Better Late Than Never - A Case of a Congenital Left Partial Anomalous Pulmonary Venous Drainage Diagnosed and Treated in The Sixth Decade of Life.

Partial anomalous pulmonary venous drainage (PAPVD) is a relatively uncommon cardiac anomaly. The diagnosis might be challenging as are the presenting symptoms. Its clinical course mimics more familiar diseases, e.g., pulmonary artery embolism. We present a case of PAPVD, which had been misdiagnosed for more than two decades. After establishing the correct diagnosis, the patient got his congenital anomaly surgically corrected and showed excellent cardiac recovery in the six months follow up.

Does the additional use of heparin-coated extracorporeal circuits (ECC) optimize the effect of modified ultrafiltration (MUF) in pediatric perfusion?

BACKGROUND: Modified ultrafiltration (MUF) has been shown to exert beneficial effects on the coagulation system and the capillary leak after pediatric cardiac surgery using extracorporeal circulation (ECC). The aim of this study was to investigate whether the additional use of heparin-coated circuits is a useful option for improving biocompatibility. METHODS: We randomized 28 children, using heparin-coated ECC circuits in group A (n = 14) and an uncoated equivalent set in group B (n = 14). After congenital heart surgery, MUF was performed post ECC in a standardized fashion. Blood samples were analyzed preoperatively, 10 min, 30 min, 1 h, and 48 h after ECC by flow cytometric analysis (FACSort) using surface antigens CD62/CD41b (platelets) and CD45/CD14 (monocytes). RESULTS: No significant difference was found with respect to mean age (20.6 months vs. 21.6 months), mean body weight (9.2 kg vs. 8.4 kg), mean ultrafiltration rate (9.1 ml/kg vs. 11.4 ml/kg), chest tube drainage, blood products, ICU stay, and 30-d survival. The percentage of CD62/CD41-positive platelets in group A (vs. B) increased up to 118 % at 60 min vs. 130 % ( P < 0.05) and declined to 98 % at 48 h postop. vs. 99 % (n. s.). The percentage of CD45/CD14-positive monocytes in group A (vs. B) increased up to 158 % at 60 min vs. 155 % (n. s.) and declined to 122 % (A) at 48 h postop. vs. 61 % (B) ( P > 0.05). CONCLUSIONS: Heparin coating of ECC in addition to MUF leads to a lower platelet activation. Monocyte surface markers CD45 and CD14 indicated a marked activation during ECC in both groups but additional heparin coating showed a better postoperative regeneration of monocyte markers in the late course indicating a beneficial additive effect.

Comparison of leptin gene expression in different adipose tissues in children and adults.

OBJECTIVE: Adipose tissue displays depot-specific metabolic properties and a predominant gene expression of leptin in subcutaneous tissue. The aim of the study was to evaluate leptin mRNA expression in various adipose tissues and to relate it to plasma leptin concentrations. Furthermore, developmental changes in leptin gene expression from childhood to adulthood were examined. DESIGN AND METHODS: Thoracic subcutaneous and intrathoracic adipose tissue specimens were obtained in 22 adults (51-81 years) and 23 children (0.1-17 years) undergoing cardiac surgery, and abdominal subcutaneous, omental and mesenterial fat specimens were collected from 21 adults (38-79 years) and 22 children (0.2-17 years) before abdominal surgery. Preoperative plasma leptin concentrations were measured by RIA. Leptin mRNA expression was quantified by TaqMan real-time PCR. RESULTS: In adults, there was no difference between leptin gene expression in subcutaneous and intrathoracic fat, whereas in children leptin mRNA expression was significantly higher in subcutaneous adipose tissue. In omental fat, leptin mRNA levels were significantly lower compared with subcutaneous and mesenterial sites in both children and adults. Adults revealed a significantly higher leptin gene expression in subcutaneous, omental and mesenterial adipose tissues than children. Subcutaneous and omental leptin gene expression are independent factors for plasma leptin concentrations in children and adults. CONCLUSION: Leptin is differentially expressed at different adipose tissue sites, a situation which is even more pronounced in children. There is a developmental increase in leptin mRNA expression in adipose tissue during childhood, reaching maximal capacity in adulthood.

Late pulmonary valve replacement after correction of Fallot's tetralogy.

BACKGROUND: The aim of this study was to investigate necessity and outcome of late pulmonary valve replacement (PVR) after repair of tetralogy of Fallot (TOF). METHODS: Hospital records from patients operated on for TOF at our institution between 1960 and 2002 were reviewed and patients were interviewed by questionnaires. RESULTS: Out of 411 long-term survivors after TOF-repair, 47 (11.4 %) patients required reoperation after 13.2 +/- 7.4 years. Preoperative right ventricular (RV) dilatation was present in 36 (76.6 %) patients including 16 (34 %) with impaired RV function. Isolated PVR was performed in 12 patients (25.5 %). Additional procedures were necessary in 35 patients (74.5 %), including closure of residual defects (VSD, n = 11), tricuspid valve replacement (n = 1) and repair (n = 3). Obstructive right ventricular or pulmonary artery lesions (34 patients, 72.3 %) were all surgically addressed. RV pressure decreased from 61.1 +/- 27.7 to 42.9 +/- 13.3 mm Hg (p < 0.01) after PVR. RV size was reduced and RV function improved compared to preoperative values. Early mortality after reoperation was 2.1 % (n = 1) with one patient dying from biventricular failure. There was no late mortality. CONCLUSIONS: PVR after Fallot repair is frequently required because of progressive RV enlargement with dysfunction. It can be performed with relatively low risk, even in the setting of multiple reoperation. Obstructive lesions (RVOTO, PA stenosis) and residual defects are frequently observed in patients needing late PVR and may play a crucial role in the development of RV failure. Timely valve replacement with repair of all obstructive lesions proximal and distal to the implanted valve is the key to preserving RV function.

Modulating IL-6 and IL-10 levels by pharmacologic strategies and the impact of different extracorporeal circulation parameters during cardiac surgery.

Postoperative morbidity after coronary artery bypass grafting (CABG) using cardiopulmonary bypass (CPB) can be influenced by pro- and anti-inflammatory cytokines like interleukin 6 (IL-6) and IL-10 triggering and balancing the acute phase response. The extent of cytokine release can be modulated by different methods. This prospective randomized study examines the effect of treatment of patients with steroid (group 1, 250 mg of prednisolone)(Solu-Decortin H)), aprotinin (group 2, 6 Mio. KIU [kallikrein inhibitory units] aprotinin [Trasylol]), and heparine coating of the artificial surface (group 3, Bioline) on the systemic release of IL-6 and IL-10 in four groups of 40 patients with coronary artery disease (CAD) scheduled for CABG. Group 4 (standard medication) served as control. Twenty hemodynamic and biochemical parameters of the CPB were analyzed regarding correlation to cytokine levels measured by enzyme-linked immunosorbent assay (ELISA). In group 1, IL-6 was suppressed compared to the control (P< 0.01). IL-10 was upregulated (P< 0.01). In group 2, cytokine release was similar to group 1. Using heparin-coated circuits in group 3 led to IL-10 upregulation (P < 0.05) and IL-6 suppression (P < 0.05). We found an exponential relationship between IL-10 levels (IL-6 levels) and cardiac ischemia time, duration of CPB, and the extent of negative base excess. An inverse relationship was found for IL-10 (IL-6) levels and venous O2 saturation (SvO2), and mean arterial pressure (MAP). Hypothermia (<34 degrees C) reduced IL-10 and IL-6 release, whereas long duration of hypothermia correlated with higher IL-10 and IL-6 release. Cytokine release after extracorporeal circulation (ECC) can be modulated pharmacologically and by distinct perfusion regimen.

Reducing the post-pump syndrome by using heparin-coated circuits, steroids, or aprotinin.

BACKGROUND: Cardiopulmonary bypass (CPB) induces a systemic inflammatory response called 'post-pump syndrome'. As a part of a complex interaction between white cells and vascular endothelium, proinflammatory cytokines IL-6 and IL-8 are part of a phased immune response that is also balanced by anti-inflammatory cytokines such as IL-10. We compared the influence of heparin-coated circuits, steroids, and aprotinin on these cytokines, looking for ways to reduce the syndrome. METHODS: 40 patients with coronary artery disease (CAD) undergoing elective CABG were prospectively studied in four randomized groups of 10. Group A received prednisolone pre- and postoperatively (2 x 250 mg), group B received aprotinin perioperatively (6 Mio. KIU). In group C, heparin-coated circuits ('Bioline' by Jostra) were used and in group D no special measures were taken (controls). Plasma levels of cytokines were measured before and during CPB and until 12 h after surgery using an ELISA technique. RESULTS: In group A IL-6 was significantly (p<0.05) suppressed in contrast to the control group (A: peak at 4 h, 155 pg/ml vs. control: peak at 8 h, 565 pg/ml). IL-8 was also suppressed (A: peak at 30', 22 pg/ml vs. control: peak at 30', 55 pg/ml). IL-10 level changed first and was markedly upregulated in contrast to the control (A: peak at 30', 1600 pg/ml vs. control: peak at 30', 130 pg/ml; p<0.05). In group B (aprotinin) the cytokine release was similar to group A. Using heparin-coated circuits (group C) also led to a significant (p<0.05) IL-10 upregulation (C: peak at 2 h, 1380 pg/ml) and IL-6 suppression (C: peak at 4 h, 290 pg/ml). IL-8 was not influenced significantly. CONCLUSIONS: The results show a similar reduction of the inflammatory cytokine release (IL-6 and IL-8 as markers) using early steroid application and aprotinin in high dosage. Heparin coating reduces IL-6 and increases IL-10 release, whereas IL-8 is not affected. Further studies should investigate the effects of a combined application for reducing inflammatory cytokine release and the post-pump syndrome.

Perioperative factors influencing interleukin-10 release under cardiopulmonary bypass.

BACKGROUND: The cytokine response to cardiopulmonary bypass (CPB) is complex and can be modified. Among several mediators, the anti-inflammatory interleukin-10 (IL-10, 'cytokine-secretion inhibitory factor') is particularly interesting because of its ability to counteract pro-inflammatory cytokines triggering endothelial and leukocyte activation in the immediate immune response to CPB. On the other hand, during the delayed phase of the immune response, IL-10 may act as a promotor of immunodeficiency in complicated courses. Therefore, it is of interest to investigate special conditions of CPB that may influence the extent of perioperative release of IL-10. METHODS: We analyzed 20 continuously registered parameters during CPB, including an analysis of subgroups in the case of application of aprotinin or steroids. 30 consecutive adult patients with coronary artery disease (CAD) and normal left-ventricular function undergoing elective CABG were prospectively studied. Arterial blood was sampled perioperatively and levels of IL-10 were determined using ELISA tests. For analysis, the time point of maximum IL-10 release was selected (30 min after end of CPB). Simultaneously, CPB-registration protocols were analyzed concerning standard parameters. RESULTS: We could state an exponential relationship between IL-10 levels 30 min after end of CPB and the ischemia time (r = 0.76), duration of CPB (r = 0.73) and the extent of negative base excess (BE, r = 0.66) in all subgroups. An inverse relationship could be seen between IL-10 plasma levels and venous O2 saturation: low values for O2 saturation correlated with high IL-10 levels as did low mean arterial pressure (MAP). Hypothermia reduced IL-10 release (r = 0.80), whereas a long duration correlated with high IL-10 release (r = 0.67). In the case of longer duration of hypothermia, the protective effect vanished. CONCLUSIONS: The results show a significant rise for IL-10 early after starting CPB. Low values for venous O2 saturation and low MAP correlated with high IL-10 levels. A good correlation could be seen between IL-10 plasma levels and the duration of CPB, ischemia time, and negative base excess. Because of the ability of persisting IL-10 production to induce a higher incidence of septic complications, all actions for maintaining an optimum of perfusion and oxygenation play an important role.