rare. The anastomosis must be limited to 3 mm in diameter in the neonate and 4 mm in older babies. Adequacy of shunt flow can be estimated by detection of a soft thrill in the right pulmonary artery and by measurement of the arterial oxygen saturation while the patient is breathing 50 per cent oxygen. With creation of a satisfactory shunt, the saturation should rise to the range of 70 to 80 per cent. A value over 85 per cent indicates excessive pulmonary flow and demands immediate narrowing of the shunt by placement of sutures at the margins of the anastomosis. Although this operation necessitates opening the pericardium and possibly causing pericardial adhesions, its central location facilitates shunt closure at the time of total cardiac repair. The descending aorta to left pulmonary artery anastomosis (Potts shunt) has fallen into disuse because of the potential difficulties in closing it at the time of subsequent complete cardiac repair [9]. Shunts between the ascending aorta and left pulmonary artery or main pulmonary artery were utilized
TABLE
IV
Transposition of the Great Arteries Diminished Pulmonary Blood Flow
with
_
Operative
Procedure
Number of Patients
Alive ~_
Waterston shunt Blalock-Taussig shunt Ascending aorta-main pulmonary artery shunt Potts shunt Brock pulmonic valvotomy Total
11 5 4 1 2 23
6 4 3 0 1 16 (70%)
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Bernhard et al
in rare cases when there was very unusual anatomy (such as pulmonary atresia with dextrocardia and situs inversus totalis or severe tetralogy of Fallot with diminutive pulmonary artery branches).
Results. Three infants were treated by a Brock procedure, with very unsatisfactory results and only one survivor. There were six who were found to have extraordinary anatomic situations, and attempts to palliate these all ended in failure. Of eighty-seven for whom some shunting procedures were performed, there was over-all survival of 82 per cent. (Table V.) A second operation was necessary in two babies to narrow a Waterston shunt in the immediate postoperative period. For the total group of ninety-six patients, a second palliative shunt was required in the late follow-up period in fourteen (after an initial Waterston shunt in six and after a Blalock-Taussig shunt in eight others). Tricuspid Atresia Atresia of the tricuspid valve was found in twenty babies. The anomaly was always associated with a patent foramen ovale or an atria1 septal defect which allowed systemic venous blood to reach the left heart. In addition, a ventricular septal defect was usually present, but pulmonary blood flow was restricted due to hypoplasia of the right ventricle. Severe hypoxia often developed in these infants at the time of spontaneous ductal closure. The operation required in all was a procedure to increase the pulmonary blood flow. In older children with this anomaly we prefer to join the superior vena cava to the right pulmonary artery. However, in infants this has seldom been satisfactory and it is better to make an arterial anastomosis from the aortic system to the pulmonary artery; we prefer to accomplish this with a Waterston shunt. Results. In fifteen babies with a Waterston shunt, nine survived. In five with a Blalock union, four have survived.
Congestive heart failure and pulmonary artery hypertension (pulmonary artery pressure two-thirds or more than aortic pressure) were present in all babies. When possible, operation was delayed until a regimen of sodium restriction as well as digitalis and diuretic administration could be undertaken. Failure to thrive or to improve on this decongestive regimen prompted surgical intervention. Operations were performed through a left thoracotomy incision, employing a 6.0 mm wide cotton tape as the banding material. Since cotton stimulates local fibrosis, the problem of a band cutting through the vessel was extremely uncommon and caused no deaths. Serial intraoperative measurements of mean pulmonary artery pressure distal to the band were recorded to help decide the degree of constriction required. The vessel was narrowed until this pressure fell to approximately 50 per cent of the original level. Measurements of arterial oxygen saturation were valuable; a lowered saturation indicated a right to left intracardiac shunt from too severe a banding.
Results. Of the seventy-six patients with a ventricular septal defect (with or without an associated patent ductus arteriosus) seventy-one (93 per cent) are alive after this palliative step. This is particularly significant since totally corrective intracardiac surgery can be offered subsequently. Of the entire ninetysix patients we have put together into this group (consisting of three categories) eighty-three (86 per cent) have survived. (Table VI.) Patent Ductus Arteriosus Because of congestive heart failure from a large left to right ductal shunt, operation was undertaken in seventy-eight infants and the ductus closed off. Ductal division was carried out in most infants, but in a few critically ill neonates double ligation was performed to reduce the length of operative time. The results of this surgery were most gratifying, with seventy-five babies surviving (96 per cent). TABLE V
Tetralogy of Fallot (Including Pulmonary Atresia
Ventricular Septal Defect, Atrioventricularis Communis, and Truncus Arteriosus These three abnormalities are grouped together because of the common method of treating each of them (surgical banding of the pulmonary artery to diminish a very large pulmonary flow and bring under control cardiac failure). Not infrequently a patent ductus had to be closed surgically in conjunction with the pulmonary banding, a combination in twentyeight of ninety-six cases in this group.
454
plus Ventricular
Septal Defect)
Number of Patients
Alive
Waterston shunt Blalock-Taussig shunt Potts shunt
51 30 3
41 (80%) 25 (83%) 2
Ascending aorta to main pulmonary or left pulmonary artery shunt Miscellaneous Total
3 9 96
2 1 71 (74%)
Operative
Procedure
The
American
Journal
of Surgery
Cardiovascular
TABLE
VI
isolated
Ventricular septal defect with or without patent ductus arteriosus Atrioventricularis canal with or without patent ductus arteriosus Truncus arteriosus with or without patent ductus arteriosus Total
Pulmonary Artery Banding Alone
Pulmonary Artery Banding plus Ductal Division
Alive
50
26
71 (93%)
9
2
8 (73%)
9 68
0 28
83 (86%)
of the Aorta
Although the vast majority of patients with coarctation of the aorta are best treated in later childhood when there are more suitable conditions for operation and there is a higher likelihood of attaining a larger anastomotic lumen, there are some babies with coarctation who are severely ill and cannot be maintained on medical management. They should be operated upon regardless of the high risk. Unfortunately, most such babies also have other intra- or extracardiac anomalies which greatly complicate the aortic obstruction problem and indeed might far overshadow it. We have found it desirable to classify the thirty infants brought to surgery into two somewhat different groups. Group I. This consisted of twenty-one babies, who had obstruction demonstrated at the aortic isthmus, but who, in about three-quarters of cases, also had other conditions such as patent ductus arteriosus, atria1 septal defect, ventricular septal defect, or considerable mitral valve deformity. In addition, twothirds of them had some degree of aortic arch hypoplasia. Included in this entire group of twenty-one, were eight babies who had preductal coarctation; a large ductus was in direct continuity with the descending aorta, which it supplied by a right to left flow. Looking at this group of twenty-one as a whole, we thought that the coarctation was the dominant lesion and there was a reasonable outlook for surgical attack on the coarctation. Group II. This consisted of nine desperately ill infants, each having a variety of other defects as hypoplastic left ventricle, aortic stenosis, and/or severe hypoplasia of the transverse arch. We recognized that in all of these children the coarctation was distinctly
Volume 123, April 1972
in Infants
Ventricular Septal Defect, Atrioventricular Canal, Truncus Arteriosus
Diagnosis
Coarctation
Surgery
4
the lesser part of the over-all picture, but decided to operate upon each hoping that correction of one part of a complex problem might save the baby. At operation, the attempt was always to remove the highly obstructed (or atretic) segment and bring remaining aortic ends together for a reasonable primary anastomosis [IO]. After aortic reconstruction, and in the known presence of ventricular septal defect, if the pulmonary artery pressure remained higher than two-thirds of the systemic pressure, the pulmonary artery was banded. Results. Of the twenty-one babies in group I, twelve had resection alone, and nine are alive. Eight had aortic resection plus pulmonary banding; four of these survived. Thus in group I, 62 per cent survived (four had preductal coarctations). Of the nine infants in group II, resection was accompanied by pulmonary artery banding in three. Of the group of nine, there was only one survivor. Some persisting mild aortic obstruction (or restenosis) is not uncommon. We have not yet had to perform secondary resections of residual obstructions although such may be necessary after longer periods of observation in some patients. Pulmonary
Atresia with Intact Ventricular
Septum
Thirty infants comprising this group had a combination of problems including right ventricular hypertension (systemic or suprasystemic pressures), hypoxemia (related to inadequate pulmonary blood flow), occasionally with tricuspid stenosis (with or without regurgitation), and rarely with obstruction at the atria1 septum. The right ventricular chamber was underdeveloped in all, varying in size from 50 per cent of normal to extreme hypoplasia. In twenty-
455
Bernhard et al
eight infants, precipitous onset of symptoms occurred at the time of spontaneous ductal closure, and operative relief was necessary in the first few weeks of life; two babies were operated upon at several months of age. Choice of Operation. When the right ventricular chamber was of reasonable size (25 to 50 per cent of normal), wide excision of the atretic pulmonary valve established continuity between the small ventricle and the main pulmonary artery (working through a main pulmonary arteriotomy with cardiac inflowocclusion). This was carried out in fourteen infants; four survived and are alive one to six years later. Deaths occurred in most of these babies within a few days after operation and were often the result of inadequate relief of the right ventricular outflow tract obstruction. Militating against a successful result was unsuspected tricuspid stenosis in some. When the ventricular chamber was poorly developed (less than 25 per cent of normal size), initially any interatrial pressure gradient was eliminated by balloon septostomy to allow free passage of blood from the right to left side of the heart. Then a systemic-pulmonary artery shunt (ascending aorta to right pulmonary artery) was performed. This relieved hypoxia by increasing pulmonary blood flow, but did not affect right ventricular hypertension. Even though thirteen of sixteen babies survived a shunt operation, ten of them succumbed within three years after surgery. Most late deaths were related to shunt failure, but three patients died of intractable arrhythmias and congestive failure. Our current policy is to recatheterize these infants with shunts at four to six months of age and visualize the right ventricle. If the right ventricular body and outflow tract are now of suitable size, resection of the atretic valve can be carried out and simultaneously the existing Waterston shunt can be reduced in size, or even completely eliminated, as the circumstances require. Valvar Pulmonic Stenosis In infants with pulmonic stenosis and an intact ventricular septum, the right ventricular pressures were greater than two-thirds of systemic pressure in all. Severe cyanosis due to right to left shunting at the atria1 level was present in most, and congestive heart failure was seen in a few. In fifteen patients, valvotomy was performed either as a closed transventricular (Brock) procedure or under direct vision down through a main pulmonary arteriotomy. The latter approach is now preferred since the fibrous commissures of the valve may be carefully incised; the use of temporary cardiac inflow-occlusion provides a dry field.
456
All of these infants with critical valvar pulmonic stenosis and an intact ventricular septum survived. Critical Aortic Stenosis Thirteen infants suffered from extreme degrees of valvular aortic stenosis. Ten were under three months of age and all were less than seven months at the time of operation. Additional congenital anomalies were present in half, including preductal coarctation, patent ductus arteriosus, endocardial fibroelastosis, mitral valve anomalies, and hypoplasia of the left ventricle. Acute left ventricular failure was present in all. For all thirteen babies, unresponsive to strict medical management, and in spite of a most forbidding outlook, operation was undertaken. Under normothermic hyperbaric conditions, open valvotomy was performed during temporary circulatory interruption (for about two minutes). The valve was exposed through a vertical aortotomy, and the tiny, irregular, verrucous-looking structures .incised at one side. Suprisingly, eleven of the thirteen survived; associated cardiovascular anomalies certainly contributed to the demise of two patients. Vascular Anomalies Producing Tracheal or Esophageal Compression Twenty-five babies had serious difficulties from vascular abnormalities which produced obstruction of either the trachea or esophagus, or both of these structures. Symptoms generally included stridor or respiratory distress or dysphagia, symptoms referable to disturbed respiratory airway far exceeding in number and severity those arising from a compressed esophagus. Diagnoses included: double aortic arch (twelve), right arch with a left ligamenturn arteriosum (six), an aberrant innominate artery pressing on the trachea (one), an aberrant subclavian artery (two), and a normally positioned aorta pushing on an abnormal trachea (trachea-malacia) (four). In all instances, the surgical approach was through a left arterolateral thoracotomy. In patients with a double aortic arch, the smaller arch (usually the anterior one) was divided. Whenever there was a right arch and left ligamenturn, the ligament was divided, as was the left subclavian artery. In both types of anomalies, even though a constricting ring had been broken by surgical section of some part of it, there generally continued to be some pressure of the aorta on the trachea. We therefore always pulled the aortic arch forward by placing three or four silk sutures through the adventitia of anterior vascular structures (anterior arch, or base of the in-
The American Journal of Surgery
Cardiovascular
nominate or carotid arteries), passing these sutures forward through the sternum (after removing most of the thymus), and then tying with appropriate tension to pull the vessels (and thus the anterior wall of the trachea) forward. In those cases in which an aberrant right subclavian artery was the sole abnormality, this vessel was doubly ligated and divided in the posterior mediastinum to relieve esophageal compression. All twenty-five infants survived. Most babies were markedly improved immediately after operation; in a few some symptoms persisted for a period of months.
Surgery in Infants
pulmonary artery with a tubular Teflon graft. Two infants with congenital heart block had pacemakers implanted successfully. One baby with a hypoplastic left heart plus a patent ductus arteriosus and two others with double outlet right ventricles survived palliative surgery. One baby underwent resection of a large intrapericardial teratoma and is well two years later. Extracorporeal Circulation and Cardiopulmonary Bypass
Extracorporeal circulation with cardiopulmonary bypass (in a standard nonpressurized operating room) 9 has been employed for twenty patients. Sixteen Aortic Arch Interruption or Aortic Atresia babies had total anomalous pulmonary venous drainage (four supracardiac, five intracardiac, and seven Four newborn infants with type B aortic arch intersubdiaphragmatic). There were six survivors (one ruption (total block between the left common carotid with supracardiac, three with cardiac, and two with artery and the left subclavian artery) were opersubdiaphragmatic drainage). The remaining four ated upon [II]. Since attempts at bridging the gap babies had various defects including incomplete endobetween segments have usually failed in patients cardial cushion defect (two), secundum atria1 septal in this age group [12], an artificial ductus (main puldefect (one), and aorticopulmonary fenestration monary artery to descending aorta) was created using (one); all of these survived. a 6 mm tubular Teflon@ prosthesis to allow perfusion Hypothermia (by surface cooling), followed by a of the lower torso. Simultaneously, the pulmonary arshort period of cold extracorporeal circulation, and tery was banded beyond the take-off of the shunt to elective circulatory arrest during the surgical proceprevent flooding of the lungs. Two infants survived, dure, provides a quiet and dry operative field. Some exand at twenty and forty months, respectively, are citing progress has been reported in which this methquite well. od was employed [11]. We have used it only on a few After initial success in infants with aortic arch inoccasions, but have been so impressed by its merits terruption, the same operation was performed withthat doubtless we will employ it more widely in the out success in seven newborn babies with aortic future. atresia (atresia of the aortic valve and marked hypoplasia of the ascending aorta and transverse arch, Comments plus a ventricular septal defect). We no longer beToday cardiovascular surgery in patients less than lieve that this surgical procedure is applicable to paone year of age can be carried out with a remarkable tients with aortic valve atresia. degree of success. Two large surgical series [4,14] have indicated over-all survival rates of 64 per cent Surgery for Unusual Problems and 72 per cent. In our own material there has been a surgical survival rate of 78 per cent. Improvements in To illustrate what can at times be accomplished in diagnostic methods, anesthetic management, postfacing what might seem to be most discouraging proboperative ventilatory support, and surgical technics lems, the following list briefly presents some isolated have all contributed to increasing success in recent but encouraging cases. years. Although totally corrective operative proFour infants had a left coronary artery arising from cedures have met with some good results recently in the main pulmonary artery; ligation of this vessel was these small patients, palliative surgery continues to successfully accomplished in all of them. Four babies be the more widely used approach for some babies had large anomalous systemic arteries feeding se[15]. It is well to point out that fully 90 per cent of questered lobes of lungs; such aberrant systemic these infants who have come through palliative therarteries were divided into two infants, and sequesapy have a good promise of having total repair in trated lung tissue was resected in two others; all surlater childhood years by technics which are now genvived. One child had an anomalous right pulmonary erally available. artery originating from the ascending aorta; the It is not possible here to elaborate upon detailed flooded lung was dramatically relieved by severing technics for carrying out the various procedures sumits artery from the aorta and attaching it to the main
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marized in this paper. However, a few remarks are in order, which have some value because of somewhat differing approaches to these matters on other surgical services. Hyperbaric conditions in the operating room have played a part in the management of babies requiring surgery for cardiovascular abnormalities. It has been our custom to perform such surgery with the entire surgical team and set-up in a hyperbaric chamber, but in only 40 per cent of the cases (severe hypoxemia or the use of circulatory interruption) was it necessary to proceed with pressurization. Whenever this was done, environmental pressure was raised to 3.0 atmospheres (absolute), with the patient breathing 100 per cent oxygen, and the surgical team breathing room air. There can be no doubt that the temporary raising of the arterial oxygen tension in the infant by such means gave a better subject to operate upon and contributed considerably to survival and avoidance of hypoxic brain damage.
Summary During a seven year period (1964 through 1970), 581 infants underwent palliative or corrective operations for congenital cardiovascular defects. Sixty-two per cent were under three months and 84 per cent under six months of age. Most babies had multiple cardiovascular anomalies and were limited by severe congestive heart failure and hypoxemia prior to operation; others had compression of the esophagus or trachea by an abnormal arterial vessel. Some had fair circulatory and pulmonary function, but failed to thrive. There was a wide variety of cardiovascular malformations, and indeed in many instances there were combinations of several defects. Although the group consisted of severely ill infants, who are rather forbidding subjects for surgery, 78 per cent of them nevertheless survived palliative corrective surgery.
References 1. MacMahon
B, McKeown T, Record RG: The incidence and life expectation of children with congenital heart disease. Brit Heart J 15: 121, 1953. 2. Coran AG, Bernhard WF: The surgical management of valvular aortic stenosis during infancy. J Thorac Cardiovasc Surg 58: 401) 1969. 3. Mustard WT, Bedard P, Trusler GA: Cardiovascular surgery in the first year of life. J Thorac Cardiovasc Surg 56: 761, 1970. 4. Stark J, Hucin B, Aberdeen E, Waterston DJ: Cardiac surgery in the first year of life: experience with 1,049 operations. Surgery 69: 463, 1971. 5. Rashkind WJ, Miller WW: Creation of an atrial septal defect without thoracotomy: a palliative approach to complete transposition of the great arteries. JAMA 196: 991, 1966.
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6. Bernhard WF, Norman J: Surgical treatment of cardiovascular defects in infants younger than one year of age, Chap 15, p 205. Cardiac Surgery. New York, AppletonCentury-Crofts, 1969. 7. Bernhard WF, Jones JE. Friedberg DZ, Litwin SB: Ascending aorta-right pulmonary shunt in infants and older children with certain types of cyanotic congenital heart disease. Circulation 43: 560, 1971. 8. Waterston DJ: The treatment of Fallot’s tetralogy in infants under the age of one year. Rozhl Chir 41: 161, 1962. 9. Gross RE, Bernhard WF. Litwin SB: Closure of Potts anastomosis in the total repair of tetralogy of Fallot. J. Thorac Cardiovasc Surg 57: 72, 1969. 10. Litwin SB, Bernhard WF, Rosenthal A, Gross RE: Surgical resection of coarctation of the aorta in infancy. J Pediat Surg 6: 307, 1971. 11. Litwin SB, Van Praagh R, Bernhard WF: A palliative operation for certain infants with aortic arch interruption. Ann Thorac Surg, in press. 12. Van Praagh R, Bernhard WF, Rosenthal A, Parisi LF, Fyler DC: Qterrupted aortic arch: surgical treatment. Amer J Cardiol27: 200, 1971. 13. Barrett-Boyes BG, Simpson M, Neutz JM: Intercardiac surgery in neonates and infants using deep hypothermia with surface cooling and limited cardiopulmonary bypass. Circulation 43: 1, 1971. 14. Hallman GL, Cooley DA: Cardiovascular suroerv in newborn infants: results in 1,050 patients less than one year old. Ann Surg 173: 1007, 1971. 15. Chung E. buShane JW, McGoon DC, Danielson GK: Total correction of cardiac anomalies using extracorporeal circulation. J Thorac Cardiovasc Surg 62: 117, 1971.
Discussion Horace C. Stansel (New Haven, Conn): A clinical series of this magnitude certainly qualifies Boston to be designated as the Houston of the East! This paper reflects a tremendous experience in the management of a most difficult group of lesions, that being congenital heart disease in the infant. This type of surgery requires the most exacting preoperative diagnostic tools, the most intensive postoperative management, and an excellence in surgical technic to achieve the results presented by Dr Litwin and his group. These are extremely difficult problems, many of which have to be faced two or more times before the infant has a reasonable lease on life. I agree with the authors’ approach to most of the lesions presented here today, but I should like to raise a couple of questions. We have had particular problems with infants with pulmonary atresia and an intact ventricular septum, especially in those with a diminutive right ventricular chamber. In our experience, simple pulmonary valvulotomy has not been sufficient to pull these children out of their immediate neonatal problems, apparently because the small volume of the right ventricular cavity does not permit sufficient pulmonary blood flow for survival, in spite of an adequate pulmonary valvulotomy. In these children, to achieve survival, we have also had to combine the pulmonary valvulotomy with a Waterston-type shunting procedure. I should like to ask the authors how often they have been able to achieve satisfactory results with only simple pulmonary valvulotomy in the management of this very difficult lesion.
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In reference to the Waterston shunt, the authors have described a rise in oxygen saturation to a level of 70 to 80 per cent as indicative of the desired degree of shunting achieved. A value of over 85 per cent saturation was interpreted by them as excessive pulmonary flow, and attempts were made to decrease the size of the shunt by placing sutures at that time. Our experience with this type of management has not been so rewarding, at least in the operating theater itself. We agree that if the child has very high oxygen saturation levels after a day or so, then certainly the possibility of excessive pulmonary blood flow exists, but our experience has frequently yielded very high saturation levels at the termination of a shunt, while the patient is under anesthesia with very high inspired oxygen levels and frequently a low cardiac output. This shunt, the technic of which must be carried out meticulously, can be easily made too large or too small, and our experience has shown that the best indication of the desirable size of the anastomosis has been by measuring the anastomosis to approximately a 4 mm length, as determined by the aortotomy. It is only with a shunt greater than this in terms of diameter that we have experienced excessive pulmonary blood flow. It is of interest that the authors carried out atria1 septectomy in two patients, presumably with normal relationship of the great vessels, because of incorrect preoperative diagnosis. This had sounded preposterous in this day of sophisticated radiographic technics or so I thought until recently when I performed this unique operation myself. However, I guess it now gives us the opportunity to follow the natural history of uncomplicated atria1 septal defects. We agree with the authors, however, that certainly balloon atria1 septostomy is the procedure of choice when it works, but unfortunately, it does not work too often. When it does, it certainly makes the subsequent Mustard operation simple when compared to surgical septectomy which creates dense adhesions between the pericardium and the heart. The excellent results that can be achieved by pulmonary artery banding in the infant with ventricular septal defect are borne out by the 93 per cent survival rate achieved by the authors. What material was used for banding? Until about six years ago, we routinely used a wide nylon tape, but experience has shown that this produces a marked deformity in the pulmonary artery, such that reconstruction of the pulmonary artery is necessary at the time of definitive repair. We now use simply a heavy braided silk suture, and have found that after removal of this suture, the constricted pulmonary artery will easily dilate to almost its normal configuration, eliminating an often difficult reconstructive procedure and a heavily scarred field. We certainly agree with the aggressive approach to the infant in failure with patent ductus arteriosus, and believe that surgical interruption of the ductus is indicated in the critically ill baby. Mortimer J. Buckley (Boston, Mass): I am greatly impressed with the results presented. Certainly, today we have
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heard a review of an effort that has produced some of the most outstanding successes in the area of surgery for congenital heart disease in infants. There are two areas of special interest to me in this account. All of us have attempted pulmonary artery banding for ventricular septal defects and have to be greatly impressed by the very low mortality seen in this group of patients. Similarly, the results obtained for palliation of transposition of the great vessels are also excellent. The effort put into this is certainly great because of the many hours required to perform this surgery on an emergency basis. We would like to ask the authors if there is any place for a wider application of the concept of total correction in early life in these children. Certainly, with the mortality that has been achieved it is hard to imagine a better result. However, because of the progression of pulmonary vascular disease, particularly in the infants with transposition, we would appreciate the authors’ comments concerning total correction as opposed to palliative procedures. S. B. Litwin (closing): Infants with pulmonary atresia and an intact ventricular septum are difficult clinical problems. We have four of thirteen operated upon by valvotomy alone who are long-term survivors. We base our choice of operation on the right ventricular chamber size as seen on the preoperative angiogram. In practically all of these infants, the right ventricle will be less than 50 per cent normal in size. If it is roughly 25 per cent of normal or larger and if the outflow tract is at least moderately well developed, we carry out pulmonic valvotomy alone as the primary procedure. This increases pulmonary blood flow and allows decompression of the hypertensive right ventricle. Unfortunately, a number of these infants will have ventricular chambers smaller than 25 per cent of normal. In such cases, an ascending aorta to right pulmonary artery anastomosis is performed initially, after balloon septostomy has been carried out at the time of cardiac catheterization (in order to open the atria1 septum widely). These babies then return at four to six months of age for a repeat cardiac catheterization. If the ventricular chamber is at all suitable at this time, a second operation is performed. The pulmonary valve is opened and the systemic-pulmonary artery anastomosis is narrowed or closed appropriately. We have recently performed staged procedures in this fashion in two infants. As to the banding material, we use a 6 mm cotton tape. This does cause a moderate amount of scarring which requires a small pericardial patch in the wall of the main pulmonary artery in order to open that vessel properly at the time of total correction. On the other hand, this local fibrosis seems to prevent the band from eroding through the vessel wall after the banding operation. In our entire series of banded patients, we are aware of only two instances of this complication. Neither child had untoward effects from such. Contrary to this, there are reports in the literature of death occurring after erosion of the band into the pulmonary artery when Teflon tape was used at the initial operation. Doctor Buckley’s comments are certainly timely. Re-
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cently, there have been reports of total correction of various malformations in early life. I think this philosophy is particularly applicable to patients with transposition. We have, in recent months, become aware of the fact that even though a successful palliative operation is performed in infants with this malformation, many develop
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pulmonary artery hypertension progressing to pulmonary vascular obstruction in early life. This militates against a successful corrective operation. Therefore I believe that, particularly in this group of infants, cardiac surgeons will have to perform totally corrective surgery in the first year of life.
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