Entwicklung der Fontan-Prozedur als Behandlungskonzept für Kinder mit angeborenen komplexen Herzfehlern

The spectrum of congenital heart defects reaches from very simple to extremely complex anomalies. 3 decades earlier, the prognosis of children with univentricular heart defects was poor. The Fontan procedure has been offered to patients with a variety of complex cyanotic heart diseases and has resulted in decreased mortality. This review summarizes the development and modifications of the Fontan procedure. The advances in preoperative, intraoperative und postoperative patient management have resulted in greatly improved survivals for even the most complex congenital defects. Over the past 3 decades, the survival of infants with critical congenital heart defects has increased to 90%.     

Distal greater trochanteric transfer in the treatment of deformity of the proximal femur caused by avascular necrosis

Background. Distal greater trochanteric transfer is one of the surgical methods used to correct proximal femoral deformity arising in the course of treatment for developmental dysplasia of the hip. Material and methods. We reviewed a series of 49 patients (55 hips) who had undergone distal greater trochanteric transfer at the mean age of 13.9 years due to deformity of the proximal femur after treatment for developmental dysplasia of the hip, in order to verify the value of the procedure. The mean follow-up was 15 years. Clinical and radiological assessment was supplemented with strain-gauging evaluation of the muscles involved. Results. Good results were achieved in those patients who had good range of movement or isolated restriction of abduction before the operation. After surgery, a 22% increase of abductor torque was found, the Trendelenburg sign disappeared in 30 individuals, and 15 patients regained normal gait. Conclusions. Distal greater trochanteric transfer improved hip joint biomechanics. Good abduction/adduction range of hip movement was essential for clinical improvement and increased muscle strength after surgery. Arthritic changes occurred primarily in those hip joints without clinical improvement. Distal transfer of the greater trochanter delayed osteoarthritis of the hip.     

Apelin: a novel marker for the patients with first ST-elevation myocardial infarction

To date, only animal studies have been concerned with apelin involvement in acute myocardial ischemia. The aim of this study was to investigate apelin measurements in low-risk patients with first ST-elevation myocardial infarction (STEMI) and to assess if apelin may feature as a marker of left ventricular (LV) injury and prognosis. In 78 consecutive patients (mean age 67 ± 11.5 years, 24 women) with first STEMI treated with primary percutaneous coronary intervention, plasma apelin-36 concentrations were measured twice: on admission and on the 5th day of hospitalization. Left ventricle ejection fraction (LVEF) was applied as marker of LV injury. Composite endpoint (CEP), which included death, stroke, and recurrent ischemic event, was assessed after 1 year follow-up. On the first day, median apelin-36 concentration was 2138.5 pg/ml and on the 5th day was significantly lower, 2008.3 pg/ml (P = 0.002). There were no significant differences found in apelin-36 concentrations between patients with normal and low LVEF. In both groups significant reductions were found in apelin-36 concentrations measured in 5-day intervals (P = 0.04 and P = 0.008, respectively). After a 1-year follow-up, only one patient died and 19 patients (24.3%) had reached CEP. No difference in baseline apelin-36 concentrations were found in the group of patients who reached CEP compared with those without CEP. However, in both groups concentrations significantly decreased after 5 days (P = 0.04 and P = 0.013, respectively). Apelin-36 concentrations are reduced in lowrisk first STEMI patients during the first days regardless of the degree of LV dysfunction and prognosis.     

Surgical treatment of patients with initially inoperable and/or metastatic gastrointestinal stromal tumors (GIST) during therapy with imatinib mesylate

BACKGROUND: The aim of the study was to analyze the surgical possibilities of unresectable and/or metastatic GIST CD117(+) patients during imatinib treatment. METHODS: We analyzed the results of surgery in 141 patients treated with imatinib for initially inoperable and/or metastatic GIST CD117(+). Median follow-up time was 12 months (range: 3-26). RESULTS: Surgery was performed as subsequent treatment in 24 patients (Group I, 17%) for resection of residual disease after complete/partial response and lack of further response to imatinib and as salvage therapy in eight patients (Group II, 6%), who progressed on initially successful imatinib therapy. In Group I, the viable GIST cells were not detected histologically in only three resection specimens. The first five patients in Group I did not receive imatinib further and we observed four recurrences. In next 19 patients, continuing imatinib after surgery, we observed only one relapse. In Group II, we continued imatinib therapy after high-risk surgical procedures, but in five patients we observed subsequent progression. CONCLUSIONS: Surgical removal of residual disease during imatinib treatment may allow for complete remission in selected GIST patients after response to therapy, theoretically prolonging durable remission, but it is necessary to continue imatinib for its maintenance.     

Free interferon-alpha/beta receptors in the circulation of patients with adenocarcinoma

BACKGROUND: Many viral and neoplastic diseases are resistant to interferon-alpha/beta (IFN-alpha/beta) therapy or develop resistance during the course of IFN treatment. In patients with viral diseases, the authors identified four IFN inhibitors, of which the most important, most likely is a free IFN receptor of type 1 appearing in the circulation that captures and neutralizes IFN-alpha/beta. METHODS: Ninety-one cancer patients and 25 healthy individuals were studied. Free circulating IFN receptor-alpha/beta type 1 was studied. The patients were ages 35-75 years. The diagnoses were 24 cases of colon carcinoma, 7 cases of prostate carcinoma, 16 cases of breast carcinoma, 8 cases of ovarian carcinoma, 9 cases of uterine carcinoma, 5 cases of lung carcinoma, 3 cases of astrocytoma, 4 cases of transitional cell carcinoma of the bladder, 1 case of osteosarcoma, 3 cases of multiple myeloma, 4 cases of Hodgkin disease, 2 cases of non-Hodgkin lymphoma, 3 cases of myelodysplastic syndrome, and 2 disseminated tumors of unknown origin. RESULTS: All patients were found to have increased free IFN receptor-alpha/beta type 1 in the circulation, with the highest levels reported in patients with adenocarcinoma. CONCLUSIONS: High IFN inhibitory activity in patients with cancer may be a significant factor in their increased susceptibility to progressive disease, infectious complications, and resistance to IFN therapy. Ongoing studies are being performed with the objective of overcoming this inhibitory activity.     

Aortic valve papillary fibroelastoma

Cardiac papillary fibroelastoma (CPF) is a rare primary benign cardiac tumor. Before the use of echocardiography, the lesion was identified at autopsy or incidentally during cardiac surgery. CPF is the third most common primary cardiac tumor after atrial myxoma and lipoma, and is the most common tumor of the valvular endothelium. Transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) permit diagnosis of the tumor in living patients. CPF may be the cause of cerebrovascular or cardiac ischemia due to embolization or occlusion of the vascular ostia. Embolic material may arise from fragments of the tumor itself, or from surrounding thrombus. The case is reported of a patient with CPF of the aortic valve in whom TEE diagnosis was conducted and the tumor removed surgically.