Don M. Hosier

Supravalvular aortic stenosis: Clinical experiences with four patients including familial occurrence☆

Abstract The clinical findings in four patients with supravalvular aortic stenosis are described, and previously reported cases reviewed. The association of this defect with the clinical findings of aortic stenosis, a marked difference in the blood pressures and pulses in the upper extremities, and the frequent absence of poststenotic dilatation of the aorta is stressed. The frequency with which one or a combination of the following defects occur, namely, aortic valve deformity, aortic regurgitation and abnormalities of coronary artery filling, is noted. The present study provides documentation of the familial occurrence of this defect, or combination of defects. Angiocardiographic studies are necessary to confirm the diagnosis. Complete surgical correction may not be possible, and there is no objective evidence available at present that partial correction will be accompanied by increased longevity.

Defects of the interventricular septum in infancy; a two-stage approach to their surgical correction.

THE high mortality of open-heart surgery for defects of the interventricular septum in infants may be avoided by a two-stage approach instead. Surgical treatment is urgently needed in many of these...

Ligation of patent ductus arteriosus for persistent respiratory distress syndrome in premature infants.

Summary Twelve patients with patent ductus arteriosus and respiratory distress syndrome have been managed by ligation of a patent ductus arteriosus. The mean weight at operation was 1246 g. All 12 patients survived operation, but three died of complications of their RDS. Two patients developed chronic pulmonary changes related to prolonged ventilatory support. Surgical intervention for patent ductus arteriosus should be done early in a premature infant patient with continued respiratory distress.

Congenital tricuspid stenosis. The diagnostic value of cineangiocardiography and hepatic pulse tracing.

Abstract 1. 1. By the use of cineangiocardiography, a three-month old patient with congenital tricuspid stenosis has been correctly diagnosed during life. All previous reports of this condition have emanated solely from postmortem findings. 2. 2. The clinical features of tricuspid stenosis are similar to those of tricuspid atresia. However, the presence of a normal or right axis deviation in the electrocardiogram in a patient suspected of having tricuspid atresia should make one suspect the existence of tricuspid stenosis. Cineangiocardiography should be undertaken to differentiate between the two conditions. An hepatic pulse tracing is helpful in correctly timing the liver pulse. In our patient, it eliminated the possibility of tricuspid incompetence being present. 3. 3. Congenital tricuspid stenosis should be treated as a clinical entity separate from tricuspid atresia because a correct diagnosis of the former can now be made during life. In addition, in keeping with the recent advances in tricuspid surgery, surgical correction may be feasible. 4. 4. It is suggested that cineangiocardiographic findings, perhaps when correlated with pressure gradients across the valve, may be of value in the diagnosis of acquired tricuspid stenosis.

Replacement of entire right atrial wall in an infant with a cardiac rhabdomyoma

Summary A 9-mo-old white female with severe supraventricular tachycardia on cineangiographic studies proved to have a space-occupying mass in the right atrium. A rhabdomyoma of the right atrium was removed and the entire right atrial wall was reconstructed with autogenous pericardium. The infant is asymptomatic 1 yr following surgery.

Anomalous Origin of Left Pulmonary Artery From the Ascending Aorta

A patient whose left pulmonary artery arose from the ascending aorta is described. The embryology and a review of the literature concerning this unusual anomaly is presented. This is the third reported case of an anomalous origin of the left pulmonary artery without associated intracardiac defects and the sixth one successfully corrected. This patient presented the interesting opportunity to compare histologically the ef fects of having one lung connected to the lesser circulation and the other to the systemic circulation in the same patient. The severe cardiac symptoms usually en countered and the otherwise fatal outcome makes surgical correction of this lesion mandatory.

The Etiology of Mitral Regurgitation: The Marfan Syndrome.

Excerpt The occurrence of varying cardiovascular abnormalities in patients with the Marfan syndrome, particularly aortic dilatation, regurgitation, and dissection, is well-recognized. However, mitr...

ANATOMICAL VENTRICULAR HYPERTROPHY PATTERNS IN COMPLETE TRANSPOSITION OF THE GREAT VESSELS

Ventricular hypertrophy in congenital heart disease is related in general to two haemodynamic changes, systolic overloading and diastolic overloading. A combination of systolic and diastolic overloading is not uncommon. Shunts like those with ventricular septal defect, atrial septal defect, and patent ductus arteriosus give rise to a diastolic overload. Obstruction to outflow in either ventricle produces a systolic type of overloading. In complete transposition of the great vessels, the right ventricle bears the higher systemic pressure while the left ventricle has the normally lower pulmonary pressure. With this arrangement hypertrophy ofthe right ventricle is expected to develop. However, the presence of shunts and other defects may bring important hamodynamic pressure changes in the two ventricles in a way that hypertrophy of one or both ventricles will be less predictable. This report shows the relation of these factors in the production of anatomical ventricular hypertrophy patterns.

Supravalvular Aortic Stenosis: Clinical Experiences with Ten Patients Including Occurrences in Two Families.

Excerpt Clinical recognition of supravalvular aortic stenosis causing left ventricular outflow obstruction is a recent event. Initially considered to be a pathologic curiosity, it is now apparent t...