Sidney Blumenthal

Congenitally corrected transposition of the great vessels.

Congenitally corrected transposition of the great vessels is an important basic anomaly of the heart which has only recently received wide clinical attention. First described by von Rokitansky in 1875 (33), the anomaly remained a clinical curiosity until modern surgical advances in the treatment of congenital heart disease made accurate diagnosis important. Less than 150 cases are described in the literature, but the condition cannot be extremely rare, since we have encountered more than 30 cases, and similar large series have been reported from other institutions (17, 27). Among our cases is the first example of congenitally corrected transposition without associated anomalies diagnosed on the basis of chest films, the second such case to be recognized in life. Basic Anatomy of Corrected Transposition of the Great Vessels The aorta and pulmonary artery are transposed so that the aorta arises on the left and ventrad, in the usual position of the pulmonary artery, while that vessel arises on the right and ...

Congenital complete heart block: A classification and experience with 18 patients

Abstract The clinical course of 18 patients with congenital complete heart block, who ranged in age up to 32 years, has been reviewed. In 17, cardiovascular diagnostic studies or necropsy were performed. The frequency of ventricular inversion (congenitally corrected transposition of the great vessels) and associated cardiovascular malformations prompted a classification based on embryologic development of the heart. Ventricular inversion occurred in 8 patients, and normal bulboventricular loop development was present in 9. Cardiovascular malformations were noted in 13 of the 17 patients studied anatomically. Prognosis for well-being and long term survival is primarily related to (1) the presence or absence of cardiovascular defects and (2) the stability of the ventricular pacemaker. The complications of congestive heart failure or Stokes-Adams attacks occurred in 9 patients, 8 of whom had associated cardiovascular defects with a stable ventricular pacemaker. The only patient without a cardiovascular anomaly who experienced syncope had an unstable ventricular focus. Cardiovascular surgery is well tolerated in patients with congenital complete heart block. Artificial cardiac pacing is valuable in the supportive management of symptomatic infants with congestive heart failure or Stokes-Adams attacks. Its application in 2 infants, in whom a cardiac pacemaker was implanted, is described.

VENTRICULAR SEPTAL DEFECT WITH SHUNT FROM LEFT VENTRICLE TO RIGHT ATRIUM BACTERIAL ENDOCARDITIS AS A COMPLICATION

During a two-year period we have seen six patients with malformations of the ventricular septum resulting in the shunting of blood between the left ventricle and the right atrium. Surgical closure of the defect has been successful in all. A history of bacterial endocarditis was established in two patients and strongly suspected in two others before operation. The surprisingly large number of patients with a malformation previously considered rare and the unusually high incidence of endocarditis as a complication prompted a review of the anatomical and clinical features of this anomaly. There have been 26 patients reported with left ventricular to right atrial shuntst on whom relatively complete clinical studies are available (Perry, Burchell, and Edwards, 1949; Stahlman et al., 1955; Kirby, Johnson, and Zinsser, 1957; Ferencz, 1957; Lynch et al., 1958; Gerbode et al., 1958; Kjellberg et al., 1959; Braunwald and Morrow, 1960; Levy and Lillehei, 1962). Symptoms were directly related to the amount of left-to-right shunting of blood with pulmonary vascular engorgement. These consisted of poor feeding in infancy and easy fatigue; tachypncea, pneumonia, and congestive heart failure were often noted. The usual findings on examination were a precordial thrill and loud systolic murmur along the lower left sternal border; a mid-diastolic apical rumble was heard in some patients. Chest radiography generally demonstrated cardiomegaly, pulmonary vascular engorgement, and in 15 of the 26 patients specific enlargement of the right atrium. The electrocardiogram was not diagnostic; an abnormal P wave was present in a few of the tracings. Except for one patient with a mean electrical axis of -90°, there were no reports of the axis being to the left or superiorly oriented. Cardiac catheterization demonstrated a left-to-right shunt at the level of the right atrium in all but 2 patients. The right atrial pressure was raised in 5 of the 9 patients in whom it was recorded. Descriptions of the cardiac defects at operation or necropsy are available in 24 patients: in 6 there was a defect high in the membranous ventricular septum with an opening in the floor of the right atrium just above the insertion of the septal leaflet of the tricuspid valve. One of these also had an abnormal mitral valve with obstruction to the left ventricular outflow tract (Ferencz, 1957). In the remaining patients a defect in the membranous ventricular septum occurred below the level of the tricuspid ring and was associated with a defect in the tricuspid valve. In 11 of these it appears that

Electrocardiographic changes associated with papillary muscle infarction in congenital heart disease

The electrocardiograms of 18 infants and children with congenital heart disease (4 with aortic stenosis and 14 with total anomalous pulmonary venous connection) and necropsy evidence of infarction of the papillary muscle or free wall, or both, were reviewed. In the presence of left ventricular hypertrophy, a Q wave in lead V3R followed by a small R wave is strong evidence of infarction of the anterior left papillary muscle or anterior left ventricular free wall, or both. When associated with serially diminishing amplitude of the R wave in lead V3R this is diagnostic of infarction. In the presence of right ventricular hypertrophy a Q wave in lead V3R may be due to the hypertrophy. The finding of a serially diminishing R wave in this lead, however, is evidence of infarction of the anterior right papillary muscles or anterior right ventricular free wall. Hence, with left ventricular hypertrophy or right ventricular hypertrophy a Q wave in lead V3R assumes diagnostic significance for infarction when accompanied by a diminishing R wave amplitude over the right precordial leads.

An Evaluation of Total Correction of Tetralogy of Fallot

Forty-one consecutive patients, ranging in age from 41/2 to 27 years, were operated upon for complete repair of tetralogy of Fallot without an operative mortality. Postoperative catheterization of 25 patients permitted objective evaluation of the results which were, by the criteria adopted, excellent in 16 patients, good in 5, and poor in 4, including the 2 late deaths.Surgical experience has shown the necessity for extensive resection of the right ventricular outflow tract. Prosthetic patches are considered necessary for closure of most of the ventricular septal defects encountered, but are infrequently needed for reconstruction of the right ventricular outflow tract. Its use is indicated in certain patients with hypoplasia of the pulmonary annulus or artery.Age has been an important factor in patient selection. It is suggested that symptomatic patients under five years of age may benefit from a systemic-to-pulmonary arterial anastomosis before definitive correction is undertaken later in childhood.

Congestive heart failure in children

H EART failure in the adult is a well-recognized symptom complex in which the hemodynamic alterations have been documented and the principles of treatment standardized. Heart failure in infants and children is less well recognized despite the fact that it is not uncommon among patients of the pediatric age group requiring hospitalization. In this age group the etiology and clinical manifestations are strikingly different from those in older children and adults and although the principles of treatment are the same, dosage and choice of drugs require special consideration because of the altered physiologic response of the young patient. These differences in the clinical manifestations and in the response to treatment have been noted by several observers,1-4 but documentation of this altered physiology utilizing hemodynamic techniques has not been reported. The study of heart failure in the young patient poses many problems including the modification of available techniques or the introduction of new ones, as well as the development of standards for the normal child. The function of the heart is to maintain adequate output of blood under all circumstances. In heart failure the. cardiac output, which may be decreased (low output failure), normal, or increased (high output failure), is inadequate to sustain normal body functions. The heart is failing when it is unable to increase its output in response to demand and when it is able to maintain its output only by increasing venous filling pressure.6 Heart failure results in inadequate delivery of oxygen to the tissues first during exercise and subsequently at rest. A number of homeostatic mechanisms directed toward increasing the delivery of oxygen to the tissues are available. These homeostatic mechanisms represent “cardiac reserve.” They include: (1) Heart rate and (2) stroke volume both of which affect cardiac output directly, (3) the ability of the tissues to extract larger amounts of oxygen from available blood as reflected by alterations in arteriovenous di

Evaluation of an enlarged cardiothymic image in infancy

erence, (4) the transport of increased amounts of oxygen to the myocardium by alteration in coronary blood flow, and (5) cardiac hypertrophy. Most of the recognizable manifestations of heart failure are due to the homeostatic mechanisms attempting to restore cardiac output, rather than to a decrease in the output.

Congenital Cardiac Defects: A Physician's Guide for Evaluation and Management: Report of the Committee on Congenital Heart Disease

Abstract The relative contribution of the thymus and of cardiovascular structures to an enlarged or deformed mediastinal image on a roentgenogram of the chest may be difficult to evaluate in infants and children. Five cases are presented which posed problems in diagnosis: in the first, the thymus obscured the presence of dextroversion of the heart; in the second, the thymus widened the mediastinal image and gave the impression of cardiomegaly and hence of heart disease, where none existed; in the third, vascular structures including a persistent left superior vena cava were responsible for the deformed supracardiac image; in the fourth, an enlarged left lobe of the thymus resulted in distortion of the left heart border and the erroneous impression of corrected transposition of the great vessels; and in the fifth, an enlarged cardiothymic image in an infant with congenital heart disease decreased in size by spontaneous shrinkage of the thymus. The administration of adrenal glucocorticoid derivatives is suggested for the induction of temporary involution of the thymus gland in selected cases. This technic is of value when there is need to clarify the composition of an unusual cardiothymic image in order to estimate the size and configuration of the heart and great vessels.

Standards for Centers Caring for Patients with Congenital Cardiac Defects Report of the Subcommittee on Education and Standards in the Field of Congenital Heart Disease

This report was designed primarily for the physician who is not a cardiologist but who encounters, in the course of his practice, patients with congenital malformations of the heart. It will help him decide whether and when such patients should have special studies done in a cardiac center or by a cardiologist familiar with these problems. It is divided into three parts: one on infants, one on children and adults, and the last on management. The first two parts are designed to assist the physician in the selection of patients for special studies, the last to aid him in the management of patients under his care. A special section in part III is devoted to care of the pregnant woman with a congenital defect. The decision as to which studies are needed and when they should be performed should be left to the cardiologist or center to which the patient is referred.

Heart disease in infancy and childhood

TI HE purpose of this report is to provide standards for services and niecessary equipment for centers responsible for the diagnosis and surgical care of patients with congenital defects of the heart and blood vessels. Skill in this field is dependent oni traininig and on continuous extensive experience which in turn is related to the caseload of an institution. Before establishing such a center, careful consideration should be given to the real need in the city or area for this highly specialized type of diagnostic workup and surgical correction. Many skills and facilities needed are not ordiniarily available outside a medical center. Accurate diagnosis of the congenital cardiac malformation is a prerequisite to cardiac surgery. To have a diagnostic center for this purpose is, therefore, of prime importance for any hospital in which cardiac surgery is performed. This type of work requires highly developed teamwork on the part of the pediatric or medical diagnostician, surgeon, anesthesiologist, roentgenologist and members of allied services. It is not advisable to have a sharp divisioii between diagnostic and surgical responsibilities. The center in which cardiac surgery is done must be approved for resident traininig in surgery and in internal medicine or pediatrics. It must be ultimately responsible for both definitive diagnosis and surgical care. The essential requirements for a diagnostic ceniter follow below.