Dorothy H. Andersen

Pulmonary hypertension and cor pulmonale in cystic fibrosis of the pancreas

The present study was concerned with the pathogenesis of pulmonary arterialhypertension in 21 patients with the pulmonary complications of cystic fibrosis. Respiratory function tests failed to distinguish between patients with normal pulmonary arterial pressure and those with pulmonary hypertension. On the other hand, the pulmonary arterial pressure was directly related to the degree of hypoxia. Relief of hypoxia, either acutely by breathing high-oxygen mixtures, or chronically by the treatment of respiratory infection and bronchial drainage, returned pulmonary arterial pressure toward, or to, normal. These physiologic observations imply a predominant role for hypoxia in the pathogenesis of pulmonary hypertension in cystic fibrosis, and indicate its potential reversibility in this disease.

Persistent venous valves, maldevelopment of the right heart, and coronary artery-ventricular communications

Abstract An unusual malformation of the heart in a newborn infant is described, consisting of persistent venous valves in the right atrium, hypoplasia of the tricuspid valve, pulmonary atresia, and multiple coronary artery-ventricular communications with the right ventricle. Coronary flow to the right heart was shunted through the right ventricle, and focal necrosis was seen in the right ventricular myocardium. The pertinent literature is reviewed and the pathogenesis of the malformation is discussed.

Aortic Origin of the Right Pulmonary Artery

The clinical course and pathologic findings in three cases of anomalous origin of the right pulmonary artery from the ascending aorta are described. The malformation is characterized by (1) absent right branch of the pulmonary artery, (2) a single large vessel arising from the ascending aorta, entering the hilus of the lung and constituting its sole arterial blood supply, and (3) normal size and structure of the right lung. A review of the literature indicates the rarity of this lesion as an isolated anomaly; patent ductus arteriosus is the most frequently described additional defect. Histologic examination of the right and left lungs reveals bilateral and symmetrical pulmonary vascular changes, despite the difference in their arterial supply. Some pathophysiologic observations based on the necropsy findings in these cases are discussed. Early diagnosis and surgical correction are imperative, since this malformation may lead to irreversible congestive heart failure and death in infancy. Surgical repair may be accomplished by transplanting the anomalous right pulmonary artery from the aorta to the main pulmonary trunk.

Celiac syndrome: VI. The relationship of celiac disease, starch intolerance, andsteatorrhea

Summary The classical picture of celiac disease is believed to result from a deficiencyof multiple nutritional factors complicating and resulting from a basic constitutional disease. The steatorrhea of celiac disease is transitory, and with intensive vitamin and dietary therapy it disappears within two months; it is viewed as a complication of celiac disease due to a specific deficiency and comparable in this regard to rickets or scurvy. A consistent and objectively demonstrable characteristic of the basic disease is a deficiency of pancreatic amylase, associated with an excess of starch in the feces when starch is fed and clinical intolerance to dietary starch. In uncomplicated cases the presenting symptom is the occurrence of repeated bouts of diarrhea with large, foul, pale, foamy stools and an enlarged abdomen. These bouts are usually initiated by an upper respiratory infection. The symptoms respond to a diet high in protein, low in starch, and abundant in vitamins. The classical picture of celiac disease with steatorrhea is the result of a multiple deficiency state superimposed on the basic disease under adverse circumstances. An attempt is made to evaluate other phenomena of the disease with respect to whether they are part of the primary defect or a result of secondary nutritional deficiency. There is suggestive evidence that the difficulty in protein metabolism, shown by the high requirement for dietary protein and a tendency to hypoproteinemia on a normal diet, may be an expression of the basic disease.

Two Cases of Total Anomalous Pulmonary Venous Return of the Supracardiac Type with Stenosis Simulating Infradiaphragmatic Drainage

Two patients who died during the neonatal period with congestive heart failure and marked pulmonary congestion were found at autopsy to have total anomalous pulmonary venous return of the supracardiac type with stenosis of the pulmonary vein. The narrowing was the result of an unusual course of the vein, which passed between the left pulmonary artery and left main bronchus and was stenosed at this point. It is emphasized that the clinical and x-ray findings were indistinguishable from those of total anomalous pulmonary drainage below the diaphragm.

CYSTIC FIBROSIS OF THE PANCREAS IN YOUNG ADULTS

Excerpt I. INTRODUCTION Cystic fibrosis has only recently become a concern of adult medicine. It is a disease with a short history, introduced to clinical pediatrics in 1938, when three series of n...

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 ...

Acute interstitial myocarditis

Abstract A case is reported in which the clinical picture simulated that seen in coronary occlusion, but the pathological findings were those of acute interstitial myocarditis. In a brief review of the literature the following points stand out: 1. 1. The diagnosis is necessarily a pathological one, since there is no uniform clinical picture. 2. 2. While sudden death sometimes occurs, in the majority of cases death is preceded by symptoms of progressive myocardial failure of variable duration. 3. 3. Although there is no known etiology, the frequent association with infections, especially pyogenic infections of the skin, is suggestive. 4. 4. Analysis of the pathological findings in thirty cases reveals the fact that the microscopic picture is the one constant finding. 5. 5. In the majority of the cases there are also cardiac hypertrophy and dilatation, a greyish-yellow mottling of the myocardium of the left or both ventricles, and acute congestion of the viscera. 6. 6. In most of the cases of more than one months duration there are mural thrombi in the left ventricle.

Glycogen storage disease of the muscles. Report of a case with unusual features.

A case of glycogen storage disease of the muscle in a 2 1/2-year-old child is reported. This may represent a fourth type of glycogen disease of striated muscle although, with the data available, a severe form of McArdles disease with the onset in infancy cannot be excluded. The clinical findings consisted of hypotonia and palatal paralysis without cardiomegaly or hepatomegaly. Blood lactate concentration failed to rise normally following epinephrine administration. Striated muscle contained massive deposits of glycogen of normal molecular structure. Amylo-1, 6-glucosidase was normal. Phosphorylase was not assayed. A summary of the types of glycogen storage disease of muscle is presented. Muscle biopsy in early cases of muscle dystrophy is urged as a means of discovering further examples of glycogen disease of striated muscle.

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