Balloon-expandable stents for recoarctation of the aorta in small children. Two centre experience.

BACKGROUND: In young patients with native aortic coarctation (CoA), the management of choice is surgery. However, in re-coarctation (re-CoA) surgery is associated with increased morbidity and even mortality. Some children with native CoA present relative contraindications for surgery. METHODS: From 2006 to 2017, thirty-four patients (male n = 20; 59%) from two centres with re-CoA (31) and native CoA (3) were managed by stent implantation with premounted balloon expandable stents. Inclusion criteria were age < 3 years and >1 month, weight < 16 kg. Median age was 6,5 months (min. 1; max. 34 months), median weight 6,2 kg (min. 3,7; max. 16 kg). Thirteen patients (38%) had Re-CoA and hypoplastic left heart syndrome (HLHS). In three patients (9%) the native CoA was stented due to contraindications for surgical treatment. RESULTS: All procedures were successful. The median peak invasive systolic pressure gradient declined from 31 mm Hg (max. 118; min. 4) to 0 mm Hg (max. 32; min.-7) (p < 0.001). The median minimal diameter of the narrowed segment of aorta increased from 3 mm (max. 6,9; min. 1,0) to 7 mm (max. 11,5; min. 3,5) (p < 0.001). There were no serious complications. The median follow-up time was 12,5 months (max. 88; min. 0 month). During this time ten patients (29%) required re-dilatation and two of them re-stenting. CONCLUSION: Percutaneous stent implantation for Re-CoA and in selected patients for native CoA can be performed successfully in very young patients with a good immediate hemodynamical result. However, repeated stent angioplasties and further on interventional 'opening' of the stent is necessary to augment the aorta to adult size.

Site of coronary sinus drainage does not significantly affect coronary flow reserve in patients long term after Fontan operation.

This study was designed to investigate the impact of postoperative coronary sinus drainage pressure on coronary flow reserve (CFR) assessed by Doppler guidewire in patients long term after Fontan operation. Twenty-nine patients (median age, 17.4 years female, 11) at a median of 10.6 years after Fontan operation were examined with intracoronary Doppler guidewire during cardiac catheterization. Fourteen patients had coronary sinus (CS) drainage to the systemic venous atrium and 15 patients had CS drainage to the pulmonary venous atrium after Fontan operation. Median CS drainage pressure was significantly higher in systemic venous CS drainage compared to pulmonary venous CS drainage (11 vs 5 mmHg, p < 0.0001). Median CFR values for the right and left coronary artery did not differ significantly with respect to CS drainage. There was a positive correlation between coronary flow reserve and pulmonary arteriolar resistance (p < 0.05) in multivariate regression analysis. The site of coronary drainage into the systemic atrium or the pulmonary venous atrium did not significantly affect CFR. Our data do not support a surgical strategy of elective redirection of coronary sinus blood to a low-pressure compartment but support an early staged approach. The positive correlation between CFR and pulmonary resistance demands further evaluation.

Stenting of a stenosed sano shunt in a neonate with hypoplastic left heart syndrome.

We report on successful stenting of a proximally stenosed Sano shunt in a newborn with hypoplastic left heart syndrome after a stage I Norwood operation.

Impaired left ventricular function after arterial switch operation: exclusion of significant coronary artery stenosis with an intravascular Doppler guidewire.

Patients after arterial switch operation for transposition of the great arteries are at risk for coronary artery stenosis or obstruction due to intraoperative manipulation. This case describes an infant with impaired left ventricular function 8 months after arterial switch operation. A hemodynamically significant left coronary artery stenosis was excluded by determination of coronary flow reserve using an intravascular Doppler guidewire.

Cardiac output determination in children: equivalence of the transpulmonary thermodilution method to the direct Fick principle.

OBJECTIVE: To show the equivalence of the transpulmonary thermodilution method to the direct Fick principle in children. DESIGN: Prospective single-centre study. SETTING: A 16-bed paediatric cardiac ICU and a cardiac catheterisation laboratory at an university affiliated centre for paediatric cardiology and congenital heart disease. PATIENTS: We consecutively investigated 18 patients (mean age 12.1 +/- 6.4 years) during cardiac catheterisation and after corrective cardiac operation. METHODS AND RESULTS: We prospectively defined limits of equivalence for cardiac index (CI) for both methods of +/- 0.25 l/min x m(2). We measured oxygen consumption for determination of CI by Fick as the clinical "gold standard" and performed a set of three transpulmonary thermodilution measurements. The mean CI(Fick) was 2.88 +/- 1.07 l/min x m(2) (range 1.10-4.62 l/min x m(2)) and CI(TPID)was 2.85 +/- 1.03 l/min x m(2)(range 1.02-4.49 l/min x m(2)). The mean difference between CI(Fick) and CI(TPID)was 0.030 +/- 0.168 l/min x m(2), and limits of agreement -0.306 to 0.366 l/min x m(2)(90% confidence interval -0.040 to 0.099 l/min x m(2)). The regression equation was : CI(Fick)=1.0244 x CI(TPID)-0.040, r(2) = 0.976, P < 0.0001. The intraclass coefficient of reliability for three repeated measurements of CI(TPID) was 0.97, the corresponding lower limit of the 95% confidence interval was 0.94. CONCLUSION: We demonstrated the equivalence of CI measurement by transpulmonary thermodilution and the Fick principle in children. This new method may improve hemodynamic monitoring and management in seriously ill children.

Mass output and particle size distribution of glucocorticosteroids emitted from different inhalation devices depending on various inspiratory parameters.

Efficient inhalation therapy depends on successful delivery of the drug to the lung. The efficacy of drug delivery is not only influenced by the characteristics of the inhalation device, but also by the patient's handling of the device and by the inspiratory maneuver achieved through the device. We analyzed the output characteristics of three different chlorofluorocarbon (CFC)-free breath-actuated inhalers for inhaled glucocorticosteroids (BUD Turbohaler, FP Diskus/Accuhaler and HFA-BDP Autohaler, respectively). Mass output and particle size distribution of drug aerosol delivered by the inhalers were determined depending on different inhalation parameters in vitro using an Andersen cascade impactor. We found that, beside the peak inspiratory flow (PIF), other factors such as flow acceleration and inhalation volume also have significant effects on aerosol generation with respect to mass output and particle size distribution. Thus, these parameters should be taken into account when a suitable device for an individual patient is to be selected. The dependency on inspiratory parameters was most pronounced for the dry powder inhalers. The Turbohaler showed by far the highest variance in particle output (fine particle fraction ranging from 3.4% to 22.1% of label claim), whereas the Diskus was less dependent on variations in inhalation (10.6% to 18.5% of label claim). The most constant aerosol output was found for the Autohaler, which also released the highest fine particle fraction (43.1% to 56.6% of label claim).

Matched pair analysis of survival after chest-wall recurrence compared to mammary recurrence: a long-term follow up.

BACKGROUND: Local recurrence remains a major concern after primary treatment of breast cancer and has a major impact on subsequent survival. While most studies report a poorer survival rate in patients with a local recurrence after mastectomy than after breast conservation, it remains controversial whether different risk profiles at the time of primary diagnosis may account for this difference. METHOD: Matched pair analysis of 134 patients with newly diagnosed locoregional recurrence of breast cancer without evidence of systemic disease. Matching criteria included the primary surgical treatment, tumor size, nodal status, and age. The significance of various prognostic parameters at the time of primary diagnosis and at the time of recurrence were evaluated, by univariate and multivariate analyses, with respect to survival after recurrence. The median follow-up was 8.4 years. RESULTS: Risk factors at the time of presentation, such as tumor size and lymph node status, were comparable between both groups. Local recurrence occurred on an average 9 months earlier in patients after mastectomy (P = 0.08). Univariate analysis showed that lymph node status (P = 0.0001) and disease-free interval from primary treatment to local recurrence (P = 0.0002) were the most significant single prognostic factors for subsequent survival after local recurrence. The primary surgical treatment modality was shown to be of marginal statistical influence (only P = 0.05). CONCLUSION: Local recurrence after mastectomy seems to be associated with worse survival than after breast-conserving therapy. Early onset of chest-wall recurrence, moreover, represents the highest independent risk for cancer-associated death.

Balloon dilation for aortic recoarctation: morphology at the site of dilation and long-term efficacy.

OBJECTIVES: We undertook this study to assess the immediate and long-term outcome of balloon angioplasty performed for recurrent or residual coarctation of the aorta, and to assess the changes in the vessel wall caused by this procedure. METHODS: Clinical, echocardiographic, angiographic and hemodynamic data from 71 patients who underwent balloon angioplasty for recoarctation between January 1987 and January 1998 were analysed retrospectively. RESULTS: Angioplasty was performed after a median of 82.6 months (range 1.4 mo-20.9 y, mean 88.5 mo) following surgery for coarctation. Mean systolic pressure gradients were reduced from 27 +/- 15 mmHg to 11 +/- 11 mmHg after angioplasty (p < 0.0001). The mean diameter at the site of recoarctation increased from 5.5 +/- 2.5 to 7.5 +/- 2.7 mm (p < 0.0001). Outpouchings of contrast agents, indicating the disruption of the inner layers of the vessel wall, were defined as extravasations. They were observed in one-quarter of the angiograms performed immediately after the intervention. Immediate success of angioplasty was achieved in 71%, and persisted in 69% of patients during long-term follow up. The main determinant for immediate success was the age at the time of the procedure (p < 0.05), while the main determinant for long-term success was the increase achieved in diameter. Extravasations did not progress to aneurysms, neither acutely nor during echocardiographic follow-up studies. For further follow-up, more sensitive imaging techniques will be necessary to delineate the morphology of the site of extravasation observed immediately after angioplasty.

Radiotherapy of the chest wall following mastectomy for early-stage breast cancer: impact on local recurrence and overall survival.

INTRODUCTION: Recent studies have renewed an old controversy about the efficacy of adjuvant radiotherapy following mastectomy for breast cancer. Radiotherapy is usually recommended for advanced disease, but whether or not to use it in pT1-T2 pN0 situations is still being debated. This study was designed to clarify whether or not routine radiotherapy of the chest wall following mastectomy reduces the risk of local recurrence and if it influences the overall survival rate. METHODS: Retrospective analysis of patients treated with mastectomy for pT1-T2 pN0 tumors and no systemic treatment. Patients treated with radiotherapy of the chest wall following mastectomy (Group A) are compared with those treated with mastectomy alone (Group B). RESULTS: A total of 918 patients underwent mastectomy. Patients who received adjuvant radiotherapy after mastectomy (n = 114) had a significantly lower risk for local recurrence. Ten years after the primary diagnosis, 98.1% of the patients with radiotherapy were disease free compared to 86.4% of the patients without radiotherapy. The average time interval from primary diagnosis until local recurrence was 8.9 years in Group A and 2.8 years in Group B. The Cox regression analysis including radiotherapy, tumor size and tumor grading found the highest risk for local recurrence for patients without radiotherapy (p < 0.0004). In terms of overall survival however, the Kaplan-Meier analysis showed no difference between the two groups (p = 0.8787) and the Cox regression analysis failed to show any impact on overall survival. CONCLUSION: With observation spanning over 35 years, this study shows that adjuvant radiotherapy of the chest wall following mastectomy reduces the risk for local recurrence in node-negative patients with pT1-T2 tumors but has no impact on the overall survival rate.

[Results of adjuvant chemotherapy of operated high risk cervix carcinoma]. / Ergebnisse der adjuvanten Chemotherapie des operierten High-Risk-Zervixkarzinoms.

Patients with carcinoma of the cervix and histologically proven blood vessel invasion have a poor prognosis. At the I. Frauenklinik der Universität München, 34 women were enrolled for a prospective study and were alternatively treated with or without adjuvant chemotherapy (carboplatin and 5-fluorouracil). Of the 28 patients included in the follow-up (14 with/14 without chemotherapy), 12 patients died (5 with/7 without chemotherapy). The disease-free interval was 18.6 months in the group treated with chemotherapy compared to 29.3 months in the group without chemotherapy. There is no statistically significant difference in the survival of patients with blood vessel invasion in relationship to adjuvant chemotherapy following radical hysterectomy.

[Isovolemic hemodilution for avoiding homologous blood transfusions: effectiveness in large gynecologic interventions]. / Isovolämische Hämodilution (IHD) zur Einsparung von Fremdbluttransfusionen: Effektivität bei grossen gynäkologischen Eingriffen.

OBJECTIVE: The practicability and efficiency of a standardized, preoperative isovolemic hemodilution was investigated during major gynecological operations (Wertheim's operation, etc.). DESIGN: Prospective clinical trial with a historical control group. SETTING: Operating room of a gynecological university hospital. PATIENTS AND INTERVENTIONS: Under general anesthesia hemodilution to a hemoglobin concentration of 9 g/dl was performed in 48 patients (mean age: 53 years). Shed blood volume amounted to 900 +/- 210 ml. Transfusion of autologous or homologous blood was provided when Hb concentration decreased beyond 7 g/dl intraoperatively. RESULTS: Compared to a control group of 57 patients without hemodilution the total number of PRBC units transfused was significantly reduced. Moreover, in 65% of all patients the transfusion of homologous blood could completely be avoided perioperatively (control group: 21% of patients). Adverse effects did not occur. CONCLUSIONS: The data reflect that acute isovolemic hemodilution before major gynecological operations represents a safe, easy to handle and effective procedure to avoid transfusion of homologous blood up to a total blood loss of 1,300-1,400 ml.

[Carcinoma in situ of the breast--is axillary lymphadenectomy necessary?]. / Carcinoma in situ der Mamma--ist eine axilläre Lymphadenektomie notwendig?

Between 1963 and 1993, 3720 women were treated both at the Universitäts-Frauenklinik Berlin-Charlottenburg and at the I. Frauenklinik der Universität München for a malignant condition of the breast. 187 of these (5%) exhibited stage p Tis, 102 (54.5%) of whom had an axillary lymphadenectomy. One patient had a micrometastasis (pN1a). During the observation period of up to 24 years, 11 patients (5.9%) developed local recurrence. In none of the patients a regional or generalised recurrence could be observed within this period. On the basis of our own results and those from the literature, we conclude that, under the aspect of a risk-adapted tumour surgery, axillary lymphadenectomy is no longer necessary under certain conditions: an invasive carcinoma should be excluded with high certainty in the tumourectomy specimen as well as in the remaining breast. Therefore, a histological work-up in serial sections must be provided to exclude multifocality (multicentricity) and a tumour diameter larger than 25 mm.

Intraductal carcinoma (DCIS) of the breast. Risk-adapted tumor surgery with axillary lymphadenectomy?

Between 1963 and April 1994, 3823 women were treated both at the Universitäts-Frauenklinik Berlin-Charlottenburg as well as at the I. Frauenklinik der Universität München, for a malignant condition of the breast gland. 161 of these (4.2%) exhibited an intraductal carcinoma stage pTis, whilst 99 (61.5%) were axillary lymphadenectomised. During the observation time-span of up to 24 years, 9 patients (5.6%) developed local recurrence. In neither patients of the group with axillary nor without axillary dissection could a regional recurrence be observed within this period. Also, a generalisation of this condition was not recorded in any patient. On the basis of our own results and those from the literature we postulate that, under the auspices of a risk adapted tumor surgery, axillary lymphadenectomy is no longer necessary under certain conditions in non-invasive breast carcinoma.

[Hepatocellular carcinoma in pregnancy]. / Hepatozelluläres Karzinom in der Schwangerschaft.

Primary hepatocellular carcinoma is a very rare disease, especially in association with a pregnancy. We report on a 22-year-old primigravida, who underwent Caesarean section in the 29th week of pregnancy in conjunction with tumour-reductive surgery for hepatocellular carcinoma. The further course of the disease was characterised by an early recurrence and lung metastases. Under palliative chemotherapy with 5-fluorouracil, the patient has been in a state of stable disease for several months. Typical risk factors for the hepatocellular carcinoma do not exist in the patient. Alternative explanations for the aetiology of the disease are discussed.

[Lymphocele and complications after pelvic/para-aortic lymph node excision in relation to closure of the peritoneum]. / Lymphozelen und Komplikationen nach pelviner/paraaortaler Lymphadenektomie in Abhängigkeit vom Verschluss des Peritoneums.

From January 1988 to December 1991, 343 women were operated for genital carcinoma at the 1st Gynaecological Department of the University Munich. Additionally to the corresponding cancer operations, pelvic or pelvic and paraaortic lymphadenectomy was performed. In a postoperative systematic and prospective study with ultrasound, we detected a high incidence of 48% of postoperative lymphocysts. Only a few were symptomatic and even less required therapy. In this study we were especially interested in the influence of closure of the pelvic peritoneum on the incidence of lymphocysts. Thus, we inaugurated a prospective study in 1991 with 49 patients with an open peritoneum and compared these results to 294 women who had standard closure of the peritoneum. There was a significantly higher rate of lymphocysts in patients with peritoneal closure (45.2%) in comparison to 20.4% lymphocysts in patients without peritoneal closure. In the study group (open peritoneum) 6.1% of the patients with lymphocysts showed symptoms, only 4.1% needed therapy. In the control group (closed peritoneum), 14.6% of the patients with lymphocysts were symptomatic and 8.2% needed therapy. These differences are not statistically significant. Other complications such as haemorrhage, fever or ileus were comparable in both groups. Our results do not supply sufficient proof, that leaving the peritoneum open significantly reduces symptomatic lymphocysts.

Deciphering the molecular genetics of congenital heart disease.

Congenital heart diseases are starting to benefit from the major advances provided by the advent of molecular biology methods. It is now possible to identify genes which are responsible for congenital heart diseases. The gene responsible for supravalvular aortic stenosis--an autosomal dominant trait--was cloned last year. It is the elastin gene. DiGeorge and Shprintzen syndromes, conotruncal anomaly face and some cardiac malformations have a common cause: a deletion of the 22q11 region resulting in a monosomy. Although the region of deletion is large, it is possible that monosomy of only one gene results in these conditions. Studies are underway to evaluate the impact of this new genetic factor on the incidence of congenital heart malformations. Studies on familial bundle branch block, and lateralization defect with midline anomalies are soon going to show a chromosomal region with the gene defect. Discovering the genes and their protein products which are implied in the cardiac morphogenesis will definitively change our understanding of these cardiac malformations.

Correlation of c-erbB-2 protein expression with histologic grade, lymph node involvement and steroid receptor status in human breast tumors.

The association of c-erbB-2 gene amplification product (p185) with histologic tumor type in 100 patients with primary breast cancer was determined. In 49 patients with infiltrating ductal carcinoma p185 detection was correlated with histologic findings (tumor grade, lymphnode status, receptor status). Strong positive staining for p185 protein was found in 10 patients (20%) with infiltrating ductal breast carcinoma and correlated with complete negative estrogen/progesterone receptor status and with histologic grade G3. There was neither an association with lymphnode involvement nor was there any to negative estrogen and progesterone receptor status alone. At present, we cannot say whether or not there is a correlation between the degree of c-erbB-2 gene amplification and prognosis. Follow-up studies are necessary to determine whether c-erbB-2 gene amplification allows definition of a specific subset of women who could benefit from adjuvant therapy.