Henry N. Neufeld

Congenital Communication of a Coronary Artery with a Cardiac Chamber or the Pulmonary Trunk ("Coronary Artery Fistula")

Anatomic, clinical, hemodynamic, and roentgenographic findings in six patients with congenital communication of a coronary artery with a cardiac chamber or the pulmonary trunk are presented. The literature is reviewed. A coronary artery may communicate anomalously, with any of the cardiac chambers, more commonly with those on the right side. In the six cases presented, the right coronary artery communicated with the right atrium in one case and with the right ventricle in another. The left coronary artery communicated with the right atrium and right ventricle in one case each, and with the pulmonary trunk in two cases. The most striking feature observed clinically was a continuous murmur. If a continous murmur is localized in an area atypical for patent ductus arteriosus, the diagnosis should be suspected. Conventional roentgenographic and electrocardiographic studies yielded no specific diagnostic features. The results of cardiac catheterization may reveal a left-to-right shunt, but they are diagnostically useful only when correlated with clinical findings. The only precise method of demonstrating the abnormality is by means of selective aortography performed by injecting medium into the very origin of the aorta. Cure is possible by surgical interruption of the fistulous tract.

SYNDROME OF CONGENITAL CARDIAC DISEASE WITH ASPLENIA. DISTINCTION FROM OTHER FORMS OF CONGENITAL CYANOTIC CARDIAC DISEASE.

Abstract The pathologic and clinical features in 17 patients with congenital cardiac disease in association with agenesis of the spleen have been presented. Pathologically, the cases fell into two groups as follows: those with two ventricles and a large ventricular septal defect (10 cases), and those with a common ventricle (7 cases). Conotruncal abnormalities were present in each case. These consisted of pulmonary stenosis or atresia (17 cases) and transposition of the great vessels (16 cases). Ventricular and infundibular inversion was common, occurring in 8 cases with two ventricles and 5 cases with common ventricle, respectively. “Endocardial cushion defects” were found in each of the 17 cases. Persistent common atrio-ventricular canal was present in 16, and an “ostium primum” atrial septal defect, in association with mitral atresia, was seen in the seventeenth case. Atrial septal defects were present in each case. In 13 instances, the only remnant of the atrial septum was a narrow strand of tissue between two large coexisting defects (the condition referred to herein as common atrium). The common occurrence of anomalies of the systemic and pulmonary veins was noted. Among the 17 cases, bilateral superior venae cavae with absent coronary sinus were found in 15 and anomalous pulmonary venous connections occurred in 12. Malposition of the viscera was represented by the finding of mirror-image dextrocardia in 1, dextroversion of the ventricles in 9 and abdominal heterotaxia in 13 cases. The finding of the cardiac apex and stomach on opposite sides was noted primarily in cases with common ventricle and may be helpful in the clinical diagnosis of this condition. There was a tendency toward bilateral symmetry of the organs characterized by the universal presence of bilateral three-lobed lungs, a large, transverse liver, absence of the spleen (the only unilateral organ in the body) and by the very frequent presence of common atrium, persistent common atrioventricular canal and bilateral superior venae cavae, and malrotation of the intestine. The patients presented, usually as young infants, with obvious cyanosis from birth. Electrocardiographically, the occurrence of left axis deviation and a counterclockwise QRS loop in the frontal plane suggesting the presence of an A-V commune defect, may help separate these patients with complicated cardiac malformations and asplenia from other patients with cyanotic congenital cardiac disease but with less complex malformations and without asplenia. In 5 cases of common ventricle and asplenia, the “A-V commune electrocardiogram” was absent, even though a complete form of persistent common atrioventricular canal was present in each. Routine thoracic and abdominal roentgenograms, as well as angiocardiographic studies, have proved to be the most effective means of establishing the clinical diagnosis of the asplenic syndrome. The angiocardiogram may identify many of the intracardiac and associated vascular anomalies found in this condition. The clinical features which are most useful in identifying cases of cyanotic congenital cardiac disease associated with asplenia include: (1) hematologic signs of asplenia; (2) electrocardiographic features of persistent common atrioventricular canal; and (3) radiologic evidence of hepatic symmetry, evidence of diminished pulmonary blood flow, and transposed great vessels.

Complete Transposition of the Great Vessels

A case of survival to the age of 11 years is reported in a patient with complete transposition of the great vessels. Additional malformations in the form of subpulmonary stenosis, ventricular septal defect, and patent ductcus arteriosus may have contributed to the unusually long period of survival. Surgical creation of an atrial septal defect and of a subclavian-pulmonary arterial anastomosis when the patient was 3 years old may have augmented the beneficial effects of the existing associated malformations.

Aorticopulmonary septal defect.

Abstract The anatomic, clinical, hemodynamic, roentgenographic and surgical problems in six cases studied and in sixty cases found in the literature of aorticopulmonary septal defect are analyzed. Anatomically, the lesion is characterized by an abnormal communication between the ascending aorta and pulmonary arterial trunk. In the aggregate of sixty-six cases, associated anomalies were as follows: (1) patent ductus arteriosus, eight cases; (2) coarctation of aorta, three cases; (3) ventricular septal defect, two cases; (4) right aortic arch, two cases; and (5) atrial septal defect, two cases. Clinically, the picture in each of the six patients studied resembled the findings of a large ventricular septal defect. The most striking clinical feature was the absence of a continuous murmur, and this finding was noted in 80 per cent of cases reviewed in the literature. The electrocardiogram is not specific and may show left, right or biventricular hypertrophy, depending on the degree of pulmonary vascular resistance. In general, hemodynamic findings are similar to those in cases of large patent ductus arteriosus. Pulmonary arterial pressure was equal or nearly equal to systemic arterial pressure in the four cases in which data for this comparison existed. A left to right shunt was demonstrated in each of the five cases subjected to cardiac catheterization. The diagnostic importance of the position of the catheter during catheterization is emphasized and illustrated. The radiologic features of the malformation are nonspecific but the diagnostic value of selective aortography is of high order. Five of the six patients underwent corrective surgical procedures, and three survived. Closure of the defect during conditions of cardio-pulmonary bypass is recommended.

Isolated Ventricular Septal Defect of the Persistent Common Atrioventricular Canal Type

Among 60 necropsy specimens of isolated ventricular septal defects and more than 300 cases of ventricular septal defect observed at operation, 15 cases demonstrating unusual anatomic positions of the defect were found. The ventricular defect differed from the ordinary ventricular septal defect in that it usually occupied the position of the ventricular component of the defect in persistent common atrioventricular canal. For this reason it was named ventricular septal defect of the persistent common atrioventricular canal type. Deformities of one or both atrioventricular valves were common (nine of 15 cases). No atrial septal defects of the ostium primum type were present. Anatomic studies of the conduction tissue revealed that this tissue skirted the posterior and inferior aspects of the ventricular septal defect and that, as in persistent common atrioventricular canal, the course taken by the conduction tissue was unusually long, as a result of the peculiar posteroinferior position of the lower edge of the defect. The electrocardiographic features were striking. In all cases the mean electrical axis of the QRS lay above the isoelectric point; the vector loop obtained in the frontal plane from the scalar electrocardiogram was directed counterclockwise, and its main mass was above the zero line. In addition, in all cases there were signs of right ventricular overload, and in some cases of left ventricular overload as well. Electrocardiographic findings of this pattern have been thought by some authors to be diagnostic of persistent common atrioventricular canal, but we observed that they also occurred in each of the cases of isolated ventricular septal defect of the variety described herein. We recognize that in the usual variety of ventricular septal defect this electrocardiographic pattern occurs, but it does so uncommonly. We have not studied its exact incidence. The anatomy, hemodynamics, and surgical considerations are different in cases with this defect from those with persistent common atrioventricular canal. The surgical risk in these cases has been higher than that in the usual type of ventricular septal defect. In the discussion of the electrophysiologic theories that seek to explain the unusual electrocardiographic patterns in this group of cases, a new theory is offered, based on studies of the conduction system. In our opinion, the different orientation of the advancing fronts of depolarization is the result of congenital displacement of the bundle of His in its relation to the ventricular septal defect.

COMBINED CONGENITAL SUBAORTIC STENOSIS AND INFUNDIBULAR PULMONARY STENOSIS

Coexistent congenital obstruction to both the right ventricular and left ventricular outflow tracts is rare. Only a few cases have been reported (Beard et al., 1957; Braunwald, 1959; Horlick and Merriman, 1957; Neufeld et al., 1960). Only two of about 800 specimens in the Mayo Clinic pathological collection of congenitally defective hearts represent this condition. It is the purpose of this communication to report these two cases.

Tetralogy of Fallot with Anomalous Tricuspid Valve Simulating Pulmonary Stenosis with Intact Septum

An anatomicopathologic entity of the tetralogy of Fallot with an additional finding of an accessory flap of tricuspid valvular tissue is described. In the 3 cases reported in this paper, the outstanding hemodynamic finding was a right ventricular systolic pressure that was significantly greater than the systemic systolic pressure. The hemodynamic findings were explained by the fact that the accessory tricuspid flap partially closed the large ventricular septal defect. The clinical picture was similar to that of either severe pulmonary stenosis with an intact ventricular septum or severe pulmonary stenosis with a small ventricular septal defect and a right-to-left shunt at the ventricular level.

Biventricular origin of the pulmonary trunk with subaortic stenosis above the ventricular septal defect

Abstract Three cases are reported that presented a complex of congenital cardiac anomalies which consisted of biventricular origin of the pulmonary trunk with subaortic stenosis above a ventricular septal defect. In Case 1 the hemodynamic findings obtained at cardiac catheterization showed consistently higher ventricular and pulmonary arterial pressures when these were compared to systemic arterial pressure. Two patients were siblings with identical anatomic findings (Cases 2 and 3). Both of these patients had associated interruption of the aortic arch. The descending aorta, which gave rise to the subclavian arteries, communicated with the right ventricle through a patent ductus arteriosus. In one of the patients (Case 3), differential cyanosis was noted: the upper and lower extremities were cyanotic but the head was not cyanotic. Mild coarctation of the aorta was present in the third patient (Case 1).

Pathology of Angina Pectoris

Among the wide variety of causes of angina pectoris, two major categories may be recognized: the anatomic, causing arterial obstruction, and the functional.The anatomic causes may be divided according to anatomic sites as follows: (1) the major coronary arterial trunks and their epicardial branches, (2) the coronary ostia at the aorta, and (3) the intramyocardial “small” arteries. The major coronary arteries are by far the most common sites for anatomic lesions. Atherosclerosis in its various anatomic manifestations is responsible for about 90% of the cases of angina. Commonly, hypertension and, less commonly, valvular disease are associated. The balance of cases of angina result from various states given below. Nonatheromatous diseases of the major coronary arteries include embolism, primary dissecting aneurysm of a coronary artery, arteritis, and anomalous communication of a coronary artery. Coronary ostial narrowing results from various diseases of the aorta including atherosclerosis, saccular and dissecting aneurysm, inflammation, and calcification of the aorta in relation to origin of a coronary artery. Lesions causing obstruction of the intramyocardial arteries are commonly part of systemic diseases such as hematologic, embolic, metabolic, and degenerative.Functional causes of angina pectoris include aortic valvular disease and functionally related conditions, thyroid disease, and pulmonary hypertension.

Complete Transposition of the Great Vessels II. An Electrocardiographic Analysis

This paper is based on a study of specimens of the heart that were obtained in 60 cases of complete transposition of the great vessels.The external relationship of the aorta and pulmonary trunk usually differed from the classic description of the aorta lying directly anterior to the pulmonary trunk (type III). This relationship was present in only two of the 60 cases. In 39 cases, the relationship of the great vessels was oblique (type I). In 19 of the 60 cases, the relationship of the great vessels was side-to-side (type II).There were two main patterns of origin of the coronary arteries. One had a tendency to be associated with the oblique (type I) relationship of the great vessels, which was most common; the other had a tendency to be associated with the side-to-side (type II) relationship of the great vessels, which was the second most common type.A ventricular septal defect was present in 22 (37 per cent) of the 60 cases. The anatomic characteristics of these defects were the same as occur in cases of an isolated defect in the ventricular septum. It was of particular interest that a left ventricular-right atrial communication was observed in three cases.Miscellaneous conditions observed include congenital obstruction of the aortic arch (eight cases), a right aortic arch in association with a ventricular septal defect and pulmonary stenosis (two cases), and juxtaposition of the atrial appendages (two cases).The atrioventricular valves resembled the homolateral valves of normal hearts. An Ebstein-like malformation of the mitral valve was present in one case.A correlative study of necropsy findings and electrocardiography in 54 cases of complete transposition of the great vessels was made to determine whether or not specific electrical patterns could predict the status of the ventricular septum. It became apparent that published criteria for distinguishing between patients with a ventricular septal defect and those with an intact ventricular septum were not uniformly reliable. Rather than the location of a communication, the important factor in differentiation depended upon the size of the opening, be it between the ventricles or through a patent ductus arteriosus. Cases were divided into two groups: In group I were those with a small communication, and in group II were those with a large communication between the greater and lesser circulations. The group with small communication showed a narrow or obliterated ductus arteriosus and an intact ventricular septum, or a small ventricular septal defect. In group II there was either a wide patent ductus arteriosus with an intact ventricular septum or a large ventricular septal defect. In group I the electrocardiogram predominantly showed isolated right ventricular hypertrophy, whereas, in group II, biventricular hypertrophy was the usual finding. Among the five patients in whom the mean manifest electrical axis was deviated to the left, two showed obstructive malformations of the aortic arch, and two left ventricular-right atrial communication. The fifth case was simply one of a large communication without complicating anomalies.