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Cor triatriatum
Cor triatricstum Correlation
of clinical
and autopsy
findings
in 3 cases
A. C. Papaioannou, M.D. J. Nihoyanopoulos, M.D. J. Vlachos, M.D. Athens, Greece
C
or triatriatum is a rare congenital heart malformation, amenable to simple corrective surgery. A preoperative diagnosis is, therefore, of paramount importance. Very few cases so far have been diagnosed during life and, to our knowledge, in no more than 9 caseshas operation been successful.1-8 Because of the rarity of this anomaly and the diversity of the symptoms with which it may be manifested, we wish to report 3 additional cases. In our discussion we have made an attempt to correlate the clinical and autopsy findings in these cases. It is noteworthy that in our third case the abnormal septum was found in the right atrium. Only 4 such cases have been reported previously. g Case reports Case 1. A.X., a 19-month-old
male infant, was admitted to the Pediatric Clinic of the University of Athens, with a history of fever, anorexia, and failure to thrive. The infant weighed 3,800 grams at birth, and his development during the first year of life was said to be normal. The patient thereafter developed “attacks” of dyspnea, which were aggravated in the last 2 months prior to his admission. Physical examination revealed a slightly cyanotic child with marked respiratory distress. The heart was enlarged to percussion and palpation. The pulmonic component of the second sound was accentuated. A Grade 2-3, short systolic murmur was best heard over the left upper sternal From the Department of Pediatrics, University Received for publication Feb. 5, 1962.
12
border. The lungs had moist rPles bilaterally. The liver was enlarged 3 fingerbreadths below the right costal margin. The peripheral pulses were well palpable. The rest of the examination was not contributory. A chest roentgenogram showed an enlarged heart and a prominent pulmonary conus (Fig. 1). The electrocardiogram indicated right atria1 enlargement and right ventricular hypertrophy of marked degree. The patient was digitalized, and oxygen, diuretics, and antibiotics were administered. However, his condition deteriorated rapidly, and he died on the second hospital day. At autopsy, the left atrium was found to be divided by an anomalous diaphragm into two chambers. The mediosuperior chamber received all the pulmonary veins and had the shape of an inverted truncated cone. Its superior surface measured 2.8 by 3.0 cm., and its inferior surface, 1.4 by 1.2 cm. In the diaphragm were two openings, about 0.7 and 0.2 cm. in diameter, which communicated with the inferior true left atrium. Another small opening, about 0.3 cm. in diameter, led the probe into the right atrium (Fig. 2), which was dilated. The left atrium was of normal size. The right ventricle was dilated and hypertrophied; the anterior wall was about 7 mm. in thickness. The wall of the left ventricle also measured 7 mm. The valves were regular. Case 2. P.S., an d-month-old boy, was first seen in Cook County Children’s Hospital, Chicago, at the age of 2 months because of “pneumonia.” Since then the child was said to have been slightly blue and to have had repeated respiratory infections. On examination, his skin had a dusky color, but no clubbing or definite cyanosis were present. The heart was enlarged to palpation. The pulmonary closure of the second sound was accentuated. A Grade 2-3, short systolic murmur was best heard over the third to fourth left intercostal spaces. No of Athens (St. Sophia’s Children’s
Hospital,
Goudi, Athens, Greece).
Cor triutriatum
13
was attached to the endocardial surface of the posterior lateral portion of the right atrium. The inferior compartment received both venae cavae in a normal manner and contained the auricular appendage, which was dilated. There were two defects, one above and the other below the diaphragm, in the septum primum, measuring 0.8 by 0.5 cm. and 1.0 by 0.8 cm., respectively. These communicated with the left atrium. The foramen ovale was closed. All the pulmonary veins entered a sac-like venous sinus which communicated with each atrium through a small ostium. The tricuspid orifice had a circumference of 5.7 cm., and the right ventricle was hypertrophied. The chambers of the left side of the heart were otherwise normally formed. The ductus was closed. Fig. 1. Case anterior view.
1.
Chest
roentgenogram,
postero-
thrill was palpable and the femoral pulses were normal. Chest x-ray examination showed an increased cardiac shadow with a prominent pulmonary artery (Fig. 3). The lung fields appeared to be somewhat congested. The electrocardiogram indicated marked right atria1 enlargement and right ventricular hypertrophy. A venous angiocardiogram was made. The right atrium was dilated and remained opacified during levoangiocardiography. No other anomalies were detected. An atria1 septal defect or anomalous venous drainage was suspected clinically. A surgical exploration was attempted; only a large septal defect was found and this was closed. Two days later the patient died. At autopsy, the left atrium was found to be separated into two chambers by an abnormal diaphragm. The upper accessory chamber communicated with the right atrium through a large septal defect, and with the left atrium through an opening in the diaphragm. As seen in retrospect, this opening was mistaken for the mitral valve by the exploring finger of the surgeon. Case 3. J.F., a f-month-old female infant, was admitted to the Pediatric Cardiology Department of Cook County Hospital, Chicago, because she had had dyspnea and cyanosis since the age of 6 weeks. On physical examination the infant appeared to be dyspneic, and mild cyanosis was present in both the upper and the lower extremities. The cardiac apex was felt over the fifth left intercostal space and at the mid-clavicular line. There was a loud pulmonary closure sound over the second and third left intercostal spaces. A Grade 3 systolic murmur accompanied by a thrill was best heard in the same area. Chest x-ray films revealed an increased diameter of the heart and a convexity of the heart shadow in the pulmonary area. The electrocardiogram was suggestive of right atria1 enlargement and biventricular hypertrophy. The patient was catheterized, and the data are shown in Table I. The patient subsequently died of pulmonary infection. At autopsy, the heart was found to be enlarged, particularly the chambers on the right side. The right atrium was divided into two compartments by a part ially fenestrated fibrous diaphragm, which
Discussion
Niwayama,g in a critical review of the literature prior to 1960, found only 38 cases reported, to which he added 5 of his own. Since then, about 11 cases have been recorded,4~6-8,10-12 which brings the total number to 54. The majority of recent workers support the explanation suggested by Griffith,I$ namely, that early embryology is responsi-
Fig. 2. Case 1. Posterior view of the heart. The pulmonary veins (P V) enter the “accessory” atrium (CP V). The vertical probe (a) is inserted through an opening from the “accessory” atrium into the left atrium. The probe (b) leads into the right atrium. The number 2 is at the orifice of the removed inferior vena cava.
14
Papaioannou,
Nihoyanopoulos,
and Vlachos
venous return. The smaller the size of the communication, the earlier the symptoms will appear. In about two thirds of the reported cases, death occurred during the first 2 years of life.gJO However, if the fenestration is very large, no obstruction at all will occur and these patients live to adult life.13j14 In some patients there were multiple fenestrations in the septum, and in 3 cases, none.g In such instances an anomalous venous drainage or defects above and below the septum are essential for survival. In 4 patients the abnormal diaphragm was found in the right atrium,Y and here is supposed to represent a persistent right valve of the sinus venosus.IL There appears to be a goodly number of anatomic variants of this anomaly, depending on the presence or absence of interatrial communications which may further complicate the clinical features. If, for instance, no interatrial communication exists and the exit from the “accessory” chamber to the left atrium is stenotic, the hemodynamic alterations and clinical manifestations may be similar to those of congenital mitral stenosis, stenosis or atresia of a common pulmonary vein, or endocardial fibroelastosis of the constrictive type.3J0,11s16 If an interatrial communication exists (open foramen ovale or atria1 defect) between the “accessory” chamber and the right atrium only, a left-to-right shunt will occur, imitating an atria1 septal defect and, in more severe cases, a Lutembacher complex.gJ6 In case of a large communication between the lower “true” atrium and the right atrium, a right-to-left shunt may occur, and, if both upper and lower chambers communicate with the right atrium, the flow may
Fig. 3. Case 2. Chest roentgenogram, posteroanterior view.
ble for this anomaly. According to this which view, the third atria1 chamber exists in this malformation represents, in fact, a dilated common pulmonary vein which failed to be incorporated into the left atrium. In car triatriatum an abnormal septum (diaphragm) divides the left atrium, or rarely the right, into two separate chambers which usually communicate through a narrow opening. The upper (accessory) atrium receives all the pulmonary veins, and the lower (true) atrium contains the atria1 appendage and the mitral (or tricuspid) valve. In the most common cases the opening of the anomalous septum is small and produces considerable obstruction to the pulmonary Table I. Catheterization
data from right side of heart in Case 3 Location
SVC
Oxygen content (vol. %) Pressure (mm. Hg) Oxygen saturation (%) Oxygen capacity (vol. 75) SVC:
Superior
vena
cava.
WC:
/
9.1
IVC
1
11.8
RA
)
RV
14.8 (4)
--
1
15.5 66/l
LA
1
FA
15.4 (7) 88.6
15.1 105/60 86.5
FA: Femoral
artery.
17.4
Inferior
vena
can.
RA:
Rirtht
atrium.
RV:
Right
ventricle.
LA:
Left
atrium.
Cor triatriatum
be bidirectional. Then a clinical cyanosis may appear, in proportion to the degree of unsaturated blood passing into the systemic circulation. Furthermore, if the abnormal septum is located in the right atrium, as in our third case, the condition may imitate an atria1 septal defect with a right-to-left shunt or an anomalous venous drainage, depending on the interatria1 communications. The clinical picture can be still more complicated if additional heart anomalies are associated.g-ll The most commonly reported manifestations in cases of car triatriatum were attacks of dyspnea and pulmonary edema, enlargement of the right side of the heart, and congestive heart failure. Analysis of the auscultatory findings in our 3 cases revealed a short systolic murmur over the upper left parasternal area and an accentuated pulmonary closure in all. Nonspecific murmurs and accentuation of the second sound have been noted in previous reports.6~R~gJ1J2 The roentgenographic and electrocardiographic findings in all our cases were suggestive of marked right ventricular (biventricular, in the third patient) hypertrophy and right atria1 dilatation. The pulmonary vascular markings and pulmonary conus were prominent; this is also commonly described in the literature. Venous angiocardiography which was performed in our second patient was not of definite help. There are not many angiocardiographic reports, but we believe that the techniques of cineangiocardiography from the pulmonary artery can be of some value in the diagnosis of car triatriatum if this condition is suspected. Catheterization of the right side of the heart of our third patient (Table I) showed an increased oxygen saturation in the chambers of the right side of the heart which was approximately the same as the oxygen saturation of the left atrium and the systemic circulation. These findings could be compatible with a total anomalous venous drainage. The pressures in the right and left atria were about normal. We were not able to measure the pulmonary wedge pressure at that time. It has been shown, however, in previously catheterized patients, that the arterial pulmonary wedge pressure when measured was
15
always found to be elevated.1,2~4~6-12 If we had been able to demonstrate a markedly increased wedge pressure, in contrast to the presence of normal atria1 pressures (Table I), it would have been almost diagnostic for the disease, because it is obvious that only an obstruction between the pulmonary arterioles and the “true” atrium could explain the difference in these pressures. In the supradiaphragmatic type of anomalous venous drainage the pulmonary wedge pressure is usually normal or slightly elevated. However, in the infradiaphragmatic type a high pulmonary wedge pressure is anticipated, but the prominent cyanosis, the more severe symptoms, and the conspicuously small size of the heart are points for differentiation of this lesion. In some rare, complicated cases with stenosis of the pulmonary veins, without an intracardiac anomalous septum, the final diagnosis can be made by catheterization. In such a case this diagnosis was made by passing the catheter into the left atrium, in which a normal pressure was found, and then into a pulmonary vein, in which a high pressure was recorded.17 In conclusion, car triatriatum can be greatly suspected during life if signs and symptoms of pulmonary venous and arterial hypertension exist and a high pulmonary wedge pressure is proved. Demonstration of a pressure difference between the left atrium and pulmonary arterioles (if these locations are accessible to the catheter) is the best evidence for this malformation. The surgical treatment consists of enlarging the opening in the abnormal septum. This can be accomplished easily, without much risk, and can be a lifesaving procedure.1-a Summary
We have reported the clinical and autopsy findings in 3 casesof car triatriatum. We discussed the possible variants of this anomaly and their clinical features. The most common manifestations include a nonspecific systolic murmur, accentuation of the second sound, right ventricular hypertrophy or failure, pulmonary congestion, and the demonstration of an
16
Papaioannou,
Nihoyanopoulos,
and Vlachos
elevated pulmonary wedge pressure by cardiac catheterization. If at the same time a normal left atria1 pressure can be obtained, this is diagnostic for the disease. In any case in which car triatriatum is suspected, a cardiotomy should be attempted, with the purpose of correcting it surgically. This may be a lifesaving procedure. The senior author is indebted to Dr. B. M. Gasul for his permission to include cases seen in his department. REFERENCES 1. Lewis, F. J., Varco, R. L., Raufic, M., and Niazi, A.: Direct vision repair of triatrial heart and total anomalous pulmonary venous drainage, Surg., Gynec. & Obst. 102:713, 1956. 2. Vineberg, A., and Gialloneto, 0.: Report of a successful operation for stenosis of common pulmonary vein (car triatriatum), Canad. M.A.J. 74:719, 1956. 3. Barrett. N. R.. and Hickie. T. B.: Cor triatriatum, Thorax 12:24, 1957: ” 4. Belcher, J. R., and Saumerville, W.: Cor triatriatum. Successful treatment of a case, Brit. M.J. 1:1280, 1959. 5. Lam. C. R.: Ouoted bv Niwavama.Q 6. Anderson, R:C., and Varcd, R. L.: Cor triatriatum, successful diagnosis and surgical correction in a three-year-old girl, Am. J. Cardiol. 7:436, 1961.
7. Gibbons, J. E., Jegier, W., and Murphy, D. R.: Cor triatriatum. Clinical, hemodynamic and autopsy studies and long-term observation after open surgical correction, Circulation 22:755, 1960. 8. Redo, F. S., and Goldberg, H. P.: Surgical management of unusual cardiovascular anomalies, Surg. Clin. North America 41:424, 1961. 9. Niwayama, G.: Cor triatriatum, AM. HEART J. 59:291, 1960. 10. Keith, J. D., Rowe, R. D., and Vlad, P.: Heart disease in infancy and childhood, New York, 1958, The Macmillan Company, pp. 352-360. 11. Gouzios, A., and Cotton, E. C.: Cor triatriatum associated with coarctation of the aorta, A.M.A. J. Dis. Child. 99:451, 1960. 12. Darke, C. S., Emery, J. L., and Lorber, J.: Triatria1 heart. Brit. Heart 1. 23:329. 1961. 13. Grifflth, T.‘W.: Note on a second example of division of the cavity of the left auricle into two compartments by a fibrous band, J. Anat. Physiol. $7:255, 1903. 14. Loeffler. E.: Unusual malformation of the left atrium:’ pulmonary sinus, Arch. Path. 48:371, 1949. I. N., Hollinshead, W. H., and Durham, 1.5. Dubin, N. C.: Congenitally insufficient tricuspid valve accompanied by an anomalous septum in the right atrium, Arch. Path. 38:225, 1944. 16. Gould, S. E., editor: Pathology of the heart, Springfield, Ill., 1960, Charles C Thomas, Publisher, pp. 484-488. 17. Burch, G. E.: Personal communication.
of clinical
and autopsy
findings
in 3 cases
A. C. Papaioannou, M.D. J. Nihoyanopoulos, M.D. J. Vlachos, M.D. Athens, Greece
C
or triatriatum is a rare congenital heart malformation, amenable to simple corrective surgery. A preoperative diagnosis is, therefore, of paramount importance. Very few cases so far have been diagnosed during life and, to our knowledge, in no more than 9 caseshas operation been successful.1-8 Because of the rarity of this anomaly and the diversity of the symptoms with which it may be manifested, we wish to report 3 additional cases. In our discussion we have made an attempt to correlate the clinical and autopsy findings in these cases. It is noteworthy that in our third case the abnormal septum was found in the right atrium. Only 4 such cases have been reported previously. g Case reports Case 1. A.X., a 19-month-old
male infant, was admitted to the Pediatric Clinic of the University of Athens, with a history of fever, anorexia, and failure to thrive. The infant weighed 3,800 grams at birth, and his development during the first year of life was said to be normal. The patient thereafter developed “attacks” of dyspnea, which were aggravated in the last 2 months prior to his admission. Physical examination revealed a slightly cyanotic child with marked respiratory distress. The heart was enlarged to percussion and palpation. The pulmonic component of the second sound was accentuated. A Grade 2-3, short systolic murmur was best heard over the left upper sternal From the Department of Pediatrics, University Received for publication Feb. 5, 1962.
12
border. The lungs had moist rPles bilaterally. The liver was enlarged 3 fingerbreadths below the right costal margin. The peripheral pulses were well palpable. The rest of the examination was not contributory. A chest roentgenogram showed an enlarged heart and a prominent pulmonary conus (Fig. 1). The electrocardiogram indicated right atria1 enlargement and right ventricular hypertrophy of marked degree. The patient was digitalized, and oxygen, diuretics, and antibiotics were administered. However, his condition deteriorated rapidly, and he died on the second hospital day. At autopsy, the left atrium was found to be divided by an anomalous diaphragm into two chambers. The mediosuperior chamber received all the pulmonary veins and had the shape of an inverted truncated cone. Its superior surface measured 2.8 by 3.0 cm., and its inferior surface, 1.4 by 1.2 cm. In the diaphragm were two openings, about 0.7 and 0.2 cm. in diameter, which communicated with the inferior true left atrium. Another small opening, about 0.3 cm. in diameter, led the probe into the right atrium (Fig. 2), which was dilated. The left atrium was of normal size. The right ventricle was dilated and hypertrophied; the anterior wall was about 7 mm. in thickness. The wall of the left ventricle also measured 7 mm. The valves were regular. Case 2. P.S., an d-month-old boy, was first seen in Cook County Children’s Hospital, Chicago, at the age of 2 months because of “pneumonia.” Since then the child was said to have been slightly blue and to have had repeated respiratory infections. On examination, his skin had a dusky color, but no clubbing or definite cyanosis were present. The heart was enlarged to palpation. The pulmonary closure of the second sound was accentuated. A Grade 2-3, short systolic murmur was best heard over the third to fourth left intercostal spaces. No of Athens (St. Sophia’s Children’s
Hospital,
Goudi, Athens, Greece).
Cor triutriatum
13
was attached to the endocardial surface of the posterior lateral portion of the right atrium. The inferior compartment received both venae cavae in a normal manner and contained the auricular appendage, which was dilated. There were two defects, one above and the other below the diaphragm, in the septum primum, measuring 0.8 by 0.5 cm. and 1.0 by 0.8 cm., respectively. These communicated with the left atrium. The foramen ovale was closed. All the pulmonary veins entered a sac-like venous sinus which communicated with each atrium through a small ostium. The tricuspid orifice had a circumference of 5.7 cm., and the right ventricle was hypertrophied. The chambers of the left side of the heart were otherwise normally formed. The ductus was closed. Fig. 1. Case anterior view.
1.
Chest
roentgenogram,
postero-
thrill was palpable and the femoral pulses were normal. Chest x-ray examination showed an increased cardiac shadow with a prominent pulmonary artery (Fig. 3). The lung fields appeared to be somewhat congested. The electrocardiogram indicated marked right atria1 enlargement and right ventricular hypertrophy. A venous angiocardiogram was made. The right atrium was dilated and remained opacified during levoangiocardiography. No other anomalies were detected. An atria1 septal defect or anomalous venous drainage was suspected clinically. A surgical exploration was attempted; only a large septal defect was found and this was closed. Two days later the patient died. At autopsy, the left atrium was found to be separated into two chambers by an abnormal diaphragm. The upper accessory chamber communicated with the right atrium through a large septal defect, and with the left atrium through an opening in the diaphragm. As seen in retrospect, this opening was mistaken for the mitral valve by the exploring finger of the surgeon. Case 3. J.F., a f-month-old female infant, was admitted to the Pediatric Cardiology Department of Cook County Hospital, Chicago, because she had had dyspnea and cyanosis since the age of 6 weeks. On physical examination the infant appeared to be dyspneic, and mild cyanosis was present in both the upper and the lower extremities. The cardiac apex was felt over the fifth left intercostal space and at the mid-clavicular line. There was a loud pulmonary closure sound over the second and third left intercostal spaces. A Grade 3 systolic murmur accompanied by a thrill was best heard in the same area. Chest x-ray films revealed an increased diameter of the heart and a convexity of the heart shadow in the pulmonary area. The electrocardiogram was suggestive of right atria1 enlargement and biventricular hypertrophy. The patient was catheterized, and the data are shown in Table I. The patient subsequently died of pulmonary infection. At autopsy, the heart was found to be enlarged, particularly the chambers on the right side. The right atrium was divided into two compartments by a part ially fenestrated fibrous diaphragm, which
Discussion
Niwayama,g in a critical review of the literature prior to 1960, found only 38 cases reported, to which he added 5 of his own. Since then, about 11 cases have been recorded,4~6-8,10-12 which brings the total number to 54. The majority of recent workers support the explanation suggested by Griffith,I$ namely, that early embryology is responsi-
Fig. 2. Case 1. Posterior view of the heart. The pulmonary veins (P V) enter the “accessory” atrium (CP V). The vertical probe (a) is inserted through an opening from the “accessory” atrium into the left atrium. The probe (b) leads into the right atrium. The number 2 is at the orifice of the removed inferior vena cava.
14
Papaioannou,
Nihoyanopoulos,
and Vlachos
venous return. The smaller the size of the communication, the earlier the symptoms will appear. In about two thirds of the reported cases, death occurred during the first 2 years of life.gJO However, if the fenestration is very large, no obstruction at all will occur and these patients live to adult life.13j14 In some patients there were multiple fenestrations in the septum, and in 3 cases, none.g In such instances an anomalous venous drainage or defects above and below the septum are essential for survival. In 4 patients the abnormal diaphragm was found in the right atrium,Y and here is supposed to represent a persistent right valve of the sinus venosus.IL There appears to be a goodly number of anatomic variants of this anomaly, depending on the presence or absence of interatrial communications which may further complicate the clinical features. If, for instance, no interatrial communication exists and the exit from the “accessory” chamber to the left atrium is stenotic, the hemodynamic alterations and clinical manifestations may be similar to those of congenital mitral stenosis, stenosis or atresia of a common pulmonary vein, or endocardial fibroelastosis of the constrictive type.3J0,11s16 If an interatrial communication exists (open foramen ovale or atria1 defect) between the “accessory” chamber and the right atrium only, a left-to-right shunt will occur, imitating an atria1 septal defect and, in more severe cases, a Lutembacher complex.gJ6 In case of a large communication between the lower “true” atrium and the right atrium, a right-to-left shunt may occur, and, if both upper and lower chambers communicate with the right atrium, the flow may
Fig. 3. Case 2. Chest roentgenogram, posteroanterior view.
ble for this anomaly. According to this which view, the third atria1 chamber exists in this malformation represents, in fact, a dilated common pulmonary vein which failed to be incorporated into the left atrium. In car triatriatum an abnormal septum (diaphragm) divides the left atrium, or rarely the right, into two separate chambers which usually communicate through a narrow opening. The upper (accessory) atrium receives all the pulmonary veins, and the lower (true) atrium contains the atria1 appendage and the mitral (or tricuspid) valve. In the most common cases the opening of the anomalous septum is small and produces considerable obstruction to the pulmonary Table I. Catheterization
data from right side of heart in Case 3 Location
SVC
Oxygen content (vol. %) Pressure (mm. Hg) Oxygen saturation (%) Oxygen capacity (vol. 75) SVC:
Superior
vena
cava.
WC:
/
9.1
IVC
1
11.8
RA
)
RV
14.8 (4)
--
1
15.5 66/l
LA
1
FA
15.4 (7) 88.6
15.1 105/60 86.5
FA: Femoral
artery.
17.4
Inferior
vena
can.
RA:
Rirtht
atrium.
RV:
Right
ventricle.
LA:
Left
atrium.
Cor triatriatum
be bidirectional. Then a clinical cyanosis may appear, in proportion to the degree of unsaturated blood passing into the systemic circulation. Furthermore, if the abnormal septum is located in the right atrium, as in our third case, the condition may imitate an atria1 septal defect with a right-to-left shunt or an anomalous venous drainage, depending on the interatria1 communications. The clinical picture can be still more complicated if additional heart anomalies are associated.g-ll The most commonly reported manifestations in cases of car triatriatum were attacks of dyspnea and pulmonary edema, enlargement of the right side of the heart, and congestive heart failure. Analysis of the auscultatory findings in our 3 cases revealed a short systolic murmur over the upper left parasternal area and an accentuated pulmonary closure in all. Nonspecific murmurs and accentuation of the second sound have been noted in previous reports.6~R~gJ1J2 The roentgenographic and electrocardiographic findings in all our cases were suggestive of marked right ventricular (biventricular, in the third patient) hypertrophy and right atria1 dilatation. The pulmonary vascular markings and pulmonary conus were prominent; this is also commonly described in the literature. Venous angiocardiography which was performed in our second patient was not of definite help. There are not many angiocardiographic reports, but we believe that the techniques of cineangiocardiography from the pulmonary artery can be of some value in the diagnosis of car triatriatum if this condition is suspected. Catheterization of the right side of the heart of our third patient (Table I) showed an increased oxygen saturation in the chambers of the right side of the heart which was approximately the same as the oxygen saturation of the left atrium and the systemic circulation. These findings could be compatible with a total anomalous venous drainage. The pressures in the right and left atria were about normal. We were not able to measure the pulmonary wedge pressure at that time. It has been shown, however, in previously catheterized patients, that the arterial pulmonary wedge pressure when measured was
15
always found to be elevated.1,2~4~6-12 If we had been able to demonstrate a markedly increased wedge pressure, in contrast to the presence of normal atria1 pressures (Table I), it would have been almost diagnostic for the disease, because it is obvious that only an obstruction between the pulmonary arterioles and the “true” atrium could explain the difference in these pressures. In the supradiaphragmatic type of anomalous venous drainage the pulmonary wedge pressure is usually normal or slightly elevated. However, in the infradiaphragmatic type a high pulmonary wedge pressure is anticipated, but the prominent cyanosis, the more severe symptoms, and the conspicuously small size of the heart are points for differentiation of this lesion. In some rare, complicated cases with stenosis of the pulmonary veins, without an intracardiac anomalous septum, the final diagnosis can be made by catheterization. In such a case this diagnosis was made by passing the catheter into the left atrium, in which a normal pressure was found, and then into a pulmonary vein, in which a high pressure was recorded.17 In conclusion, car triatriatum can be greatly suspected during life if signs and symptoms of pulmonary venous and arterial hypertension exist and a high pulmonary wedge pressure is proved. Demonstration of a pressure difference between the left atrium and pulmonary arterioles (if these locations are accessible to the catheter) is the best evidence for this malformation. The surgical treatment consists of enlarging the opening in the abnormal septum. This can be accomplished easily, without much risk, and can be a lifesaving procedure.1-a Summary
We have reported the clinical and autopsy findings in 3 casesof car triatriatum. We discussed the possible variants of this anomaly and their clinical features. The most common manifestations include a nonspecific systolic murmur, accentuation of the second sound, right ventricular hypertrophy or failure, pulmonary congestion, and the demonstration of an
16
Papaioannou,
Nihoyanopoulos,
and Vlachos
elevated pulmonary wedge pressure by cardiac catheterization. If at the same time a normal left atria1 pressure can be obtained, this is diagnostic for the disease. In any case in which car triatriatum is suspected, a cardiotomy should be attempted, with the purpose of correcting it surgically. This may be a lifesaving procedure. The senior author is indebted to Dr. B. M. Gasul for his permission to include cases seen in his department. REFERENCES 1. Lewis, F. J., Varco, R. L., Raufic, M., and Niazi, A.: Direct vision repair of triatrial heart and total anomalous pulmonary venous drainage, Surg., Gynec. & Obst. 102:713, 1956. 2. Vineberg, A., and Gialloneto, 0.: Report of a successful operation for stenosis of common pulmonary vein (car triatriatum), Canad. M.A.J. 74:719, 1956. 3. Barrett. N. R.. and Hickie. T. B.: Cor triatriatum, Thorax 12:24, 1957: ” 4. Belcher, J. R., and Saumerville, W.: Cor triatriatum. Successful treatment of a case, Brit. M.J. 1:1280, 1959. 5. Lam. C. R.: Ouoted bv Niwavama.Q 6. Anderson, R:C., and Varcd, R. L.: Cor triatriatum, successful diagnosis and surgical correction in a three-year-old girl, Am. J. Cardiol. 7:436, 1961.
7. Gibbons, J. E., Jegier, W., and Murphy, D. R.: Cor triatriatum. Clinical, hemodynamic and autopsy studies and long-term observation after open surgical correction, Circulation 22:755, 1960. 8. Redo, F. S., and Goldberg, H. P.: Surgical management of unusual cardiovascular anomalies, Surg. Clin. North America 41:424, 1961. 9. Niwayama, G.: Cor triatriatum, AM. HEART J. 59:291, 1960. 10. Keith, J. D., Rowe, R. D., and Vlad, P.: Heart disease in infancy and childhood, New York, 1958, The Macmillan Company, pp. 352-360. 11. Gouzios, A., and Cotton, E. C.: Cor triatriatum associated with coarctation of the aorta, A.M.A. J. Dis. Child. 99:451, 1960. 12. Darke, C. S., Emery, J. L., and Lorber, J.: Triatria1 heart. Brit. Heart 1. 23:329. 1961. 13. Grifflth, T.‘W.: Note on a second example of division of the cavity of the left auricle into two compartments by a fibrous band, J. Anat. Physiol. $7:255, 1903. 14. Loeffler. E.: Unusual malformation of the left atrium:’ pulmonary sinus, Arch. Path. 48:371, 1949. I. N., Hollinshead, W. H., and Durham, 1.5. Dubin, N. C.: Congenitally insufficient tricuspid valve accompanied by an anomalous septum in the right atrium, Arch. Path. 38:225, 1944. 16. Gould, S. E., editor: Pathology of the heart, Springfield, Ill., 1960, Charles C Thomas, Publisher, pp. 484-488. 17. Burch, G. E.: Personal communication.