Balloon-Atrioseptostomy in Small Children Using an Embolectomy Catheter: Preliminary Data.

Interventional treatment of restrictive atrial septal defects in complex heart disease is considered state-of-the-art therapy up to date. Nevertheless, dedicated balloons are lacking so far, as several products have been withdrawn from the market. We report on off-label use of a balloon embolectomy catheter used successfully in a preterm patient and discuss whether this device might be used in other patients as well as it seems to be promising due to its shape and versatility.

[Stroke alarm-Arterial ischemic stroke as one of the most time-critical emergencies in children and adolescents]. / Notfall-Neuropädiatrie ­ Der arteriell ischämische Schlaganfall als einer der zeitkritischsten Notfälle bei Kindern und Jugendlichen.

Arterial ischemic stroke in childhood and adolescence is one of the most time-critical emergencies in pediatrics. Nevertheless, it is often diagnosed with a considerable time delay which may be associated with low awareness, the sometimes nonspecific clinical presentation with a wide variety of differential diagnoses, and less established 'acute care structures'. The revascularisation strategies in adult stroke care are also potential and promising treatment options for children, even if available evidence is still limited. In the post-acute phase, the etiological work-up is complex due to the multitude of risk factors to be considered. But it is essential to identify each child's individual risk profile as it determines secondary prevention, risk of recurrence and outcome. Long-term care in a multiprofessional, interdisciplinary team must take into account the bio-psycho-social aspects to integrate the child into its social and educational, and later professional environment.

Microcatheter-assisted stenting of the tortuous vertical ductus arteriosus via femoral access in a duct-dependent pulmonary circulation.

BACKGROUND: Stenting of the patent ductus arteriosus (PDA) has been accepted as alternative option to surgical shunting to enable additional pulmonary blood flow or for palliation of patients with a truly duct-dependent pulmonary circulation. The procedure can be challenging given the variable and often tortuous anatomy of the PDA and various technical approaches are reported. OBJECTIVE: To report an alternative technique to treat tortuous ducts with microcatheter assistance and by transfemoral approach. METHODS: We applied this technique of PDA stenting in 5 consecutive patients (4/5 age < 1 week, weight 2,7-3,2 kg; 1/5 re-do PDA stenting at 6,5 month and 5,9 kg). A soft coronary guidewire was advanced by microcatheter assistance into the branch pulmonary arteries and thereafter replaced by an extrastiff guidewire to enable the placement of long coronary stents. RESULTS: Successful PDA stenting with this stepwise approach and with femoral access only could be achieved in all patients (n = 5/5). A single stent was used in 2 patients (one with re-do stenting and previous stents). 3/5 patients had 2 stents implanted by telescopic technique. Stent sizes used were 4,5 × 15 mm (n = 2) and 4,5 × 18 mm (n = 6). No guide wire or stent dislodgement appeared through all procedures with microcatheter assistance. CONCLUSIONS: This technique enables PDA stenting via transfemoral approach in complex and tortuous ducts and thereby offers an attractive addition to the interventional management of truly duct-dependent pulmonary circulation.

Transient severe tricuspid regurgitation after transplantation of an extremely oversized donor heart in a child-Does size matter? A case report.

In pediatric heart transplantation, the size of the donor organ is an important criterion for organ allocation. Oversized donor hearts are often accepted with good results, but some complications in relation to a high donor-recipient ratio have been described. Our patient was transplanted for progressive heart failure in dilated cardiomyopathy. The donor-to-recipient weight ratio was 3 (donor weight 65 kg, recipient weight 22 kg). The intra-operative echocardiography before chest closure showed excellent cardiac function, no tricuspid valve regurgitation, and a normal central venous pressure. After chest closure, central venous pressure increased substantially and echocardiography revealed a severe tricuspid insufficiency. As other reasons for right ventricular dysfunction, that is, myocardial ischemia, pulmonary hypertension, and rejection, were excluded, we assumed that the insufficiency was caused by an alteration of the right ventricular geometry. After 1 week, the valve insufficiency regressed to a minimal degree. In pediatric heart transplant patients with a high donor-to-recipient weight ratio, the outlined complication may occur. If other reasons for right ventricular heart failure can be ruled out, this entity is most likely caused by an acute and transient alteration of the right ventricular geometry that may disappear over time.

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.

Eosinophilic oesophagitis: relationship of quality of life with clinical, endoscopic and histological activity.

BACKGROUND: Knowledge about determinants of quality of life (QoL) in eosinophilic oesophagitis (EoO) patients helps to identify patients at risk of experiencing poor QoL and to tailor therapeutic interventions accordingly. AIM: To evaluate the impact of symptom severity, endoscopic and histological activity on EoE-specific QoL in adult EoE patients. METHODS: Ninety-eight adult EoE patients were prospectively included (64% male, median age 39 years). Patients completed two validated instruments to assess EoE-specific QoL (EoO-QoL-A) and symptom severity (adult EoE activity index patient-reported outcome) and then underwent esophagogastroduodenoscopy with biopsy sampling. Physicians reported standardised information on EoE-associated endoscopic and histological alterations. The Spearman's rank correlation coefficient was calculated to determine the relationship between QoL and symptom severity. Linear regression and analysis of variance was used to quantify the extent to which variations in severity of EoE symptoms, endoscopic and histological findings explain variations in QoL. RESULTS: Quality of life strongly correlated with symptom severity (r = 0.610, P < 0.001). While the variation in severity of symptoms, endoscopic and histological findings alone explained 38%, 35% and 22% of the variability in EoE-related QoL, respectively, these together explained 60% of variation. Symptom severity explained 18-35% of the variation in each of the five QoL subscale scores. CONCLUSIONS: Eosinophilic oesophagitis symptom severity and biological disease activity determine QoL in adult patients with eosinophilic oesophagitis. Therefore, reduction in both eosinophilic oesophagitis symptoms as well as biological disease activity is essential for improvement of QoL in adult patients. Clinicaltrials.gov number, NCT00939263.

Value of postmortem magnetic resonance imaging for fatal neonatal congenital heart disease: a case report.

Autopsies after neonatal death due to suspected or insufficient diagnosed cardiac malformations provide important information to parents and the involved obstetric and pediatric physicians. The autopsy rate, however, is declining. Postmortem magnetic resonance imaging may be an alternative option for selected cases, providing detailed morphologic information about the cardiovascular system. This case report demonstrates the beneficial use of postmortem magnetic resonance imaging in a newborn with a suspected complex cardiac malformation. The magnetic resonance imaging was able to complete the diagnosis of situs inversus totalis, with transposition of great arteries, and to provide valuable information about the underlying physiology.

Successful treatment of oleander intoxication (cardiac glycosides) with digoxin-specific Fab antibody fragments in a 7-year-old child: case report and review of literature.

UNLABELLED: A 7-year-old girl presented six hours after ingestion of a yellow oleander seed (Thevetia peruviana) with severe emesis, change in colour vision and complete heart block. Initial treatment with phenytoin and isoprenalin infusion led to intermittent supraventricular and ventricular tachycardia. The patient was then treated with two intravenous doses of 190 mg of digoxin-specific Fab antibody fragments (Digibind). Subsequently the patient's rhythm reverted to sinus rhythm and the symptoms resolved within 2 hours. CONCLUSION: administration of digoxin-specific Fab antibody fragments in an otherwise healthy child after oleander intoxication is safe and without adverse reactions.

Initial experience using the NuMED Cheatham Platinum (CP) stent for interventional treatment of coarctation of the aorta in children and adolescents.

We report the immediate results in a group of selected patients with native or recurrent coarctation of the aorta who underwent endovascular stent implantation using the newly designed Cheatham-Platinum (CP)-stent. The balloon-expandable stents were implanted in 6 patients (mean age 12.7 years) with coarctation of the aorta (5 native, 1 recurrent). The maximal systolic peak pressure gradient was decreased from 49 to 3 mmHg (p <0.001). There was a 350% increase in the mean diameter at the original coarctation site (3.8 to 13.8 mm, p <0.01). Although the maximal diameter varied from 8 to 18 mm, there was only a minor reduction in the length of the CP-stents used (max. 11%). The dilatation was successful in all patients and there were no complications during balloon dilatation or stent implantation. All patients were hypertensive prior to stent implantation, with three of them requiring antihypertensive drug therapy. In 2 patients only a moderate dilatation diameter was chosen initially due to the extremely small coarctation site (1 mm) and repeat dilatation after 12 months was performed in order to obtain a maximal aortic diameter. At a mean of 18 months of follow-up, 5 of 6 patients are normotensive. There is no recurrence of coarctation, aortic dissection or aneurysm formation and no stent displacement. These findings suggest that the implantation of CP-stents for coarctation of the aortamay cover a wide spectrum of aortic diameters and consequently hereby offer an effective alternative approach to surgery or ballon dilatation alone even in infancy and childhood. The potential for redilatation of CP-stents in a wide range of diameters without significant shortening adds to the benefit of this device in growing children.

Postoperative junctional ectopic tachycardia (JET).

Postoperative junctional ectopic tachycardia (JET) is a potential life-threatening tachycardia that mainly occurs after surgical correction of congenital heart defects. The arrhythmia itself or the related treatment has significant clinical impact on the postoperative course and intensive care stay. In general, JET is a self-limiting disorder that usually resolves within one week. However, JET occurs usually within the first 24 to 48 hours after corrective surgery, when systolic and diastolic function of the heart is impaired. Thus, the rapid heart rate leads to an acute further deterioration of cardiac output that requires adequate treatment. The diagnosis of JET is made by the typical ECG-appearance with narrow QRS-configuration at a rate of 170 to 260 bpm and AV-dissociation. A variety of different therapeutic strategies have been tested in postoperative and congenital/spontaneous JET. Treatment success is usually defined as a stable decrease in the ventricular rate below 140-150/min, the possibility of atrial pacing and thereby the improvement of cardiac output. Optimal success is the reinstitution of sinus rhythm. Many of the treatment strategies reported are based on specific institutional treatment protocols. These include conventional supportive treatment, specific medical antiarrhythmic therapy, specific forms of pacing and surface cooling. Today, the administration of high doses of amiodarone usually leads to adequate control of the rate and enables pacing. Surgical intervention or catheter ablation of the HIS-bundle is rarely necessary. This article reviews the literature about JET over the past years and offers a specific treatment protocol.

[Isolated myocardial noncompaction in childhood-case report and review of the literature]. / Isolated myocardial noncompaction als seltene Ursache einer Synkope im KindesalterFallbericht mit Literaturübersicht.

The diagnostic work-up for the first syncopal episode of a 14-year-old female adolescent revealed the morphology of an isolated myocardial non-compaction on echography. Angiography and biopsy of the left ventricle confirmed the diagnosis. Despite a reduced shortening fraction (FS 21%) and frequent premature atrial beats, there were no further cardiac or extracardiac symptoms. After establishment of therapy with beta-blockade, digitalis, angiotensin converting enzyme inhibition and acetylsalic acid, the follow-up over 24 months was good; the implantation of an automatic implantable cardioverter-defibrillator is planned.

Comparison of analgesic/sedative effect of racemic ketamine and S(+)-ketamine during cardiac catheterization in newborns and children.

The hypnotic and analgesic effect of ketamine with maintained spontaneous breathing is used for analgesic/sedative anesthesia without the need of intubation. The intention of this study was to compare the efficacy and side effects of racemic ketamine and its enantiomer S(+)-ketamine during cardiac catheterization in newborns and children. One hundred children (ages 0-11 years) were randomly assigned to groups of equal size. The differences between the racemic ketamine/midazolam and the S(+)-ketamine/midazolam groups were investigated regarding the total dosage of sedative drugs, side effects, and the awakening period. The dosage of S(+)-ketamine (2.28 mg/kg/h) was significantly lower than that needed for racemic ketamine (3.12 mg/kg/h) (p = 0.037) with an analgesic/sedative potency ratio of 1.4:1. Balloon dilatation required significantly higher dosages in both groups (p = 0.043). Significantly more patients were excluded because of ineffective analgesia/sedation or severe side effects in the racemic ketamine group. The awakening period did not show significant differences between the two groups. S(+)-ketamine proved to be a more efficient analgesic/sedative drug in newborns and children. It was shown to be useful in diagnostic and interventional procedures and allows spontaneous breathing. Moderate side effects occurred in both groups; severe side effects seemed to occur more often with the racemic solution.

[Difficulties in diagnosing Marfan's syndrome in childhood and adolescence]. / Diagnostische Schwierigkeiten bei Patienten mit Marfan-Syndrom im Kindes- und Jugendalter.

BACKGROUND: The diagnosis of Marfan's syndrome in childhood and adolescence is made by the criteria of the Gent nosology, which evaluates genetic data, family history and a spectrum of clinical criteria. Due to the age dependent manifestations of the clinical symptoms, combined with the extreme heterogeneity of Marfan's syndrome diagnosis in early childhood remains sometimes difficult. PATIENTS: Prospectively, we analyzed the clinical symptoms of all patients where Marfan's syndrome was suspected. We evaluated those patients between January 1997 and April 2002 by an interdisciplinary approach. METHODS: We compared the clinical datas of the patients by using the Gent nosology and the Berlin nosolgy. RESULTS: 34 patients underwent full follow-up. The median age was 10,32 years with a range of 0,01 to 37,31 years, 19 patients were male, 15 patients were female. In eight patients Marfan's syndrome could be rouled out, 9 of 26 patients (34,6 %) fullified the criteria of the Gent nosology, in 17 of 26 patients (65,4 %) Marfan's syndrome remained just suspected, but was not fullified by the criteria of the Gent nosology. Concerning the Berlin nosology 14 of 26 patients (53,8 %) fullified the criteria, 12 of 26 patients (46,2 %) failed. Due to the criteria of the Gent nosology 14 patients (53,8 %) fullified the criteria of skeletal involvement, 21 patients (80,8 %) fullified cardiovascular major manifestation, 6 patients (23,1 %) had an ophthalmic major manifestation, and 9 patients (34,6 %) had an affected first degree relative or were genetically determined. CONCLUSIONS: On the basis of the data of our patients the diagnosis of Marfan's syndrome in childhood and adolescence can be made more sensitive by the criteria of the Berlin nosology compared to the Gent nosology. This seems to be caused by the age dependent manifestations of the symptoms. Until diagnostic algorhythms of Marfan's syndrome in childhood remain suboptimal, continuous clinical follow-up for all cases even those only in the case of suspected Marfan's syndrome are necessary to exclude complicated course and to improve outcome.

[Pulmonary plasma catecholamine levels and pulmonary hypertension in congenital heart disease]. / Pulmonale Plasmakatecholaminspiegel bei Patienten mit angeborenen Herzfehlern und erhöhtem pulmonalarteriellen Fluss.

Plasma catecholamines may play a role in the pathogenesis of pulmonary hypertension in congenital heart disease with increased pulmonary blood flow. At cardiac catheterization, blood samples were obtained before and after passage of the lung in patients with congenital heart disease with normal pulmonary blood flow (n=39), elevated pulmonary blood flow with normal pulmonary pressure and normal pulmonary vascular resistance (PVR) in patients with atrial septal defect (ASD) (n=57) or ventricular septal defect (VSD) (n=12), and increased pulmonary blood flow, pressure and vascular resistance in patients with ASD, VSD or both (n=22), or in patients with primary pulmonary hypertension (n=4). Plasma catecholamines were determined by reversed phase high performance liquid chromatography and electrochemical detection (HPLC-ECD). In patients with elevated PVR elevated norepinephrine levels (NE) were observed. In contrast, epinephrine levels (E) were not associated with the development of pulmonary hypertension. Patients with primary pulmonary hypertension had the highest levels of NE while patients with associated Down's syndrome had significantly lower epinephrine levels. Pulmonary plasma norepinephrine levels are increased in patients with elevated pulmonary vascular resistance. Whether this phenomena is the cause or sequalae of pulmonary hypertension needs further examination. In the future, plasma catecholamines may serve as a diagnostic feature or may result in further therapeutic options.

Acute hemodynamic effects of post cardiotomy peritoneal dialysis in neonates and infants.

OBJECTIVE: Evaluation of the acute hemodynamic changes during peritoneal dialysis in patients with low cardiac output syndrome and acute renal failure after open heart surgery. PATIENTS: Three newborns and three infants after corrective surgery of congenital heart disease with post cardiotomy dialysis. Five of these had an open thorax during dialysis. METHODS: Cardiac output measurements using the thermodilution technique on two consecutive days at four different times during the peritoneal dialysis cycle. RESULTS: We did not find a deterioration of the cardiac index or systemic vascular resistance measured over two cycles in each of the six patients. Pulmonary artery pressure rose slightly after instillation of the dialysate solution in all patients. Changes in central venous pressure and left atrial pressure were not clinically meaningful. In all patients fluid removal by peritoneal dialysis was effective. All five surviving patients recovered renal function. CONCLUSIONS: Peritoneal dialysis can be performed in newborns and infants following cardiac surgery without causing acute hemodynamic imbalances. An open chest may have a significant impact on hemodynamic stability during peritoneal dialysis by blunting any possible negative alterations of increased intraabdominal pressure.

[The clinical diagnosis of aortic isthmus stenosis]. / Klinische Diagnostik der Aortenisthmusstenose.

BACKGROUND AND OBJECTIVE: Unfortunately, congenital coarctation of the aorta (CoA) is in many cases not diagnosed until adulthood., even though this defect is known to cause serious complications if treated too late. This retrospective study of patients was undertaken to ascertain whether early, exclusively clinical, diagnosis would have been possible. PATIENTS AND METHODS: The case notes of 61 patients with native CoA (n = 45) or restenosis in adulthood after earlier surgical repair (n = 16) were analysed with regard to the findings on physical examination and the patients' symptoms. RESULTS: The patients' age ranged from 15 to 54 years (mean 23 +/- 15.5 years). 48 of 58 patients (83%; incomplete data excluded three patients) had hypertension in the brachial arteries and 50 of 58 (86%) had a pressure difference between the arms. 51 of 53 patients (96%) had a heart murmur, while a pressure gradient between the arms and legs was recorded in in 49 of 51 patients (96%). Notching of the ribs was noted in the chest radiogram of 47 of the 58 patients in whom it was taken. 35 patients had reported one, 23 more than one symptom. CONCLUSION: In all patients (with native CoA or restenosis after surgical repair) it would have been possible to make the correct diagnosis of CoA on the basis of hypertension in the arms, a difference in pulse amplitude and/or a pressure gradient between arms and legs, as well as a cardiac murmur. Normal pressures in ten patients could be explained by marked collateral circulation (rib notching), but even in these patients their symptoms plus at least two other main signs could have provided the correct diagnosis at an earlier time.

Peritoneal dialysis after infant open heart surgery: observations in 27 patients.

BACKGROUND: The role of peritoneal dialysis (PD) in the management of infants after heart operation is under discussion. The aim of this study was to investigate the effect of PD on fluid balance and outcome. METHODS: Twenty-seven (33%) of 81 consecutive infants who underwent heart operation required PD. In 22 patients (81%), PD was started prophylactically at the end of the operation. We recorded hemodynamic data and fluid balance. Patients experiencing acute renal failure (ARF) were compared with the remaining infants. RESULTS: Eleven of 81 patients (14%) experienced ARF; 3 of them died (4% of all patients undergoing operation, 27% of those with ARF). Complications of PD, present in 33%, were transitory and of minor significance. Patients with ARF had decreased cardiac function compared with those without ARF but similar fluid balance. CONCLUSIONS: Peritoneal dialysis is an effective and safe method for the treatment of ARF in infants after open heart operation. As PD is helpful in modulating postoperative fluid balance, prophylactic use of PD can be recommended for selected patients who are at risk for low cardiac output syndrome.

Renal impairment in patients with long-standing cyanotic congenital heart disease.

Nephropathy is known to occur in patients with long-standing cyanotic congenital heart disease (CCHD). In order to assess the incidence, nature and degree of the problem among such patients, discriminating urine analyses were performed in 26 patients with CCHD, with a mean age of 22 (10-42) y. Ten patients showed reduced glomerular function, six of whom also had advanced glomerulopathy. Glomerular filtration rates were below normal in half of the patients and occurred with glomerular-type proteinuria in five, with tubular-type proteinuria in one and without pathological proteinuria in four. An elevated haematocrit and duration of cyanosis were identified as the main risks factors for the development of glomerulopathy. The risk of developing glomerular lesions rose sharply during the second decade of life. Nephropathy in CCHD is common and the dominant feature is glomerular damage, which is related to the duration of cyanosis and the extent to which the haematocrit is elevated.