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The Surgical Treatment of Congenital Heart Disease Accompanied by Cyanosis
Symposium on Pediatric Surgery FROM THE JOHNS HOPKINS HOSPITAL, BALTIMORE, MARYLAND
The Surgical Treatment of Congenital Heart Disease Accompanied by Cyanosis DAVID C. SABISTON, JR., M.D.* ALFRED BLALOCK, M.D., F.A.C.S., F.R.C.S. (HON.)t
IN ORDER to understand the recent developments in the treatment of patients with congenital heart disease, it is essential to be familiar with the normal and abnormal structure and function of the circulatory system. Although the normal structure of the heart and vessels was known to Galen in 179 A.D., it was not until 1628 that William Harvey recognized the course of the blood, through the arteries and its return by veins. Physiologists of the nineteenth century demonstrated the function of the lungs in oxygenating the venous blood and returning it to the left auricle for circulation through the systemic vessels. A consideration of the fetal circulation is essential for the understanding of congenital circulatory defects. Prior to birth the infant derives From the Department of Surgery, The Johns Hopkins Hospital, and The Johns Hopkins University School of Medicine, Baltimore, Maryland.
* Assistant in Surgery, Johns Hopkins University School of Medicine; Assistant Resident in Surgery, Johns Hopkins Hospital.
t Professor of Surgery and Director, Department of Surgery, The Johns Hopkins University School of Medicine; Surgeon-in-Chief, The Johns Hopkins Hospital. 1273
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its oxygen from the maternal circulation through the placenta. The lungs are collapsed. Oxygenated blood is conducted through the umbilical vein to the right auricle of the fetus where a portion enters the right
Key
Venous blood Arleri ..l blood Mixed blood Venous ~!, ,.n--I"-:
!~~~re:; (Closes at birth)
Duclus
OXYGlenaled blood
from mother vIa _ _- - ; c
umbtUcal vein
Fig. 391. Diagram of fetal circulation. Normal circulation is established with closure of the foramen ovale and ductus arteriosus.
ventricle to be propelled into the pulmonary circulation, and the remainder is shunted through the interauricular communication (patent foramen ovale) into the left auricle and thence into the systemic circulation. In addition the systemic circulation also receives blood shunted directly from the pulmonary artery, where the pressure is high (due to the high resistance offered by the collapsed lungs) through the ductus arteriosus into the aorta (Fig. 391).1
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However, with birth or shortly thereafter, two adjustments occur. Both the foramen ovale and ductus arteriosus normally close. This completely separates the pulmonary and systemic circulations and the normal postnatal circulatory plan is established. In most instances the etiology of congenital cardiac lesions is not known. Certain viral infections occurring in the mother in the early months of pregnancy are now recognized etiologic factors. The majority of these defects do occur relatively early in embryonic life. The persistent patent ductus arteriosus is an exception since it is a developmental error which occurs late. The malformations show great variation, and many are incompatible with extra-uterine life. Congenital heart disease has been divided into two groups, "cyanotic" and "noncyanotic." Cyanosis (kuavos, "dark blue") is a result of an abnormally high amount of unoxygenated blood as observed in the superficial vessels of the integument. This phenomenon does not appear until at least 5 gm. of unoxygenated hemoglobin are present per 100 cc. of blood. As a result of their low oxygen saturation, patients with cyanotic heart disease are usually markedly retarded in development and activity. Many of them are able to take only a few steps before the onset of fatigue and dyspnea. In an attempt to compensate for this the body produces an excessive number of erythrocytes. This number is usually about 8,000,000, but the count can be considerably higher. Another associated though poorly explained phenomenon is the high incidence of "clubbed" digits in these patients. There are numerous anatomical defects which may occur in congenital cyanotic heart disease. In most instances the pulmonary artery arises from the right ventricle in an abnormal manner. In addition, there are various associated intracardiac defects. These malformations may be of such severity as to allow only a small portion of the venous blood to enter the pulmonary system, much of the blood being shunted directly to the systemic system. TETRALOGY OF FALLOT AND ASSOCIATED DISORDERS
The tetralogy of Fallot is the most common congenital cardiac anomaly in the cyanotic group. There are a number of variations which may occur in this condition. As originally described in 1888 by Fallot2 the essential features are pulmonary stenosis, overriding of the aorta, high interventricular septal defect,· and hypertrophy of the right ventricle (Fig. 392). This group may be subdivided into those with (a) infundibular stenosis, (b) pulmonary atresia, (c) infundibular and valvular stenosis, (d) valvular stenosis with an interventricular septal defect, and (e) complex variations including nonfunctioning right ventricle, single ventricle with pulmonic stenosis, and tricuspid atresia.
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Anatomy 1. Infundibular Stenosis. This type of pulmonary stenosis is the most common in the tetralogy of Fallot. It is found in more than half the
Fig. 392. Diagram of the tetralogy of Fallot demonstrating infundibular pulmonic stenosis, an interventricular septal defect, an overriding aorta, and hypertrophy of the right ventricle.
instances. Here the stenosis is in the conus arteriosus, proximal to the pulmonary valve. It is produced by a prominent muscular band, the crista supraventricularis, which extends from the anteromedial wall of the right ventricle to the interventricular septum. The relative position and size of this muscular structure determine the nature of the chamber
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present between it and the pulmonary valve ring. Thus, when the stenosis is high, there is a small chamber, when intermediate, a medium chamber, and when low, a large one. Each of these occurs with approximately the same incidence. 3 2. Pulmonary Atresia. There may be an absence of communication of the right ventricle with the pulmonary artery, an atresia. This occurs in less than one-fifth of the instances of tetralogy of Fallot. The atresia is usually at the most proximal portion of the conus arteriosus, and it is !J.lmost always associated with a diminutive pulmonary artery. Further, the aorta is frequently far to the right and considerably enlarged. 3. Infundibular and Valvular Stenosis. Stenosis o"ccurring at both the conus and valvular levels is encountered less frequently. The infundibular chamber is usually large, and often the aorta overrides far to the right. 4. Valvular Stenosis with an Interventricular Septal Defect. Valvular pulmonic stenosis in association with an interventricular septal defect is less common than the types previously described. The valvular anatomy here is similar to that in isolated valvular stenosis. However, the pulmonary artery is usually normal in size or slightly dilated. 5. Complex Variations. There are several additional anomalies more complex in nature which can possibly be considered variations of this group. The entity of a nonfunctioning right ventricle is occasionally encountered, and is almost always associated with anomalies of tllfJ . . tricuspid valve and pulmonary artery. The tricuspid valve may be markedly hypoplastic and the right ventricle so diminutive as to be virtually nonfunctional. There is usually pulmonary atresia or marked stenosis with a small artery. The single ventricle with pulmonic stenosis is a further variation. Here the auricles empty into a single ventricle with a stenotic pulmonary artery emerging either from a rudimentary ventricular chamber or from the common chamber. In tricuspid atresia there is defective development of the right ventricle. The primary lesion is atresia of the tricuspid valve, and the blood must pass through a patent foramen ovale or an interauricular septal defect to the left auricle into the left ventricle. To enter the lung the blood must pass through a ventricular septal defect to the diminutive right ventricle or it may course into the aorta and reach the lungs by a patent ductus arteriosus or the bronchial arteries. ,~
Diagnosis
The diagnosis of the tetralogy of Fallot and its variations is given in detail by Taussig and others.4 The important symptoms in the classic type are diminished exercise tolerance, squatting, and retarded growth. Cerebrovascular accidents are not uncommon. The positive physical
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findings include cyanosis, a small heart, a systolic murmur in the pulmonary area and along the left sternal border, a loud aortic second sound and a weak pulmonary second sound, and clubbing of the digits. Examination of the blood reveals polycythemia (usually between 6 and 9 million), a high hematocrit (60 to 90), and the platelets are sometimes diminished. The arterial oxygen saturation is low, being 65 to 75 per cent in most instances. However, in severe examples it may be below 25 per cent. The electrocardiogram nearly always shows a right axis deviation. The P waves may be high and pointed. In the usual tetralogy the radiographic findings include a concavity in the region of the pulmo-
A
B
Fig. 393. A, Roentgenogram of patient with tetralogy of Fallot. Note concavity in region of pulmonary conus. B, Roentgenogram of same patient following operation.
nary conus, diminished size and pulsation of the pulmonary arteries, and a clear pulmonary window (Fig. 393). Since the aorta descends on the right in 20 to 25 per cent of these patients, an esophagogram should always be done to ascertain this point before operation. Special laboratory procedures such as angiocardiography and cardiac catheterization give invaluable aid in certain patients. The angiocardiogram in the tetralogy demonstrates the overriding aorta and gives an indication of the degree of pulmonary stenosis. However, it is to be stressed that angiocardiography is not without hazard in the cyanotic patient with reduced cardiac reserve. In the Johns Hopkins Hospital over 300 angiocardiograms have been performed in patients with congenital heart disease with cyanosis. There have been two deaths definitely attributable to the procedure. Two additional deaths may possibly have
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been related to the angiocardiography. There have been no deaths in the past two years.6 The figures obtained by cardiac catheterization are sometimes of invaluable aid in attaining the correct diagnosis. Bing and associates found that in patients with pulmonic stenosis (confirmed at operation) 80 per cent show with exercise a fall in oxygen consumption per liter of ventilation. 6 In the classic tetralogy of Fallot, tricuspid atresia, and single ventricle with pulmonic stenosis, the pulmonary flow is less than the systemic flow, and this is accompanied by a reduced pulmonary artery pressure. A high right ventricular pressure is present in the usual tetralogy of Fallot. The typical tetralogy of Fallot presents a few difficulties in diagnosis. Patients with pulmonary atresia usually give a history of extreme limitation of activity dating from early infancy. A systolic murmur is barely audible or absent. Angiocardiography and cardiac catheterization are seldom of diagnostic aid. In tricuspid atresia left axis deviation on the electrocardiogram is nearly always found. Also, fluoroscopy shows that the heart does not project as far anteriorly toward the retrosternal space, but it projects posteriorly toward the spine (hypoplastic right ventricle). Treatment
The first attempt to treat pulmonic stenosis by operative means was by Doyen in 1911.7 He attempted to divide the valve with a tenotome but was unsuccessful. In 1944, following extensive experimental studies in animals dating some years previously,S the first successful systemicpulmonary anastomosis was performed. 9 This operation consists of an anastomosis between a branch of the aortic arch (subclavian, carotid or innominate) to one of the pulmonary arter.ies. The procedure most frequently performed in this clinic is a subclavian-pulmonary artery anastomosis (end-to-side). Since the first procedure in November 1944, over 1150 such operations have been performed in the Johns Hopkins Hospital. Potts described a special clamp which permits partial occlusion of the aorta, and by its use an anastomosis may be performed between the side of the aorta and the side of the pulmonary artery. The first such procedure was performed by him in 1946.1° He prefers this method for cases in which the aortic arch descends on the left. Brock and others have felt that a direct attack on the stenosis, that is, an infundibular resection, is the operative procedure of choice in many instances. This subject is receiving considerable attention, and the Brock technic is being applied in selected cases. However, at present the shunt operations, either subclavian-pulmonary or aortico-pulmonary, appear to be preferred.
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There are several possible choices in the anastomotic procedures. In this clinic anastomoses between the following vessels have been employed: (a) the proximal end of the right or left subclavian artery and the side of the right or left pulmonary artery; (b) the proximal end of the right or left subclavian artery and the end of the right or left pulmonary artery; (c) the proximal end of the carotid or innominate artery and the side or distal end of the right or left pulmonary artery; and (d)
Fig. 394. Demonstration of the dissection of the innominate artery and its branches. The position of the pulmonary artery is shown (right).
the side of the aorta and the side of one of the pulmonary arteries. The anastomosis of choice is determined by the age of the patient, the descent of the aorta, and by the local anatomical configuration found at operation. The great majority of our patients have had an anastomosis between the end of the subclavian branch of the innominate artery and the side of the corresponding pulmonary artery. This is our procedure of choice for the usual patient, and it is illustrated in Figures 394 through 397. The alteration in the circulation following creation of an artificial ductus arteriosus is demonstrated in Figure 398. In infants (under the
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age of 2) the incision is usually made on the side on which the aorta descends. Thus, if the subclavian artery is not of suitable size, a Potts'
Fig. 395. The pulmonary artery is occluded and an incision in it has been made. The subclavian artery (center) has been divided and turned down to be anastomosed to the pulmonary artery (right).
Fig. 396. The anastomosis is completed. The occluding devices have been removed (right).
procedure may be employed. This procedure may be quite difficult in cases with a right aortic arch. It is in the extremely ill infant we feel that the aortico-pulmonary anastomosis has its greatest usefulness. In
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patients 12 years of age or older, usually the incision is made on the left and either an end-to-side or end-to-end subclavian pulmonary anastomosis is performed. An alternative would be an aortico-pulmonary anastomosis (Fig. 399). Mention should be made of certain anomalies of the great vessels
Fig. 397. The steps of the anastomosis are demonstrated, reading from left to right. The suture is an everting, continuous one (5-0 Deknatel) which includes the entire thickness of the artery. These are taken about 1 mm. apart. The posterior row is placed before it is drawn taut. The ends are then tied to stay sutures. The anterior row is interrupted at one point. This row is pulled taut as it is placed.
which may occur. In 20 to 25 per cent of cases the aorta descends on the right, and the innominate and its subclavian branch are found on the left. A single pulmonary artery is occasionally encountered. A retroesophageal subclavian is not infrequently seen and is sometimes suspected preoperatively by the deformity on the esophagram. Usually this vessel may be left in a retroesophageal position for the anastomosis, although occasionally this relationship is altered. The innominate artery may rarely be absent. In this instance the carotids and subclavians arise directly from the aortic arch. Bilateral superior vena cavae are some-
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times found. An aortic ring about the trachea,12 functional truncus arteriosus with a blind pulmonary artery, and anomalies of the pulmonary veins are rarely encountered (Figs. 400 and 401).
R.caroHd
Rsubc1avian
R.pulm.
Fig. 398. The alteration in the circulation following creation of an artificial ductus arteriosus is demonstrated. A right subclavian pulmonary anastomosis has been performed. Since the systemic arterial pressure is considerably higher than the pulmonary pressure, a large volume of blood may flow through a relatively small communication into the lungs.
In those instances in which there is valvular stenosis in association with an interventricular defect, valvulotomy is the treatment of choice. It is believed that the infundibular resection of Brock may be found most suitable in those instances where a well developed infundibular chamber is present.
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Results
Taussig and her associates have recently completed a report of the results of operation in the first 1000 cases operated upon in the Johns Hopkins HospitaP3 These results were classified into four groups, (a) good, (b) fair, (c) unimproved, and (d) dead. The results are as follows: Good result: This means a marked decrease in cyanosis, a rise in arterial oxygen saturation to 75 to 90 per cent, reduction in erythrocyte
Fig. 399. Technic of aortico-pulmonary anastomosis.
count and hematocrit reading towards normal, and marked subjective improvement. Those falling in this category represent 78.1 per cent of the total. Fair result: In this group there was definite subjective improvement, but cyanosis and polycythemia of varying degree persisted. The percentage of the total was 3.7. Unimproved: This means the course was not altered by operation and includes those instances in which operation proved to be technically impossible. This group comprises 2.6 per cent of the total. Dead: This group consists of all who died during or shortly after operation and is 15.6 per cent of the total.
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Thus, the over-all result in these patients, many of whom were extremely ill, is remarkably gratifying. In certain age groups the results following operation on patients with the typical tetralogy of Fallot are excellent. For example in those patients aged 10 to 15 years the results classified as "good" were 87 per cent with a mortality of only 8 per cent. In the more complex anomalies associated with pulmonary stenosis and atresia, the results are also good when the severity of the malforma-
Fig. 400. Specimen showing a single pulmonary artery.
tion is considered. For example, of 63 patients with tricuspid atresia, 68 per cent obtained a "good" result" with an additional 10 per cent classified as "fair". Interauricular septal defects are now being created in addition to the usual systemic-pulmonary anastomosis in selected cases. This group is now being evaluated. Since Brock has had considerable experience with the direct infundibular resection, his results should be considered. He has recently reported 24 cases of tetralogy of Fallot with infundibular stenosis in which an infundibular resection was performed with 7 deaths, a mortality of 29 per cent.u Most of the surviving patients were greatly benefited. In the tetralogy of Fallot with valvular stenosis, he reports 25 cases of
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valvulotomy with only 2 deaths. Both of these were in adults who were 21 and 24 years of age. He emphasizes the increased risk in this adult group and suggests that anastomoses are probably preferable. Glover,
Tretchea
R. subclavian
1
ctlspsl
Fig. 401. Double aortic arch in association with tetralogy of Fallot.
Bailey and their associates have reported infundibular resection in 12 patients with 4 operative deaths (33.3 per cent).14 ISOLATED PULMONARY VALVULAR STENOSIS WITH INTACT INTERVENTRICULAR SEPTUM
This type of congenital pulmonic stenosis has become of increasing interest in the past several years, principally due to the increased recogni-
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tion of the condition and to the excellent prognosis offered by valvulotomy. It is now felt that this condition is approximately 10 per cent as frequent as the tetralogy of Fallot and its variations. Since 1949 over
R.pulm.
Fig. 402. "Pure" pUlmonic valvular stenosis with intact interventricular septum.
60 patients with this anomaly have had pulmonary valvulotomy performed in the Johns Hopkins Hospital. Anatomy
In isolated ("pure") pulmonic stenosis, the primary lesion is the markedly deformed valve (Fig. 402). The cusps are all fused into a domelike
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structure. There is a small perforation in its center through which a small jet of blood is ejected with each systole (Fig. 403). This jet, with its high pressure, is felt to be responsible for the marked dilatation of the pulmonary artery. At times this dilatation may result in an artery which is several times normal in size. In addition, approximately 70 per cent of these patients have a foramen ovale or an interauricular defect which allows a right to left shunt and hence cyanosis. The tremendous work carried out by the right ventricle in emptying results in extreme degrees of myocardial hypertrophy. In those instances of patent auricular de-
Fig. 403. Demonstration of the fusion of the pulmonary valve cusps into a domelike structure in pure pulmonic stenosis. The incision made by a valvulotome is seen.
fects, adding more work for the left side of the heart, left ventricular hypertrophy occurs. Thus, while pulmonic valvular stenosis is the primary lesion, there are also additional anatomical features which are associated with it. Diagnosis
In the evaluation of this anomaly the history and physical examination are frequently adequate for diagnosis. However, a definite diagnosis cannot be made in many cases without angiocardiography or cardiac catheterization. Among the symptoms exercise intolerance is the most prominent. Examination reveals precordial prominence with a heave, cardiac
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enlargement (chiefly of the right side), and a precordial systolic murmur and thrill. In those with an associated interauricular defect (about 70 per cent), cyanosis and clubbing are present. The second pulmonary sound is quite soft. The liver may be enlarged and pulsating. The blood usually shows polycythemia, depending on the amount of cyanosis. A prolonged circulation time occurs if there is no auricular defect. Right
Fig. 404. Typical roentgenogram of patient with pure pUlmonic stenosis. Pulmonary artery dilatation and cardiac enlargement are present.
axis deviation and right ventricular hypertrophy are characteristically noted on electrocardiographic examination, and in cases of long standing a right bundle branch block is sometimes present. The chest film shows cardiac enlargement, chiefly of the right ventricle (Fig. 404). There is marked prominence of the pulmonary artery and a diminished vascularity of the lung fields. In the presence of an auricular shunt, the left auricle may be enlarged and may produce posterior displacement of the barium-filled esophagus in the right anterior oblique position.
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Frequently the roentgenograms obtained after the injection of an opaque medium are sufficiently characteristic to be diagnostic. The dye passes slowly through the heart (unless there is an auricular defect) due to obstruction of the right ventricular outflow. The right ventricle is large, and there is poststenotic dilatation of the pulmonary artery. One particularly characteristic feature is the lingering of the dye in the pulmonary artery which is usually present in several successive films taken at intervals of one second (Fig. 405). The right ventricular pressure is elevated, frequently to 100 mm. Hg and pressures over 200 mm. Hg have been recorded. The pulmonary artery pressure may be normal or low
A
B
Fig. 405. Angiocardiograms of patient with pure pulmonic stenosis. A, This film is taken 2 seconds after injection of dye. The pulmonary artery shows dilatation. B, Taken 2 seconds later shows lingering of dye in pulmonary artery.
and there is a diminished pulmonary blood flow. If an auricular defect is present, a low oxygen saturation is found. Treatment
The first attempt to treat pulmonic stenosis by surgical means has been previously mentioned.7 With recent renewed interest in this condition Brock has devised an attack which is quite successful. His report in 1948 of 3 patients treated by this method was the introduction to the successful treatment of this condition. Is Credit should also be given Sellors who performed a similar procedure with good result several months prior to Brock's first case. I6 The procedure is performed through an anterior incision with entry into the chest through the third left intercostal space. The pericardium is opened and the pulmonary artery is inspected. Distal to the stenotic
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valve, the artery is usually dilated. One can usually palpate the stenotic valve and a strong jet of blood can be felt as it strikes the pulmonary artery near its point of bifurcation. Two stay sutures are placed in the myocardium of the right ventricle and a small incision is then made between them. A probe is then introduced which is directed through the stenosis into the pulmonary artery (Fig. 406). ·For the performance of the valvulotomy, we favor the use of collapsible dilators and valvulotomes such as described by Potts 17 and Muller and Longmire. 18 Following division of the valves the jet and thrill are more diffuse. It must be em-
Fig. 406. Operative technic in isolated pulmonary stenosis. The incision in the right ventricle is shown as is the position of the knife. In this instance the Brock flat valvulotome is being employed.
phasized that should the patient's condition become grave shortly after the chest is opened (hypotension, cardiac arrhythmia, or cardiac arrest), it is important to proceed immediately with division of the valve as rapidly as possible. It has been observed it is unlikely in these instances that a failing heart can be revived without first alleviating the valvular stenosis. Results
Our experience in the division of the valve in the treatment of pulmonary valvular stenosis consists of operations on 61 patients. An account of the earlier cases has been reported previously.19 The results in
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the main have been excellent. In most instances the increase in exercise tolerance has been remarkable. Eight deaths have occurred in the series (13 per cent), in 4 of which there were additional lesions. Two had previously had a subclavian-pulmonary anastomosis for an incorrectly diagnosed tetralogy of Fallot (with an unfavorable postoperative course), a third was found at autopsy to have a tetralogy of Fallot, and the fourth died on the third postoperative day of a brain abscess demonstrated at autopsy to have been present for some time. The age range in our group has varied between 6 months and 33 years. Technically, this operation is not difficult. There is as yet no evidence that the divided valve leaflets will scar and fuse again. Present experience indicates that this type of malformation is relatively easily attacked and the expectation for good results is high. TRANSPOSITION OF THE GREAT ARTERIES
Anatomy
Complete transposition of the aorta and pulmonaty artery is a relatively common congenital anomaly. In this malformation the aorta arises from the right ventricle and the pulmonary artery from the left ventricle. Since this implies basically that there are two separate circulations, it is obvious that some communication between the two sides of the heart or the great blood vessels must exist to be compatible with life. Such communications include: (a) patent foramen ovale, (b) interauricular septal defect, (c) interventricular septal defect, (d) patent ductus arteriosus, and (e) partial transposition of pulmonary veins. Each of these may allow oxygenated blood to enter the systemic circulation. These communications may exist either singly or in combination, but at least one must be present if the infant is to survive following birth. In addition to these defects, both ventricles are usually hypertrophied. A variant of this malformation is transposition of the pulmonary artery and extreme dextroposition of the aorta combined with pulmonic stenosis. Clinically this condition more nearly resembles the tetralogy of Fallot, as the basic features of the latter are present. Another closely related anomaly is the Taussig-Bing syndrome. 22 Here the aorta is transposed and a large pulmonary artery arises primarily from the right ventricle and partially overrides a high ventricular septal defect. The chance of survival with complete transposition of the aorta and pulmonary artery is poor. In a collection of 123 cases, the average duration of life was nineteen months. 20 Only 6 survived longer than the tenth year. The longest average survival was found in those with interventricular defects, and those with an interauricular defect and patent foramen ovale ranked second.
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Diagnosis
As a rule infants with transposition of the great arteries are cyanotic at birth. They are usually severely incapacitated, although in the presence of a large interventricular septal defect the signs and symptoms are less pronounced. On examination intense cyanosis is present. The heart
Fig. 407. Roentgenogram demonstrating the narrow shadow cast by the great vessels in transposition of the great arteries.
is enlarged, the enlargement involving both ventricles. Murmurs and thrills may be present but are of no diagnostic importance. It is the radiographic examination which is mos(helpful in determining the diagnosis. The characteristic..narrow shadow cast by the great vessels in the anterior-posterior plane is highly significant (Fig, 407). This results from the altered positions of the aorta and pulmonary artery, such that the aorta is anterior, with the pulmonary artery almost directly behind it. The lung fields show increased vascularity. Both ventricles and the right auricle are enlarged. The electrocardiogram usually shows right axis devi-
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ation, although this is not consistent. The polycythemia is very marked. Angiocardiograms will show the anterior origin of the aorta and sometimes the shunt is demonstrated. Cardiac catheterization is not often of diagnostic aid. Thus, one of the outstanding features in the diagnosis of this condition is the narrow shadow cast by the great vessels in the anterior-posterior plane. When this is seen in association with intense cyanosis, enlargement of both ventricles and vascular lung fields, the most likely diagnosis is transposition of the great arteries. In certain instances it is possible to predict the shunt by clinical observation. For example, the relative absence of cyanosis in the lower extremities may indicate a patent ductus in association with transposition of the great vessels. Treatment
Various procedures directed towards accomplishing greater admixture of the two circulations have been attempted. The experience in The Johns Hopkins Hospital has been previously reported. 20 The operations fall into four main groups: (a) construction of extra cardiac shunts, either venous or arterial (anastomosis of the pulmonary veins to the vena cava or the vena cava to the pulmonary artery), (b) creation of an interauricular septal defect, (c) an auricular septal defect plus an extracardiac shunt, and (d) an interventricular defect. The first group, that of venous and arterial extracardiac shunts, has not proved to be satisfactory to date. The creation of an interauricular septal defect has been the most successful. The procedure is dependent upon the fact that the proximal portion of the wall of the right pulmonary veins is adherent to the posterior wall of the right auricle. It is possible to excise a portion of the septum between the pulmonary veins and the right auricle by appropriate occlusion of the lateral portion of the right auricle and of the right superior pulmonary veins (Figs. 408 and 409). This forms an interauricular communication, and in fact virtually the entire interauricular septum may be removed by this procedure. We have created interventricular septal defects in several patients, but this is still in an experimental stage. Certain variations of transposition of the great arteries have been noted to give more favorable results. In those instances in which there is an associated pulmonic stenosis, quite gratifying results have been obtained in some patients when an interauricular defect has been created and a pulmonary-subclavian anastomosis performed. Those patients with Taussig-Bing syndrome also have a better postoperative prognosis than those with the typical transposition of the aorta and pulmonary artery. Unfortunately the mortality is high and the results are poor in some of the survivors. This implies that a better procedure is desirable, such as division of the aorta and pulmonary artery with anastomosis to their
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proper ventricles. Another possible procedure is the transposition of the veins. It is hoped with further advances in the mechanical heart-lung
Fig. 408. Transposition of the great arteries. The right auricle and the pulmonary veins have been opened and the septum is being cut away with scissors. On the right a clamp is shown in place with a segment of interatrial septum prepared for excision.
Fig. 409. Demonstration of completion of the interauricular defect. The anterior wall of the auricle is being sutured to the anterior wall of the pulmonary veins. At the right the completed defect is shown.
preparation that a more satisfactory procedure for the treatment of this condition may be devised. MISCELLANEOUS CONDITIONS
Space will not permit us to consider other conditions such as transposition of the pulmonary veins, the Eisenmenger complex, patent duc-
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tus arteriosus with reverse flow, pulmonary arteriovenous fistulas, Ebstein's disease, and auricular and ventricular septal defects. SUMMARY
A review of the various types of congenital cyanotic heart disease amenable to surgery has been presented. The tetralogy of Fallot has been considered with reference to the various anatomical types which may be encountered and the appropriate treatment for each. The overall results following operation are good. A similar consideration has been given isolated valvular pulmonic stenosis with intact interventricular septum. This condition is now relatively easily diagnosed and treated. The survival rate and postoperative results are good. Transposition of the aorta and pulmonary artery are discussed. The various methods employed in its treatment are evaluated. At present the creation of an interauricular septal defect sometimes combined with a subclavian-pulmonary anastomosis is the proceQ.ure of choice. The results in this anomaly indicate that further inve'3tigation is necessary. NOTE: We are indebted to Surgery, Gynecology and Obstetrics, Annals of Surgery and The Scientific Monthly for their generous permission to reproduce illustrations presented in this paper.
REFERENCES 1. Blalock, A., Hanlon, C. R. and Scott, iI. W.: The Surgical Treatment of Congenital Cyanotic Heart Disease. The Scientific Monthly 69: 360-367, 1949. 2. Fallot, E.: Contribution a l'anatomic pathologique de la maladie bleue (cyanose cardique). Marseille-med. 25: 77-93, 138-158, 207-223, 270-286,341354, 402-420, 1888. 3. Blalock, A., Johns, T. N. P. and Williams, G. R., Jr.: The Anatomy of Pulmonary Stenosis and Atresia with Comments on Surgical Therapy. To be published. 4. Taussig, H. B.: Congenital Malformations of the Heart. The Commonwealth Fund, New York, 1947. 5. Cooley, R. N.: Personal communication. 6. Bing, R. J.: The Physiology of Congenital Heart Disease. Nelson Loose-Leaf Medicine, Vol. 4, Chap. 6, p. 423. 7. Dumont, J.: Chirurgie der Malformations Congenitales ou Acquises du Coeur. La Presse Medicale, 21: 860, 1913. 8. Levy, Sanford E. and Blalock, A.: Experimental Observations on the Effects of Connecting by Suture the Left Main Pulmonary to the Systemic Circulation. J. Thoracic Surg. 8: 525-530, 1939. 9. Blalock, A. and Taussig, H. B.: The Surgical Treatment of Malformations of the Heart in Which There is Pulmonary Stenosis and Pulmonary Atresia. J.A.M.A. 128: 109-202, 1945. 10. Potts, W. J., Smith S. and Gibson, S. J.: Anastomosis of Aorta to Pulmonary Artery; Certain Types in Congenital Heart Disease. J.A.M.A. 132: 627631, 1946. 11. Brock, R. C.: Cardiac Surgery. Ann. Roy. ColI. Surgeons England 9: 1-12, 1951.
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12. Bahnson, H. T. and Blalock, A.: Aortic Vascular Rings Encountered in the Surgical Treatment of Congenital Pulmonic Stenosis. Ann. Surg. 131: 356-362, 1950. 13. Taussig, H. B., King, J. T., Bauersfeld, R. and Swaramakrishne-Padvamah. Iyer: Results of Operation for Pulmonary Stenosis and Atresia: Report ~ of 1000 Cases. Tr. A. Am. Phys. 64: 67, 1951. 14. Glover, R. P., Bailey, C. P., O'Neill, T. J. F., Downing, D. F. and Wells, C. R. E.: The Direct Intracardiac Relief of Pulmonary Stenosis in the Tetralogy of Fallot. J. Thoracic Surg. 23: 14-41, 1952. 15. Brock, R. C.: Pulmonary Valvulotomy for the Relief of Congenital Pulmonic Stenosis. Brit. M. J. 1: 1121,1948. 16. Sellors, T. H.: Surgery of Pulmonary Stenosis. Lancet 254: 988, 1948. 17. Potts, W. J. and Riker, W. L.: Surgical Treatment of Pulmonary Stenosis with Intact Interventricular Septum. Arch. Surg. 62: 776, 1951. 18. Muller, W. H. and Longmire, W. P.: The Surgical Treatment of Pure Pulmonic Stenosis. A Description of an Expansible Valvulotome. Surgery 30: 275, 1951. 19. Blalock, A. and Kieffer, R. F., Jr.: Valvulotomy for the Relief of Congenital Valvular Pulmonic Stenosis with Intact Ventricular Septum: Report of Nineteen Operations by the Brock Method. Ann. Surg. 132: 496, 1950. 20. Hanlon, C. R. and Blalock, A.: Complete Transposition of the Aorta and Pulmonary Artery. Experimental Observations on Venous Shunts as Corrective Procedures. Ann. Surg. 127: 385, 1948. 21. Blalock, A. and Hanlon, C. R.: Interatrial Septal Defect. Its Experimental Production under Direct Vision without Interruption of the Circulation. Surg., Gynec. & Obst. 87: 183, 1948. 22. Taussig, H. B. and Bing, R. J.: Complete Transposition of the Aorta and a Levoposition of the Pulmonary Artery. Am. Heart J. 37: 55, 1949.
The Surgical Treatment of Congenital Heart Disease Accompanied by Cyanosis DAVID C. SABISTON, JR., M.D.* ALFRED BLALOCK, M.D., F.A.C.S., F.R.C.S. (HON.)t
IN ORDER to understand the recent developments in the treatment of patients with congenital heart disease, it is essential to be familiar with the normal and abnormal structure and function of the circulatory system. Although the normal structure of the heart and vessels was known to Galen in 179 A.D., it was not until 1628 that William Harvey recognized the course of the blood, through the arteries and its return by veins. Physiologists of the nineteenth century demonstrated the function of the lungs in oxygenating the venous blood and returning it to the left auricle for circulation through the systemic vessels. A consideration of the fetal circulation is essential for the understanding of congenital circulatory defects. Prior to birth the infant derives From the Department of Surgery, The Johns Hopkins Hospital, and The Johns Hopkins University School of Medicine, Baltimore, Maryland.
* Assistant in Surgery, Johns Hopkins University School of Medicine; Assistant Resident in Surgery, Johns Hopkins Hospital.
t Professor of Surgery and Director, Department of Surgery, The Johns Hopkins University School of Medicine; Surgeon-in-Chief, The Johns Hopkins Hospital. 1273
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its oxygen from the maternal circulation through the placenta. The lungs are collapsed. Oxygenated blood is conducted through the umbilical vein to the right auricle of the fetus where a portion enters the right
Key
Venous blood Arleri ..l blood Mixed blood Venous ~!, ,.n--I"-:
!~~~re:; (Closes at birth)
Duclus
OXYGlenaled blood
from mother vIa _ _- - ; c
umbtUcal vein
Fig. 391. Diagram of fetal circulation. Normal circulation is established with closure of the foramen ovale and ductus arteriosus.
ventricle to be propelled into the pulmonary circulation, and the remainder is shunted through the interauricular communication (patent foramen ovale) into the left auricle and thence into the systemic circulation. In addition the systemic circulation also receives blood shunted directly from the pulmonary artery, where the pressure is high (due to the high resistance offered by the collapsed lungs) through the ductus arteriosus into the aorta (Fig. 391).1
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However, with birth or shortly thereafter, two adjustments occur. Both the foramen ovale and ductus arteriosus normally close. This completely separates the pulmonary and systemic circulations and the normal postnatal circulatory plan is established. In most instances the etiology of congenital cardiac lesions is not known. Certain viral infections occurring in the mother in the early months of pregnancy are now recognized etiologic factors. The majority of these defects do occur relatively early in embryonic life. The persistent patent ductus arteriosus is an exception since it is a developmental error which occurs late. The malformations show great variation, and many are incompatible with extra-uterine life. Congenital heart disease has been divided into two groups, "cyanotic" and "noncyanotic." Cyanosis (kuavos, "dark blue") is a result of an abnormally high amount of unoxygenated blood as observed in the superficial vessels of the integument. This phenomenon does not appear until at least 5 gm. of unoxygenated hemoglobin are present per 100 cc. of blood. As a result of their low oxygen saturation, patients with cyanotic heart disease are usually markedly retarded in development and activity. Many of them are able to take only a few steps before the onset of fatigue and dyspnea. In an attempt to compensate for this the body produces an excessive number of erythrocytes. This number is usually about 8,000,000, but the count can be considerably higher. Another associated though poorly explained phenomenon is the high incidence of "clubbed" digits in these patients. There are numerous anatomical defects which may occur in congenital cyanotic heart disease. In most instances the pulmonary artery arises from the right ventricle in an abnormal manner. In addition, there are various associated intracardiac defects. These malformations may be of such severity as to allow only a small portion of the venous blood to enter the pulmonary system, much of the blood being shunted directly to the systemic system. TETRALOGY OF FALLOT AND ASSOCIATED DISORDERS
The tetralogy of Fallot is the most common congenital cardiac anomaly in the cyanotic group. There are a number of variations which may occur in this condition. As originally described in 1888 by Fallot2 the essential features are pulmonary stenosis, overriding of the aorta, high interventricular septal defect,· and hypertrophy of the right ventricle (Fig. 392). This group may be subdivided into those with (a) infundibular stenosis, (b) pulmonary atresia, (c) infundibular and valvular stenosis, (d) valvular stenosis with an interventricular septal defect, and (e) complex variations including nonfunctioning right ventricle, single ventricle with pulmonic stenosis, and tricuspid atresia.
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Anatomy 1. Infundibular Stenosis. This type of pulmonary stenosis is the most common in the tetralogy of Fallot. It is found in more than half the
Fig. 392. Diagram of the tetralogy of Fallot demonstrating infundibular pulmonic stenosis, an interventricular septal defect, an overriding aorta, and hypertrophy of the right ventricle.
instances. Here the stenosis is in the conus arteriosus, proximal to the pulmonary valve. It is produced by a prominent muscular band, the crista supraventricularis, which extends from the anteromedial wall of the right ventricle to the interventricular septum. The relative position and size of this muscular structure determine the nature of the chamber
Congenital Heart Disease with Cyanosis
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present between it and the pulmonary valve ring. Thus, when the stenosis is high, there is a small chamber, when intermediate, a medium chamber, and when low, a large one. Each of these occurs with approximately the same incidence. 3 2. Pulmonary Atresia. There may be an absence of communication of the right ventricle with the pulmonary artery, an atresia. This occurs in less than one-fifth of the instances of tetralogy of Fallot. The atresia is usually at the most proximal portion of the conus arteriosus, and it is !J.lmost always associated with a diminutive pulmonary artery. Further, the aorta is frequently far to the right and considerably enlarged. 3. Infundibular and Valvular Stenosis. Stenosis o"ccurring at both the conus and valvular levels is encountered less frequently. The infundibular chamber is usually large, and often the aorta overrides far to the right. 4. Valvular Stenosis with an Interventricular Septal Defect. Valvular pulmonic stenosis in association with an interventricular septal defect is less common than the types previously described. The valvular anatomy here is similar to that in isolated valvular stenosis. However, the pulmonary artery is usually normal in size or slightly dilated. 5. Complex Variations. There are several additional anomalies more complex in nature which can possibly be considered variations of this group. The entity of a nonfunctioning right ventricle is occasionally encountered, and is almost always associated with anomalies of tllfJ . . tricuspid valve and pulmonary artery. The tricuspid valve may be markedly hypoplastic and the right ventricle so diminutive as to be virtually nonfunctional. There is usually pulmonary atresia or marked stenosis with a small artery. The single ventricle with pulmonic stenosis is a further variation. Here the auricles empty into a single ventricle with a stenotic pulmonary artery emerging either from a rudimentary ventricular chamber or from the common chamber. In tricuspid atresia there is defective development of the right ventricle. The primary lesion is atresia of the tricuspid valve, and the blood must pass through a patent foramen ovale or an interauricular septal defect to the left auricle into the left ventricle. To enter the lung the blood must pass through a ventricular septal defect to the diminutive right ventricle or it may course into the aorta and reach the lungs by a patent ductus arteriosus or the bronchial arteries. ,~
Diagnosis
The diagnosis of the tetralogy of Fallot and its variations is given in detail by Taussig and others.4 The important symptoms in the classic type are diminished exercise tolerance, squatting, and retarded growth. Cerebrovascular accidents are not uncommon. The positive physical
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findings include cyanosis, a small heart, a systolic murmur in the pulmonary area and along the left sternal border, a loud aortic second sound and a weak pulmonary second sound, and clubbing of the digits. Examination of the blood reveals polycythemia (usually between 6 and 9 million), a high hematocrit (60 to 90), and the platelets are sometimes diminished. The arterial oxygen saturation is low, being 65 to 75 per cent in most instances. However, in severe examples it may be below 25 per cent. The electrocardiogram nearly always shows a right axis deviation. The P waves may be high and pointed. In the usual tetralogy the radiographic findings include a concavity in the region of the pulmo-
A
B
Fig. 393. A, Roentgenogram of patient with tetralogy of Fallot. Note concavity in region of pulmonary conus. B, Roentgenogram of same patient following operation.
nary conus, diminished size and pulsation of the pulmonary arteries, and a clear pulmonary window (Fig. 393). Since the aorta descends on the right in 20 to 25 per cent of these patients, an esophagogram should always be done to ascertain this point before operation. Special laboratory procedures such as angiocardiography and cardiac catheterization give invaluable aid in certain patients. The angiocardiogram in the tetralogy demonstrates the overriding aorta and gives an indication of the degree of pulmonary stenosis. However, it is to be stressed that angiocardiography is not without hazard in the cyanotic patient with reduced cardiac reserve. In the Johns Hopkins Hospital over 300 angiocardiograms have been performed in patients with congenital heart disease with cyanosis. There have been two deaths definitely attributable to the procedure. Two additional deaths may possibly have
Congenital Heart Disease with Cyanosis
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been related to the angiocardiography. There have been no deaths in the past two years.6 The figures obtained by cardiac catheterization are sometimes of invaluable aid in attaining the correct diagnosis. Bing and associates found that in patients with pulmonic stenosis (confirmed at operation) 80 per cent show with exercise a fall in oxygen consumption per liter of ventilation. 6 In the classic tetralogy of Fallot, tricuspid atresia, and single ventricle with pulmonic stenosis, the pulmonary flow is less than the systemic flow, and this is accompanied by a reduced pulmonary artery pressure. A high right ventricular pressure is present in the usual tetralogy of Fallot. The typical tetralogy of Fallot presents a few difficulties in diagnosis. Patients with pulmonary atresia usually give a history of extreme limitation of activity dating from early infancy. A systolic murmur is barely audible or absent. Angiocardiography and cardiac catheterization are seldom of diagnostic aid. In tricuspid atresia left axis deviation on the electrocardiogram is nearly always found. Also, fluoroscopy shows that the heart does not project as far anteriorly toward the retrosternal space, but it projects posteriorly toward the spine (hypoplastic right ventricle). Treatment
The first attempt to treat pulmonic stenosis by operative means was by Doyen in 1911.7 He attempted to divide the valve with a tenotome but was unsuccessful. In 1944, following extensive experimental studies in animals dating some years previously,S the first successful systemicpulmonary anastomosis was performed. 9 This operation consists of an anastomosis between a branch of the aortic arch (subclavian, carotid or innominate) to one of the pulmonary arter.ies. The procedure most frequently performed in this clinic is a subclavian-pulmonary artery anastomosis (end-to-side). Since the first procedure in November 1944, over 1150 such operations have been performed in the Johns Hopkins Hospital. Potts described a special clamp which permits partial occlusion of the aorta, and by its use an anastomosis may be performed between the side of the aorta and the side of the pulmonary artery. The first such procedure was performed by him in 1946.1° He prefers this method for cases in which the aortic arch descends on the left. Brock and others have felt that a direct attack on the stenosis, that is, an infundibular resection, is the operative procedure of choice in many instances. This subject is receiving considerable attention, and the Brock technic is being applied in selected cases. However, at present the shunt operations, either subclavian-pulmonary or aortico-pulmonary, appear to be preferred.
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There are several possible choices in the anastomotic procedures. In this clinic anastomoses between the following vessels have been employed: (a) the proximal end of the right or left subclavian artery and the side of the right or left pulmonary artery; (b) the proximal end of the right or left subclavian artery and the end of the right or left pulmonary artery; (c) the proximal end of the carotid or innominate artery and the side or distal end of the right or left pulmonary artery; and (d)
Fig. 394. Demonstration of the dissection of the innominate artery and its branches. The position of the pulmonary artery is shown (right).
the side of the aorta and the side of one of the pulmonary arteries. The anastomosis of choice is determined by the age of the patient, the descent of the aorta, and by the local anatomical configuration found at operation. The great majority of our patients have had an anastomosis between the end of the subclavian branch of the innominate artery and the side of the corresponding pulmonary artery. This is our procedure of choice for the usual patient, and it is illustrated in Figures 394 through 397. The alteration in the circulation following creation of an artificial ductus arteriosus is demonstrated in Figure 398. In infants (under the
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age of 2) the incision is usually made on the side on which the aorta descends. Thus, if the subclavian artery is not of suitable size, a Potts'
Fig. 395. The pulmonary artery is occluded and an incision in it has been made. The subclavian artery (center) has been divided and turned down to be anastomosed to the pulmonary artery (right).
Fig. 396. The anastomosis is completed. The occluding devices have been removed (right).
procedure may be employed. This procedure may be quite difficult in cases with a right aortic arch. It is in the extremely ill infant we feel that the aortico-pulmonary anastomosis has its greatest usefulness. In
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patients 12 years of age or older, usually the incision is made on the left and either an end-to-side or end-to-end subclavian pulmonary anastomosis is performed. An alternative would be an aortico-pulmonary anastomosis (Fig. 399). Mention should be made of certain anomalies of the great vessels
Fig. 397. The steps of the anastomosis are demonstrated, reading from left to right. The suture is an everting, continuous one (5-0 Deknatel) which includes the entire thickness of the artery. These are taken about 1 mm. apart. The posterior row is placed before it is drawn taut. The ends are then tied to stay sutures. The anterior row is interrupted at one point. This row is pulled taut as it is placed.
which may occur. In 20 to 25 per cent of cases the aorta descends on the right, and the innominate and its subclavian branch are found on the left. A single pulmonary artery is occasionally encountered. A retroesophageal subclavian is not infrequently seen and is sometimes suspected preoperatively by the deformity on the esophagram. Usually this vessel may be left in a retroesophageal position for the anastomosis, although occasionally this relationship is altered. The innominate artery may rarely be absent. In this instance the carotids and subclavians arise directly from the aortic arch. Bilateral superior vena cavae are some-
Congenital Heart Disease with Cyanosis
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times found. An aortic ring about the trachea,12 functional truncus arteriosus with a blind pulmonary artery, and anomalies of the pulmonary veins are rarely encountered (Figs. 400 and 401).
R.caroHd
Rsubc1avian
R.pulm.
Fig. 398. The alteration in the circulation following creation of an artificial ductus arteriosus is demonstrated. A right subclavian pulmonary anastomosis has been performed. Since the systemic arterial pressure is considerably higher than the pulmonary pressure, a large volume of blood may flow through a relatively small communication into the lungs.
In those instances in which there is valvular stenosis in association with an interventricular defect, valvulotomy is the treatment of choice. It is believed that the infundibular resection of Brock may be found most suitable in those instances where a well developed infundibular chamber is present.
1281,-
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Results
Taussig and her associates have recently completed a report of the results of operation in the first 1000 cases operated upon in the Johns Hopkins HospitaP3 These results were classified into four groups, (a) good, (b) fair, (c) unimproved, and (d) dead. The results are as follows: Good result: This means a marked decrease in cyanosis, a rise in arterial oxygen saturation to 75 to 90 per cent, reduction in erythrocyte
Fig. 399. Technic of aortico-pulmonary anastomosis.
count and hematocrit reading towards normal, and marked subjective improvement. Those falling in this category represent 78.1 per cent of the total. Fair result: In this group there was definite subjective improvement, but cyanosis and polycythemia of varying degree persisted. The percentage of the total was 3.7. Unimproved: This means the course was not altered by operation and includes those instances in which operation proved to be technically impossible. This group comprises 2.6 per cent of the total. Dead: This group consists of all who died during or shortly after operation and is 15.6 per cent of the total.
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Thus, the over-all result in these patients, many of whom were extremely ill, is remarkably gratifying. In certain age groups the results following operation on patients with the typical tetralogy of Fallot are excellent. For example in those patients aged 10 to 15 years the results classified as "good" were 87 per cent with a mortality of only 8 per cent. In the more complex anomalies associated with pulmonary stenosis and atresia, the results are also good when the severity of the malforma-
Fig. 400. Specimen showing a single pulmonary artery.
tion is considered. For example, of 63 patients with tricuspid atresia, 68 per cent obtained a "good" result" with an additional 10 per cent classified as "fair". Interauricular septal defects are now being created in addition to the usual systemic-pulmonary anastomosis in selected cases. This group is now being evaluated. Since Brock has had considerable experience with the direct infundibular resection, his results should be considered. He has recently reported 24 cases of tetralogy of Fallot with infundibular stenosis in which an infundibular resection was performed with 7 deaths, a mortality of 29 per cent.u Most of the surviving patients were greatly benefited. In the tetralogy of Fallot with valvular stenosis, he reports 25 cases of
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valvulotomy with only 2 deaths. Both of these were in adults who were 21 and 24 years of age. He emphasizes the increased risk in this adult group and suggests that anastomoses are probably preferable. Glover,
Tretchea
R. subclavian
1
ctlspsl
Fig. 401. Double aortic arch in association with tetralogy of Fallot.
Bailey and their associates have reported infundibular resection in 12 patients with 4 operative deaths (33.3 per cent).14 ISOLATED PULMONARY VALVULAR STENOSIS WITH INTACT INTERVENTRICULAR SEPTUM
This type of congenital pulmonic stenosis has become of increasing interest in the past several years, principally due to the increased recogni-
Congenital Heart Disease with Cyanosis
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tion of the condition and to the excellent prognosis offered by valvulotomy. It is now felt that this condition is approximately 10 per cent as frequent as the tetralogy of Fallot and its variations. Since 1949 over
R.pulm.
Fig. 402. "Pure" pUlmonic valvular stenosis with intact interventricular septum.
60 patients with this anomaly have had pulmonary valvulotomy performed in the Johns Hopkins Hospital. Anatomy
In isolated ("pure") pulmonic stenosis, the primary lesion is the markedly deformed valve (Fig. 402). The cusps are all fused into a domelike
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David C. Sabiston, Jr., Alfred Blalock
structure. There is a small perforation in its center through which a small jet of blood is ejected with each systole (Fig. 403). This jet, with its high pressure, is felt to be responsible for the marked dilatation of the pulmonary artery. At times this dilatation may result in an artery which is several times normal in size. In addition, approximately 70 per cent of these patients have a foramen ovale or an interauricular defect which allows a right to left shunt and hence cyanosis. The tremendous work carried out by the right ventricle in emptying results in extreme degrees of myocardial hypertrophy. In those instances of patent auricular de-
Fig. 403. Demonstration of the fusion of the pulmonary valve cusps into a domelike structure in pure pulmonic stenosis. The incision made by a valvulotome is seen.
fects, adding more work for the left side of the heart, left ventricular hypertrophy occurs. Thus, while pulmonic valvular stenosis is the primary lesion, there are also additional anatomical features which are associated with it. Diagnosis
In the evaluation of this anomaly the history and physical examination are frequently adequate for diagnosis. However, a definite diagnosis cannot be made in many cases without angiocardiography or cardiac catheterization. Among the symptoms exercise intolerance is the most prominent. Examination reveals precordial prominence with a heave, cardiac
Congenital Heart Disease with Cyanosis
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enlargement (chiefly of the right side), and a precordial systolic murmur and thrill. In those with an associated interauricular defect (about 70 per cent), cyanosis and clubbing are present. The second pulmonary sound is quite soft. The liver may be enlarged and pulsating. The blood usually shows polycythemia, depending on the amount of cyanosis. A prolonged circulation time occurs if there is no auricular defect. Right
Fig. 404. Typical roentgenogram of patient with pure pUlmonic stenosis. Pulmonary artery dilatation and cardiac enlargement are present.
axis deviation and right ventricular hypertrophy are characteristically noted on electrocardiographic examination, and in cases of long standing a right bundle branch block is sometimes present. The chest film shows cardiac enlargement, chiefly of the right ventricle (Fig. 404). There is marked prominence of the pulmonary artery and a diminished vascularity of the lung fields. In the presence of an auricular shunt, the left auricle may be enlarged and may produce posterior displacement of the barium-filled esophagus in the right anterior oblique position.
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Frequently the roentgenograms obtained after the injection of an opaque medium are sufficiently characteristic to be diagnostic. The dye passes slowly through the heart (unless there is an auricular defect) due to obstruction of the right ventricular outflow. The right ventricle is large, and there is poststenotic dilatation of the pulmonary artery. One particularly characteristic feature is the lingering of the dye in the pulmonary artery which is usually present in several successive films taken at intervals of one second (Fig. 405). The right ventricular pressure is elevated, frequently to 100 mm. Hg and pressures over 200 mm. Hg have been recorded. The pulmonary artery pressure may be normal or low
A
B
Fig. 405. Angiocardiograms of patient with pure pulmonic stenosis. A, This film is taken 2 seconds after injection of dye. The pulmonary artery shows dilatation. B, Taken 2 seconds later shows lingering of dye in pulmonary artery.
and there is a diminished pulmonary blood flow. If an auricular defect is present, a low oxygen saturation is found. Treatment
The first attempt to treat pulmonic stenosis by surgical means has been previously mentioned.7 With recent renewed interest in this condition Brock has devised an attack which is quite successful. His report in 1948 of 3 patients treated by this method was the introduction to the successful treatment of this condition. Is Credit should also be given Sellors who performed a similar procedure with good result several months prior to Brock's first case. I6 The procedure is performed through an anterior incision with entry into the chest through the third left intercostal space. The pericardium is opened and the pulmonary artery is inspected. Distal to the stenotic
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valve, the artery is usually dilated. One can usually palpate the stenotic valve and a strong jet of blood can be felt as it strikes the pulmonary artery near its point of bifurcation. Two stay sutures are placed in the myocardium of the right ventricle and a small incision is then made between them. A probe is then introduced which is directed through the stenosis into the pulmonary artery (Fig. 406). ·For the performance of the valvulotomy, we favor the use of collapsible dilators and valvulotomes such as described by Potts 17 and Muller and Longmire. 18 Following division of the valves the jet and thrill are more diffuse. It must be em-
Fig. 406. Operative technic in isolated pulmonary stenosis. The incision in the right ventricle is shown as is the position of the knife. In this instance the Brock flat valvulotome is being employed.
phasized that should the patient's condition become grave shortly after the chest is opened (hypotension, cardiac arrhythmia, or cardiac arrest), it is important to proceed immediately with division of the valve as rapidly as possible. It has been observed it is unlikely in these instances that a failing heart can be revived without first alleviating the valvular stenosis. Results
Our experience in the division of the valve in the treatment of pulmonary valvular stenosis consists of operations on 61 patients. An account of the earlier cases has been reported previously.19 The results in
I
I
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the main have been excellent. In most instances the increase in exercise tolerance has been remarkable. Eight deaths have occurred in the series (13 per cent), in 4 of which there were additional lesions. Two had previously had a subclavian-pulmonary anastomosis for an incorrectly diagnosed tetralogy of Fallot (with an unfavorable postoperative course), a third was found at autopsy to have a tetralogy of Fallot, and the fourth died on the third postoperative day of a brain abscess demonstrated at autopsy to have been present for some time. The age range in our group has varied between 6 months and 33 years. Technically, this operation is not difficult. There is as yet no evidence that the divided valve leaflets will scar and fuse again. Present experience indicates that this type of malformation is relatively easily attacked and the expectation for good results is high. TRANSPOSITION OF THE GREAT ARTERIES
Anatomy
Complete transposition of the aorta and pulmonaty artery is a relatively common congenital anomaly. In this malformation the aorta arises from the right ventricle and the pulmonary artery from the left ventricle. Since this implies basically that there are two separate circulations, it is obvious that some communication between the two sides of the heart or the great blood vessels must exist to be compatible with life. Such communications include: (a) patent foramen ovale, (b) interauricular septal defect, (c) interventricular septal defect, (d) patent ductus arteriosus, and (e) partial transposition of pulmonary veins. Each of these may allow oxygenated blood to enter the systemic circulation. These communications may exist either singly or in combination, but at least one must be present if the infant is to survive following birth. In addition to these defects, both ventricles are usually hypertrophied. A variant of this malformation is transposition of the pulmonary artery and extreme dextroposition of the aorta combined with pulmonic stenosis. Clinically this condition more nearly resembles the tetralogy of Fallot, as the basic features of the latter are present. Another closely related anomaly is the Taussig-Bing syndrome. 22 Here the aorta is transposed and a large pulmonary artery arises primarily from the right ventricle and partially overrides a high ventricular septal defect. The chance of survival with complete transposition of the aorta and pulmonary artery is poor. In a collection of 123 cases, the average duration of life was nineteen months. 20 Only 6 survived longer than the tenth year. The longest average survival was found in those with interventricular defects, and those with an interauricular defect and patent foramen ovale ranked second.
Congenital Heart Disease with Cyanosis
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Diagnosis
As a rule infants with transposition of the great arteries are cyanotic at birth. They are usually severely incapacitated, although in the presence of a large interventricular septal defect the signs and symptoms are less pronounced. On examination intense cyanosis is present. The heart
Fig. 407. Roentgenogram demonstrating the narrow shadow cast by the great vessels in transposition of the great arteries.
is enlarged, the enlargement involving both ventricles. Murmurs and thrills may be present but are of no diagnostic importance. It is the radiographic examination which is mos(helpful in determining the diagnosis. The characteristic..narrow shadow cast by the great vessels in the anterior-posterior plane is highly significant (Fig, 407). This results from the altered positions of the aorta and pulmonary artery, such that the aorta is anterior, with the pulmonary artery almost directly behind it. The lung fields show increased vascularity. Both ventricles and the right auricle are enlarged. The electrocardiogram usually shows right axis devi-
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ation, although this is not consistent. The polycythemia is very marked. Angiocardiograms will show the anterior origin of the aorta and sometimes the shunt is demonstrated. Cardiac catheterization is not often of diagnostic aid. Thus, one of the outstanding features in the diagnosis of this condition is the narrow shadow cast by the great vessels in the anterior-posterior plane. When this is seen in association with intense cyanosis, enlargement of both ventricles and vascular lung fields, the most likely diagnosis is transposition of the great arteries. In certain instances it is possible to predict the shunt by clinical observation. For example, the relative absence of cyanosis in the lower extremities may indicate a patent ductus in association with transposition of the great vessels. Treatment
Various procedures directed towards accomplishing greater admixture of the two circulations have been attempted. The experience in The Johns Hopkins Hospital has been previously reported. 20 The operations fall into four main groups: (a) construction of extra cardiac shunts, either venous or arterial (anastomosis of the pulmonary veins to the vena cava or the vena cava to the pulmonary artery), (b) creation of an interauricular septal defect, (c) an auricular septal defect plus an extracardiac shunt, and (d) an interventricular defect. The first group, that of venous and arterial extracardiac shunts, has not proved to be satisfactory to date. The creation of an interauricular septal defect has been the most successful. The procedure is dependent upon the fact that the proximal portion of the wall of the right pulmonary veins is adherent to the posterior wall of the right auricle. It is possible to excise a portion of the septum between the pulmonary veins and the right auricle by appropriate occlusion of the lateral portion of the right auricle and of the right superior pulmonary veins (Figs. 408 and 409). This forms an interauricular communication, and in fact virtually the entire interauricular septum may be removed by this procedure. We have created interventricular septal defects in several patients, but this is still in an experimental stage. Certain variations of transposition of the great arteries have been noted to give more favorable results. In those instances in which there is an associated pulmonic stenosis, quite gratifying results have been obtained in some patients when an interauricular defect has been created and a pulmonary-subclavian anastomosis performed. Those patients with Taussig-Bing syndrome also have a better postoperative prognosis than those with the typical transposition of the aorta and pulmonary artery. Unfortunately the mortality is high and the results are poor in some of the survivors. This implies that a better procedure is desirable, such as division of the aorta and pulmonary artery with anastomosis to their
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proper ventricles. Another possible procedure is the transposition of the veins. It is hoped with further advances in the mechanical heart-lung
Fig. 408. Transposition of the great arteries. The right auricle and the pulmonary veins have been opened and the septum is being cut away with scissors. On the right a clamp is shown in place with a segment of interatrial septum prepared for excision.
Fig. 409. Demonstration of completion of the interauricular defect. The anterior wall of the auricle is being sutured to the anterior wall of the pulmonary veins. At the right the completed defect is shown.
preparation that a more satisfactory procedure for the treatment of this condition may be devised. MISCELLANEOUS CONDITIONS
Space will not permit us to consider other conditions such as transposition of the pulmonary veins, the Eisenmenger complex, patent duc-
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tus arteriosus with reverse flow, pulmonary arteriovenous fistulas, Ebstein's disease, and auricular and ventricular septal defects. SUMMARY
A review of the various types of congenital cyanotic heart disease amenable to surgery has been presented. The tetralogy of Fallot has been considered with reference to the various anatomical types which may be encountered and the appropriate treatment for each. The overall results following operation are good. A similar consideration has been given isolated valvular pulmonic stenosis with intact interventricular septum. This condition is now relatively easily diagnosed and treated. The survival rate and postoperative results are good. Transposition of the aorta and pulmonary artery are discussed. The various methods employed in its treatment are evaluated. At present the creation of an interauricular septal defect sometimes combined with a subclavian-pulmonary anastomosis is the proceQ.ure of choice. The results in this anomaly indicate that further inve'3tigation is necessary. NOTE: We are indebted to Surgery, Gynecology and Obstetrics, Annals of Surgery and The Scientific Monthly for their generous permission to reproduce illustrations presented in this paper.
REFERENCES 1. Blalock, A., Hanlon, C. R. and Scott, iI. W.: The Surgical Treatment of Congenital Cyanotic Heart Disease. The Scientific Monthly 69: 360-367, 1949. 2. Fallot, E.: Contribution a l'anatomic pathologique de la maladie bleue (cyanose cardique). Marseille-med. 25: 77-93, 138-158, 207-223, 270-286,341354, 402-420, 1888. 3. Blalock, A., Johns, T. N. P. and Williams, G. R., Jr.: The Anatomy of Pulmonary Stenosis and Atresia with Comments on Surgical Therapy. To be published. 4. Taussig, H. B.: Congenital Malformations of the Heart. The Commonwealth Fund, New York, 1947. 5. Cooley, R. N.: Personal communication. 6. Bing, R. J.: The Physiology of Congenital Heart Disease. Nelson Loose-Leaf Medicine, Vol. 4, Chap. 6, p. 423. 7. Dumont, J.: Chirurgie der Malformations Congenitales ou Acquises du Coeur. La Presse Medicale, 21: 860, 1913. 8. Levy, Sanford E. and Blalock, A.: Experimental Observations on the Effects of Connecting by Suture the Left Main Pulmonary to the Systemic Circulation. J. Thoracic Surg. 8: 525-530, 1939. 9. Blalock, A. and Taussig, H. B.: The Surgical Treatment of Malformations of the Heart in Which There is Pulmonary Stenosis and Pulmonary Atresia. J.A.M.A. 128: 109-202, 1945. 10. Potts, W. J., Smith S. and Gibson, S. J.: Anastomosis of Aorta to Pulmonary Artery; Certain Types in Congenital Heart Disease. J.A.M.A. 132: 627631, 1946. 11. Brock, R. C.: Cardiac Surgery. Ann. Roy. ColI. Surgeons England 9: 1-12, 1951.
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12. Bahnson, H. T. and Blalock, A.: Aortic Vascular Rings Encountered in the Surgical Treatment of Congenital Pulmonic Stenosis. Ann. Surg. 131: 356-362, 1950. 13. Taussig, H. B., King, J. T., Bauersfeld, R. and Swaramakrishne-Padvamah. Iyer: Results of Operation for Pulmonary Stenosis and Atresia: Report ~ of 1000 Cases. Tr. A. Am. Phys. 64: 67, 1951. 14. Glover, R. P., Bailey, C. P., O'Neill, T. J. F., Downing, D. F. and Wells, C. R. E.: The Direct Intracardiac Relief of Pulmonary Stenosis in the Tetralogy of Fallot. J. Thoracic Surg. 23: 14-41, 1952. 15. Brock, R. C.: Pulmonary Valvulotomy for the Relief of Congenital Pulmonic Stenosis. Brit. M. J. 1: 1121,1948. 16. Sellors, T. H.: Surgery of Pulmonary Stenosis. Lancet 254: 988, 1948. 17. Potts, W. J. and Riker, W. L.: Surgical Treatment of Pulmonary Stenosis with Intact Interventricular Septum. Arch. Surg. 62: 776, 1951. 18. Muller, W. H. and Longmire, W. P.: The Surgical Treatment of Pure Pulmonic Stenosis. A Description of an Expansible Valvulotome. Surgery 30: 275, 1951. 19. Blalock, A. and Kieffer, R. F., Jr.: Valvulotomy for the Relief of Congenital Valvular Pulmonic Stenosis with Intact Ventricular Septum: Report of Nineteen Operations by the Brock Method. Ann. Surg. 132: 496, 1950. 20. Hanlon, C. R. and Blalock, A.: Complete Transposition of the Aorta and Pulmonary Artery. Experimental Observations on Venous Shunts as Corrective Procedures. Ann. Surg. 127: 385, 1948. 21. Blalock, A. and Hanlon, C. R.: Interatrial Septal Defect. Its Experimental Production under Direct Vision without Interruption of the Circulation. Surg., Gynec. & Obst. 87: 183, 1948. 22. Taussig, H. B. and Bing, R. J.: Complete Transposition of the Aorta and a Levoposition of the Pulmonary Artery. Am. Heart J. 37: 55, 1949.