Carney's syndrome: complex myxomas. Report of four cases and review of the literature.

Cardiac myxomas are rare tumors. They usually appear as a sporadic isolated condition in the left atrium of middle-aged women with no other coincidental pathology. Carney and others have described in young people a special complex group of cardiac myxomas associated to a distinctive complex pathology, giving identity to the "Syndrome Myxoma" or "Carney's Syndrome". Four additional cases of this syndrome, treated from 1977 to 1999 at the Hospital Clínico de la Universidad de Chile are presented here with a comprehensive review of the literature, accumulating 100 cases. The main features of our cases include the presence of malignant non cardiac tumors, a familial trend, follow-up of 23 years and an iterative recurrence in the elder case. To date all patients are tumor free. Reviewing the literature, patients with Carney's Syndrome were younger, with a mean age of 26 years and female predominance (62%). Cardiac myxomas affected the four chambers of the heart: 64% the left atrium; 44% the right atrium; 14% the left ventricle and 12% the right ventricle. They were multiple tumors in 41% and involved more than one chamber in 31%, being synchronous or metachronous. There was a marked familial trend (52%), a high incidence of recurrence (20%), with more than one occurring in half the cases. Extra-cardiac involvement consisted of: 68% pigmented skin lesions, 40% cutaneous myxomas, 37% adrenal cortical disease, 27% myxoid mammary fibroadenoma and 34% male patients with testes tumors. A low percentage had pituitary adenoma, melanotic schwannomas and thyroid disease. The diagnosis is made when two or more of these criteria are present. In agreement with these findings the four chambers of the heart should be examined at surgery for atypical myxoma locations, right atriotomy and combined superior-transseptal approach improve exposure of the cavities, careful screening of the first degree family members should be conducted, and closed short and long term follow up controls are important. Complex myxoma appears as a multi-systemic disorder, occasionally having an ominous prognosis and malignant potentiality, and is still undergoing investigation for better understanding and identification.

Producción comparativa de prostaciclina en arteria epigástrica inferior, arteria mamaria interna y vena safena: implicancias en el pronóstico de la revascularización miocárdica / Prostacycline production by the inferior epigastric artery, compared to the internal mammary artery and the saphenous vein

Se usó un sistema in vitro para medir la producción de PG12 en anillos aislados de la arteria mamaria interna, la arteria epigástrica inferior y la vena safena obtenidos en 5 pacientes durante cirugía de revascularización. La muestras fueron incubadas en solución Krebs'Ringer a pH 7.4. Por radioinmuoensayo (Amersham) se midió el 6-keto-PGF la, metabolito de PG12 en alicuotas del sobrenadante. La producción de arterias epigástrica fue 12,5 2,0 ng/mg/30 min, la de arteria mamaria interna 9,3 1,7 y la de vena safena5,1 0,7 (p 0,01). Por lo tanto, la arteria epigástrica inferior produce PG12 en cantidades similares a las de la arteria mamaria interna y considerablemente mayores que las de la vena safena. Ello podría traducirse en un mejor pronóstico de los puentes coronarios efectuados con arteria epigástrica comparados con las de la vena safena

[The wall in persistent ductus arteriosus (author's transl)]. / Uber den Wandbau des persistierenden Ductus arteriosus

A light microscopy study of the wall in ductus arteriosus was performed in 13 cases of isolated persistent ductus. The specimens were obtained surgically in 11 cases and by necropsy in 2. Four patients were male, 9 female. The eldest was a 9-year-old girl and the youngest, a 4-month-old girl. Morphologically, a progressive transformation of the duct wall to the elastic-type artery was observed. In this transformation three stages were determined; stage I: laminar elastosis of the intima; stage II: same as stage I plus incomplete elastic transformation of the media; and stage III: fully developed elastic-type artery. A neat correlation between morphologic stages and clinical data was not found. The course of events in this transformation is apparently determined in great measure by individual factors.