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Biomedical subjects

J A Noonan

Publications and source records attributed to J A Noonan.

15 recordsLinked to original sources
PubMed1991

Changes in left ventricular diastolic filling patterns by Doppler echocardiography in cystic fibrosis.

The onset of cor pulmonale is a common terminal finding in patients with cystic fibrosis. Since Doppler echocardiography can detect changes in diastolic filling patterns prior to the onset of either systolic dysfunction or clinical symptoms, we utilized this technique to determine whether detectable changes in left ventricular diastolic filling patterns exist in patients with cystic fibrosis. Among 25 patients, the proportion of left ventricular filling attributable to atrial contraction was significantly increased when compared with age-matched control individuals. When filling patterns were compared with severity of pulmonary disease, worsening pulmonary disease was directly correlated to shifts in left ventricular filling patterns. We conclude that changes in left ventricular patterns of relaxation are detectable early in the course of cystic fibrosis and that such changes are probably progressive. Early detection could lead to therapeutic trials designed to improve left ventricular filling and delay the onset of overt cor pulmonale.

Adolescent↗
PubMed1987

Down's syndrome.

We discuss the ethical, psychosocial, economic, and medical dimensions of the treatment and management of a child with Down's syndrome and a congenital heart defect.

Down Syndrome↗
PubMed1978

Lactic acidosis associated with cerebellar vermal atrophy and cardiomyopathy.

The association of fluctuating neurological signs and congestive cardiomyopathy with chronic lactic acidosis is described in a 5 1/2 year-old-boy who ultimately succumbed to congestive heart failure. The autopsy findings included severe atrophy of the anterior cerebellar vermis and a hypertrophied heart with left sided endocardial fibroelastosis. Skeletal and cardial muscle calcification was prominent and probably due to the effect of intracellular metabolic alterations associated with lactic acidosis. A review of the literature shows that the combination of cardiomyopathy, isolated atrophy of cerebellar vermis and muscle fiber calcification have not been reported in association with idiopathic lactic acidosis previously.

Acidosis↗
PubMed1977

Association of maternal systemic lupus erythematosus with congenital complete heart block.

Infants born to mothers with disseminated lupus erythematosus occasionally have transient manifestations of the maternal disease. In six infants with congenital heart block born to mothers with systemic lupus erythematosus we postulated a causative relation. In one of the infants a post-mortem study of the conduction system suggested faulty embryonic development of the atrioventricular node with an abnormally thick annulus fibrosus and the effects of early inflammatory changes. Two of the infants had a cardiomyopathy and three, associated congenital heart disease.

Adult↗
PubMed1977

Propranolol therapy during pregnancy, labor, and delivery: evidence for transplacental drug transfer and impaired neonatal drug disposition.

The administration of 160 mg of propranolol during pregnancy, labor, and delivery was associated with profound hypoglycemia and respiratory depression in a newborn infant. The neonate's plasma propranolol level rose from 40 ng/ml at the time of birth to 90 ng/ml four hours later. This increase in plasma propranolol concentration might be due to redistribution of the drug in the neonate as well as to different elimination mechanisms than in adults. The elevated propranolol level four hours after delivery was not associated with any signs or symptoms of drug toxicity, but drug effect was apparent on the electrocardiogram. The administration of propranolol during pregnancy in doses capable of producing therapeutic maternal blood levels may be dangerous to the neonate.

Adult↗
PubMed1976

Total anomalous pulmonary venous return with associated patent ductus arteriosus. Two cases with successful correction.

A large patent ductus arteriosus associated with total anomalous pulmonary venous return results in a unique hemodynamic pattern, and long-term survival has been rare. Two patients, aged five and 18 years, underwent successful surgical repair of supracardiac total anomalous pulmonary venous return and an associated large patent ductus arteriosus. The association of the Noonan syndrome in the 18-year-old woman is of additional interest.

Adolescent↗
PubMed1975

Closed aortic valvotomy and simultaneous correction of associated anomalies in infants.

A small infant with severe congenital aortic stenosis presents a difficult therapeutic problem. Both operative and nonoperative treatment are hazardous--especially when other cardiovascular anomalies are present. This report describes a simple, effective technique for dilating the stenotic aortic valve and simultaneously repairing certain associated defects. The procedure has been used successfully in 3 infants with a postoperative follow-up of 6 months to 4 years. There were no deaths, and the functional results have been excellent. This technique is presented as an alternative to the more hazardous approach to open valvotomy and/or stage correction of associated anomalies.

Abnormalities, Multiple↗