Edwards, Jesse E.

Thirty-three cases of corrected transposition of the great vessels are analyzed: 31 of corrected transposition in situs solitus, and two of corrected transposition in situs inversus. In 18 of these 33 cases, of which 64% were males, diagnosis was confirmed by necropsy or operation. There was no history of congenital heart disease in the families of any of these patients. The person whose sole anomaly is corrected transposition has no hemodynamic basis for symptoms. Symptoms are the result of associated lesions, the most common of which are anomalies of the left atrioventricular valve usually causing incompetence, ventricular septal defects, pulmonary valvular stenosis, and advanced disturbances of atrioventricular conduction. Physical findings in corrected transposition depend upon these associated defects. Noncardiac associated anomalies are rare. Anteroposterior thoracic roentgenograms may suggest the diagnosis, particularly when a narrow waist is present at the base of the heart, accompanied by the absence of a pulmonary-artery "knob" and the presence of an enlarged left atrium. Electrocardiographic features that should suggest corrected transposition are atrioventricular block (particularly 2:1 or complete heart block), abnormal P waves, absent or low R voltage in aVR, an initial Q wave in leads 3, aVR, aVF and V1, absence of a Q wave in lead V6, upright T waves across the precordium in children, and a clockwise spatial loop in the frontal plane. Cardiac catheterization may be diagnostic in this malformation when careful documentation is made of the location of the atrioventricular valves and their relation to the semilunar valves, together with the position of the great vessels in the anteroposterior and lateral views. Alteration of hemodynamics is secondary to associated malformations. Inability to enter the pulmonary artery during catheterization should arouse suspicion, and in these cases, particularly, angiocardiography can be diagnostic. The decision as to whether the most frequently associated malformations exist as isolated entities, or whether they are present in combination with corrected transposition, appears to be the main differential problem–together with the differentiation of corrected transposition with associated cyanosis from complete (that is, uncorrected) transposition. Surgical treatment of corrected transposition has been difficult because of complete atrioventricular block before or after operation, residual incompetence when deformed left atrioventricular valves were present, and the mirror-image arrangement for the coronary arteries. The most common cause of death was cardiac failure, often with a terminal bout of pulmonary edema. The average age at death in this series was about 14 years, with a median age at death of about 4 years. A review of the literature again emphasizes the association of corrected transposition with some degree of atrioventricular block evidenced on the electrocardiogram. The most frequently reported associated cardiac lesions were ventricular septal defect, pulmonary stenosis or atresia, patent ductus arteriosus, and dextrocardia. The incidence of anomalies of the left atrioventricular valve in these reported cases has probably been underestimated.

A case of the Taussig-Bing complex with necropsy findings is reported. Of additional interest was the unusual occurrence of associated malformations of the aortic-arch system, including coarctation of the aorta and anomalous origin of the right subclavian artery proximal to the coarctation. A narrow patent ductus arteriosus was present and probably represented a temporary phase in the process of normal anatomic closure. A single coronary artery that divided into the two main coronary arteries shortly after its origin also was present. Origin of the anomalous right subclavian artery proximal to a zone of aortic coarctation is an extremely rare arrangement among malformations of the aortic-arch system.

Simple plastic constricting devices for producing experimental arterial stenosis may be prepared from formalinized polyvinyl alcohol (surgical ivalon) sponge. Not a single instance of arterial rupture, a common sequel of stenosis produced by rigid devices, was encountered in 56 experiments.

Among certain patients with cardiovascular malformations, a common phenomenon is a ventricular septal defect and absence of anatomic origin of the pulmonary arterial supply from the heart. In this situation, true pulmonary arteries may be present or absent. In the latter case, the pulmonary arterial supply comes through bronchial arteries. When true pulmonary arteries are present, the left and right pulmonary arteries either may be in continuity at their origins (confluence) or they may arise from separate sites (nonconfluence). When confluence is present, the two pulmonary arteries may arise either from an atretic pulmonary trunk or from certain forms of persistent truncus arteriosus. Pulmonary atresia may be localized to the proximal end of the pulmonary trunk or diffuse in this vessel. When nonconfluence is present, each pulmonary artery arises separately from one of three possible sources as follows: (1) a form of persistent truncus arteriosus, (2) through ducti arteriosi from the aortic arch system, or (3) through bronchial arteries from the descending aorta.

The major parts of the atrioventricular conduction system of the human heart were traced in 21 instances of ventricular septal defect: 19 were examples of variously located uncomplicated ventricular septal defects and two of the tetralogy of Fallot. In the presence of a ventricular septal defect, the conduction system was found to have a normal course, except when the ventricular septal defect lay in a position normally occupied by the conduction system. In each specimen with a defect posterior and inferior to the crista supraventricularis, the conduction system occupied a position posterior and inferior to the defect. In no instance did the conduction system occupy a position superior to a defect of this type. Defects located in the posterobasal portion of the muscular part of the ventricular septum sometimes were posterior to the main parts of the conduction system, so that the conduction tissue was related to the anterior edge of the defect. No example of a defect lying superior to the crista supraventricularis was studied. In our two examples of tetralogy of Fallot, the position of the conduction system was essentially similar to that occurring in the usual variety of ventricular septal defect, that is, posterior and inferior to the crista supraventricularis.

Among 13,314 necropsies following death from neoplastic and nonneoplastic diseases performed at the Mayo Clinic from 1942 through 1958, 189 cases of secondary malignant lesions of the pericardium were demonstrated, an incidence of 1.42 per cent. Carcinoma of the lung and breast, the lymphomas, and leukemia constituted the large majority of the primary malignant lesions that caused the pericardial metastasis. Fifty-five of the 189 patients were adjudged to have had some impairment of cardiac function as a consequence of the pericardial metastasis and the following conclusions concern this group. 1. In 47 patients (86 per cent), the lesions were either the immediate or a contributory cause of death. 2. The most common mechanism whereby metastatic lesions in the pericardium interfered with cardiac function was by the development of pericardial effusion. Next in frequency was effusion combined with myocardial invasion and then cardiac constriction by tumor. Combinations of these processes with malignant encroachment on the great vessels and with invasion of coronary veins were the other mechanisms noted. Involvement of coronary arteries and the cardiac nerve supply was not noted. 3. Pericardial fluid was significant in causing tamponade in 31 cases. The fluid was frankly hemorrhagic in a small minority of these cases although fibrinous pericarditis was present in 45 (82 per cent) of the group. Pleural effusion was present in 48 (92 per cent) of 52 cases and was usually bilateral. Ascites was present in 22 (50 per cent) of 44 cases; the quantity was usually rather small. 4. Only one patient had metastasis to the pericardium without metastasis to other thoracic structures; exclusive of patients with leukemia, 43 (92 per cent) of 47 patients had extrathoracic metastatic lesions. 5. Dyspnea, cough, pleural effusion, hepatomegaly, and thoracic pain were the most commonly recorded signs and symptoms that might be related to the pericardial lesions. However, more specific signs and symptoms directing attention to pericardial or cardiac involvement were not commonly recorded. 6. The diagnosis of pericardial metastatic disease was suspected or established in only 16 (29 per cent) of the 55 patients, although 34 (62 per cent) either received treatment for congestive heart failure or were given roentgen therapy to mediastinal structures. 7. Of the 27 patients whose electrocardiograms were available, 25 had T-wave abnormalities, 22 had low QRS voltage, and 15 showed sinus tachycardia. 8. Thoracic roentgenograms, made on 53 patients, supplied clues to the diagnosis of pericardial malignancy in 28.

The records of 66 necropsied cases of periarteritis nodosa in which there was no associated collagen disease were reviewed with reference specifically to the heart. Clinically, congestive heart failure and its manifestations were the most important cardiac findings. Congestive heart failure developed some time during the illness in 57 per cent of the patients, and 44 per cent died as a direct result of this cardiac condition. Myocardial infarction was diagnosed clinically in only three cases. The electrocardiogram was abnormal in 35 of 41 cases but was diagnostic of left ventricular hypertrophy in only six cases and of acute myocardial infarction in only three cases; in the other cases the changes were of a nonspecific nature. Pathologically, 41 of the 66 hearts had evidence of necrotizing arteritis of the coronary arteries; in 39 of these the arteritis had been acute, and in two it had healed. Forty-one of the 66 hearts exhibited myocardial infarcts. Evidence of acute pericarditis was found in 22 hearts; nine of these cases were attributed to uremia, one was due to transmural myocardial infarction, three could not be classified, and the remaining nine were attributed directly to the periarteritis nodosa. Forty-one of the 64 hearts that had been weighed were hypertrophied.

The high rate of necropsy in deaths among permanent residents of Rochester, Minnesota, provided a unique opportunity to study the prevalence of coronary heart disease and the frequency and mode of death resulting from this disease. In this community of approximately 30,000 population, necropsy was done in 73 per cent of all resident deaths during the years 1947 through 1952. Included in this group were 691 necropsies that represented 67 per cent of the 1,026 deaths of persons 20 years of age or older. Coronary heart disease caused death in 221 patients (23 per cent of all necropsies and 32 per cent of all necropsies on adults). These 221 coronary deaths in adults represented 41 per cent of the men and 22 per cent of the women. The coronary deaths were attributed to acute coronary failure (sudden death) in 94 patients (43 per cent), acute myocardial infarction in 87 patients (39 per cent), congestive heart failure in 32 patients (14 per cent) and thromboembolism in eight patients (4 per cent). The 87 patients dying during acute myocardial infarction died of congestive heart failure (30 per cent), myocardial rupture (24 per cent), acute coronary failure (23 per cent), and thromboembolism (14 per cent), with the remaining 9 per cent dying of a combination of acute myocardial infarction and additional serious systemic disease. The greatest number of deaths from coronary heart disease occurred during the seventh decade of life in men and the eighth decade in women. At least one coronary artery exhibited from 25 to 100 per cent obstruction from atherosclerosis in 513 hearts (74 per cent of the adult necropsies). These necropsies, representing two thirds of all deaths in adults in this community, disclosed that significant coronary-artery disease was present in three of four adults and was the cause of death in four of 10 men and two of 10 women.