1980 Journal - The Changing Role of Palliative Procedur in Treatment of Infant With CHD

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J THORAC CARDIOV ASC SURG 79: 194-20 1, 1980

The changing role of palliative procedures In


the treatment of infants with congenital
heart disease
The use of palliative procedures in the treatment of infants with congenital heart disease has been an
established and accepted form of therapy. With progression toward more corrective operations in the first
year of life, the more recent use of palliative procedures is examined. Five hundred two infants underwent
cardiac operation in the first year of life at the University of California, San Francisco, during the period
from Jan. 1, 1975, through June I, 1979. Four hundred twenty-three (84.3%) underwent "corrective"
procedures. One hundred forty-seven of them had tetraology of Fallot, ventricular septal defect (VSD),
transposition of the great arteries (TGA), and truncus arteriosus types 1 and 11. These patients, commonly
treated in the past with conventional means of palliation, underwent either' 'correction" or an outflow
patch procedure. The operative survival rate was 91%. During the same period, only 26 (5.2%)
conventional palliative procedures were performed. Pulmonary artery banding was performed in 13,
closed atrial septectomy in two, Blalock-Taussig shunt in five, and Waterston shunt in six. An additional
53 (10.4%) unconventional palliations were performed. Lesions palliated by conventional or
unconventional procedures included the complex malformations of pulmonary, tricuspid, and mitral
atresia, single ventricle, endocardial cushion defect, TGA with VSD, Taussig-Bing deformity, and
Ebstein's anomaly. These data demonstrate the changing role for the palliative procedure in the treatment
of infants with congenital heart disease. Selection of the proper palliative procedure for the complex
lesions not amenable to early correction should be predicated on both the nature of the lesion and the
factors which influence the ultimate surgical repair, the most important of which is induction of
symmetrical growth and development.

Kevin Turley, M.D., William Y. Tucker, M.D., and Paul A. Ebert, M.D.,
San Francisco, Calif.

Conventional palliative procedures in the form of procedures can be accomplished in early infancy with
pulmonary artery banding, closed atrial septectomy, acceptable risk. The magnitude of this change and its
and peripheral shunts have formed the standard by effect on both the use and selection of operative proce-
which survival in infants with congenital heart disease dures in early infancy are examined.
has been judged. They were necessitated by inability to
produce more physiological palliation (' 'correction") Patients and methods
in early infancy without an extremely high operative During the period from Jan. 1, 1975, through June 1,
mortality rate. 1979, 502 patients in the first year of life underwent
Recently, the role of conventional palliative proce- operative procedures on the Cardiothoracic Surgery
dures has changed markedly. Survival is now possible Service at the University of California, San Francisco,
with many types of "correction, " central shunting, and California. The age distribution is noted in Fig. 1.
other unconventional forms of central palliation which These patients can be divided into four groups (Table
result in a more physiological type of palliation. Such I). Group 1 includes those in whom no palliative proce-
dure is possible, and thus correction in infancy is nec-
From the University of California, San Francisco, Department of
Surgery, San Francisco, Calif. 94143.
essary. Group 2 includes patients with four lesions
Read at the Fifth Annual Meeting of The Samson Thoracic Surgical
commonly palliated in the past, in which efficacy of
Society, Sun Valley, Idaho, June 5 to 8, 1979. such conventional palliation has been judged; in this
Address for reprints: Kevin Turley, M.D., Department of Surgery, group correction in early infancy has recently become
University of California, San Francisco, California 94143. common. Group 3 includes patients in whom either
194 0022-5223/80/020194+08$00.80/0 © 1980 The C. V. Mosby Co.
Volume 79
Number 2 Palliation of congenital heart disease 195
February, 1980

Table I. Congenital heart disease 300

Group I Lesions 260


Pump 0
Non-pump ~
Group I Aortic stenosis, pulmonary stenosis, mitral stenosis, 220
total anomalous pulmonary venous connection, cor C-Correction

triatriatum, coarctation, interrupted arch, patent duc-


'"
I-
z P·Paliiation
w 180
tus arteriosus i=
Group 2 Tetralogy of Fallot, transposition of the great arteries
«
Q..
140
(simple), ventricular septal defect, truncus arteriosus 0
types I and II Z
100
Group 3 Transposition of the great arteries with ventricular septal
defect, pulmonary atresia, endocardial cushion defect, 60
truncus arteriosus types III and IV
Group 4 Single ventricle, double-outlet right or left ventricle,
Ebstein's anomaly, tricuspid atresia, mitral atresia,
20
c P c pCP
CL
c P
hypoplastic right or left ventricle
0-3 4-6 7-9 10-12
AGE IN MONTHS
Table II. Modes of therapy Fig. 1. Age distribution with type of cardiopulmonary sup-
Palliation port and type of procedure. Included are 502 infants, treated
in the first year of life, between Jan. I, 1975, and June I,
"Correction' ,
Con-
ventional
I Uncon-
ventional Totals
1979.

in only 26 (5.2%) of the patients; an additional


CPB 271 I 36 308 (61%)
Non-CPB 152
53 (10.6%) underwent unconventional palliative ap-
25 17 194 (38%)
proaches. Table II illustrates these relationships.
Totals 423 (84.3%) 26 (5.1%) 53 (10.6%) 502 One hundred forty-seven of the 308 patients treated
Legend: CPB, Cardiopulmonary bypass. with cardiopulmonary bypass technique had Group 2
lesions. These included 37 with tetralogy of FaIlot, 50
Table III. "Corrective" operations in Group 2 with transposition of the great arteries (TGA), 31 with
infants in the first year of life ventricular septal defects (VSD), and 29 with truncus
arteriosus types I and II. All of these patients were
Lesion No.
severely symptomatic, requiring operative intervention
Tetralogy of Fallot 25 (12*) in early infancy. The approach to these four lesions
Ventricular septal defect 31 (Group 2) is described in Table III. Of note, only one
Transposition of the great arteries 50t conventional palliative procedure was performed in this
Truncus arteriosus types I and II 29
group.
Total 135 During this same period only 26 conventional pal-
*Twelve of the 37 patients with tetralogy of Fallot underwent unconven- liative procedures were performed: pulmonary artery
tional palliative procedures. banding in 13 patients, closed atrial septectomy in
tOne patient underwent a Blalock-Hanlon septectomy at the age of 2 days. A two, and shunting procedures in the form of Blalock-
"corrective" operation (intra-atrial baffle procedure) was performed at the age
of 8 months. Taussig anastomoses in five and Waterston shunts in
six (Table IV).
correction or palliation may be indicated depending on Unconventional procedures for palliation were per-
the anatomy of the lesion and the need for further formed in 53 patients. These include open pulmonary
growth before correction can be performed. Finally, artery outflow tract patching in 12 of the patients with
Group 4 includes patients with lesions for which no tetralogy of Fallot and nine patients with pulmonary
corrective procedure is usually available in infancy and atresia, pulmonary arterial conduit for palliation in one
only with a palliative procedure producing symmetrical patient with pulmonary atresia, main aorta-pulmonary
growth of the pulmonary arteries and cardiac chambers shunts in 10 patients, combined open septectomy and
may any correction be possible at a later date. pulmonary artery bandings in four, and intra-atrial
Cardiopulmonary bypass was utilized in 308 patients baffle procedures combined with pulmonary artery
(61% of the total group) and nonbypass techniques banding in four, In addition, 13 infants underwent for-
in 194 (38%). "Correction" was accomplished in 423 malin injections of the ductus arteriosus early in this
(84.3%) of the patients and conventional palliation, series; however, because of the short-term effective-
The Journal of
196 Turley, Tucker, Ebert Thoracic and Cardiovascular
Surgery

Table IV. Conventional palliative procedures Table VI. Results of "correction" /unconventional
Procedure '-N-O.-o-if-c-a-se-s- palliation in Group 2 infants
Percent
Pulmonary artery banding 13 Lesion survival
Closed atrial septectomy 2
TGA and VSD* Tetralogy of Fallot 37 33 90
TGAt Ventricular septal defect 31 28 90
Blalock-Taussig shunt 5 Transposition of the great arteries 50 50 100
Pulmonary atresia 2 Truncus arteriosus types I and" 29 23 79
Pulmonary atresia with single ventricle I
Pulmonary atresia with TGA I Totals 147 134 91
Pulmonary atresia, tricuspid atresia, and hypo- I
plastic right ventricle
Waterston shunt 6
Table VD. Deaths in Group 2 patients
Pulmonary atresia 4
Pulmonary atresia and mitral stenosis I Lesion Complicating factors
Pulmonary atresia, tricuspid atresia, and hypo- I
plastic right ventricle Tetralogy of 7 mo 7 Outflow patch rupture
Fallot 6 mo 21 PA: Ao ratio 0.25, NEC,
Legend: TGA, Transposition of the great arteries. VSD, Ventricular septal
defect. postop. pulm. hemorrhage,
*Closed atrial septectomy combined with pulmonary artery banding. terratoma
tOne Group 2 patient. 5 mo 2 PA:Ao ratio 0.27, rneningo-
myelocele
(OTP) 6 mo 4 Illeal atresia, preop. seizures
Table V. Palliated lesions Ventricular 14 days OR Severe CHF, on respirator
septal from birth
No. of defect 25 days OR Severe CHF, on respirator
Lesions patients from birth
45 days 8 Gastroschisis, severe CHF
Pulmonary atresia 37
Tricuspid atre- 106 days I PVOD, R,,:Rs 0.48, Q,,:Qs
Ebstein's anomaly I
sia types I 1.5: I
Hypoplastic left ventricle I
and " 40 days Truncal valve incompetence
Tricuspid atresia 7
requiri ng replacement
Mitral atresia 5
14 days Severe CHF, on respirator
Endocardial cushion defect 4
from birth, weight 2,580
Single ventricle 2
gm
TGA with single ventricle 4
5 days OR Preop. arrest, PVOD; at au-
TGA with VSD 3
topsy, marked medial hy-
Corrected TGA I
pertrophy of pulm. vessels
Previous Taussig-Bing shunt I
137 days 2 PVOD; at autopsy, marked
Total 66 medial hypertrophy of
pulm. vessels
Legend: TGA, Transposition of the great arteries. VSD, Ventricular septal
20 days OR PVOD; at autopsy, marked
defect.
medial hypertrophy of
pulm. vessels
ness of this procedure, it rarely is used currently.
Legend: PA:Ao, Pulmonary artery to aorta (anteroposterior projection-
The 66 complex lesions (Groups 3 and 4) in which right PA-Ao diameter). NEC, Necrotizing enterocolitis. CHF, Congestive
palliative procedures were used are described in heart failure. OTP. Outflow tract patch. OR. Operating room. PVOD, Pul-
monary vascular obstructive disease. RI':R s, Ratio of pulmonary to systemic
Table V; the diversity and complex nature of these resistance. Qp:Qs. Ratio of pulmonary to systemic flow.
lesions can be appreciated.
survival. Overall, the operative survival rate was 91%.
Results These results are described in Table VI. Only a single
One hundred forty-seven patients underwent "cor- patient with a Group 2 lesion underwent a conventional
rection " or unconventional palliation for the four le- palliative procedure during this period. This patient,
sions once commonly palliated in early infancy, This who had TGA, subsequently required correction in the
group included 37 patients with tetralogy of Fallot, form of an intra-atrial baffle procedure in the first year
with a 90% survival; 31 patients with VSDs, 90% sur- of life. Twelve patients in this group with tetralogy of
vival; 50 patients with TGA, 100% survival; and 29 Fallot underwent unconventional palliative operations
patients with truncus arteriosus types I and II, 79% in the form of outflow tract patches, as described by
Volume 79
Number 2 Palliation of congenital heart disease I 97
February, 1980

Table VIII. Palliative procedures


Conventional" Unconventional"

Lesion PAB I B-H I B-T IWat, RVOTP


IA-P shunt
IConduit
IOpen sept.j Baffie
and banding and banding
IDuctal
injection

Pulmonary atresia 2 (0) 4 (1) 9 (3) 1 (1) 1 (0) 8 (0)


Pulmonary atresia, single ventricle 1 (0) 2 (1) 2 (0)
Pulmonary atresia, TGA, single ven- 1 (0) 2 (0)
tricle
Pulmonary atresia, mitral stenosis 1 (0)
Pulmonary and tricuspid atresia, hypo- 1 (0) 1 (0) 1 (0)
plastic right ventricle
Endocardial cushion defect 4 (1)
Single ventricle 2 (0)
Tricuspid atresia 5 (2) 2 (1)
Mitral atresia 4 (1) I (1)
TGA, VSD 2 (1) 1(0)
TGA, single ventricle 1 (0) 3 (0)
Corrected TGA 1 (1)
Hypoplastic left ventricle I (0)
Taussig-Bing I (0)
Ebstein's anomaly 1(0)
[TGA]t I (0)
[Tetralogy of Fallot]'t 12 (1)
Totals 13 (3) 2 (0) 5 (0) 6 (1) 21 (4) 10 (4) 1(0) 4 (2) 4 (1) 13 (0)

Legend: PAB, Pulmonary artery band. B-H, Blalock-Hanlon septectomy. B-T, Blalock-Taussig shunt.Wat. Waterston shunt. RVOTP, Right ventricular outflow
tract patch. A-P, Aorta-Pulmonary shunt. TGA, Transposition of the great arteries. VSD, Ventricular septal defect.
"Figures in parentheses indicate deaths.
tBrackets indicate Group 2 lesions.

Tucker and associates, t and will require subsequent (41) palliative procedures during the same period
VSD closure. (excluding the 12 patients subjected to outflow tract
The 13 deaths are reviewed in Table VI. Improved patching who had tetralogy of Fallot and the single
survival through the use of a two-stage approach may patient with TGA and a Blalock-Hanlon anastomosis).
have been possible in the two patients with tetralogy of The majority of these patients (37) had variants of pul-
Fallot with right pulmonary artery-ascending aortic monary atresia, including pulmonary atresia with single
ratios below 0.3 (diameter in anterior projection'). In ventricle, pulmonary atresia with TGA, pulmonary at-
these two, right ventricular outflow tract patches with- resia with mitral stenosis, and pulmonary atresia with
out VSD closure might have resulted in survival. A tricuspid atresia and hypoplastic right ventricle. The
technical problem that occurred in one patient with te- operative survival rate was 76%. Four patients had se-
tralogy of Fallot, outflow patch disruption, might have vere endocardial cushion defects (75% survival); two
been avoidable had a shunt been used. The severity of patients had single ventricle (100% survival) ; seven
the congestive failure present in the three infants with patients had tricuspid atresia (57% survival); five pa-
VSDs necessitated their operations in early infancy. tients had mitral atresia (60% survival); four patients
Two of the three had received respirator support from had TGA and single ventricle (100% survival); and
birth. Pulmonary vascular obstructive disease was pre- three patients had TGA with large VSD (67% survi-
sent in four of the six patients with truncus arteriosus val). A single patient with corrected TGA failed to
who died. Finally, one patient had trucus valvular in- survive, and one patient with Ebsteins anomaly, one
competence, and four of the six patients were 2 months with hypoplastic left ventricle, and one with Taussig-
old or younger, so that the ventricular reconstructi ve Bing anomaly had successful palliation. Overall survi-
operation was more difficult. These deaths are noted in val for the palliated group was 74%. The operative
Table VII. None of these latter patients were better approaches and results (both conventional and uncon-
candidates for a two-stage approach. ventional) in the complex Group 3 and 4 lesions in
Sixty-six patients, all with Group 3 or 4 lesions, which palliation was employed are summarized in
underwent either conventional (25) or unconventional Table VIII.
The Journal of
198 Turley, Tucker. Ebert Thoracic and Cardiovascular
Surgery

Results of this series demonstrate the change in the encouraging results'-" Finally, in patients with truncus
use of palliative procedures which occurs when an early arteriosus types I and II, in whom a greater than 50%
corrective approach is employed. They demonstrate mortality from banding had been commonplace,": 9 a
that correction or central palliation of Group 2 lesions "corrective" procedure is now available. to
can be performed with acceptable survival (Table VI). These "corrections," it should be noted, represent
When such an approach was employed, the need for pal- only a more physiological form of palliation. Although
liative procedures was confined to certain Group 3 pa- some may result in a nearly normal heart (e.g., VSD),
tients who were poor candidates for early correction, in a persistence of some pathological condition (e.g.,
whom a better two-stage result was anticipated, and pulmonary insufficiency in tetralogy of Fallot, and
also to those Group 4 patients in whom no early correct- ventricular reversal in Mustard and Senning procedures
ive procedure was currently available. Selection of the for TGA) or the necessity for subsequent operations
mode of palliation of these patients was based on the (e.g., conduit replacement in truncus arteriosus) may
goal of maximal long-term palliation and symmetrical be expected. These procedures, however, do allow for
growth, and a central palliative approach was employed normal growth and development while fulfilling the
whenever possible. Comparable survival rates are goals for which the conventional palliative procedures
noted in the complex lesions treated by both these un- were originally designed. Furthermore, the infant sur-
conventional palliative procedures and the few conven- vival rate is high.
tional procedures performed, further supporting their The question arises, which lesions now necessitate
use (Table VIII). palliation and how might this best be accomplished?
Table V lists the types of lesions necessitating pallia-
Discussion tion when the "corrective" approach is employed.
Treatment of infants in the first year of life with These include lesions in which the anatomy in infancy
congenital heart disease until recently has depended is inadequate to allow a "corrective" procedure and
upon conventional palliative procedures in the form of further growth is necessary (e.g., pulmonary atresia),
pulmonary artery bands, closed atrial septectomy, and lesions for which both correction and palliation result in
pulmonary-systemic shunt procedures (Blalock-Taus- a high mortality rate but later correction yields a more
sig, Waterston, Potts, and Glenn). These procedures satisfactory long-term result (e.g., certain types of
are designed, to allow survival in the infant groups so TGA with large VSD, certain endocardial cushion de-
that correction may be accomplished at an older age. fects, and certain types of single ventricles), and those
With the exception of those lesions for which no pallia- lesions for which no satisfactory "corrective" ap-
tion is applicable (Group I), this two-stage approach proach is now available and maximal palliation is de-
has been commonly applied. sired. The selection of the proper mode of palliation
Conventional palliative procedures are designed to requires attention to these goals and an appreciation of
alter the hemodynamic effects which result in infant the limitations as well as the advantages of each ap-
death. These include increased pulmonary blood flow proach.
with subsequent development of pulmonary vascular Pulmonary arterial banding is the only modality cur-
obstructive disease or progressive congestive heart rently available to decrease pulmonary arterial flow in
failure, inadequate intracardiac mixing in lesions with patients with severe congestive heart failure. Without
pulmonary and systemic systems in parallel, and in- banding or "correction, " pulmonary vascular obstruc-
adequate pulmonary arterial flow owing to right ven- tive disease causes death from progressive congestive
tricular outflow obstructive lesions. Although such heart failure or else growth retardation occurs. There is
conventional palliative procedures have been applied in a substantial operative mortality rate both with the
all patients in whom palliation is possible (Groups 2, 3, banding procedure itself (in those without simple
and 4), in the past their efficacy has been judged by VSDs) and when subsequent "correction" is per-
results in several commonly treated lesions, namely formed.s: 9 The source of these problems lies in the
tetralogy of Fallot, VSD, TGA, and truncus arteriosus nature of the procedure itself; an inadequate or overly
types I and II. constrictive band may be placed, and pulmonary arte-
Primary "correction" of these Group 2 lesions has rial stenosis or aneurysm formation, pulmonary arterial
recently been reported. The results of "correction" of rupture, or kinking of one of the distal vessels may
tetralogy of Fallot reveal both a lower initial mortality result. 6 Thus the operation should be restricted to those
rate and a lower cumulative mortality rate.t' Like- in whom no correction is available or significantly
wise, treatment of symptomatic VSD and TGA yields improved survival results with its use.
Volume 79
Number 2 Palliation of congenital heart disease I 99
February, 1980

Fig. 2. Central palliations: A, Outflow tract patch. B, Conduit. C. Polytetrafluoroethylene tube.

Atrial or ventricular septectomy or intra-atrial baffle monary arterial hypertension, pulmonary vascular ob-
procedures can produce intra-atrial mixing in those pa- structive disease, kinking of the pulmonary artery, uni-
tients with parallel systemic and pulmonary systems or lateral pulmonary arterial hypoplasia, progressive
those in whom parallel flow is present (single ventricle) stomal stenosis, and stomal growth with congestive
or atrial outflow is obstructed. The closed atrial septec- heart failure have been reported.tv 16, 17 Further, dis-
tomy (Blalock-Hanlon) was developed to achieve this tortion of the pulmonary arterial anatomy and the
aim without the use of cardiopulmonary bypass. Use of necessity for reconstructive procedures for stomal clo-
this approach prior to the development of the balloon sure at the time of correction are common, particularly
atrial septostomy and infant "corrective" procedures in patients with Waterston shunts, and their use in
allowed some salvage and produced acceptable pallia- Group 3 and 4 patients yields the poorest results.!"
tion (in simple transposition) until "correction" could Central shunts (Fig. 2) in the form of outflow patches,
be accomplished. II With the advent of "correction" in pulmonary arterial conduits (rarely used except when
infancy, its usefulness must be questioned. 12 Those le- the need for pulmonary valve placement exists), or
sions which now require mixing procedures (Table V) main aorta-pulmonary arterial communications utiliz-
are those in which the potential for correction is ing a polytetrafluoroethylene tube provide systemic
limited. Thus a procedure producing maximal mixing flow and symmetrical growth of the pulmonary arterial
and long-term palliation (i.e., open septectomy or system. In the latter, anastomosis size can be restricted
baffle procedure) appears warranted. both by cross-sectional area and length of the graft
Finally, there remain those lesions, the most com- placed. 19, 20 These methods allow central palliation,
mon group, in which pulmonary arterial blood flow is avoid the problems of stomal growth and arterial kink-
inadequate and those in which growth of the pulmonary ing, and decrease the necessity for subsequent recon-
tree must be stimulated for "correction" to be possible. struction procedures. These central shunts can provide
Several conventional shunting procedures have been many of the advantages afforded by •'corrective" pro-
utilized toward this end. The Blalock-Taussig, Water- cedures in Group 2 patients until "correction" can be
ston, and Potts procedures all produce aorta-pulmonary performed in Group 3 and 4 patients.
continuity with resultant increased flow. However, Thus the role of palliative procedures and particu-
each often is a unilateral shunt which ideally (as re- larly the conventional form of such procedures has
ported in some patients with tetralogy of Fallor'") markedly changed. No longer can they be appraised
should produce symmetrical growth of the pulmonary against survival in infancy alone. No longer do the
artery system, but more commonly has a high compli- good risk (Group 2) patients form the standard by
cation rate when applied to the infant group. This is which their efficacy is judged. Normal growth and de-
true in particular in those Group 3 and 4 lesions in velopment, long-term palliation in those in whom no
which such palliations must now be employed. 14, 15 correction is available, and the quality of the palliative
Shunt occlusion, unilateral pulmonary edema, pul- results when •'correction " is performed form the stan-
The Journal of
200 Turley, Tucker, Ebert Thoracic and Cardiovascular
Surgery

dard by which each procedure should be evaluated in 16 Greenwood RD, Nadas AS, Rosenthal A, Freed MD,
the treatment of congenital heart disease in infancy. Bernhard WF, Castenada AR: Ascending aorta-pulmo-
nary artery anastomosis for cyanotic congenital heart dis-
REFERENCES ease. Am Heart 194:14-20, 1977
17 Laks H, Marco 10, Willman VL: The Blalock-Taussig
Tucker WY, Turley K, Ullyot 01, Ebert PA: Manage-
shunt in the first six months of life. 1 THoRAc CAR-
ment of symptomatic tetralogy of Fallot in the first year of
DIOVASC SURG 70:687-691,1975
life. 1 THoRAc CARDIOVASC SURG 78:494-502, 1979
18 Lennox SC: Late results of aortopulmonary shunts,
2 Calder AL, Barratt-Boyes BG, Brandt PWT, Neutze 1M:
Modern Cardiac Surgery, DB Longmore, ed., Baltimore,
Postoperative evaluation of patients with tetralogy of Fal-
1978, University Park Press, pp 219-221
lot repaired in infancy. 1 THoRAc CARDIOVASC SURG
19 Gazzaniga AB, Lamberti 11, Siewers RD, Sperling DR,
77:704-720, 1979
Dietrick WR, Arcilla RA, Replogle RL: Arterial prosthe-
3 Daily PO, Stinson EB, Griepp RB, Shumway NE: Tetral-
sis of microporous expanded polytetrafluoroethylene for
ogy of Fallot. Choice of surgical procedure. 1 THoRAc
construction of aorto-pulmonary shunts. 1 THoRAc CAR-
CARDIOVASC SURG 75:338-343, 1978
DIOVASC SURG 72:357-363, 1976
4 Starr A, Bonchek LI, Sunderland CO: Total correction of
20 Miyamoto K, Zavanella C, Lewin AN, Subramanian S:
tetralogy of Fallot in infancy. 1 THoRAc CARDIOVASC
Aorta-pulmonary artery shunts with expanded polytet-
S URG 65:45-57, 1973
rafluoroethylene (PTFE) tube. Ann Thorac Surg 27:413-
5 Blackstone EH, Kirklin rw, Bradley EL, DuShane JW,
417, 1979
Applebaum A: Optimal age and results in repair of large
ventricular septal defects. 1 THoRAc CARDIOVASC SURG
72:661-679, 1976
6 Turley K, Ebert PA: Total correction of transposition of Discussion
the great arteries. Conduction disturbances in infants less DR. GEORGE G. LINDESMITH
than three months of age. 1 THoRAc CARDIOVASC SURG Los Angeles. Calif.
76:312-320, 1978 I would like to congratulate Dr. Turley and his colleagues
7 Zavanella A, Subramanian S: Review. Surgery for trans- for truly outstanding results in the surgical management of
position of the great arteries in the first year of life. Ann infants with cardiovascular disease.
Surg 187:143-150, 1978 At the Childrens Hospital of Los Angeles, we basically
8 Mahle S, Nicoloff OM, Knight L, Moller lH: Pulmonary agree with the concept of performing an intracardiac repair as
artery banding. Long-term results in 63 patients. Ann an initial procedure when feasible. A brief look at the trends
Thorac Surg 27:216-224, 1979 in our experience with systemic-pulmonary shunts will rein-
9 Dooley Kl, Parisi-Buckley L, Fyler DC, Nadas AS: Re- force this agreement.
sults of pulmonary arterial banding in infancy. Survey of The first slide shows our total experience with systemic-
5 years' experience in the New England regional infant pulmonary artery shunts, the bulk of which have been
cardiac program. Am 1 Cardiol 36:484-488, 1975 Taussig-Blalock shunts. During the interval from 1946 to
10 Ebert PA, Robinson sr, Stanger P, Engle MA: Pulmonary 1973, 84% of the patients were over I year of age at the time
artery conduits in infants younger than six months of age. of the shunt operation: beyond that time, 62% of the patients
1 THoRAc CARDIOVASC SURG 72:351-356, 1976 were under I year of age, with 28% of them under I month.
II Gutgesell HP, McNamara DG: Transposition of the great The next slide shows our experience with the Blalock-
arteries. Results of treatment with early palliation and late Taussig operation for children under I year of age, during the
intracardiac repair. Circulation 51 :32-37, 1975 years surveyed by the authors. This slide simply lists the
12 Clarkson PM, Barratt-Boyes BG, Neutze 1M, Lowe JB: diagnoses for which this operation was performed. The diag-
Results over a ten-year period of palliation followed by noses were generally those for which there was no intracar-
corrective surgery for complete transposition of the great diac repair readily available for the patient. The majority of
arteries. Circulation 45:1251-1258, 1972 these patients were in the first 2 months of life.
13 Gale AW, Arciniegas E, Green EW, Blackstone EH, Finally, I would like to comment on some of the concepts
Kirklin lW: Growth of the pulmonary anulus and pulmo- which were touched upon by the authors, hoping that this will
nary arteries after the Blalock-Taussig shunt. J THORAC generate a response from Dr. Turley.
CARDIOVASC SURG 77:459-465, 1979 Those of us involved in the care of children with congenital
14 Idriss FS, Cavallo CA, Nikaidoh H, Paul MH, Koopot R, heart disease must be very careful in our use of the terms
Muster Al: Ascending aorta-right pulmonary artery "palliative" and "corrective. " Dr. Turley has addressed this
shunt. 1 THoRAc CARDIOVASC SURG 71:49-57, 1976 point in the manuscript, but I think further emphasis is war-
15 Aziz KU, Olley PM, Rowe RD, Trusler GA, Mustard ranted. The fact is that relatively few of the lesions which we
WT: Survival after systemic to pulmonary arterial shunts treat surgically can be proved as yet to be other than palliated
in infants less than 30 days old with obstructive lesions of by the surgical procedures which we perform. This applies in
the right heart chambers. Am 1 Cardiol 36:79-482, 1975 particular to the diagnosis of TGA and the treatment of trun-
Volume 79
Number 2 Palliation of congenital heart disease 20 1
February, 1980

cus arteriosus, Tetralogy of Fallot, for that matter, probably In regard to surgical therapy, these patients all required
also can be included in this category. I realize that this con- operation in infancy, not as an elective approach, because
cern is purely semantic. However, the inappropriate use of they were severely symptomatic. In all four of the Group 2
the term "corrective" frequently supplies an incorrect impli- lesions, we maintain that the severely symptomatic patient
cation to both the referring doctor and the patient's family. can be most successfully treated by an early corrective repair.
Second, I would like to clarify our position on the use of As to the question of the relation of age to death rate, there
intracardiac repair in children under I year of age. In several was no difference between patients with TGA who were less
of the diagnoses discussed by the authors, this age grouping than 3 months of age and those older than 3 months of age. In
provides an unrealistic view of the overall results. In TGA, the tetralogy group no deaths occurred in the 14 patients less
for instance, we find that the results of intracardiac repair than 3 months of age, and all three deaths in the group with
beyond the age of 3 months are exactly comparable with the VSD occurred in severely ill infants, two of whom had re-
results of this operation in any older age group. With tetral- quired ventilator support from birth. The patients in whom
ogy of Fallot, we have found the break point to be somewhere complex intraventricular repair is necessary, for example,
around 6 months of age. With intracardiac repair of this lesion those with truncus arteriosus, are at a higher risk. The prob-
beyond age 6 months, the results obtained are comparable lem lies with the ventriculotomy and the nature of the ven-
with results obtained in older age groups. tricular tissue in the first 6 weeks, for the ventricle in these
With this in mind, I think it would be appropriate for the infants is edematous and much more difficult to suture ef-
authors to comment more specifically on their results in the fectively.
very young age groups, specifically, those under 2 months of As I stated, however, these were severely symptomatic
age. patients and the alternative would have been pulmonary artery
In viewing the reports from other centers addressing this banding, which in our hands has been unsuccessful in the
particular age group, we believe that the overall results fail to infant with truncus arteriosus.
justify a tremendous enthusiasm to press for intracardiac re- Our method of cardiopulmonary support in these patients
pair in these very young infants. had been to restrict the use of deep hypothermia and circula-
Finally, I would like to report that at this time we still favor tory arrest to those less than 6 months of age, in whom the
the Blalock-Taussig operation as the most desirable operative repair can be done during less than 40 minutes of
systemic-pulmonary shunt; we use it almost exclusively. I circulatory arrest, for example, the transposition group.
believe this operation can be performed with a high degree of In the case of the ventricular repairs, for example, the
success and with minimal mortality and morbidity. We prefer truncus group, we have used core cooling to approximately
to use it on the right side, and we use interrupted suture 28° C and low flow during the intraventricular portion of the
technique much in the same fashion as we do with coronary repair, flows of about 10 cc/kg, allowing a relatively blood-
arteries. At the time of anastomosis, we administer heparin less field during the time of actual ventricular repair.
before clamping the vessels.

DR. T U R LEY (Closing)


Dr. Lindesmiths recent data support our finding that in-
creasingly Group 3 and group 4 patients constitute the paral-
leled group.

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