Background: Supravalvular aortic stenosis (SVAS) can be focal with a distinct stenosis localization or with a diffuse narrowing of the ascending aorta. Surgical techniques consist of the single patch repair technique (McGoon's repair), the pantaloon- or Y-shaped patch which extends into two aortic valve sinuses (Doty's technique and the three-patch technique where three separate triangular patches are inserted in the three sinuses (Brom's technique).

Background: Pediatric coronary artery bypass is performed for ischemic complications of Kawasaki disease, congenital coronary anomalies, and iatrogenic pediatric coronary artery problems.Methods: We performed myocardial revascularization using the internal mammary artery in 6 children. A review of outcomes is presented here. Patient ages ranged from 7 days to 10 years.Results: There was no operative mortality. All coronary bypass grafts were patent at 3 months to 3 years postoperatively. No patient had recurrent angina or showed signs of myocardial ischemia.Conclusion: Internal mammary artery bypass grafting can be successfully performed in infants and children with good results. Long-term patency and growth of the anastomosis of the distal vessel are not clear, but good clinical and angiographic results have been reported even after 25 years.

The significance of cytomegalovirus (CMV) infection after lung transplantation was investigated in 20 patients (ten women and ten men; mean age 46 [21-67] years). Indications for transplantation were emphysema (n = 6), cystic fibrosis (n = 2), primary pulmonary hypertension (n = 2), pulmonary fibrosis (n = 5), obliterating bronchiolitis (n = 2), cystic lung (n = 2) and bronchiectasis (n = 1). Incidence, diagnostic parameters (serology, virus isolation and histology) and efficacy of prophylactic and therapeutic measures were recorded. 16 of the 20 patients developed a CMV infection, which in 12 was clinically significant. CMV pneumonia developed in two patients, proving fatal in one. The infection occurred a median of 47 (17-200) days after the transplantation. Administration of Ganciclovir (5 mg/kg twice daily intravenously) brought about remission of symptoms in all but one of the patients and improved the clinical parameters.--This experience demonstrates that regular monitoring of the patients for possible CMV infection and its early therapy can achieve a low death rate.

Karth, Georg Delle; Buberl, Anton; Meyer, Brigitte; Wojta, Johann; Wollenek, Gregor; Grimm, Michael; Lassnigg, Andrea; Brannath, Werner; Hiesmayr, Michael; Heinz, Gottfried Author Information

Excimer lasers have been demonstrated to provide a very precise and circumscribed ablation of synthetic polymers and biological tissues. We investigated in vitro the use of ultrashort pulsed ultraviolet excimer-laser energy for controlled removal of meniscus cartilage under the aspects of arthroscopic meniscectomy. A krypton-fluorine gas mixture was used to achieve laser emission of 248-nm wavelength. A total of 22 human menisci obtained either by operation or necropsy were irradiated over a range of energy fluence (2.15–3.07 J/cm2/pulse), repetition rates (5–20 Hz), and exposure time (15–60 s). Ablation rates of 4.00–5.76 μm per pulse were obtained. Light-microscopic examinations demonstrated tissue ablation without any evidence of pathological changes associated with continuous-wave laser irradiation. Effects of laser energy were clearly limited to the target of the laser beam, and tissue removal proceeded without production of heat or smoke. Due to the lack of pathological alterations observed, excimer-laser irradiation of meniscus cartilage may prove to be advantageous for precisely cutting and removing menisci without injury to the surrounding normal tissue. Clinical application of excimer-laser irradiation includes the development of suitable fiberoptics and laser coupling, as well as modification of fiber tips.

An oxyhaemoglobin dissociation curve (ODC) quantifies the most important function of red blood cells and that is the affinity for oxygen and its delivery to the tissues. Oxygen affinity for haemoglobin plays a critical role in the delivery of oxygen to the tissues and is changed by shifting to the left or right. A shift to the left implies an increased oxygen affinity and, hence, tighter binding due to the higher oxygen saturation in relation to the pO2.On the other hand, a shift to the right corresponds to a decreased oxygen affinity and easier release of oxygen to the tissues. It is well known that the ODC shifts in response to changes in pH, pCO2 and 2,3 diphosphoglycerate. However, how much the ODC shifts has never been quantified. Arterial and venous blood gases were taken during cardiopulmonary bypass and two indices were used to quantify the shift of the ODC; the p50 shift and the SO2 difference. Arterial blood shifted to the right by 49-0.1 mmHg at a pH of 7.24 and shifted to the left by -3.59-0.05 mmHg at a pH of 7.51. The change in arterial saturation was minimal, rising by 0.8% and dropping by -5% and did not correlate to p50 shifting and changes in pH, but demonstrated changes dependent on the concentration of dyshaemoglobins. The venous blood exhibited a greater range of p50 shifting at each pH value. At a pH of 7.24, the p50 shifted to the right by 4.89-2 mmHg and at a pH of 7.51 the p50 shifted to the left by -4±1.8 mmHg. Unlike the arterial blood, the change in saturation correlated well to p50 shifting. It is shown here for the first time how much the curve shifts with changes in pH and how this may be used to evaluate treatment strategies.

OBJECTIVE:Inhaled nitric oxide (NO) is used as a therapy of pulmonary hypertension in children after cardiac surgery. Hemoglobin binds to NO with great affinity and forms methemoglobin by oxidation in the erythrocyte. Once produced, methemoglobin is unable to transport and unload oxygen in the tissues. The amount of available hemoglobin in the body for oxygen transport is thereby reduced. Anemia, acidosis, respiratory compromise and cardiac disease may render patients more susceptible than expected for a given methemoglobin level. The goal of the present study was to review the cumulative effect of inhaled NO on methemoglobin formation in critically ill children. We therefore looked for methemoglobin levels in children with congenital heart disease after cardiac surgery who were treated with inhaled NO in a range of 5-40 ppm.METHODS:We retrospectively reviewed the medical charts of 38 children with congenital heart disease after cardiac surgery. We extracted demographic data and physiological measurements at the following time points: (1) T0 = before starting inhaled NO therapy, (2) T1 = 24 h after the beginning of inhaled NO therapy, (3) T2 = half-time therapy, (4) T3 = end of therapy, (5) T4 = 24 h after finishing inhaled NO therapy.RESULTS:The median duration of inhaled NO therapy was 5.5 days (interquartile range 6, range 2-29), NO concentrations at T1 and T2 were 16 ppm (10, 5-40) and 12.5 ppm (12.3, 2-40), respectively. The median cumulative dose of inhaled NO was 1699 ppm (2313, 193-7018). Methemoglobin levels increased moderately, but significantly, during therapy ( T0 vs T1 p<0.05 and T0 vs T2 p<0.001). The highest methemoglobin level measured was 3.9%. Methemoglobin levels correlated positively with the inhaled NO doses applied at T1 ( r(2)=0.8376; p<0.01) and at T2 ( r(2)=0.8945; p<0.01). At T1 the methemoglobin level correlated negatively with the T1 blood pH value. The overall mortality rate was 13.2% (5 of 38 study patients died). There was no significant difference in methemoglobin levels between survivors and non-survivors.CONCLUSION:We conclude from our data that the use of inhaled NO therapy for children with congenital heart disease after cardiac surgery in the described range of 5-40 ppm, resulting in a maximum of 4% methemoglobin blood level, is feasible and safe. However, we recommend the use of the minimal effective dose of inhaled NO and continuous monitoring of methemoglobin levels, especially in cases of anemia or sepsis in critically ill children.

Background. Cerebral damage is a serious complication of pediatric cardiac surgery. Early prediction of actual risk can be useful in counseling of parents, and in early diagnosis and rehabilitation therapy. Also, if all children at risk could be identified therapeutic strategies to limit perioperative cerebral damage might be developed. The aim of this study is to create a mathematical model to predict risk of neurologic sequelae within 24 hours after surgery using simple and readily available clinical measurements.Methods. The hospital records of 534 children after cardiac surgery were reviewed. Variables examined were age at operation, diagnosis, use of cardiopulmonary bypass, arterial and central venous oxygen saturation, serum glucose, lactate and creatine kinase, mean arterial pressure, and body temperature. The endpoint for each study patient was the occurence or lack of occurence of seizures, movement or developmental disorders, cerebral hemorrhage, infarction, hydrocephalus, or marked cerebral atrophy. Univariate and multivariate regression analyses were used to evaluate the predictive power of the investigated factors as well as to create a predictive model.Results. In 6.26% of children symptoms of cerebral damage were found. Significant risk factors were age at surgery, more complex malformations, metabolic acidosis, and increased lactate (odds ratio: age, 0.882/yr [0.772-1.008]; complex malformations, 10.32 [1.32-80.28]; arterial pH more than 7.35 to 0.4 [0.18-0.89]; lactate -1.018 per mg/dL [1.006-1.03]).Conclusions. It is possible to quantify the risk of appearance of symptoms of cerebral damage after cardiac surgery within 24 hours using simple and readily available clinical measurements. (C) 2003 by The Society of Thoracic Surgeons.

J Thorac Cardiovasc Surg Horvat, H. Gabriel, E. Wolner and M. Grimm M. Gorlitzer, H. J. Ankersmit, G. Wollenek, W. Wisser, G. Wieselthaler, M. Haisjackl, R. concomitant domino heart transplantation Backtable ventricular and atrial septal defect repair of an Eisenmenger allograft with http://jtcs.ctsnetjournals.org/cgi/content/full/123/2/374 the World Wide Web at: The online version of this article, along with updated information and services, is located on

We have previously reported that the outcome of infants with functional single ventricle and total anomalous pulmonary venous connection is poor relative to that of other single ventricle patients. Younger age at initial operation and obstructed total anomalous pulmonary venous connection were found to be risk factors for mortality. A review of our recent experience was undertaken to determine whether results in these patients are improving.Medical records of 18 patients admitted after 1997 were reviewed (group B) and compared with the previous group of 73 patients admitted between 1984 and 1997 (group A). Data were analyzed using a Cox proportional hazards model.Median age at first operation was the same for both groups. The incidence of obstructed total anomalous pulmonary venous connection was not significantly different between groups (29% versus 33%, p = 0.70). Early survival is significantly improved for group B compared with group A (p = 0.015). Only group and younger age at initial operation were found to be risk factors for mortality. In the current group, 5 patients have undergone superior cavopulmonary connection with one death, 3 have undergone Fontan completion with no deaths, and heart or heart-lung transplantation was performed in 5 patients with two deaths.Early survival in patients with single ventricle and total anomalous pulmonary venous connection has improved significantly in recent years, but intermediate survival is still approximately 50%. Selective management using staged reconstructive surgery and transplantation may result in improved survival. Further study may identify risk factors for mortality and improve selection of appropriate patients for each therapy.

Objective: Excellent hemodynamic performance has been demonstrated after aortic valve replacement using the autologous pulmonary valve as described by D. Ross. However, in the pediatric population there is concern in regard to growth of the autograft and late dilatation in the systemic circulation. Methods: Since 1991, 30 children (mean age, 11.3 +/- 3.1 years) had aortic valve replacement with the pulmonary autograft as a Not replacement. All children had yearly clinical and echocardiographic follow-up. Results: Then were no perioperative deaths; one child died late in a car accident. At the last follow-up (mean follow-up, 4.3 +/- 2.6 years), all patients: were in NYHA class I. There was one early reoperation, in which the autograft had to be reconstructed due to a leaflet perforation. There were no major valve related events. All children showed normal somatic growth. The annulus diameter increased significantly from 18 +/- 2 at surgery to 20 +/- 3.5 mm at the latest follow-up (P < 0.004). The sinus also increased significantly in diameter from 29 +/- 4 at surgery to 34 +/- 2 mm at the last follow-up (P < 0.001). This increase in autograft size, both for the annulus and the sinus, paralleled the increase in body surface area with no evidence for unproportional dilatation. Hemodynamic measurements demonstrated physiological peak gradients of 6.8 +/- 2.9 mmHg and no or trivial aortic insufficiency in 95% of this rapidly growing patient population. Conclusion: These data demonstrate growth of the pulmonary autograft parallel to somatic growth without undue dilatation in the systemic circulation. The hemodynamics are excellent with regard to physiological gradients and no increase in aortic insufficiency. (C) 2001 Elsevier Science B.V. All rights reserved.

Studies performed in mice together with the demonstration of increased levels of heart-specific autoantibodies, cytokines and cytokine receptors in sera from cardiomyopathy (CMP) patients argued for a pathogenic role of autoimmune mechanisms in CMP. This study was designed to analyse the presence of IgG anti-heart antibodies in sera from patients suffering from hypertrophic and dilatative forms of CMP as well as from patients with ischaemic heart disease and healthy individuals. Patients' sera were analysed for IgG reactivity to Western-blotted extracts prepared from human epithelial and endothelial cells, heart and skeletal muscle specimens as well as from Streptococcus pyogenes. The IgG subclass (IgG1-4) reactivity to purified human cardiac myosin was analysed by ELISA. While sera from CMP patients and healthy individuals displayed comparable IgG reactivity to a variety of human proteins, cardiac myosin represented the prominent antigen detected strongly and preferentially by sera from CMP patients. Pronounced IgG anti-cardiac myosin reactivity was frequently found in sera from patients with dilatative CMP and reduced ventricular function. ELISA analyses revealed a prominent IgG2/IgG3 anti-cardiac myosin reactivity in CMP sera, indicating a preferential Th1-like immune response. Elevated anti-cytomegalovirus, anti-enterovirus IgG titres as well as IgG reactivity to nitrocellulose-blotted S. pyogenes proteins were also frequently observed in the group of CMP patients. If further work can support the hypothesis that autoreactivity to cardiac myosin represents a pathogenic factor in CMP, specific immunomodulation of this Th1- towards a Th2-like immune response may represent a promising therapeutic strategy for CMP.

Background: Ebstein's anomaly is a rare congenital cardiac defect of the tricuspid valve (TV) leading to severe tricuspid insufficiency (TI). Methods: In ten patients, 6 adults (39-53 years) and 4 children (5-10 years), operated between 1989 and 1995 echocardiography was performed pre and post repair and at follow-up. Patients were assessed in our institution at two cut-off points, resulting in a mean first follow-up of 17 +/- 15 months and a mean second follow-up of 53 +/- 23 months. All patients had additional congenital cardiac defects (ASD,VSD). In all patients the TV was repaired by techniques described by Carpentier et a[. with some modifications. The goal of this reparative attempt is to mobilize restricted leaflet tissue and aid coaptation through implantation of a ring. Results: Echocardiographically we were able to identify significant characteristics for the successful repair of Ebstein's anomaly. The severity of the disease is represented by the size and function of the right ventricle and the atrialized chamber, the most advanced cases exhibiting a dilated right ventricle with poor contractility. There was severe preoperative TI ( mean grade 3.2 +/- 0.3). Postoperatively TI was significantly reduced to a mean grade of 2 +/- 0.2. 60% of the patients demonstrated an improvement in the ratio of atrialized chamber to functional right ventricle. Right-ventricular function was improved, the mean score being 2.8 +/- 0.1. At follow-up I and II right-ventricular function and tricuspid insufficiency was improved in most patients and all patients benefited in quality of life. Conclusions: These results suggest that surgical correction should not be delayed until severe right heart failure develops as, particularly in children, good results are achieved, improving the quality of life.

BACKGROUND The major current concept for the pathogenesis of the Down Syndrome (DS) phenotype including congenital heart disease (CHD) is the so-called "gene dosage effect." According to this hypothesis, genes encoded by chromosome 21 at the "critical region" (which is thought to be crucial for the development of the DS phenotype) are overexpressed in the trisomic state, thus leading to an imbalance of genes as, e.g., the protooncogene ets-2, superoxide dismutase, etc. METHODS We studied heart biopsies obtained at surgery from 6 patients with DS and 7 patients with congenital heart disease. ets-2-mRNA steady state levels were determined by a competitive reverse transcription-polymerase chain reaction (RT-PCR) technique which allowed the determination of this gene at the attomol level. RESULTS ets-2 mRNA in total ventricular tissue of DS patients showed concentrations of 0.60 +/- 0.42 fg/10 ng total RNA (mean, +/- SD). When normalized versus the housekeeping gene beta-actin to rule out general transcriptional changes in that disorder, the ratio of 0.56 +/- 0.28 (mean, +/- SD) was calculated. ets-2 mRNA in total ventricular tissue of patients with non-DS CHD showed concentrations of 0.45 +/- 0.22 fg/10 ng total RNA (mean, +/-SD) and ratios of 0.48 +/- 0.35 (mean, +/-SD). No differences could be found at the p<0.05 level. CONCLUSION No absolute quantification of a gene incriminated in the "gene dosage effect-hypothesis" was performed so far and the only approach to (semi-) quantitative determination of the ets-2 gene using northern blotting was published on one individual DS sample only. This is the first report to clearly show that no overexpression of ets-2 can be found in heart of patients with DS, thus providing evidence against the current gene dosage effect-hypothesis.

BACKGROUNDThe high incidence of aortic disease in subjects with congenital aortic valve malformations suggests a causative relationship between these 2 conditions. The histological observation in aortic dilatation/aneurysm/dissection is Erdheim cystic medial necrosis (CMN), a noninflammatory loss of smooth muscle cells (SMCs), fragmentation of elastic fibers, and mucoid degeneration.METHODS AND RESULTSTo examine whether apoptosis is 1 of the mechanisms underlying CMN and aortic medial layer SMC loss, ascending aortic wall specimens from 32 patients were collected at cardiothoracic surgery and examined by histochemical staining and terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphate nick end labeling. From echocardiography results, 4 groups of patients were identified: bicuspid valve carriers with (bi/dil) or without (bi/0) aortic dilatation and tricuspid valve carriers with (tri/dil) or without (tri/0) aortic dilatation. Massive focal apoptosis was observed in the medial layers of bi/dil (mean apoptotic index [mAI], 8.1+/-6.0) and tri/dil (mAI, 8.1+/-8.3) compared with tri/0 (mAI, 0.9+/-1.2; P=0.0079 and P=0.037). In bi/0 (mAI, 9.1+/-5.7) compared with tri/0 (mAI, 0.9+/-1.2), rates of medial SMC apoptosis were increased (P=0.0025). Bi/dil (mean age, 40. 6+/-15.7 years) were significantly younger than tri/dil (mean age, 56.4+/-12.8 years) undergoing the same operation (P=0.0123).CONCLUSIONSPremature medial layer SMC apoptosis could be part of a genetic program underlying aortic disease in patients with aortic valve malformations.

Although the anatomy, histology and pathology of human coronary arteries have been studied extensively, little is known about the functional relationship between vessel radius and wall thickness. It is the purpose of this study to present detailed measurements and to describe this relationship covering the range from the feeding coronary artery to the arterioles. Human hearts of 10 adults less than 36 +/- 3 years old were investigated immediately postmortem. Ten cubic tissue blocks, measuring about 10 mm in length on each side, were dissected from the left ventricular wall. After fixation by immersion, 15-microm sections were prepared and outer and inner perimeters of 52 arterial segments were digitalized. Vessel radius and wall thickness were calculated and plotted to show their relationship over the whole range of vessel calibers. Outer vessel radii ranged from 100 to 3,000 microm and wall thickness from 80 to 800 microm. Plotting the outer vessel radius against the wall thickness, the data points were found to cluster around a straight line. A significant correlation between the two parameters was found (R2 = 0.79). This mathematical correlation and the good agreement of the presented results with data from other species indicate a common physiologic concept.