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Byline: Dierk Thomas (1), Kezhong Wu (1), Anna-Britt Wimmer (1), Edgar Zitron (1), Bettina C. Hammerling (1), Sven Kathofer (1), Sonja Lueck (1), Ramona Bloehs (1), Volker A. W. Kreye (2), Johann Kiehn (1), Hugo A. Katus (1), Wolfgang Schoels (1), Christoph A. Karle (1) Keywords: Adrenergic (ant)agonists; Arrhythmia; K-channel; hERG channel; Long QT syndrome Abstract: Patients with cardiac disease typically develop life-threatening ventricular arrhythmias during physical or emotional stress, suggesting a link between adrenergic stimulation and regulation of the cardiac action potential. Human ether-a-go-go related gene (hERG) potassium channels conduct the rapid component of the repolarizing delayed rectifier potassium current, I.sub.Kr. Previous studies have revealed that hERG channel activation is modulated by activation of the [beta]-adrenergic system. In contrast, the influence of the [alpha]-adrenergic signal transduction cascade on hERG currents is less well understood. The present study examined the regulation of hERG currents by [alpha].sub.1A-adrenoceptors. hERG channels and human [alpha].sub.1A-adrenoceptors were heterologously coexpressed in Xenopus laevis oocytes, and currents were measured using the two-microelectrode voltage clamp technique. Stimulation of [alpha].sub.1A-receptors by applying 20 AuM phenylephrine caused hERG current reduction due to a 9.6-mV shift of the activation curve towards more positive potentials. Simultaneous application of the [alpha].sub.1-adrenoceptor antagonist prazosin (20 AuM) prevented the activation shift. Inhibition of PKC (3 AuM Ro-32-0432) or PKA (2.5 AuM KT 5720) abolished the [alpha]-adrenergic activation shift, suggesting that PKC and PKA are required within the regulatory mechanism. The effect was still present when the PKA- and PKC-dependent phosphorylation sites in hERG were deleted by mutagenesis. In summary, cardiac repolarizing hERG/I.sub.Kr potassium currents are modulated by [alpha].sub.1A-adrenoceptors via PKC and PKA independently of direct channel phosphorylation. This novel regulatory pathway of [alpha].sub.1-adrenergic hERG current regulation provides a link between stress and ventricular arrhythmias, in particular in patients with heart disease. Author Affiliation: (1) Department of Cardiology, Medical University Hospital, Im Neuenheimer Feld 410, 69120, Heidelberg, Germany (2) Department of Physiology and Pathophysiology, Medical School, University of Heidelberg, Im Neuenheimer Feld 326, 69120, Heidelberg, Germany Article History: Registration Date: 15/07/2004 Received Date: 29/04/2004 Accepted Date: 07/07/2004 Online Date: 08/09/2004 Article note: Electronic Supplementary Material Supplementary material is available for this article if you access the article at http://dx.doi.org/10.1007/s00109-004-0582-8. A link in the frame on the left on that page takes you directly to the supplementary material.

Byline: G. P. Taylor (1) Author Affiliation: (1) Department of Pathology, Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G 1X8, Canada Article History: Registration Date: 01/01/2004 Received Date: 24/05/2004 Accepted Date: 26/07/2004 Online Date: 17/11/2004 Article note: Presented at the US-CAP Pediatric Pathology Specialty Conference March 23, 2003.

Byline: R. J. Ascuitto (1), N. T. Ross-Ascuitto (1) Keywords: Flow-energy loss; collaterals; Fontan anatomy Abstract: Patients with Fontan-modified, single-ventricle heart frequently have systemic collaterals that increase pulmonary blood flow. Competitive flow from these auxiliary vessels can also elevate pulmonary artery pressure, a process leading to erosion of flow's mechanical energy. An analogous analytical description of mixing fluid streams was used to provide insight into flow energetics associated with systemic-to-pulmonary collaterals in Fontan-type circulation. We find that theoretical pressure increases and flow energy losses due to mixing vary quadratically as the velocity differences of the interacting fluid streams. Moreover, the predicted flow energy loss is shown to depend directly on the resultant pressure increase. Based on studies of aortopulmonary collaterals in patients with Fontan anatomy, we provide an estimate of pulmonary artery pressure elevation and flow energy loss, factors that are of considerable clinical importance. Author Affiliation: (1) Departments of Pediatrics and Physics, Tulane University, 1430 Tulane Avenue, New Orleans, LA, 70112, USA Article History: Registration Date: 01/01/2004 Online Date: 30/07/2004

Byline: Hideo A. Baba (1), Jeremias Wohlschlaeger (1), Henning D. Stubbe (2), Florian Grabellus (1), Hugo Van Aken (2), Klaus J. Schmitz (1), Friedrich Otterbach (1), Kurt W. Schmid (1), Christian August (3), Bodo Levkau (4), Frank Hinder (2) Keywords: Myocardium; Heat shock protein; Sepsis; Multiple organ failure; Vasoconstrictor therapy; Apoptosis Abstract: Objective Inducible heat shock protein 72 (HSP 72) preserves myocardial function and prevents apoptosis. We investigated the expression and localization of HSP 72 and apoptosis in our previously described new model of multiple organ failure. Design Eighteen adult-instrumented sheep and three healthy controls were randomly assigned to one of three groups: (a) norfenefrine-masked hypovolemia plus endotoxemia (NMH+ENDO) (b) norfenefrine-masked hypovolemia without endotoxemia (NMH) (c) recurrent endotoxemia during normovolemia (ENDO) and (d) normovolemia without endotoxemia (CONTROLS). Measurements and results Hearts were analyzed by light microscopy, Western blots, immunohistochemistry, and TUNEL staining. HSP 72 expression was approximately threefold increased in NMH+ENDO compared with the other groups (p Conclusion HSP 72 overexpression and apoptosis, but not necrosis, indicate cardiovascular decompensation and poor outcome during early multiple organ failure. Author Affiliation: (1) Institut fur Pathologie, Universitat Essen, Hufelandstrasse 55, 45147, Essen, Germany (2) Klinik und Poliklinik fur Anasthesiologie und operative Intensivmedizin, Universitat Munster, Munster, Germany (3) Gerhard-Domagk Institut fur Pathologie, Universitat Munster, Munster, Germany (4) Institut fur Pathophysiologie, Zentrum fur Innere Medizin, Universitat Essen, Essen, Germany Article History: Registration Date: 05/01/2004 Received Date: 07/10/2003 Accepted Date: 22/12/2003 Online Date: 24/02/2004 Article note: H.A. Baba and J. Wohlschlaeger contributed equally to the study.

Byline: A. Rydberg (1), P. Rask (2), R. Hornsten (2), D. Teien (1) Keywords: Heart rate variability; Fontan circulation Abstract: Heart rate variability (HRV) can be used to study cardiovascular autonomic control. This study examines HRV in children with Fontan circulation and its change over time. Thirty-four children in two groups were examined. Group A consisted of 10 patients who had undergone total cavopulmonary connection. Group B consisted of 24 healthy children/adolescents matched for gender, height, and weight. To analyze HRV parameters, all examinations included echocardiography and 24-hour ambulatory electrocardiogram. Comparing all patients and controls, there were no significant differences in HRV parameters. Analyzing subgroups of patients younger than 10 years old, two of the HRV parameters were significantly different compared to controls. For the group of patients older than 10 years, eight of the HRV parameters were significantly reduced. Most significant differences were found regarding low-frequency range (p &lt 0.008) and high-frequency range p &lt 0.008. This study confirms the finding of earlier studies that patients with Fontan circulation have a reduced HRV, and our findings indicate that there is a progressive reduction of HRV over time. Author Affiliation: (1) Department of Clinical Sciences, Paediatrics, Section of Paediatric Cardiology, Umea University Hospital, S-901 85 Umea, Sweden (2) Department of Surgery and Perioperative Science, Umea University Hospital, S-901 85 Umea, Sweden Article History: Registration Date: 01/01/2003 Online Date: 20/01/2004

Byline: N. T. Ross-Ascuitto (1), J. J. Joyce (1), A. Z. M. Arif Hasan (1), R. J. Ascuitto (1) Keywords: Myocardial performance; Chronic hypoxia; Immature hearts; Contracture Abstract: Hearts isolated from 30 rabbits, raised from birth to ~5 weeks of age under either hypoxic (FIO.sub.2, 0.10) or normoxic (FIO.sub.2, 0.21) conditions, underwent retrograde aortic perfusion using a nonrecirculating, well-oxygenated crystalloid solution. The left ventricular end diastolic pressure was initially set at ~5 mmHg. Aerobic performance was studied by measuring peak systolic pressure (PSP), coronary flow, glucose oxidation, and oxygen consumption. Anaerobic function was assessed by determining time for the onset of contracture (TOC) in the presence of zero coronary flow. Hypoxic vs normoxic hearts (mean +- SEM): heart rate, 197 +- 6 vs 190 +- 5 beats per minute PSP, 136 +- 4* vs 108 +- 4 mmHg (+) dP/dt .sub.max, 2294 +- 125* vs 1549 +- 144 mmHg/sec relaxation time constant (Tau), 26.9 +- 1.1* vs 41.6 +- 4.8 msec (-) dP/dt .sub.max, 1422 +- 43* vs 1001 +- 63 mmHg/sec coronary flow, 86.3 +- 4.2* vs 59.9 +- 2.9 ml/min/g.sub.dry glucose oxidation, 3511 +- 118* vs 2979 +- 233 nmol/min/g.sub.dry oxygen consumption, 28.2 +- 1.4* vs 22.7 +- 1.4 Aumol/min/g.sub.dry and TOC, 11.8 +- 1.2* vs 22.9 +- 2.2 min (*p &lt 0.05). Hearts isolated from young rabbits, exposed to hypoxia from birth, exhibited enhanced ventricular systolic and diastolic mechanical function, elevated coronary flow, retained capacity for aerobic metabolism, and a shorter TOC compared to their normoxic counterparts. Author Affiliation: (1) Department of Pediatric Cardiology, Tulane University School of Medicine, 1430 Tulane Avenue, New Orleans, LA 70112, USA Article History: Registration Date: 01/01/2003 Online Date: 28/01/2004

Byline: H. Isaacs (1) Keywords: Fetal cardiac tumors; Neonatal cardiac tumors; Tuberous sclerosis Abstract: Primary tumors of the heart are uncommon in the fetus and neonate. Nevertheless, the widespread use of new imaging techniques has contributed significantly to earlier diagnosis, treatment, and thus improved survival. The clinical findings, imaging studies, pathology, and outcome of 224 fetuses and neonates with cardiac tumors collected from the literature are evaluated and discussed. Most tumors are benign, and of these rhabdomyoma is the most common, followed by teratoma, fibroma, oncocytic cardiomyopathy, vascular tumors, and myxoma. Malignant and metastatic tumors are described but are rare. Murmurs, arrhythmias, cyanosis, respiratory distress, and cardiac failure are the main presenting signs of cardiac tumors in the perinatal period. Disturbances in hemodynamic function are correlated with the size and location of the tumor. Cardiac vascular tumors have the best outcome, whereas malignant tumors have the worst. The purpose of this review is to concentrate on the fetus and neonate in an attempt to determine the various ways cardiac tumors differ clinically and morphologically in this age group from those occurring in older children and adults and to show that certain types of tumors have a better prognosis than others. Author Affiliation: (1) Department of Pathology, Children's Hospital San Diego, 3020 Children's Way, MC 5007, San Diego, CA 92123 and University of California San Diego School of Medicine, La Jolla, CA 92093-0612, USA Article History: Registration Date: 01/01/2003 Online Date: 19/04/2004

Byline: S. Sanatani (1), C. Chiu (1), D. Nykanen (1), J. Coles (2), L. West (1), R. Hamilton (1) Keywords: Cardiac transplantation; Heart rate variability; Congenital heart disease Abstract: In cardiac transplantation, the donor organ is not initially innervated and demonstrates decreased heart rate variability (HRV). However, HRV may improve after several months. The mechanism for HRV improvement has not been elucidated autonomic 'reinnervation' of the donor heart has been proposed. The role of atrioatrial conduction from recipient to donor organ has not been evaluated. We prospectively evaluated cardiac transplant patients with a limited electrophysiology study at the time of their surveillance biopsies. Recordings were made of recipient and donor signals, observing conduction properties between recipient and donor atria. Holter recordings were analyzed and HRV was determined using spectral analysis techniques, recording mean RR interval, low-frequency power (LF), high-frequency power (HF), and the LF/HF ratio. These were compared to published norms. From November 1999 to May 2000, 21 patients (6 female) who underwent cardiac transplantation participated at a median age of 101 months (range, 4.1--217 months). Time posttransplant ranged from 26 days to 71 months. Holter data were available for 20 patients and demonstrated dissociated P waves in 13 (65%). The mean heart rate on Holter was 111 beats per minute (bpm) (range, 85--161 bpm). We were able to record distinct recipient atrial signals in 16 of 21 (76%) patients. The average recipient tissue heart rate was 55% that of the donor heart rate. We documented atrioatrial association in only 1 patient. HRV did not reach normal values for most patients and did not increase with time posttransplantation. The LF values were in the normal range for most patients, whereas 3 patients had normal HF values and 2 patients had values just below normal. Recipients of heart transplantation have a predominantly sympathetic influence of HRV. These preliminary data suggest that atrioatrial conduction does not play a role in reestablishing normal heart rate control following pediatric cardiac transplantation. Author Affiliation: (1) Division of Cardiology, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada (2) Division of Cardiovascular Surgery, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada Article History: Registration Date: 01/01/2003 Online Date: 04/12/2003

Byline: Ayush Dagvadorj (1), Montse Olive (2), Jean-Andoni Urtizberea (3), Martin Halle (4), Alexey Shatunov (1), Carsten Bonnemann (5), Kye-Yoon Park (1), Hans H. Goebel (6), Isidro Ferrer (2), Patrick Vicart (7), Marinos C. Dalakas (1), Lev G. Goldfarb (1) Keywords: cardiomyopathy; skeletal myopathy; desmin gene; de novo mutation Abstract: Abstract. Desminopathy is a familial or sporadic cardiac and skeletal muscular dystrophy associated with mutations in desmin. We have previously characterized a de novo desmin R406W mutation in a patient of European origin with early onset muscle weakness in the lower extremities and atrioventricular conduction block requiring a permanent pacemaker. The disease relentlessly progressed resulting in severe incapacity within 5 years after onset. We have now identified three other patients with early onset rapidly progressive cardiac and skeletal myopathy caused by this same desmin R406W mutation. The mutation was present in each studied patient, but not in their parents or other unaffected family members, indicating that the mutation in all four cases was generated de novo. The patients' mutationcarrying chromosomes showed no similarity, suggesting that the R406W mutation has occurred independently. These observations strongly confirm that the de novo R406W desmin mutation is the genetic basis for early-onset cardiac and skeletal myopathy in patients with sporadic disease and indicate that desmin position 406 is a hot spot for spontaneous mutations. The high pathogenic potential of this mutation can be explained by its location in the highly conserved YRKLLEGEE motif at the C-terminal end of the 2B helix that has a critical role in the process of desmin filament assembly. Author Affiliation: (1) National Institute of Neurological Disorders and Stroke, National Institutes of Health Bldg 10, Room 4B37, 10 Center Dr., MSC 1361, Bethesda, Maryland, 20892-1361, USA (2) Institut de Neuropatologia Ciutat Sanitaria i Universitaria de Bellvitge, Hospitalet de Llobregat, Barcelona, Spain (3) Hopital Raymond Poincare, 92380, Garches, France (4) Medizinische Klinik, Georg-August-Universitaet, 37075, Goettingen, Germany (5) Division of Neurology, The Children's Hospital of Philadelphia, Philadelphia, PA, 19104, US (6) Department of Neuropathology, Johannes Gutenberg-University, Medical Center, Langenbeckstrasse 1, 55131, Mainz, Germany (7) Laboratoire Cytosquelette et Developpement, UMR CNRS 7000 Faculte de Medecine Pitie-Salpetriere, 105 Bd de l'Hopital, 75013, Paris, France Article History: Registration Date: 01/01/2004 Received Date: 05/03/2003 Accepted Date: 18/09/2003

Abstract. Flow energy dissipation reduces cardiac efficiency, particularly in the Fontan-modified, single-ventricle heart. To provide insight into flow energetics relevant to Fontan-type anatomy, a simple, analytical description of fluid motion [...]

Byline: Esther de Beus (1), Walther N. K. A. van Mook (1), Graham Ramsay (1), Jan L. M. Stappers (3), Hans W. H. M. van der Putten (4) Keywords: Peripartum Cardiomyopathy Intensive care African Eclampsia Echocardiography Abstract: We use an illustrative case of severe peripartum cardiomyopathy with congestive heart failure to introduce this topic and proceed to cover its pathophysiology, incidence, management and outcome. Author Affiliation: (1) Department of Intensive Care Medicine, University Hospital Maastricht, P.O. Box 5800, 6202 AZ, Maastricht, The Netherlands (2) Department of Internal Medicine, University Hospital Maastricht, 6202 AZ, Maastricht, The Netherlands (3) Department of Cardiology, University Hospital Maastricht, 6202 AZ, Maastricht, The Netherlands (4) Department of Gynecology, University Hospital Maastricht, 6202 AZ, Maastricht, The Netherlands Article History: Received Date: 08/09/2002 Accepted Date: 24/10/2002 Article note: Electronic Publication

Byline: M. Skeels (), D. Taylor (), J. Park (), M. Parrish (), M. Choy () Abstract: At the University of California Davis Medical Center, a screening fetal ultrasound examination (level I or II) incorporates a comprehensive segmental evaluation of the fetal heart. This study evaluated the reliability of the fetal ultrasound exam in the detection of abnormal heart anatomy. Our retrospective study reviewed results of 614 antenatal patients that had a screening fetal ultrasound exam. All patients subsequently underwent a detailed targeted fetal cardiac ultrasound exam performed by a pediatric cardiac sonographer and reviewed by a board-certified pediatric cardiologist. Of these 614 patients, 60 fetuses had structural heart disease by the targeted fetal exam. The screening fetal ultrasound exam correctly identified 55 of the 60, with 5 false negatives (8.3% false-negative rate) and 1 false positive (1.7% false-positive rate). Our study suggests that if a screening fetal ultrasound exam incorporates a segmental evaluation of the fetal heart it can reliably detect abnormal heart anatomy. At our institution a targeted fetal cardiac exam is now used to confirm and provide detailed assessment of the heart anatomy when a screening fetal exam is positive for heart disease. Author Affiliation: () Division of Pediatric Cardiology, University of California Davis Medical Center, 2516 Stockton Boulevard, Sacramento, CA 95817, USA, US

Byline: S. Pipitone (), G. Giudice (), M. Giuffre (), V. Sperandeo () Abstract: Pulsus alternans was observed in a child with left ventricular dysfunction secondary to systemic hypertension. The Echo-Doppler of supravalvular aorta and a simultaneous electrocardiographic trace (ECG) clearly demonstrated the mechanism of an apparent abrupt halving of pulse frequency compared with the ECG, similar to an electromechanical dissociation 2:1. Besides the extreme pulsus alternans, a P wave and a PP cycle length change were also observed. Both mechanical and electrical alternans disappeared with afterload reduction by nitroprusside treatment. Author Affiliation: () Divisione di Cardiologia, Ospedale Casa del Sole, Via Roccazzo, 83, Palermo, Italy, IT

Byline: J. Soongswang (), K. Durongpisitkul (), S. Ratanarapee (), W. Leowattana (), A. Nana (), D. Laohaprasitiporn (), S. Akaniroj (), N. Limpimwong (), C. Kangkagate () Abstract: This study was conducted to assess the use of the serum cardiac troponin T (cTnT) level as a noninvasive indicator to differentiate acute myocarditis and chronic dilated cardiomyopathy in pediatric patients. Myocarditis and dilated cardiomyopathy are clinically difficult to differentiate. Endomyocardial biopsy proved to be quite useful. However, the nature of the procedure--invasiveness, time-consuming, and limited sensitivity--caused some concerns, especially in pediatric patients. Hence, we attempted to find an alternative method that could give a prompt diagnosis of acute myocarditis. Twenty cases with clinically suspected myocarditis or dilated cardiomyopathy and a control group of 21 cases with moderate left-to-right shunt and congestive heart failure were recruited. History, physical examination, electrocardiogram, chest roentgenogram, echocardiogram, cTnT, creatine kinase MB isoenzyme (CK-MB mass), and/or endomyocardial biopsy were compared. The gold standard used to diagnose myocarditis is endomyocardial biopsy (Dallas criteria) and/or recovery from cardiovascular problems within 6 months of follow-up. Ten patients were diagnosed as having myocarditis (group 1) and 10 with chronic dilated cardiomyopathy (group 2). The control group of 21 cases was designated as group 3. The median serum cTnT levels were 0.088 (0.04--3.11), 0.010 (0.010--0.990), and 0.010 (0.010--0.550) ng/ml in groups 1, 2, and 3, respectively. The mean CK-MB mass level for groups, 1, 2, and 3 were 18.35 (7.14--70.00), 4.80 (0.54--108.00), and 2.26 (0.95--7.06) ng/ml. The study showed that both the cTnT and CK-MB mass levels were significantly higher in group 1 than either group 2 or group 3. Histopathology was studied in 9 cases. In 2 of 5 cases and in all 4 cases in group 1 and group 2 histopathology was pathologically proved. Levels of cTnT and CK-MB were significantly higher for myocarditis than for dilated cardiomyopathy and left-to-right shunt with CHF. Further study is needed to assess the optimum cTnT level for differentiating both conditions. Author Affiliation: () Department of Pediatrics, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand 10700, TH () Department of Pathology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand 10700, TH () Department of Clinical Pathology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand 10700, TH () Her Majesty Cardiac Center, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand 10700, TH

Byline: A. Gerdes (), T. Hanke (), V. Ahrens (), G. Pfister (), H.-H. Sievers () Abstract: Optimally designed cavopulmonary connections are desirable to reduce transanastomotic power loss. Since aplanarity of the total cavopulmonary connections results from the natural anterior--posterior position of the superior vena cava and the right pulmonary artery, the aim of this study was to investigate the influence of aplanarity of caval offset on transanastomotic power losses. Two types of cavopulmonary connections, a planar cross-like connection and a nonplanar V-shaped anastomosis, were studied in a mock circulation, comparing for each type native porcine vessels and Perspex glass models. Total flow varied between 1200 and 6000 ml/min at superior to inferior caval flow ratios of SVC/IVC = 50/50% and 33/67%, respectively. Pressures and flows were measured and power losses calculated. No significant differences in power losses were found between the planar and aplanar connections for native vessels or for Perspex glass models. Power losses across the native preparations in each configuration were significantly higher than those in the corresponding Perspex glass model. The simulation of the natural aplanar V-shaped caval offset in total cavopulmonary connections used to bypass the right ventricle seems to have no relevant additional effect on in vitro power losses compared to planar connections. Author Affiliation: () Department of Cardiac Surgery, Medical University of Lubeck, Ratzeburger Allee 160, D-23538 Lubeck, Germany, DE () Institute for Applied Physics, Christian-Albrechts-University, Olshausenstr. 40, D-24098 Kiel, Germany, DE

Byline: G. Arsos (), E. Moralidis (), N. Karatzas (), I. Iakovou (), S. Georga (), D. Koliouskas (), G. Langazalis (), C. Karakatsanis () Abstract: Left ventricular diastolic filling is a fundamental constituent of cardiac performance. Diastolic function in both adults and children can be routinely assessed by radionuclide ventriculography (RNV). It has previously been shown that factors such as heart rate (HR) and age can significantly modify diastolic performance in adults, thus limiting the clinical applicability of RNV diastolic indices. The aim of this study was to investigate various factors that may affect diastolic function in childhood. Seventy-nine children, aged 40 days to 15 years, were enrolled in the study their HR ranged from 45 to 160 beats per minute (bpm). All had intact cardiac function and were submitted to baseline RNV prior to chemotherapy initiation for malignancies. Using stepwise linear regression analysis, HR was identified as the major factor affecting RNV diastolic indices during growth. Applying univariate regression models, diastolic indices were corrected for a referrence HR of 100 bpm this substantially reduced variability of RNV diastolic indices along age increments, allowing for the establishment of reference ranges. In conclusion, HR was shown to be the major determinant of RNV diastolic indices during growth. Adjustment for this variable alone can offer reference ranges for the assessment of left ventricular filling in childhood. Author Affiliation: () Department of Nuclear Medicine, Aristotle University Medical School, Hippokration Hospital, Thessaloniki, Greece, GR () Department of Pediatric Oncology, Hippokration Hospital, Thessaloniki, Greece, GR

Byline: T. Kohl () Abstract: Author Affiliation: () Department of Obstetrics and Gynecology, Division of Prenatal Medicine, University of Lubeck Medical School, Ratzeburger Allee 60, 23538 Lubeck, Germany, DE

Byline: Raul Altman (1), Jorge Rouvier (1), Alejandra Scazziota (1), Claudio Gonzalez (2) Keywords: C-reactive protein; fibrinogen; Chlamydia pneumoniae; coronary disease; cardiac prosthesis Abstract: The C-reactive protein, Chlamydia-specific IgG antibody, and fibrinogen were assayed in the serum of 159 patients with arterial disease (the arterial group) and 203 patients with heart valve prostheses (the valvular group) and no demonstrable coronary disease. In the arterial group, the Chlamydia pneumoniae antibody was aY=1 : 32 for 67.3% (107/159) of the patients, the C-reactive protein was elevated in 41.5% (66/159), and the fibrinogen was elevated in 27.7% (44/159). In the valvular group, the C. pneumoniae antibody was aY=1 : 32 for 59.1% (120/203) of the patients the C-reactive protein was elevated in 34.0% (69/203), and the fibrinogen was elevated in 17.2% (35/203). Of 107 patients in the arterial group with C. pneumoniae titers aY=1 : 32, only 26 (24.3%) had elevated fibrinogen (426 +- 29 mg/dL) and 44 (41.1%) had elevated C-reactive protein (1.06 +- 0.52 mg/dL). Similarly, of the 120 patients in the valvular group with C. pneumoniae titers aY=1 : 32, 17 (14.2%) had elevated fibrinogen (409 +- 29 mg/dL) and 34 had elevated C-reactive protein (0.99 +- 1.1 mg/dL). Correlated poorly was C. pneumoniae with C-reactive protein and fibrinogen levels. Only the fibrinogen level could be discriminated between the arterial and the valvular group. These results suggest that no causal association exists between inflammation and C. pneumoniae. A highly significant correlation between C-reactive protein and fibrinogen levels was found. Author Affiliation: (1) Centro de Trombosis de Buenos Aires, Viamonte 2008, 1056, Buenos Aires, Argentina (2) Department of Pharmacology, School of Medicine, University of Buenos Aires, Argentina Article History: Registration Date: 12/10/2004

Byline: P.C. Frommelt (), E.N. Whitstone (), M.A. Frommelt () Abstract: A digital pediatric echocardiography laboratory, without videotape redundancy was established at Children's Hospital of Wisconsin in December 1998. To characterize the experience, 1198 consecutive patient studies were reviewed-50% from the first 2 months after establishing the digital protocol and 50% from the last 2 months available. Each study was stored using a protocol that was based on capture of single beat clips of relevant two-dimensional/color Doppler imaging and static frame spectral Doppler tracings. Studies were digitally compressed using a DICOM-compatible JPEG algorithm at 20:1 and edited with deletions of redundant clips to minimize archival storage needs. Study quality was uniformly excellent, and no errors were attributable to the digital protocol or compression-related loss of information. The average study required 21.5 +- 11.4 MB of storage space with 35.4 +- 12.3 total clips/study captured. Studies reviewed from the earlier experience were not significantly larger (23.6 +- 14 vs 19.7 +- 8.1 MB, 35.6 +- 12.5 vs 35.2 +- 12 clips) than those done recently. Studies in patients with isolated ventricular septal defect used comparable storage (23.7 +- 8.9 MB, 42.8 +- 11.5 clips) to that of the group as a whole. More complex congenital heart disease studies were slightly larger-tetralogy of Fallot (28.2 +- 19.5 MB, 43.4 +- 13.9 clips), transposition of the great arteries (30.6 +- 17.4 MB, 40.3 +- 16.7 clips), and single ventricle (29.7 +- 19.6 MB, 39.9 +- 12 clips)-although this trend was not significant. This study suggests that digital pediatric echo is feasible using a DICOM-compatible protocol with maintenance of diagnostic integrity despite compression of study size to allow rapid archival storage and retrieval. Author Affiliation: () Division of Pediatric Cardiology, Department of Pediatrics, Children's Hospital of Wisconsin, Medical College of Wisconsin, 9000 W. Wisconsin Avenue, Milwaukee, WI 53226, USA, US

Byline: M.T. Donofrio (), D.R. Allen (), M. Tekin (), J. Bodurtha () Abstract: We describe an inherited form of a disorder in which four patients spanning three generations were affected with congenital myocardial disease. The youngest member of the family, diagnosed as a fetus with a large aneurysm of the muscular interventricular septum, demonstrates an antenatal pathogenic process. Study of the specific findings in each patient suggests that congenital aneurysms of the muscular interventricular septum may be in some way associated with specific developmental pathways of the heart. We believe that screening family members of patients with muscular interventricular septal aneurysms may be indicated to assess for silent myocardial disease. Author Affiliation: () Department of Pediatrics, Division of Pediatric Cardiology, The Medical College of Virginia Hospitals of the Virginia Commonwealth University, Box 980342, Richmond, VA 23298, USA, US () Department of Human Genetics, The Medical College of Virginia Hospitals of the Virginia Commonwealth University, Box 980342, Richmond, VA 23298, USA, US

Byline: G. Vaksmann (), R. Gutierrez (), A. Duhamel (), B. Nelken (), C. Francart (), C. Kouakam (), F. Mazingue (), C. Rey () Abstract: The aim of the present study was to determine if signal-averaged ECG of patients with anthracycline-induced left ventricular dysfunction could differentiate between patients with anthracycline-induced cardiotoxicity and those without. Sixteen children with anthracycline-induced cardiomyopathy, aged 6.5 to 15.5 years (anthracycline dose = 198-737 mg/m2), and 31 patients aged 5.0 to 16.7 years, who received anthracyclines without evidence of left ventricular dysfunction (anthracycline dose = 120-517 mg/m2), were studied with signal averaged ECG. The two groups were comparable in age, body surface area, and time since completion of chemotherapy. Signal averaged ECG parameters of the patients were compared with data obtained from 530 healthy children. These parameters were converted to z-scores to account for growth-related changes in signal averaged ECG recordings. Z-scores for filtered QRS duration and low amplitude terminal signal &lt 40 mV were significantly lower (p = 0.002 and p = 0.015, respectively), and Z-score for root mean square voltage of the last ?30 ms of filtered QRS tended to be higher (p = 0.06) in patients with left ventricular dysfunction. Filtered QRS duration lower than ?1.5 SD was found in 4 of 16 patients with left ventricular dysfunction and in only 1 of 31 patients without (p &lt 0.05) yielding a sensitivity of 25% and a specificity of 97% to detect left ventricular dysfunction. Only 1 patient had late potentials his left ventricular function was normal. Left ventricular mass index tended to be lower in patients with left ventricular dysfunction (p = 0.07), whereas left ventricular diastolic diameter was similar in the two groups. The mechanism that accounted for the difference in signal averaged-ECG between the two groups of patients could be linked with the decrease in left ventricular mass in patients with left ventricular dysfunction. In conclusion, children with left ventricular dysfunction following anthracycline therapy have a SA ECG different from those without left ventricular dysfunction, which is mainly characterized by a lower filtered QRS duration. A prospective study is needed in order to determine if this modification of SA ECG recordings precedes alteration of left ventricular function, and, therefore, if it could help in early detection of cardiac toxicity of anthracyclines. Author Affiliation: () Department of Pediatric Cardiology, Cardiologic Hospital, F59037, Lille, France, FR () Department of Biostatistics, University of Lille II, Lille, France, FR () Department of Pediatric Hematology, Jeanne de Flandre Hospital, F59037, Lille, France, FR

Byline: Alyson M. Booth (1), Robin D. LeGallo (1), Mark H. Stoler (1), Peter E. Waldron (2), Lisa A. Cerilli (1) Keywords: Key words: heart, angiosarcoma, malignant hemangioendothelioma, metastatic, pediatric, ovary, vascular tumor, sarcoma, cardiac Abstract: We report the seventh case of angiosarcoma of the heart in a child. The patient was a 23-month-old female who presented for lower extremity limping and underwent open surgical biopsy of the femur. Immediately postoperatively, she developed pericardial tamponade, and a bulky intracardiac mass was discovered as the underlying cause. The mass was composed of highly pleomorphic tumor cells reactive for the endothelial markers CD31, CD34, and factor VIII--related antigen (FVIII-RA). Staging evaluation revealed widespread metastases involving the brain, ovaries, and bone marrow. She died of complications of metastatic disease 8 months following initial presentation. Unusual features of this case include the young age of the patient, left-sided nature of the cardiac tumor, presentation secondary to metastatic disease, and the pattern of metastases. The literature on cardiac angiosarcoma, which is limited to six case reports in the pediatric population, is also reviewed. Author Affiliation: (1) Department of Pathology, University of Virginia Health System, Box 800214, Charlottesville, VA 22908, USA, US (2) Department of Pediatrics, University of Virginia Health System, Box 800386, Charlottesville, VA 22908, USA, US Article note: Received September 15, 2000 accepted February 9, 2001.

Byline: S.A. Subahi (1) Keywords: Key words: Salih congenital muscular dystrophy -- Electrocardiography -- Echocardiography -- Multigated acquisition scan Abstract: The cardiac features of a novel form of congenital muscular dystrophy (Salih CMD) are described in two adolescent siblings. The patients presented with severe hypotonia at birth, associated with delayed development. They could walk independently and managed to maintain walking after 13 years of age. Their muscle immunohistochemistry differed from that seen in Duchenne and Becher muscular dystrophy (DMD and BMD), severe childhood autosomal recessive muscular dystrophy (SCARMD) due to sarcoglycan deficiency (sarcoglycanopathies), and laminin[alpha]2 (merosin)-deficient CMD. However, both patients had associated cardiomyopathy. Electrocardiography (ECG) in Salih CMD was characterized by delayed atrioventricular (AV) conduction, left anterior fascicular block (left axis deviation), and left atrial enlargement without evidence of atrial dysarrhythmia. Echocardiography showed features of severe left ventricular dysfunction with estimated left ventricle ejection fraction (LVEF) of 25% at 16 years-of-age in the older patient. A year later, multigated aquisition MUGA scan showed LVEF of 21% and dilatation of the right ventricle. Echocardiography and MUGA scan were normal in the younger patient at 15 years-of-age. ECG, echocardiography, and MUGA scan are effective techniques for diagnosing and monitoring the cardiomyopathy in Salih CMD. They can also distinguish it from features seen in the other common forms of MD, including DMD, BMD, and sarcoglycanopathies. Author Affiliation: (1) Division of Cardiology, Department of Medicine, King Khalid University Hospital, Riyadh, Saudi Arabia, SA

Byline: D.B. McElhinney (1), A.K. Hoydu (1), J.W. Gaynor (1), T.L. Spray (1), E. Goldmuntz (1), P.M. Weinberg (1) Keywords: Key words: Vascular ring -- Right aortic arch -- Bilateral ductus arteriosus -- Cardiovascular development Abstract: It is unusual to have a right aortic arch with mirror-image branching of the brachiocephalic vessels and no associated congenital cardiac anomalies. Pathogenetic factors of this anomaly, and associated development of the derivatives of the pharyngeal arches, have not been explored extensively. Eleven patients with a mirror-image right aortic arch and no intracardiac anomalies have undergone evaluation at our institution since 1987. Of these, 4 had stenosis (n= 2) or atresia (n= 2) of the proximal left pulmonary artery. These included 2 with bilateral ductus arteriosus. Six patients had a vascular ring formed by a ductus arteriosus or ligamentum arteriosum from the descending aorta to the left pulmonary artery. Six patients were evaluated for deletion within the DiGeorge critical region of chromosome 22q11. All 6 of the patients with a vascular ring underwent division of the ductus arteriosus or ligamentum through a left lateral thoracotomy. None of the patients with stenosis or atresia of the LPA have undergone intervention. Two of the 6 patients who underwent evaluation for chromosome 22q11 deletion were found to have the deletion. At a median follow-up of 7 years (15 months to 12 years), the patients who underwent division of the ring are all alive and asymptomatic. Two of these patients have undergone repeat magnetic resonance imaging, 1 of whom had mild residual tracheal compression. A right aortic arch with mirror-image branching of the brachiocephalic vessels and no associated intracardiac anomalies is extremely uncommon. We have observed two basic patterns of this lesion: (1) with associated abnormalities of the proximal left pulmonary artery, (2) with a vascular ring formed by a ductus arteriosus or ligamentum from the descending aorta to the left pulmonary artery. Pathogenetic considerations are explored, with a focus on flow-related vascular development and the role of deletions within chromosome 22q11. Author Affiliation: (1) Divisions of Pediatric Cardiology and Pediatric Cardiothoracic Surgery, The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Room 9557, and University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA, US

Byline: Manuel Ruiz-Bailen (1), Asuncion Lopez-Martinez (2), Jose Angel Ramos-Cuadra (1), Miguel Angel Diaz-Castellanos (1), Antonio Cardenas-Cruz (1), Manuel Rodriguez-Elvira (3), Angel Montiel-Trujillo (2) Keywords: Peripartum cardiomyopathy Dilated cardiomyopathy Myocarditis Pregnancy Idiopathic cardiomyopathy Abstract: Objectives: We describe our experience with peripartum cardiomyopathy. Design and setting: A case series in intensive care units (ICU) of a district hospital and a referral center. Patients: Six patients who required admission to an ICU after the onset of peripartum cardiomyopathy. Results: Five of the six patients survived, with total recovery of ventricular function. After 1 year of follow-up all five survivors were symptom free with a normal ventricular function. Conclusions: There is a low rate of ICU admissions for peripartum cardiomyopathy, which has a potentially fatal prognosis. However, this disease can be detected by echocardiography among patients without the semiology. Author Affiliation: (1) Intensive Care Unit, Critical Care and Emergencies Department, Hospital de Poniente, El Ejido, Almeria, Spain (2) Cardiology Unit, Hospital de Poniente, El Ejido, Almeria, Spain (3) Intensive Care Unit, Critical Care and Emergencies Department, Virgen de las Nieves University Hospital, Granada, Spain (4) Mailing address: C/. Las Torres 57, 23650 Torredonjimeno, Jaen, Spain, e-mail: MRB1604@teleline.es, Tel.: +34-950-572020, Fax: +34-950-570702 Article History: Received Date: 08/06/2000 Accepted Date: 02/11/2000 Article note: Final revision received: 15 September 2000 Electronic Publication

Byline: Enid Gilbert-Barness (1), Lewis A. Barness (3) Keywords: Key words: cardiomyopathy, myopathy, metabolic, storage, arrhythmias Author Affiliation: (1) Department of Pathology, University of South Florida, Tampa General Hospital, P.O. Box 1289, Tampa, FL 33601-1289, USA , US (2) Department of Obstetrics and Gynecology, University of South Florida, Tampa General Hospital, P.O. Box 1289, Tampa, FL 33601-1289, USA , US (3) Department of Pediatrics, University of South Florida, 17 Davis Boulevard, Tampa, FL 33601, USA , US Article note: Received February 6, 1998 accepted May 24, 1999.

Byline: Elizabeth A. Leistikow (0), Robert P. Bolande (0) Keywords: Key words: atherosclerosis, atheroma precursors, coronary arteriosclerosis, human newborn, infant, fetus, smooth muscle, perinatal stress Author Affiliation: (0) Department of Pathology and Laboratory Medicine, East Carolina University School of Medicine, Brody Building, Greenville, NC 27858-4354, USA , US Article note: Received August 1, 1998 accepted August 20, 1998.

Byline: Ricardo Drut (1), Rosa Monica Drut (1), M. Alba Greco (2) Keywords: Key words: calcification, heart, myocardium, non-immune hydrops Abstract: Massive myocardial calcification (MMC) in the perinatal period is an unusual finding considered to be a unique tissue reaction. This report summarizes the clinical and pathologic findings of seven cases of perinatal MMC. All patients presented clinical evidence of myocardial damage. In two cases arrhythmia was detected in utero. Four cases presented with hydrops, one of which was associated with major heart malformation. One case was a trisomy 13. Three cases had polyhydramnios. Our results demonstrate that calcification follows progressive stages from patches of calcified myocardial cells (stage I), to coagulative and colliquative myocytolysis with clusters of interstitial mononucleated cells (stage II), to collapsing fibrosis with granulation tissue and multinucleated regenerative myocardial cells (stage III), and finally to fibrous scars containing entrapped remaining myocardial cells (stage IV). Literature review and our findings suggest that perinatal MMC results from different conditions inducing hypoxic--ischemic damage that later is followed by progressive scarring if the patient survives the acute stage. The lesion may represent the human counterpart of the so-called dystrophic cardiac calcinosis in mice. This disease is related to an abnormality at the Dyscalc locus of proximal chromosome 7 (syntenic with human chromosome 19q13 and 11p15). Author Affiliation: (1) Department of Pathology, Hospital de Ninos 'Sor Maria Ludovica,' 1900 La Plata, Argentina , AR (2) Department of Pathology, New York University--Bellevue Hospital Medical Center, 462 First Avenue, Room 4 North 22, New York, NY 10016, USA , US Article note: Received June 4, 1997 accepted November 13, 1997.

Byline: R. M. Grocott-Mason (1), A. M. Shah (1) Author Affiliation: (1) Department of Cardiology, University of Wales College of Medicine, Heath Park, Cardiff CF4 4XN, UK, GB Article note: Received: 6 August 1997 Accepted: 16 September 1997

Byline: R. Braams (1), H. P. F. Koppeschaar (2), H. D. W. M. van de Pavoordt (3), T. J. M. V. van Vroonhoven (1) Keywords: Key words Cortisol; ACTH; Synacthen test; Ruptured abdominal aortic aneurysm; Adrenocortical insufficiency Abstract: Objective: To investigate adrenocortical function in patients with ruptured aneurysm of the abdominal aorta. Design: Prospective clinical investigation. Setting: Surgical intensive care unit in a university teaching hospital and intensive care unit in a general hospital. Patients and participants: 54 patients with a documented rupture of the abdominal aorta. Interventions: A short adrenocorticotrophic hormone (ACTH) stimulation test was performed. Measurements and results: Patients were studied within 24 h of admission to the hospital. Blood samples for the measurement of cortisol and ACTH were collected at 0800 h. Subsequently 0.25 mg tetracosactrin (Synacthen) was injected i. v. and after 60 min cortisol measurement was repeated. The criterion for a normal short ACTH test was: stimulated or unstimulated cortisol levels aY= 0.55 umol/l. For the group as a whole, an unstimulated plasma cortisol level of 0.76 umol/l was comparable to that in other groups of critically ill patients with similar severity of illness. Between survivors and non survivors, significant differences were found between unstimulated plasma cortisol levels (0.70 vs 1.03 umol/l), stimulated plasma cortisol levels (1.00 vs 1.30 umol/l), and plasma ACTH levels (72 vs 133 ng/l). One patient did not meet the criteria for normal adrenocortical function: unstimulated plasma cortisol 0.26 umol/l, stimulated plasma cortisol 0.47 umol/l. Conclusions: In the patients studied with ruptured aneurysm of the abdominal aorta, adrenocortical response was comparable to that in other groups of critically ill patients with similar severity of illness. High cortisol levels were associated with mortality. One patient did not meet the criteria for normal adrenocortical function but survived without steroid treatment. Author Affiliation: (1) Department of Surgery, University Hospital Utrecht, The Netherlands, NL (2) Department of Endocrinology, University Hospital Utrecht, The Netherlands, NL (3) Department of Surgery, Antonius Hospital Nieuwegein, The Netherlands, NL Article note: Received: 27 January 1997 Accepted: 24 September 1997

Byline: Friedrich C. Luft (1) Author Affiliation: (1) Franz Volhard Clinic, HELIOS Kliniken Berlin, Medical Faculty of the Charite, Humboldt University, Wiltbergstrasse 50, 13125, Berlin-Buch, Germany Article History: Registration Date: 17/12/2003 Online Date: 23/01/2004

Byline: K. A. Milhoan (1), D. J. Levy (1), N. Shields (1), A. Rothman (1) Keywords: Pulmonary artery pressure; Fontan procedure Abstract: After the bidirectional cavopulmonary or Fontan operation, the physiologic consequence is passive flow of the systemic venous return to the pulmonary arteries. Knowledge of pulmonary artery pressure (PA) is valuable in the management of these patients, and obtaining this information without the need for a central line or cardiac catheterization would be advantageous. The aim of this study was to evaluate the correlation between upper extremity peripheral venous (PV) pressures and PA or superior vena cava (SVC) pressures in patients who have undergone cavopulmonary or Fontan connections. During cardiac catheterization, 19 patients with complex cyanotic heart disease who had undergone a cavopulmonary shunt or Fontan procedure were studied. Simultaneous pressure measurements were obtained from the peripheral intravenous line placed prior to the procedure and the SVC or PA. The mean pressures were compared. The mean PV pressure was 17.5 +- 5.6 mmHg. The mean SVC or PA pressure was 16.1 +- 5.4 mmHg. The mean difference was 1.5 +- 1.5 mmHg (p &lt 0.001). The correlation coefficient PV to SVC or PA pressure was 0.97 (p &lt 0.001). PV pressure measurements taken from an upper extremity accurately reflect PA pressures in patients who have undergone a cavopulmonary shunt or Fontan procedure. Author Affiliation: (1) Division of Pediatric Cardiology, Children's Hospital San Diego and the University of California, San Diego, CA 92123, USA Article History: Registration Date: 01/01/2003 Online Date: 04/09/2003

Byline: M.S. Horenstein (), R.D. Ross (), T.P. Singh (), M.L. Epstein () Abstract: There is little experience using b-blocker therapy for the management of congestive heart failure (CHF) in children. We present the case of a child with idiopathic dilated cardiomyopathy and CHF, referred for cardiac transplantation, in whom carvedilol reversed elevated pulmonary vascular resistance. Author Affiliation: () Division of Cardiology, Children's Hospital of Michigan, Wayne State University School of Medicine, 3901 Beaubien Boulevard, Detroit, MI 48201, USA, US

Byline: T.S. Hornung (1), A. Heads (1), A.S. Hunter (1) Keywords: Key words: Fetal echocardiography -- Right ventricular dilatation Abstract: Right ventricular dilatation is an infrequent finding at fetal echocardiography. Previous studies have documented an association with aortic coarctation. However, there are associations with other congenital abnormalities. We reviewed our experience of fetal right heart dilatation in order to recognize concurrent anomalies and to assess the outcome of the affected fetuses. We studied all fetuses with right ventricular dilatation over a 5-year period. We documented associated cardiac and noncardiac lesions, and outcome data were assessed in terms of the number of fetuses that were born live and the number surviving to 2 months of age. Forty-three fetuses with right heart dilatation were seen. Fifteen had associated cardiac abnormalities: most commonly coarctation (n= 4) and VSD (n= 4). Seven had associated noncardiac abnormalities. There were seven fetuses who also had chromosomal abnormalities. In total, there were three terminations of pregnancy, four intrauterine deaths, one stillbirth and 35 live-births. Twenty-eight were alive at 2 months of age (70% of the nonterminated pregnancies). Fetal right heart dilatation is frequently associated with both cardiac and noncardiac congenital abnormalities. Our outcome figures suggest a guarded prognosis be given during counseling of parents of fetuses with right heart dilatation. Author Affiliation: (1) Department of Pediatric Cardiology, Freeman Hospital, Newcastle upon Tyne, UK, GB

Byline: J. Kusa (1), A. Pyrkosz (1), A. Skiba (1), M. Szkutnik (1) Keywords: Carbohydrate-deficient glycoprotein syndrome; Cardiomyopathy; Pericarditis Abstract: A 2-month-old girl with exudative pericarditis and hypertrophic obstructive cardiomyopathy is presented. The child had characteristic dysmorphic features of carbohydrate-deficient glycoprotein syndrome, which was confirmed by serum levels of carbohydrate-deficient transferrin. Author Affiliation: (1) Department of Congenital Heart Defects and Pediatric Cardiology, Silesian Centre for Heart Disease, 41-800 Zabrze, ul. Szpitalna 2, Poland Article History: Registration Date: 01/01/2002 Online Date: 14/03/2003 Article note: Present address: A. Pyrkosz, Department of General and Molecular Biology and Genetics, Silesian Medical University, 40-752 ul. Medykow 16, Katowice, Poland

Byline: O. Cakir (), N. Eren (), N. Kilinc () Abstract: Cardiac hydatic cyst is rarely encountered. In this article, a case of hydatid cyst localized in the left ventricle causing cerebral emboli is reported. Author Affiliation: () Department of Thoracic Cardiovascular Surgery, Dicle University Medical Faculty, Diyarbakir, Turkey, TR () Department of Pathology, Dicle University Medical Faculty, Diyarbakir, Turkey, TR

Byline: T. Hishitani (1), K. Ogawa (1), K. Hoshino (1), S. Kido (1), Y. Nakamura (2), Y. Ogawa (3) Keywords: Key words: Cardiac tumor -- Fibrosarcoma -- Myxoma Abstract: Left atrial tumor, diagnosed as myxoma at first investigation, was later demonstrated to be a malignant fibrosarcoma with myxoid change. Myxoma should be diagnosed and managed carefully because of its wide range of histopathologic features. Author Affiliation: (1) Department of Cardiology, Saitama Children's Medical Center, Magome 2100, Iwatsuki, Saitama 339-8551, Japan, JP (2) Department of Cardiovascular Surgery, Magome 2100, Iwatsuki, Saitama 339-8551, Japan, JP (3) Department of Pathology, Saitama Children's Medical Center, Magome 2100, Iwatsuki, Saitama 339-8551, Japan, JP

Byline: M. Akcam (1), F.Z. Akcam (2) Author Affiliation: (1) Department of Pediatrics, Suleyman Demirel University, Medical School, Isparta, Turkey (2) Department of Infectious Disease, Suleyman Demirel University, Medical School, Isparta, Turkey Article History: Registration Date: 01/01/2003 Online Date: 12/05/2004