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Biomedical subjects

D Z Friedberg

Publications and source records attributed to D Z Friedberg.

At least 19 recordsLinked to original sources

Aortic outflow obstruction in visceral heterotaxy: a study based on twenty postmortem cases.

Aortic outflow tract obstruction can complicate the clinical course and surgical management of patients with heterotaxy syndromes, but its anatomic basis has not been described in detail. In 20 postmortem cases with asplenia (n = 4) or polysplenia (n = 16), the anatomic causes of aortic outflow tract obstruction were absence of the subaortic conus in association with (1) narrowing of the subaortic outflow tract between the conal septum anteriorly and the common atrioventricular (AV) valve posteriorly in six (30%) patients; (2) aortic valvar atresia in four (25%), three with asplenia and one with polysplenia; (3) redundant AV valve leaflets in four (20%); (4) excessive AV valve fibrous tissue in four (20%); (5) marked hypoplasia of the mitral valve and left ventricle in two (10%); and (6) aneurysm of membranous septum in one (5%). One patient belonged to group (1) and (4). Aortic outflow tract obstruction was much more common with polysplenia (28%) than with asplenia (4%) (p < 0.001).

Abnormalities, Multiple

Twenty-year experience with repair of complete atrioventricular septal defects.

BACKGROUND: To determine factors predicting mortality and morbidity after repair of complete atrioventricular septal defect, we retrospectively analyzed preoperative, operative, and postrepair factors on the outcome of 115 consecutive complete atrioventricular septal defect repairs at The Children's Hospital of Wisconsin between January 1974 and December 1993. METHODS: For the entire experience the operative mortality was 13.9% (16 patients). During the most recent era, January 1988 to December 1993, operative mortality was 3.6% (2 of 55 patients). This was significantly improved from the two previous eras, January 1974 to December 1980, 28% (7 of 25) and January 1981 to December 1987, 20% (7 of 35 patients) (p = 0.02). There were seven late deaths; 10-year actuarial survival, including operative mortality was 81%. Age at complete repair decreased; before 1982 all patients were more than 12 months of age, whereas after 1982 64% (56 of 88 patients) were 12 months of age or less. RESULTS: Moderate or severe preoperative left atrioventricular valve regurgitation was not a risk factor for operative mortality. For operative survivors with moderate to severe preoperative left atrioventricular valve regurgitation (n = 17), late postoperative left atrioventricular valve regurgitation (follow-up data available on 15 patients) was significantly reduced (severe = 1, moderate = 5, mild = 9; p = 0.007). CONCLUSIONS: Early mortality was predicted by the era of surgical repair. Conversion to routine repair during infancy was achieved with a simultaneous decrease in operative mortality. For patients with moderate to severe preoperative left atrioventricular valve regurgitation, significant improvement in the degree of left atrioventricular valve regurgitation can be expected without an increase in operative or late mortality or morbidity.

Actuarial Analysis

Percutaneous balloon pulmonary valvuloplasty in children.

This report describes the results and follow-up of 27 patients undergoing pulmonary balloon valvuloplasty at Children's Hospital of Wisconsin where this procedure has been performed since 1985. The valvuloplasty data at catheterization as well as follow-up echocardiograms when available were retrospectively reviewed. For 26 patients, the transvalvar gradient changed from a mean of 72 mm Hg + 5.2 (SEM) to 29 mm Hg + 4.4 (SEM) (p 0.00001) a mean gradient reduction of 58%. No major complications were reported and no patient went on to surgical valvotomy. Follow up echocardiograms were available for 26 patients. Average estimated transvalvar pressure gradients by continuous wave Doppler echocardiography were 28 mm Hg at one day (13 patients), 20 mm Hg at mean 1.5 months (18 patients), 23 mm Hg at mean 8.8 months (12 patients), and 19 mm Hg at mean 26 months (8 patients). Percutaneous balloon valvuloplasty gives good results in nearly all patients with pulmonary valve stenosis.

Adolescent

Pulmonary artery plication: a new surgical procedure for small infants with type I truncus arteriosus.

A new technique is reported for constriction of the pulmonary artery ostium as a palliative procedure in infants with type I truncus arteriosus. Pulmonary artery plication was performed without cardiopulmonary bypass, and this allowed intraoperative evaluation of the procedure. The lungs were protected from developing pulmonary vascular obstructive disease, and congestive heart failure resolved. The pulmonary arteries and the ostium grew with the child so that corrective surgery could be delayed until 4 1/2 years of age, when a large (20 mm) valved extracardiac conduit could be placed. We believe this is a useful palliative operation, particularly for small, sick infants, and it deserves further evaluation.

Heart Failure

Cor triatriatum.

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Heart Atria

Syncope with prolonged QT interval.

Four children with syncope had a prolonged QT interval on the electrocardiogram. Neurologic studies were negative. One patient had associated deaf mutism, one had a family history of sudden death and prolonged QT interval, and two had ventricular arrhythmias while being monitored in the hospital. Treatment with propranolol hydrochloride eliminated the syncope in all patients, although the ECGs remained abnormal.

Adolescent

The medical and surgical management of patients with congenital heart disease. A survey of current knowledge and practices.

Today, techniques for diagnosis,medical preparation, and definitive surgical repair are available for most cardiac anomalies, if needed. These permit successful treatment and carry a low mortality. Such children can now be expected to lead normal and meaningful lives as contributors to society. When serious symptoms occur in infancy, as discussed herein, an emergency exists and prompt diagnosis and treatment are mandatory for survival.

Age Factors