Early and Late Results of Surgical Repair of Truncus Arteriosus CARLO MARCELLETTI, M.D., DWIGHT C. MCGOON, M.D., GORDON K. DANIELSON, M.D., ROBERT B. WALLACE, M.D., AND DOUGLAS D. MAIR, M.D. tom-free, with all but two of the remainder (38%) being in NYHA functional class II. The late result is suggestively less satisfactory in patients with significant preoperative truncal valve regurgitation. Some late complications related to deterioration of the earlier aortic homograft conduit may be avoided by use of a porcine valve Dacron conduit. The current operative mortality of 9% (last 33 operations), the overall late mortality of 9%, and the well-being of 97% of surviving patients suggest the continued advisability of recommending operation for appropriate patients.

SUMMARY Ninety-two patients had corrective operation for truncus arteriosus between 1%7 and 1975. During the first 30 days

after surgery, 23 patients died. No significant differences appear between early mortality and sex, type of truncus, variations in pulmonary arterial anatomy, truncal valve regurgitation, associated anomalies, previous operation, or duration of extracorporeal circulation. Greater risk is probably encountered with the higher but still operable levels of pulmonary resistance. Reoperation has been required in three patients. Fifty-nine percent of survivors are symp-

TRUNCUS ARTERIOSUS is an

uncommon

the pulmonary artery (or arteries) from the truncus and closure of the resulting truncal defect; 2) longitudinal right ventriculotomy and patch closure of the ventricular septal defect; and 3) establishment of continuity between the right ventricle- and the pulmonary artery. In the initial group of 59 patients, a homograft of ascending aorta, aortic valve, and anterior leaflet of the mitral valve was used to establish such continuity; since November 1972 a Dacron conduit hearing a porcine semilunar valve, prepared and preserved in glutaraldehyde,* has been inserted between the right ventricle and the pulmonary artery in 33 consecutive patients. Truncal valve regurgitation was sufficiently severe to require intraoperative attention in 23 cases. This was managed by temporary approximation of cusps in 13, replacement of the truncal valve in six, repair of the valve in two, coronary perfusion in one, and maintenance of competence by digital

lesion. It ac-

counts for 0.4 to 3.9% of autopsy-proven cases of congenital

cardiac defects." 2 It is characterized' by a single, large arterial trunk originating from the base of the heart directly superior to a high ventricular septal defect; the pulmonary arteries originate from the trunk. This defect is now correctable by closing the ventricular septal defect and inserting a conduit between the right ventricle and the pulmonary arteries.6-

The late results of this operation have not been reported. This is a study of the complete Mayo Clinic experience with surgical repair of truncus arteriosus.

Clinical Material From September 1967 through January 1975, 92 patients underwent corrective surgery for truncus arteriosus at the Mayo Clinic. Fifty-six patients were males and 36 were females, ranging in age at the time of operation from 14 months to 21 years (median 7.3 years). The anatomic findings of the truncus arteriosus complex at the time of surgery and their relative incidence are summarized in table 1. According to the classification recently proposed by Edwards and McGoon12 for anomalies in which the pulmonary arterial supply has no direct origin from the heart, conditions previously termed truncus arteriosus type IV are not included in this series since no true pulmonary arteries are present,'2, Is and the more appropriate name is absence or atresia of the pulmonary arteries. We reviewed the operative reports in the two patients we had previously classified as truncus arteriosus type III and found that, in both, the pulmonary arteries originate from the posterolateral aspect of the ascending aorta. Therefore, we have reclassified these two patients as truncus arteriosus type II.

pressure in one.14

Results Early Results

Twenty-three patients died within 30 days of operation (25%). The postoperative complications and causes of early death are detailed in tables 2 and 3, respectively. No statistically significant relationship exists between the risk of early mortality and the sex of the patient (male, 21% mortality and female, 31%), the anatomic type of truncus arteriosus (25% in type I and 24% in type II), the anatomy of pulmonary blood supply, the presence of truncal valve regurgitation, the presence of associated cardiovascular anomalies, previous operation for palliation or exploration (30 cases), or even the duration of extracorporeal circulation. A positive correlation (P < 0.01) exists between the age of the patient at the time of surgical repair and the early mortality, which was 83% in patients under two years of age and 21% in patients over two years of age (table 4). None of the above factors was found predominantly in the earlier or more recent periods of this experience, with the exception of age; most patients less than two years of age were encountered in the earlier period. The preoperative systemic arterial oxygen saturation

Operative Technique

The basic steps of the operative repair as originally described64 remain essentially unchanged: 1) separation of From the Mayo Clinic and Mayo Foundation, Rochester, Minnesota. Presented at the American Heart Association Scientific Sessions, November 1975. Address for reprints: Dr. C. Marcelletti, c/o Section of Publications, Mayo Clinic, 200 First Street SW, Rochester, MN 55901. Received August 20, 1976; revision accepted November 26, 1976.

*Supplied by Hancock Laboratories, Inc. 636

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SURGICAL REPAIR OF TRUNCUS ARTERIOSUS/Marcelletti

TABLE 1. The Anatomy of the Truncus Arteriosus Complex No.

Truncus arteriosus Type I 67 Type II 25 Aortic arch Left 61 Right 28 3 Unstated Pulmonary arteries Normal diameter 46 3 Bilateral hypoplasia 4 RPA hypoplasia 13 Bilateral stenosis due to banding 14 RPA stenosis (band or natural) 3 LPA stenosis (band or natural) 2 Absence of RPA 6 Absence of LPA 1 LPA originating from PDA Truncal valve 10 Bicuspid 59 Tricuspid 8 Quadricuspid 1 Pentacuspid 4 Undetermined Truncal valve regurgitation 28 Mild (no intraoperative treatment) Mild to severe (intraoperative treatment) 23 Ventricular septal defect 69 Not reaching tricuspid anulus 17 Reaching tricuspid anulus Not reaching tricuspid anulus with 1 muscular defect also 5 Not described Associated defects 8 ASD 2 PAPVC 5 PDA 3 Interrupted aortic arch

Percent

73 27 69 31

637

al.

TABLE 3. Causes of Early Surgical Death Low cardiac output Cardiac arrest before perfusion (anesthetic) Respiratory insufficiency Arrhythmia (ventricular tachycardia or fibrillation) Hemorrhage Heart block Cardiac arrest

10* 2 4 3 2

1 1

*Including four patients unable to be discontinued from bypass. 50 3 4 14

15 3 2 7 1 11 67

9 1

30 25

79 20 1 9 2 5 3

Abbreviations: RPA = right pulmonary artery; LPA = left pulmonary artery; PAPVC = partial anomalous pulmonary venous connection; PDA = patent ductus arteriosus; ASD = atrial septal defect.

provides an indication of the volume of pulmonary flow, and in the absence of pulmonary stenosis, banding, or arterial hypoplasia, it reflects the level of pulmonary resistance.15 In 55 patients of this type, there was no influence on the risk of operation sufficient to attain statistical significance (early mortality of 50% in patients with arterial saturation lower than 85% and 28% in patients with arterial saturation of 85% or higher). Tables 5 and 6 show an increasing mortality rate TABLE 2. Postoperative Complications Low output syndrome Arrhythmia Respiratory insufficiency (tracheostomy required in 15) Febrile course without identified infection Bleeding requiring reoperation CNS injury Coagulation deficit with thrombocytopenia Postcardiotomy syndrome Gastrointestinal bleeding Paralysis of left hemidiaphragm Wound infection

et

23 17

35* lot 5 2 1 1 1 1 1

*Defined as requiring endotracheal intubation 72 hours or more postoperatively. tEight of these patients had received a Dacron conduit bearing a porcine valve.

pulmonary resistance is increased over 8 units m2 or the ratio of pulmonary vascular resistance to systemic vascular resistance is above 0.60. The ratio of systolic right ventricular pressure to systolic left ventricular pressure (RV/LV) measured intraoperatively after repair had an opposite effect on the risk of operation than that expected (i.e., it was found that the lower the ratio, the higher the risk of operation) but did not reach statistical significance. A gratifying reduction in the risk of operation during the evolution of this experience (table 7) is emphasized by comparing the 9% risk in the recent series of 33 patients, in whom a valved Dacron conduit was used, to the 34% risk in the earlier group, in whom continuity between the right ventricle and the pulmonary artery (or arteries) had been established by means of an aortic homograft. as

Late Results

Follow-up information to May 1975 was obtained for all 69 survivors, either by re-examination at the Mayo Clinic or by response from the referring physician or the parents to a special questionnaire. The follow-up varied from four to 92 months after repair, with a median period of 36 months. Six patients have died 2-38 months after the operation, a late mortality rate of 9%. Details of these patients are given in table 8. The actuarial survival curve for the patients surviving the first postoperative month is shown in figure 1. Table 9 summarizes the late complications and their relative incidence. Three patients required reoperation (4%): the first for residual ventricular septal defect; the second for associated residual ventricular septal defect, incompetent homograft valve, and perforation of noncoronary cusp of the truncal valve, probably secondary to Candida sepsis; and the third for dehiscence of the proximal anastomosis of the homograft resulting in a large false aneurysm that compressed the graft. Closure of the residual ventricular septal defect was performed in the first two patients, with additional repair of the truncal valve cusp and replacement of the aortic homograft with a valved Dacron conduit in the second, and insertion of a new homograft from the right ventricle to the distal graft in the third patient. The clinical status of the 63 late survivors is as follows: 37 TABLE 4. Age at Time of Operation and Surgical Risk No. of cases

Age (yr)

12 Total

6 20 52 14 92

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Hospital deaths

5 83% 5 25%

10 19% 21% 3 21%J 23 25%

CIRCULATION

638 TABLE 5. Pulmonary Resistance and Surgical Risk* Rp before repair

(units M2)

No. of cases

58

13

Total

100

Hospital deaths

0%

0

14%to

Discussion Experimental animal work6', 17 followed by successful clinical application"' has proven that the surgical repair of truncus arteriosus can be achieved by repairing the ventricular septal defect and restoring anatomic continuity between the right ventricle and the pulmonary artery or arteries. The early and late risks of this type of operation are of such magnitude that the advisability of recommending repair must be considered carefully. This report attempts to identify factors that may relate to the early and late postoperative results. The surgical risk is apparently not influenced by the

Rp/Rs

Hospital deaths

No. of cases

0.6 0.7

4

1

*P

P

0.01.

)

18%*

54%*

(23) ---- Observed

60

patients are classified as being in functional class 1 (59%), 24 in class 11 (38%), one in class III (having overt signs of congestive heart failure), and one patient cannot be classified because he is confined to bed by central nervous system injury incurred at the time of operation. This last patient suffered a cardiac arrest before institution of cardiopulmonary bypass and again during closure of the sternotomy, with subsequent development of hyperthermia to 104°F and seizures, followed by cortical blindness. Twenty of the 63 survivors (32%) are receiving digitalis therapy. The late clinical status of the survivors in relation to the preoperative presence of truncal valve regurgitation is given in table 10. The results for those with truncal regurgitation sufficiently significant to require special intraoperative management are suggestively worse (P = 0.078) than for patients having no or only mild truncal valve regurgitation. Histologic evidence of microcalcification of the homograft tissue has been found on postmortem study as early as 56 days after the operation. Calcification of the homograft is roentgenologically evident in 35 patients (83% of those about whom data on this point are available) (fig. 2). Postoperative cardiac catheterization, at least six months after repair, has been performed at this institution or elsewhere in 13 patients. Since recatheterization was not done in a systematic manner, and was also often carried out among patients in whom residual defects were suspected, relevant observations with respect to the overall series of patients cannot be made.

Resistance/Systematic

Expected*

-----

'14

*Includes only patients having data available and the presence of both right and left pulmonary arteries. tP

80

6 20%J 11 39%t 17 24%

29 28 70

VOL 55, No 4, APRIL 1977

Re-

40 26..

20 nI

0

1

I 2

I

3

4

6

5

7

Years after surgery *Under Minnesota death rates of 1970

FIGURE 1. Actuarial survival curve for patients surviving first month after corrective surgery; observedfour-year survivorship (23 patients) is below expected (P < 0.01). Six deaths have been observed less than four years after surgery, when only 1.5 would be expected.

of associated congenital cardiovascular defect, by anatbmic alteration of the pulmonary arteries, by the occurrence of previous operation, or by the duration of extracorporeal circulation. The presence of significant truncal valve regurgitation in 25% of our patients constitutes an increased technical difficulty during operation" but does not appear to be a substantial early risk factor. This study (table 10) suggests that significant uncorrected truncal valve regurgitation may detract from the late result. Furthermore, success with truncal valve replacement combined with complete repair, in our recent experience, gives encouragement for this approach whenever truncal regurgitation is judged to be more than mild to moderate. The ideal candidate for repair of truncus arteriosus appears to be a patient 5 to 12 years of age with normal or mildly elevated pulmonary resistance."8 As the degree of pulmonary vascular obstructive disease advances, the operative mortality and the probability of poor late results or late death are increased. It appears from our experience that a value for pulmonary resistance greater than 8 units m2 carries a significantly greater surgical risk, as previously reported,18 but the potential benefits of repair probably outpresence

TABLE 7. Year of Operation and Relation to Surgical Risk Year

1967

1968 1969

No. of cases

2 7 15

4 59*

7 1971 15 1972 (Jan-Oct) 13 1972 (Nov-Dec) 1 1973 9 1970

Hospital deaths

0 2 5

0

29 33

20 1 7 5 0 2

14 47 38 0 22

34%t

33 3 '9%t 1 20 5 3 0 *Patients in the top brackets in ch column received an sortie homograft; patients in the ottom brackets are the more recent group receiving 1974 1975

a Dacron conduit containing a porcine valve. One patient operated on at the end of 1972 received a Dacron conduit.

tP 42% 1 2 Repair of valve 40 1 0 Truncal valve replacement 3 8 2 1 *Not precisely class IV on a cardiac basis since confined to bed by injury of the central nervous system.

0 0 0 0 1 0

1*1

66%

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34%

0 0

0 0 0

58%

TRUNCUS WITH SINGLE PULMONARY ARTERY/Mair 14. De Leval MR, McGoon DC, Wallace RB, Danielson GK, Mair DD: Management of truncal valvular regurgitation. Ann Surg 180: 427, 1974 15. Mair DD, Ritter DG, Davis GD, Wallace RB, Danielson GK, McGoon DC: Selection of patients with truncus arteriosus for surgical correction: Anatomic and hemodynamic considerations. Circulation 49: 144, 1974 16. Arai T, Tsuzuki Y, Nogi M, Kurashige K, Koyanagi H, Nishida H, Ikeda Y, Ichikawa H: Experimental study on bypass between the right ventricle and pulmonary artery, left ventricle and pulmonary artery, and left ventricle and aorta by means of homograft with valve. Bull Heart Inst Japan 9: 49, 1965 17. Rastelli GC, Titus JL, McGoon DC: Homograft of ascending aorta and aortic valve as a right ventricular outflow: An experimental approach to the repair of truncus arteriosus. Arch Surg 95: 698, 1967 18. DuShane JW: Cited by DuShane JW, Kirklin JW: Late results of the repair of ventricular septal defect on pulmonary vascular disease. In Advances in Cardiovascular Surgery. Editedby JW Kirklin. New York, Grune & Stratton, 1973, pp 9-16 19. Parker RK, McGoon DC, Danielson GK, Wallace RB, and Mair DD: Repair of truncus arteriosus in patients with prior banding of the pulmonary artery. Surgery 78: 761, 1975 20. Gelband H, Van Meter S, Gersony WM: Truncal valve abnormalities in

21. 22.

23. 24. 25.

26. 27.

et

al.

641I

infants with persistent truncus arteriosus: A clinicopathologic study. Circulation 45: 397, 1972 McGoon DC, Wallace RB, Danielson GK: Homografts in reconstruction of congenital cardiac anomalies: Expanded operability in complex congenital heart disease. Mayo Clin Proc 47: 101, 1972 McGoon DC, Wallace RB, Danielson GK: The Rastelli operation: Its indications and results. J Thorac Cardiovasc Surg 65: 65, 1973 Brawley RK, Gardner TJ, Donahoo JS, Neill CA, Rowe RD, Gott VL: Late results after right ventricular outflow tract reconstruction with aortic root homografts. J Thorac Cardiovasc Surg 64: 314, 1972 Kaplan S, McKinivan CE, Helmsworth JA, Benzing G III, Schwartz DC, Schreiber JT: Complications following homograft replacement of the right ventricular outflow tract. Ann Thorac Surg 18: 250, 1974 Merin G, McGoon DC: Reoperation after insertion of aortic homograft as a right ventricular outflow tract. Ann Thorac Surg 16: 122, 1973 Moodie DS, Mair DD, Fulton RE, Wallace RB, Danielson GK, McGoon DC: Aortic homograft obstruction. J Thorac Cardiovasc Surg 72: 553, 1976 Bowman FO, Hancock WD, Malm JR: A valve-containing Dacron prosthesis: Its use in restoring pulmonary artery-right ventricular continuity. Arch Surg 107: 724, 1973

Truncus Arteriosus with Unilateral Absence of a Pulmonary Artery Criteria for Operability and Surgical Results DOUGLAS D. MAIR, M.D., DONALD G. RITTER, M.D., GORDON K. DANIELSON, M.D., ROBERT B. WALLACE, M.D., AND DWIGHT C. MCGOON, M.D. SUMMARY In 15 of 126 (12%) patients with truncus arteriosus who were catheterized at the Mayo Clinic from 1967 through 1975, natural agenesis of one pulmonary artery was present. Ten truncus patients with either natural or acquired absence of one pulmonary artery have undergone definitive operation. The criteria for operability, based on a calculation of pulmonary resistance, are different in patients with single pulmonary artery than in patients with two pulmonary arteries. Study revealed that, if the calculated pulmonary resistance in the patient with single pulmonary artery is halved, this new value provides a more reliable assessment of the reac-

tivity of the pulmonary arteriolar bed than does the total calculated pulmonary resistance value. Follow-up information suggests that the patient with single pulmonary artery may be at potentially high risk of continued progression of pulmonary vascular disease after surgical correction, possibly because of the postoperative obligatory increased flow through the single pulmonary arteriolar bed as a result of the entire cardiac output passing through it. Surgical correction of truncus arteriosus during infancy, before significant pulmonary vascular disease has developed, appears to be particularly desirable in this subgroup of patients with single pulmonary artery.

TETRALOGY OF FALLOT and persistent truncus arteriosus are the two congenital cardiovascular malformations most often associated with complete agenesis of one pulmonary artery. In tetralogy of Fallot, the pulmonary circuit is under low pressure and the risk of the development of pulmonary vascular obstructive disease is minimal. Therefore, assessment of suitability for operation, insofar as the status of the pulmonary resistance vessels is concerned, is not complicated in tetralogy by the additional feature of absence of one pulmonary artery. In truncus arteriosus, however, the pulmonary circuit is generally exposed to a pressure equal to that in the systemic circuit (the uncommon exception being the patient with stenosis at the pulmonary artery ostia), and as a result there is a tendency to develop

early severe pulmonary arteriolar changes. Thus, the hemodynamic assessment becomes critical in the selection of patients for corrective operation. The criteria for operability are different for truncus associated with single pulmonary artery than for truncus associated with two pulmonary arteries. We wish to report our experience regarding selection for surgery of patients with truncus arteriosus and associated unilateral absence of a pulmonary artery and the early and late results that have been achieved in such patients.

From the Mayo Clinic and Mayo Foundation, Rochester, Minnesota. Address for reprints: Douglas D. Mair, M.D., Mayo Clinic and Mayo Foundation, Rochester, Minnesota 55901. Received July 28, 1976; revision accepted November 4, 1976.

Material and Methods This report is concerned exclusively with types I and II truncus arteriosus as defined by Collett and Edwards,' conditions in which a single pulmonary artery arises or two pulmonary arteries arise from the truncus a short distance above the truncal valve. From August 1967 through

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Early and late results of surgical repair of truncus arteriosus. C Marcelletti, D C McGoon, G K Danielson, R B Wallace and D D Mair Circulation. 1977;55:636-641 doi: 10.1161/01.CIR.55.4.636 Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © 1977 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539

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Early and late results of surgical repair of truncus arteriosus.

Early and Late Results of Surgical Repair of Truncus Arteriosus CARLO MARCELLETTI, M.D., DWIGHT C. MCGOON, M.D., GORDON K. DANIELSON, M.D., ROBERT B...
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