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Upright T wave in V1: A useful diagnostic sign of mild pulmonic valve stenosis in children
March, 1969 T h e J o u r n a l o[ P E D I A T R I C S
413
Upright T wave in A useful diagnostic sign of miM pulmonic valve stenosis in children In 9 o[ 10 children aged 5 to 10 years with mild pulmonary value stenosis, confirmed at cardiac catheterization and cineangiocardiography, the T wave in V~ was upright in an otherwise normal electrocardiogram. This compared with absence o[ an upright T wave concluded that the presence o[ an upright T wave in V1 in children with a systolic in V1 in 6 cases o[ atrial septal deject and in 100 cases o[ Junctional murmurs. It is murmur and an otherwise normal electrocardiogram is strongly suggestive o[ mild pulmonary valve stenosis.
j. M. Celermajer, M.B.,* T. Izukawa, M.D., P. J. Varghese, M.B., and R. D. Rowe, M.D. B A L T I M O R E , 1VID.
TI-IE CLINICAL DIAGNOSIS Of pulmonary valve stenosis in children is simple
in the presence of classical physical signs and characteristic electrocardiogram and chest roentgenogram. However, in some patients with mild pulmonic stenosis, the physical signs are inconclusive and the roentgenogram and electrocardiogram are relatively normal, making the lesion difficult to distinguish from other conditions such as functional murmur and atrial septal defect. An upright T wave in lead V1 in children after the immediate neonatal period has
From the Department o[ Pediatrics, Cardiac Clinic, Children's Medical and Surgical Center, The ]ohns Hopkins Hospital. Supported in part by United States Public Health Service Grants Nos. 5 TO1 H E 0 5 2 3 7 and L R O L HEI0718. *Supported by an Overseas Clinical Fellowship 0C7 or the National Heart Foundation oJ Australia.
been described as abnormaP -3 and representing evidence of right ventricular hypertrophy?, 4-7 We have observed that this electrocardiographic finding may be helpful in the diagnosis of mild pulmonary valve stenosis when the electrocardiogram shows no other sign of right ventricular hypertrophy. M A T E R I A L S AND M E T H O D S The records of children with isolated pulmonary valve stenosis who underwent cardiac catheterization and cineangiocardiography at The Johns Hopkins Hospital, and in whom the electrocardiogram showed no evidence of right ventricular hypertrophy with the possible exception of upright T wave in V~, were examined. The age group selected was restricted to patients between 5 and 10 years for two reasons: (1) In younger children with mild pulmonary valve stenosis, cardiac catheterization is not performed. (2) Vol. 74, No. 3, pp. 413-415
4 14
Celerma]er et al.
The Journal of Pediatrics March 1969
After the age of 10 years the finding of an upright T wave in V, has no significance.* A total of 10 patients satisfied the criteria. T h e right ventricular systolic pressure ranged from 34 to 63 mm. Hg, the peak systolic pressure difference across the valve being 15 to 48 mm. H g in 9 cases and 10 mm. H g in the tenth patient. For control groups, we examined the records and electrocardiograms of children in the corresponding age group who had the following conditions: (a) functional murmur, diagnosed clinically--100 children, (b) isolated atrial septal defect, confirmed by cardiac catheterization, with no evidence of right ventricular hypertrophy--6 cases; the right ventricular systolic pressure was in the same range as in the patients with mild pulmonary valve stenosis. In all patients, standard 13 lead scalar electrocardiograms were recorded with a Sanborn Visocardiette model 100 direct writer. In the catheterized patients, the elec-
trocardiograms were almost invariably obtained during the 24 hours preceding catheterization. RESULTS
The incidence of the T wave pattern in the different diagnostic groups is shown in Table I. Upright T waves in V, were present in 9 of the 10 children with mild pulmonary valve stenosis (Fig. 1). By contrast, none among 6 with atrial septal defect and 100 with functional m u r m u r had this electrocardiographic finding. The frequency of upright T wave in V , in patients with pulmonary valve stenosis was compared with its incidence in all other groups taken singly or in combination. In all instances the difference was statistically highly significant (p < 0.001). DISCUSSION
In none of our 10 patients with mild pulmonary valve stenosis, confirmed by cardiac
Fig. 1. Electrocardiogram of a child aged 9 years with mild pulmonary valve stenosis, showing upright T wave in Vs.
Table I
Diagnosis
Pulmonic stenosis Functional murmur Atrial septal defect
Diagnostic criteria
Cardiac cathetei~ization Clinical Cardiac catheterization
_
T wave in V1 Upright
I
Inverted
1 99 6
t
Biphasic
Total
0 1 0
10 100 6
Volume 74 Number 3
catheterization, was the diagnosis firmly established on clinical grounds. O t h e r diagnoses considered in these patients included atrial septal defect, idiopathic dilatation of the pulmonary artery, and functional murmur. T h e importance of making the diagnosis of mild pulmonary valve stenosis is the benign and nonprogressive nature of this lesion in childhood.* Bassingthwaite and associates 5 briefly alIuded to the occasional presence of upright T wave in V1 in children with mild pulmonic stenosis. I-ternandez, 6 reporting on right ventricular pressure work, illustrated 3 of 4 cases of mild pulmonary valve stenosis, aged less than 10 years and with right ventricular systolic pressures of 40 to 56 mm. Hg, who h a d upright T waves in V1 but otherwise normal electrocardiograms. O n the other hand, Cayler and associates, 7 in reviewing the electrocardiographic findings in 105 patients with pulmonic stenosis, failed to mention upright T waves in V1 in their mild cases. T h e configuration of the T wave in V~ normally varies with age. 1-s A n upright T wave is seen in V1 during the neonatal period up to the age of 4 days? -3 Later the T wave is normally inverted throughout childhood until the age of 12 to 16 years, when it frequently becomes upright in normal people? An upright T wave in V1 in the absence of T wave inversion in V5 and V6 in neonates has been attributed to elevated right ventricular systolic pressure? -3 I n children, an upright T wave in V1, in conjunction with other criteria, has been described as indicative of right ventricular hypertrophy 1, ~, 5, 3, 10 or pressure overwork.5, 6, s, 10 T h e right ventricular systolic pressure was elevated in all of our patients, but such mild elevation m a y occur in situations of increased flow in which the upright T wave in V1 is not usually present, e.g., atrial septal defect. It is possible, although purely speculative, that this sign m a y therefore be a reflection of the pattern of work of the ven-
Pulmonic valve stenosis
4 15
tricle against a fixed mild obstruction, rather than a direct indication of an increased systolic pressure alone or any anatomical abnormality of the m y o c a r d i u m of the right ventricle. W h e n other electrocardiographic criteria of right ventricular hypertrophy are present, the direction of the T wave in V1 is no longer found to be helpful. I n fact, in severe right ventricular hypertrophy the T waves are usually deeply inverted. ~, 7 REFERENCES
1. Ziegler, R. F.: Electrocardiographic studies in normal infants and children, Springfield, Ill., 1951, Charles C Thomas, Publisher, p. 77. 2. DePasquale, N. P., and Burch, G. E.: The electrocardiogram, ventricular gradient and spatial vectorcardiogram during the first week of life, Am. J. Cardiol. 12: 482, 1963. 3. Hait, G., and Gasul, M." The evolution and significance of T wave changes in the normal newborn during the first seven days of life, Am. J. Cardiol. 12: 494, 1963. 4. Proudfit, W. L., and Tapia, F. A.: Significance of upright T waves in right precordial leads in infants and children, Circulation 20: 753, 1959. (Abst.) 5. Bassingthwaite, ]'. B., Parkln, T. W., DuShane, J. W., Wood, E. H., and Burchell, H. B.: The electrocardiographic and hemodynamic findings in pulmonary stenosis with intact ventricular septum, Circulation 28: 893, 1963. 6. Hernandez, F. A.: Right ventricular pressure work and hypertrophy in Cassels, D. E., and Ziegler, R. F., editors: Electrocardiography in infants and children, New York, I966, Grune & Stratton, Inc., p. 182. 7. Cayler, G. C., Ongley, P., and Nadas, A.: Relation of systolic pressure in the right ventricle to the electrocardiogram. A study of patients with pulmonary stenosis and intact ventricular septum, New England J. Med. 258: 979, 1958. 8. Moller, J. H., and Adams, P., Jr.: The natural history of pulmonary valvular stenosis. Serial catheterizations of 21 children, Am. J. Cardiol. 16: 654, 1965. 9. Ziegler, R. F.: The importance of positive T waves in the right precordial electrocardiogram during the first year of life, Am. Heart J. 52: 533, 1956. 10. Liebman, J.: The normal electrocardiogram in newborns and infants (a critical review), in Cassels, D. E,, and Ziegler, R. F., editors: Electrocardiography in infants and children, New York, 1966, Grune & Stratton, Inc., p. 79.
413
Upright T wave in A useful diagnostic sign of miM pulmonic valve stenosis in children In 9 o[ 10 children aged 5 to 10 years with mild pulmonary value stenosis, confirmed at cardiac catheterization and cineangiocardiography, the T wave in V~ was upright in an otherwise normal electrocardiogram. This compared with absence o[ an upright T wave concluded that the presence o[ an upright T wave in V1 in children with a systolic in V1 in 6 cases o[ atrial septal deject and in 100 cases o[ Junctional murmurs. It is murmur and an otherwise normal electrocardiogram is strongly suggestive o[ mild pulmonary valve stenosis.
j. M. Celermajer, M.B.,* T. Izukawa, M.D., P. J. Varghese, M.B., and R. D. Rowe, M.D. B A L T I M O R E , 1VID.
TI-IE CLINICAL DIAGNOSIS Of pulmonary valve stenosis in children is simple
in the presence of classical physical signs and characteristic electrocardiogram and chest roentgenogram. However, in some patients with mild pulmonic stenosis, the physical signs are inconclusive and the roentgenogram and electrocardiogram are relatively normal, making the lesion difficult to distinguish from other conditions such as functional murmur and atrial septal defect. An upright T wave in lead V1 in children after the immediate neonatal period has
From the Department o[ Pediatrics, Cardiac Clinic, Children's Medical and Surgical Center, The ]ohns Hopkins Hospital. Supported in part by United States Public Health Service Grants Nos. 5 TO1 H E 0 5 2 3 7 and L R O L HEI0718. *Supported by an Overseas Clinical Fellowship 0C7 or the National Heart Foundation oJ Australia.
been described as abnormaP -3 and representing evidence of right ventricular hypertrophy?, 4-7 We have observed that this electrocardiographic finding may be helpful in the diagnosis of mild pulmonary valve stenosis when the electrocardiogram shows no other sign of right ventricular hypertrophy. M A T E R I A L S AND M E T H O D S The records of children with isolated pulmonary valve stenosis who underwent cardiac catheterization and cineangiocardiography at The Johns Hopkins Hospital, and in whom the electrocardiogram showed no evidence of right ventricular hypertrophy with the possible exception of upright T wave in V~, were examined. The age group selected was restricted to patients between 5 and 10 years for two reasons: (1) In younger children with mild pulmonary valve stenosis, cardiac catheterization is not performed. (2) Vol. 74, No. 3, pp. 413-415
4 14
Celerma]er et al.
The Journal of Pediatrics March 1969
After the age of 10 years the finding of an upright T wave in V, has no significance.* A total of 10 patients satisfied the criteria. T h e right ventricular systolic pressure ranged from 34 to 63 mm. Hg, the peak systolic pressure difference across the valve being 15 to 48 mm. H g in 9 cases and 10 mm. H g in the tenth patient. For control groups, we examined the records and electrocardiograms of children in the corresponding age group who had the following conditions: (a) functional murmur, diagnosed clinically--100 children, (b) isolated atrial septal defect, confirmed by cardiac catheterization, with no evidence of right ventricular hypertrophy--6 cases; the right ventricular systolic pressure was in the same range as in the patients with mild pulmonary valve stenosis. In all patients, standard 13 lead scalar electrocardiograms were recorded with a Sanborn Visocardiette model 100 direct writer. In the catheterized patients, the elec-
trocardiograms were almost invariably obtained during the 24 hours preceding catheterization. RESULTS
The incidence of the T wave pattern in the different diagnostic groups is shown in Table I. Upright T waves in V, were present in 9 of the 10 children with mild pulmonary valve stenosis (Fig. 1). By contrast, none among 6 with atrial septal defect and 100 with functional m u r m u r had this electrocardiographic finding. The frequency of upright T wave in V , in patients with pulmonary valve stenosis was compared with its incidence in all other groups taken singly or in combination. In all instances the difference was statistically highly significant (p < 0.001). DISCUSSION
In none of our 10 patients with mild pulmonary valve stenosis, confirmed by cardiac
Fig. 1. Electrocardiogram of a child aged 9 years with mild pulmonary valve stenosis, showing upright T wave in Vs.
Table I
Diagnosis
Pulmonic stenosis Functional murmur Atrial septal defect
Diagnostic criteria
Cardiac cathetei~ization Clinical Cardiac catheterization
_
T wave in V1 Upright
I
Inverted
1 99 6
t
Biphasic
Total
0 1 0
10 100 6
Volume 74 Number 3
catheterization, was the diagnosis firmly established on clinical grounds. O t h e r diagnoses considered in these patients included atrial septal defect, idiopathic dilatation of the pulmonary artery, and functional murmur. T h e importance of making the diagnosis of mild pulmonary valve stenosis is the benign and nonprogressive nature of this lesion in childhood.* Bassingthwaite and associates 5 briefly alIuded to the occasional presence of upright T wave in V1 in children with mild pulmonic stenosis. I-ternandez, 6 reporting on right ventricular pressure work, illustrated 3 of 4 cases of mild pulmonary valve stenosis, aged less than 10 years and with right ventricular systolic pressures of 40 to 56 mm. Hg, who h a d upright T waves in V1 but otherwise normal electrocardiograms. O n the other hand, Cayler and associates, 7 in reviewing the electrocardiographic findings in 105 patients with pulmonic stenosis, failed to mention upright T waves in V1 in their mild cases. T h e configuration of the T wave in V~ normally varies with age. 1-s A n upright T wave is seen in V1 during the neonatal period up to the age of 4 days? -3 Later the T wave is normally inverted throughout childhood until the age of 12 to 16 years, when it frequently becomes upright in normal people? An upright T wave in V1 in the absence of T wave inversion in V5 and V6 in neonates has been attributed to elevated right ventricular systolic pressure? -3 I n children, an upright T wave in V1, in conjunction with other criteria, has been described as indicative of right ventricular hypertrophy 1, ~, 5, 3, 10 or pressure overwork.5, 6, s, 10 T h e right ventricular systolic pressure was elevated in all of our patients, but such mild elevation m a y occur in situations of increased flow in which the upright T wave in V1 is not usually present, e.g., atrial septal defect. It is possible, although purely speculative, that this sign m a y therefore be a reflection of the pattern of work of the ven-
Pulmonic valve stenosis
4 15
tricle against a fixed mild obstruction, rather than a direct indication of an increased systolic pressure alone or any anatomical abnormality of the m y o c a r d i u m of the right ventricle. W h e n other electrocardiographic criteria of right ventricular hypertrophy are present, the direction of the T wave in V1 is no longer found to be helpful. I n fact, in severe right ventricular hypertrophy the T waves are usually deeply inverted. ~, 7 REFERENCES
1. Ziegler, R. F.: Electrocardiographic studies in normal infants and children, Springfield, Ill., 1951, Charles C Thomas, Publisher, p. 77. 2. DePasquale, N. P., and Burch, G. E.: The electrocardiogram, ventricular gradient and spatial vectorcardiogram during the first week of life, Am. J. Cardiol. 12: 482, 1963. 3. Hait, G., and Gasul, M." The evolution and significance of T wave changes in the normal newborn during the first seven days of life, Am. J. Cardiol. 12: 494, 1963. 4. Proudfit, W. L., and Tapia, F. A.: Significance of upright T waves in right precordial leads in infants and children, Circulation 20: 753, 1959. (Abst.) 5. Bassingthwaite, ]'. B., Parkln, T. W., DuShane, J. W., Wood, E. H., and Burchell, H. B.: The electrocardiographic and hemodynamic findings in pulmonary stenosis with intact ventricular septum, Circulation 28: 893, 1963. 6. Hernandez, F. A.: Right ventricular pressure work and hypertrophy in Cassels, D. E., and Ziegler, R. F., editors: Electrocardiography in infants and children, New York, I966, Grune & Stratton, Inc., p. 182. 7. Cayler, G. C., Ongley, P., and Nadas, A.: Relation of systolic pressure in the right ventricle to the electrocardiogram. A study of patients with pulmonary stenosis and intact ventricular septum, New England J. Med. 258: 979, 1958. 8. Moller, J. H., and Adams, P., Jr.: The natural history of pulmonary valvular stenosis. Serial catheterizations of 21 children, Am. J. Cardiol. 16: 654, 1965. 9. Ziegler, R. F.: The importance of positive T waves in the right precordial electrocardiogram during the first year of life, Am. Heart J. 52: 533, 1956. 10. Liebman, J.: The normal electrocardiogram in newborns and infants (a critical review), in Cassels, D. E,, and Ziegler, R. F., editors: Electrocardiography in infants and children, New York, 1966, Grune & Stratton, Inc., p. 79.