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Tetralogy of Fallot with absent pulmonary valve
J THoRAc CARDIOVASC SURG 89:280-287, 1985
Tetralogy of Fallot with absent pulmonary valve Early and late results of surgical treatment From 1957 through September, 1983, 35 patients with tetralogy of Fallot and absent pulmonary valve mtderwent operation. Two subgroups of patients were recognized: minimally symptomatic (Group A, n = 21) and markedlysymptomatic (Group B, n = 14).Group B patients were symptomatic at an earlier age and were younger at operation.Ages ranged from 1 day to 42 years (mean = 7.8 ± 7.4 SD). Repair consisted of closure of the ventricular septal defect and relief of the right ventricle-pulmonary artery pressuregradient (nine patients received a tissuevalve), In five patients,partial resection and/or plication of the aneurysmalpulmonary arteries also was performed. Operative mortalitywas 4.8% in GroupA and 35.7 % in GroupB. In up to 25 years of fonow-up, there was onelate death in GroupA (an early case with complete heart block); the remaining 19 patients are asymptomatic. In Group B, there were four late deaths, three related to cardiopulmonary insufficiency. These results indicate pulmonary valve insufficiency is tolerated wen in minimally symptomatic patients. For markedly symptomatic patients, results may be improved if repair includes establishment of pulmonary valvular competence and reduction of size and length of aneurysmal pulmonary arteries.
B. C. McCaughan, M.B., B.S., Gordon K. Danielson, M.D., David J. Driscoll, M.D., and Dwight C. McGoon, M.D., Rochester, Minn.
h e syndrome of tetralogy of Fallot with absent pulmonary valve is characterized by a ventricular septal defect, overriding of the aorta, right ventricular hypertrophy, and pulmonary annular stenosis in association with absent or rudimentary pulmonary valve. Pulmonary regurgitation and aneurysmal dilatation of the pulmonary arteries uniformly are present. Symptoms vary from life-threatening respiratory obstruction or cardiac failure in neonates to absence of symptoms in patients who may live unrestricted lives for many years. As with uncomplicated tetralogy of Fallot, surgical repair has been demonstrated to be effective treatment. To assess the early and late results of operation, we reviewed all patients undergoing repair of this syndrome at the Mayo Clinic. From the Department of Thoracic and Cardiovascular Surgery and the Department of Pediatric Cardiology, Mayo Clinic, Rochester, Minn. Presented at the American Heart Association Fifty-sixth Scientific Sessions, Anaheim, California, Nov. 15, 1983. Received for publication Jan. 26, 1984. Accepted for publication March 12, 1984. Address for reprints: Gordon K. Danielson, M.D., Mayo Clinic/ Foundation, Rochester, Minn. 55905.
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Patients From 1957 through September, 1983, 35 patients with tetralogy of Fallot and absent pulmonary valve underwent repair at this institution. The first 18 patients have been reported previously.' There were 20 males and 15 females (male/female ratio = 1.3). Ages at operation ranged from 1 day to 42 years (mean = 7.8 ± 7.4 SD). There was a wide spectrum in the severity of symptoms. At one extreme was a critically ill l-day-old neonate with cyanosis, acidosis, and low cardiac output. At the other end of the spectrum was a 42-year-old woman whose murmur had been noted in childhood and whose only symptom was occasional palpitations from extrasystoles. The patients could be classified into two groups according to their clinical status:
Group A: Asymptomatic or minimally symptomatic-2l patients. These patients had been noted to have cyanosis, a cardiac murmur, or both, in the neonatal period. Growth and development were normal in infancy and childhood. Symptoms were absent in 10 patients and minimal (such as upper respiratory tract infection or fatigability on severe exertion) in 10 patients. One additional patient had frequent premature beats and runs of ventricular tachycardia. Ages at operation
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ranged from 3 to 42 years (mean = 10.2 ± 8.5 SD). Nine of the 21 patients were male (43%). Group B: Markedly symptomatic-14 patients. These patients had one or more of the following symptoms within the first 6 months of life: cardiac failure necessitating treatment (eight patients), marked developmental delay (nine patients), and episodes of respiratory obstruction, infection, or both, necessitating hospitalization (13 patients). Two patients had required tracheostomy during episodes of severe respiratory distress. Three patients had undergone lobectomy (one a right middle lobectomy, one a right middle and right lower lobectomy, and one a left upper lobectomy) prior to intracardiac repair because of lobar emphysema secondary to bronchial compression. The condition of all three had improved after lobectomy. The ages of the 14 patients at operation ranged from 1 day to 9 years (mean = 4.3 ± 3.1 SD). Eleven of the 14 patients were male (79%). Physical findings in all 35 patients were remarkably similar and consisted of an overactive heart and a right ventricular impulse. There was a systolic thrill associated with a harsh systolic murmur along the left upper sternal border. An early diastolic murmur, often accompanied by a thrill, was present at the left sternal border. These murmurs were characteristically "to-and-fro," but in some patients they were thought initially to be continuous. The second sound in the pulmonary area was absent in all cases. The electrocardiograms showed normal sinus rhythm in all 35 patients; three had associated first-degree atrioventricular block. Premature ventricular contractions were noted on three tracings. V leads were taken in 34patients; eight had right bundle branch block, 23 had rightventricular hypertrophy alone, one had left ventricular hypertrophy alone, and two had biventricular hypertrophy. Cardiomegaly was present in nearly all of the chest roentgenograms. Aneurysmal dilatation of the central pulmonary arteries and normal peripheral pulmonary vasculature were present bilaterally except in the seven patients with unilateral pulmonary artery anomalies (Table I). Ten patients (29%) had a right-sided aortic arch. Cardiac catheterization was performed prior to operation in all but one patient. The one exception was a 3-month-old infant who had critical obstruction of the left main bronchus that necessitated continuous semiprone positioning. The clinical diagnosis was confirmed by two-dimensional echocardiography. In Group A, all patients had a predominant left-to-right shunt; the mean Qp/Qs in 15 patients in whom it was measured was 1.8
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Table I. Associated cardiac anomalies (20 patients) Anomaly Right aortic arch Interatrial communication Ostium secundum defect Patent foramen ovale Absent LPA Origin LPA from ascending aorta Origin RP A from descending aorta Patent ductus arteriosus Bilateral superior venae cavae Double outlet right ventricle Multiple ventricular septal defects
Pt. No. 10 10 6 4 5 I I I 1 I
I
Legend: LPA, Left pulmonary artery. RPA, Right pulmonary artery.
(range = 1.1 to 3.2). The peak systolic right ventriclepulmonary artery pressure gradient ranged from 35 to 86 mm Hg (mean = 60) (N = 20), the peak pulmonary artery systolic pressure ranged from 18 to 40 mm Hg (mean = 29) (N = 20), and the systemic arterial oxygen saturation ranged from 90% to 96% (mean = 93%) (N = 18). Within Group B, the predominant shunt was left-to-right in five (mean Qp/Qs = 1.9), right-to-left in four (mean Qp/Qs = 0.5), and essentially balanced in four. The peak systolic right ventricle-pulmonary artery pressure gradient ranged from 28 to 75 mm Hg (mean = 52) (N = 13), the peak pulmonary artery systolic pressure ranged from 20 to 52 mm Hg (mean = 31) (N = 13), and the systemic arterial oxygen saturation ranged from 68% to 94% (mean = 86) (N = 12). Associated cardiac anomalies included absent left pulmonary artery in five patients and anomalous origin of a pulmonary artery from the aorta in two patients (Table I). Operative findings and procedures Right ventricular hypertrophy and pulmonary annular stenosis were present in all 35 patients. True pulmonary valvular leaflets were absent. Remnants of leaflet tissue were present in some cases as tags of tissue or a ridge at the anulus. The limbs of the crista were hypertrophied in 31 patients, but in only six were they thought to be causing significant obstruction. A paramembranous ventricular septal defect (sited high posteriorly in the ventricular septum adjacent to the septal leaflet of the tricuspid valve) was present in all patients. In one patient, there was a second, muscular, ventricular septal defect inferior to the first. Pulmonary arterial dilatation was a feature in all cases. In 24 patients, the main pulmonary artery and proximal portions of the right and left pulmonary
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Fig. 1. Diagram of operative technique. A, A longitudinal incision is made in the main pulmonary artery and extended proximally across the pulmonary anulus into the right ventricular outflow tract (inset). The ventricular septaldefectis patchedand a tissue-valved conduit is anastomosed to the distal pulmonary artery. B, The proximal conduitis fashioned with an anterior flap and anastomosed to the right ventricular outflow tract. Inset, Transverse plication sutures reduce the diameter of the aneurysmal right and left pulmonary arteries.
Table II. Repair of right ventricular outflow tract Repair
Pt, No.
Radial annular incisions Excision of rudimentary valve tissue only Valved conduit Transannular patch Valve with patch
11 10
Total
35
7 5 2
arteries were dilated markedly. In four patients, the main pulmonary artery was enlarged slightly, the right pulmonary artery was enlarged markedly, and the left pulmonary artery was normal in size. In six patients, the main pulmonary artery and right pulmonary artery were dilated markedly; in five of these the left pulmonary artery was absent and in the sixth it arose from the ascending aorta. In one patient, the main pulmonary artery and left pulmonary artery were dilated markedly and the right pulmonary artery arose from the descending thoracic aorta. Other associated cardiac findings are shown in Table I. The mitral, tricuspid, and aortic valves were normal in all cases.
Operation was performed via median sternotomy using cardiopulmonary bypass with moderate hypothermia. In our earlier experience, intermittent aortic crossclamping was employed. Since 1978, the use of cold potassium cardioplegic solution combined with topical application of cold saline has been preferred. Total circulatory arrest under profound hypothermia was utilized to facilitate repair in two infants. A right ventriculotomy was performed and the intracardiac anatomy was defined. In some patients, a second incision was made in the main pulmonary artery. Hypertrophied cristal bands were resected. The paramembranous ventricular septal defect was closed by direct suture in six patients (all prior to 1964) and by a patch technique in 29 patients. Any leaflet tissue remnants were resected. In 10 patients (six in Group A and four in Group B), this relieved the right ventriclepulmonary artery gradient and the pulmonary anulus was left intact. In 11 patients, radial incisions were made in the anulus to reduce the degree of pulmonary stenosis (six in Group A and five in Group B). In five patients (two in Group A, three in Group B), transannular patch enlargement of the outflow tract was performed (pericardium in four and a Teflon patch in one). In the
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Fig. 2. Operative technique, continued. A, An alternate and more effective way to reduce the diameter of the pulmonary arteriesis to resectredundantarterial wall, as shown. B, For additional reliefof bronchial compression, the pulmonary arteries are shortened by plicating them with the arterial repair. C, In this example, after patch closure of the ventricular septaldefect, a tissue valve has beensewn to the pulmonary anulusand the anterior wall of the reconstruction is made with a pericardial patch. remaining nine patients (seven in Group A and two in Group B), all of whom have been operated upon since 1973, a glutaraldehyde-preserved tissue valve was inserted between the right ventricle and the main pulmonary artery. In two of the nine patients, a tissue valvewas placed at the level of the anulus and the roof of the outflow tract was reconstructed with a pericardial patch; in the remaining seven, a tissue-valved conduit* was inserted (Fig. 1) (Table II). In five patients, aneurysmally dilated pulmonary arteries were plicated, partially resected, or both (four in Group A and one in Group B) (Figs. 1 and 2). In one patient, in whom the left pulmonary artery arose from the proximal aorta, the former was detached and reimplanted into the main pulmonary artery. In another patient, in whom the right pulmonary artery arose from the descending aorta, it was not technically feasible to reimplant the right pulmonary artery so its origin was narrowed with a partially occlusive ligature in order to reduce shunting. The interatrial communication was closed in all 10 patients by direct suture, and the patent ductus arteriosus in one patient was ligated. The double-outlet right ventricle was repaired in the course of closure of the "Hancock Laboratories, Anaheim, Calif.
ventricular septal defect. Intraoperative electrophysiological mapping in the patient who had multiple premature ventricular beats and episodes of ventricular tachycardia demonstrated a reentry point in the right ventricular outflow tract caudal to the pulmonary anulus. This portion of the outflow tract was excised, and the endocardium of the right ventricle downstream to the moderator band was electrocoagu1ated. The postrepair right ventricle-pulmonary artery gradients were reduced from preoperative values in all patients except one. The mean preoperative peak systolic pressure gradient was 58 ± 14 SD mm Hg (range = 28 to 86), and the mean postrepair gradient was 23 ± 13 SD mm Hg (range = 0 to 45) (Fig. 3).
Results Group A. Of the 21 patients who were asymptomatic or minimally symptomatic preoperatively, one died within 30 days of operation (4.8%). This death occurred in a 3-year-old girl who had a technically uncomplicated procedure (in 1967), but who developed progressive coma postoperatively. The cause of the cerebral damage was not revealed by autopsy examination. Two patients required exploration for excessive postoperative bleeding. One patient, operated upon early in the series, developed complete heart block immediately following
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100,....-------------,100
mmHg
Mean
50
o
Preoperative
0
Postoperative
Fig. 3. Preoperative and postoperative peak systolic pressure gradients between the right ventricle and pulmonary artery.
direct closure of the ventricular septal defect. This patient and two additional ones in Group B were described in our earlier report. I There have been no further cases of permanent complete heart block in the past 20 years at this institution after operations for this condition. No patient required prolonged respiratory support. At the closing date of the study, follow-up had been obtained on all 20 patients surviving operation; the follow-up intervals ranged from 1 month to 25 years (median = 10 years). There was one late death which occurred in the patient who had complete heart block. She had remained well for 31h years without a pacemaker but then died suddenly. One patient who developed congestive heart failure from a recurrent ventricular septal defect underwent successful reoperation 4 months after the initial procedure. All 19 late survivors are currently well without apparent physical limitations. Of the 12 patients who have pulmonary insufficiency, all remain well 2 to 25 years later (median = 15 years). Partial resection or plication of the aneurysmal arteries was not employed in any of these patients. The patient with recurrent ventricular tachycardia has remained asymptomatic without antiarrhythmic therapy. One of the seven patients who received a tissue-valved conduit was reoperated upon elsewhere 8 years after repair for replacement of the conduit with another tissue-valved conduit because of valvular calcification and stenosis. Group B. Of the 14 patients having marked symptoms during their first 6 months of life, five died within 30 days of operation (35.7%). The causes of death were heart block in one (prior to the availability of pacemakers), ventricular fibrillation in the immediate postopera-
tive period in one, and low cardiac output in two. One of the latter patients was the l-day-old infant who was operated upon as an emergency. She was hypoxic, cyanotic, and acidotic and had a cardiac arrest prior to operation. Repair was accomplished with a No. 12 mm porcine-valved conduit, but she could not be weaned from cardiopulmonary bypass. The fifth patient died suddenly, presumably from an arrhythmia, 2 weeks after hospital discharge (but within 30 days of operation). His electrocardiogram showed sinus rhythm and right bundle branch block only; there were no postoperative arrhythmias. Autopsy showed the repair to be intact. Right ventricular hypertrophy was present (wall thickness 1.3 em); the average left ventricle thickness was 0.9 em. Four patients required prolonged intensive respiratory care; tracheostomy was necessary in two. Ultimately, all four left the hospital free of respiratory symptoms. A recent case illustrates many of the features of the symptomatic infant. A 3-month-old boy required continuous semi-prone positioning, narcotic sedation, and bronchodilator therapy. Preoperative cardiac catheterization could not be performed because of the infant's clinical condition and marked deterioration when turned from the prone position. Two-dimensional echocardiography confirmed the clinical diagnosis. Repair consisted of patch closure of the ventricular septal defect, reduction of the size of the aneurysmal pulmonary arteries by partial resection and plication, and insertion of a valved conduit in the right ventricular outflow tract. Postoperatively, copious secretions were aspirated from the previously obstructed bronchi. Intensive respiratory care was necessary for 5 days, but the patient subsequently was discharged on postoperative day 12 free of respiratory symptoms. He has remained well and requires no medication. Follow-up was obtained on all nine patients surviving operation; the follow-up intervals ranged from 1 to 22 years (median = 12 years). One of the patients died 6 weeks after operation from complications of complete heart block. Three additional late deaths occurred, all 8 years postoperatively. At operation, the pulmonary anulus had been enlarged by radial incision in all three patients and in none was partial resection or plication of the dilated pulmonary arteries performed. All had episodic respiratory infections and congestive heart failure within 6 months after hospital discharge. Reoperation was performed for placement of a porcine tissue valve in the pulmonary position in two patients 2 and 4 years postoperatively, respectively.The third patient had worsening restrictive lung disease secondary to thoracic scoliosis. Episodic respiratory difficulties persisted in all
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three patients until sudden death occurred in each, 8 years after operation. Autopsy was performed in two; the surgical repairs were intact and no apparent cause for the sudden deaths was found. If one excludes the three patients whose deaths were related to permanent heart block (all operated upon prior to 1964), the combined early and late mortality is one of 20 (5.0%) in Group A and seven of 12 (58.3%) in Group B (p < 0.001). Excluding the l-day-old infant (Patient 12 in Group B) who had a cardiac arrest preoperatively, was operated upon as an emergency, and died intraoperatively, the remaining six patients in Group B who died all had repairs that left them with pulmonary insufficiency postoperatively.
Discussion The rare anomaly of absent pulmonary valve in association with tetralogy of Fallot has been noted in 3% to 6% of autopsy cases.v' The 35 patients in this series are from a total of 1,354 patients with tetralogy of Fallot undergoing repair at our institution during the same time interval, a surgical incidence of 2.6% in our experience. The incidence of the additional associated anomaly of absent pulmonary artery (14.3%) or anomalous origin of one pulmonary artery from the aorta (5.7%) in this series is higher than expected in a general population of patients having tetralogy of Fallot. Aneurysmal dilatation of the pulmonary arteries begins in utero; bronchial obstruction from the aneurysmal arteries, when present, probably always begins then or during the first year of life.' Osman, Meng, and Girdany' have observed at bronchography partial bronchial or tracheal compression on inspiration and complete obstruction during expiration in some patients. The cardiac lesion is detected at birth in most patients because of the characteristic murmur. Preoperative cardiac catheterization was performed in all previously reported patients who underwent operative repair. Catheterization was performed also on all patients in this series except for the most recent one, who was dependent on semi-prone positioning for maintenance of a patent airway. Two-dimensional echocardiography confirmed the clinical diagnosis in this patient and accuratelydefined the anatomy subsequently found at operation. As previously suggested by Cheatham, Latson, and Gutgesell," the echocardiographic features of this anomaly are diagnostic; they and others' have shown that it is possible to eliminate preoperative cardiac catheterization with its attendant risks in many critically ill infants with congenital heart disease. A subset of patients with tetralogy of Fallot and absent pulmonary valve enter a critical period during
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infancy in which symptoms of severe respiratory distress, hypoxic spells, or congestive heart failure develop. For these patients, some type of surgical intervention is usually required. The pulmonary problems may be the primary reason for clinical deterioration. If significant lobar emphysema has occurred, lobectomy is one alternative to help the patient through this difficult period and to allow his condition to stabilize for later definitive repair. Although three of our patients underwent prior lobectomies, our current approach would be to proceed with total repair, to be followed by lobectomy only if the lobar emphysema persists. We have not used other palliative procedures such as isolated pulmonary artery aneurysmorrhaphy,' superior vena cava-right pulmonary artery anastomosis," division of the right or left pulmonary artery and placement in an anterior position using a prosthetic graft," simultaneous interposition of a polytetrafluoroethylene shunt and main pulmonary artery ligation,'? II or main pulmonary artery banding." For those patients who do not become symptomatic in infancy, growth and development are normal and repair can be undertaken electively. The correct management of the stenotic and incompetent pulmonary anulus remains controversial. Ilbawi and colleagues" contend that a valve should be inserted in all patients. Arensman's group" use a transannular patch, accepting the inevitable pulmonary insufficiency. Pinsky and associates" recommend a valve only when pulmonary hypertension is present. Although it is attractive in principle to provide pulmonary valvular competence by insertion of a valve between the right ventricular outflow tract and the dilated main pulmonary artery, mechanical valves have not proved reliable in this position and tissue valves have a limited life expectancy. All currently available tissue-valved conduits placed in the right ventricular outflow tract have a significant incidence of late calcification and stenosis necessitating their replacement.":" One of the patients in this series required replacement of the conduit 8 years after implantation. In our experience, pulmonary valvular insufficiency is present in the majority of patients after repair of tetralogy of Fallot, but this has not resulted in significant early harmful effects. The long-term results of pulmonary insufficiency are not fully known, but in a review of the late results of repair of tetralogy of Fallot at our institution," most patients have enjoyed excellent late results. Of those who have required reoperation," none had pulmonary valvular incompetence as the sole hemodynamic abnormality. Those patients who received a prosthetic pulmonary valve at reoperation all had associated residual lesions such as tricuspid insufficiency,
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ventricular septal defect, peripheral pulmonary arterial stenoses, atrial septal defect, right ventricular outflow tract gradient, or a combination of these. In the review of our first 18 patients, I we reported that none received a pulmonary valve and none had reconstruction of the aneurysmal pulmonary arteries. Subsequent to submission of the manuscript, as noted in the addendum, late deterioration was noted in one patient and a subsequent patient died suddenly at home. Subsequent analysis of our experience, as reported herein, suggests that for those patients minimally symptomatic preoperatively (Group A), it is not necessary to insert a pulmonary valve for long-term symptom-free survival. On the other hand, those patients who are signifIcantly symptomatic early in life (Group B) have a relatively high early and late mortality when a pulmonary valve is not implanted. The persistence of significant respiratory symptoms in three of our patients, which occurred even after reoperation for placement of a porcine valve in the pulmonary position in two of them, suggests that some type of reconstruction of the aneurysmal pulmonary arteries is indicated, in addition to a pulmonary valve, in order to relieve chronic bronchial compression. We now believe the best operation for Group B patients is patch closure of the ventricular septal defect; reduction of the size of the pulmonary arteries by partial resection, plication, or both; and insertion of a valve in the pulmonary area. Others have recently advocated reduction in pulmonary arterial size as an important part of the repair in symptomatic infants.14.21.22 Currently, we would also consider placement of a pulmonary valve in all patients with this syndrome who have associated absence of one pulmonary artery. In our series, of those patients with tetralogy of Fallot and absent pulmonary valve who had significant symptoms early in life, the majority were male, 79% in Group B compared to 43% in Group A (p = 0.036). This association has not been noted previously and its significance is not clear. Our current concepts for the management of the patient with tetralogy of Fallot and absent pulmonary valve syndrome are as follows: 1. The minimally symptomatic patient should undergo a corrective operation electively in childhood. The ventricular septal defect is closed with a patch and pulmonary stenosis is relieved. The insertion of a pulmonary valve prosthesis and resection or plication of aneurysmal pulmonary arteries are not essential. 2. The markedly symptomatic neonate or infant should undergo intensive medical management, including ventilator support when necessary. lithe patient fails
Thoracic and Cardiovascular Surgery
to respond, a corrective operation involving patch closure of the ventricular septal defect, insertion of a valve or valved conduit in the right ventricular outflow tract, and partial resection, plication, or both of the aneurysmal pulmonary arteries are performed. This philosophy of management has been employed in 13 consecutive patients over the past 10 years; there has been only one early death, which occurred in the neonate who had a cardiac arrest and underwent emergency operation. There are no late deaths in this latter group and the patients are clinically well on follow-up. We are indebted to William M. O'Fallon, Ph.D., of the Medical Statistics Department for assistance with the statistical analysis. REFERENCES
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Stafford EG, Mair DD, McGoon DC, Danielson GK: Tetralogy of Fallot with absent pulmonary valve. Surgical considerations and results. Circulation 47:Suppl 3:24-30, 1973 Lev M, Eckner FAO: The pathologic anatomy of tetralogy of Fallot and its variations. Chest 45:251-261, 1964 Nagao GI, Daoud GI, McAdams AJ, Schwartz DC, Kaplan S: Cardiovascular anomalies associated with tetralogy of Fallot. Am J Cardiol 20:206-215, 1967 D'Cruz lA, Lendrum BL, Novak G: Congenital absence of the pulmonary valve. Am Heart J 68:728-740, 1964 Osman MZ, Meng CCL, Girdany BR: Congenital absence of the pulmonary valve. Report of eight cases with review of the literature. AJR 106:58-69, 1969 Cheatham JP, Latson LA, Gutgesell HP: Echocardiographic pulsed Doppler features of absent pulmonary valve syndrome in the neonate. Am J Cardiol 49: 1773-1777, 1982 Rice MJ, Seward JB, Hagler DJ, Mair DD, Feldt RH, Puga FJ, Danielson GK, Edwards WD, Tajik AJ: Impact of 2-dimensional echocardiography on the management of distressed newborns in whom cardiac disease is suspected. Am J Cardiol 51:288-292, 1983 Waldhausen JA, Friedman S, Nicodemus H, Miller WW, Rashkind W, Johnson J: Absence of the pulmonary valve in patients with tetralogy of Fallot. Surgical management. J THORAC CARDIOVASC SURG 57:669-674, 1969 Litwin SB, Rosenthal A, Fellows K: Surgical management of young infants with tetralogy of Fallot, absence of the pulmonary valve, and respiratory distress. J THORAC CARDIOVASC SURG 65:552-558, 1973 Byrne JP, Hawkins JA, Battiste CE, Khoury GH: Palliative procedures in tetralogy of Fallot with absent pulmonary valve. A new approach. Ann Thorac Surg 33:499502, 1982 Kirklin JW, Blackstone EH, Kirklin JK, Pacifico AD, Aramendi J, Bargeron LM Jr: Surgical results and protocols in the spectrum of tetralogy of Fallot. Ann Surg 198:251-265, 19l53
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12 Opie JC, Sandor GGS, Ashmore PG, Patterson MWH: Successful pal1iation by pulmonary artery banding in absent pulmonary valve syndrome with aneurysmal pulmonary arteries. J THORAC CARDIOVASC SURG 85: 125182, 1983 13 Ilbawi MN, Idriss FS, Muster AJ, Wessel HU, Paul MH, Deleon SY: Tetralogy of Fallot with absent pulmonary valve. Should valve insertion be part of the intracardiac repair? J THORAC CARDIOVASC SURG 81:906-913, 1981 14 Arensman FW, Francis PD, Helmsworth JA, Benzing G III, Schreiber JT, Schwartz DC, Kaplan S: Early medical and surgical intervention for tetralogy of Fallot with absence of pulmonic valve. J THORAC CARDIOVASC SURG 84:430-436, 1982 15 Pinsky WW, Nihill MR, Mullins CE, Harrison G, McNamara DG: The absent pulmonary valve syndrome. Considerations of management. Circulation 54: 159-162, 1978 16 Agarwal KC, Edwards WD, Feldt RH, Danielson GK, Puga FJ, McGoon DC: Clinicopathological correlates of obstructed right-sided porcine-valved extracardiac conduits. J THORAC CARDIOVASC SURG 81:591-601, 1981
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17 McGoon DC, Danielson GK, Puga FJ, Ritter DG, Mair DD, Ilstrup DM: Late results after extracardiac conduit repair for congenital cardiac defects. Am J Cardiol 49:1741-1749,1982 18 Bissett GS III, Schwartz DC, Benzing G III, Helrnsworth J, Schreiber JT, Kaplan S: Late results of reconstruction of the right ventricular outflow tract with porcine xenografts in children. Ann Thorac Surg 31:437-443, 1981 19 Poirier RA, McGoon DC, Danielson GK, Wallace RB, Ritter DG, Moodie DS, Wiltse CG: Late results after repair of tetralogy of Fallot. J THORAC CARDIOVASC SURG 73:900-908, 1977 20 Uretzky G, Puga FJ, Danielson GK, Hagler DJ, McGoon DC: Reoperation after correction of tetralogy of Fallot. Circulation 66:Suppl 1:202-208, 1982 21 Dunnigan A, Oldham HN, Benson DW Jr: Absent pulmonary valve syndrome in infancy. Surgery reconsidered. Am J Cardiol 48: 117-122, 1981 22 Stel1in G, Jonas RA, Goh TH, Brawn WJ, Venables AW, Mee RBB: Surgical treatment of absent pulmonary valve syndrome in infants. Relief of bronchial obstruction. Ann Thorac Surg 36:468-475, 1983
Tetralogy of Fallot with absent pulmonary valve Early and late results of surgical treatment From 1957 through September, 1983, 35 patients with tetralogy of Fallot and absent pulmonary valve mtderwent operation. Two subgroups of patients were recognized: minimally symptomatic (Group A, n = 21) and markedlysymptomatic (Group B, n = 14).Group B patients were symptomatic at an earlier age and were younger at operation.Ages ranged from 1 day to 42 years (mean = 7.8 ± 7.4 SD). Repair consisted of closure of the ventricular septal defect and relief of the right ventricle-pulmonary artery pressuregradient (nine patients received a tissuevalve), In five patients,partial resection and/or plication of the aneurysmalpulmonary arteries also was performed. Operative mortalitywas 4.8% in GroupA and 35.7 % in GroupB. In up to 25 years of fonow-up, there was onelate death in GroupA (an early case with complete heart block); the remaining 19 patients are asymptomatic. In Group B, there were four late deaths, three related to cardiopulmonary insufficiency. These results indicate pulmonary valve insufficiency is tolerated wen in minimally symptomatic patients. For markedly symptomatic patients, results may be improved if repair includes establishment of pulmonary valvular competence and reduction of size and length of aneurysmal pulmonary arteries.
B. C. McCaughan, M.B., B.S., Gordon K. Danielson, M.D., David J. Driscoll, M.D., and Dwight C. McGoon, M.D., Rochester, Minn.
h e syndrome of tetralogy of Fallot with absent pulmonary valve is characterized by a ventricular septal defect, overriding of the aorta, right ventricular hypertrophy, and pulmonary annular stenosis in association with absent or rudimentary pulmonary valve. Pulmonary regurgitation and aneurysmal dilatation of the pulmonary arteries uniformly are present. Symptoms vary from life-threatening respiratory obstruction or cardiac failure in neonates to absence of symptoms in patients who may live unrestricted lives for many years. As with uncomplicated tetralogy of Fallot, surgical repair has been demonstrated to be effective treatment. To assess the early and late results of operation, we reviewed all patients undergoing repair of this syndrome at the Mayo Clinic. From the Department of Thoracic and Cardiovascular Surgery and the Department of Pediatric Cardiology, Mayo Clinic, Rochester, Minn. Presented at the American Heart Association Fifty-sixth Scientific Sessions, Anaheim, California, Nov. 15, 1983. Received for publication Jan. 26, 1984. Accepted for publication March 12, 1984. Address for reprints: Gordon K. Danielson, M.D., Mayo Clinic/ Foundation, Rochester, Minn. 55905.
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Patients From 1957 through September, 1983, 35 patients with tetralogy of Fallot and absent pulmonary valve underwent repair at this institution. The first 18 patients have been reported previously.' There were 20 males and 15 females (male/female ratio = 1.3). Ages at operation ranged from 1 day to 42 years (mean = 7.8 ± 7.4 SD). There was a wide spectrum in the severity of symptoms. At one extreme was a critically ill l-day-old neonate with cyanosis, acidosis, and low cardiac output. At the other end of the spectrum was a 42-year-old woman whose murmur had been noted in childhood and whose only symptom was occasional palpitations from extrasystoles. The patients could be classified into two groups according to their clinical status:
Group A: Asymptomatic or minimally symptomatic-2l patients. These patients had been noted to have cyanosis, a cardiac murmur, or both, in the neonatal period. Growth and development were normal in infancy and childhood. Symptoms were absent in 10 patients and minimal (such as upper respiratory tract infection or fatigability on severe exertion) in 10 patients. One additional patient had frequent premature beats and runs of ventricular tachycardia. Ages at operation
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ranged from 3 to 42 years (mean = 10.2 ± 8.5 SD). Nine of the 21 patients were male (43%). Group B: Markedly symptomatic-14 patients. These patients had one or more of the following symptoms within the first 6 months of life: cardiac failure necessitating treatment (eight patients), marked developmental delay (nine patients), and episodes of respiratory obstruction, infection, or both, necessitating hospitalization (13 patients). Two patients had required tracheostomy during episodes of severe respiratory distress. Three patients had undergone lobectomy (one a right middle lobectomy, one a right middle and right lower lobectomy, and one a left upper lobectomy) prior to intracardiac repair because of lobar emphysema secondary to bronchial compression. The condition of all three had improved after lobectomy. The ages of the 14 patients at operation ranged from 1 day to 9 years (mean = 4.3 ± 3.1 SD). Eleven of the 14 patients were male (79%). Physical findings in all 35 patients were remarkably similar and consisted of an overactive heart and a right ventricular impulse. There was a systolic thrill associated with a harsh systolic murmur along the left upper sternal border. An early diastolic murmur, often accompanied by a thrill, was present at the left sternal border. These murmurs were characteristically "to-and-fro," but in some patients they were thought initially to be continuous. The second sound in the pulmonary area was absent in all cases. The electrocardiograms showed normal sinus rhythm in all 35 patients; three had associated first-degree atrioventricular block. Premature ventricular contractions were noted on three tracings. V leads were taken in 34patients; eight had right bundle branch block, 23 had rightventricular hypertrophy alone, one had left ventricular hypertrophy alone, and two had biventricular hypertrophy. Cardiomegaly was present in nearly all of the chest roentgenograms. Aneurysmal dilatation of the central pulmonary arteries and normal peripheral pulmonary vasculature were present bilaterally except in the seven patients with unilateral pulmonary artery anomalies (Table I). Ten patients (29%) had a right-sided aortic arch. Cardiac catheterization was performed prior to operation in all but one patient. The one exception was a 3-month-old infant who had critical obstruction of the left main bronchus that necessitated continuous semiprone positioning. The clinical diagnosis was confirmed by two-dimensional echocardiography. In Group A, all patients had a predominant left-to-right shunt; the mean Qp/Qs in 15 patients in whom it was measured was 1.8
28 1
Table I. Associated cardiac anomalies (20 patients) Anomaly Right aortic arch Interatrial communication Ostium secundum defect Patent foramen ovale Absent LPA Origin LPA from ascending aorta Origin RP A from descending aorta Patent ductus arteriosus Bilateral superior venae cavae Double outlet right ventricle Multiple ventricular septal defects
Pt. No. 10 10 6 4 5 I I I 1 I
I
Legend: LPA, Left pulmonary artery. RPA, Right pulmonary artery.
(range = 1.1 to 3.2). The peak systolic right ventriclepulmonary artery pressure gradient ranged from 35 to 86 mm Hg (mean = 60) (N = 20), the peak pulmonary artery systolic pressure ranged from 18 to 40 mm Hg (mean = 29) (N = 20), and the systemic arterial oxygen saturation ranged from 90% to 96% (mean = 93%) (N = 18). Within Group B, the predominant shunt was left-to-right in five (mean Qp/Qs = 1.9), right-to-left in four (mean Qp/Qs = 0.5), and essentially balanced in four. The peak systolic right ventricle-pulmonary artery pressure gradient ranged from 28 to 75 mm Hg (mean = 52) (N = 13), the peak pulmonary artery systolic pressure ranged from 20 to 52 mm Hg (mean = 31) (N = 13), and the systemic arterial oxygen saturation ranged from 68% to 94% (mean = 86) (N = 12). Associated cardiac anomalies included absent left pulmonary artery in five patients and anomalous origin of a pulmonary artery from the aorta in two patients (Table I). Operative findings and procedures Right ventricular hypertrophy and pulmonary annular stenosis were present in all 35 patients. True pulmonary valvular leaflets were absent. Remnants of leaflet tissue were present in some cases as tags of tissue or a ridge at the anulus. The limbs of the crista were hypertrophied in 31 patients, but in only six were they thought to be causing significant obstruction. A paramembranous ventricular septal defect (sited high posteriorly in the ventricular septum adjacent to the septal leaflet of the tricuspid valve) was present in all patients. In one patient, there was a second, muscular, ventricular septal defect inferior to the first. Pulmonary arterial dilatation was a feature in all cases. In 24 patients, the main pulmonary artery and proximal portions of the right and left pulmonary
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Fig. 1. Diagram of operative technique. A, A longitudinal incision is made in the main pulmonary artery and extended proximally across the pulmonary anulus into the right ventricular outflow tract (inset). The ventricular septaldefectis patchedand a tissue-valved conduit is anastomosed to the distal pulmonary artery. B, The proximal conduitis fashioned with an anterior flap and anastomosed to the right ventricular outflow tract. Inset, Transverse plication sutures reduce the diameter of the aneurysmal right and left pulmonary arteries.
Table II. Repair of right ventricular outflow tract Repair
Pt, No.
Radial annular incisions Excision of rudimentary valve tissue only Valved conduit Transannular patch Valve with patch
11 10
Total
35
7 5 2
arteries were dilated markedly. In four patients, the main pulmonary artery was enlarged slightly, the right pulmonary artery was enlarged markedly, and the left pulmonary artery was normal in size. In six patients, the main pulmonary artery and right pulmonary artery were dilated markedly; in five of these the left pulmonary artery was absent and in the sixth it arose from the ascending aorta. In one patient, the main pulmonary artery and left pulmonary artery were dilated markedly and the right pulmonary artery arose from the descending thoracic aorta. Other associated cardiac findings are shown in Table I. The mitral, tricuspid, and aortic valves were normal in all cases.
Operation was performed via median sternotomy using cardiopulmonary bypass with moderate hypothermia. In our earlier experience, intermittent aortic crossclamping was employed. Since 1978, the use of cold potassium cardioplegic solution combined with topical application of cold saline has been preferred. Total circulatory arrest under profound hypothermia was utilized to facilitate repair in two infants. A right ventriculotomy was performed and the intracardiac anatomy was defined. In some patients, a second incision was made in the main pulmonary artery. Hypertrophied cristal bands were resected. The paramembranous ventricular septal defect was closed by direct suture in six patients (all prior to 1964) and by a patch technique in 29 patients. Any leaflet tissue remnants were resected. In 10 patients (six in Group A and four in Group B), this relieved the right ventriclepulmonary artery gradient and the pulmonary anulus was left intact. In 11 patients, radial incisions were made in the anulus to reduce the degree of pulmonary stenosis (six in Group A and five in Group B). In five patients (two in Group A, three in Group B), transannular patch enlargement of the outflow tract was performed (pericardium in four and a Teflon patch in one). In the
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Fig. 2. Operative technique, continued. A, An alternate and more effective way to reduce the diameter of the pulmonary arteriesis to resectredundantarterial wall, as shown. B, For additional reliefof bronchial compression, the pulmonary arteries are shortened by plicating them with the arterial repair. C, In this example, after patch closure of the ventricular septaldefect, a tissue valve has beensewn to the pulmonary anulusand the anterior wall of the reconstruction is made with a pericardial patch. remaining nine patients (seven in Group A and two in Group B), all of whom have been operated upon since 1973, a glutaraldehyde-preserved tissue valve was inserted between the right ventricle and the main pulmonary artery. In two of the nine patients, a tissue valvewas placed at the level of the anulus and the roof of the outflow tract was reconstructed with a pericardial patch; in the remaining seven, a tissue-valved conduit* was inserted (Fig. 1) (Table II). In five patients, aneurysmally dilated pulmonary arteries were plicated, partially resected, or both (four in Group A and one in Group B) (Figs. 1 and 2). In one patient, in whom the left pulmonary artery arose from the proximal aorta, the former was detached and reimplanted into the main pulmonary artery. In another patient, in whom the right pulmonary artery arose from the descending aorta, it was not technically feasible to reimplant the right pulmonary artery so its origin was narrowed with a partially occlusive ligature in order to reduce shunting. The interatrial communication was closed in all 10 patients by direct suture, and the patent ductus arteriosus in one patient was ligated. The double-outlet right ventricle was repaired in the course of closure of the "Hancock Laboratories, Anaheim, Calif.
ventricular septal defect. Intraoperative electrophysiological mapping in the patient who had multiple premature ventricular beats and episodes of ventricular tachycardia demonstrated a reentry point in the right ventricular outflow tract caudal to the pulmonary anulus. This portion of the outflow tract was excised, and the endocardium of the right ventricle downstream to the moderator band was electrocoagu1ated. The postrepair right ventricle-pulmonary artery gradients were reduced from preoperative values in all patients except one. The mean preoperative peak systolic pressure gradient was 58 ± 14 SD mm Hg (range = 28 to 86), and the mean postrepair gradient was 23 ± 13 SD mm Hg (range = 0 to 45) (Fig. 3).
Results Group A. Of the 21 patients who were asymptomatic or minimally symptomatic preoperatively, one died within 30 days of operation (4.8%). This death occurred in a 3-year-old girl who had a technically uncomplicated procedure (in 1967), but who developed progressive coma postoperatively. The cause of the cerebral damage was not revealed by autopsy examination. Two patients required exploration for excessive postoperative bleeding. One patient, operated upon early in the series, developed complete heart block immediately following
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100,....-------------,100
mmHg
Mean
50
o
Preoperative
0
Postoperative
Fig. 3. Preoperative and postoperative peak systolic pressure gradients between the right ventricle and pulmonary artery.
direct closure of the ventricular septal defect. This patient and two additional ones in Group B were described in our earlier report. I There have been no further cases of permanent complete heart block in the past 20 years at this institution after operations for this condition. No patient required prolonged respiratory support. At the closing date of the study, follow-up had been obtained on all 20 patients surviving operation; the follow-up intervals ranged from 1 month to 25 years (median = 10 years). There was one late death which occurred in the patient who had complete heart block. She had remained well for 31h years without a pacemaker but then died suddenly. One patient who developed congestive heart failure from a recurrent ventricular septal defect underwent successful reoperation 4 months after the initial procedure. All 19 late survivors are currently well without apparent physical limitations. Of the 12 patients who have pulmonary insufficiency, all remain well 2 to 25 years later (median = 15 years). Partial resection or plication of the aneurysmal arteries was not employed in any of these patients. The patient with recurrent ventricular tachycardia has remained asymptomatic without antiarrhythmic therapy. One of the seven patients who received a tissue-valved conduit was reoperated upon elsewhere 8 years after repair for replacement of the conduit with another tissue-valved conduit because of valvular calcification and stenosis. Group B. Of the 14 patients having marked symptoms during their first 6 months of life, five died within 30 days of operation (35.7%). The causes of death were heart block in one (prior to the availability of pacemakers), ventricular fibrillation in the immediate postopera-
tive period in one, and low cardiac output in two. One of the latter patients was the l-day-old infant who was operated upon as an emergency. She was hypoxic, cyanotic, and acidotic and had a cardiac arrest prior to operation. Repair was accomplished with a No. 12 mm porcine-valved conduit, but she could not be weaned from cardiopulmonary bypass. The fifth patient died suddenly, presumably from an arrhythmia, 2 weeks after hospital discharge (but within 30 days of operation). His electrocardiogram showed sinus rhythm and right bundle branch block only; there were no postoperative arrhythmias. Autopsy showed the repair to be intact. Right ventricular hypertrophy was present (wall thickness 1.3 em); the average left ventricle thickness was 0.9 em. Four patients required prolonged intensive respiratory care; tracheostomy was necessary in two. Ultimately, all four left the hospital free of respiratory symptoms. A recent case illustrates many of the features of the symptomatic infant. A 3-month-old boy required continuous semi-prone positioning, narcotic sedation, and bronchodilator therapy. Preoperative cardiac catheterization could not be performed because of the infant's clinical condition and marked deterioration when turned from the prone position. Two-dimensional echocardiography confirmed the clinical diagnosis. Repair consisted of patch closure of the ventricular septal defect, reduction of the size of the aneurysmal pulmonary arteries by partial resection and plication, and insertion of a valved conduit in the right ventricular outflow tract. Postoperatively, copious secretions were aspirated from the previously obstructed bronchi. Intensive respiratory care was necessary for 5 days, but the patient subsequently was discharged on postoperative day 12 free of respiratory symptoms. He has remained well and requires no medication. Follow-up was obtained on all nine patients surviving operation; the follow-up intervals ranged from 1 to 22 years (median = 12 years). One of the patients died 6 weeks after operation from complications of complete heart block. Three additional late deaths occurred, all 8 years postoperatively. At operation, the pulmonary anulus had been enlarged by radial incision in all three patients and in none was partial resection or plication of the dilated pulmonary arteries performed. All had episodic respiratory infections and congestive heart failure within 6 months after hospital discharge. Reoperation was performed for placement of a porcine tissue valve in the pulmonary position in two patients 2 and 4 years postoperatively, respectively.The third patient had worsening restrictive lung disease secondary to thoracic scoliosis. Episodic respiratory difficulties persisted in all
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three patients until sudden death occurred in each, 8 years after operation. Autopsy was performed in two; the surgical repairs were intact and no apparent cause for the sudden deaths was found. If one excludes the three patients whose deaths were related to permanent heart block (all operated upon prior to 1964), the combined early and late mortality is one of 20 (5.0%) in Group A and seven of 12 (58.3%) in Group B (p < 0.001). Excluding the l-day-old infant (Patient 12 in Group B) who had a cardiac arrest preoperatively, was operated upon as an emergency, and died intraoperatively, the remaining six patients in Group B who died all had repairs that left them with pulmonary insufficiency postoperatively.
Discussion The rare anomaly of absent pulmonary valve in association with tetralogy of Fallot has been noted in 3% to 6% of autopsy cases.v' The 35 patients in this series are from a total of 1,354 patients with tetralogy of Fallot undergoing repair at our institution during the same time interval, a surgical incidence of 2.6% in our experience. The incidence of the additional associated anomaly of absent pulmonary artery (14.3%) or anomalous origin of one pulmonary artery from the aorta (5.7%) in this series is higher than expected in a general population of patients having tetralogy of Fallot. Aneurysmal dilatation of the pulmonary arteries begins in utero; bronchial obstruction from the aneurysmal arteries, when present, probably always begins then or during the first year of life.' Osman, Meng, and Girdany' have observed at bronchography partial bronchial or tracheal compression on inspiration and complete obstruction during expiration in some patients. The cardiac lesion is detected at birth in most patients because of the characteristic murmur. Preoperative cardiac catheterization was performed in all previously reported patients who underwent operative repair. Catheterization was performed also on all patients in this series except for the most recent one, who was dependent on semi-prone positioning for maintenance of a patent airway. Two-dimensional echocardiography confirmed the clinical diagnosis in this patient and accuratelydefined the anatomy subsequently found at operation. As previously suggested by Cheatham, Latson, and Gutgesell," the echocardiographic features of this anomaly are diagnostic; they and others' have shown that it is possible to eliminate preoperative cardiac catheterization with its attendant risks in many critically ill infants with congenital heart disease. A subset of patients with tetralogy of Fallot and absent pulmonary valve enter a critical period during
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infancy in which symptoms of severe respiratory distress, hypoxic spells, or congestive heart failure develop. For these patients, some type of surgical intervention is usually required. The pulmonary problems may be the primary reason for clinical deterioration. If significant lobar emphysema has occurred, lobectomy is one alternative to help the patient through this difficult period and to allow his condition to stabilize for later definitive repair. Although three of our patients underwent prior lobectomies, our current approach would be to proceed with total repair, to be followed by lobectomy only if the lobar emphysema persists. We have not used other palliative procedures such as isolated pulmonary artery aneurysmorrhaphy,' superior vena cava-right pulmonary artery anastomosis," division of the right or left pulmonary artery and placement in an anterior position using a prosthetic graft," simultaneous interposition of a polytetrafluoroethylene shunt and main pulmonary artery ligation,'? II or main pulmonary artery banding." For those patients who do not become symptomatic in infancy, growth and development are normal and repair can be undertaken electively. The correct management of the stenotic and incompetent pulmonary anulus remains controversial. Ilbawi and colleagues" contend that a valve should be inserted in all patients. Arensman's group" use a transannular patch, accepting the inevitable pulmonary insufficiency. Pinsky and associates" recommend a valve only when pulmonary hypertension is present. Although it is attractive in principle to provide pulmonary valvular competence by insertion of a valve between the right ventricular outflow tract and the dilated main pulmonary artery, mechanical valves have not proved reliable in this position and tissue valves have a limited life expectancy. All currently available tissue-valved conduits placed in the right ventricular outflow tract have a significant incidence of late calcification and stenosis necessitating their replacement.":" One of the patients in this series required replacement of the conduit 8 years after implantation. In our experience, pulmonary valvular insufficiency is present in the majority of patients after repair of tetralogy of Fallot, but this has not resulted in significant early harmful effects. The long-term results of pulmonary insufficiency are not fully known, but in a review of the late results of repair of tetralogy of Fallot at our institution," most patients have enjoyed excellent late results. Of those who have required reoperation," none had pulmonary valvular incompetence as the sole hemodynamic abnormality. Those patients who received a prosthetic pulmonary valve at reoperation all had associated residual lesions such as tricuspid insufficiency,
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28 6 McCaughan et al.
ventricular septal defect, peripheral pulmonary arterial stenoses, atrial septal defect, right ventricular outflow tract gradient, or a combination of these. In the review of our first 18 patients, I we reported that none received a pulmonary valve and none had reconstruction of the aneurysmal pulmonary arteries. Subsequent to submission of the manuscript, as noted in the addendum, late deterioration was noted in one patient and a subsequent patient died suddenly at home. Subsequent analysis of our experience, as reported herein, suggests that for those patients minimally symptomatic preoperatively (Group A), it is not necessary to insert a pulmonary valve for long-term symptom-free survival. On the other hand, those patients who are signifIcantly symptomatic early in life (Group B) have a relatively high early and late mortality when a pulmonary valve is not implanted. The persistence of significant respiratory symptoms in three of our patients, which occurred even after reoperation for placement of a porcine valve in the pulmonary position in two of them, suggests that some type of reconstruction of the aneurysmal pulmonary arteries is indicated, in addition to a pulmonary valve, in order to relieve chronic bronchial compression. We now believe the best operation for Group B patients is patch closure of the ventricular septal defect; reduction of the size of the pulmonary arteries by partial resection, plication, or both; and insertion of a valve in the pulmonary area. Others have recently advocated reduction in pulmonary arterial size as an important part of the repair in symptomatic infants.14.21.22 Currently, we would also consider placement of a pulmonary valve in all patients with this syndrome who have associated absence of one pulmonary artery. In our series, of those patients with tetralogy of Fallot and absent pulmonary valve who had significant symptoms early in life, the majority were male, 79% in Group B compared to 43% in Group A (p = 0.036). This association has not been noted previously and its significance is not clear. Our current concepts for the management of the patient with tetralogy of Fallot and absent pulmonary valve syndrome are as follows: 1. The minimally symptomatic patient should undergo a corrective operation electively in childhood. The ventricular septal defect is closed with a patch and pulmonary stenosis is relieved. The insertion of a pulmonary valve prosthesis and resection or plication of aneurysmal pulmonary arteries are not essential. 2. The markedly symptomatic neonate or infant should undergo intensive medical management, including ventilator support when necessary. lithe patient fails
Thoracic and Cardiovascular Surgery
to respond, a corrective operation involving patch closure of the ventricular septal defect, insertion of a valve or valved conduit in the right ventricular outflow tract, and partial resection, plication, or both of the aneurysmal pulmonary arteries are performed. This philosophy of management has been employed in 13 consecutive patients over the past 10 years; there has been only one early death, which occurred in the neonate who had a cardiac arrest and underwent emergency operation. There are no late deaths in this latter group and the patients are clinically well on follow-up. We are indebted to William M. O'Fallon, Ph.D., of the Medical Statistics Department for assistance with the statistical analysis. REFERENCES
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6
7
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9
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Stafford EG, Mair DD, McGoon DC, Danielson GK: Tetralogy of Fallot with absent pulmonary valve. Surgical considerations and results. Circulation 47:Suppl 3:24-30, 1973 Lev M, Eckner FAO: The pathologic anatomy of tetralogy of Fallot and its variations. Chest 45:251-261, 1964 Nagao GI, Daoud GI, McAdams AJ, Schwartz DC, Kaplan S: Cardiovascular anomalies associated with tetralogy of Fallot. Am J Cardiol 20:206-215, 1967 D'Cruz lA, Lendrum BL, Novak G: Congenital absence of the pulmonary valve. Am Heart J 68:728-740, 1964 Osman MZ, Meng CCL, Girdany BR: Congenital absence of the pulmonary valve. Report of eight cases with review of the literature. AJR 106:58-69, 1969 Cheatham JP, Latson LA, Gutgesell HP: Echocardiographic pulsed Doppler features of absent pulmonary valve syndrome in the neonate. Am J Cardiol 49: 1773-1777, 1982 Rice MJ, Seward JB, Hagler DJ, Mair DD, Feldt RH, Puga FJ, Danielson GK, Edwards WD, Tajik AJ: Impact of 2-dimensional echocardiography on the management of distressed newborns in whom cardiac disease is suspected. Am J Cardiol 51:288-292, 1983 Waldhausen JA, Friedman S, Nicodemus H, Miller WW, Rashkind W, Johnson J: Absence of the pulmonary valve in patients with tetralogy of Fallot. Surgical management. J THORAC CARDIOVASC SURG 57:669-674, 1969 Litwin SB, Rosenthal A, Fellows K: Surgical management of young infants with tetralogy of Fallot, absence of the pulmonary valve, and respiratory distress. J THORAC CARDIOVASC SURG 65:552-558, 1973 Byrne JP, Hawkins JA, Battiste CE, Khoury GH: Palliative procedures in tetralogy of Fallot with absent pulmonary valve. A new approach. Ann Thorac Surg 33:499502, 1982 Kirklin JW, Blackstone EH, Kirklin JK, Pacifico AD, Aramendi J, Bargeron LM Jr: Surgical results and protocols in the spectrum of tetralogy of Fallot. Ann Surg 198:251-265, 19l53
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12 Opie JC, Sandor GGS, Ashmore PG, Patterson MWH: Successful pal1iation by pulmonary artery banding in absent pulmonary valve syndrome with aneurysmal pulmonary arteries. J THORAC CARDIOVASC SURG 85: 125182, 1983 13 Ilbawi MN, Idriss FS, Muster AJ, Wessel HU, Paul MH, Deleon SY: Tetralogy of Fallot with absent pulmonary valve. Should valve insertion be part of the intracardiac repair? J THORAC CARDIOVASC SURG 81:906-913, 1981 14 Arensman FW, Francis PD, Helmsworth JA, Benzing G III, Schreiber JT, Schwartz DC, Kaplan S: Early medical and surgical intervention for tetralogy of Fallot with absence of pulmonic valve. J THORAC CARDIOVASC SURG 84:430-436, 1982 15 Pinsky WW, Nihill MR, Mullins CE, Harrison G, McNamara DG: The absent pulmonary valve syndrome. Considerations of management. Circulation 54: 159-162, 1978 16 Agarwal KC, Edwards WD, Feldt RH, Danielson GK, Puga FJ, McGoon DC: Clinicopathological correlates of obstructed right-sided porcine-valved extracardiac conduits. J THORAC CARDIOVASC SURG 81:591-601, 1981
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17 McGoon DC, Danielson GK, Puga FJ, Ritter DG, Mair DD, Ilstrup DM: Late results after extracardiac conduit repair for congenital cardiac defects. Am J Cardiol 49:1741-1749,1982 18 Bissett GS III, Schwartz DC, Benzing G III, Helrnsworth J, Schreiber JT, Kaplan S: Late results of reconstruction of the right ventricular outflow tract with porcine xenografts in children. Ann Thorac Surg 31:437-443, 1981 19 Poirier RA, McGoon DC, Danielson GK, Wallace RB, Ritter DG, Moodie DS, Wiltse CG: Late results after repair of tetralogy of Fallot. J THORAC CARDIOVASC SURG 73:900-908, 1977 20 Uretzky G, Puga FJ, Danielson GK, Hagler DJ, McGoon DC: Reoperation after correction of tetralogy of Fallot. Circulation 66:Suppl 1:202-208, 1982 21 Dunnigan A, Oldham HN, Benson DW Jr: Absent pulmonary valve syndrome in infancy. Surgery reconsidered. Am J Cardiol 48: 117-122, 1981 22 Stel1in G, Jonas RA, Goh TH, Brawn WJ, Venables AW, Mee RBB: Surgical treatment of absent pulmonary valve syndrome in infants. Relief of bronchial obstruction. Ann Thorac Surg 36:468-475, 1983