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OPERATIVE CORRECTION OF VENTRICULAR SEPTAL DEFECT WITH ORIGIN OF BOTH GREAT VESSELS FROM THE RIGHT VENTRICLE
OPERATIVE CORRECTION OF VENTRICULAR SEPTAL DEFECT W I T H ORIGIN OF B O T H GREAT VESSELS FROM THE RIGHT VENTRICLE S. Frank Redo, M.D., Mary Allen Engle,
M.D.,
George R. Holswade, M.D., and Henry P. Goldberg, M.D., New York, N. Y.
O
of both great vessels from the right ventricle with a normally placed pulmonary artery and an associated subaortic ventricular septal defect is an entity that has only recently been recognized during life. The anomaly was considered rare, the few reported cases having been diagnosed at autopsy. Witham 1 in 1957 and Neufeld and his associates2 in 1961 divided these patients into two groups; those with and those without pulmonic stenosis. It is to the group with increased pulmonary blood flow that we wish to direct attention, for to the cardiologist and to the surgeon this syndrome masquerades as acvanotic ventricular septal defect.3 After unexpectedly encountering 2 such patients at open-heart surgery for closure of the ventricular septal defect, we discovered, in a review of autopsy specimens at The New York Hospital—Cornell Medical Center, 9 additional instances of this anomaly in children not subjected to operation. The diagnosis was made during life in 7 other patients who are currently being followed. One of these, diagnosed preoperatively, has been successfully operated upon. The pathologic findings and details of diagnostic methods, including angiocardiography, will be reported in a separate paper. 4 In general, this syndrome should be suspected in patients with a ventricular septal defect who have a systemic pressure in the right ventricle. As a rule, these patients do not show cyanosis, and most of the findings in them resemble those of a large ventricular septal defect with normal aortic origin. The purpose of this paper is to present 3 patients with this anomaly who were operated upon. In 2 instances, the entity was not recognized and both patients died when closure of the ventricular septal defect left no outlet for the left ventricle. In the third patient this anomaly was diagnosed preoperatively and confirmed at operation. The defect was successfully corrected. RIGIN
CASE REPORTS CASE 1.—J. H. was born on Oct. 9, 1956; the mother's pregnancy was uncomplicated. The i n f a n t ' s weight gain was poor, and, at the age of 6 weeks, she was admitted to The Prom the Departments of Surgery and Pediatrics. The New York Hospital—Cornell Medical Center, 525 East 68th Street, New York 21, N. Y. Received for publication June 19, 1962. 52fi
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New York Hospital in respiratory distress and congestive heart failure. She had a hoarse cry, retraction of the suprasternal notch and diaphragms, and a bulging, overactive precordium. No cyanosis was visible. Diagnosis of ventricular septal defect with a large leftto-right shunt was made on the basis of a holosystolic thrill and murmur maximal along the lower left sternal border, and on radiologie evidence of pulmonary overcireulation and enlargement of the main pulmonary artery, as well as of all four cardiac chambers. Electrocardiogram before digitalization showed a 90 degree electrical axis and evidence of combined left and right ventricular hypertrophy with inverted T waves over the left precordial and related limb leads. The patient responded to treatment for heart failure and was maintained on digitoxin therapy until operation. Although the second sound at the base was not heard initially, it became louder and was considered to be accentuated and not split. Other cardiac findings remained unchanged; physical growth was retarded. On Sept. 8, 1958, she was admitted for cardiac eatheterization in anticipation of operation for the ventricular septal defect. The results of this study (Table I ) supported that diagnosis.
TABLE I. CARDIAC CATHETERIZATION, CASE BLOOD OXYGEN CONTENT (VOL.
Femoral artery Bight pulmonary artery Main pulmonary artery Right ventricle (outflow tract) Bight ventricle (inflow tract) Eight atrium Superior vena cava Inferior vena cava
%)
14.1 13.3 13.3 13.4 12.4 10.7 10.5 10.7
1
1
PRESSURES
(MM.
Femoral artery Pulmonary capillary Pulmonary artery Bight ventricle
Hg)
88/4 6 66/26 65/1
Bight atrium
-2
Arterial oxyhemoglobin saturation: 97.2% Pulmonary blood flow: 17.47 L./min./M.2 Systemic blood flow: 3.91 L./min./M.2 Pulmonary vascular resistance: 312 dynes/sec./cm.- 5 Indocyanine green injection into the right ventricle and right pulmonary artery gave no indication of a right-to-left shunt
The increased oxygenation of blood a t the ventricular level was consistent with the diagnosis of ventricular septal defect with a large left-to-right shunt. Pressures in the pulmonary and femoral arteries were not measured simultaneously. On April 16, 1959, the patient underwent open-heart surgery with extracorporeal circulation with the use of a rotating disc-type oxygenator. The pulmonary artery was large and in the normal position. The origin of the aorta was more anterior than usual, although it was still behind and to the right of the pulmonary artery. This was interpreted as some degree of dextroposition of the aorta. Potassium citrate (2.5 per cent) was used to induce cardioplegia. Exploration after ventriculotomy revealed a high, posterior ventricular septal defect. The margins of the defect were approximated by direct suture. In addition, right atriotomy revealed a patent foramen ovale which was sutured closed. The patient survived operation and had satisfactory return of cardiac action. However, the pulse pressure was narrow and a thrill was palpable over the aorta. Her condition deteriorated rapidly, and she died in the recovery room 2 hours after completion of the operation. At postmortem examination, the true nature of the malformation became apparent only after the left ventricle was opened. I t was then seen that the aortic valve and the origin of the aorta were transposed to the inflow tract of the right ventricle, and t h a t the base of the left ventricle came to a blind end in the area of the septal defect, which was only partially closed (Fig. 1 ) . In the intervpntricular septum, just below the point where the crista supraventricularis joined the interventricular septum on the right and in the membranous
528
REDO E T AL.
J. Thoracic and Cardiovas. Surg.
Fig. 1.—Case 1, J. H. A, View of left ventricle. Note that septal defect Is only outlet from left ventricle. B, View of right ventricle, apex lifted. Sutures closing right ventriculotomy incision parallel cut surface of anterior wall of right ventricle. Outflow tract to pulmonary artery anteriorly is separated from that to aorta by a muscular ridge. Sutures partially close Inferior aspect of the channel to aortic valve. Musculature of right ventricle resembles that of left ventricle in thickness.
portion on the left, was a defect recently closed on the right side by sutures along a line 1.5 cm. in length. J u s t posterior to this suture line and to the right of the insertion of the crista supraventricularis was a channel about 1 cm. in diameter that connected the right ventricle with the aortic valve. The left coronary artery and its main branches were in normal position. The right coronary artery vertically traversed the middle of the wall of the right ventricle and was not in the atrioventricular groove. The left and right ventricles were hypertrophied. The tricuspid, mitral, and pulmonic valves were in normal anatomic position. The foramen ovale was closed by sutures.
Comment: The defect in this child was considered to be a ventricular septal defect complicated only by a large left-to-right shunt and pulmonary hypertension. Although dextroposition of the aorta was noted at operation, the true nature of the abnormality was not recognized. Closure of the defect was not complete, and therefore some means of egress of blood from the left ventricle remained. However, even partial closure of the defect led to marked obstruction of flow from left ventricle to aorta and led to death within a short time.
CASE 2.—J. V. was born on Oct. 9, 1956; the mother's pregnancy was uneventful. A heart murmur was noted a t birth, and a t the age of 2 months the infant was referred to The New York Hospital because of heavy breathing. She was tachypneic, without cyanosis or clubbing, and had an enlarged heart with a holosystolic thrill and murmur along the lower left sternal border. H e a r t sounds a t the base could not be heard initially. Fluoroscopy disclosed enlargement of all four cardiac chambers and of the main pulmonary artery. Pul-; monary arterial markings were engorged. The aorta was not dilated. The electrocardiogram showed no deviation of the electrical axis. Precordial leads indicated left ventricular hypertrophy and " s t r a i n , " as well as right ventricular hypertrophy. By the age of 5 months, the second sound in the second left intercostal space was loud and snapping, and there was a markedly bulging precordium. Poor feeding, slow weight
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gain, heavy breathing, and excess perspiration characterized the first 2 years. The clinical impression was of ventricular septal defect with excessive pulmonary blood flow and pulmonary hypertension. Cardiac Catheterization.—At the age of 2 years the child was admitted for cardiac catheterization. These studies confirmed the diagnosis (Table I I ) . TABLE I I . CARDIAC CATHETERIZATION, CASE 2 BLOOD OXYGEN CONTENT (VOL. % )
Brachial artery Pulmonary artery
14.4 12.6
Right ventricle (outflow tract) Right ventricle (inflow tract) Right atrium Superior vena cava Inferior vena cava
12.6 11.1 10.9 9.4 —
|
PRESSURES
(MM.
Brachial artery Pulmonary capillary Pulmonary artery Right ventricle
Hg)
— 3 70/15 70/-2
Right atrium
2
Arterial oxyhemoglobin saturation: 96.7% Left-to-right shunt — 49 per cent of pulmonary blood flow Injection of methylene blue into the pulmonary artery and right atrium confirmed the left-to-right shunt and gave no evidence of a right-toleft shunt Angiocardiography was performed because of a suggestion of, branch stenosis of the pulmonary artery. There was slight enlargement of the right atrium and ventricle, and more marked enlargement of the left atrium and ventricle. The large, convex pulmonary artery was in its normal position. A t the time of left heart filling, there was continued opacification of the pulmonary arterial system, indicating a left-to-right shunt. The aorta opacified only at the time of left ventricular filling. • On Nov. 19, 1959, the patient underwent closure of the ventricular septal defect by the open-heart technique with the use of a rotating disc-type oxygenator. When the right ventricle was opened, a large defect high in the membranous portion of the septum was identified. This extended down to the annulus of the tricuspid valve and up to the lower margin of the aortic valve cusps. The defect was closed with a series of interrupted sutures. Intermittent cross-clamping of the aorta was utilized to obtain a bloodless field. After the ventriculotomy wound was closed, attempts to remove the patient from the pump were unsuccessful, and she died on the operating table about 2 hours after completion of the procedure. Postmortem examination revealed complete dextroposition of the aortic valve which was located in the inflow tract of the right' ventricle. There was a ventricular septal defect 2 cm. in diameter high in the membranous portion of the septum. This had been the only outlet from the left ventricle. The ventricular septal defect had been only closed in its anterior aspect. Some of the sutures passed through the base of the aortic leaflet of the mitral valve and the upper portion of the muscular ventricular septum, with a resultant narrowing of the left ventricular outflow tract to 40 per cent of the original diameter (Fig. 2 ) . All valves were normal. The coronary arteries were of normal size and distribution. Both ventricles and atria were hypertrophied and dilated.
Comment: As in the preceding case, closure of the ventricular septal defect led to obstruction of left ventricular outflow. The degree of obstruction that resulted in this instance was of such magnitude that sufficient output could not be obtained, and it was impossible to remove the patient from the pump. In both cases, the basic pathology was not understood either before or during operation. Although the condition was considered rare, it was evident that a means of recognizing it before operation should be sought, and a different operative
530
EEDO ET AL.
J. Thoracic and Cardiovas. Surg.
Fig. 2.—Case 2, J. V. A, Anterosuperior view of heart with opening in outflow tract of right ventricle extending into pulmonary artery and paralleling right ventriculotomy incision. Pulmonary artery is in normal position and aorta is posterior and medial to it. B, Left ventricle, septal aspect. Probe passes through septal defect, which has been partially closed by sutures, thus obstructing the outlet by which left ventricular blood reaches the aorta. C, Interior of right ventricle. Probe extends behind septal leaflet of tricuspid valve into aorta. Muscular band separates aorta and pulmonary artery. Note hypertrophy of right ventricular musculature.
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technique of correcting it be devised. Review of the pathologic anatomy in these 2 acyanotic patients Avith ventricular septal defect and pulmonary hypertension indicated that the clue to diagnosis lay in demonstrating, by lateral or left anterior oblique x-ray projections, that the aorta originated anterior rather than posterior to the plane of the ventricular septum. Review of the angiocardiograms on Case 2 revealed what had previously been overlooked, that when the left heart opacified in the left anterior oblique projection, the aortic valve was clearly visualized anterior to the plane of the ventricular septum and above the right ventricle. 3 This observation led to a search for aortic transposition in other patients with a ventricular septal defect and systemic pressure in the right ventricle, and thus to preoperative diagnosis in Case 3. Meanwhile, postmortem specimens were reviewed and a surgical technique devised that would obliterate the left-to-right shunt by a hammock-like prosthesis, leaving the aorta in unobstructed continuity with the left ventricle. 3 CASE 3.—J. F . was 7 years old when first seen at The New York Hospital in 1958. A heart murmur had been discovered at age of one month. She had always been small in size and weight but had been asymptomatic except for slight fatigability. At the age of 6, she had undergone cardiac catheterization and angiocardiography at another institution, where a ventricular septal defect with a left-to-right shunt was diagnosed. Pressure in the pulmonary artery was as high as that in a systemic artery. She was a noncyanotic child with a left precordial bulge and a holosystolic thrill and a murmur characteristic of a ventricular septal defect. The second sound at the base on the left was accentuated. Phonocardiograms (Fig. 3) recorded a holosystolic murmur maximal in the fourth left intercostal space. Electrocardiograms demonstrated right ventricular hypertrophy. Fluoroscopy showed a large pulsating pulmonary artery and some enlargement of the right and left ventricles and left atrium. Two unusual features were a coin-shaped density in the region of the right or left atrium and an abnormal shadow, presumed to be vascular, at the right base. Because of these two shadows, angiocardiography was advised. Several months elapsed before the recommendation was accepted, and by this time radiographic survey revealed that the shadow at the right base had become larger and now contained flecks of calcium ( F i g . 4 ) . Skeletal survey, intravenous pyelogram, and bone marrow studies were normal. Angiocardiography revealed a large posterior mediastinal tumor t h a t did not opacify. The pulmonary arteries were large; the aorta was of average size, and it filled only at the time of left ventricular opacification. There w-as evidence of a left-to-right sliunt only. The coin-shaped density was caused by a common pulmonary vein seen ' ' end o n . ' ' On April, 22, 1959, the mediastinal tumor was resected and proved to be a neuroblastoma.
TABLE
III.
CARDIAC
BLOOD OXYGEN CONTENT (VOL.
Brachial artery Pulmonary artery Right ventricle (outflow tract) Right ventricle (inflow tract) Right atrium Superior vena cava
%)
18.4 15.4 15.6 14.5 13.6 13.1
CATHETERIZATION,
!
CASE
PRESSURES
Brachial artery Pulmonary artery Right ventricle
3 (MM.
Hg)
97/61 (mean 77) 57/34 (mean 45) 97/3
Right atrium -3 Superior vena cava —
Arterial oxyhemoglobin saturation: 9 3 . 1 % Systemic blood flow: 3.44 L./min. (3.62 L./min./M.2) Pulmonary blood flow: 5.49 L./min. (5.78 L./min./M.2) Left-to-right shunt: 2.05 L./min. Pulmonary vascular resistance: 597 dynes/sec./cm.-o
532
KEDO E T AL.
J. Thoracic and Cardiovas. Surg.
Fig. 3.—Case 3, J. F. Phonocardiogram from second left intercostal space and apex (A) and second right intercostal space and fourth left intercostal space (B) records holosystolic murmur with midsystolic accentuation, maximal at fourth left intercostal space. The second sound in second left intercostal space is split (0.04 second) and the pulmonic component is accentuated. After the skin sutures were removed, a course of radiation therapy to the tumor bed was begun. She received 3,400 r over a period of 4 weeks. After discharge there was no recurrence of the mediastiual lesion and no change in the cardiac findings. One year later, she was admitted for evaluation of the results of tumor therapy and for cardiac catheterization (Table I I I ) . Skeletal and chest x-rays showed no evidence of recurrence or metastases of the neuroblastoma, and cystoscopy and retrograde pyelography were unremarkable. Bone marrow studies were normal. These data were interpreted to indicate a ventricular septal defect with left-to-right shunt, pulmonary arterial hypertension, and infundibular pulmonic stenosis. The chief change from the findings 3 years earlier was the evidence of the development of infundibular pulmonic stenosis. Nonetheless, pulmonary hypertension was present. Because there was no sign of recurrent or metastastic neuroblastoma, surgical correction of the cardiac lesion was recommended. However, the father, the lone surviving parent, did not accept this recommendation until one year later, in May, 1961. Once more she underwent skeletal, chest, bone marrow, and kidney surveys. These failed to show evidence of neuroblastoma. From the cardiac standpoint her condition had not changed.
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Pig. 4.—Case 3. A, Roentgenogram made prior to removal of calcified mediastinal mass on right, a neuroblastoma. Coin-shaped density within atrlal shadow was shown on angiocardiogram to be pulmonary vein "end on." Main pulmonary artery is in usual position and is unusually prominent. B, Roentgenogram made prior to open-heart surgery .for ventricular septal defect, 2 years after operation for neuroblastoma.
Pig. 5.—Case 3. Angiocardiogram in left lateral position. Angiocardiogram is shown to the left and tracing on the right. Posterior to plane of ventricular septum, left ventricle is opacined. At upper aspect of septum, contrast medium spills through defect into right ventricle and into aorta. Aortic valves are seen anterior to plane of septum, above right ventricle.
534
REDO E T AL.
J. Thoracic and Cardiovas. Surg.
The angiocardiograms made 2 years earlier were reviewed to determine the origin of the aorta in this noneyanotic patient with a right ventricular pressure at systemic levels. The aorta opacified only at the time of left ventricular filling. I n frontal projection, it appeared normal, but in the left anterior oblique projection, the aortic valve and ascending aorta were seen anterior to the plane of the ventricular septum, arising from the right ventricle (Fig. 5 ) . Transposition of the aorta with subaortic ventricular septal defect was diagnosed. Appropriate surgical correction was planned^ through a ventriculotomy or atriotomy. On May 9, 1961, she underwent correction of the ventricular septal defect. Extracorporeal circulation was maintained by a rotating disc-type oxygenator and pump. A bilateral inframammary incision was used. Determinations performed on the operating table prior to caval cannulation revealed the same systolic pressure in the right ventricle and aorta and a lower pressure in the pulmonary artery (see Table I V ) . The right side of the heart was enlarged. The aorta arose on the same coronal and cross-sectional plane as did the pulmonary artery. I t was to the right of the pulmonary artery. There was a large right coronary artery that passed directly across the right ventricle in such a fashion as to preclude the usual right ventriculotomy approach to the ventricular septal defect. Following this inspection, soft, thin rubber tubes were placed about the pulmonary artery and the aorta. Linen tapes were placed about the superior and inferior venae cavae. Suitable catheters were introduced into the femoral artery bilaterally for inflow of oxygenated blood. Caval catheters were inserted through two openings in the right atrial appendage and secured with purse-string sutures of heavy braided silk. Perfusion was begun. When a perfusion rate of 100 ml. per kilogram of body weight was attained, the tapes on the inferior and superior venae cavae were tightened. Because of the anomalous right coronary artery, the ventricular septal defect was explored through the right atrium employing a large incision. The septal leaflet of the tricuspid valve was retracted, revealing a 2 cm. septal defect high and posteriorly placed in the membranous portion. Both the aorta and the pulmonary artery arose from the right ventricle. The left ventricle was in continuity with the aorta only through the septal defect (Fig. 6). At this point the aorta was cross-clamped to provide a dryer field. Interrupted sutures of 3-0 silk were placed about the rim of the defect infcriorly, anteriorly, and posteriorly, care being taken to place them 2 mm. from the actual margin of the defect to avoid injuring the bundle of His. Superiorly, sutures were placed in the tissue forming the margin of the aortic origin. After these had been placed, an Ivalon patcli (compressed 10 to 1) was cut in such a way as to form a hammock or elbow to provide a conduit for left ventricular blood to reach the aorta while obliterating the interventricular shunt of blood. The sutures that had been previously laid in the heart were passed through the margins of the Ivalon patch. When this was completed, the patch was pushed down to the rim of the defect and the sutures were tied (Fig. 6 ) . During this portion of the procedure, the aorta had been intermittently cross-clamped for periods of 10 to 12 minutes with undamped intervals lasting 2 to 5 minutes. Following final placement of the prosthesis, the aortic clamp was permanently released. No red blood appeared to be leaking into the right ventricle, and it was believed that good closure of the septal defect had been obtained. The atriotomy was then closed with two continuous sutures of 2-0 silk, one starting at each end, and both were tied in the middle after all air had been evacuated from the right atrium. Perfusion was then stopped after a total cardiac bypass time of 61 minutes. The heart functioned well on its own without evidence of block. The aorta appeared to have an adequate volume without evidence of obstruction to flow. With the caval catheters still in place, determinations showed a decrease in right ventricular and pulmonary arterial pressures in comparison with those in the left ventricle and aorta (Table I V ) . Oxygen saturation of blood from the right heart was measured by oximetry and was venous; aortic saturation was 100 per cent. On the basis of these studies, it was thought that the ventricular septal defect had been well closed and that the aortic orifice had not been compromised. Caval and femoral artery catheters were removed and the wound was closed. The chest was drained bilaterally with No. 22 catheters brought out through the sixth inter-
Vol.45, No. 4 April, 1963
CORRECTION O F V E N T R I C U L A R S E P T A L D E F E C T TABLE IV.
BEFORE (MM. Hg) Aorta Left ventricle Right ventricle Pulmonary artery
535 ^ua
PRESSURES I N OPERATING ROOM
| 80/50 80/0 80/0 60/0
AFTER (MM. Hg) Aorta Left ventricle Right ventricle Pulmonary artery
85/50 85/0 50/0 32/17
costal space. Pericostal sutures of chromic catgut were used to reapproximate the intercostal spaces. The divided sternum was approximated with the use of No. 25 stainless steel wires. Dry sterile dressings were applied and the patient was taken to the recovery room, having withstood the procedure well. Postoperatively, the patient did well except for daily temperature elevations to 38° C. rectally for the first 2 weeks. Careful examination failed to reveal any cause for this. She received antibiotics prophylactically (aqueous penicillin 1,200,000 units once daily; novobiocin 125 mg. every 6 hours) for the first week. On the fourteenth postoperative day the hemoglobin fell to 8.3 Gm. and continued to fall for the next 3 days, dropping to a low level of 6.8 Gm. Prior to open-heart surgery, this child was known to have been previously immunized against the Kell factor. Blood compatability studies at this time revealed immunization to C as well. I t was believed that she had a hemolytic anemia on this basis. A transfusion of 200 c.c. of compatible packed cells was given on the fourteenth postoperative day.
Fig. 6.—Case 3. Artist's sketch of operative findings. In cut-away of right ventricle (A), arrow indicates flow of blood from left ventricle through defect into aorta. Hammockshaped prosthesis is shown alone ( £ ) , and sutured into place (C). Diagram (D) in left anterior oblique position shows manner in which prosthesis interrupts interventricular shunt while left ventricular outflow into aorta is maintained.
REDO E T AL.
J. Thoracic and Cardiovas. Surg.
Pig. 7.—Case 3. Postoperative phonocardiogram, recorded as in Fig. 3. Note decrease in intensity and change in quality of systolic murmur. Early systolic ejection sound is now present.
Following this she became afebrile and the hemoglobin level stabilized at 9.5 Gm. for the remainder of her hospital stay. At no time postoperatively did she have any cardiac difficulties. She was discharged well 32 days after operation. Since discharge she has been followed in the cardiac clinic. She is on full activity without symptoms or medication. The hemoglobin is 12.3 Gm. There is no evidence of recurrence of the neuroblastoma. A soft systolic murmur, different from the preoperative one, is heard along the left sternal border but there is no thrill. The second sound on the left remains accentuated (Fig. 7). There is no cyanosis, peripheral edema, or evidence of pulmonary congestion, and the heart size is not enlarged. She has gained 3 kilograms in weight in the first 9 months since discharge, and her condition is apparently much improved over what it had been preoperatively.
Comment: Having had experience with the preceding 2 cases and after careful review of the pathologic conditions demonstrated by the additional 9 autopsy cases, this patient was operated upon with the surgeons both forewarned and forearmed. With the information thus obtained, operation was carried out
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"°'
in a pre-planned manner. The prosthesis used to correct the ventricular septal defect was fashioned and sutured into place in such a way as to form an elbowshaped conduit, allowing blood to pass through the septal defect into the aorta while still closing off the defect from the right ventricle. DISCUSSION
Although ventricular septal defect with origin of both great vessels from the right ventricle is an entity only recently recognized, it apparently is not so rare as previously supposed. Furthermore, it is important to make the correct diagnosis if operation is planned. The third case illustrates that diagnosis and surgical correction during life can be accomplished. The diagnosis should be suspected in those patients in whom the right ventricular systolic pressure approximates that in the aorta and the left ventricle. Failure to obtain these measurements simultaneously (as in Case 1) or to obtain a systemic artery pressure (as in Case 2) may lead to a missed diagnosis. Diagnosis can then be more strongly suggested or indeed established by using angiocardiographic techniques, as in Cases 2 and 3. Surgical correction is possible, utilizing extracorporeal circulatory methods. Failure to understand the nature of the lesion may lead to improper closure of the ventricular septal defect and resultant failure to provide an exit for blood from the left ventricle. The use of an elbow or hammock-shaped prosthesis sutured to the inferior margins of the defect and to the right border of the root of the aorta successfully provides an outlet for blood in the left ventricle and at the same time obliterates the interventricular shunting of blood. Thus, communication between the left and right ventricles is interrupted, but left ventricular and aortic continuity is maintained. Shortly after the operation on Case 3, McGoon5 reported a similar case in which the aortic transposition was recognized at operation when the aorta appeared to arise more anteriorly than usual. A patch was placed in a manner similar to that described in Case 3. The patient did well. SUMMARY
Three cases are reported of aortic origin from the right ventricle with a normally placed pulmonary artery and subaortic ventricular septal defect. Clinically the findings were those of ventricular septal defect and a large leftto-right shunt. Systemic oxygen saturation was normal. Diagnosis was not suspected in the first 2 cases preoperatively or at operation. The patients died after the margins of the defect were sutured together due to obstruction of left ventricular outflow. Preoperative diagnosis in the third patient led to operative repair which successfully eliminated the interventricular shunt and left the aortic flow unobstructed. Cardiac catheterization was performed in the Cardio-Pulmonary Laboratory under the direction of Dr. Daniel 8. Lukas. Angiocardiograms were performed by Dr. Israel Steinberg. We are indebted to Dr. Wallace G. Campbell for the- postmortem data and photographs.
538
REDO E T AL.
J. Thoracic and
Cardiovas. Surg.
ADDENDUM
Twenty months postoperative, on Oct. 8, 1962, the patient again underwent cardiac catheterization which demonstrated a decrease in pressures in the pulmonary artery and right ventricle and obliteration of the shunt.
BLOOD OXYGEN CONTENT (VOL. % )
Brachial artery Pulmonary artery Bight ventricle (outflow tract) Eight ventricle (inflow tract) Right atrium Superior vena cava Inferior vena cava
19.5 14.7 14.6 14.6 14.1 13.9 14.2
PRESSURES (MM. H g )
Brachial artery Pulmonary artery
Right ventricle (inf undibulum) Right ventricle Right atrium Arterial oxyhemoglobin saturation: 96.6%
112/70 (mean 88) 43/13 (mean 38) 43/0 55/3 2
REFERENCES
1. Witham, A. C.: Double Outlet Right Ventricle: A Partial Transposition Complex, Am. Heart J . 5 3 : 928-939, 1957. 2. Neuf eld, H. N., DuShane, J . W., Wood, E. H., Kirklin, J . W., and Edwards, J . E . : Origin of Both Great Vessels From Right Ventricle. I. Without Pulmonary Stenosis, Circulation 2 3 : 399-412, 1961. 3. Engle, M. A., Holswade, G. R., Campbell, W. G., and Goldberg, H. P . : Ventricular Septal Defect With Transposition of Aorta Masquerading as Acyanotic Ventricular Septal Defect. (Abstracts of 33rd Scientific Sessions of American Heart Association), Circulation 2 2 : 745, 1960. 4. Engle, M. A., Steinberg, I., Campbell, W. G., and Goldberg, H. P . : In preparation. 5. McGoon, D. C.: Origin of Both Great Vessels From the Right Ventricle, Surg. Clin. North America 4 1 : 1113-1120, 1961.
M.D.,
George R. Holswade, M.D., and Henry P. Goldberg, M.D., New York, N. Y.
O
of both great vessels from the right ventricle with a normally placed pulmonary artery and an associated subaortic ventricular septal defect is an entity that has only recently been recognized during life. The anomaly was considered rare, the few reported cases having been diagnosed at autopsy. Witham 1 in 1957 and Neufeld and his associates2 in 1961 divided these patients into two groups; those with and those without pulmonic stenosis. It is to the group with increased pulmonary blood flow that we wish to direct attention, for to the cardiologist and to the surgeon this syndrome masquerades as acvanotic ventricular septal defect.3 After unexpectedly encountering 2 such patients at open-heart surgery for closure of the ventricular septal defect, we discovered, in a review of autopsy specimens at The New York Hospital—Cornell Medical Center, 9 additional instances of this anomaly in children not subjected to operation. The diagnosis was made during life in 7 other patients who are currently being followed. One of these, diagnosed preoperatively, has been successfully operated upon. The pathologic findings and details of diagnostic methods, including angiocardiography, will be reported in a separate paper. 4 In general, this syndrome should be suspected in patients with a ventricular septal defect who have a systemic pressure in the right ventricle. As a rule, these patients do not show cyanosis, and most of the findings in them resemble those of a large ventricular septal defect with normal aortic origin. The purpose of this paper is to present 3 patients with this anomaly who were operated upon. In 2 instances, the entity was not recognized and both patients died when closure of the ventricular septal defect left no outlet for the left ventricle. In the third patient this anomaly was diagnosed preoperatively and confirmed at operation. The defect was successfully corrected. RIGIN
CASE REPORTS CASE 1.—J. H. was born on Oct. 9, 1956; the mother's pregnancy was uncomplicated. The i n f a n t ' s weight gain was poor, and, at the age of 6 weeks, she was admitted to The Prom the Departments of Surgery and Pediatrics. The New York Hospital—Cornell Medical Center, 525 East 68th Street, New York 21, N. Y. Received for publication June 19, 1962. 52fi
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New York Hospital in respiratory distress and congestive heart failure. She had a hoarse cry, retraction of the suprasternal notch and diaphragms, and a bulging, overactive precordium. No cyanosis was visible. Diagnosis of ventricular septal defect with a large leftto-right shunt was made on the basis of a holosystolic thrill and murmur maximal along the lower left sternal border, and on radiologie evidence of pulmonary overcireulation and enlargement of the main pulmonary artery, as well as of all four cardiac chambers. Electrocardiogram before digitalization showed a 90 degree electrical axis and evidence of combined left and right ventricular hypertrophy with inverted T waves over the left precordial and related limb leads. The patient responded to treatment for heart failure and was maintained on digitoxin therapy until operation. Although the second sound at the base was not heard initially, it became louder and was considered to be accentuated and not split. Other cardiac findings remained unchanged; physical growth was retarded. On Sept. 8, 1958, she was admitted for cardiac eatheterization in anticipation of operation for the ventricular septal defect. The results of this study (Table I ) supported that diagnosis.
TABLE I. CARDIAC CATHETERIZATION, CASE BLOOD OXYGEN CONTENT (VOL.
Femoral artery Bight pulmonary artery Main pulmonary artery Right ventricle (outflow tract) Bight ventricle (inflow tract) Eight atrium Superior vena cava Inferior vena cava
%)
14.1 13.3 13.3 13.4 12.4 10.7 10.5 10.7
1
1
PRESSURES
(MM.
Femoral artery Pulmonary capillary Pulmonary artery Bight ventricle
Hg)
88/4 6 66/26 65/1
Bight atrium
-2
Arterial oxyhemoglobin saturation: 97.2% Pulmonary blood flow: 17.47 L./min./M.2 Systemic blood flow: 3.91 L./min./M.2 Pulmonary vascular resistance: 312 dynes/sec./cm.- 5 Indocyanine green injection into the right ventricle and right pulmonary artery gave no indication of a right-to-left shunt
The increased oxygenation of blood a t the ventricular level was consistent with the diagnosis of ventricular septal defect with a large left-to-right shunt. Pressures in the pulmonary and femoral arteries were not measured simultaneously. On April 16, 1959, the patient underwent open-heart surgery with extracorporeal circulation with the use of a rotating disc-type oxygenator. The pulmonary artery was large and in the normal position. The origin of the aorta was more anterior than usual, although it was still behind and to the right of the pulmonary artery. This was interpreted as some degree of dextroposition of the aorta. Potassium citrate (2.5 per cent) was used to induce cardioplegia. Exploration after ventriculotomy revealed a high, posterior ventricular septal defect. The margins of the defect were approximated by direct suture. In addition, right atriotomy revealed a patent foramen ovale which was sutured closed. The patient survived operation and had satisfactory return of cardiac action. However, the pulse pressure was narrow and a thrill was palpable over the aorta. Her condition deteriorated rapidly, and she died in the recovery room 2 hours after completion of the operation. At postmortem examination, the true nature of the malformation became apparent only after the left ventricle was opened. I t was then seen that the aortic valve and the origin of the aorta were transposed to the inflow tract of the right ventricle, and t h a t the base of the left ventricle came to a blind end in the area of the septal defect, which was only partially closed (Fig. 1 ) . In the intervpntricular septum, just below the point where the crista supraventricularis joined the interventricular septum on the right and in the membranous
528
REDO E T AL.
J. Thoracic and Cardiovas. Surg.
Fig. 1.—Case 1, J. H. A, View of left ventricle. Note that septal defect Is only outlet from left ventricle. B, View of right ventricle, apex lifted. Sutures closing right ventriculotomy incision parallel cut surface of anterior wall of right ventricle. Outflow tract to pulmonary artery anteriorly is separated from that to aorta by a muscular ridge. Sutures partially close Inferior aspect of the channel to aortic valve. Musculature of right ventricle resembles that of left ventricle in thickness.
portion on the left, was a defect recently closed on the right side by sutures along a line 1.5 cm. in length. J u s t posterior to this suture line and to the right of the insertion of the crista supraventricularis was a channel about 1 cm. in diameter that connected the right ventricle with the aortic valve. The left coronary artery and its main branches were in normal position. The right coronary artery vertically traversed the middle of the wall of the right ventricle and was not in the atrioventricular groove. The left and right ventricles were hypertrophied. The tricuspid, mitral, and pulmonic valves were in normal anatomic position. The foramen ovale was closed by sutures.
Comment: The defect in this child was considered to be a ventricular septal defect complicated only by a large left-to-right shunt and pulmonary hypertension. Although dextroposition of the aorta was noted at operation, the true nature of the abnormality was not recognized. Closure of the defect was not complete, and therefore some means of egress of blood from the left ventricle remained. However, even partial closure of the defect led to marked obstruction of flow from left ventricle to aorta and led to death within a short time.
CASE 2.—J. V. was born on Oct. 9, 1956; the mother's pregnancy was uneventful. A heart murmur was noted a t birth, and a t the age of 2 months the infant was referred to The New York Hospital because of heavy breathing. She was tachypneic, without cyanosis or clubbing, and had an enlarged heart with a holosystolic thrill and murmur along the lower left sternal border. H e a r t sounds a t the base could not be heard initially. Fluoroscopy disclosed enlargement of all four cardiac chambers and of the main pulmonary artery. Pul-; monary arterial markings were engorged. The aorta was not dilated. The electrocardiogram showed no deviation of the electrical axis. Precordial leads indicated left ventricular hypertrophy and " s t r a i n , " as well as right ventricular hypertrophy. By the age of 5 months, the second sound in the second left intercostal space was loud and snapping, and there was a markedly bulging precordium. Poor feeding, slow weight
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CORRECTION OF VENTRICULAR SEPTAL DEFECT
^9Q
gain, heavy breathing, and excess perspiration characterized the first 2 years. The clinical impression was of ventricular septal defect with excessive pulmonary blood flow and pulmonary hypertension. Cardiac Catheterization.—At the age of 2 years the child was admitted for cardiac catheterization. These studies confirmed the diagnosis (Table I I ) . TABLE I I . CARDIAC CATHETERIZATION, CASE 2 BLOOD OXYGEN CONTENT (VOL. % )
Brachial artery Pulmonary artery
14.4 12.6
Right ventricle (outflow tract) Right ventricle (inflow tract) Right atrium Superior vena cava Inferior vena cava
12.6 11.1 10.9 9.4 —
|
PRESSURES
(MM.
Brachial artery Pulmonary capillary Pulmonary artery Right ventricle
Hg)
— 3 70/15 70/-2
Right atrium
2
Arterial oxyhemoglobin saturation: 96.7% Left-to-right shunt — 49 per cent of pulmonary blood flow Injection of methylene blue into the pulmonary artery and right atrium confirmed the left-to-right shunt and gave no evidence of a right-toleft shunt Angiocardiography was performed because of a suggestion of, branch stenosis of the pulmonary artery. There was slight enlargement of the right atrium and ventricle, and more marked enlargement of the left atrium and ventricle. The large, convex pulmonary artery was in its normal position. A t the time of left heart filling, there was continued opacification of the pulmonary arterial system, indicating a left-to-right shunt. The aorta opacified only at the time of left ventricular filling. • On Nov. 19, 1959, the patient underwent closure of the ventricular septal defect by the open-heart technique with the use of a rotating disc-type oxygenator. When the right ventricle was opened, a large defect high in the membranous portion of the septum was identified. This extended down to the annulus of the tricuspid valve and up to the lower margin of the aortic valve cusps. The defect was closed with a series of interrupted sutures. Intermittent cross-clamping of the aorta was utilized to obtain a bloodless field. After the ventriculotomy wound was closed, attempts to remove the patient from the pump were unsuccessful, and she died on the operating table about 2 hours after completion of the procedure. Postmortem examination revealed complete dextroposition of the aortic valve which was located in the inflow tract of the right' ventricle. There was a ventricular septal defect 2 cm. in diameter high in the membranous portion of the septum. This had been the only outlet from the left ventricle. The ventricular septal defect had been only closed in its anterior aspect. Some of the sutures passed through the base of the aortic leaflet of the mitral valve and the upper portion of the muscular ventricular septum, with a resultant narrowing of the left ventricular outflow tract to 40 per cent of the original diameter (Fig. 2 ) . All valves were normal. The coronary arteries were of normal size and distribution. Both ventricles and atria were hypertrophied and dilated.
Comment: As in the preceding case, closure of the ventricular septal defect led to obstruction of left ventricular outflow. The degree of obstruction that resulted in this instance was of such magnitude that sufficient output could not be obtained, and it was impossible to remove the patient from the pump. In both cases, the basic pathology was not understood either before or during operation. Although the condition was considered rare, it was evident that a means of recognizing it before operation should be sought, and a different operative
530
EEDO ET AL.
J. Thoracic and Cardiovas. Surg.
Fig. 2.—Case 2, J. V. A, Anterosuperior view of heart with opening in outflow tract of right ventricle extending into pulmonary artery and paralleling right ventriculotomy incision. Pulmonary artery is in normal position and aorta is posterior and medial to it. B, Left ventricle, septal aspect. Probe passes through septal defect, which has been partially closed by sutures, thus obstructing the outlet by which left ventricular blood reaches the aorta. C, Interior of right ventricle. Probe extends behind septal leaflet of tricuspid valve into aorta. Muscular band separates aorta and pulmonary artery. Note hypertrophy of right ventricular musculature.
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technique of correcting it be devised. Review of the pathologic anatomy in these 2 acyanotic patients Avith ventricular septal defect and pulmonary hypertension indicated that the clue to diagnosis lay in demonstrating, by lateral or left anterior oblique x-ray projections, that the aorta originated anterior rather than posterior to the plane of the ventricular septum. Review of the angiocardiograms on Case 2 revealed what had previously been overlooked, that when the left heart opacified in the left anterior oblique projection, the aortic valve was clearly visualized anterior to the plane of the ventricular septum and above the right ventricle. 3 This observation led to a search for aortic transposition in other patients with a ventricular septal defect and systemic pressure in the right ventricle, and thus to preoperative diagnosis in Case 3. Meanwhile, postmortem specimens were reviewed and a surgical technique devised that would obliterate the left-to-right shunt by a hammock-like prosthesis, leaving the aorta in unobstructed continuity with the left ventricle. 3 CASE 3.—J. F . was 7 years old when first seen at The New York Hospital in 1958. A heart murmur had been discovered at age of one month. She had always been small in size and weight but had been asymptomatic except for slight fatigability. At the age of 6, she had undergone cardiac catheterization and angiocardiography at another institution, where a ventricular septal defect with a left-to-right shunt was diagnosed. Pressure in the pulmonary artery was as high as that in a systemic artery. She was a noncyanotic child with a left precordial bulge and a holosystolic thrill and a murmur characteristic of a ventricular septal defect. The second sound at the base on the left was accentuated. Phonocardiograms (Fig. 3) recorded a holosystolic murmur maximal in the fourth left intercostal space. Electrocardiograms demonstrated right ventricular hypertrophy. Fluoroscopy showed a large pulsating pulmonary artery and some enlargement of the right and left ventricles and left atrium. Two unusual features were a coin-shaped density in the region of the right or left atrium and an abnormal shadow, presumed to be vascular, at the right base. Because of these two shadows, angiocardiography was advised. Several months elapsed before the recommendation was accepted, and by this time radiographic survey revealed that the shadow at the right base had become larger and now contained flecks of calcium ( F i g . 4 ) . Skeletal survey, intravenous pyelogram, and bone marrow studies were normal. Angiocardiography revealed a large posterior mediastinal tumor t h a t did not opacify. The pulmonary arteries were large; the aorta was of average size, and it filled only at the time of left ventricular opacification. There w-as evidence of a left-to-right sliunt only. The coin-shaped density was caused by a common pulmonary vein seen ' ' end o n . ' ' On April, 22, 1959, the mediastinal tumor was resected and proved to be a neuroblastoma.
TABLE
III.
CARDIAC
BLOOD OXYGEN CONTENT (VOL.
Brachial artery Pulmonary artery Right ventricle (outflow tract) Right ventricle (inflow tract) Right atrium Superior vena cava
%)
18.4 15.4 15.6 14.5 13.6 13.1
CATHETERIZATION,
!
CASE
PRESSURES
Brachial artery Pulmonary artery Right ventricle
3 (MM.
Hg)
97/61 (mean 77) 57/34 (mean 45) 97/3
Right atrium -3 Superior vena cava —
Arterial oxyhemoglobin saturation: 9 3 . 1 % Systemic blood flow: 3.44 L./min. (3.62 L./min./M.2) Pulmonary blood flow: 5.49 L./min. (5.78 L./min./M.2) Left-to-right shunt: 2.05 L./min. Pulmonary vascular resistance: 597 dynes/sec./cm.-o
532
KEDO E T AL.
J. Thoracic and Cardiovas. Surg.
Fig. 3.—Case 3, J. F. Phonocardiogram from second left intercostal space and apex (A) and second right intercostal space and fourth left intercostal space (B) records holosystolic murmur with midsystolic accentuation, maximal at fourth left intercostal space. The second sound in second left intercostal space is split (0.04 second) and the pulmonic component is accentuated. After the skin sutures were removed, a course of radiation therapy to the tumor bed was begun. She received 3,400 r over a period of 4 weeks. After discharge there was no recurrence of the mediastiual lesion and no change in the cardiac findings. One year later, she was admitted for evaluation of the results of tumor therapy and for cardiac catheterization (Table I I I ) . Skeletal and chest x-rays showed no evidence of recurrence or metastases of the neuroblastoma, and cystoscopy and retrograde pyelography were unremarkable. Bone marrow studies were normal. These data were interpreted to indicate a ventricular septal defect with left-to-right shunt, pulmonary arterial hypertension, and infundibular pulmonic stenosis. The chief change from the findings 3 years earlier was the evidence of the development of infundibular pulmonic stenosis. Nonetheless, pulmonary hypertension was present. Because there was no sign of recurrent or metastastic neuroblastoma, surgical correction of the cardiac lesion was recommended. However, the father, the lone surviving parent, did not accept this recommendation until one year later, in May, 1961. Once more she underwent skeletal, chest, bone marrow, and kidney surveys. These failed to show evidence of neuroblastoma. From the cardiac standpoint her condition had not changed.
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CORRECTION OF VENTRICULAR SEPTAL DEFECT
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Pig. 4.—Case 3. A, Roentgenogram made prior to removal of calcified mediastinal mass on right, a neuroblastoma. Coin-shaped density within atrlal shadow was shown on angiocardiogram to be pulmonary vein "end on." Main pulmonary artery is in usual position and is unusually prominent. B, Roentgenogram made prior to open-heart surgery .for ventricular septal defect, 2 years after operation for neuroblastoma.
Pig. 5.—Case 3. Angiocardiogram in left lateral position. Angiocardiogram is shown to the left and tracing on the right. Posterior to plane of ventricular septum, left ventricle is opacined. At upper aspect of septum, contrast medium spills through defect into right ventricle and into aorta. Aortic valves are seen anterior to plane of septum, above right ventricle.
534
REDO E T AL.
J. Thoracic and Cardiovas. Surg.
The angiocardiograms made 2 years earlier were reviewed to determine the origin of the aorta in this noneyanotic patient with a right ventricular pressure at systemic levels. The aorta opacified only at the time of left ventricular filling. I n frontal projection, it appeared normal, but in the left anterior oblique projection, the aortic valve and ascending aorta were seen anterior to the plane of the ventricular septum, arising from the right ventricle (Fig. 5 ) . Transposition of the aorta with subaortic ventricular septal defect was diagnosed. Appropriate surgical correction was planned^ through a ventriculotomy or atriotomy. On May 9, 1961, she underwent correction of the ventricular septal defect. Extracorporeal circulation was maintained by a rotating disc-type oxygenator and pump. A bilateral inframammary incision was used. Determinations performed on the operating table prior to caval cannulation revealed the same systolic pressure in the right ventricle and aorta and a lower pressure in the pulmonary artery (see Table I V ) . The right side of the heart was enlarged. The aorta arose on the same coronal and cross-sectional plane as did the pulmonary artery. I t was to the right of the pulmonary artery. There was a large right coronary artery that passed directly across the right ventricle in such a fashion as to preclude the usual right ventriculotomy approach to the ventricular septal defect. Following this inspection, soft, thin rubber tubes were placed about the pulmonary artery and the aorta. Linen tapes were placed about the superior and inferior venae cavae. Suitable catheters were introduced into the femoral artery bilaterally for inflow of oxygenated blood. Caval catheters were inserted through two openings in the right atrial appendage and secured with purse-string sutures of heavy braided silk. Perfusion was begun. When a perfusion rate of 100 ml. per kilogram of body weight was attained, the tapes on the inferior and superior venae cavae were tightened. Because of the anomalous right coronary artery, the ventricular septal defect was explored through the right atrium employing a large incision. The septal leaflet of the tricuspid valve was retracted, revealing a 2 cm. septal defect high and posteriorly placed in the membranous portion. Both the aorta and the pulmonary artery arose from the right ventricle. The left ventricle was in continuity with the aorta only through the septal defect (Fig. 6). At this point the aorta was cross-clamped to provide a dryer field. Interrupted sutures of 3-0 silk were placed about the rim of the defect infcriorly, anteriorly, and posteriorly, care being taken to place them 2 mm. from the actual margin of the defect to avoid injuring the bundle of His. Superiorly, sutures were placed in the tissue forming the margin of the aortic origin. After these had been placed, an Ivalon patcli (compressed 10 to 1) was cut in such a way as to form a hammock or elbow to provide a conduit for left ventricular blood to reach the aorta while obliterating the interventricular shunt of blood. The sutures that had been previously laid in the heart were passed through the margins of the Ivalon patch. When this was completed, the patch was pushed down to the rim of the defect and the sutures were tied (Fig. 6 ) . During this portion of the procedure, the aorta had been intermittently cross-clamped for periods of 10 to 12 minutes with undamped intervals lasting 2 to 5 minutes. Following final placement of the prosthesis, the aortic clamp was permanently released. No red blood appeared to be leaking into the right ventricle, and it was believed that good closure of the septal defect had been obtained. The atriotomy was then closed with two continuous sutures of 2-0 silk, one starting at each end, and both were tied in the middle after all air had been evacuated from the right atrium. Perfusion was then stopped after a total cardiac bypass time of 61 minutes. The heart functioned well on its own without evidence of block. The aorta appeared to have an adequate volume without evidence of obstruction to flow. With the caval catheters still in place, determinations showed a decrease in right ventricular and pulmonary arterial pressures in comparison with those in the left ventricle and aorta (Table I V ) . Oxygen saturation of blood from the right heart was measured by oximetry and was venous; aortic saturation was 100 per cent. On the basis of these studies, it was thought that the ventricular septal defect had been well closed and that the aortic orifice had not been compromised. Caval and femoral artery catheters were removed and the wound was closed. The chest was drained bilaterally with No. 22 catheters brought out through the sixth inter-
Vol.45, No. 4 April, 1963
CORRECTION O F V E N T R I C U L A R S E P T A L D E F E C T TABLE IV.
BEFORE (MM. Hg) Aorta Left ventricle Right ventricle Pulmonary artery
535 ^ua
PRESSURES I N OPERATING ROOM
| 80/50 80/0 80/0 60/0
AFTER (MM. Hg) Aorta Left ventricle Right ventricle Pulmonary artery
85/50 85/0 50/0 32/17
costal space. Pericostal sutures of chromic catgut were used to reapproximate the intercostal spaces. The divided sternum was approximated with the use of No. 25 stainless steel wires. Dry sterile dressings were applied and the patient was taken to the recovery room, having withstood the procedure well. Postoperatively, the patient did well except for daily temperature elevations to 38° C. rectally for the first 2 weeks. Careful examination failed to reveal any cause for this. She received antibiotics prophylactically (aqueous penicillin 1,200,000 units once daily; novobiocin 125 mg. every 6 hours) for the first week. On the fourteenth postoperative day the hemoglobin fell to 8.3 Gm. and continued to fall for the next 3 days, dropping to a low level of 6.8 Gm. Prior to open-heart surgery, this child was known to have been previously immunized against the Kell factor. Blood compatability studies at this time revealed immunization to C as well. I t was believed that she had a hemolytic anemia on this basis. A transfusion of 200 c.c. of compatible packed cells was given on the fourteenth postoperative day.
Fig. 6.—Case 3. Artist's sketch of operative findings. In cut-away of right ventricle (A), arrow indicates flow of blood from left ventricle through defect into aorta. Hammockshaped prosthesis is shown alone ( £ ) , and sutured into place (C). Diagram (D) in left anterior oblique position shows manner in which prosthesis interrupts interventricular shunt while left ventricular outflow into aorta is maintained.
REDO E T AL.
J. Thoracic and Cardiovas. Surg.
Pig. 7.—Case 3. Postoperative phonocardiogram, recorded as in Fig. 3. Note decrease in intensity and change in quality of systolic murmur. Early systolic ejection sound is now present.
Following this she became afebrile and the hemoglobin level stabilized at 9.5 Gm. for the remainder of her hospital stay. At no time postoperatively did she have any cardiac difficulties. She was discharged well 32 days after operation. Since discharge she has been followed in the cardiac clinic. She is on full activity without symptoms or medication. The hemoglobin is 12.3 Gm. There is no evidence of recurrence of the neuroblastoma. A soft systolic murmur, different from the preoperative one, is heard along the left sternal border but there is no thrill. The second sound on the left remains accentuated (Fig. 7). There is no cyanosis, peripheral edema, or evidence of pulmonary congestion, and the heart size is not enlarged. She has gained 3 kilograms in weight in the first 9 months since discharge, and her condition is apparently much improved over what it had been preoperatively.
Comment: Having had experience with the preceding 2 cases and after careful review of the pathologic conditions demonstrated by the additional 9 autopsy cases, this patient was operated upon with the surgeons both forewarned and forearmed. With the information thus obtained, operation was carried out
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April, 1963
CORRECTION OF VENTRICULAR S E P T A L D E F E C T
537
"°'
in a pre-planned manner. The prosthesis used to correct the ventricular septal defect was fashioned and sutured into place in such a way as to form an elbowshaped conduit, allowing blood to pass through the septal defect into the aorta while still closing off the defect from the right ventricle. DISCUSSION
Although ventricular septal defect with origin of both great vessels from the right ventricle is an entity only recently recognized, it apparently is not so rare as previously supposed. Furthermore, it is important to make the correct diagnosis if operation is planned. The third case illustrates that diagnosis and surgical correction during life can be accomplished. The diagnosis should be suspected in those patients in whom the right ventricular systolic pressure approximates that in the aorta and the left ventricle. Failure to obtain these measurements simultaneously (as in Case 1) or to obtain a systemic artery pressure (as in Case 2) may lead to a missed diagnosis. Diagnosis can then be more strongly suggested or indeed established by using angiocardiographic techniques, as in Cases 2 and 3. Surgical correction is possible, utilizing extracorporeal circulatory methods. Failure to understand the nature of the lesion may lead to improper closure of the ventricular septal defect and resultant failure to provide an exit for blood from the left ventricle. The use of an elbow or hammock-shaped prosthesis sutured to the inferior margins of the defect and to the right border of the root of the aorta successfully provides an outlet for blood in the left ventricle and at the same time obliterates the interventricular shunting of blood. Thus, communication between the left and right ventricles is interrupted, but left ventricular and aortic continuity is maintained. Shortly after the operation on Case 3, McGoon5 reported a similar case in which the aortic transposition was recognized at operation when the aorta appeared to arise more anteriorly than usual. A patch was placed in a manner similar to that described in Case 3. The patient did well. SUMMARY
Three cases are reported of aortic origin from the right ventricle with a normally placed pulmonary artery and subaortic ventricular septal defect. Clinically the findings were those of ventricular septal defect and a large leftto-right shunt. Systemic oxygen saturation was normal. Diagnosis was not suspected in the first 2 cases preoperatively or at operation. The patients died after the margins of the defect were sutured together due to obstruction of left ventricular outflow. Preoperative diagnosis in the third patient led to operative repair which successfully eliminated the interventricular shunt and left the aortic flow unobstructed. Cardiac catheterization was performed in the Cardio-Pulmonary Laboratory under the direction of Dr. Daniel 8. Lukas. Angiocardiograms were performed by Dr. Israel Steinberg. We are indebted to Dr. Wallace G. Campbell for the- postmortem data and photographs.
538
REDO E T AL.
J. Thoracic and
Cardiovas. Surg.
ADDENDUM
Twenty months postoperative, on Oct. 8, 1962, the patient again underwent cardiac catheterization which demonstrated a decrease in pressures in the pulmonary artery and right ventricle and obliteration of the shunt.
BLOOD OXYGEN CONTENT (VOL. % )
Brachial artery Pulmonary artery Bight ventricle (outflow tract) Eight ventricle (inflow tract) Right atrium Superior vena cava Inferior vena cava
19.5 14.7 14.6 14.6 14.1 13.9 14.2
PRESSURES (MM. H g )
Brachial artery Pulmonary artery
Right ventricle (inf undibulum) Right ventricle Right atrium Arterial oxyhemoglobin saturation: 96.6%
112/70 (mean 88) 43/13 (mean 38) 43/0 55/3 2
REFERENCES
1. Witham, A. C.: Double Outlet Right Ventricle: A Partial Transposition Complex, Am. Heart J . 5 3 : 928-939, 1957. 2. Neuf eld, H. N., DuShane, J . W., Wood, E. H., Kirklin, J . W., and Edwards, J . E . : Origin of Both Great Vessels From Right Ventricle. I. Without Pulmonary Stenosis, Circulation 2 3 : 399-412, 1961. 3. Engle, M. A., Holswade, G. R., Campbell, W. G., and Goldberg, H. P . : Ventricular Septal Defect With Transposition of Aorta Masquerading as Acyanotic Ventricular Septal Defect. (Abstracts of 33rd Scientific Sessions of American Heart Association), Circulation 2 2 : 745, 1960. 4. Engle, M. A., Steinberg, I., Campbell, W. G., and Goldberg, H. P . : In preparation. 5. McGoon, D. C.: Origin of Both Great Vessels From the Right Ventricle, Surg. Clin. North America 4 1 : 1113-1120, 1961.