Surgical repair of univentricular heart (double inlet left ventricle) with obstructed anterior subaortic outlet chamber

JACC V. ,I 4, No.4 October '184:771-8

771

Surgical Repair of Univentricular Heart (Double Inlet Left Ventricle) With Obstructed Anterior Subaortic Outlet Chamber GERALD BARBER, MD, DONALD J. HAGLER, MD, FACC, WILLIAM D. EDWARDS, MD, FACC, FRANCISCO J. PUGA, MD, FACC, GORDON K. DANIELSON, MD, FACe, DWIGHT C. McGOON, MD, FACC, DAVID J. DRISCOLL, MD, FACC Rochester, Minnesota

The results of operation in all patients with univentricular heart and an obstructed anterior subaortic outlet chamber who were operated on utilizing extracorporeal circulation at the Mayo Clinic from 1973 through 1983 were reviewed. Ten of the 18 patients died during the immediate postoperative period and there was one late death. Factors significantly related to operative and immediate postoperative mortality were age at operation, cardiothoracic ratio on X-ray examination, degree of ST

Univentricular heart (double inlet left ventricle) is a congenital cardiac malformation with a poor natural history. In one study 0), more than 50% of the patients died within 14 years of diagnosis. For this reason, various palliative (2-15) and "corrective" 06-36) surgical approaches have been advocated. However, in those patients with an anterior subaortic outlet chamber, the outlet foramen frequently becomes obstructed after pulmonary artery banding (12, 15,36). Moreover, these patients have a particularly high mortality rate with attempted "corrective" surgery. Reports of operation on 15 patients with this lesion have been published previously (12,15,27,33,35,36) and 9 of these patients (60%) died during the immediate postoperative period. We report our surgical results in 18 patients with this lesion and describe the factors predictive of outcome.

Methods Study patients. The records for all patients with univentricular heart who were operated on at the Mayo Clinic utilizing extracorporeal circulation from 1973 through 1983 were reviewed. Patients who had a univentricular heart with

From the Mayo Chnic and Mayo Foundation, Rochester, Minnesota. Manuscript received April 2, 1984, revised rnanuscnpt received May 23. 1984. accepted May 24, 1984. Address for reprInts: David J Driscoll, MD, Mayo Clinic, 200 First Street, Southwest, Rochester, Minnesota 55905 © 198-+ by the Amencan College of Cardiology

depression on electrocardiogram and pressure gradient across the outlet foramen at catheterization. Autopsy in eight cases revealed significant hypertrophy of ventricular myocardium and a small outlet foramen that was considered stenotic relative to either body surface area or aortic root area. The ventricular myocardium showed histologicchanges of chronic ischemia that predated the surgical procedure.

an anterior subaortic outlet chamber whose pressure gradient across the outlet foramen was 10 mm Hg or greater by cardiac catheterization before surgery were included in the study. Patients with tricuspid atresia and transposition of the great arteries, regardless of the pressure gradient across the ventricular septal defect, were excluded. Eighteen patients (11 male, 7 female) met the criteria of the study. Study procedure. The clinic records of the 18 patients were examined for details of the history, physical examination, chest roentgenogram, cardiac catheterization, operation and postoperative course. When available, two-dimensional echocardiograms were reviewed to determine the size of the outlet foramen, diameter of the aortic root and thickness of the ventricular wall. Pathologic specimens at autopsy, available in eight cases, were reviewed to determine these same measurements and also myocardial histopathologic features. For comparison, we also reviewed the histopathologic features of myocardium from patients younger than 20 years of age who had a univentricular heart with an anterior subaortic outlet chamber but without an outlet foramen obstruction. Specimens were available from eight such patients who died during the immediate postoperative period. Statistical methods. To determine factors that would predict a poor surgical outcome, the surviving group was compared with the nonsurviving group by means of Student's t test, with degrees of freedom adjusted for unequal variances when appropriate. Only those differences with a 0735-1097/84/$3 00

772

BARBER ET AL. SURGICAL REPAIR OF UNIVENTRICULAR HEART

lACC Vol 4. No 4 October 1984 771-8

Table 1. Results of Surgery in Patients With Univentricular Heart and Obstructed Anterior Subaortic Outlet Chamber Previously Reported Cases*

Mayo Clinic Cases

Procedure

Patients

Nonsurvivors

%

Enlarge foramen Aortopulmonary anastomosis Ventricular septation Modified Fontan Total

2 7 5 15

I 0 7 I 9

100 0 100 20 60

Patients 0 2t 3 13 18

Nonsurvivors

%

0 2 2 6 10

100 67 46 56

*From references 12, 15, 27. 33. 35 and 36. tOne patient had both enlargement of the foramen and aortopulmonary anastomosis.

two-tailed probability of less than 0.05 were considered significant. All data were expressed as mean ± standard deviation.

Results Preoperative data. Thirteen of the 18 patients previously had banding of the pulmonary artery, and the remaining 5 had natural pulmonary or subpulmonary stenosis. Three of these five had undergone a prior systemic to pulmonary artery shunt procedure. The pressure gradient across

the outlet foramen in the 18 patients ranged from 12 to 136 mm Hg (mean 63). Operations (Table 1). The operations performed were: a modified Fontan procedure in 13 patients, ventricular septation in 3 patients, transection of the main pulmonary artery with a proximal pulmonary artery to aorta anastomosis and a Goretex conduit from aorta to distal pulmonary artery in 1 patient and resection of the outlet foramen and side to side aorta to main pulmonary artery anastomosis in 1 patient. This latter patient had a double outlet outlet chamber with both outlet foramen obstruction and subpulmonary obstruc-

Table 2. Data From Patients With Univentricular Heart and Obstructed Anterior Subaortic Outlet Chamber

Age at operation (yr) Prior pulmonary artery band Taking digoxin before operation Chest roentgenogram Pulmonary vascularity increased Cardiothoracic ratio Electrocardiogram Rate (beats/min) Axis COl Absolute maximal ST deviation (mm) Echocardiogram Outlet foramen diameter (mm) Outlet foramen area/body surface area ( x 105 ) Outlet foramen area/aortic root area (%) % predicted of normal left ventricular wall thickness Catheterization Qp:Qs Gradient across outlet foramen (mm Hg) Operation Aortic cross-clamp time (min) Bypass time (min) Outlet foramen gradient (mm Hg) Begmrung procedure Ending procedure

Survivors

Nonsurvivors

16.0 ± 5.1 4/8 (50%) 3/8 (38%)

6.5 ± 4.2* 9/10 (90%) 5/10 (50%)

5/8 (62%) 0.50 ± 0.07

5/10 (50%) 0.61 ± 0.05*

87 ± 23 114 ± 12 1.9 ± 1.2

103 ± 10 99 ± 24 4.0 ± 1.8t

133 ± 4.2 12.2 ± 6.3 13.3 156 ± 14

4.3 ± 22§ 2.3 ± 2.2§ 7.7§ 190 ± 59§

1.4 ± 0.6 37 ± 20

1.3 ± 0.4

82 ± 24 140 ± 26

77 ± 33 153 ± 52

24 ± 17 4 ± 16

84 ± 36t

57 ± 19t 10 ± II

*p < 0.001; tp < 0.01; tp < 0.05; §statistical calculation not performed because of the small number of patients. Data are reported as mean values ± standard deviation. Qp:Qs = ratio of pulmonary to systemic blood flow.

lACC v • 4, No 4 October I ~84'771-8

BARBER ET AL SURGICAL REPAIR OF UNIVENTRICULAR HEART

100

773

,..-------------------::l

c:

g co Qj

• •• ,:-1

I

I'

I

j

~" t ~., -". I

• •

75

0.

o

•

•

•

•

25

r ,-

•

50

75

125

100

150

Pressure gradient Preop cardiac cathetenzanon

\

(mm Hg)

Figure 1. Electrocardiogram of a nonsurvivor demonstrating typical ST segment changes, This patient was not on digoxin therapy. I mm = 0,1 JLV,

tion. Ten of the 18 patients died within 72 hours of operation. Our surgical mortality rate of 56% is comparable with the 60% obtained from other previously reported studies (12,1 -; .27 ,33,35 ,36), In contrast. the surgical mortality rate was 14% for the last 50 patients with all types of univentricular heart operated on at the Mayo Clinic.

Figure 3. Relation between pressure gradient across the outlet foramen obtained by catheterization and at surgery. The regression equation is: surgical gradient = 0.63 times catheterization gradient. r = 0.83 (p < 0.001). Preop = preoperative.

differentiated these two groups. Nonsurvivors had a larger cardiothoracic ratio on chest roentgenogram and greater ST segment deviation on the electrocardiogram than did survivors (Fig. 1 and 2). No patient with ST depression of more than 3 mm in any electrocardiographic lead survived, By two-dimensional echocardiography, survivors had a larger outlet foramen than nonsurvivors, and this relation remained valid when the size of the outlet foramen was

adjusted for the differences in age and size between the two groups by indexing it to either body surface area or aortic root area. The left ventricular wall thickness expressed as a percent of predicted for weight was greater for nonsurvivors than for survivors. Valid statistical analysis of the echocardiographic data was impossible because of the retrospective nature of the study. Most patients in the first half of the study either never had echocardiography or the echocardiogram was not satisfactory to accurately measure the aortic root, outlet foramen and left ventricular posterior wall. During the second half of the study, only two of the three measurements could be determined on some of the echocardiograms. Therefore. outlet foramen and left ventricular wall thickness were measured in only three survivors and six nonsurvivors and aortic root dimension for only two survivors and four nonsurvivors.

Figure 2. Differences between survivors and nonsurvivors with respect to ST segment deviation. ECG = electrocardiographic: 0 sun ivor; • = nonsurvivor.

Figure 4. Relations among age, outcome and pressure gradient across the outlet foramen at cardiac catheterization. /':, = survivor Mayo Clinic: .. = nonsurvivor Mayo Clinic.

Comparisons between survivors and nonsurvivors (Table 2). No item of the history or physical examination

4

r-- - - - - - - - - - - - - - - , . . . , . - - -

--,

30

r---------------------,

25 I-

20 I00 ~

;>, Q)

15 I-

Ol


t:.

t:.

t:. t:.

t:.

o:

t:.

A A

A

10 I-

A

A A

51-

A [A

0 0

25

A

I

I

~

I

50

75

100

125

Pressure gradient Preop cardiac catheterization (mm Hg)

150

BARBER ET AL. SURGICAL REPAIR OF UNIVENTRICULAR HEART

774

Table 3. Comparison Between Available Echocardiographic and Autopsy Data for Nonsurvivors

Outlet foramen diameter (mm) Outlet foramen area/body surface area (X

Echocardiogram

Autopsy

(n = 6)

(n = 8)

4.3 ± 2.2 2.3 ± 2.2

8.7 ± 4.5 6.0 ± 4.2

7.7

27 ± 18 177 ± 22

10- 5)

Outlet foramen area/aortic root area (%) % predicted of normal left ventricular wall thickness

190 ± 59

Data are reported as mean values ± standard deviation.

The pressure gradient measured across the outlet foramen at cardiac catheterization was one of the most reliable predictors of outcome. Survivors had a significantly lower gradient than did nonsurvivors, and no patient with a gradient greater than 66 mm Hg survived. There was no difference between survivors and nonsurvivors with respect to aortic cross-clamp time, bypass time or ventricular-aortic pressure gradient at the end of the surgical procedure. The gradient measured across the outlet foramen at the beginning of operation was significantly lower

Figure 5. Univentricular heart in a 22 monthold boy. The outlet foramen (arrows) is severely obstructed. The right (R) and left (L) atrioventricular valves insert into the left ventricle. The pulmonary trunk (PT) arises from the left ventricle, and the aorta (Ao) emanates from the outlet chamber.

lACC Vol 4, No 4 October 1984 771-8

for survivors than for nonsurvivors. The correlation between the pressure gradient measured at catheterization and that at operation is shown in Figure 3. Survivors were significantly older than nonsurvivors at the time of operation. No patient in our series younger than 10 years of age survived, regardless of the pressure gradient (Fig. 4). Autopsy data. Analysis of the eight available autopsy specimens from patients with outlet foramen obstruction who died during the immediate postoperative period revealed findings similar to those obtained with echocardiography. However, the size of the outlet foramen was greater and the values for aortic root area and percent of predicted left ventricular wall thickness were smaller than those obtained by echocardiography (Table 3). A typical example of the hypertrophied myocardium with a stenotic outlet foramen, as seen in this condition, is shown in Figure 5. Histologic specimens from each of these eight patients showed changes of chronic myocardial ischemia that predated the surgical procedure. Most of the ischemic changes were subendocardial, but some were subepicardial. There were four types of chronic ischemic changes: vacuolization of myocardial cells, patchy fibrosis, nonpatchy or diffuse fibrosis and interstitial granulation tissue (Fig. 6). An ischemic index was calculated by summing the four types of ischemic changes from five slides for each patient (mild = 1, moderate = 2, and severe = 3). This index correlated positively with ST depression for patients who died during the immediate postoperative period (Fig. 7). The ischemic changes for nonsurvivors with and without outlet obstruction are compared in Table 4. The ischemic index was significantly greater (p < 0.05) for patients with outlet obstruction (5.6 ± 3.8) than for those without obstruction (2.1 ± 1.6). The specimens also showed signs of acute ischemia. One patient had evidence of an approximately 1 day old myocardial infarction involving the outlet chamber, although the degree of compromise of myocardial function by this infarction could not be estimated. In the other seven patients, the microfocal changes caused by acute ischemia were not considered severe enough to account for the significant myocardial dysfunction apparent after operation. Follow-up of survivors. The eight patients who survived the immediate postoperative period have been evaluated for a mean of 28 months (minimum 2 weeks, maximum 63 months) after operation. Despite relief of the outlet obstruction as evidenced by a mean pressure gradient of 4 mm Hg across the outlet foramen after the operation, the majority of survivors have continued to have cardiac problems. One patient died 33 months after undergoing a Fontan procedure and resection of his outlet foramen. He had undergone cardiac catheterization 2 weeks before his death, and the pressure gradient across the outlet foramen was 11 mm Hg. He also had a 15% left to right shunt at the oversewn valve, but his repair was otherwise intact. Supraventricular tachycardia had developed 1 month before his death, and

BARBER ET AL SURGICAL REPAIR OF UNIVENTRICULAR HEART

JACC Vo 4, No 4

October I 1'4 771-8

Figure 6. Photomicrographs showing four types of chronic myocardial Ischemia, A, Vacuolization of myocardial cells (magnification x 350), B, Patchy interstitial fibrosis (magnification X 35), C. Diffuse interstitial fibrosis (magnification x 90), D, Active interstitial granulation tissue (magnification x 175). (All hematoxylin-eosin; reduced by 25%.)

Figure 7. Relation between ischemic index (calculated by histologic changes) and maximal ST segment depression. The regression equation is: ischemic index = 1.9 + 1.5 times maximal ST depression. (r = 0.85, P < 0,001). Solid lines represent predicted ischemic index and 5th and 95th percentiles. 18 16

x Q)

'0

s U

E Q)

14

-

12

-

10 8

-

s:

6

-

.!!l

4

-

2

-

0

-

o

-2

o

2

3

4

5

6

MaXimum ST depression (mm)

8

9

775

he had a cardiac arrest after receiving verapamil intravenously during an episode of tachycardia. Autopsy revealed hemorrhagic pulmonary edema, congestive hepatic fibrosis and bilateral thalamic hemorrhages, At the time of death, the left ventricular wall thickness was 140% of predicted normal thickness and there was histologic evidence of chronic myocardial ischemia with mild nonpatchy and moderate patchy fibrosis and vacuolated myocytes. Also, this patient had had the highest preoperative pressure gradient across the outlet foramen (66 mm Hg) of any operative survivor. Another survivor experienced dehiscence of his atrioventricular valve patch and required reoperation 11 months after undergoing a modified Fontan procedure. Subsequent catheterization revealed a 2 mm Hg pressure gradient across his outlet foramen. Fifty months after the modified Fontan procedure, he developed atrial flutter, but he is otherwise doing well. A third operative survivor has exertional dyspnea 18 months after undergoing a modified Fontan procedure. and a fourth remains cyanotic 56 months after a modified Fontan procedure. A fifth patient was doing well 63 months after ventricular septation, but recently, he developed a murmur consistent with a ventricular septal defect. He has not yet undergone repeat catheterization to confirm this diagnosis, The sixth, seventh and eighth patients are

776

l ACC Vol 4. No 4

BARBER ET AL SURGICAL REPAIR OF UNIVENTRICULAR HEART

Oc tobe r I'I X4 771 -X

Table 4. Compari son of Myocardial Ischemic Changes for Nonsurvivors With or Without Outlet Obstruction Chron ic Ischemic Change s Patchy Fibrosis

Vacuolizati on

Diffuse Fibrosis

Granulation Tissue

Grade

Obst*

NorrObsrl'

Obst

NonObst

Obst

NonObst

Obst

NonObst

None Mild Moder ate Severe

6 I 0

7

I 4 I

3

2 4 I I

3 4 I 0

5

5

8 0 0 0

I

I 0 0

2

0 0

2 0 I

Number of patients with obstructed (Obst*) or nonobstructed (NonObstt ) outlet foramen for each grade of chrome ischemic chan ge .

Univentricular heart is a cardiac malformation that has a poor natural history and usually requires surgical treatment. One form of univentricular heart-that with obstructed anterior subaortic outlet chamber-has a particularly high incidence of surgical mortality. This high mortality rate may be due to myocardial dysfunction caused by severe ventricular hypertrophy and ischemia resulting from ventricular outflow obstruction. Our 10 nonsurvivors had a mean left ventricular wall thickne ss that was 190% of the predicted normal value determined by echocardiography. They had significant cardiomegaly by chest roentgeno gram and ST deviation by electrocardiograph y. The ST deviation correlated with signs of chronic subendocardial ischemia histologically. Exclusion of tricuspid atresia. Because the right ventricle in tricu spid atresia may have all the components of a normal though hypoplastic ventricle (37- 42), the question of whether tricuspid atresia constitutes a form of univentricular heart is unresol ved (42- 45). Since the ventricular septal defect may close spontaneously in tricuspid atresia (4 1,46-48) but not, to our knowledge , in the univentricul ar heart , it is unknown whether the natural history or surgical morbidity and mortality of these two lesions is equivalent. Therefore, we excluded patients with tricuspid atresia from our study. We also excluded , when possible , patients with tricuspid atresia from the data of the referenced authors in our comparison charts and tables. Accord ingly , our study include s only those forms of univentricul ar heart that may

tery banding and subsequent development of obstruction of a subaortic outlet foramen (12,15,36). Penkoske et al. (36) suggested that pulmonary artery bandin g in the neonate with univentricular heart and anterior subaortic outlet chamber may be avoided by transection of the main pulmonary artery , anastomosing the proximal pulmonary artery to the aorta and providing a controlled shunt from aorta to the distal pulmonary artery. We have had little experience with this procedure and believe that until more data are available, pulmonary artery banding still may be an acceptable means of treating pulmonary edem a in the neonate with univentricular heart and anterior subaortic outlet chamber. Once pulmonary artery banding has been performed , the patient must be monitored carefull y for development of subaortic outlet obstruction . Further operation is indicated at .the first sign of subaortic outlet obstruction . Penkoske et al. (36) suggested that even a pressure gradient provoked only by isoproterenol may identify the patient at increased operative risk. Elimination of obstruction. Which type of operation is best once outlet foramen obstruction has occurred is unknown . Como et al. (33) recommended that the outlet foramen be enlarged. The use of this techn ique resulted in the death of one patient previou sly reported on (15). Como et al. also recommended bypassing the obstructed outlet foramen by using the proximal pulmonary artery to perfuse the aorta . Penkoske et al. (36) used the techn ique of Neches et a1. (49) to successfull y create an aortopulmonary window in two of their patients . One patient is currently await ing a modified Fontan procedure , and the other died of low cardiac output failure when a modified Fontan procedure was attempted 4 \14 years later. Both of the patients in whom we attempted an aortopulmonary anastomo sis died. One of the

be designated double inlet left ventricle .

patients, however, had a double outlet outlet chamber with

Palliative operations. Many patients with univentricular heart and anterior subaortic outlet chamber require some procedure to control pulmonary edema before 1 year of age. Because of the reported association between pulmonary ar-

severe subpulmonary obstruction . In this patient. a simultaneous resection of the outlet foramen was attempted . "Corrective" operations. McKay et al. (35) used septation of the ventricle as a means of repairing the univen-

asymptomatic 2 weeks, 1 month and 9 months . respectively , after undergoing a modified Fontan procedu re.

Discussion

JACC 'I"l 4, No 4 Octobe 1984:771-8

tricular heart, but they have had no success in the five patients with obstructed outlet foramen, Two other patients with septation described previously (12,31), as well as two of our three patients with septation, died during the immediate postoperative period, From their experience, McKay et al. (35) theorized that reduction in ventricular cavity size and decreased compliance secondary to myocardial hypertrophy, as well as an increased susceptibility to myocardial necrosis at the time of surgery, were the causes of death in their patients with obstructed outlet foramen. Because of the high risk with septation, they recommend a modified Fontan procedure for these patients. Unfortunately, decreased ventricular compliance also make ~ the patient a poor candidate for a modified Fontan procedure. The filling pressure required by a noncompliant left ventricle results in excessive right atrial pressure. In our group, 6 of the 13 patients in whom a modified Fontan procedure was attempted died of low cardiac output within 72 hours, with left atrial pressures as high as 30 mm Hg. In addition, one of the five patients reported on (27,38) also died of low cardiac output during the immediate postoperative period. Resection of outlet foramen by aortopulmonary anastomosis as initial procedure. On the basis of our findings that patients with high grade obstruction (as evidenced by catheterization and surgical gradients across the outlet foramen and suggested by the outlet foramen area referable to aortic root area or body surface area) develop a hypertrophied ventricle and that patients with severe ventricular hypertrophy develop subendocardial ischemia (as evidenced by ST depression and histologic evaluation of our nonsurvivors), we concur with McKay et al. (35) that a satisfactory repair by septation or modified Fontan procedure is unlikely in these patients. A reasonable alternative is to attempt to reduce the outlet obstruction. Thus, although enlargement of the outlet foramen or bypass of it by an aortopulmonary anastomosis may be associated with a high mortality rate, the rate is still less than that of "more corrective," that is, Fontan or septation, procedures. We, therefore, favor resection of the outlet foramen or an aortopulmonary anastomosis in patients younger than 10 years old or those with a high pressure gradient across the foramen. We hope that by reducing the obstruction to aortic flow, the ventricular myocardium will return to a more normal state with improved compliance and less ischemia; at that time, the morbidity and mortality of a modified Fontan procedure or septation may be more acceptable. Although there were no successful repairs in this series in a patient with univentricular heart and obstructed anterior subaortic outlet chamber by this approach, we have used such resection successfully in one patient with tricuspid atresia, transposition of the great arteries and restrictive ventricular septal defect. After pulmonary artery banding, the patient had a 116 mm Hg

BARBER ET AL SURGICAL REPAIR OF UNIVENTRICULAR HEART

777

pressure gradient across his ventricular septal defect. The outlet foramen was resected, and 8 1/ 2 years later with a 17 mm Hg pressure gradient across the ventricular septal defect, he successfully underwent a modified Fontan procedure. Cardiac status of survivors. Even if the patient survives operation, he or she usually is symptomatic. Only our three survivors with the shortest follow-up are asymptomatic. The symptoms in our patients may be related to operation on patients with complicated congenital heart disease in general, such as patch dehiscence or cyanosis, or they may be a direct result of chronic myocardial hypertrophy and ischemia, such as dyspnea or arrhythmias. Only one of two survivors reported on by Moreno-Cabral et al. (27) was asymptomatic I year after operation. The other developed congestive heart failure with poor ventricular function. Penkoske et al. (36) reported that one of two patients who survived a Fontan procedure developed mild to moderate aortic insufficiency and the other has subsequently died (Freedom RM, personal communication). Part of the reason for the poor prognosis in survivors may be decreased ventricular function secondary to the fibrosis caused by chronic myocardial ischemia. The myocardium of our patient who survived his initial repair, but subsequently died, continued to show moderate changes of fibrosis and myocyte vacuolization. Conclusions. Univentricular heart with obstructed anterior subaortic outlet chamber secondary to pulmonary artery banding or natural pulmonary stenosis has a poor natural history and a high operative morbidity and mortality. We hope that newer techniques, such as direct relief of the outlet obstruction or bypass of the obstructed outlet foramen by an aortopulmonary anastomosis as a first stage with a subsequent Fontan or septation procedure, will improve the otherwise dismal outlook in this condition.

References I. Moodie DS. Ritter DG, Tajik JA, O'Fallon WM. Long-term followup in the unoperated umventricular heart. Am J Cardiol 1984;53:1124-8.

2 Muller WH Jr. Dammann JF Jr. The treatment of certain congenital malformations of the heart by the creation of pulmonic stenosis to reduce pulmonary hypertension and excessive pulmonary blood flow: a preliminary report. Surg Gynecol Obstet 1952;95:213-9. 3. Ochsner JL, Cooley DA, McNamara 00, Kline A. Surgical treatment of cardiovascular anomalies in 300 infants younger than one year of age. J Thorac Cardiovasc Surg 1962;43:182-97. 4. WIlham, GR. Cayler GG, Richardson WR, Tassopolous N, Carter DR. Campbell GS. Pulmonary artery banding in infants with congemtal heart defects other than ventricular septal defect. Am Surg 1963;29:160-5.

5 Craig TV, Sirak HD. Pulmonary artery banding: an analysis of 38 cases. J Thorac Cardiovasc Surg 1963;45:599-604. 6. Idnss FS, Riker WL, Paul MH. Banding of the pulmonary artery: a palliative surgical procedure. J Pediatr Surg 1968;3:465-74. 7 Stark J. Aberdeen E. Waterston DJ, Bonham-Carter RE, Tynan M.

778

BARBER ET AL. SURGICAL REPAIR OF UNIVENTRICULAR HEART

lACC Vol 4. No 4 October !'ig4 771·g

Pulmonary artery constriction (banding): a report of 146 cases. Surgery 1969.65:808-18.

atIon in the presence of severe hypoplasia of a pulmonary artery J Thorac Cardiovasc Surg 1980.80:424-6

8. Horsley BL. Zuberbuhler JR. Bahnson HT. Factors influencing survival after banding of the pulmonary artery. A review of 89 cases. Arch Surg 1970:101:776-9.

29. Marcelletti C, Mazzera E, Olthof H, et at. Fontan's operation: an expanded horizon. J Thorac Cardiovasc Surg 1980:80:764-9

9. Hunt CEoFormanek G. Levine MA. Castaneda A. Moller JH. Banding of the pulmonary artery. Results in III children Circulation 1971:43:395-406. 10. Oldham HN Jr. Kakos GS. Jarmakani MM. Sabiston DC Jr. Pulmonary artery banding in infants with complex congenital heart defects. Ann Thorac Surg 1972:13:342-50. 11. Truccone N1. Bowman FO Jr. Maim JR, Gersony WM. Systemicpulmonary arterial shunts in the first year of life. Circulation 1974:49:508-11. 12. Somerville 1. Becu L. Ross D. Common ventricle with acquired subaortic obstruction. Am J Cardiol 1974:34:206-14. 13. Dooley KJ. Parisi-Buckley L. Fyler DC. Nadas AS. Results of pulmonary arterial banding in infancy: survey of 5 years' experience in the New England Regional Infant Cardiac Program. Am J Cardiel 1975:36:484-8.

30. Feldt RH. Mair DD. Danielson GK. Wallace RB, McGoon DC, Current status of the septation procedure for univentricular heart. J Thorac Cardiovasc Surg 1981:82:93-7. 31. Kreutzer GO, Vargas FJ. Schlichter AJ, et at. Atnopulmonary anastomosis. J Thorac Cardiovasc Surg 1982:83:427-36. 32. Nunez L, Aguado MG. Celemm D. Larrea JL. Closure of the right atrioventricular valve in the modified Fontan operation for univentricular heart. Ann Thorac Surg 1982;34:714-5. 33. Como A, Becker AE, Bultenjs AHK, et at. Univentricular heart: can we alter the natural history? Ann Thorac Surg 1982:34:716-26. 34. Eijgelaar A. Hess J, Hardjowijono R. Karliczek GF. Rating W. Homan van der Heide IN. Experiences with the Fontan operation. Thorac Cardiovasc Surg 1982:30:63-8 35. McKay R. Pacifico AD, Blackstone EH, KirklIn JW, Bargeron LM Jr. Septation of the univentncular heart with left anterior subaortic outlet chamber. J Thorac Cardiovasc Surg 1982;84:77-87.

14. Taussig HB. Long-time observations on the Blalock-Taussig operation. IX. Single ventricle (with apex to the left). Johns Hopkins Med J 1976;139:69-76.

36. Penkoske PA, Freedom RM. Williams WG. Trusler GA, Rowe RD. The surgical palliation of subaortic stenosis in the univentricular heart. J Thorac Cardiovasc Surg 1984:87:767-81.

15. Freedom RM. Sondheimer H. Dische R. Rowe RD. Development of "subaortic stenosis" after pulmonary arterial banding for common ventricle. Am J Cardiol 1977;39:78-83.

37. Jordan Jc, Sanders CA. Tricuspid atresia WIth prolonged survival: a report of two cases with a review of the world literature. Am J Cardiol 1966;18:112-9.

16. Sakakibara S. Tominaga S, Imai Y, Uehara K. Matsumura M Successful total correction of common ventricle. Chest 1972:61:192-4.

38. Guller B, Titus JL. Morphological studies in tricuspid atresia. Circulation 1968:38:977-86.

17. Ionescu Ml, Macartney FJ, Wooler GH. Intracardiac repair of single ventricle with pulmonary stenosis. J Thorac Cardiovasc Surg 1973;65:602-7.

39. Folger GM Jr, Witham AC, Ellison RG. Tricuspid atresia with transposition of the great vessels. J Pediatr 1969;74:946-52.

18. Edie RN , Ellis K. Gersony WM. Krongrad E, Bowman FO Jr, Maim JR. Surgical repair of single ventricle. J Thorac Cardiovasc Surg 1973;66.350-9. 19. Somerville J. Ross DN, Yacoub M, Radley-Smith R. Primitive ventricle with acquired subpulmonary stenosis. Eur J Cardiol 1975;3:193-203. 20. Lamberti 11. Thilenius 0, de la Fuente D, Lin CY, Arcilla R, Replogle RL. Right atrial partition and right ventricular exclusion: another surgical approach for complex cyanotic congenital heart disease. J Thorac Cardiovasc Surg 1976;71:386-91. 21. Sharratt GP, Sbokos CG, Johnson AM, Anderson RH, Monro JL. Surgical "correction" of solitus-concordant. double-outlet left ventricle with L-malposition and tricuspid stenosis WIth hypoplastic right ventricle. J Thorac Cardiovasc Surg 1976:71:853-8. 22. Kawashima Y, Mori T. Matsuda H, Miyamoto K, Kozuka T. Manabe H. Intraventricular repair of single ventricle associated with transposition of the great arteries. J Thorac Cardiovasc Surg 1976:72:21-7.

40. Marcano BA, Riemenschneider TA. Ruttenberg HD. Goldberg SJ. Gyepes M. Tricuspid atresia with increased pulmonary blood flow: an analysis of 13 cases Circulation 1969;40:399-410. 41. Rao PS, Sissman NJ. Spontaneous closure of physiologically advantageous ventncular septal defects. Circulation 1971:43:83-90. 42. Van Praagh R. Wise J, Dahl B, Van Praagh S. Single left ventricle with infundibular outlet chamber and tricuspid valve opening only into outlet chamber in a 44-year-old man with thoracoabdommal ectopia cordis without diaphragmatic or pericardral defect: importance of myocardial morphologic method of chamber identification in congenital heart disease. In: Van Praagh R, Takao A. eds. Etiology and Morphogenesis of Congenital Heart Disease. New York: Futura, 1980:379-489. 43. Van Praagh R, Ongley PA. Swan HJC, Anatomic types of single or common ventricle in man. Morphologic and geometric aspects of 60 necropsied cases. Am J Cardiol 1964;13:367-86. 44. Lev M, Liberthson RR, Kirkpatrick JR, Eckner FAO, Arcilla RA. Single (primitive) ventricle. Circulation 1969:39:577-91.

23. Yacoub MH. Radley-Smith R. Use of a valved conduit from nght atrium to pulmonary artery for "correction" of single ventncle. Circulation 1976:54(suppl III):I11-63-70.

45. Anderson RH, Becker AE. Macartney FJ, Shineboume EA. Wilkmson JL. Tynan MJ. Is "tricuspid atresia" a univentricular heart? Pediatr Cardiol 1979: I:51-6

24. McGoon DC. Danielson GK, Ritter 00. Wallace RB. Maloney JD. Marcelletti C. Correction of the univentricular heart having two atrioventricular valves. J Thorac Cardiovasc Surg 1977:74:218-26.

46. Roberts WC, Morrow AG. Mason DT, Braunwald E Spontaneous closure of ventricular septal defect. Anatomic proof in an adult with tricuspid atresia. Circulation 1963;27:90-4.

25 Dory DB, Schieken RM, Lauer RM. Septation of the univentricular heart Transatrial approach. J Thorac Cardiovasc Surg 1979:78:423-30.

47. Gallaher ME. Fyler DC. Observations on changing hemodynamics in tricuspid atresia without associated transposition of the great vessels. Circulation 1967;35:381-8.

26. Gale AW, Danielson GK, McGoon DC. Mair DD. Modified Fontan operation for univentricular heart and complicated congenital lesions. J Thorac Csrdiovasc Surg 1979:78:831-8. 27. Moreno-Cabral RJ, Miller DC. Oyer PE, Stinson EB, Reitz BA, Shumway NE. A surgical approach for S,L,L single ventncle incorporating total nght atrium-pulmonary artery diversion. J Thorac Cardiovasc Surg 1980;79:202-9 28. Sade RM, RIOpel DA, Taylor AB Orthoterminally corrective oper-

48 Meng CCL. Spontaneous closure of ventricular septal defect m tricuspid atresia. J Pediatr 1969;75:697-700. 49. Neches. WHo Park SC, Lenox CC, Zuberbuhler JR, Bahnson HT. Tricuspid atresia with transposition of the great arteries and closing ventncular septal defect. Successful palliation by banding of the pulmonary artery and creation of an aorticopulmonary wmdow J Thorac Cardiovasc Surg 1973;65:538-42