- Email: [email protected]
Complete atrioventricular canal associated with tetralogy of Fallot
Complete atrioventricular canal associated with tetralogy of Fallot Clinical experience and operative methods Data on 8 children with the combined anomalies of complete atrioventricular canal and tetralogy of Fallot are presented. Six of the children had only a palliative procedure, and only 1 of these patients is doing relatively well at the present time from a clinical standpoint. Two of the remaining 5 children undergoing palliative procedures have not been helped significantly, and 3 of the children in this group subsequently died. Two children have had an attempted total correction of this complicated intracardiac problem. One of these children, in whom the diagnosis of atrioventricular canal was not made preoperatively, died in the early postoperative period. In the other child, the correct diagnosis was made preoperatively, and a satisfactory, totally corrective procedure was achieved. The patient is asymptomatic at this time.
R. Darryl Fisher, M.D., David K. Bone, M.D., Richard D. Rowe, M.D., and Vincent L. Gott, M.D., Baltimore, Md.
he
association of the complete form of atrioventricular canal with tetralogy of Fallot is a recognized, but rare, cardiac malformation. This uncommon associated anomaly occurs in less than five per cent of patients with complete endocardial cushion defects. Until recently, attempts at total correction of atrioventricular canal were often unsuccessful; however, with newer surgical techniques " 2 and a more precise definition of the intracardiac anatomy," the mortality rate for repair of this serious defect has been reduced.' Despite success with total correction of atrioventricular canal, attempts at repair of this malformation associated with other cardiac defects have not been as successful. Between 1960 and 1974, 8 patients have required surgical treatment for complete From the Departments of Surgery and Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, Md. 21205. Supported in part by the National Institutes of Health Training Grant No. 5TOI GM 01541. Received for publication March 11, 1975.
atrioventricular canal and associated tetralogy of Fallot at The Johns Hopkins Hospital. The diagnostic experience and the operative methods with this group form the basis of this report.
Oinical material Eight patients evaluated at The Johns Hopkins Hospital from 1960 to 1974 with complete atrioventricular canal and associated tetralogy of Fallot have undergone palliative or corrective operations (Table I). Three patients were younger than 2 years at the time of their first operative procedure, whereas the remaining patients ranged in age from 3 to 13 years. All patients experienced severe cyanosis and diminished exercise tolerance in the period before operation; rising hematocrit values (ranging from 46 to 81 per cent) and increasing heart size were found in each patient before operation. The electrocardiogram in all except Patient J. D. had left axis deviation with a counterclockwise loop in the frontal plane. Five of 8 patients had Down's syndrome and 1
265
266
The Journol of Thorocic ond Cordiovosculor Surgery
Fisher et al.
Table I. Summary of the experience with 8 patients undergoing operative procedures for complete atrioventricular canal and associated tetralogy of Fallot Patient
M.e.
C.H.
Preoperative cath diagnosis
Atrioventricular canal and tetralogy of Fallot Tetralogy of Fallot
Operative procedures (age at time ofoperation)
Associated disease
Down's syndrome
Brock procedure (10 yr.)
None
Result
Doing relatively well clinically
W.O.
Atrioventricular canal and tetralogy of Fallot
Mental retardation
A.N.
Atrioventricular canal and tetralogy of Fallot Atrioventricular canal and tetralogy of Fallot Atrioventricular canal and tetralogy of Fallot Tetralogy of Fallot
Down's syndrome
Died 3 months after operation with severe congestive heart failure Blalock-Taussig shunt (3 yr.) Temporary relief after first shunt Brock procedure (13 yr.) Died in postop. period after Brock procedure Blalock-Taussig shunt (20 mo.); No improvement Waterston shunt 2 days later
Down's syndrome
Blalock-Taussig shunt (4 mo.); Waterston shunt 15days later
Down's syndrome
Waterston shunt (4 mo.)
Died during surgery before shunt constructed
Down's syndrome
Right Blalock-Taussig shunt (5 yr.); left Blalock-Taussig shunt (7.5 yr.); total correction (9 yr.)
Temporary relief from shunts
W. J.
L. D.
J. D.
D.M.
Atrioventricular canal and tetralogy of Fallot
None
Brock procedure (8 yr.)
Blalock-Taussig shunt (4 yr.); total correction (8 yr.)
other was mentally retarded. The remaining 2 patients were of normal intelligence. Preoperative cardiac catheterization revealed tetralogy of Fallot malformation in each patient. In 6 of the 8 patients, the presence of a complete atrioventricular canal was established by preoperative cardiac catheterization. In the other 2 patients, the presence of the associated atrioventricular canal was not suspected prior to the operative procedure. In Patient J. D. the diagnosis was made during the open-heart procedure, and in Patient C. H. the diagnosis was made at postmortem examination 3 months after a Brock procedure. Retrospective analysis of the cineangiograms suggested the diagnosis in 1 of these patients, whereas in the other the cineangiograms could not be interpreted as suggesting an endocardial cushion
No improvement
Died in early postop. period after attempted total correction Temporary relief from shunt; asymptomatic after total correction
defect despite knowledge of the malformation. A variety of operative procedures was employed for this group. An infundibulectomy was carried out in 3 patients as a palliative procedure. One patient (M. C.) was relieved of symptoms and is now leading a relatively normal but restricted life. Two patients died after the Brock procedure. One of these patients (W. D.) with these combined malformations died early in our experience; at the present time, he certainly would be submitted to a totally corrective procedure. The other patient (C. H.) died 3 months following the Brock procedure. At the time of his death, he was being treated for moderately severe congestive failure with pulmonary valve regurgitation. Five patients underwent systemic-pulmo-
Volume 70
Atrioventricular canal with tetralogy of Fallot
Number 2
267
August, 1975
Fig. 1. Preoperative chest film for Patient D . M. before the totally corrective procedure.
Table II. Preoperative and postoperative cardiac catheterization findings in an 8-year-old boy (D. M.) undergoing correction of tetralogy of Fallot and complete atrioventricular canal* Preoperative Pulmonary arter y pressure (mm. Hg) 14/5 Right ventricular pressure (mm. Hg) 90/0 Infundibular gradient (mm. Hg) 76 Left ventricular angiogram Mild mitral regurgitation
Postop erative
18/5 29/0
II
Mild mitral regurgitation
'There were no shunts.
nary artery shunting procedures as their primary operation. In 2 patients (A . N. and W. J .) the Blalock-Taussig shunts were not adequate and were followed in a few days with a Waterston shunt. In both of these patients, no significant improvement was obtained. Three patients having a BlalockTaussig shunt (W. D., J. D., and D. M.) obtained temporary relief of symptoms ranging from 3 months to 10 years. Patient W. D. required a Brock procedure 10 years
Fig. 2. Preoperative left (A) and right (B) ventriculograms for Patient D . M.
later and died during the early postoperative period. Patients J. D. and D. M. both obtained temporary improvement until total correction was undertaken. The final patient to have an attempted shunt procedure (L. D. ) died during the operation before the shunt could be constructed. Complete correction of this combined anomaly was attempted in 2 of the patients. One patient (1. D.) underwent open-heart surgery at the age of 9 years after 2 previous
268
The Journol 01 Thoracic and Ccrdiovosculcr
Fisher et al.
Surgery
I I
R .<:l. h ·ium
R i m of
ait'iul ::lep ial defect
ri'l -::is i o n in an terior a n d p os i e r io r leaflets Fig. 3. A, View of complete endocardial cushion defect through right atriotomy. B, Incisions in the common leaflets of the atrioventricular valve permit exposure of the top of the ventricular septal defect. Interrupted sutures have been placed in the cleft of the mitral valve.
Blalock-Taussig shunts (age 5 and 7 years, 6 months). Unfortunately, the diagnosis of an associated endocardial cushion defect had not been made before the operation and he died in the early postoperative period of low cardiac output. In the other patient (D. M.), an 8-year-old boy, this complex malformation was correctly diagnosed preoperatively. His clinical summary is presented in detail. Case report This child was the product of an uneventful pregnancy and delivery. At 6 weeks of age a cardiac murmur was first heard, and a diagnosis of ventricular septal defect and infundibular pulmonic stenosis was made by cardiac catheterization. At 6 months of age, the child developed severe cyanosis and tachypnea when an episode of
supraventricular tachycardia occurred. These symptoms were relieved by digitalis and he remained well for the next 3 years. At the age of 4 years, he was first seen at The Johns Hopkins Hospital with mild cyanosis and tachypnea. Cardiac catheterization revealed a systemic right ventricular pre ssure and a dextraposed aorta. In addition, a complete atrioventricular canal malformation was noted. A right Blalock-Taussig anastomosis was then performed and afforded complete relief of symptoms. The hematocrit value fell from 75 to 45 per cent in the months following the operation. Because of decreasing exercise tolerance, radiographic evidence of cardiac enlargement, and right ventricular strain by electrocardiography, he was admitted at the age of 8 years for total correction of the tetralogy of Fallot and the endocardial cushion malformation. Upon admission, he was an active, alert boy with moderate cyanosis and clubbing. His weight was 21.6 kilograms. A right ventricular heave was present, and the second
Volume 70 Number 2
Atrioventricular canal with tetralogy of Fallot
August , 1975
269
T o p of ve n f v-i c u laY' .: sept u m
71'1".:. / jj;,:
~
"Da c ~' o n
patch to be 5u fu Y'ed fa ventY'icutar- sep t u m
SUf UY' e pa 5 ses Iht"ouQh patch z;nd h'1'cu sp i d
leaflet
,,
,
1 /
"
/
Mlh': : I
I
/
P ~tch
Ie fle l
Fig. 4. A , Placement of interrupted sutures in the ventricular septum and in turn into the Dacron patch. B, Approximation of the medial leaflets of the mitral and tricuspid valves to the Dacron patch. heart sound was single. A Grade 3/6 ejection murmur was present at the lower left sternal margin. The hematocrit value was 54 per cent. The electrocardiogram revealed left axis deviat ion, right ventricular hypertrophy, and right bundle branch block . Moderate biventricular enlargement was noted by chest roentgenography (Fig. 1). Cardiac catheterization pro vided the pressure data listed in Table II and the ventriculographic studies in Fig. 2. On Oct. 18, 1972, the patient underwent total correction of the atrioventricular canal and right ventricula r outflow tract obstruction. The right Blalock-Taussig shunt was ligated after extracorporeal circulation was begun. A complete endo cardial cushion defect (Type III) was identified through a right atriotomy (Fig. 3, A), and this was repaired by the method originated by Rastelli and his associates. > The common leaflets of the atrioventricular valve were incised in the plane of the septum to yield a mitral and tricuspid leaflet (Fig. 3, B) . A tail ored septal patch of Dacron fabr ic was sutured to the margin of the ventricula r defect , and the leaflet segments were then att ached to the patch to reconstruct the atrioventricular valve mechanism (Fig. 4, A and B ) .
The patch was then used to close the ostium primum defect in the atrial septum (Fig. 5, A and B). A right ventricular infundibulectomy was also performed, and the commissures of the pulmonary valve were incised (Fig. 5, C). Intraoperative pressure measurements revealed a right ventricular pressure of 40/0 mm. Hg and a pulmonary artery pressure of 20/10 mm. Hg . Mild congestive failure necessitated fluid restriction and diuretics in the early postoperative period. Cardiac catheterization was carried out 7 months after the operation, and the data are presented in Table II . Two years after operation, he is well with no cyanosis and with no limitation in activity.
Discussion The association of tetralogy of Fallot and complete endocardial cushion defect was first described by Bull" in 1885 and more recently by Monquin." In a series of autopsy specimens with complete endocardial cushion defect , Lev ' was able to report 3 cases of this combined defect. Early palliative attempts with this complex problem were
The Journol 01
270
Fisher et al.
Thoracic a nd Cardiovascular Surgery
:,0
Pa.lch. to ~e ti~pl ·o. xim aled •< . l'lm o r A .:;j.D. "
-- R e a H a c:: h m e n i of m\tral leaflet to p a tch abov e top of v en h' l c u~ al~ sep tum
, C~",fl ~e af1
el
ill IIli t"al c lo s o d
-Hypel'irophled infundioulal' mue, resecte d 'Pa l c h Tr i cus p i d leaflet app"oxima leJ \ 0 pafch above top of venh·i.; ulal~ s eptum
-.: ~ o s i n g
V. S.D.
Fig. 5. A and B, Completion of the closure of the ostium primum defect with the Dacron patch . C, Incisions have been made in the main pulmonary artery and in the right ventricle for pulmonary valve commissurotomy and resection of the hypertrophied infundibular muscle. A .S.D., Atrial septal defect. V.S.D ., Ventricular septal defect.
not successful, " and more recent attempts at total correction of this combined defect have rarely been successful. 8 In fact, there is only a single reported example of complete correction of this combined anomaly." The reasons for lack of successful repair may be due either to failure to appreciate the complex and combined nature of the defects or failure to relieve the infundibular stenosis . Rastelli" did much to clarify the anatomy of the complete endocardial cushion defect. He divided the complete endocardial cushion defects into three distinct types. In Type I, the anterior common leaflet is divided into mitral and tricuspid portions with chordal attachments from the rim of the ventricular septal defect to the margins of the leaflet. In Type II , the anterior common leaflet is divided, but its components are
both attached medially to an abnormal papillary muscle arising in the right ventricle. Type III is seen most frequently in those cases in which associated intracardiac defects are noted. This type is characterized by an undivided common anterior leaflet with no chordal attachments to the intraventricular septum." The Type III defect was the anatomic defect seen in our patients at operation and autopsy. Careful preoperative evaluation of these patients is essential to their management and successful operative treatment. Occasionally, the diagnosis of tetralogy of Fallot will be made without the realization of the associated atrioventricular anomalies. Precise diagnosis is not always established before operation, as in 2 of our patients, and this lack of information complicates the intracardiac repair. The frequent association of endo-
Volume 70 Number
2
Atrioventricular canal with tetralogy of Fallot
27 1
August, 1975
cardial cushion defects and Down's syndrome is borne out by the small experience reported here. Five of the 8 patients with combined anomalies had Down's syndrome. The diagnosis of infundibular stenosis and the tetralogy of Fallot malformation in a child with Down's syndrome should alert one to the possibility of an associated endocardial cushion defect. Careful review of the preoperative left ventricular angiocardiogram will usually reveal the characteristic findings of the endocardial cushion defect,"?- 11 The timing of complete surgical repair is of extreme importance. Previous experience> has demonstrated high mortality rates with attempts at complete correction of the atrioventricular canal deformity in patients less than 2 years of age. For this reason, we favor an early palliative procedure (BlalockTaussig anastomosis or a Waterston anastomosis) as the initial procedure. In view of the complex nature of this intracardiac problem, it seems prudent, when possible, to delay the total correction until the child is older than 2 years of age or weighs more than 15 kilograms.
3
4
5 6 7
8
9
10
REFERENCES Rastelli, G., Ongley, P., and McGoon, D.: Surgical Repair of Complete Atrioventricular Canal With Anterior Common Leaflet Undivided and Unattached to the Ventricular Septum, Mayo Clin. Proc. 44: 335, 1969. 2 Rastelli, G., Ongley, P., Kirklin, J., and McGoon, D.: Surgical Repair of the Complete Form of Persistent Common Atrioventricular
11
12
Canal, J. THORAc. CARDIOVASC. SURG. 55: 299, 1968. Rastelli, G., Kirklin, J., and Titus, J.: Anatomic Observations on Complete Form of Persistent Common Atrioventricular Canal With Special Reference to Atrioventricular Valves, Mayo Clin. Proc. 41: 296, 1966. McMullan, M., Wallace, R., Weidman, W., and McGoon, D.: Surgical Treatment of Complete Atrioventricular Canal, Surgery 72: 905, 1972. Bull, C.: Anomalic congenital du coeur avec transposition des vesceres, Jemaine Med. 5: 318, 1885. Monquin, M., Metainu, c., Durand, M., and Beyda, E.: Cardiopathic Congenital Complex, Cardiologia 29: 145, 1956. Lev, M., Agustsson, M., and Arcilla, R.: The Pathologic Anatomy of Common Atrioventricular Orifice Associated With Tetralogy of Fallot, Am. J. Clin. PathoI. 36: 408, 1961. Gerbode, F., Sanchez, P., Arguero, R., Kerth, W., Hill, J., DeVries, P., Selzer, A., and Robinson, S.: Endocardial Cushion Defects, Am. Surg. 166: 486, 1967. Colvez, P., and Levasseur, F.: Une cardiopathie congenitale rare. L'association d'une tetralogie et d'un canal atrioventricularie complete, Arch. Mal. Coeur 62: 996, 1969. Girod, D., Raghib, G., Wang, Y., and Adams, P.: Angiocardiographic Characteristics of Persistent Common Atrioventricular Canal, Radiology 85: 442, 1965. Rastelli, G., Kirklin, J., and Kincaid, 0.: Angiocardiography of Persistent Common Atrioventricular Canal, Mayo Clin. Proc. 42: 200, 1967. Mustard, W., Niquidulas, F., and Truster, G.: Endocardial Cushion Defects in Infants and Children: Ten-Year Surgical Experience, Br. Heart J. 27: 768, 1965.
R. Darryl Fisher, M.D., David K. Bone, M.D., Richard D. Rowe, M.D., and Vincent L. Gott, M.D., Baltimore, Md.
he
association of the complete form of atrioventricular canal with tetralogy of Fallot is a recognized, but rare, cardiac malformation. This uncommon associated anomaly occurs in less than five per cent of patients with complete endocardial cushion defects. Until recently, attempts at total correction of atrioventricular canal were often unsuccessful; however, with newer surgical techniques " 2 and a more precise definition of the intracardiac anatomy," the mortality rate for repair of this serious defect has been reduced.' Despite success with total correction of atrioventricular canal, attempts at repair of this malformation associated with other cardiac defects have not been as successful. Between 1960 and 1974, 8 patients have required surgical treatment for complete From the Departments of Surgery and Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, Md. 21205. Supported in part by the National Institutes of Health Training Grant No. 5TOI GM 01541. Received for publication March 11, 1975.
atrioventricular canal and associated tetralogy of Fallot at The Johns Hopkins Hospital. The diagnostic experience and the operative methods with this group form the basis of this report.
Oinical material Eight patients evaluated at The Johns Hopkins Hospital from 1960 to 1974 with complete atrioventricular canal and associated tetralogy of Fallot have undergone palliative or corrective operations (Table I). Three patients were younger than 2 years at the time of their first operative procedure, whereas the remaining patients ranged in age from 3 to 13 years. All patients experienced severe cyanosis and diminished exercise tolerance in the period before operation; rising hematocrit values (ranging from 46 to 81 per cent) and increasing heart size were found in each patient before operation. The electrocardiogram in all except Patient J. D. had left axis deviation with a counterclockwise loop in the frontal plane. Five of 8 patients had Down's syndrome and 1
265
266
The Journol of Thorocic ond Cordiovosculor Surgery
Fisher et al.
Table I. Summary of the experience with 8 patients undergoing operative procedures for complete atrioventricular canal and associated tetralogy of Fallot Patient
M.e.
C.H.
Preoperative cath diagnosis
Atrioventricular canal and tetralogy of Fallot Tetralogy of Fallot
Operative procedures (age at time ofoperation)
Associated disease
Down's syndrome
Brock procedure (10 yr.)
None
Result
Doing relatively well clinically
W.O.
Atrioventricular canal and tetralogy of Fallot
Mental retardation
A.N.
Atrioventricular canal and tetralogy of Fallot Atrioventricular canal and tetralogy of Fallot Atrioventricular canal and tetralogy of Fallot Tetralogy of Fallot
Down's syndrome
Died 3 months after operation with severe congestive heart failure Blalock-Taussig shunt (3 yr.) Temporary relief after first shunt Brock procedure (13 yr.) Died in postop. period after Brock procedure Blalock-Taussig shunt (20 mo.); No improvement Waterston shunt 2 days later
Down's syndrome
Blalock-Taussig shunt (4 mo.); Waterston shunt 15days later
Down's syndrome
Waterston shunt (4 mo.)
Died during surgery before shunt constructed
Down's syndrome
Right Blalock-Taussig shunt (5 yr.); left Blalock-Taussig shunt (7.5 yr.); total correction (9 yr.)
Temporary relief from shunts
W. J.
L. D.
J. D.
D.M.
Atrioventricular canal and tetralogy of Fallot
None
Brock procedure (8 yr.)
Blalock-Taussig shunt (4 yr.); total correction (8 yr.)
other was mentally retarded. The remaining 2 patients were of normal intelligence. Preoperative cardiac catheterization revealed tetralogy of Fallot malformation in each patient. In 6 of the 8 patients, the presence of a complete atrioventricular canal was established by preoperative cardiac catheterization. In the other 2 patients, the presence of the associated atrioventricular canal was not suspected prior to the operative procedure. In Patient J. D. the diagnosis was made during the open-heart procedure, and in Patient C. H. the diagnosis was made at postmortem examination 3 months after a Brock procedure. Retrospective analysis of the cineangiograms suggested the diagnosis in 1 of these patients, whereas in the other the cineangiograms could not be interpreted as suggesting an endocardial cushion
No improvement
Died in early postop. period after attempted total correction Temporary relief from shunt; asymptomatic after total correction
defect despite knowledge of the malformation. A variety of operative procedures was employed for this group. An infundibulectomy was carried out in 3 patients as a palliative procedure. One patient (M. C.) was relieved of symptoms and is now leading a relatively normal but restricted life. Two patients died after the Brock procedure. One of these patients (W. D.) with these combined malformations died early in our experience; at the present time, he certainly would be submitted to a totally corrective procedure. The other patient (C. H.) died 3 months following the Brock procedure. At the time of his death, he was being treated for moderately severe congestive failure with pulmonary valve regurgitation. Five patients underwent systemic-pulmo-
Volume 70
Atrioventricular canal with tetralogy of Fallot
Number 2
267
August, 1975
Fig. 1. Preoperative chest film for Patient D . M. before the totally corrective procedure.
Table II. Preoperative and postoperative cardiac catheterization findings in an 8-year-old boy (D. M.) undergoing correction of tetralogy of Fallot and complete atrioventricular canal* Preoperative Pulmonary arter y pressure (mm. Hg) 14/5 Right ventricular pressure (mm. Hg) 90/0 Infundibular gradient (mm. Hg) 76 Left ventricular angiogram Mild mitral regurgitation
Postop erative
18/5 29/0
II
Mild mitral regurgitation
'There were no shunts.
nary artery shunting procedures as their primary operation. In 2 patients (A . N. and W. J .) the Blalock-Taussig shunts were not adequate and were followed in a few days with a Waterston shunt. In both of these patients, no significant improvement was obtained. Three patients having a BlalockTaussig shunt (W. D., J. D., and D. M.) obtained temporary relief of symptoms ranging from 3 months to 10 years. Patient W. D. required a Brock procedure 10 years
Fig. 2. Preoperative left (A) and right (B) ventriculograms for Patient D . M.
later and died during the early postoperative period. Patients J. D. and D. M. both obtained temporary improvement until total correction was undertaken. The final patient to have an attempted shunt procedure (L. D. ) died during the operation before the shunt could be constructed. Complete correction of this combined anomaly was attempted in 2 of the patients. One patient (1. D.) underwent open-heart surgery at the age of 9 years after 2 previous
268
The Journol 01 Thoracic and Ccrdiovosculcr
Fisher et al.
Surgery
I I
R .<:l. h ·ium
R i m of
ait'iul ::lep ial defect
ri'l -::is i o n in an terior a n d p os i e r io r leaflets Fig. 3. A, View of complete endocardial cushion defect through right atriotomy. B, Incisions in the common leaflets of the atrioventricular valve permit exposure of the top of the ventricular septal defect. Interrupted sutures have been placed in the cleft of the mitral valve.
Blalock-Taussig shunts (age 5 and 7 years, 6 months). Unfortunately, the diagnosis of an associated endocardial cushion defect had not been made before the operation and he died in the early postoperative period of low cardiac output. In the other patient (D. M.), an 8-year-old boy, this complex malformation was correctly diagnosed preoperatively. His clinical summary is presented in detail. Case report This child was the product of an uneventful pregnancy and delivery. At 6 weeks of age a cardiac murmur was first heard, and a diagnosis of ventricular septal defect and infundibular pulmonic stenosis was made by cardiac catheterization. At 6 months of age, the child developed severe cyanosis and tachypnea when an episode of
supraventricular tachycardia occurred. These symptoms were relieved by digitalis and he remained well for the next 3 years. At the age of 4 years, he was first seen at The Johns Hopkins Hospital with mild cyanosis and tachypnea. Cardiac catheterization revealed a systemic right ventricular pre ssure and a dextraposed aorta. In addition, a complete atrioventricular canal malformation was noted. A right Blalock-Taussig anastomosis was then performed and afforded complete relief of symptoms. The hematocrit value fell from 75 to 45 per cent in the months following the operation. Because of decreasing exercise tolerance, radiographic evidence of cardiac enlargement, and right ventricular strain by electrocardiography, he was admitted at the age of 8 years for total correction of the tetralogy of Fallot and the endocardial cushion malformation. Upon admission, he was an active, alert boy with moderate cyanosis and clubbing. His weight was 21.6 kilograms. A right ventricular heave was present, and the second
Volume 70 Number 2
Atrioventricular canal with tetralogy of Fallot
August , 1975
269
T o p of ve n f v-i c u laY' .: sept u m
71'1".:. / jj;,:
~
"Da c ~' o n
patch to be 5u fu Y'ed fa ventY'icutar- sep t u m
SUf UY' e pa 5 ses Iht"ouQh patch z;nd h'1'cu sp i d
leaflet
,,
,
1 /
"
/
Mlh': : I
I
/
P ~tch
Ie fle l
Fig. 4. A , Placement of interrupted sutures in the ventricular septum and in turn into the Dacron patch. B, Approximation of the medial leaflets of the mitral and tricuspid valves to the Dacron patch. heart sound was single. A Grade 3/6 ejection murmur was present at the lower left sternal margin. The hematocrit value was 54 per cent. The electrocardiogram revealed left axis deviat ion, right ventricular hypertrophy, and right bundle branch block . Moderate biventricular enlargement was noted by chest roentgenography (Fig. 1). Cardiac catheterization pro vided the pressure data listed in Table II and the ventriculographic studies in Fig. 2. On Oct. 18, 1972, the patient underwent total correction of the atrioventricular canal and right ventricula r outflow tract obstruction. The right Blalock-Taussig shunt was ligated after extracorporeal circulation was begun. A complete endo cardial cushion defect (Type III) was identified through a right atriotomy (Fig. 3, A), and this was repaired by the method originated by Rastelli and his associates. > The common leaflets of the atrioventricular valve were incised in the plane of the septum to yield a mitral and tricuspid leaflet (Fig. 3, B) . A tail ored septal patch of Dacron fabr ic was sutured to the margin of the ventricula r defect , and the leaflet segments were then att ached to the patch to reconstruct the atrioventricular valve mechanism (Fig. 4, A and B ) .
The patch was then used to close the ostium primum defect in the atrial septum (Fig. 5, A and B). A right ventricular infundibulectomy was also performed, and the commissures of the pulmonary valve were incised (Fig. 5, C). Intraoperative pressure measurements revealed a right ventricular pressure of 40/0 mm. Hg and a pulmonary artery pressure of 20/10 mm. Hg . Mild congestive failure necessitated fluid restriction and diuretics in the early postoperative period. Cardiac catheterization was carried out 7 months after the operation, and the data are presented in Table II . Two years after operation, he is well with no cyanosis and with no limitation in activity.
Discussion The association of tetralogy of Fallot and complete endocardial cushion defect was first described by Bull" in 1885 and more recently by Monquin." In a series of autopsy specimens with complete endocardial cushion defect , Lev ' was able to report 3 cases of this combined defect. Early palliative attempts with this complex problem were
The Journol 01
270
Fisher et al.
Thoracic a nd Cardiovascular Surgery
:,0
Pa.lch. to ~e ti~pl ·o. xim aled •< . l'lm o r A .:;j.D. "
-- R e a H a c:: h m e n i of m\tral leaflet to p a tch abov e top of v en h' l c u~ al~ sep tum
, C~",fl ~e af1
el
ill IIli t"al c lo s o d
-Hypel'irophled infundioulal' mue, resecte d 'Pa l c h Tr i cus p i d leaflet app"oxima leJ \ 0 pafch above top of venh·i.; ulal~ s eptum
-.: ~ o s i n g
V. S.D.
Fig. 5. A and B, Completion of the closure of the ostium primum defect with the Dacron patch . C, Incisions have been made in the main pulmonary artery and in the right ventricle for pulmonary valve commissurotomy and resection of the hypertrophied infundibular muscle. A .S.D., Atrial septal defect. V.S.D ., Ventricular septal defect.
not successful, " and more recent attempts at total correction of this combined defect have rarely been successful. 8 In fact, there is only a single reported example of complete correction of this combined anomaly." The reasons for lack of successful repair may be due either to failure to appreciate the complex and combined nature of the defects or failure to relieve the infundibular stenosis . Rastelli" did much to clarify the anatomy of the complete endocardial cushion defect. He divided the complete endocardial cushion defects into three distinct types. In Type I, the anterior common leaflet is divided into mitral and tricuspid portions with chordal attachments from the rim of the ventricular septal defect to the margins of the leaflet. In Type II , the anterior common leaflet is divided, but its components are
both attached medially to an abnormal papillary muscle arising in the right ventricle. Type III is seen most frequently in those cases in which associated intracardiac defects are noted. This type is characterized by an undivided common anterior leaflet with no chordal attachments to the intraventricular septum." The Type III defect was the anatomic defect seen in our patients at operation and autopsy. Careful preoperative evaluation of these patients is essential to their management and successful operative treatment. Occasionally, the diagnosis of tetralogy of Fallot will be made without the realization of the associated atrioventricular anomalies. Precise diagnosis is not always established before operation, as in 2 of our patients, and this lack of information complicates the intracardiac repair. The frequent association of endo-
Volume 70 Number
2
Atrioventricular canal with tetralogy of Fallot
27 1
August, 1975
cardial cushion defects and Down's syndrome is borne out by the small experience reported here. Five of the 8 patients with combined anomalies had Down's syndrome. The diagnosis of infundibular stenosis and the tetralogy of Fallot malformation in a child with Down's syndrome should alert one to the possibility of an associated endocardial cushion defect. Careful review of the preoperative left ventricular angiocardiogram will usually reveal the characteristic findings of the endocardial cushion defect,"?- 11 The timing of complete surgical repair is of extreme importance. Previous experience> has demonstrated high mortality rates with attempts at complete correction of the atrioventricular canal deformity in patients less than 2 years of age. For this reason, we favor an early palliative procedure (BlalockTaussig anastomosis or a Waterston anastomosis) as the initial procedure. In view of the complex nature of this intracardiac problem, it seems prudent, when possible, to delay the total correction until the child is older than 2 years of age or weighs more than 15 kilograms.
3
4
5 6 7
8
9
10
REFERENCES Rastelli, G., Ongley, P., and McGoon, D.: Surgical Repair of Complete Atrioventricular Canal With Anterior Common Leaflet Undivided and Unattached to the Ventricular Septum, Mayo Clin. Proc. 44: 335, 1969. 2 Rastelli, G., Ongley, P., Kirklin, J., and McGoon, D.: Surgical Repair of the Complete Form of Persistent Common Atrioventricular
11
12
Canal, J. THORAc. CARDIOVASC. SURG. 55: 299, 1968. Rastelli, G., Kirklin, J., and Titus, J.: Anatomic Observations on Complete Form of Persistent Common Atrioventricular Canal With Special Reference to Atrioventricular Valves, Mayo Clin. Proc. 41: 296, 1966. McMullan, M., Wallace, R., Weidman, W., and McGoon, D.: Surgical Treatment of Complete Atrioventricular Canal, Surgery 72: 905, 1972. Bull, C.: Anomalic congenital du coeur avec transposition des vesceres, Jemaine Med. 5: 318, 1885. Monquin, M., Metainu, c., Durand, M., and Beyda, E.: Cardiopathic Congenital Complex, Cardiologia 29: 145, 1956. Lev, M., Agustsson, M., and Arcilla, R.: The Pathologic Anatomy of Common Atrioventricular Orifice Associated With Tetralogy of Fallot, Am. J. Clin. PathoI. 36: 408, 1961. Gerbode, F., Sanchez, P., Arguero, R., Kerth, W., Hill, J., DeVries, P., Selzer, A., and Robinson, S.: Endocardial Cushion Defects, Am. Surg. 166: 486, 1967. Colvez, P., and Levasseur, F.: Une cardiopathie congenitale rare. L'association d'une tetralogie et d'un canal atrioventricularie complete, Arch. Mal. Coeur 62: 996, 1969. Girod, D., Raghib, G., Wang, Y., and Adams, P.: Angiocardiographic Characteristics of Persistent Common Atrioventricular Canal, Radiology 85: 442, 1965. Rastelli, G., Kirklin, J., and Kincaid, 0.: Angiocardiography of Persistent Common Atrioventricular Canal, Mayo Clin. Proc. 42: 200, 1967. Mustard, W., Niquidulas, F., and Truster, G.: Endocardial Cushion Defects in Infants and Children: Ten-Year Surgical Experience, Br. Heart J. 27: 768, 1965.