TITLE

THE DIFFERENTIATION OF THE TETRALOGY OF FALLOT FROM SEVERE PULMONARY STENOSIS WITH INTACT VENTRICULAR SEPTUM AND RIGHT-TO-LEFT INTERATRIAL SHUNT

AUTHOR(S)
Vogelpoel, Louis; Schrire, Velva; Nellen, Maurice; Goetz, Robert H.
PUB. DATE
June 1957
SOURCE
Angiology;Jun1957, Vol. 8 Issue 3, p215
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
1. Fourteen cases of severe pulmonary stenosis with intact ventricular septum and right-to-left interatrial shunt and 48 cases of the tetralogy of Fallot have been analyzed to assess the points of value in the differential diagnosis. 2. The methods used in establishing conclusive proof of the diagnosis in each ease are described. 3. The most important and constant clinical differentiating features are the findings on auscultation. Thus, in severe pulmonary stenosis with intact ventricular septum the systolic murmur at the site of maximal intensity is so prolonged that it extends beyond and obscures the normal aortic component of the very widely split second sound. It stops, however, before the markedly delayed diminutive pulmonary component, which may or may not be audible. In the tetralogy of Fallot on the other hand, the systolic murmur starts soon after the first sound, reaches maximal intensity by midsystole and then diminishes markedly usually ending before the single loud, often palpable aortic component of the second sound. Other auscultatory findings of some importance are described. 4. Examination of the neck veins is second only to auscultation in importance in the clinical differentiation. A giant ‘a’ wave excludes uncomplicated tetralogy of Fallot where the ‘a’ wave is usually normal. A dominant ‘a’ wave strongly favors severe pulmonary stenosis with intact ventricular septum. 5. In severe pulmonary stenosis with intact ventricular septum the heart is usually normal in size but may be considerably enlarged; there is usually a marked sustained lift over the right ventricular outflow tract and the second heart sound is not palpable. In the tetralogy the heart is normal in size as a rule and the lift over the second right ventricle is insignificant. However, the second sound is often easily palpable and a systolic thrust in the pulmonary area, presumably due to the anteroposed aorta, is occasionally felt. 6. A delayed onset of central cyanosis favors severe pulmonary stenosis with intact ventricular septxim but may occasionally occur in the tetralogy. Cyanosis developing in the first year favors the tetralogy but may also occur in severe pulmonary stenosis with intact t ventricular septum. The intensity of cyanosis at rest is on the whole greater in the tetralogy than in severe pulmonary stenosis with intact ventricular septum. 7. A history of squatting occurs more commonly in the tetralogy than in severe pulmonary stenosis with intact ventricular septum, but is of no help in the diagnosis of the individual case. 8. Dyspnea, clubbing, pulycythemia, effort syncope, and stunting of growth are common to both conditions. 9. A history of angina pectoris favors severe pulmonary stenosis with intact ventricular septum. 10. Whereas right atrial and right ventricular hypertrophy are electrocardiographic features common to both conditions, the pattern of extreme right ventricular hypertrophy favors severe pulmonary stenosis with intact ventricular septum. 11. In a large proportion of cases the diagnosis can be suggested on radiologic grounds alone. In severe pulmonary stenosis with intact ventricular septum, the heart is usually larger and there is marked poststenotic dilatation of the pulmonary arteries. In the tetralogy the heart is often sabot shaped. The aorta is generally unusually wide and may be right-sided. However, many features overlap in both conditions. Moreover, in a number of cases the cardiac silhouette is within normal limits. Pulmonary oligemia and right ventricular hypertrophy are common to both conditions. 12. The diagnosis can usually be made by cardiac catheterization. The demonstration of an infundibular stenosis favors the tetralogy but valvular stenosis is common to both. Passage of the catheter from the right ventricle into the aorta excludes severe pulmonary stenosis with intact ventricular septum. The presence of an ‘a’ wave of 1) mm Hg or more excludes uncomplicated tetralogy of Fallot. Passage of the catheter from the right atrium to the left does not prove the diagnosis of severe pulmonary stenosis. A pressure gradient from right-to-left atrium and/or the demonstration of a right-to-left interatrial shunt favors the latter diagnosis. 13. Selective angiocardiography is a useful method for establishing the diagnosis of the tetralogy of Fallot. The aorta usually can be filled by injecting diodone into the right ventricle and the infundibular chamber can often be outlined. 14. Intravenous angiocardiography usually produces simultaneous opacification of the aorta and pulmonary arteries in both conditions. Differentiation of the two is best seen in the left oblique projection. In severe pulmonary stenosis with intact ventricular septum, marked early opacification of the left atrium and left ventricle accounts for the premature opacification of the aorta. In the tetralogy the aorta is filled from the right ventricle. In the anteroposterior projection the infundibular chamber can sometimes be outlined favoring the tetralogy. An unusually large aorta and anomalies of the aortic arch favor the tetralogy. 15. An accurate diagnosis can usually be made at the bedside. Cardiac catheterization and angiocardiography provide special anatomic; information useful to the surgeon and may be essential for the diagnosis in the occasional case.
ACCESSION #
16351806

 

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