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Aortic coarctation was diagnosed in a 27-year-old man with Klippel-Feil syndrome, an inborn skeletal defect of the vertebral column associated with anomalies of various organs. The presented findings are discussed in the context to the theory of vascular artery supply disruption sequence during embryogenisis as a potential explanation for the pathogenesis of morphological defects of Klippel-Feil and associated syndromes., (Copyright 2003 Elsevier Inc.)

Objective: Surface-modifying additives (SMA) have been suggested for improving cardiopulmonary bypass (CPB) circuit biocompatibility, potentially minimizing inflammatory complications and bleeding associated with CPB. The purpose of this prospective, randomized clinical study was to compare a novel copolymer surface-modified CPB circuit (SMARXT; COBE Cardiovascular) against the unmodified circuit., Methods: We randomized 122 patients with isolated coronary artery disease subjected to first-time surgery on CPB into either the SMA (n = 62) or the control group (n = 60). Exclusion criteria included renal insufficiency, liver disease, coagulopathy, anticoagulation therapy < 6 days preop, carotid artery stenosis > 70 %, and a history of stroke. We collected perioperative clinical data including drainage blood loss, transfusion requirements, duration of mechanical ventilation, and ICU stay. Platelet function was determined pre- and post-CPB., Results: SMA patients received 3.2 +/- 0.9 (SD) grafts during 48 +/- 16 min of aortic cross clamp and 91 +/- 30 min CPB (Control: 3.0 +/- 0.9 grafts; p = 0.33, 46 +/- 14 min AXC; p = 0.36, and 84 +/- 23 min CPB; p = 0.14). In the SMA group, 23 patients (37 %) received red blood-cell transfusions, 9 patients (15 %) fresh frozen plasma, and 3 patients (5 %) received platelets (control: n = 27 [46 %], p = 0.44; n = 10 [17 %], p = 0.91; and n=4 [7 %], p = 0.71, respectively). Platelet count on CPB fell to the same level in both groups. In SMA patients, platelet function decreased from 94.2 +/- 24.9 % pre-CPB to 79.5 +/- 32.8 % post-CPB (p = 0.043) (control: from 87.7 +/- 25.6 % to 69.4 +/- 34.7 %; p = 0.001). Postoperative drainage blood loss, mechanical ventilation duration, and ICU stay were similar in both groups (p > 0.3). One patient of the control group was excluded due to surgical bleeding, and one SMA patient died., Conclusions: Our results show that the surface-modified CPB circuit decreased neither blood loss nor transfusions despite slightly better platelet function preservation compared to the unmodified circuit. This type of CPB circuit surface modification does not appear to improve clinical outcome in low-risk coronary artery surgery patients.

Background: The colloid osmotic pressure (COP) is not routinely assessed during pediatric heart surgery. Two cases of unrecognized hyperoncotic states associated with renal failure have been observed after pediatric heart surgery. We studied the hypothesis that the COP cannot be estimated from the total plasma protein (TPP) or albumin level., Methods: The course of COP and its correlation to the TPP and albumin level were investigated in 25 children undergoing elective heart surgery. Infusion therapy was performed solely on the basis of clinical parameters and TPP/albumin levels. COP values were determined in a blinded fashion at the end of the study., Results: No correlation between TPP/albumin and the COP could be determined preoperatively. On arrival at the ICU correlation was strong. A weak correlation was observed at 24 hours and 48 hours after surgery. However, the observed wide range of the confidential bands indicates that the COP cannot be estimated correctly, neither from the TPP, nor from the albumin level. Due to colloidal oversubstitution COP was significantly increased compared to preoperative level at 48 hrs following surgery., Conclusions: As estimation of COP from TPP or albumin level is inaccurate, oncometry should be performed during pediatric heart surgery.

Objective: Colloidal infusion therapy during cardiac surgery has changed from the principal use of human albumin to the preference for synthetic colloids. Despite the possible interference of synthetic plasma expanders with the biuret determination of total plasma protein (TPP), perioperative infusion therapy is frequently directed on the basis of TPP and albumin levels. The hypothesis that the level of TPP or albumin does not reflect the plasma colloid osmotic pressure (COP) if synthetic plasma expanders are used was studied., Materials and Methods: In 61 patients undergoing elective cardiac surgery the course of COP and its correlation to the TPP and albumin levels were investigated. Natural and artificial colloids were used for colloidal infusion therapy., Results: No correlation between TPP/albumin levels and COP was found preoperatively and on arrival at the ICU, only a weak correlation was observed at 24 hours and 48 hours postoperatively. The wide range of the confidential interval indicates that the COP cannot be estimated correctly neither from the TPP nor the albumin level. The postoperative COP was significantly increased compared to the preoperative levels indicating oncotic overdosage., Conclusions: In order to avoid oncotic disturbances, indication for colloidal volume replacement during cardiac surgery should be controlled by oncometry if natural and synthetic colloids are administered.

Postpericardiotomy syndrome (PPS) is a frequent complication after cardiac surgery. In a recent study, elevated anticardiolipin antibody (ACLA) titres were observed in patients with PPS. The value of anti-heart muscle antibodies (AHA) for the diagnosis of PPS remains controversial. Therefore, a prospective double-blind study was performed to test the sensitivity and specificity of ACLA and AHA for the diagnosis of PPS. ACLA titres (ELISA) and AHA, elevated by immunofluorescence, the clinical course and routine laboratory parameters were assessed in 57 patients before and after elective cardiac surgery. ACLA increased and AHA first appeared after surgery in patients both with and without PPS. The sensitivities of a > or = 1.5-fold increase in IgM-ACLA titres, of a > or = 2-fold increase in IgG-ACLA titres and of the occurrence of AHA > or = 2+ for the diagnosis of PPS were 60%, 20% and 20%. The respective specificities were 43%, 79% and 85%. Thus, after cardiac surgery, increased ACLA titres and the occurrence of AHA, as assessed by immunofluorescence, may only contribute to the diagnosis of PPS to a limited extent.

An 18-year-old female patient was admitted with ascites, right upper abdominal tenderness and peripheral edema. Angiography showed complete occlusion of the vena cava inferior up to the level of the right atrium. By open heart surgery, masses of thrombotic material were pulled out of the v. cava inferior/vv. iliacae which histologically contained tumor cell populations consistent with a hepatocellular carcinoma. Celiacography showed a highly vascularized tumor in the right hepatic lobe. Histologically, it proved to be fibrolamellar subtype hepatocellular carcinoma.

The diagnosis of left hemitruncus and large patent ductus arteriosus was made by magnetic resonance imaging in an adult patient with recurrent haemoptysis and dyspnoea on exertion. Previous cardiac catheterization and echocardiography failed to establish the complete diagnosis. Magnetic resonance imaging using spin-echo and gradient-echo pulse sequences is a useful imaging modality to evaluate anatomical and functional abnormalities in patients with complex congenital heart disease.

The clinical, hemodynamic, angiocardiographic, and postmortem findings of a previously unreported type of subaortic stenosis are presented in two patients who also had straddling tricuspid valve and transposition of the great arteries. The subaortic stenosis became apparent after banding of the main pulmonary artery and was due to hypertrophy of a probably abnormally positioned moderator band resulting in a double-chambered right ventricle. Fibrous tissue accumulation at the stenotic os infundibuli also contributed to the subaortic obstruction. Both patients had situs solitus of the atria. Patient 1 had ventricular inversion (L-loop ventricles) and atresia of the right-sided mitral valve. Patient 2 had normally positioned ventricles (D-loop) and two atrioventricular valves. The presence of a large left ventricle and a small right ventricle in the angiocardiogram led to the erroneous diagnosis of a single left ventricle with an infundibular outlet chamber in both patients. Consequently, the subaortic obstruction was thought preoperatively to be at the site of a restrictive bulboventricular foramen. Patient 1 died 36 hours after placement of a valved conduit from the left ventricle to the descending aorta. Patient 2 was operated on successfully and the surgical procedures performed are described.

A noninvasive method of quantitative nuclear angiocardiography was developed using a camera system and a computer. The investigation, which can be performed on an out-patient basis within 10-15 min, includes the following steps: injection of 10 mCi 99mTc-labelled human serum albumin into a femoral vein, and external recording of the passage of the bolus through the central circulation by a sequence of scintigraphic frames taken during diastole. From time--activity curves over the right ventricle, the pulmonary artery and the left ventricle, peak-to-peak times and mean circulation times were calculated. The method was applied to 424 patients with valvular heart disease proven by heart catherization. The following results were obtained: (1) The method proved to be highly sensitive in stating whether there was a hemodynamically significant valvular disease or not. But differential diagnosis concerning the different forms of valvular disease was not possible. (2) The prolongation of the circulation times correlated with the hemodynamic severity of the disease. (3) The circulation times were directly related to the cardiac index, the enddiastolic volume of the left ventricle and the pressures in the pulmonary vascular system. (4) The method could be used to evaluate the effect of surgery and for follow-up after operation. Quantitative nuclear cardiography may help to fill the gap between the nonquantitative, rather unspecific and the specific but invasive methods of cardiologic investigation.

The present report of a malignant metastasizing ameloblastoma and a critical review of literature was undertaken in an attempt to better understand the biological potential and behavior of this rare tumor and thus to facilitate its clinical management. Most of the 26 patients with a proven malignant ameloblastoma including the present case had developed multiple recurrences. The lung was the most frequent metastatic site (88%) followed by regional lymph nodes (27%). Furthermore metastases were observed in some cases in the bone, brain, kidney, small intestine and liver. The interval between diagnosis of tumor and manifestation of metastases was long with a median of 11.1 years. The average survival time was 13.1 years. By contrast, the interval between diagnosis of metastatic disease and death was relatively short (median: 2.6 years). The histologic and cytologic pattern of malignant ameloblastoma and of its metastases was not significantly different from that of non-metastatic ameloblastoma. Because of the lack of morphological criteria of malignancy the biological behavior of ameloblastomas cannot be predicted. It is difficult to be certain which factors are important in the delayed induction of metastases. It is suspected that ameloblastomas possess an inherent low grade malignancy which is stimulated by multiple recurrences. It is further assumed that the metastatic tumor cells have a slow growth rate resulting in late clinical manifestation of metastases. When lung metastases occur we recommend their surgical removal in order to prolong live expectancy or even to obtain a curative effect.

Unlabelled: In patients undergoing thoracic surgery central blood volume is subject to large variations and extravascular lung water may change critically due to fluid shifts. Therefore, an accurate monitoring of these parameters, in particular under perioperative conditions, seems to be desirable. This study describes an improved method for the measurement of intrathoracic volumes. Experiments were carried our in 9 mongrel dogs under piritramide-N2O anesthesia. In order to produce low cardiac output in combination with uneven distribution of perfusion, measurements were performed under base line conditions and after postural changes. Indicators (cold and indocyanine green dye) were injected into the v. cava and indicator kinetics were recorded from the pulmonary artery and aorta using thermistor-fiberoptic catheters. The transport functions of cold and dye were computed from the corresponding pairs of dilution curves. From the transport functions, the mean transit times of the intravascular (dye) and diffusible (cold) indicator were determined. Central blood volume and extravascular lung thermal volume were calculated from the mean transit times and a thermodilution cardiac output., Results: Under base line conditions, central blood volume was 15.3 +/- 2.5 ml/kg body weight. In orthostasis, a significant and reversible reduction to 11.6 +/- 2.4 ml/kg body weight was found. Cardiac output fell significantly from 3.3 +/- 0.5 to 2.4 +/- 1.1. l/min. In contrast, the slight decrease of extravascular lung thermal volume was not statistically significant. It is concluded that the method presented is sensitive enough to detect even small changes of central blood volume.(ABSTRACT TRUNCATED AT 250 WORDS)

The value of cross-sectional sector echocardiography in the visualization of discrete subaortic stenosis (DSS) was investigated using an 80 degrees phased-array sector scanner with simultaneously derived M-mode outputs. Thirty-three patients (including 22 postoperative patients) with proven DSS were investigated and divided into two groups: group I--discrete membranous type; and group II--diffuse fibromuscular type. In group I (15 patients), two isolated subaortic ridges were identified as discrete linear echoes in the long axis of the outflow tract in five patients. A single distinct and isolated linear echo was observed in three patients, whereas in another five the ridges appeared to be attached to the ventricular septum and/or to the base of the anterior mitral leaflet. In two patients with mild DSS, a false-negative diagnosis was made. Only 2 out of 12 patients in this group showed a short remnant of the ridge after surgical revision. In group II, a more generalized or long-segment narrowing was present in 18 patients; in 14 of them the ridges were continuous with the walls of the outflow tract. Eight out of ten patients reexamined following resection of fibromuscular tissue still had residual obstruction. Analysis of the simultaneous M-mode recordings revealed that the transient appearance of the ridge in the outflow tract depended on the normal movement of the heart within the chest, on the lengthening of the membrane itself, and on the angle between the ultrasound beam and the membrane during different cardiac cycles. Our study shows that a spectrum of one- and two-dimensional patterns exists depending on the nature of the obstructing lesion. Cross-sectional sector echocardiography provides more direct visualization and characterization of various types of DSS than M-mode echocardiography does.