C.A. Wagenvoort

Cardiovascular system in Marfan's syndrome and in idiopathic dilatation of the ascending aorta.

Abstract The gross and histopathological findings in the cardiovascular system in four cases of Marfans syndrome were compared with those in four cases of idiopathic dilatation of the aorta, and they were found to be virtually identical. No changes were observed that could be considered to be characteristic of one condition that did not occur in the other. The lesions in the arterial system, although most pronounced in the ascending aorta, were present also in other elastic arteries, including the pulmonary artery and its elastic intrapulmonary branches. The principal features of these lesions were interruption, fragmentation, and rarefaction of elastic fibers of the media with mucoid degeneration.

Hypoplasia of ascending aorta. An unusual form of supravalvular aortic stenosis with special reference to localized coronary arterial hypertension.

Abstract Anomalies causing obstruction to blood flow in the aorta above the aortic valve, though rare, are of three types: (1) A localized zone of obstruction resembling a diaphragm in the ascending aorta; (2) localized narrowing of the ascending aorta and (3) uniform narrowing of the entire ascending aorta. In the case of the 2 year old boy reported, the last type of anomaly was present. In this case clinical data were suggestive of aortic stenosis, electrocardiographic findings of left and right ventricular overwork, and catheterization data of obstruction to the blood flow from the left ventricle, some degree of left ventricular failure, and left ventricular and pulmonary hypertension of moderate degree. An attempt to relieve the obstruction was not successful. At necropsy left ventricular hypertrophy, a suggestion of mitral insufficiency and uniform narrowing of the ascending aorta were found. In the coronary arteries the media was thickened, and deposition of elastic fibers was evident. These changes are attributed to systolic hypertension in the coronary vessels.

The Pulmonary Arterial Tree in Ventricular Septal Defect: A Quantitative Study of Anatomic Features in Fetuses, Infants, and Children

The pulmonary vascular tree was studied in 12 cases of uncomplicated atrial septal defect in fetuses, infants, and children. The ages of these subjects ranged from 24 weeks of fetal life to 11 years. A quantitative analysis was made of the medial thickness and of the index of medial surface area (ratio of medial tissue to pulmonary parenchyma) of the pulmonary arterial branches. In contrast to subjects with ventricular septal defect in corresponding age groups, the three fetuses in this series had medial hypertrophy, as indicated by a high index of medial surface area. In the postnatal group, both the medial thickness and the index of medial surface area were also usually greater than in the controls of corresponding ages, notably in two patients, who, at the ages of 7 weeks and 4 months, died of cardiac failure. No satisfactory explanation for these unexpected findings can be offered. Slight to moderate intimal proliferation was observed in some muscular and elastic pulmonary arteries, in bronchial arter...

Origin of Right Pulmonary Artery from Ascending Aorta

A case is reported of a 20-day-old infant with origin of the right pulmonary artery from the ascending aorta. Besides its abnormal origin, the artery was stenotic in its proximal half. We believe the malformation results from persistence of the distal (instead of the proximal) part of the primitive sixth aortic arch and of a portion of the right dorsal aorta. The presence of ductal tissue in the wall of the right pulmonary artery supports this concept. The pulmonary vasculature in the two lungs showed great differences in medial and intimal characteristics. A second case of origin of the right pulmonary artery from the aorta, deseribed elsewhere, provided material for comparative study of the structure of the right main pulmonary arteries and the intrapulmonary branches.

The Pulmonary Arterial Tree in Ventricular Septal Defect

A quantitative analysis was made of the pulmonary arterial medial thickness and index of medial surface area (ratio of medial tissue to pulmonary parenchyma) in 50 cases of uncomplicated ventricular septal defect in fetuses, infants, and children. The ages of the subjects ranged from 26 weeks of fetal life to 12 years. The results were compared with those obtained from a control group of the same age. During the fetal and newborn period, both the thickness and the index of surface area of the media were generally within normal limits, although in an occasional case pulmonary arterial branches were considerably thinner than those in corresponding controls. The subjects between 1 week and 5 weeks of age had a medial thickness and an index of medial surface area that were lower than the average values at the time of birth. In this regard the pulmonary arterial tree in cases of ventricular septal defect corresponded with that in normal newborn infants. Soon after, however, and most clearly from 8 weeks on, th...