Application of Dual-Source-Computed Tomography in Pediatric Cardiology in Children Within the First Year of Life.

PURPOSE: To assess fields of application and value of dual source computed tomography (DSCT) for diagnostics and therapy in patients with congenital heart disease during their first year of life. Evaluation of image quality, surgical use and radiation exposure of 2nd and 3 rd generation DSCT. MATERIALS AND METHODS: DSCT was applied in 118 cases between January 2012 and October 2014 for diagnostics of congenital heart defects. 2nd generation was used in 91 cases until April 2014 and 3 rd generation in 27 cases during the period thereafter. 3 D reconstructions of the image data were created for clinical diagnostics and planning of interventions. Image quality was assessed using a 4-point-scale. The visibility of the mammary arteries was analyzed, and signal-to-noise-ratio (SNR) and contrast-to-noise-ratio (CNR) were calculated. The usefulness of 3D-reconstructions for surgical planning was rated using a 5-point-scale. Radiation exposure and contrast dye consumption were determined. All cases were analyzed retrospectively. RESULTS: DSCT was successfully used in 118 cases. All image data obtained were interpretable. More than 60 percent of cases did not show any artifacts. The mammary arteries were visible down to the diaphragmatic arch in more than 80 percent of cases. Diagnostic value and surgical benefit were evaluated as "useful" or as "essential" in all cases. Median radiation dose was 0.4 mSv and 0.27 mSv for the 2nd and 3 rd generation DSCT, respectively. Consumption of contrast dye was 2 ml/kg in all cases. CONCLUSION: DSCT is a modern and extremely helpful technique for diagnostics and planning of interventions in patients with complex congenital heart defects. Extracardiac vascular structures in particular can be depicted three-dimensionally at high resolution. The use of iterative reconstruction with 3 rd generation DSCT yielded image quality similar to that of 2nd generation DSCT at considerably reduced radiation exposure level compared to 2nd generation DSCT. 3 rd generation DSCT is a low risk, accurate and extremely fast technique for diagnosing unstable patients with CHD. KEY POINTS: Expanded scope of indications for DSCT in diagnosing critically ill infants. Effective radiation dose is considerably lower than 0.5 mSv. Extremely rapid image acquisitions with high image quality. Possibility of optimized 3D-based surgical planning

Cardiac MRI in Children and Adolescents Who Have Undergone Surgical Repair of Right-Sided Congenital Heart Disease: Automated Left Ventricular Volumes and Function Analysis and Effects of Different Manual Adjustments.

PURPOSE: To evaluate automated segmentation and the effects of different manual adjustments regarding left ventricular parameter quantification in cardiac magnetic resonance (MR) data on children and adolescents who have undergone surgical repair of right-sided congenital heart disease (CHD). MATERIALS AND METHODS: Dedicated software (syngo.via, Siemens AG) was used to automatically segment and/or manually adjust the end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV), myocardial mass (MM) and ejection fraction (EF) before/after manual apex/base adjustment (ADJ-step 1) and after manual apex/base/myocardial contour adjustment (ADJ-step 2; reference standard). MR data of 40 patients (13.1 ± 3. y, 4 - 17 y) with repaired CHD with decreased pulmonary blood flow (CHD-DPBF) were evaluated. Intra- and inter-rater reliability was determined for 10 randomly selected patients. RESULTS: The software correctly detected the left ventricle in 38/40 (95%) patients. EDV after automated segmentation: 119.1 ± 44.0 ml; after ADJ-step 1: 115.8 ± 9.5 ml; after ADJ-step 2: 116.2 ± 39.4 ml. The corresponding results for ESV were 52.0 ± 18.5/49.6 ± 6.9/49.7 ± 16.4 ml; for SV 67.1 ± 28.5/66.2 ± 25.4/66.5 ± 25. ml; for EF 55.5 ± 7.3/56.7 ± 6.6/56.7 ± 6.3%; for MM 83.7 ± 35.9/76.2 ± 8.3/74.6 ± 27.2 g. Significant differences were found for ESV/MM/EF comparing the automated segmentation results with these after ADJ-step 1 and ADJ-step 2. No significant differences were found when comparing all results of ADJ-step 1 and ADJ-step 2 or when comparing EDV/SV results. Intra- and inter-rater reliability was excellent. The mean time effort was 63.4 ± 6.9 s for the automated segmentation, 74.2 ± 8.9 s for ADJ-step 1 and 269.5 ± 39.4 s for ADJ-step 2. CONCLUSION: Automated left ventricular volumes and function analysis in children and adolescents with surgically treated CHD proved to be feasible with excellent intra- and inter-rater reliability. Automated segmentation with manual apex/base adjustment provided clinically acceptable results. KEY POINTS: Automated left ventricular volume and function analysis in children and adolescents with surgically treated right-sided heart disease is feasible with excellent intra- and inter-rater reliability. Automated segmentation with manual apex/base adjustment provides clinically acceptable results. Additional manual myocardial contour adjustment does not significantly improve the results.

Cystatin C and neutrophil gelatinase-associated lipocalin: biomarkers for acute kidney injury after congenital heart surgery.

QUESTIONS UNDER STUDY: To evaluate the diagnostic value of serum Cystatin C and urine neutrophil gelatinase-associated lipocalin (NGAL) for the detection of acute kidney injury in patients undergoing congenital heart surgery. METHODS: Serial samples of serum Cystatin C and urine NGAL were collected from 139 consecutive patients with congenital heart defects aged 3 days to 30 years after admission to the intensive care unit, 2 and 6 hours after the end of cardiopulmonary bypass. Biomarker levels were compared to perioperative data retrospectively. Acute kidney injury was defined according to the paediatric-modified RIFLE classification. RESULTS: According to the paediatric-modified RIFLE criteria 53% of patients developed evidence of acute kidney injury. Serum Cystatin C concentrations were strongly correlated with severity of acute kidney injury. Optimal sensitivity of 80% and specificity of 66% for the prediction of acute kidney injury occurred at a cut-off value of 0.995 mg/l, 2 hours after the end of cardiopulmonary bypass. The 2 hour urine NGAL concentration was significantly correlated to the duration of cardiopulmonary bypass, time of aortic cross clamping, and serum lactate concentration. Moreover a significant correlation was found between urine NGAL and both length of hospital stay and mechanical ventilation. CONCLUSIONS: In patients after congenital heart surgery, urine NGAL indicates the damaging force of cardiopulmonary bypass and serum Cystatin C is a valuable predictive biomarker for resulting acute kidney injury.

Functional null mutations in the gonosomal homologue gene TBL1Y are associated with non-syndromic coarctation of the aorta.

In patients with congenital heart defects, chromosomal anomalies are 100 times more frequent than in control subjects. Coarctation of the aorta can be detected in 15-20% of patients with Ullrich-Turner syndrome. By extensively reviewing literature involving breakpoint analysis of gonosomal deletions in Ullrich- Turner syndrome patients with and without coarctation of the aorta, we identified several gonosomal homolgous gene pairs of interest. Four of these homologous gene pairs were investigated by standard DNA sequencing in a cohort of 83 patients with non-syndromic coarctation of the aorta. Subsequently stability of mutant RNA and protein was analyzed to verify functional relevance of detected mutations. We identified two unreported missense mutations in Exon 8 (p.D69H) and 9 (p.R176W) of TBL1Y. Bioinformatic analysis and 3D modelling predicted that both mutations lead to TBL1Y loss of function. In RT-PCR and Western blot analyses of HEK293 cells transfected with a vector carrying the full-length TBL1Y (wild-type and mutant), we documented the predicted protein instability by showing protein decay for both mutant proteins. TBL1Y is similar to its gonosomal homologue, TBL1X, and its autosomal homologue, TBLR1, on chromosome 3. Both genes are part of co-repressor machineries and required for transcriptional activation by transcription factors that involve CtBP1/2, which contributes to Notch signaling. Several studies have shown that Notch signalling is important for proper development of the left ventricular outflow tract. Our findings suggest that TBL1Y is involved in the genesis of non-syndromic coarctation of the aorta.

Pericardial cyst or teratoma? Change of strategy during mediastinal tumor surgery.

This report describes the management of an 8 × 8-cm cystic mass that arose from the anterior mediastinum and prolapsed into the right pleural cavity adherent to the pericardium in an asymptomatic 16-year-old girl. The patient was scheduled for a video-assisted thoracoscopic operation with exposure, puncture and suction of a suspected pericardial cyst. However, during the procedure the strategy was changed due to the solid consistency of the mass, and the lesion was extirpated in toto by a short anterolateral thoracotomy. The complete histopathological investigation showed a highly fibrous, cystic, mature teratoma.

Aortic arch repair: let it beat!

Objective aortic arch repair (AAR) on the beating heart may reduce cross-clamping times and offer improved postoperative cardiac function.Methods A single-center review of all patients (n = 24) who underwent surgical AAR during biventricular repair between 01/2006 and 01/2008 was done. All patients were operated on under cardiopulmonary bypass (CPB) with antegrade cerebral perfusion (ACP). During AAR, 13 patients (group 1) received cardioplegic arrest, and were compared to 11 patients (group 2) who underwent a beating-heart modification with selective myocardial perfusion. Seventeen patients had additional intracardiac lesions and underwent simultaneous correction during the procedure.Results Durations of CPB, AAR and ACP did not differ statistically between groups. Cardioplegic arrest time was significantly lower in group 1 (34 ± 13 vs. 76 ± 11 min, p = 0.02) and resulted in a subsequent reduction of myocardial ischemic damage as borne out by lower postoperative levels of troponin T and CK-MB (2.5 ± 0.7 vs. 7.1 ± 1.4 ng/mL, p = 0.02; 68.7 ± 11.5 vs. 149.1 ± 27.2 U/l, p = 0.03). We observed an enhanced patient recovery with shorter inotropic and ventilatory support times (p < 0.05).Conclusion Pediatric aortic arch correction on a CPB beating heart with selective myocardial perfusion is technically feasible and safe. The reduction of the myocardial ischemic time is effective and results in less myocardial damage.

The Norwood procedure - does the type of shunt determine outcome?

BACKGROUND: Stage I palliation of hypoplastic left heart syndrome (HLHS) and its variants is usually performed by a Norwood operation. The management of pulmonary blood flow during this procedure remains controversial. The RV-to-PA conduit (RVPAC) has been proposed as the better alternative compared to a systemic-to-pulmonary shunt (SPS). METHODS: A retrospective single center chart review of consecutive patients who underwent a Norwood I procedure between 01/1997 and 09/2006 was performed. All patients were operated in deep hypothermia, with or without circulatory arrest, using different shunt modifications according to surgeon's preference. Patients were divided into two groups depending on surgical management for pulmonary blood flow (modified BT shunt [BT] and non-valved RVPAC [Sano]). RESULTS: Fifty-four patients were included in the study (BT: 31 patients vs. Sano: 23 patients). Diastolic blood pressure during the first 24 hours postoperatively was significantly lower in the BT group (BT: 38.6 +/- 6.9 mmHg vs. Sano: 42.4 +/- 7.2 mmHg; P < 0.01) with a trend towards a higher systolic blood pressure (BT: 74.1 +/- 13.5 mmHg vs. Sano: 69.8 +/- 12.1 mmHg; P = 0.08). Mean circulatory arrest time in the BT group was significantly longer compared to the Sano patients (BT: 41 +/- 21 min vs. Sano: 25 +/- 23 min; P < 0.01). The mean hospital stay was 18.5 days for BT patients and 20 days for Sano patients ( P = 0.45). Early mortality for the total cohort was 14.8 % (n = 8) (BT 19.4 % [n = 6] vs. Sano 8.7 % [n = 2]; P = 0.12). There was no significant difference in inter-stage mortality between the two groups (BT: 18.2 % vs. Sano: 21.1 %; P = 0.47). CONCLUSION: The results for both established surgical methods (BT and Sano) for the palliation of HLHS and its variants have improved over time and are reaching acceptable early mortality rates. There was a trend towards a favorable early outcome for Sano patients, which did not reach statistical significance in this study due to the low patient numbers.

No evidence for angiotensin type 2 receptor gene polymorphism in intron 1 in patients with coarctation of the aorta and Ullrich-Turner syndrome.

In male patients with congenital anomalies of the kidney and urinary tract, an increased incidence of a polymorphism in the angiotensin type 2 receptor gene (AT2R) has been identified. The AT2R has been shown to be involved in apoptosis, particularly during embryogenesis. The aim of this study was to examine the A-->1675G transition polymorphism in intron 1 of the AT2R gene that is located on the X chromosome in patients with coarctation of the aorta (CoA) with and without Ullrich-Turner syndrome (UTS). Screening of DNA samples was performed with restriction fragment length polymorphism analysis. Ninety-seven patients with CoA, 28 girls with UTS, 10 girls with UTS and CoA, and 96 control individuals were studied. There was no significant difference in the distribution of A and G-genotypes in any of the patient groups compared to controls. An A-->1675G transition in the AT2R gene seems not to be involved in the pathogenesis of aortic coarctation.

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.

Congenital coronary artery fistula draining into the right ventricle: successful surgical closure after failed transcatheter coil embolization.

Coronary artery aneurysms and fistulae are very rare congenital anomalies. They occur in 0.2-0.4 % of all congenital heart diseases. In this article, we report a case of a four-year-old girl with a right coronary artery aneurysm and fistula draining into the right ventricle. Since the transcatheter coil embolization was not successful, surgical occlusion was considered in this case. We describe and discuss the handling of the fistula and the aneurysmatic enlargement of the proximal coronary artery.

Plasmatic [corrected] factor XIII reduces severe pleural effusion in children after open-heart surgery.

Chylous effusions frequently occur after cardiac surgery due to severe damage to the lymphatic system, thus indicating that the insertion of a chest tube may be necessary. Factor XIII (FXIII) is discussed as being essential for wound healing. The aim of this retrospective study was to evaluate whether the application of a single dose of FXIII results in a reduced amount of pleural effusion, leading to an earlier release of patients from the hospital. The cases of 40 children with severe chylous effusions after open-heart surgery were examined. Twenty patients received FXIII and were compared to 20 age- and weight-matched patients who did not receive FXIII. Major parameters included the amount of effusion before and 1 and 3 days after the application of FXIII; the duration of chest tubes; the total amount of fluid loss via drainage; and the period of hospitalization. FXIII levels in plasma showed an inverse correlation with fluid loss. After application of a single dose of FXIII, a significant reduction of pleural effusion within the first 24 hours was detected. However, no difference was observed between the two groups when comparing the total amount of pleural effusions within the first 72 hours. Finally, the duration of hospitalization did not differ between the FXIII-treated and the control group. A single application of FXIII rapidly reduces the amount of chylous effusions in the early period after open-heart surgery. This effect is detectable only for 24 hours after the treatment and does not alter the further clinical outcome. Prospective clinical trials are warranted to determine if repeated application or a higher dose of FXIII may improve the clinical outcome of chylous leakages in children after open-heart surgery.

The use of preoperative intra-aortic balloon pump in open heart surgery.

AIM: Intra-aortic balloon pump (IABP) therapy before open heart surgery has been suggested for ''high risk'' patients. METHODS: Records from patients undergoing open heart surgery at our institution between June 1999 and February 2002 were reviewed. Indication for IABP insertion was severely impaired left ventricular function, acute myocardial infarction (MI) or unstable angina. RESULTS: Fifty-five patients were included in the study: 41 male, 14 female, age 64+/-9 years. Fifty-one (92.7%) required coronary artery bypass brafting (CABG) alone or as a combined procedure, 2 (3.6%) required mitral valve surgery, and 2 (3.6%) needed more complex cardiac procedures. Thirty-two patients (58%) underwent emergency cardiac surgery and 11 patients (20%) suffered from acute preoperative MI. The overall 30 days mortality was 9%. Mean intensive care unit (ICU) stay was 6+/-8 days. Four patients (7.2%) developed postoperative renal failure requiring temporary hemodialysis. Three patients (5.4%) developed IABP related peripheral vascular complications. CONCLUSIONS: Perioperative morbidity and mortality is increased despite preoperative IABP, particularly in patients with acute MI. In contrast to studies not using this approach, preoperative IABP reduces morbidity and mortality of high risk patients. IABP related complications are low. Our data suggest that high risk patients profit from preoperative IABP therapy, however, prospective studies are needed to confirm these findings.

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.

Aortic root abscess complicated by fistulization and intracerebral hemorrhagic infarction.

We report an unusual case of a 12-year-old male with aortic root abscess complicated by fistulization and intracerebral hemorrhagic infarction. Transesophageal echocardiography provided exact information about the location and dimension. Color-flow mapping demonstrated fistulous communication between the abscess and both the aorta and the left ventricle, causing severe diastolic regurgitation through the paravalvular leak. Pulmonary autograft aortic root replacement was performed with good result.

Media calcification, low erythrocyte magnesium, altered plasma magnesium, and calcium homeostasis following grafting of the thoracic aorta to the infrarenal aorta in the rat--differential preventive effects of long-term oral magnesium supplementation alone and in combination with alkali.

Calcifications in arterial media are clinically well documented, but the role played by magnesium in pathophysiology and therapy is uncertain. To clarify this, an animal model in which the juxtacardial aorta was grafted to the infrarenal aorta, and the subsequent calcifications in the media of the graft and their response to oral supplementation with three magnesium-containing and alkalinizing preparations was investigated. Groups of highly inbred rats were formed as follows: sham-operation (Sham, n = 12), aorta transplantation (ATx, n = 12), ATx + magnesium citrate (MgC, n = 12), ATx + MgC + potassium citrate (MgCPC, n = 12), ATx + MgC + MgCPC (MgCPCSB, n = 12). At 84 (+/-2) days after ATx with or without treatment the following observations were made: (1) weight gain and general status were normal; (2) ATx rats developed massive media calcification, mineral accumulation in the graft, decreased erythrocyte magnesium and plasma parathyroid hormone, and increased plasma ionized magnesium and calcium, and uric acid; (3) Mg-treated rats developed variable degrees of metabolic alkalosis, but only MgCPCSB supplementation prevented calcifications. Additional findings after ATx alone were: imbalance in endothelin and nitric oxide production, the mineral deposited in media was poorly crystallized calcium phosphate, calcium exchange between plasma and graft, and bone resorption were unchanged. The superior anti-calcification effect of MgCPCSB was characterized by complete restoration of normal extracellular mineral homeostasis and uric acid, but sub-optimal normalization of erythrocyte magnesium. It was concluded that in the rat: (1) ATx causes loss of cellular magnesium, excess of extracellular magnesium and calcium in the presence of apparently unchanged bone resorption, and increased uricemia; (2) ATx facilitates enhanced influx of calcium into vascular tissue, leading to calcium phosphate deposition in the media; (3) ATx-induced calcification is prevented by dietary supplementation with a combination of magnesium, alkali citrate and bases. Although the described circulatory model of media calcification in the rat requires further investigation, the data allow ascribing a fundamental role to magnesium and acid-base metabolism.

Early increase of procalcitonin after cardiovascular surgery in patients with postoperative complications.

OBJECTIVE: Type and frequency of postoperative abnormalities were registered after cardiovascular surgery to evaluate the aetiology and diagnostic value of increased concentrations of procalcitonin (PCT) and C-reactive protein (CRP) during the early postoperative period. DESIGN: Prospective, observational study. PATIENTS: Two hundred and eight patients undergoing coronary artery bypass grafting or valve replacement requiring cardiopulmonary bypass were monitored for 7 days postoperatively for various types of infectious or non-infectious complications. Plasma PCT and CRP levels were measured on day 1 and day 2 after surgery and, when increased, until day 7. RESULTS: More patients with PCT above 2 ng/ml on day 1 or 2 (n=55) had postoperative abnormalities (95%) than patients with lower PCT (59%). Specifically, the incidence of three or more criteria of the "systemic inflammatory response syndrome" was 45% versus 4% (area under the curve of the receiver operating characteristic 0.866); positive inotropic support was needed in 65% versus 9% (0.870); respiratory insufficiency (PaO(2)/FIO(2)<200) 38% versus 12% (0.704); proven and suspected bacterial infection 9% versus 1% (0.900) and 24% versus 1% (0.897), respectively. For CRP, the respective areas under the curve were all below 0.63, while all patients had elevated CRP levels, whether they had a complication or not. CONCLUSIONS: Elevated PCT, but not CRP, correlates with evidence of systemic inflammation and other complications early postoperatively after cardiac surgery. Although the PCT levels do not rise as quickly as the criteria of the systemic inflammatory response syndrome appear, they do reflect systemic inflammation. Early identification and quantification of a systemic inflammatory response may help reduce postoperative complications.

[Aortic root abscess without involvement of the aortic valve: diagnosis and therapy in a 2.5-year-old child]. / Aortenwurzelabszess ohne Beteiligung der Aortenklappe: Diagnostik und Therapie bei einer 2 1/2-jährigen Patientin.

Although formation of an aortic root abscess is a frequent complication of aortic valve endocarditis in adults, this complication has been rarely observed in children. In the majority of cases it has been described in children without underlying congenital heart disease. Due to the rarity of this complication, diagnosis and treatment is frequently delayed in childhood. We report a 2 1/2 year old girl who developed pericardial effusion in the course of pneumonia. Echocardiographic examinations, which were performed because of the pericardial effusion, revealed after 6 days the development of a cystic structure posterior to the aortic root. There was a perforation of this aortic root abscess to the left ventricular outflow tract; the aortic and mitral valves however were normal without endocarditic vegetations. Surgery was performed on the 10th day following a rapid increase in the size of the abscess. During surgery the abscess was drained and the perforation to the left ventricle was closed with direct sutures. Intraoperative transesophageal echocardiography confirmed a good surgical result. Blood cultures remained negative; in the material from the abscess however we found staphylococcus aureus. The postoperative course was uneventful. Our case demonstrates the necessity of detailed and repeated echocardiographic examinations in children with possible symptoms of bacterial endocarditis (in our case pericardial effusion) as well as the requirement of cultures of the abscess for identification of the infective organism. Intraoperative transesophageal echocardiography allows exact description of an aortic root abscess, its relation to other cardiac structures and immediate evaluation of the surgical result.

A case of aortic valve disease associated with Tropheryma whippelii infection in the absence of other signs of Whipple's disease.

A case of endocarditis caused by Tropheryma whippelii is reported. The 69-year-old patient was diagnosed as suffering from severe aortic regurgitation requiring aortic valve replacement, but showed no other symptoms of Whipple's disease. T. whippelii was detected in the explanted aortic valve by broad-range PCR amplification of the 16S rDNA and subsequent sequence analysis of the product. The etiologic agent was classified as a type 2A sequence variant based on the 16S-23S intergenic spacer and the 23S rDNA (domain III) sequences. The histological examination of the aortic valve was compatible with Whipple's disease. A duodenal biopsy revealed an infection with Giardia lamblia, but T. whippelii and histological signs of Whipple's disease were not detectable.