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At least 1,027 records · Page 57Linked to original sources
PubMed1998

Technical considerations for valve repair in patients with congenital heart disease.

Valve repair is a critical element in the surgical treatment of congenital heart disease. Growing children require a longitudinal management strategy not commonly necessary in adult cardiac surgery. The use of valve prostheses poses unique problems in children, not only due to size limitations, but also to difficulties with chronic medical management. For these reasons, considerable effort is given to repairing even severely malformed valves in pediatric patients. This article provides a brief overview of some commonly used techniques in reparative valve surgery in children.

Aortic Valve Insufficiency↗
PubMed1998

Noonan syndrome associated with central giant cell granuloma.

We report a case of Noonan syndrome associated with central giant cell granuloma. The patient was a 10 1/2-year-old boy with the chief complaint of proptosis of the right eye. He also had various malformations such as short stature, webbed neck, pectus excavatum, cubitus valgus, pulmonary valve stenosis and patent foramen ovale, a characteristic face appearance and cryptorchidism and so on. Chromosome analysis showed a 46,XY karyotype. A computed tomographic scan and magnetic resonance imaging showed a mass originated from the lateral wall of the right maxillary sinus. The patient underwent Caldwell-Luc operation. Histological examination of the mass showed the characteristics of central giant cell granuloma. This case report describes a patient with the features of the recently described Noonan-like/multiple giant cell lesion syndrome.

Child↗
PubMed2004

Atrial standstill with subnodal conduction defect and diffuse ventricular fibrosis managed with low right atrial pacing.

In patients with congenital heart disease presenting with bradyarrhythmia, many electrophysiological abnormalities are found. This report presents a case of a man with surgically corrected single atrium and pulmonary valve stenosis, atrial standstill, delayed AV node conduction, first-degree intra- and second-degree infra-His block, who was permanently paced from a restricted excitable area of the low right atrium, as the pacing threshold was unacceptable at any ventricular site.

Arrhythmias, Cardiac↗
PubMed1993

Noonan's and DiGeorge syndromes with monosomy 22q11.

A boy with the dysmorphic features of Noonan's syndrome and pulmonary valve stenosis who had evidence of hypoparathyroidism and abnormal T lymphocyte numbers in the neonatal period is reported. He had a normal karyotype but molecular analysis revealed a submicroscopic deletion within chromosome 22q11, the region deleted in DiGeorge syndrome. Thus this child has both Noonan's syndrome and DiGeorge syndrome; 22q11 is a candidate region for a gene defective in Noonan's syndrome.

Blotting, Southern↗
PubMed1980

Congenital hepatic fibrosis with congenital heart disease. A family study with ultrastructural features of the liver.

A family with congenital hepatic fibrosis (CHF) and congenital heart disease (CHD) is presented. The consanguineous healthy parents gave birth to 12 children of whom 10 survived. One son had CHF and CHD, one daughter had CHF and a second daughter had CHD. Three other siblings probably had small a ventricular septal defect and another one probably had mild pulmonary valve stenosis. Development of portal hypertension and hypersplenism necessitated performing shunt operation on both siblings suffering from congenital hepatic fibrosis. Ultrastructural findings were giant mitochondria with large laminar inclusions in hepatocytes, and excess of villi and whorls of membranes and collagen fibrils between hepatocytes.

Adolescent↗
PubMed1991

Fatal pneumococcal septicaemia associated with asplenia and isomerism of the right atrial appendages.

A 22 month old male infant with isomerism of the right atrial appendages (associated with a complete atrioventricular septal defect, double outlet right ventricle, and pulmonary valve stenosis) died from pneumococcal septicaemia after a 36 hour illness. He had not been given penicillin prophylaxis. Asplenia was confirmed at necropsy. In a survey of 50 paediatric cardiologists in the United Kingdom, 13 (33%) of the 40 (80%) who replied did not advise any prophylactic measures against pneumococcal infection in patients with isomerism of the right atrial appendages.

Heart Atria↗
PubMed1997

Multiple lentigines syndrome (LEOPARD syndrome or progressive cardiomyopathic lentiginosis).

The multiple lentigines syndrome is an autosomal dominant condition which has many similarities to Noonan syndrome, except in the most striking feature from which its name is derived. The less neutral but very apt mnemonic, LEOPARD syndrome, was first used by Gorlin et al to whom the major debt in the definition of this syndrome lies, that is, Lentigines, ECG abnormalities, Ocular hypertelorism/Obstructive cardiomyopathy, Pulmonary valve stenosis, Abnormalities of genitalia in males, Retardation of growth, and Deafness. Not previously included in the mnemonic is cardiomyopathy which is an important feature because it is associated with significant mortality.

Abnormalities, Multiple↗