Abstract
Striking variability has been observed in the presenting features in infancy of patients subsequently shown to have tetralogy of Fallot. Some patients presented with severe cyanosis in the neonatal period while others had a systolic murmur and cyanosis only on crying. In these latter patients cyanosis became present at rest over the subsequent months. Yet others presented with episodic attacks of unconsciousness, and a final group presented with dyspnoea and heart failure, accompanying a left-to-right shunt. Angiography demonstrates corresponding variations in the anatomy of the ventricular outflow tracts. In the severely cyanosed patients, the conus septum was deviated so as to obstruct the pulmonary outflow tract, and was best visualized in the lateral projection. In the patients with increasing cyanosis or episodic attacks of unconsciousness, the conus septum again obstructed the pulmonary infundibulum, but was obliquely orientated, not being seen clearly on either lateral or frontal projections. The episodic attacks were considered to be related to infundibular spasm, as previously shown to occur in Fallot's tetralogy. In contrast, increasing cyanosis was believed to be related to hypertrophy of infundibular musculature. In the patients with an initial left-to-right shunt, the arteries were orientated side-by-side so that the conus septum was observed in the frontal projection. Again, subsequent cyanosis was related to ventricular hypertrophy producing outflow tract obstruction. These findings are interpreted in the setting of a recent study, indicating that Fallot's tetralogy is produced by rotation of the embryonic conus together with sinistro-anterior deviation of the conus septum.
Full text
PDF
Images in this article
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Anderson R. H., Wilkinson J. L., Arnold R., Becker A. E., Lubkiewicz K. Morphogenesis of bulboventricular malformations. II. Observations on malformed hearts. Br Heart J. 1974 Oct;36(10):948–970. doi: 10.1136/hrt.36.10.948. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Becker A. E., Connor M., Anderson R. H. Tetralogy of Fallot: a morphometric and geometric study. Am J Cardiol. 1975 Mar;35(3):402–412. doi: 10.1016/0002-9149(75)90034-x. [DOI] [PubMed] [Google Scholar]
- Goor D. A., Edwards J. E. The spectrum of transposition of the great arteries: with specific reference to developmental anatomy of the conus. Circulation. 1973 Aug;48(2):406–415. doi: 10.1161/01.cir.48.2.406. [DOI] [PubMed] [Google Scholar]
- Gotsman M. S. Increasing obstruction to the outflow tract in Fallot's tetralogy. Br Heart J. 1966 Sep;28(5):615–621. doi: 10.1136/hrt.28.5.615. [DOI] [PMC free article] [PubMed] [Google Scholar]
- HONEY M., CHAMBERLAIN D. A., HOWARD J. THE EFFECT OF BETA-SYMPATHETIC BLOCKADE ON ARTERIAL OXYGEN SATURATION IN FALLOT'S TETRALOGY. Circulation. 1964 Oct;30:501–510. doi: 10.1161/01.cir.30.4.501. [DOI] [PubMed] [Google Scholar]
- Rudolph A. M., Heymann M. A., Spitznas U. Hemodynamic considerations in the development of narrowing of the aorta. Am J Cardiol. 1972 Oct;30(5):514–525. doi: 10.1016/0002-9149(72)90042-2. [DOI] [PubMed] [Google Scholar]
- Shinebourne E. A., Elseed A. M. Relation between fetal flow patterns, coarctation of the aorta, and pulmonary blood flow. Br Heart J. 1974 May;36(5):492–498. doi: 10.1136/hrt.36.5.492. [DOI] [PMC free article] [PubMed] [Google Scholar]
- VANMIEROP L. H., ALLEY R. D., KAUSEL H. W., STRANAHAN A. PATHOGENESIS OF TRANSPOSITION COMPLEXES. I. EMBRYOLOGY OF THE VENTRICLES AND GREAT ARTERIES. Am J Cardiol. 1963 Aug;12:216–225. doi: 10.1016/0002-9149(63)90311-4. [DOI] [PubMed] [Google Scholar]
- VANMIEROP L. H., WIGLESWORTH F. W. PATHOGENESIS OF TRANSPOSITION COMPLEXES. II. ANOMALIES DUE TO FAULTY TRANSFER OF THE POSTERIOR GREAT ARTERY. Am J Cardiol. 1963 Aug;12:226–232. doi: 10.1016/0002-9149(63)90312-6. [DOI] [PubMed] [Google Scholar]
- Van Praagh R. What is the Taussig-Bing malformation? Circulation. 1968 Sep;38(3):445–449. doi: 10.1161/01.cir.38.3.445. [DOI] [PubMed] [Google Scholar]
- WOOD P. Attacks of deeper cyanosis and loss of consciousness (syncope) in Fallot's tetralogy. Br Heart J. 1958 Apr;20(2):282–286. [PubMed] [Google Scholar]