Ray C. Anderson

The developmental complex of "parachute mitral valve," supravalvular ring of left atrium, subaortic stenosis, and coarctation of aorta.

Abstract A developmental complex is described in which four obstructive anomalies of the left side of the heart and aorta coexist. These anomalies are (1) parachute mitral valve; (2) supravalvular ring of left atrium; (3) subaortic stenosis of either the muscular or membranous type; and (4) coarctation of the aorta. Eight cases form the basis of this report. In 2 cases, each of the four anomalies was present; all of the other 6 cases represent partial forms of the complex, or formes frustes, in that only two or three of the anomalies were present. The clinical picture is compounded of the effects of the several anomalies of the complex and of the frequent association with still other anomalies, including ventricular septal defect.

Congenital Cardiac Disease Associated with Polysplenia A Developmental Complex of Bilateral "Left-Sidedness"

The well-recognized association of congenital cardiac disease with asplenia has been termed “bilateral right-sidedness” or dextro-isomerism, since the spleen is absent, the liver is symmetrical, and each lung has three lobes and an epiarterial bronchus. In a study of pathological material from 12 patients with congenital cardiac disease associated with multiple spleens (as contrasted to accessory spleen), we found a definite tendency for the symmetrical development of organs but with a tendency for bilateral left-sidedness or levo-isomerism.The abnormalities assumed one of three forms as follows: (1) absence of a normal right-sided structure, (2) bilateral organs, each with the structure of a left-sided organ, or (3) excessive tissue of a left-sided organ. Thus, in polysplenia we observed a tendency for (1) absence of the hepatic segment of the inferior vena cava and absence of the gallbladder, (2) two lobes in each lung with hyparterial bronchi, and (3) multiple spleens. Other noncardiac abnormalities were partial or complete abdominal heterotaxia and partial malrotation of the bowel. The cardiac malformations included dextrocardia, bilateral superior venae cavae, anomalous pulmonary venous connection with malposition of the atrial septum, and defects in the atrial septum and in the ventricular septum.Our study suggests that the developmental complex of multiple spleens is closely related to the asplenic syndrome, with the important difference being left-sided symmetry rather than right-sided symmetry.

Termination of Left Superior Vena Cava in Left Atrium, Atrial Septal Defect, and Absence of Coronary Sinus A Developmental Complex

Eight cases of termination of the left superior vena cava in the left atrium are reviewed pathologically and clinically.In three of the five patients who died, pathologic examination revealed three anomalies which, together, are considered to form a developmental complex. The anomalies are (1) termination of the left superior vena cava in the left atrium, (2) absence of the coronary sinus, and (3) an atrial septal defect lying in the postero-inferior angle of the atrial septum. In the two remaining fatal cases, the aforementioned anomalies were associated with persistent common atrioventricular canal. In this situation, the atrial septal defect of the latter malformation was confluent with the atrial septal defect of the anomalous complex described.The coexistence of three anomalies-the left superior vena cava terminating in the left atrium, absent coronary sinus, and atrial septal defect-is considered to result from a single developmental abnormality. This takes the form of failure of complete formation of the left atriovenous fold, that fold which normally develops along the left side of the junction of the sinus venosus and the atrial portion of the heart.Clinically, features of increased pulmonary blood flow, coupled with duskiness of the skin, were the significant abnormalities.When cardiac catheterization was attempted through a right-sided vein, the data revealed a left-to-right shunt at atrial level, while levels of arterial oxygen desaturation were present.Except in cases with coexistent ventricular septal defect, pulmonary hypertension was absent.In each of the four patients in whom cardiac catheterization was performed through a left antecubital vein, the catheter was advanced into the left superior vena cava, left atrium, and left ventricle. This was substantiated by angiocardiography in three of the four patients. In our experience, angiocardiography was the most reliable procedure to substantiate the termination of the left superior vena cava in the left atrium.The present study suggests that, in the absence of pulmonary hypertension, a left-to-right transatrial shunt associated with systemic arterial oxygen desaturation may indicate the presence of a persistent left superior vena cava terminating in the left atrium. Moreover, an atrial septal defect identified surgically as lying in the postero-inferior angle of the atrial septum should suggest that an additional anomaly may be present in the form of termination of the left superior vena cava in the left atrium.

Anomalous inferior vena cava with azygos continuation (infrahepatic interruption of the inferior vena cava). Report of 15 new cases.

Summary Fifteen new cases of anomalous inferior vena cava with azygos or hemiazygos continuation are presented, together with a review of 26 cases previously published. The incidence of this anomaly approximates 0.6 per cent in patients with congenital heart defects. The commonly encountered cardiac defects include cor biloculare, atrioventricular canal, anomalously connecting pulmonary veins, double outflow right ventricle, large atrial septal defect, pulmonary stenosis or atresia, or combinations of these. Abnormal positions of the heart and abdominal viscera are often present. An antemortem diagnosis can be established by cardiac catheterization or angiocardiography. A typical “candy cane” appearance of the anomalous venous pathway occurs when angiocardiography is performed from the leg veins. The most common pattern is azygos continuation with normal position of the heart and abdominal viscera. There is no evident relationship between the subtypes of this anomaly and the intracardiac defects that may be present. The embryologic development of the venous system is discussed, particularly in regard to the origin of the anomalies under discussion. Anomalous inferior vena cava with azygos or hemiazygos continuation is best interpreted as interruption or failure of fusion of the hepatic and prerenal segments of the inferior vena cava, combined with persistence of either the right lumbar azygos vein or left lumbar hemiazygos vein. Thus, a better term for the anomaly would be “infrahepatic interruption of the inferior vena cava with azygos (hemiazygos) continuation.”

Aortico-Left Ventricular Tunnel

To be distinguished from aneurysm of an aortic sinus (Valsalva) is the rare condition in which an abnormal channel begins in the ascending aorta (above the right coronary artery), bypasses the aortic valve, and leads through the ventricular septum into the left ventricular cavity. This entity, which has not previously been diagnosed during life, to our knowledge, is herein named aortico-left ventricular tunnel. The cases of three patients (aged 3, 5, and 10 years) with this rare condition are described. The clinical, electrocardiographic, and radiological features were similar in each case and resembled those in aortic valvular insufficiency. The diagnosis may be established clinically through selective aortography. This reveals that the origin of the abnormal communication lies in the anterior wall of the ascending aorta above the levels of the coronary arterial origins. The abnormal communication leads to the left ventricle. The condition is distinguished from aortic sinus aneurysm by its origin above t...

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.

Anomalous Muscle Bundle of the Right Ventricle Hemodynamic Consequences and Surgical Considerations

Seven examples of anomalous muscle bundles of the right ventricle have been presented. In one patient having an associated ventricular septal defect, spaces between the anomalous muscle were confused surgically with the ventricular septal defect. In the remaining six cases, the anomalous muscle bundle caused obstruction within the right ventricular cavity. Five of these patients had associated ventricular septal defect, with a clinical picture resembling that of the cyanotic or acyanotic tetralogy of Fallot. The cases served to demonstrate that the surgeon who exposes the right ventricle to relieve an obstructive lesion in the right ventricular infundibulum or in the pulmonary valve, may find no such lesions in these locations but instead may be dealing with an obstruction more proximal than these areas. Relief of the obstruction within the right ventricle may be attained by resecting the anomalous muscle mass. In one patient having intact ventricular septum and pulmonary valvular stenosis, an anomalous muscle bundle within the right ventricle which lay proximal to the infundibulum was responsible for persistent right ventricular obstruction after pulmonary valvotomy. Anomalous muscle bundles in the right ventricle may be suspected when, in the angiocardiogram, they cause filling defects within the right ventricle.

ENDOCARDIAL FIBROELASTOSIS. A CLINICAL AND ANATOMIC STUDY OF 47 PATIENTS WITH EMPHASIS ON ITS RELATIONSHIP TO MITRAL INSUFFICIENCY.

A clinical and pathologic study of 47 cases with endocardial fibroelastosis is presented. These cases have been classified according to the associated cardiac anomaly and the anatomic condition of the left ventricle. In so doing, a better understanding is had of the role the cardiac anomaly associated with endocardial fibroelastosis plays in the production of cardiac signs and symptoms.In each of the specimens available for review, a change of the mitral valve was present that rendered it insufficient. Those cases grouped as primary endocardial fibro-elastosis had this as the only abnormality present, other than the endocardial fibroelastosis. Many of the clinical and laboratory findings in this group could be explained on the basis of mitral insufficiency, and one cannot necessarily assign the cardiac signs and symptoms to the endocardial process itself. In those cases with associated cardiac anomalies the hemodynamic consequences appear to be a summation of the combined effects of the mitral insufficiency, endocardial fibroelastosis, and the associated cardiac anomaly.In this review, we were unable to distinguish, by either gross or microscopic methods, between primary and secondary endocardial fibroelastosis. Since use of these terms suggests etiologic relations, it is preferable to classify cases of endocardial fibroelastosis on the basis of the structural abnormalities present.