Biomedical subjects

R M Freedom

Publications and source records attributed to R M Freedom.

At least 325 records · Page 18Linked to original sources

PubMed1976

Aneurysm of the atrial septum in tricuspid atresia: diagnosis during life and therapy.

Massive aneurysmal dilatation of the foramen ovale was diagnosed angiocardiographically in a patient with tricuspid atresia. The angiographic findings are distinct for this condition, and the pathogenesis appears to be related to a restrictive atrial communication in the patient with obligatory right to left shunting at atrial level. The anatomic potential for atrial restriction in the patient with tricuspid or pulmonary atresia, intact ventricular septum and diminutive right ventricle necessitates balloon atrial septostomy at the initial diagnostic cardiac catheterization. In the patient with aneurysmal dilatation of the foramen ovale, satisfactory decompression may be achieved by Blalock-Hanlon atrial septectomy, open atrial septectomy, or, possibly, balloon septostomy.

Cardiac Catheterization↗

PubMed1976

Anatomical variants in aortic atresia. Potential candidates for ventriculoaortic reconstitution.

Aortic valve atresia is usually associated with severe hypoplasia of the left ventricle and mitral apparatus. Palliative procedures, including atrial septectomy, pulmonary artery-aorta anastomosis, and pulmonary artery banding, have been performed in some patients. However, because of the distinct underdevelopment of the left heart, the long-term prognosis must be guarded even in the survivors of these procedures. Rarely, aortic valve atresia is found with adequate left heart chambers. Because very few of these patients are described, the clinical, angiocardiographic, and the pathological findings in two patients are presented, with comments on palliation in the neonatal period, and the potential for ventriculoaortic reconstitution.

Aortic Valve↗

PubMed1976

The angiocardiographic appearance of the endocardial cushion defect in selected transposition and malposition complexes.

Although the angiocardiographic features of the endocardial cushion defect (ECD) have been well described in the patient with two normally related ventricles (D-ventricular loop in situs solitus) and normally related great arteries, little attention has been focused on the angiocardiographic appearance of the ECD in patients with complex cardiovascular disease. Because of recent surgical advances in the treatment of single ventricle and double outlet right ventricle complexes, it has become increasingly important to document the status of the atrioventricular valves in these patients. The angiocardiographic features of the ECD are therefore described in two patients, one with D-loop, single left ventricle and outlet chamber, D-transposition of the great arteries, bilateral conus and pulmonary stenosis and the other with dextrocardia, situs inversus, double outlet right ventricle, common atrioventricular canal and pulmonary stenosis. In both patients, the left ventricular outflow tract, despite aortic-mitral discontinuity, is characterized by elongation, scalloping and serration, and the classic appearance of the "gooseneck" deformity is observed on left ventricular angiography. It thus appears that aortic-mitral continuity and two well-formed, normally related ventricles and great arteries are not invariable necessary to the manifestation of the "gooseneck" deformity in the patient with complex cardiovascular disease and associated endocardial cushion defect.

Abnormalities, Multiple↗

PubMed1976

Nonoperative management of late failure of a Glenn anastomosis. Transvenous wafer occlusion of patent superior vena cava--right atrial junction.

A case is presented illustrating delayed failure of a right Glenn anastomosis due to patency of the superior vena cava-right atrial (SVC-RA) junction. Clinical deterioration was rectified by a unique nonoperative approach. A transvenous catheter was designed, constructed, and used to accurately place an umbrella-like occlusive wafer at the SVC-RA junction. In this manner the integrity of the cavopulmonary shunt was re-established. A gratifying and uncomplicated palliation has thus far been achieved.

Cardiac Catheterization↗

PubMed1976

Selective aortic root angiography in the hypoplastic left heart syndrome.

Selective aortic root angiography was performed in 18 patients with the clinical diagnosis of hypoplastic left heart syndrome in order to allow more precise correlation with echocardiography. Aortic valve atresia and severe hypoplasia of the left heart chambers subsequently were substaniated at necropsy in each instance. Wide variation in the size and shape of the aortic root and ascending aorta was demonstrated angiographically in these patients. In some patients, despite aortic valve atresia, the dimensions of the aortic root and sinuses of Valsalva at angiography were almost normal. In addition, this technique allowed the demonstration of primitive vascular communications between the coronary artery and left ventricular cavity in 1 patient (coronary-cameral fistula). The realization that the aortic root may approach nearly normal size in some patients with the hypoplastic left heart syndrome is essential to the echocardiographic diagnosis of this disease and to the differential diagnosis from other cardiac and noncardiac causes of severe neonatal congestive heart failure.

Angiocardiography↗

PubMed1975

Echocardiography in atrioventricular canal defect: a clinical spectrum.

Echocardiographic examinations were performed in 30 children with atrioventricular (A-V) canal defect to determine the diagnostic value of recording A-V valve motion. Although a wide spectrum of abnormal movement was observed, four main diagnostic types emerged. Type 1, prolonged mitral-septal approximation during diastole, resulted from anterior displacement of the cleft anterior mitral leaflet. Type 2, the most diagnostic motion, was recorded when the common anterior A-V leaflet traversed the interventricular septum. Type 3 motion reflected posterior displacement of the septal tricuspid leaflet, which appeared to arise from the interventricular septum. Normal posterior diastolic motion of the septal leaflet was never recorded. Type 4, double contour of the mitral valve during systole and marked duplication of the systolic image, corresponded to irregularities in the mitral valve. When these patterns are recorded in a patient with congenital heart disease the diagnosis of A-V canal defect can be made with confidence.

Adolescent↗

PubMed1975

Acute renal failure: an important complication of cardiac surgery in infants.

Acute renal failure occurred in association with cardiac surgery in 20 of 248 infants (8%). Hypotension, poor tissue perfusion, and hypoglycemia were the most important factors recognized in the pathogenesis and outcome of the ARF. However, many infants were extremely ill preoperatively. The most frequent operative procedures performed in the 20 patients were open-heart surgery with total correction under deep hypothermia and repair of coarctation of the aorta. Thirteen of the 20 infants with ARF died. The combination of a major operative procedure, cardiac failure, hypoglycemia, hypotension, and compromised renal function imposes important constraints in the treatment of hyperkalemia, hypoglycemia, correction of acid-base distrubances, and the administration of fluids.

Acute Kidney Injury↗

PubMed1975

Identification of congenital malformations of the great arteries in infants by real-time two-dimensional echocardiography.

Real-time, two dimensional echocardiography was used to identify great artery relations in 23 infants and small children, including 16 patients with angiography documented transposition of the great arteries, tetralogy of Fallot, or pulmonary aresia. Using this technique, the heart was scanned perpendicular to its long axis at the origin of the great arteries. Great arteries cross-sectioned perpendicular to their long axes appear as circles; when sectioned longitudinally these arteries appeared as elongated sausage-shaped structures. I- patients with normally related great arteries, a curcular structure (aorta) always was positioned posterior to an elongated, sausage-shaped structure (distal right ventricular outflow tract and proximal main pulmonary artery). In transposition of the great arteries, two adjacent circular structures were observed; the anterior circle (aorta) was located to the right, left or directly anterior to the posterior circle (pulmonary artery). In pulmonary atresia or hypoplasis, a large posterior circle (aorta) was associated with an anteriorly positioned structure that was either short and small (atretic right ventricular outflow tract) or elongated with an area of severe narrowing (hypoplastic right ventricular outflow tract). Thus, real-time two-dimensional echocardiography provides a rapid, noninvasive means of accurately identifying congenital malformations of the great arteries in infants and small children and may be a useful adjunct to cardiac catheterization in the diagnosis of cyanotic congenital heart disease.

Child, Preschool↗

PubMed1974