Helen B. Taussig

Complete transposition of the aorta and a levoposition of the pulmonary artery: Clinical, physiological, and pathological findings☆

Abstract A new clinical syndrome is described. The malformation consists of a transposed aorta, a large pulmonary artery which arises primarily from the right ventricle and partially overrides the ventricular septum, a high ventricular septal defect, and right ventricular hypertrophy. Clinically, in this instance the heart was but slightly, if at all, enlarged; there was a systolic murmur and thrill. Cyanosis dated from birth. Clubbing of the extremities developed at an early age. The red blood cell count, the level of the available hemoglobin, and the hematocrit reading were increased. The electrocardiogram showed evidence of right ventricular hypertrophy. The x-ray films of the heart showed fullness of the pulmonary conus and increased hilar shadows. Upon fluoroscopy, the pulmonary vessels showed faint expansile pulsations. The oxygen saturation of the arterial blood was abnormally low and fell still further with exercise. In brief, the clinical syndrome associated with this malformation resembled that of an Eisenmenger complex, except that cyanosis dated from birth. The two conditions showed a further difference in that, in the Eisenmenger complex, exercise causes an increase in the consumption of oxygen per liter of ventilation, whereas in this malformation, exercise causes a decline in the oxygen consumption per liter of ventilation. Intracardiac catheterization studies revealed a higher oxygen content in the pulmonary artery than in the femoral artery. It is probable that the volume of blood which entered the pulmonary artery from the right ventricle was equal to that directed from the left ventricle into the aorta. The extensive intimal changes in pulmonary arterioles appeared to be sufficient to account for the increased resistence in the pulmonary vascular bed.

Death from lightning--and the possiblity of living again

Abstract This article reviews a number of examples of persons who were struck by lightning and recovered. Statistics indicated that more persons recover after being struck than are killed. A person...

Ebstein's Anomaly of the Tricuspid Valve Report of Three Cases and Analysis of Clinical Syndrome

In Ebsteins anomaly the tricuspid valve is displaced downward so that the upper portion of the right ventricle is incorporated in the right auricle. This imipairs the efficiency of the right side of the heart and produces a distinctive syndrome, which is described here for the first time. Diagnosis is important because this malformation, which is not amenable to surgery, may be confused with the tetralogy of Fallot.In Ebstein9s anomaly the tricuspid valve is displaced downward so that the upper portion of the right ventricle is incorporated in the right auricle. This imipairs the efficiency of the right side of the heart and produces a distinctive syndrome, which is described here for the first time. Diagnosis is important because this malformation, which is not amenable to surgery, may be confused with the tetralogy of Fallot.

Possible Injury to the Cardiovascular System from Vitamin D

Excerpt The interest in vitamin D as a possible source of injury to the cardiovascular system lies in the recent advances in our knowledge of supravalvular aortic stenosis. When, in 1958, Denie and...

Late Hemodynamic Complications of Anastomotic Surgery for Treatment of the Tetralogy of Fallot

Seventeen patients with late hemodynamic complications of anastomotic surgery for the tetralogy of Fallot have been studied. Two syndromes have been noted and both seem related to shunts of greater than ideal size. The clinical characteristics of these syndromes are delineated.

Ten to Thirteen Year Follow-up on Patients after a Blalock-Taussig Operation

Analysis of 224 patients who were living 5 years after operation showed that at the end of 10 to 13 years, 40 per cent were still doing well, 46 per cent had failed to maintain improvement, 2 per cent had developed cardiac failure, and 12 per cent had died. The majority of patients who had failed to maintain improvement suffered from increasing cyanosis and dyspnea associated with a small heart and clear lung fields; hence these patients can be helped by a second operation:— either a second anastomosis or total correction. The results of a second anastomosis have, however, not been as satisfactory as the first anastomosis. Clearly total correction is the ideal. The age at which total correction is indicated depends not only on the skill of the surgeon but on the severity of the abnormality. The long-time results of total correction are unknown. Inasmuch as abnormal tissue does not grow as fast as normal tissue, the possibility exists that the area in which tissue has been excised and a patch inserted may fail to grow or even contract and cause the right ventricle to become abnormally small. Analysis of the results at the end of 5, 10, and 13 years showed that only 10 patients (less than 5 per cent) had required a second operation at the end of a years and only 20 per cent had required a second operation in the ensuing 5 years. Older patients, i.e., those between 8 and 12 years, had done far better than younger children. Only two of a group of 38 children operated upon between 8 and 12 years of age had required a second operation within 10 years. Thus a Blalock-Taussig operation offers the child an excellent chance to attain the major part of his growth before total correction is undertaken. A further advantage of an early anastomotic procedure is that it restores the pulmonary vascular bed to normal and thereby renders it easier for the lungs to adjust to the full circulatory load placed on them at the time of the total correction. The conclusion is reached, therefore, that for infants and small children a Blalock-Taussig operation is preferable. For children who have attained or nearly attained full growth, total correction is the ideal. For many patients, especially those between 8 and 12 years of age for whom total correction is not possible, a simple Blalock-Taussig operation offers an extremely good prognosis.

Valvular Pulmonic Stenosis with Intact Ventricular Septum and Patent Foramen Ovale Report of Illustrative Cases and Analysis of Clinical Syndrome

In this malformation the difficulty in sending blood through the stenosed pulmonary valve leads to right heart enlargement and to functional patency of the foramen ovale, through which a venousarterial shunt occurs. The onset of symptoms and their severity depend on the size of the opening in the pulmonary valve. Characteristic physical findings and results of fluoroscopy, electrocardiogram, circulation time, arterial blood studies, exercise test, angiocardiogram, and cardiac catheterization are presented. Diagnosis is important because these patients develop cardiac failure following at Blalock-Taussig operation, but may be greatly helped by pulmonic valvulotomy.

Five-Year Postoperative Results of First 500 Patients with Blalock-Taussig Anastomosis for Pulmonary Stenosis or Atresia

The evaluation of surgical corrective procedures depends to an important degree on the long-term benefits derived from the operation. Three hundred and eighty-nine of the first 500 patients with a Blalock-Taussig anastomosis for pulmonary stenosis or atresia survived 6 months or longer. Detailed information is now available on 244 of these patients, who were originally improved by operation and who have been followed for 5 years or longer. The results obtained on patients with tetralogy of Fallot are compared with those with other malformations. The alteration of the size of the heart over a period of years has been studied. A comparison between the preoperative and postoperative hemograms and arterial oxygen saturations is presented. The optimum age for operation, the causes of late deaths, and the incidence of bacterial endocarditis are discussed.

CONGENITAL HEART DISEASE IN THE NEONATAL PERIOD: AUTOPSY STUDY OF 170 CASES.

A review of the autopsy records of The Johns Hopkins Hospital between 1927 and 1959 revealed 170 infants who died under 1 month of age with congenital cardiovascular malformations. The records were analyzed for the incidence of the different cardiac anomalies, the time of death, and the frequency of noncardiac malformations. The most frequent cardiac malformations encountered were ventricular septal defect, transposition of the great vessels, coarctation of the aorta, and mitral and aortic atresias. Infants with ventricular septal defects had a high incidence of prematurity, infection, and noncardiac malformations. Most of these deaths in the neonatal period occurred in the first week of life. Infants with transposition of the great vessels lived longer, and the incidence among them of prematurity and of noncardiac defects was low. The data from two similar series are compared with those from this study.

Complete transposition of the great vessels

Abstract We have had the opportunity to study four cases of complete transposition of the great vessels clinically and post mortem. In one case there was, in addition, a patent foramen ovale, a patent interventricular septum, and a patent ductus arteriosus; in the second, a patent interventricular septum; in the third, a patent foramen ovale and a patent ductus arteriosus; and in the fourth, a patent foramen ovale, a patent ductus arteriosus, and complete interruption of the isthmus of the aorta. Each had its own individual variations, but all showed the characteristic features of complete transposition of the great vessels, namely, (1) persistent cyanosis; (2) cardiac enlargement, especially of the right ventricle; (3) a narrow aortic shadow in the anteroposterior roentgenogram; and (4) an increase in the width of the roentgenographic shadow cast by the great vessels when the patient is placed in the left anterior oblique position.