The role of FDG-PET-CT in pediatric cardiac patients and patients with congenital heart defects.

AIM: Medical imaging by using FDG-PET/CT (PET-CT) can detect, confirm or eliminate with high sensitivity areas of suspected infections in case of persistent fever of unknown origin in combination with other bacteriological examinations. The aim of this study was to assess the potential role of PET-CT in detecting or excluding infections or other inflammatory processes in patients with congenital heart defects (CHD). In addition we wanted to evaluate the practical impact of PET-CT on the subsequent clinical management. METHODS: In this retrospective study we analyzed the data of all CHD patients who underwent PET-CT over a 5year period in our institution. The results were then evaluated with regard to the potential impact on clinical decision making. RESULTS: Between 2010 and 2015 PET-CT was performed in 30 patients. The mean age was 26years (SD 15years, range 1 to 66years). The diagnoses covered a large field of CHD. 11 patients (4/11 with assist device) were assessed before heart transplantation; suspected malignancies or infections were excluded and transplant listing was possible. In another 5/6 patients suspected assist device infection could be confirmed with PET/CT. Endocarditis was suspected in 13 patients, 2 of whom underwent previous MRI without confirmation and ECHO was inconclusive. Endocarditis was finally excluded in 5/13 patients but confirmed in 8/13 patients by PET-CT. CONCLUSION: In this study we could show a high sensitivity of PET-CT for specific localization of infections and with high impact on subsequent therapy. Based on this results clinical management could be targeted and adapted. We could demonstrate that PET-CT has a high impact on the subsequent clinical therapy.

Three decades of follow-up of aortic and pulmonary vascular lesions in the Williams-Beuren syndrome.

The diagnostic criteria of the Williams-Beuren syndrome (WBS) were established almost 3 decades ago. Until now there has been little knowledge about the natural and post-surgical history of vascular lesions in this syndrome. In order to evaluate the long term follow-up of aortic and pulmonary vascular lesions, we have analysed the catheterization data, angiocardiograms, and Doppler-echo measurements in 59 patients who were seen at least twice in our institution between 1961 and 1993. Their follow-up periods ranged from 2.1 to 28.2 years. Of 45 patients with supravalvular aortic stenosis (SVAS) with a mean follow-up period of 12.9 years, it became evident that pressure gradients of less than 20 mm Hg in infancy generally remained unchanged during the first two decades of life. Pressure gradients exceeding 20 mm Hg increased from an average of 35.5 mm Hg to 52.7 mm Hg in 13 patients. Of these, 8 required surgical relief of the narrowing. In 7 patients aortic hypoplasia was documented. In 5 of them the caliber of the aorta showed a tendency towards normalisation within a period of 11.9 to 23.9 years. Of 6 individuals with aortic hypoplasia and surgical relief of SVAS, 4 patients developed restenosis at the distal end of the aortoplasty patch. In contrast, 9 patients with operated SVAS-but without aortic hypoplasia-remained free of restenosis over a period of 11 years (mean). Coarctation occurred in 4/59 patients; restenosis was seen in 2 after 5 and 16 years. Peripheral pulmonary stenosis was followed in 23 patients over 14.4 years (mean).(ABSTRACT TRUNCATED AT 250 WORDS)

Left atrial thrombus in a 10-month-old boy--successful thrombolysis with recombinant tissue-type plasminogen activator after open-heart surgery: review of the literature.

A 10-month-old boy with major left atrial thrombus following cardiac surgery was treated with intravenously administered recombinant tissue-type plasminogen activator (rt-PA; Actilyse, Thomae-Behring, Germany). The left atrial thrombus was diagnosed by Doppler echocardiography 8 days after complete correction of a ventricular septal defect. rt-PA therapy was administered over a 10-day period. Significant hemopericardium occurred 50 h after the start of thrombolytic therapy. rt-PA infusion was discontinued for 20 h to insert a pericardial drainage. The initial rt-PA dose was 0.1 mg/kg over 10 min followed by a continuous daily infusion of 1.7 mg/kg together with low-dose heparin. Thrombolytic therapy was restarted 20 h after pericardial drainage was inserted. The daily rt-PA dose was gradually raised to 3 mg/kg (total dose: 18 mg/kg). On day 7 and 8 a clear decrease in P-plasminogen and P-antithrombin occurred, requiring additional fresh frozen plasma and P-antithrombin concentrate substitution. One day later, without further side effects, complete thrombolysis occurred. Although hemopericardium demanded discontinuation of thrombolytic therapy, rt-PA administration, closely monitored by Doppler echocardiography, was continued, leading to complete thrombolysis of the left atrial thrombus in the early postoperative period. We consider the literature dealing with rt-PA thrombolysis in infancy we discuss this case report.

Direct fetal administration of adenosine for the termination of incessant supraventricular tachycardia.

BACKGROUND: Adenosine terminates supraventricular reentry tachycardia safely and effectively in the pediatric age group. CASE: The recurrence of pretreated incessant tachycardia led to severe hydrops in a 28-week-old fetus. The tachycardia was terminated instantly with direct fetal administration of adenosine via the umbilical vein. Normal heart rate and rhythm were then preserved temporarily with digoxin and flecainide. CONCLUSION: Direct fetal adenosine administration might be helpful in the treatment of fetal reentry tachycardias if the sinus rhythm achieved quickly can be preserved by long-acting antiarrhythmic drugs. Such a combined therapeutic approach might be especially advantageous in hydropic fetuses.

Biological bridging after repair of an anomalous origin of a left coronary artery.

We report on an unusual case of surgical repair for anomalous origin of the coronary artery from the pulmonary artery, in a young patient who underwent tunnel repair with concomitant heterotopic heart transplantation to support severely impaired left ventricular function. Four years later, the graft was removed after confirmation of ventricular arrest and excellent recovery of the patient's own heart.

Cardiopulmonary bypass in patients with heparin-induced thrombocytopenia using Org 10172.

BACKGROUND: In patients with heparin-induced thrombocytopenia undergoing cardiac operations, anticoagulation with heparin should be avoided. The low-molecular-weight glycosaminoglycan Orgaran has been used as an alternative, but the overall experience is limited. METHODS: Two patients with heparin-induced thrombocytopenia underwent cardiopulmonary bypass using Orgaran for anticoagulation. A 30-year-old woman suffered from emboli to her brain through a secondary atrial septal defect, a 14-year-old boy from ischemia of his left leg due to recurrent embolism originating from the mitral valve. In both cases, cardiopulmonary bypass was performed in a routine manner, except for using low-dose Orgaran instead of heparin. Anticoagulation was monitored during cardiopulmonary bypass by measuring Orgaran plasma levels and activated clotting time. RESULTS: No thromboembolic or bleeding complications occurred during and after atrial septal defect repair and mitral valve replacement, respectively. In the former case, thrombotic material from the inferior vena cava was removed during hypothermic circulatory arrest within the same procedure. Activated clotting time did not correlate with plasma levels of Orgaran. CONCLUSIONS: Orgaran might be a useful alternative for anticoagulation during extracorporeal circulation. Adequate dosages and measurement of plasma levels are recommended for its use in cardiopulmonary bypass.

Neonatal mechanical bridging to total orthotopic heart transplantation.

BACKGROUND: Until recently, newborns with medically intractable cardiac failure caused by congenital malformations were mostly doomed to death because of the severity of the disease, which precludes a palliative operation, or because of fatal deterioration before availability of a suitable donor heart. METHODS: The recently developed paracorporeal pneumatically driven Medos HIA ventricular assist device offers a therapeutic option for these small infants because it is manufactured in various sizes and is even suitable for cardiac assistance in neonates with a body surface area less than 0.3 m2. RESULTS: We report our initial experience with this device, which we used for univentricular bridging to total orthotopic cardiac transplantation in 3 infants. The device was inserted to support the left ventricle in two instances and to support the right heart in one. Successful bridging to transplantation was achieved in 2 infants for periods of 2 and 7 weeks. CONCLUSIONS: Our experience demonstrates the feasibility of univentricular mechanical support followed by successful cardiac transplantation in infants and newborns.

Percutaneous closure of secundum atrial septal defect with a new self centering device ("angel wings").

OBJECTIVE: To investigate the safety, efficacy, and clinical application of a new self centering device ("angel wings") for closure of secundum atrial septal defects (ASD II) and persistent foramen ovale in all age groups. DESIGN: Multicentre, prospective, nonrandomised study. INCLUSION CRITERIA: defects with an occlusive diameter of < or = 20 mm and a surrounding rim of > 4 mm; body weight > 10 kg; and an indication for surgical closure of secundum atrial septal defect. Additionally, there were compassionate indications for closure in patients with persistent foramen ovale. INTERVENTIONS: Defects were closed by a transcatheter device consisting of two square frames made of superelastic nitinol wire. The frames are covered by elastic polyester fabric, which is sewn together at a central circle. All procedures, except for three interventions that were carried out under sedation, were performed under general anaesthesia using transoesophageal echocardiography and fluoroscopy to monitor intervention. RESULTS: Closure was attempted in 75 (71%) of 105 patients. An ASD II was present in 35 children and 15 adults. A persistent foramen ovale was present in 25 adults with suspected paradoxical embolism. Transcatheter closure was unsuccessful in three children and crossover to surgery was required. Residual shunts were found in 20 patients (27%) immediately after the procedure. A transient atrioventricular third degree block occurred in three patients (4%) and the right atrial disk was not fully deployed in three. A minor shunt (< 3 mm) was present in only three (4%) of 72 patients during follow up of 1-17 months. Blood clots on the right atrial disks in two patients (one required lysis) were seen during follow up transoesophageal echocardiography. Serious complications demanding surgical removal of the device occurred in three patients. One patient had haemopericardial tamponade because of an aortic lesion. Left atrial thrombus formation due to an unfolded right atrial disk was found in a second patient and dislodgement of the left atrial disk resulted in a large residual shunt in a third. CONCLUSIONS: Percutaneous closure of a central ASD with a diameter < or = 20 mm in paediatric and adult patients is feasible and effective with this new device. It is a promising alternative to surgical closure. Modifications of the design, however, seem to be mandatory as 4% of patients developed serious complications.

Interaction of fibrinolysis and prothrombotic risk factors in neonates, infants and children with and without thromboembolism and underlying cardiac disease. a prospective study.

UNLABELLED: To evaluate the role of plasminogen activator inhibitor-1 (PAI-1) and tissue-type plasminogen activator (t-PA) in children with an estimated risk of vascular occlusion reported to range from 7% to 16%, we conducted a prospective study in infants and children with underlying cardiac disease. One hundred and twenty-five children (neonate - 16 years) were investigated. In 9 infants out of the 125 children vascular occlusion occurred, closely related to cardiac catheterisation and arterial or venous lines during major cardiac surgery. Six of the nine neonates and infants with (n=6) and without (n=3) prothrombotic risk factors showed evidence of a basically impaired fibrinolytic system. Five of the nine infants showed increased PAI-1 clearly correlated to the 4G/4G genotype of the plasminogen activator-1 promoter polymorphism along with elevated t-PA concentration before the first diagnostic cardiac catheterisation was performed. One infant presented with increased t-PA concentration only. Five of the six children with reduced fibrinolytic capacity had further prothrombotic risk factors. CONCLUSION: Data of this study indicate that neonates and infants with underlying cardiac disease and basically increased PAI-1 due to the 4G/4G variant of the PAI-1 promoter polymorphism along with elevated t-PA levels in combination with further prothrombotic risk factors are at high risk of developing early thromboembolism during cardiac catheterisation.

Flush heparin during cardiac catheterisation prevents long-term coagulation activation in children without APC-resistance-preliminary results.

This study was designed to prospectively evaluate haemostatic activation in 75 children undergoing cardiac catheterisation with intermittent flush heparin (10 IU/ml saline) and to relate these data to clinical findings and inherited risk factors for thrombophilia. In addition to flush heparin in infants < 6 months of age in whom additional arterial catheterisation was performed (n = 5) or patients with thrombophilia, heparin (300-400 IU/kg/d) was administered for a further 24 h. APTT was prolonged and anti Xa activity was significantly increased at the end of catheterisation and returned to normal 24 hours later. Whereas thrombin generation (F1 + 2) showed a significant coagulation activation at the end of catheterisation, no concomitant fibrinolytic activation (D-Dimer) was observed. Four children showed resistance to APC: one of them in whom stroke had occurred before and one additional child heterozygous for APCR received further prophylactic heparin. Two neonates with APCR and flush heparin only suffered from thrombosis after catheterisation. No further thrombotic events occurred. This study indicates that low-dose flush heparin during catheterisation may prevent long-term haemostatic activation in children without thrombophilia. Whether further heparin after cardiac catheterisation in children with APCR prevents vascular insults requires a more intensive study.

Reproducibility of quantitative pediatric transesophageal echocardiography.

Transesophageal echocardiography (TEE) is commonly used to monitor cardiac function and to assess cavitary size. For the interpretation of quantitative echocardiographic data, the degree of their reproducibility should be considered. The variability of quantitative TEE was evaluated in this study. To assess intraobserver, beat-to-beat, interobserver, and repositioning variability, TEE examinations of 46 patients with congenital heart defects were analyzed. The mean beat-to-beat variability of 8.5% (range 4.2% to 12.3%) exceeded the mean intraobserver variability of 4.9% (1.9% to 8.1%). The mean interobserver difference between two observers was 3.4% (0.2% to 11.9%). Differences in image acquisition caused by repositioning of the transesophageal probe contributed the most (6.4% to 13.3%; mean 10.5%) to the variability of two-dimensional TEE. Changes seen on TEE studies should be interpreted as abnormal only when they exceed the total variability of this method.

Effects of ultrafiltration and peritoneal dialysis on proinflammatory cytokines during cardiopulmonary bypass surgery in newborns and infants.

OBJECTIVES: To assess the impact of balanced ultrafiltration and peritoneal dialysis (PD) on plasma and urinary cytokines and renal dysfunction after cardiopulmonary bypass (CPB) surgery in newborns and infants. METHODS: Twenty-three newborns and infants weighing less than 7 kg and scheduled for operation on congenital malformation were enrolled in this descriptive open clinical study. All patients received conventional ultrafiltration in the CPB rewarming period. Eleven newborns underwent Tenckhoff-catheter implantation in the operation theatre as a routine institutional procedure and received PD after admission to the ICU (the PD [+] group). No PD was used in another 12 patients (the PD [-] group). Interleukins (IL) 6 and 8 were measured four times pre- and post-operatively. Kidney function was assessed by creatinine clearances and urine protein and enzyme analyses. RESULTS: All patients had an uneventful clinical course. Age (10+/-2 days, PD [+] vs. 96+/-19 days, PD [-]), CPB duration (215+/-23 vs. 143+/-20 min), and degree of hypothermia (26+/-1.3 vs. 31+/-0.1 degrees C) differed significantly between the groups. Age, CPB duration and ultrafiltration influenced post-operative IL-levels in an analysis of variance. While there were few differences immediately after the end of ultrafiltration, post-operative levels of IL-6 and IL-8 were higher and more sustained in the newborns (PD [+]) than in the older infants (PD [-]). The median amount of IL-6 and IL-8 removed by ultrafiltration came to 28 and 59% compared to the amount of IL-6 and IL-8 remaining in the blood at the end of CPB. IL-clearance by ultrafiltration was more than 1000-fold and by PD more than 100-fold as effective as IL-clearance by the kidney. While the kidneys showed an unselective mixed glomerular and tubular pattern of injury, during CPB higher serum IL-concentrations correlated with lower urinary IL-clearances in both study groups. CONCLUSIONS: Ultrafiltration and PD are highly effective in removing proinflammatory cytokines. Impaired kidney function was associated with proinflammatory IL-serum concentrations. Thus, we raise the hypothesis that glomerular-filtered proinflammatory ILs damage the proximal tubular cells of the kidney in newborns and infants, thus contributing to post-operative renal dysfunction. Conversely, we conclude that removing proinflammatory ILs by ultrafiltration and PD acts renoprotectively. A future prospective randomised study could demonstrate whether this can indeed improve clinical outcome.

Cesarean section in a patient with non-compaction cardiomyopathy managed with ECMO.

Isolated ventricular non-compaction is a rare cardiomyopathy associated with left heart failure, severe arrhythmias and thromboembolism. We report about our interdisciplinary strategy in a patient with severe isolated ventricular non-compaction cardiomyopathy scheduled for caesarean section in general anaesthesia. Monitoring included placement of an arterial line, a central venous catheter and a pulmonary artery catheter with pacing option. Small introducer gates were placed in the femoral artery and vein to facilitate quick percutaneous institution of extracorporeal life support via extracorporeal membrane oxygenation in case of acute cardiac failure refractory to medical treatment. Inotropic pharmacological therapy with 3 µg/kg/min dobutamine and 0.25 mg/kg/min milrinone was started before surgery. Induction of general anesthesia and rapid sequence intubation was performed with an analgesic dose of 0.5 mg/kg S ketamine, 0.25 mg/kg etomidate and 5 mg rocoronium followed by 1.5 mg/kg succinylcholine. This regimen provided completely stable hemodynamics in this critical period until delivery of the child and continuation of anaesthesia with continuous infusion of propofol and remifentanyl. The current strategies, particularly the preparation for femoro-femoral extracorporeal membrane oxygenation, may be considered in similar cases with a high risk of acute cardiac decompensation which may be refractory to medical treatment. Anaesthesiologist involved in performing caesarean section in women with complex cardiac disease, should encompass extracorporeal membrane oxygenation standby in management of the perioperative period.

Pediatric patients with Kawasaki disease and a case report of Kitamura operation.

Kawasaki disease (KD), also called mucocutaneous lymph node syndrome, is an acute, self-limiting, small-vessel vasculitis with an unknown cause that affects children between the ages of 6 months and 5 years. It is the most common cause of acquired coronary artery disease in childhood. Acute myocardial infarction and coronary artery aneurysm are major complications. We present a cohort of patients with KD who were followed up and treated in the Heart Center, North Rhine-Westphalia. Included is a review of important relevant items common to cases of KD, such as clinical data and management, including medical management of the acute condition and the diagnosis and management of coronary vasculitis and aneurysms as well as the application of coronary artery bypass grafting (CABG) in those conditions. Between January 2002 and January 2006, we evaluated the findings and characteristics of 18 pediatric patients with a history of KD and their long-term outcome. The acute illness occurred between the ages of 4 months and 14 years of age. Anomalies of the coronary arteries were found in 6 patients ranging in age from 5 months to 10 years. One patient had acute myocardial infarction; another underwent CABG after 5 years from disease onset at the age of 15 years. Kitamura operation was performed successfully. The other patients are still under observation.Coronary artery aneurysms and stenosis requiring surgery are rare in KD; nevertheless, CABG is the standard therapy when myocardial ischemia is detected. Kitamura operation provides good growth potential and long-term graft patency.

Echocardiographic and electrocardiographic findings of cardiomyopathy in Duchenne and Becker-Kiener muscular dystrophies.

We assessed the cardiac findings in Duchenne muscular dystrophy (DMD) and Becker-Kiener muscular dystrophy (BMD) patients in the large outpatient group of our single-center institution. The analysis included the revision of patient records (retrospectively) and current echocardiography, electrocardiogram (ECG), and Holter ECG data (prospectively). Reduced left ventricular fraction shortening (FS) < 25% was found in 24% of all patients (50 DMD, 12.1 +/- 4.7 years: 20 BMD, 17.1 +/- 8.5 years). Median age of onset of FS < 25% was 16.8 +/- 1.0 in DMD and 30.4 +/- 3.4 in BMD; (p < 0.05). Holter ECG in DMD demonstrated sinus tachycardia in 26% deprivation of circadian rhythm in 31%, and reduced heart rate variability in 51%. For these reasons, we recommend carrying out echocardiography annually in DMD and BMD > 10 years. Because the rate of disturbances in the cardiac autonomic nervous system is high in DMD, we also recommend Holter ECG annually. Further investigation should be conducted to determine if treatment with ss-blockers and ACE inhibitors in combination reduces cardiac autonomic nervous imbalance, thus improving patient outcome in DMD.

New aspects of the ventricular septum and its function: an echocardiographic study.

OBJECTIVES: To examine whether the line dividing the septum into two layers is found consistently by conventional echocardiography and to evaluate functional differences in the right and left side of the septum in terms of wall thickening, strain rate, and strain imaging. DESIGN: In a systematic study in 30 normal subjects, M mode and Doppler myocardial imaging data from the interventricular septum (IVS) were recorded. Velocity curves, regional strain rate, and strain profiles were obtained. Systolic deformation (wall thickening, radial and longitudinal strain rate, and strain) of both sides were assessed. Furthermore, three patients with one sided abnormalities were studied. RESULTS: A bright echo consistently segmented the IVS into a left and right part. In this normal population radial deformation was different for the left and right side of the septum (mean (SD) wall thickening on the left, 49 (46)%, and on the right, 17 (38)%; strain rate on the left, 3.8 (0.6) 1/s, and on the right, 2.1 (1.9) 1/s; strain on the left, 41 (17)%, and on the right, 22 (14)%), whereas longitudinal deformation was found to be similar (strain rate on the left, -2.2 (0.7) 1/s, and on the right, -2.0 (0.6) 1/s; strain on the left, -28 (12)%, and on the right, -25 (12)%). The presented clinical examples show that abnormalities can be strictly limited to one layer. CONCLUSIONS: Differential radial deformation and knowledge of fibre architecture showing an abrupt change in the middle of the septum, together with the clinical cases, suggest the septum to be a morphologically and functionally bilayered structure potentially supplied by different coronary arteries.

[Degree of narrowing of regional and non-regional coronary arteries of hearts with myocardial infarction. A postmortem study (author's transl)]. / Postmortale Untersuchungen über die Lichtungsweiten von Infarktarterien und Nichtinfarktarterien von Infarkterzen.

137 hears with 185 infarctions with a diameter of at least 2.5 cm were investigated. Sometimes the infarcted areas were supplied by two arteries. Altogether there were 198 arteries supplying infarcted areas (= IA) and 213 arteries supplying non-infarcted areas (= NIA). The cross-sectional area of most severe stenosis of each coronary artery was determined and expressed in per cent of the area surrounded by the lamina elastica interna. Only two groups of infarctions were formed: recent infarcts (without and with organization) and old infarcts (completely scarred). Regarding all IA and NIA there were highly significant differences between the mean values. The rest of the lumen amounted in the IA 7.48% and in the NIA 38.20%. In hearts with recent infarcts, IA were significantly smaller (4.13%) than in hearts with old infarcts 8.75%). This difference is due to recanalizations in the group of old infarcts. Differences between the degree of stenosis of sole and repeated infarcts were missed and there was no correlation between the weight of the heart and the cross-sectional area of IAs. The IA of hearts with transmural infarction were constricted more strongly than those of hearts with subendocardial infarcts. 21 IA were larger than one NIA of the same heart. Most of these hearts included old infarcts and it is most probably that during the origin of the infarctions almost all of these IA were smaller than the NIA. The findings strongly support the coronarogenic origin of myocardial infarctions.