February 1975
The Journal o f P E D I A T R I C S
189
Operative repair of atrial septal defect without cardiac catheterization Thirty-three children with secundum or sinus venosus atrial septal defects underwent operative correction at the University of Minnesota Hospitals" between January, 1972, and December, 1973. Nineteen o f these patients (58%) did not have cardiac catheterization as part of their preoperative evaluation, since clinical assessment o f the child, with or without corroborative evidence obtained by echocardiography and radionuclide angiocardiography, provided st4fficient information to permit recommendation of operation. In the ma/ority o[' patients with uncomplicated atrial septal defect, cardiac catheterization is not a necessary part of the preoperative evaluation and may be reserved for those patients with unusual clinical or laboratory features.
W i l l i a m A. N e a l , M . D . , Morganto~vn, W. Va., J a m e s H . M o i l e r , M . D . ,
Richard L. Varco, M.D., and Ray C. Anderson, M . D . , * M i n n e a p o l i s , M i n n .
OPINIONS differ r e g a r d i n g the a m o u n t and type o f diagnostic data needed before recommending that a child with congenital heart disease undergo an operation. With few exceptions, it has been our policy to perform cardiac catheterization on all children prior to operations involving cardiopulmonary bypass. We have not routinely catheterized pediatric patients whose clinical and laboratory findings were considered diagnostic for patent ductus arteriosus or coarctation of the aorta. At least one institution has considered cardiac catheterization not indicated in a significant n u m b e r of patients undergoing open heart surgery. ~ Since 1971, we have omitted preoperative cardiac catheterization studies in children whose clinical and laboratory findings a p p e a r e d typical for atrial septal defect of secundum or sinus venosus type. More recently, we have used the noninvasive techniques of echocardiography and nuclearoangiocardiography, p e r m i t t i n g an e v e n g r e a t e r degree of accuracy in identifying atrial septal defect in pediatric patients. The purpose of this report is to pres e n t our clinical e x p e r i e n c e with the p r e o p e r a t i v e evaluation of children with atrial septal defect.
From the Department o f Pediatrics and Surgery, University of Minnesota Hospitals. *Reprint address: Box 94, Mayo Memorial Building, University of Minnesota Hospitals, Minneapolis, Minn. 55455.
MATERIAL
AND METHODS
Patient selection. The clinical and laboratory data of 33 pediatric patients operated upon for uncomplicated atrial septal defect at the University of Minnesota Hospitals between January, 1972, and December, 1973, have been reviewed. In each case the diagnosis was confirmed at operation or by cardiac catheterization. Patients with ostium primum atrial defects have been excluded from this series. See related article, p. 319. Clinical evaluation, Each patient suspected of having an atrial septal defect was examined by at least two members of the pediatric cardiology staff. After the history was obtained and physical examination performed, cardiac sounds were viewed on a phonocardioscope. A s t a n d a r d 12-lead e l e c t r o c a r d i o g r a m and thoracic roentgenograms were obtained from each patient. Vectorcardiograms, as well as echocardiograms a n d technetium 99 radionuclide scintillation studies, were obtained from many patients. The application of these techniques in the evaluation of atrial septal defect has been previously described. 25 The clinical criteria considered necessary to establish the diagnosis of atrial septal defect were: (1) a systolic ejection m u r m u r at the upper left sternal border of
VoL 86, No. 2, pp. 189-193
190
Neal et al.
The Journal of Pediatrics February 1975
Table I. Indications for cardiac catheterization in 14 patients operated upon for atrial septal defect Patient 1 2 3 4 5 6 7 8* 9* 10 11 12 13 14
J
Reason.[or catheterization
Cardiac defects
Variable splitting $2; no IRBBB Variable splitting $2; no IRBBB Var!able splitting $2; ejection click Roentgenogram suggestive of L-TGV Holt-Oram syndrome; atypical systolic murmur Variable splitting $2; no IRBBB; ejection click History of CHF during infancy Variable splitting $2; no IRBBB; no diastolic murmur Variable splitting S2 Variable splitting $2; atypical systolic murmur History ofcyanosis and CHF during infancy Narrow, fixed splitting $2; atypical systolic murmur Variable splitting $2; abnormal P wave axis; no IRBBB No atypical findings, catheterization prior to referral
ASD (secundum) ASD (secundum) ASD (secundum) ASD (secundum) ASD (secundum) ASD (secundum) ASD (secundum) ASD (secundum) ASD (secundum) ASD (secundum) ASD (sinus venosus) PAPVC ASD (sinus venosus) ASD (fenestrated) ASD (secundum)
Qp:Qs = p u l m o n a r y to systemic flow ratio; s 2 = second h e a r t sound; I RBBB = incomplete right b u n d l e branc h block; ASD = atrial septal defect; L - T G V = L-transposition o f the great vessels; C H F = congestive heart failure; P A P V C = partial a n o m a l o u s p u l m o n a r y v e n o u s connection; PS = p u l m o n a r y
stenosis. *Qp:Qs) 1.5:1.0,( 2.0:1.0.Allother patientsQp:Qs) 2.0:t.0.
Radioisotope Flow Study KW, ASD Age 6yrs. svc RL
"'~ ~
'
i'~
'
LL
PV
Fig. 1. Radionuclide angiocardiogram in a patient with a secundum type atrial septal defect. Regions of interest, such as the right atrium (RA) and lung fields (RL and LL), are selected for computer scanning and time-activity curves derived. The atrial septal defect is not visualized, but recirculation of isotope through the lungs produces an abnormal time-activity curve. Grade 2-3/6 intensity; (2) wide splitting of the second heart sound with little or no respiratory variation; and (3) a tricuspid flow m u r m u r in diastole. Confirmatory laboratory evidence included: (1) an electrocardiogram showing mild right axis deviation in the frontal plane, a n d an i n c o m p l e t e right b u n d l e branch block pattern in VaR and V1; and (2) a thoracic roentgenogram interpreted either as normal or, as is
more often the case, showing mild cardiomegaly and increased pulmonary vascular markings. All patients r e c o m m e n d e d for operative repair of atrial septal defect w i t h o u t cardiac c a t h e t e r i z a t i o n fulfilled the criteria for diagnosis described above. As our experience with echocardiography and technetium 99 cardiac flow studies has increased, we have increasingly relied on these noninvasive techniques to substantiate the clinical diagnosis. Positive confirmat i o n of the presence of a left-to-right s h u n t by radionuclide cardiac angiography is now considered advisable in each patient scheduled for operation without cardiac catheterization. Cardiac catheterization. W h e n the diagnosis of uncomplicated atrial septal defect with a pulmonary to systemic flow ratio of greater than 1.5:1 could not be established with sufficient certainty, cardiac catheterization was performed. An increase in oxygen saturation at the atrial level greater than 1.5 volumes percent on at least two right-sided oximetry series was considered necessary to establish the presence of a left-to-right shunt. Additionally, confirmation was obtained by the indicator dilution technique, and frequently by pulmonary arteriography. RESULTS Thirty-three pediatric patients were operated upon for closure of atrial septal defects of s e c u n d u m or sinus venosus type during the two-year period of this study. All had defects greater than 2.0 cm in diameter. Their ages ranged between 5 and 18 years. None had additional cardiac defects with the exception of one patient
Volume 86 Number 2
R e p a i r o f atrial septal defect without catheterization
191
Radioisotope Flow Study Activity Curves KW Age 6yrs. ASD Rt. Atrium
Pulm
Rt. Vent
Art.
~
,e,
.:
=
," II
...,
~ ",:..2::.,./..l.~::t:;v~.:.:.,.~."
II
Rt. Lung
"*"
Lt. Lung
t
9.
"r162
;.,;;;P"l % . t - ;
I
,
**'..%
9 , . . . . *:,,.,
J
Fig. 2. Time-activity curves derived from various regions of interest in a radionuclide angiocardiographic study of a child with an atrial septal defect. The curves obtained by scanning the lung fields are abnormal due to recirculation of 99mTc secondary to a left-to-right shunt. who had c o e x i s t e n t partial a n o m a l o u s p u l m o n a r y venous connection. N i n e t e e n of 33 p a t i e n t s (58%) had clinical and laboratory features typical of atrial septal defect and were operated upon without prior cardiac catheterization. The 14 remaining patients (42%) required cardiac catheterization to confirm the diagnosis of uncomplicated atrial septal defect" and quantitate the magnitude of the left-to-right shunt. Significant respiratory variation in the degree of splitting of the second heart sound (eight patients) and absence of incomplete right bundle branch block pattern on the electrocardiogram (five patients) were the most frequently encountered atypical findings in these children (Table I). Excluded from this study are two additional patients who did not fulfill the criteria for operation without hemod}namic study and were found to have small secund u m atrial defects (Qp:Qs (1.5:1.0) at catheterization. Surgery was not performed. DISCUSSION Before palliative or corrective operations on patients with congenital heart disease can be safely performed, an accurate diagnosis is necessary. Usually this necessitates p r e o p e r a t i v e cardiac c a t h e t e r i z a t i o n , i n c l u d i n g selective angiography. There are situations, however,
when it is possible to make a diagnosis on the basis of clinical and laboratory findings with a high degree of accuracy, obviating the need for cardiac catheterization 9 It may be questioned whether this approach can be safely recommended for operations requiring cardiopulmonary bypass. Analysis of the pre- and postoperative diagnoses of 196 patients with congenital heart disease referred to the Mayo Clinic suggests that cardiac catheterization is not always necessary, l Operation was advised on the basis of clinical findings alone in 106 patients (54%), though the series included a variety of types of congenital heart disease. In only four cases were unsuspected lesions e n c o u n t e r e d which significantly a l t e r e d the surgical approach. Twelve patients were operated upon with a clinical diagnosis of atrial septal defect, one of whom was found to have additional partial anomalous pulmonary venous connection. The association o f partial a n o m a l o u s p u l m o n a r y venous connection with atrial septal defect, particularly defects of the sinus venosus type, is well known. In the majority of cases it is not possible to suspect this additional a n o m a l y w i t h o u t cardiac c a t h e t e r i z a t i o n , and even then it may be missed unless specifically looked for. The surgeon, however, can readily identify anomalous venous connection a n d can nearly always correct
192
~
Neal et al.
-
-
The Journal of Pediatrics February 1975
I
CHEST
WAL
I
I
~rlrfldo•
.10
Db
~
Fig. 3. Simultaneous recording of an echocardiogram, ECG, and phonocardiogram in a child with an atrial septal defect. The right ventricle (R V) is enlarged and there is simultaneous anterior motion of the septum and posterior left ventricular wall during systole, referred to as "paradoxical" septal motion. These echocardiographic features are frequently present in volume overload of the RV, such as with atrial septal defect.
the defect regardless of whether or not the diagnosis was known preoperatively. The location of the atrial septal defect can frequently be predicted with accuracy by clinical and laboratory means. 60stium primum atrial defects usually have electrocardiographic evidence of left axis deviation in the frontal plane with a figure of eight or counterclockwise vector loop. Sinus venosus defects have left axis deviation of the P wave vectors in nearly half the c a s e s ] and suspicion of this defect alerts one to the possibility of associated partial anomalous pulmonary venous connection. Nevertheless, with the exception of ostium primum lesions, knowledge of the exact location of the atrial defect preoperatively has not been considered necessary. Coexistent atrial and ventricular septal defects occasionally occur, but the physical findings, electrocardiogram, and chest roentgenogram are similar to those of a moderate to large ventricular septal defect. 8 Lutembacher's syndrome (atrial septal defect and rheumatic mitral valve disease) 9 is very u n c o m m o n in children and would have different auscultatory findings; moreover, echocardiography can be usefully applied to rule out coexistent mitral valve disease. Technetium pertechnetate cardiac flow studies provide a relatively sensitive means of determining the
presence or absence of a left-to-right shunt, but it is not possible to determine the location of the defect (Figs. 1 and 2). Echocardiography reveals paradoxical septal motion and increased right ventricular internal dimension in many patients with volume overload of the right ventricle (Fig. 3). However, paradoxical septal motion also has been described with normal right heart hemodynamics, 1~and normal septal motion has been found in patients with atrial septal defect. 1~By combining the information obtained by noninvasive techniques with the clinical and laboratory data, operative repair of atrial septal defect without prior cardiac catheterization can be safely recommended. The advantages of eliminating cardiac catheterization when possible are obvious. Though the risk is minimal when performed on a child beyond infancy, it is still an invasive procedure potentially complicated by arrhythmias, perforation of the heart, and allergic reactions to contrast media. Femoral or brachial artery thrombosis occurs frequently enough to warrant concern, regardless of whether the percutaneous or open arteriotomy technique is used. Finally, it is an expensive procedure which usually requires one or two days of hospitalization. We think that the pediatric patient with typical clinical and laboratory features of atrial septal defect can be
Volume 86 Number 2
referred for operation w i t h o u t cardiac catheterization. If atypical features are present, t h e n additional study is advisable. REFERENCES
l.
2.
3.
4.
5.
Pestana C, Weidman WH, Swan HJC, and McGoon DC: Accuracy of preoperative diagnosis in congenital heart disease, Am Heart J 72:446, 1966. Diamond MA, Dillon JC, Haine CL, Chang S, and Feigenbaum H: Echocardiogr.aphic features of atrial septal defect, Circulation 43:129, 1971. Tajik AJ, Gau GT, Ritter DG, and Schattenberg TT: Echocardiographic pattern of right ventricular diastolic volume overload in children, Circulation 46:36, 1972. Folse R, and Braunwald E: Pulmonary vascular dilution curves recorded by external detection in the diagnosis of left-to-right shunts, Br Heart J 24:166, 1962. Rosenthal L: Nucleographic screening of patients with left-to-right cardiac shunts, Nucl Med 99:601, 1971.
Repair of atrial septal defect without catheterization
19 3
6. DuShane JW, Weidman WH, Brandenburg RO, and Kirklin JW: Differentiation of interatrial communications by clinical methods, Circulation 21:363, 1960. 7. Davia JE, Cheitlin MD, and Bedynek JL: Sinus venosus atrial septal defect: Analysis of fifty cases, Am Heart J 85:177, 1973. 8. Prado S, Adams P Jr, Amplatz K, Girod D, and Anderson RC: Coexistent atrial and ventricular septal defects: A report of twenty-two cases, Dis Chest 51:412, 1967. 9. Bashour RA, and Simmons DH: Atrial septal defect with mitral valvulitis: Clinical and catheterization diagnosis, Ann Intern Med 48:1194, 1957. 10. Cody MC, Giuliani ER, and Mair DD: "Paradoxical" septal motion by echocardiography in a patient with normal right heart hemodynamics, Mayo Clin Proc 48:31, 1973. 11. Tajik AJ, Gau GT, Schattenberg TT, and Ritter DG: Normal ventricular septal motion in atrial septal defect, Mayo Clin Proc 47:635, 1972.
The Journal o f P E D I A T R I C S
189
Operative repair of atrial septal defect without cardiac catheterization Thirty-three children with secundum or sinus venosus atrial septal defects underwent operative correction at the University of Minnesota Hospitals" between January, 1972, and December, 1973. Nineteen o f these patients (58%) did not have cardiac catheterization as part of their preoperative evaluation, since clinical assessment o f the child, with or without corroborative evidence obtained by echocardiography and radionuclide angiocardiography, provided st4fficient information to permit recommendation of operation. In the ma/ority o[' patients with uncomplicated atrial septal defect, cardiac catheterization is not a necessary part of the preoperative evaluation and may be reserved for those patients with unusual clinical or laboratory features.
W i l l i a m A. N e a l , M . D . , Morganto~vn, W. Va., J a m e s H . M o i l e r , M . D . ,
Richard L. Varco, M.D., and Ray C. Anderson, M . D . , * M i n n e a p o l i s , M i n n .
OPINIONS differ r e g a r d i n g the a m o u n t and type o f diagnostic data needed before recommending that a child with congenital heart disease undergo an operation. With few exceptions, it has been our policy to perform cardiac catheterization on all children prior to operations involving cardiopulmonary bypass. We have not routinely catheterized pediatric patients whose clinical and laboratory findings were considered diagnostic for patent ductus arteriosus or coarctation of the aorta. At least one institution has considered cardiac catheterization not indicated in a significant n u m b e r of patients undergoing open heart surgery. ~ Since 1971, we have omitted preoperative cardiac catheterization studies in children whose clinical and laboratory findings a p p e a r e d typical for atrial septal defect of secundum or sinus venosus type. More recently, we have used the noninvasive techniques of echocardiography and nuclearoangiocardiography, p e r m i t t i n g an e v e n g r e a t e r degree of accuracy in identifying atrial septal defect in pediatric patients. The purpose of this report is to pres e n t our clinical e x p e r i e n c e with the p r e o p e r a t i v e evaluation of children with atrial septal defect.
From the Department o f Pediatrics and Surgery, University of Minnesota Hospitals. *Reprint address: Box 94, Mayo Memorial Building, University of Minnesota Hospitals, Minneapolis, Minn. 55455.
MATERIAL
AND METHODS
Patient selection. The clinical and laboratory data of 33 pediatric patients operated upon for uncomplicated atrial septal defect at the University of Minnesota Hospitals between January, 1972, and December, 1973, have been reviewed. In each case the diagnosis was confirmed at operation or by cardiac catheterization. Patients with ostium primum atrial defects have been excluded from this series. See related article, p. 319. Clinical evaluation, Each patient suspected of having an atrial septal defect was examined by at least two members of the pediatric cardiology staff. After the history was obtained and physical examination performed, cardiac sounds were viewed on a phonocardioscope. A s t a n d a r d 12-lead e l e c t r o c a r d i o g r a m and thoracic roentgenograms were obtained from each patient. Vectorcardiograms, as well as echocardiograms a n d technetium 99 radionuclide scintillation studies, were obtained from many patients. The application of these techniques in the evaluation of atrial septal defect has been previously described. 25 The clinical criteria considered necessary to establish the diagnosis of atrial septal defect were: (1) a systolic ejection m u r m u r at the upper left sternal border of
VoL 86, No. 2, pp. 189-193
190
Neal et al.
The Journal of Pediatrics February 1975
Table I. Indications for cardiac catheterization in 14 patients operated upon for atrial septal defect Patient 1 2 3 4 5 6 7 8* 9* 10 11 12 13 14
J
Reason.[or catheterization
Cardiac defects
Variable splitting $2; no IRBBB Variable splitting $2; no IRBBB Var!able splitting $2; ejection click Roentgenogram suggestive of L-TGV Holt-Oram syndrome; atypical systolic murmur Variable splitting $2; no IRBBB; ejection click History of CHF during infancy Variable splitting $2; no IRBBB; no diastolic murmur Variable splitting S2 Variable splitting $2; atypical systolic murmur History ofcyanosis and CHF during infancy Narrow, fixed splitting $2; atypical systolic murmur Variable splitting $2; abnormal P wave axis; no IRBBB No atypical findings, catheterization prior to referral
ASD (secundum) ASD (secundum) ASD (secundum) ASD (secundum) ASD (secundum) ASD (secundum) ASD (secundum) ASD (secundum) ASD (secundum) ASD (secundum) ASD (sinus venosus) PAPVC ASD (sinus venosus) ASD (fenestrated) ASD (secundum)
Qp:Qs = p u l m o n a r y to systemic flow ratio; s 2 = second h e a r t sound; I RBBB = incomplete right b u n d l e branc h block; ASD = atrial septal defect; L - T G V = L-transposition o f the great vessels; C H F = congestive heart failure; P A P V C = partial a n o m a l o u s p u l m o n a r y v e n o u s connection; PS = p u l m o n a r y
stenosis. *Qp:Qs) 1.5:1.0,( 2.0:1.0.Allother patientsQp:Qs) 2.0:t.0.
Radioisotope Flow Study KW, ASD Age 6yrs. svc RL
"'~ ~
'
i'~
'
LL
PV
Fig. 1. Radionuclide angiocardiogram in a patient with a secundum type atrial septal defect. Regions of interest, such as the right atrium (RA) and lung fields (RL and LL), are selected for computer scanning and time-activity curves derived. The atrial septal defect is not visualized, but recirculation of isotope through the lungs produces an abnormal time-activity curve. Grade 2-3/6 intensity; (2) wide splitting of the second heart sound with little or no respiratory variation; and (3) a tricuspid flow m u r m u r in diastole. Confirmatory laboratory evidence included: (1) an electrocardiogram showing mild right axis deviation in the frontal plane, a n d an i n c o m p l e t e right b u n d l e branch block pattern in VaR and V1; and (2) a thoracic roentgenogram interpreted either as normal or, as is
more often the case, showing mild cardiomegaly and increased pulmonary vascular markings. All patients r e c o m m e n d e d for operative repair of atrial septal defect w i t h o u t cardiac c a t h e t e r i z a t i o n fulfilled the criteria for diagnosis described above. As our experience with echocardiography and technetium 99 cardiac flow studies has increased, we have increasingly relied on these noninvasive techniques to substantiate the clinical diagnosis. Positive confirmat i o n of the presence of a left-to-right s h u n t by radionuclide cardiac angiography is now considered advisable in each patient scheduled for operation without cardiac catheterization. Cardiac catheterization. W h e n the diagnosis of uncomplicated atrial septal defect with a pulmonary to systemic flow ratio of greater than 1.5:1 could not be established with sufficient certainty, cardiac catheterization was performed. An increase in oxygen saturation at the atrial level greater than 1.5 volumes percent on at least two right-sided oximetry series was considered necessary to establish the presence of a left-to-right shunt. Additionally, confirmation was obtained by the indicator dilution technique, and frequently by pulmonary arteriography. RESULTS Thirty-three pediatric patients were operated upon for closure of atrial septal defects of s e c u n d u m or sinus venosus type during the two-year period of this study. All had defects greater than 2.0 cm in diameter. Their ages ranged between 5 and 18 years. None had additional cardiac defects with the exception of one patient
Volume 86 Number 2
R e p a i r o f atrial septal defect without catheterization
191
Radioisotope Flow Study Activity Curves KW Age 6yrs. ASD Rt. Atrium
Pulm
Rt. Vent
Art.
~
,e,
.:
=
," II
...,
~ ",:..2::.,./..l.~::t:;v~.:.:.,.~."
II
Rt. Lung
"*"
Lt. Lung
t
9.
"r162
;.,;;;P"l % . t - ;
I
,
**'..%
9 , . . . . *:,,.,
J
Fig. 2. Time-activity curves derived from various regions of interest in a radionuclide angiocardiographic study of a child with an atrial septal defect. The curves obtained by scanning the lung fields are abnormal due to recirculation of 99mTc secondary to a left-to-right shunt. who had c o e x i s t e n t partial a n o m a l o u s p u l m o n a r y venous connection. N i n e t e e n of 33 p a t i e n t s (58%) had clinical and laboratory features typical of atrial septal defect and were operated upon without prior cardiac catheterization. The 14 remaining patients (42%) required cardiac catheterization to confirm the diagnosis of uncomplicated atrial septal defect" and quantitate the magnitude of the left-to-right shunt. Significant respiratory variation in the degree of splitting of the second heart sound (eight patients) and absence of incomplete right bundle branch block pattern on the electrocardiogram (five patients) were the most frequently encountered atypical findings in these children (Table I). Excluded from this study are two additional patients who did not fulfill the criteria for operation without hemod}namic study and were found to have small secund u m atrial defects (Qp:Qs (1.5:1.0) at catheterization. Surgery was not performed. DISCUSSION Before palliative or corrective operations on patients with congenital heart disease can be safely performed, an accurate diagnosis is necessary. Usually this necessitates p r e o p e r a t i v e cardiac c a t h e t e r i z a t i o n , i n c l u d i n g selective angiography. There are situations, however,
when it is possible to make a diagnosis on the basis of clinical and laboratory findings with a high degree of accuracy, obviating the need for cardiac catheterization 9 It may be questioned whether this approach can be safely recommended for operations requiring cardiopulmonary bypass. Analysis of the pre- and postoperative diagnoses of 196 patients with congenital heart disease referred to the Mayo Clinic suggests that cardiac catheterization is not always necessary, l Operation was advised on the basis of clinical findings alone in 106 patients (54%), though the series included a variety of types of congenital heart disease. In only four cases were unsuspected lesions e n c o u n t e r e d which significantly a l t e r e d the surgical approach. Twelve patients were operated upon with a clinical diagnosis of atrial septal defect, one of whom was found to have additional partial anomalous pulmonary venous connection. The association o f partial a n o m a l o u s p u l m o n a r y venous connection with atrial septal defect, particularly defects of the sinus venosus type, is well known. In the majority of cases it is not possible to suspect this additional a n o m a l y w i t h o u t cardiac c a t h e t e r i z a t i o n , and even then it may be missed unless specifically looked for. The surgeon, however, can readily identify anomalous venous connection a n d can nearly always correct
192
~
Neal et al.
-
-
The Journal of Pediatrics February 1975
I
CHEST
WAL
I
I
~rlrfldo•
.10
Db
~
Fig. 3. Simultaneous recording of an echocardiogram, ECG, and phonocardiogram in a child with an atrial septal defect. The right ventricle (R V) is enlarged and there is simultaneous anterior motion of the septum and posterior left ventricular wall during systole, referred to as "paradoxical" septal motion. These echocardiographic features are frequently present in volume overload of the RV, such as with atrial septal defect.
the defect regardless of whether or not the diagnosis was known preoperatively. The location of the atrial septal defect can frequently be predicted with accuracy by clinical and laboratory means. 60stium primum atrial defects usually have electrocardiographic evidence of left axis deviation in the frontal plane with a figure of eight or counterclockwise vector loop. Sinus venosus defects have left axis deviation of the P wave vectors in nearly half the c a s e s ] and suspicion of this defect alerts one to the possibility of associated partial anomalous pulmonary venous connection. Nevertheless, with the exception of ostium primum lesions, knowledge of the exact location of the atrial defect preoperatively has not been considered necessary. Coexistent atrial and ventricular septal defects occasionally occur, but the physical findings, electrocardiogram, and chest roentgenogram are similar to those of a moderate to large ventricular septal defect. 8 Lutembacher's syndrome (atrial septal defect and rheumatic mitral valve disease) 9 is very u n c o m m o n in children and would have different auscultatory findings; moreover, echocardiography can be usefully applied to rule out coexistent mitral valve disease. Technetium pertechnetate cardiac flow studies provide a relatively sensitive means of determining the
presence or absence of a left-to-right shunt, but it is not possible to determine the location of the defect (Figs. 1 and 2). Echocardiography reveals paradoxical septal motion and increased right ventricular internal dimension in many patients with volume overload of the right ventricle (Fig. 3). However, paradoxical septal motion also has been described with normal right heart hemodynamics, 1~and normal septal motion has been found in patients with atrial septal defect. 1~By combining the information obtained by noninvasive techniques with the clinical and laboratory data, operative repair of atrial septal defect without prior cardiac catheterization can be safely recommended. The advantages of eliminating cardiac catheterization when possible are obvious. Though the risk is minimal when performed on a child beyond infancy, it is still an invasive procedure potentially complicated by arrhythmias, perforation of the heart, and allergic reactions to contrast media. Femoral or brachial artery thrombosis occurs frequently enough to warrant concern, regardless of whether the percutaneous or open arteriotomy technique is used. Finally, it is an expensive procedure which usually requires one or two days of hospitalization. We think that the pediatric patient with typical clinical and laboratory features of atrial septal defect can be
Volume 86 Number 2
referred for operation w i t h o u t cardiac catheterization. If atypical features are present, t h e n additional study is advisable. REFERENCES
l.
2.
3.
4.
5.
Pestana C, Weidman WH, Swan HJC, and McGoon DC: Accuracy of preoperative diagnosis in congenital heart disease, Am Heart J 72:446, 1966. Diamond MA, Dillon JC, Haine CL, Chang S, and Feigenbaum H: Echocardiogr.aphic features of atrial septal defect, Circulation 43:129, 1971. Tajik AJ, Gau GT, Ritter DG, and Schattenberg TT: Echocardiographic pattern of right ventricular diastolic volume overload in children, Circulation 46:36, 1972. Folse R, and Braunwald E: Pulmonary vascular dilution curves recorded by external detection in the diagnosis of left-to-right shunts, Br Heart J 24:166, 1962. Rosenthal L: Nucleographic screening of patients with left-to-right cardiac shunts, Nucl Med 99:601, 1971.
Repair of atrial septal defect without catheterization
19 3
6. DuShane JW, Weidman WH, Brandenburg RO, and Kirklin JW: Differentiation of interatrial communications by clinical methods, Circulation 21:363, 1960. 7. Davia JE, Cheitlin MD, and Bedynek JL: Sinus venosus atrial septal defect: Analysis of fifty cases, Am Heart J 85:177, 1973. 8. Prado S, Adams P Jr, Amplatz K, Girod D, and Anderson RC: Coexistent atrial and ventricular septal defects: A report of twenty-two cases, Dis Chest 51:412, 1967. 9. Bashour RA, and Simmons DH: Atrial septal defect with mitral valvulitis: Clinical and catheterization diagnosis, Ann Intern Med 48:1194, 1957. 10. Cody MC, Giuliani ER, and Mair DD: "Paradoxical" septal motion by echocardiography in a patient with normal right heart hemodynamics, Mayo Clin Proc 48:31, 1973. 11. Tajik AJ, Gau GT, Schattenberg TT, and Ritter DG: Normal ventricular septal motion in atrial septal defect, Mayo Clin Proc 47:635, 1972.
