0000000000175222
AUTHOR
Ina Michel-behnke
Microdeletion 22q11 in complex cardiovascular malformations.
Besides DiGeorge, velocardiofacial and conotruncal anomaly face syndromes, some of the isolated congenital heart diseases have also been associated with a chromosomal deletion in 22q11. These disease entities, which had originally been considered to have a different genetic background, are now included in the CATCH-22 microdeletion complex. CATCH 22 is an acronym for cardiac defect, abnormal facies, thymic hypoplasia or aplasia and T-cell deficiency, cleft palate, hypoparathyroidism, and hypocalcemia. In the present study, we focused on the complex cardiovascular defects (CCVD) and screened 40 patients for a microdeletion of 22q11 by fluorescence in situ hybridization using the D22S75 DNA p…
Metabolische Alkalose nach herzchirurgischen Eingriffen im Kindesalter
Transcatheter radiofrequency perforation and stent implantation for palliation of pulmonary atresia in a 3060-g infant
In a 3060-g infant with fibromuscular pulmonary atresia an open right ventricular outflow tract was created by means of interventional cardiological methods. Following two inadvertent perforations without sequelae or clinical symptoms, radiofrequency perforation and subsequent balloon dilatation were successfully performed. The implantation of a Palmaz iliac stent led to a predictable communication between the right ventricle and pulmonary artery.
Mitral and aortic regurgitation in 84 patients with mucopolysaccharidoses
In echocardiographic and necropsy studies nodular thickening of the mitral valve and, less frequently, of the aortic valve has been found in 60%-90% of patients with mucopolysaccharidoses (MPS). Little is known about the haemodynamic consequences of these morphological changes. In this study 84 unselected patients with different enzymatically proven MPS and 84 age and sex matched, healthy persons were studied prospectively by colour Doppler flow mapping. The patients' age ranged from 1 to 47 years (median 8.1 years). Mitral and aortic regurgitation were defined as a holosystolic or holodiastolic jet originating from the valve into the left atrium or the left ventricular outflow tract, respe…
Gradual angioplasty and stent implantation to treat complete superior vena cava occlusion after Mustard procedure
A 16-year-old male was admitted with complete occlusion of the superior vena cava pathway 14 years after Mustard procedure for transposition of the great arteries. From a left subclavian vein approach, the atretic vein segment was perforated using a straight guidewire, and was followed by sequential balloon dilation. Implantation of a 30 mm Palmaz-stent through a femoral vein approach resulted in a widely patent channel of the vena cava superior into the systemic venous atrium. © 1996 Wiley-Liss, Inc.
Analgosedierung mit Fentanyl/Midazolam nach Korrektur angeborener Herzfehler
Abstract There is no standard therapy in the management of postoperative pain control following corrective cardiac surgery of congenital heart disease. Assessment in the preverbal age is difficult. In a randomized study we compared a combined treatment of fentanyl and midazolam, given as continuous infusion versus single dose application. A pain assessment score was used to measure the effectiveness of analgosedation in addition to recording nurseries observations. Fentanyl and midazolam are an appropriate combination for postoperative pain treatment. Continuous application is considered to be more effective concerning basic anxiety, cumulative dosage and to avoid volume overload in infants…