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Tetralogy of Fallot: Correlation of clinical, roentgenologic, and post-mortem findings
Department TETRALOGY
OF
of Clinical
FALLOT
ROENTGENOLOGIC, JOSEPH
A.
: CORRELATION AND
PESCATORE, AND
Reports
VICTOR.
OF CLINICAL,
POST-MORTEM
M.D.,
A.
PHILADELPE-11-4,
JOSEPH DIGILIO,
B. WOLFFE, M.D.
FINDINGS M.D.,
PA.
TRUE case of tetralogy, as described by Fallot,l includes the following : (1) stenosis of the pulmonary artery, (2) enlarged and hypertrophied right ventricle, (3) displaced aorta, and (4) interventricular septal defect. The following case is a typical illustration of the tetralogy of Fallot and offered an opportunity to study the relationship of the chambers and great vessels to one another and to the chest wall.
A
J. B., a man 20 years old, died of septic endocarditis complicating the tetralogy of Fallot. This patient had been under the care of one of us (J. B. TV.) for many years. In the summer of 1933 he developed a septic endocarditix, which was the cause of his death in September of the same year, A roentgenogram taken three years before death (Fig. 1) reveals the typical cardiac silhouette usually seen in these cases, with blunting of the apex and a roughly rectangular heart. We were interested in ascertaining what anatomic structures were responsible in our ease for the typical cardiac silhouette, and therefore an autopsy was performed in the following manner: On opening the chest by removing the sternum and ribs, the size, shape, and position of the heart and its anatomic relation to the lungs were carefully studied. These organs, together with the liver, were removed in tolo, and were arranged on the x-ray table in exactly the same anatomic relationship which they had occupied in the body. h cannula was inserted into the superior vena cava and tied to prevent leakage. A large metal syringe filled with barium emulsion was then connected to this cannula and the barium slowly injected until the right auricle was completely filled (seen tluoroscopically). A roentgenogram (Fig. 2) showed complete filling of the right auricle, with some of the barium going through the tricuspid opening. The valve and trabeculae carneae are also visualized. This roentgenogram definitely shows that the right auricle forms the right border of the heart and a goodly portion of its anterior surface. In addition, the inferior vena cava, with its contributing branches, is seen coming from the liver and emptying into the right auricle. The injection of barium was continued and another roentgenogram taken (Fig. 3). It reveals partial filling of the right ventricle, with some of the barium going into the aorta; as expected, marked narrowing of the pulmonary artery is very evident. Again we continued the injection of barium until we completely filled the right ventricle (Fig. 4). Here we can see that the anterior surface of the heart is formed almost completely by the right auricle and right ventricle, while only a very small portion of the left ventricle can be seen. The apex is blunted and is formed eomFrom the Department Received for publication
of Medicine, Temple Sept. 3, 1938.
University
Hospital.
490
‘THE
AYIERI(‘AN
HEART
JOCRSAL
pletely by the right ventricle, instead of the left, as in normal hearts. Again the marked stenosis of the pulmonary artery is very evident and the aorta contains more barium. dfter roentgenograms of the right heart were taken, the organ was washed out as well as possible and a hypodermic needle substituted for the cannula (superior vena cava) as a landmark. The cannula was then inserted into one of the pulmonary The barium-filled syringe was conveins and another roentgenogram taken (Fig. 5). nected to the cannula and the material slowly injected until the left auricle was Fig.
1.
Fig.
3.
Fig.
Fig.
2.
4.
filled (Fig. G). This roentgenogram shows the filled-out left auricle with some of the material going through the mitral opening into the left ventricle, and some going The pulmonary and hepatic arterial trees are also shown. through to the aorta. If Fig. 2 be superimposed upon Fig. 6, it will be seen that the left auricle occupies a position posterior and somewhat medial to the right auricle. The injection of barium was then cont.inued until the left ventricle was completely filled (Fig. 7). This roentgenogram shows that the left ventricle lies almost entirely behind and is
PESCATORE
ET
AL.
:
TETRAL’OGY
OF
491
FALLOT
small as compared to the right ventricle. It is also very interesting to note that, although there exists an opening in the membranous portion of the interventricular septum, the barium fails to go into the right ventricle. It may be possible that nature prevents the blood from going from the left to the right ventricle by developing the moderator band, which may act as a valve in closing off the septal opening
during cardiac Fig. 8 taken. interventricular artery. Next, ventricle (Fig.
Fig.
5.
Fig.
6.
Fig.
7.
Fig.
8.
‘?
systole. The barium was then forced in under great pressure and This shows that under pressure the barium finally went through the opening into the right ventricle, and then into the pulmonary the heart was dissected and washed and a window cut into the right 9). This shows the enlarged right ventricle with its hypertrophied
Fig.
10.
walls and, in addition, the interventricular opening with the displaced aorta and stenosed pulmonary artery. A window was then cut into the left vent,ricle (Fig. lo), showing the small size of this chamber as compared to a normal one; a probe \ which is running through the interventricular opening is also seen. Finally, a dra .wing of the anterior aspect of the heart was made in such a way that th6 picture Bias a trifle smaller t,han the original. This was then superimposed on a roentgeno gram taken ante mortem (Fig. ll), in order to show the reason for the typical silho uette and the position of the various chambers. COMMENT
From a diagnostic point of view, Blackford stresses the rectang ‘ular appearance of the heart and its increased transverse diameter. He also
PESCATORE
ET
AL.:
TEsTRALOGY
Fig.
OF
FALLOT
493
11.
points out that the apex of the heart is formed entirely by the right ventricle, instead of by the left, as is the case in the normal heart. These observations deserve emphasis as an aid in the diagnosis of this condition. We attach significance to the blunted appearance of the apex roentgenographically. Popp3 establishes the following criteria for the roentgenologic diagnosis of tetralogy of Fallot : (1) Absence of the pulmonic conus as a sign of hypoplasia of the pulmonary artery ; (2) deviation of the vascular pedicle to the right-aorta &L cheuat; (3) masking of the aorta by the trachea in the right anterior oblique position, and (4) crossing of the aorta by the right bronchus. Abbott,4 largely on the basis of the studies by Sir Arthur Keith, ascribes the lesions of the tetralogy of Fallot to an arrest of development of the cardiac apparatus before the eighth week of embryonic life.
We have presented a case of tetralogy of Fallot, and have endeavored to show which anatomic structures are responsible for the typical cardiac silhouette. REFERENCES 1. Fallot: Original monograph (1888). 2. Blackford, L. M.: Tetralogy of Fallot: Report of a Case, Arch. Int. Med. 45: 631, 1930. 3. Popp, c.: Radiologic Diagnosis of Tetralogy of Fallot, Arch. d. mal. du coeur 24: 249, 1931. 4. Abbott, M. E.: Congenital Cardiac Disease (in Osler and McCrae): Modern Medicine, Ed. 3, Val. 4, p. 613, Philadelphia, 1927, Lea & Febiger.
OF
of Clinical
FALLOT
ROENTGENOLOGIC, JOSEPH
A.
: CORRELATION AND
PESCATORE, AND
Reports
VICTOR.
OF CLINICAL,
POST-MORTEM
M.D.,
A.
PHILADELPE-11-4,
JOSEPH DIGILIO,
B. WOLFFE, M.D.
FINDINGS M.D.,
PA.
TRUE case of tetralogy, as described by Fallot,l includes the following : (1) stenosis of the pulmonary artery, (2) enlarged and hypertrophied right ventricle, (3) displaced aorta, and (4) interventricular septal defect. The following case is a typical illustration of the tetralogy of Fallot and offered an opportunity to study the relationship of the chambers and great vessels to one another and to the chest wall.
A
J. B., a man 20 years old, died of septic endocarditis complicating the tetralogy of Fallot. This patient had been under the care of one of us (J. B. TV.) for many years. In the summer of 1933 he developed a septic endocarditix, which was the cause of his death in September of the same year, A roentgenogram taken three years before death (Fig. 1) reveals the typical cardiac silhouette usually seen in these cases, with blunting of the apex and a roughly rectangular heart. We were interested in ascertaining what anatomic structures were responsible in our ease for the typical cardiac silhouette, and therefore an autopsy was performed in the following manner: On opening the chest by removing the sternum and ribs, the size, shape, and position of the heart and its anatomic relation to the lungs were carefully studied. These organs, together with the liver, were removed in tolo, and were arranged on the x-ray table in exactly the same anatomic relationship which they had occupied in the body. h cannula was inserted into the superior vena cava and tied to prevent leakage. A large metal syringe filled with barium emulsion was then connected to this cannula and the barium slowly injected until the right auricle was completely filled (seen tluoroscopically). A roentgenogram (Fig. 2) showed complete filling of the right auricle, with some of the barium going through the tricuspid opening. The valve and trabeculae carneae are also visualized. This roentgenogram definitely shows that the right auricle forms the right border of the heart and a goodly portion of its anterior surface. In addition, the inferior vena cava, with its contributing branches, is seen coming from the liver and emptying into the right auricle. The injection of barium was continued and another roentgenogram taken (Fig. 3). It reveals partial filling of the right ventricle, with some of the barium going into the aorta; as expected, marked narrowing of the pulmonary artery is very evident. Again we continued the injection of barium until we completely filled the right ventricle (Fig. 4). Here we can see that the anterior surface of the heart is formed almost completely by the right auricle and right ventricle, while only a very small portion of the left ventricle can be seen. The apex is blunted and is formed eomFrom the Department Received for publication
of Medicine, Temple Sept. 3, 1938.
University
Hospital.
490
‘THE
AYIERI(‘AN
HEART
JOCRSAL
pletely by the right ventricle, instead of the left, as in normal hearts. Again the marked stenosis of the pulmonary artery is very evident and the aorta contains more barium. dfter roentgenograms of the right heart were taken, the organ was washed out as well as possible and a hypodermic needle substituted for the cannula (superior vena cava) as a landmark. The cannula was then inserted into one of the pulmonary The barium-filled syringe was conveins and another roentgenogram taken (Fig. 5). nected to the cannula and the material slowly injected until the left auricle was Fig.
1.
Fig.
3.
Fig.
Fig.
2.
4.
filled (Fig. G). This roentgenogram shows the filled-out left auricle with some of the material going through the mitral opening into the left ventricle, and some going The pulmonary and hepatic arterial trees are also shown. through to the aorta. If Fig. 2 be superimposed upon Fig. 6, it will be seen that the left auricle occupies a position posterior and somewhat medial to the right auricle. The injection of barium was then cont.inued until the left ventricle was completely filled (Fig. 7). This roentgenogram shows that the left ventricle lies almost entirely behind and is
PESCATORE
ET
AL.
:
TETRAL’OGY
OF
491
FALLOT
small as compared to the right ventricle. It is also very interesting to note that, although there exists an opening in the membranous portion of the interventricular septum, the barium fails to go into the right ventricle. It may be possible that nature prevents the blood from going from the left to the right ventricle by developing the moderator band, which may act as a valve in closing off the septal opening
during cardiac Fig. 8 taken. interventricular artery. Next, ventricle (Fig.
Fig.
5.
Fig.
6.
Fig.
7.
Fig.
8.
‘?
systole. The barium was then forced in under great pressure and This shows that under pressure the barium finally went through the opening into the right ventricle, and then into the pulmonary the heart was dissected and washed and a window cut into the right 9). This shows the enlarged right ventricle with its hypertrophied
Fig.
10.
walls and, in addition, the interventricular opening with the displaced aorta and stenosed pulmonary artery. A window was then cut into the left vent,ricle (Fig. lo), showing the small size of this chamber as compared to a normal one; a probe \ which is running through the interventricular opening is also seen. Finally, a dra .wing of the anterior aspect of the heart was made in such a way that th6 picture Bias a trifle smaller t,han the original. This was then superimposed on a roentgeno gram taken ante mortem (Fig. ll), in order to show the reason for the typical silho uette and the position of the various chambers. COMMENT
From a diagnostic point of view, Blackford stresses the rectang ‘ular appearance of the heart and its increased transverse diameter. He also
PESCATORE
ET
AL.:
TEsTRALOGY
Fig.
OF
FALLOT
493
11.
points out that the apex of the heart is formed entirely by the right ventricle, instead of by the left, as is the case in the normal heart. These observations deserve emphasis as an aid in the diagnosis of this condition. We attach significance to the blunted appearance of the apex roentgenographically. Popp3 establishes the following criteria for the roentgenologic diagnosis of tetralogy of Fallot : (1) Absence of the pulmonic conus as a sign of hypoplasia of the pulmonary artery ; (2) deviation of the vascular pedicle to the right-aorta &L cheuat; (3) masking of the aorta by the trachea in the right anterior oblique position, and (4) crossing of the aorta by the right bronchus. Abbott,4 largely on the basis of the studies by Sir Arthur Keith, ascribes the lesions of the tetralogy of Fallot to an arrest of development of the cardiac apparatus before the eighth week of embryonic life.
We have presented a case of tetralogy of Fallot, and have endeavored to show which anatomic structures are responsible for the typical cardiac silhouette. REFERENCES 1. Fallot: Original monograph (1888). 2. Blackford, L. M.: Tetralogy of Fallot: Report of a Case, Arch. Int. Med. 45: 631, 1930. 3. Popp, c.: Radiologic Diagnosis of Tetralogy of Fallot, Arch. d. mal. du coeur 24: 249, 1931. 4. Abbott, M. E.: Congenital Cardiac Disease (in Osler and McCrae): Modern Medicine, Ed. 3, Val. 4, p. 613, Philadelphia, 1927, Lea & Febiger.