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Biomedical subjects

C M McCue

Publications and source records attributed to C M McCue.

At least 19 recordsLinked to original sources

Pulmonary arteriovenous malformations related to Rendu-Osler-Weber syndrome.

Interest in Rendu-Osler-Weber (ROW) syndrome has been renewed because of new treatment for the pulmonary artery fistulae that occur in approximately one-half of the patients. Pulmonary arteriovenous malformations (AVM) can be occluded safely by the transvenous placement of a silicone balloon thus avoiding the many potential complications of thoracotomy. Thirty-three members of four generations of a family with ROW illustrate the varied manifestations of the syndrome and provide a basis for review of clinical findings and therapeutic approaches to the management of pulmonary AVMs during the last 25 years. Special attention is given to early detection and prevention of major complications. Since ROW syndrome is an inherited trait, informed genetic counseling is an important component of the overall management of families with this disorder.

Adolescent

Adolescent blood pressure in Richmond, Virginia, schools.

The blood pressure of 3,166 adolescents was measured periodically in Richmond during the period 1974-1977. The subjects were students in public and private schools and ranged in age from 14 to 18 years. Analysis of the readings showed minimal differences relating to age, sex, school and race but a strong correlation with obesity. Only two organic renal causes of hypertension were discovered. A 3 1/2-year followup of 902 students indicated significant tracking, with a correlation coefficient of .385 for systolic and .305 for diastolic pressure. At the conclusion of the study, 340 students (10.7%) were identified as needing followup for hypertension.

Adolescent

Lack of connection between the atria and the peripheral conduction system in a case of corrected transposition with congenital atrioventricular block.

This is the first documented histologic study of the heart of a patient with corrected transposition of the great vessels and congenital atrioventricular (A-V) block with no connection between the atria and an anterior type of peripheral conduction system. Musculature in the superior (anterior) walls of both atria was absent, as was the anterior A-V node. The peripheral conduction system began with the bundle of His. In place of the absent atrial musculature, fibrosis and calcification were present. The relation of laboratory evidence of connective tissue dyscrasia in the mother to the congenital A-V block in the child is discussed.

Adult

Successful intracardiac repair of the Taussig-Bing malformation in 2 children.

Surgical repair of the Taussig-Bing deformity has been successful in 2 children weighting 7.7 and 11.1 kg, respectively. In the first case the right ventricular patch divered left ventricular blood to the pulmonary arter, thus creating a physiologically complete transposition which was then corrected by an interatrial Mustard baffle. In the second case a right ventricular tunnel diverted left ventricular blood to the aorta, and right ventricular blood was directed to the distal pulmonary artery by means of an external conduit bypassing a subpulmonic stenosis. These patients represent the seventh and eighth successful corrections of the Taussig-Bing anomaly. One case (Patient 2) is the youngest to undergo repair and the first to receive an external conduit for bypass of an associated subpulmonic stenosis.

Age Factors

Congenital heart block in newborns of mothers with connective tissue disease.

Of 22 children with congenital complete heart block (CCHB) available for study, 14 (63.6%) were born to 11 mothers with clinical or laboratory evidence of connective tissue disease, primarily lupus erythematosus (LE). Seven mothers had both clinical and laboratory evidence of disease while four had only positive laboratory studies including fluorescent antinuclear antibody, rheumatoid factor, and depressed complement levels. In adults with systemic LE, pathologic changes in the collagen surrounding the conduction system have led to the fibrosis and death from heart block. Antinuclear antibodies of the IgG class cross the placental barrier and newborn infants have been reported with transient skin lesions of lupus. Placental transmission of such antibodies may affect the fetal cardiac conduction system, surrounding collagen, and myocardium, leading in some cases to CCHB. This is probably one important etiologic factor in CCHB even though the mother is asymptomatic during her pregnancy.

Antibodies, Antinuclear

Rhabdomyoma of the heart: ultrastructural study of three cases.

The electron microscopic features of three primary cardiac rhabdomyomas were studied for the first time. Distinct striated muscle cells arranged in a disorderly fashion are described. The cases demonstrated a spectrum of possible myobibril differentiation with one case having only Z-bands and myosin filaments present while the other two had well formed thick and thin filaments with all of the bands present including Z, I, A, H, and M. Glycogen bodies, numerous mitochondria with tubular cristae, lipid deposits, and peculiar striated structures resembling zebra bodies were also seen. Desmonsomal attachments suggestive of Purkinje-type fibers were seen in all of the cases and were the only types seen in cases 2 and 3. In addition, intercalated discs characteristic of myocardial fibers were noted in case 1. This finding still leaves the exact histogenesis of the lesion uncertain although origin from two types of fibers is possible.

Heart Neoplasms

Correction of platelet dysfunction and bleeding in cyanotic congenital heart disease by simple red cell volume reduction.

Red cell volume reduction corrected the platelet aggregation abnormality and bleeding tendency in four boys, aged 5 to 16 years, with severe cyanotic congenital heart disease and polycythemia. Red cell volume was reduced by replacing 15 to 20 ml/kg body weight of the patient's blood with plasma in 50 ml increments over a 1 to 2 hour period. Within 3 days, platelet aggregation was restored essentially to normal, and previous bleeding symptoms disappeared. Platelet aggregation remained normal during 3 weeks of follow-up study and hematocrit values remained at palliative levels. The procedure was safe and simple, and it had beneficial effects not only on bleeding but also on other symptoms related to polycythemia. These preliminary observations suggest that red cell volume reduction may be useful preoperatively in patients with cyanotic heart disease and defects in platelet function to lessen the risk of serious bleeding during the early postoperative period. Palliation in severely cyanotic children whose condition is inoperable is another possible indication for this procedure.

Adolescent

Associated coarctation of the aorta and mitral valve disease: nine cases with surgical correction of both lesions in three.

Nine cases of the combination of coarctation of the aorta and mitral stenosis were evaluated over a seven-year period. Symptoms did not usually cause distress in infancy, but began subtly with pneumonia or cardiac failure at about 2 years of age. Important clues were differences in blood pressure between the arms and legs, paroxysmal dyspnea, congestive heart failure, right ventricular hypertrophy, and left atrial enlargement. Cardiac catheterization studies showed elevated right ventricular and main pulmonary artery wedge pressures. These features in patients with coarctation of the aorta should suggest associated mitral valve disease. The importance of demonstrating associated valvular lesions, particularly mitral stenosis, is emphasized. Two of our children had successful repair of the coarctation of the aorta and mitral stenosis simultaneously. In a third child, resection of the coarctation was followed in six years by mitral valve replacement.

Aortic Coarctation