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 Nevertheless, dedicated balloons are lacking so far, as several products have been withdrawn from the market.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.

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.

[Cardiovascular risk factors in childhood. An anamnestic guideline]. / Kardiovaskuläre Risikofaktoren im Kindes- und Jugendalter. Eine anamnestische Handlungsanleitung.

Being overweight in childhood causes several cardiovascular risk factors which in turn contribute to accelerated atherosclerosis. Being overweight itself represents a risk factor, but also contributes to an increased prevalence of arterial hypertension, dyslipidemia and impaired glucose tolerance. Thus, cardiovascular prevention should be included in the management of obese children. Most of all, therapy of adiposity should be performed, as weight reduction and increased fitness represent protective factors. Moreover, a detailed cardiovascular workup and therapy of secondary vascular disease must also be performed. Subclinical changes at the level of the endothelium may be diagnosed using modern imaging techniques such as the measurement of the intima-media thickness of the carotid artery. In general, the overweight child should be considered as a future patient with vascular disease! The following article focuses on the prevalence, diagnostics and therapeutic options in the cardiovascular management of overweight children.

Total pubertal growth in patients with juvenile idiopathic arthritis treated with growth hormone: analysis of a single center.

OBJECTIVES: Growth failure is a permanent sequelae in juvenile idiopathic arthritis (JIA). The aim of the study was to compare pubertal growth in control and growth hormone (GH) treated JIA subjects. DESIGN: 64 children with JIA at a mean age of 10.38 ± 2.80 years were enrolled and followed until final height (measured in standard deviation (SD) scores). 39 children (20 m) received GH therapy and 24 (9 m) served as controls. GH dose was 0.33 mg/kg/week. Linear regression analysis was performed to identify factors influencing total pubertal growth. RESULTS: Mean total pubertal growth was 21.1 ± 1.3 cm (mean ± SD) in GH treated JIA patients and 13.8 ± 1.5 cm in controls. Final height was significantly higher with GH treatment (-1.67 ± 1.20 SD) compared to controls (-3.20 ± 1.84 SD). Linear regression model identified age at onset of puberty (ß=-4.2,CI: -5.9, -2.6 in controls and ß=-2.3,CI: -3.6, -1.1 in GH treated) as the main factor for total pubertal growth. Final height SDS was determined by the difference to target height at onset of puberty (ß=-0.59;CI: -0.80, -0.37 in controls and ß=-0.30,CI: -0.52, -0.08 in GH treated), age at onset of puberty (ß=0.47;CI:0.02,0.93 in controls and 0.23;CI: -0.00,0.46 in GH treated) and height gain during puberty (ß=0.13;CI:0.05,0.21 in controls and ß=0.11;CI:0.07,0.16 in GH treated). CONCLUSION: Total pubertal growth in JIA patients treated with GH was increased by a factor of 1.5 greater in comparison to controls leading to a significantly better final height. To maximize final height GH treatment should be initiated early to reduce the height deficit at onset of puberty.

[MRI for therapy control in patients with tetralogy of Fallot]. / MRT zur Therapiekontrolle bei Patienten mit Fallot-Tetralogie.

With prevalences ranging from 0.26 to 0.8‰ of all live births tetralogy of Fallot (TOF) is the most common congenital heart disease with primary cyanosis. Due to improvements in surgical techniques, nearly all patients can nowadays expect to reach adulthood. After surgical repair, pulmonary regurgitation (PR) occurs in almost every child and is an important contributing factor in long-term morbidity and mortality. Cardiac magnetic resonance imaging is well established for functional assessment and flow measurements and is an ideal tool for serial post-surgical follow-up examinations, as it is non-invasive and does not expose patients to ionizing radiation. The timing of pulmonary valve replacement is crucial as right ventricular (RV) volumes have only proven to normalize when preoperative end-diastolic volumes are <170 ml/m(2) and end-systolic volumes are <85 ml/m(2). After surgical repair up to 15% of patients have residual or recurrent pulmonary artery stenosis. Distal pulmonary branch stenosis can aggravate PR and lead to right heart failure due to combined pressure and volume overload. Therefore, it has to be diagnosed in time and treated by angioplasty with or without stenting.

[Modern therapy of congenital heart defects]. / Moderne Therapieverfahren bei angeborenen Herzfehlern.

Modern therapy of congenital heart defects consists of interventional and surgical procedures. The following report provides information about the most common congenital heart defects and the corresponding therapeutic options. Furthermore, the combined application of interventional and surgical procedures, so-called hybrid procedures, is described in detail as well as the latest developments in percutaneous valve replacement therapy.

Effects of growth hormone treatment in juvenile idiopathic arthritis: bone and body composition.

BACKGROUND: Inflammation and glucocorticoid therapy are major factors influencing growth and bone maturation in patients with juvenile idiopathic arthritis (JIA). In addition to alterations in total bone mineral density and bone geometry, longitudinal data confirm that the main contributors to errant bone maturation in patients with JIA are reductions in muscle mass and force. Growth hormone (GH) therapy, which has shown efficacy in controlling disease, may also positively influence body composition. For several years, GH therapy has been used to treat growth retardation in patients with JIA receiving glucocorticoids. GH therapy normalizes growth velocity, increases height, bone mineral density and bone mass and changes bone geometry. Despite ongoing glucocorticoid therapy, muscle mass and bone size substantially increase with GH therapy. Increased bone size suggests improved bone stability, which may reduce fracture risk. Along with the increase in muscle mass, patients experience stabilized or slightly decreased fat mass during GH therapy. CONCLUSIONS: All these effects suggest an anabolic effect of GH therapy on bone and body composition.

Successful ABO-incompatible heart transplantation in a child despite blood-group sensitization after ventricular assist device support.

In the first two yr of life blood-group incompatible (ABO-incompatible) heart transplantation can be performed leading to immune tolerance to donor blood group. Antibody titers should be below 1:4. VAD use is correlated with sensitization toward blood-group antigens. A boy was diagnosed with dilated cardiomyopathy at nine months of age and listed for 0-compatible transplantation. Progressive heart failure required implantation of a left VAD. His listing was extended for ABO-incompatible transplantation despite antibody titers of 1:32 anti-A and 1:8 anti-B. After 26 days on VAD, he was transplanted with a B donor heart. No hyperacute or acute rejection occurred in 12 months post-transplant. Anti-B antibodies rose to a maximum of 1:2. No use of rituximab or plasmapheresis was required. There are no signs of graft vasculopathy. This indicates that inclusion criteria for ABO-incompatible transplantation may be extended to immediate cases. This is the first case with a healthy immune system to show signs of tolerance development after ABO-incompatible heart transplantation with increased prior antibody titers and without specific treatment.

Gastric outlet obstruction after long-term prostaglandin administration mimicking hypertrophic pyloric stenosis.

Prostaglandin E1 (PGE1) is widely used in neonates with cyanotic congenital heart disease who depend on the patency of the ductus arteriosus for oxygenation. Side effects of prostaglandin therapy are common and include respiratory depression, generalized flushing, and cardiovascular and neurological effects. Little is known about the complex effects on the gastrointestinal tract. We report on an infant with gastric outlet obstruction after long-term prostaglandin administration. At the age of 1 month, feeding problems developed with projectile vomiting. Ultrasonography showed progressive elongation of the antropyloric channel without wall thickening, which was causing gastric outlet obstruction. Three days after cardiac surgery and cessation of prostaglandin therapy, the infant fed normally and rapidly gained weight. The clinical signs in such patients can mimic hypertrophic pyloric stenosis. Therefore, the sonographic findings should not be confused with pyloric wall thickening to avoid a false diagnosis and unnecessary surgery. The symptoms diminish with cessation of the prostaglandin therapy after a corrective cardiac operation.

Impaired short-term blood pressure regulation and autonomic dysbalance in children with type 1 diabetes mellitus.

AIMS: Because reduction in baroreceptor sensitivity (BRS) has been associated with hypertension in the normal population and with increased cardiovascular morbidity and mortality in patients with diabetes mellitus, we measured BRS in a patient cohort of children with type 1 diabetes mellitus. METHODS: Two hundred and eight children (150 patients with type 1 diabetes mellitus, mean age 13.9 +/- 2.8 years, 70 boys, mean HbA(1c) 7.8 +/- 1.4%; and 58 healthy controls, mean age 14.1 +/- 3.1 years, 32 boys) were studied. BRS and heart rate variability (HRV) were analysed from a short-time ECG and BP recording using the sequence method (BRS) and the frequency domain method (HRV). RESULTS: There were 111 of 150 patients (74%) and 5 of 58 controls (8.6%) that showed impaired BRS. Mean BRS differed significantly between patients and controls (18.4 +/- 7.2 vs 25.8 +/- 8.2 ms/mm, p < 0.001). BRS correlated inversely with systolic BP (r = -0.23, p = 0.009) and was related to diabetes duration (r = -0.194, p = 0.027). Analysis of HRV showed greater sympathetic and less parasympathetic influence in patients than in controls (low frequency/high frequency ratio 1.3 +/- 0.8 vs 0.9 +/- 0.6, p < 0.05); the low frequency/high frequency ratio was inversely correlated with BRS (r = -0.28, p = 0.001). CONCLUSIONS/INTERPRETATION: Diabetic children show reduced BRS. In our patient group, the single risk factor for this finding was found to be the disease duration. The degree of BRS impairment was related to the degree of autonomic dysbalance.

Recurrent pericarditis in children: elevated cardiac autoantibodies.

UNLABELLED: Recurrent pericarditis is a rare disease in childhood. Nevertheless, it may represent a challenge to the clinician due to its resistance to anti-inflammatory treatment. The initial etiology often remains unclear; specific laboratory parameters predicting the frequency or severity of the recurrences are lacking. We report on four patients with recurrent pericarditis in whom antimyolemmal antibodies (AMLAs) were detected. A prolonged persistence of IgM-type AMLAs was found in three patients: two of them presented with acute inflammation as the initial event and one with 48 recurrences during 5.5 years. The fourth patient showed a fast conversion from IgM to IgG-type AMLAs after a less acute initial presentation and showed 4 mild recurrences during the 48-month follow-up. CONCLUSION: We were able to detect AMLAs in four children with recurrent pericarditis. This finding may be attributed to an auto-immunological disease following a first, acute event. We propose the detection of AMLAs in all children with unexplained recurrent pericarditis. Pediatric patients with a persistence of IgM-type AMLAs may face frequent recurrences and should be monitored therefore more closely. In addition, medical treatment may be changed in these patients with a slower tapering of the dosage of steroidal and non-steroidal antiinflammatory drugs.

Pubertal height gain in Ullrich-Turner syndrome.

Short stature and ovarian failure are the main features in Ullrich-Turner syndrome (UTS). The aim of this retrospective analysis was to evaluate the influence of age at initiation of puberty on final height. Sixty-five girls were treated with growth hormone (GH) and had a final height of 150.6 +/- 5.7 cm; 12/65 entered puberty spontaneously. A non-GH treated group of 12 girls with UTS reached a final height of 147.3 +/- 6.6 cm. Subdividing the GH treated group (n = 53) based on the age at induction of puberty, before or after 13 years, there was no significant difference in final height. Final height was affected by the age at which GH treatment was initiated, height SDS at the beginning of puberty, and by the duration of GH therapy. Therefore, early treatment with GH for short stature in UTS should be attempted so that an age adequate initiation of puberty will be feasible.

First experience with the 2005 modified Gore Helex ASD occluder system.

We report about our initial experience for the in 2005 modified Helex (Gore) device for closure of atrial septal defects (ASD) and persistent foramen ovale (PFO). Major changes were made at the delivery system for simplifying the Helex implantation procedure. We treated 11 patients, 8 children and 3 adults, with ages between 3 and 62 years. In 10 patients the diagnosis was a relevant ASD with volume overload of the right heart (Left to right shunts between 30 and 50%). One adult (age 58 years) have had a small left to right shunt with a PFO-like defect and the history of 2 neurologic embolic events. In 3 patients we found 2 defects. In all patients a Helex occluder was implanted successfully. The mean fluoroscopy time was 8,4 minutes. The immediate occlusion rate after 24 hours was 91%. In all cases there was a very good adaptation of the device to the anatomical structures. In this small series, the Helex occluder appears to offer a reliable system of occlusion for small and moderate ASDs and for PFO with minimal risk of major complications.

Short-term testing of heart rate variability in heart-transplanted children: equal to 24-h ECG recordings?

INTRODUCTION: Heart rate variability (HRV) is reduced in adults and children after cardiac transplantation. Testing of HRV has been used to assess re-innervation of the cardiac graft; its reliability in ruling out acute graft rejection is still under investigation. This study used a short-term test on HRV in 23 heart and heart-lung transplanted children and adolescents and compared the results with 24-h ECG recordings. PATIENTS AND METHODS: Twenty-three subjects (16.3+/-4.2 yr; 10 females) underwent a 10-min HRV test at two occasions and one 24-h ECG. HRV was calculated according to the time domain method (RR interval, standard deviation of RR interval) and the frequency domain method (total power, LF and HF for assessment of sympathovagal modulation of heart rate). RESULTS: Correlation between the short-term tests and 24-h ECG was high with regard to the frequency domain analysis of HRV. Correlation was less pronounced in the time domain method. CONCLUSIONS: In heart and heart-lung-transplanted children and adolescents, due to reduced overall HRV short-term testing may give as reliable data as 24-h ECG. Therefore, especially when power spectral analysis has to be performed as a longitudinal assessment of re-innervation of the cardiac graft, short-term testing may offer an easily applicable and non-invasive diagnostic tool. Further studies are warranted to investigate whether HRV testing may contribute to rule out acute graft rejection.

Young adults born small for gestational age: is reduced baroreceptor sensitivity a risk factor for hypertension?

UNLABELLED: BACKGROUND. Adults born small for gestational age (SGA) are at increased risk for the metabolic syndrome and cardiovascular disease. HYPOTHESIS: Impaired short-term blood pressure regulation may contribute to the development of hypertension in patients born SGA. METHODS: In all, 43 patients born SGA (18 female, age 19.4 +/- 0.3 years) were evaluated by beat-to-beat blood pressure and heart rate registration during rest and mental and orthostatic stress. The study group was divided into Group 1 with normal resting blood pressure (n=32) and Group 2 with slightly elevated blood pressure (n=11). Baroreceptor sensitivity (BRS) was calculated. Fasting insulin as well as lipid levels were correlated with hemodynamic parameters. RESULTS: Eleven of the 43 study patients (25%) had a slightly elevated resting systolic blood pressure (SBP) rising during mental and orthostatic stress. Body mass index (BMI) and fasting insulin levels correlated strongly with SBP in Group 2. Baroreceptor sensitivity was lower in Group 2 at rest (p < 0.05). CONCLUSIONS: Three components of metabolic syndrome (elevated BP, high BMI, elevated insulin levels) correlate strongly in young adolescents born SGA; BRS is reduced in prehypertensive patients. Close follow-up is warranted during adult life as they are predisposed for developing a metabolic syndrome with elevated cardiovascular risk.

Impedance cardiography and beat-to-beat blood pressure monitoring in diagnosis of syncope in long-QT syndrome.

Syncope represents a diagnostic challenge in patients affected by long-QT syndrome (LQTS). It is crucial for the therapeutic decision to distinguish between potentially life-threatening ventricular tachycardias (Torsadede-pointes) and-especially during adolescence-neurocardiogenic syncopes. This case report presents a patient with LQTS2 (mutation in the HERG gene) on medication with beta-blocker, in whom a head-up-tilt table test was performed after syncope of unknown origin. The test was chosen in order to reproduce the circumstances under which the syncope had happened. The monitoring device consisted of impedance cardiography as well as non-invasive beat-to-beat blood-pressure measurement. By these means it was possible to register a reduced peripheral vascular resistance after tilting the patient, a reduced cardiac output and bradycardia leading to syncope after four minutes of upright posture. This was suggestive for neurocardiogenic syncope as a cause for the spell experienced. Further non-invasive diagnostic methods were normal. As the patient's family history was negative for syncopes, Torsade-de-pointes seemed unlikely.In this special case the non-invasive monitoring system of cardiac output, peripheral vascular resistance and beat-to-beat blood pressure measurements was useful as a supplemental tool during evaluation of syncope and helpful in decision-making against implantation of an ICD and for a more intense treatment with betablockers. Such monitoring devices offer new insights into the orthostatic regulation in young patients.

Head-up-tilt testing in children: new perspectives using beat-to-beat blood-pressure monitoring.

The head-up-tilt-test in pediatric patients for the evaluation of syncope shows a sensitivity of 35-85% and often requires pharmacological stimulation in order to improve its diagnostic value. We used a new device for beat-to-beat blood pressure monitoring combined with impedance cardiography in a 12-year-old girl during tilt testing. A seven seconds asystolia was provoked. The haemodynamic parameters showed clearly the drop in heart rate as well as in cardiac output, and returned to normal values after tilting back the patient. With the help of this new monitoring device, the sensitivity and specificity of head-up-tilt-testing can probably be improved.

Musculoskeletal and functional muscle-bone analysis in children with rheumatic disease using peripheral quantitative computed tomography.

Bone demineralization is a severe complication of juvenile idiopathic arthritis (JIA) and other rheumatic diseases. To identify patients, who are at risk of bone disease, musculoskeletal analysis is performed. Furthermore, a more functional approach is needed to assess, whether bone strength is adequate for muscle force and whether muscle force is adequate for body size. In patients with a chronic disease it is most important to differentiate between primary bone problems and those that are secondary to low muscle force. To implement this approach, we measured musculoskeletal parameters of the radius in 94 patients with juvenile idiopathic arthritis of different subtypes and connective tissue disease using peripheral quantitative computed tomography. The four groups consisted of patients with oligoarticular (n = 31), polyarticular (n = 27), systemic JIA (n = 20) and connective tissue disease (CTD) (n = 16). All patients with systemic JIA and CTD and 56% of the patients with polyarticular JIA were under treatment with glucocorticoids. In general, the longer the duration of the disease and the more severe the subtype of the rheumatic disease, the shorter the height and the lower the bone density and bone strength parameters. Mean height, bone mineral content (BMC) and muscle cross-sectional area (CSA) were low for age, but muscle CSA was normal for height with the exception of patients with polyarticular disease. In the systemic JIA group the ratio of BMC per muscle CSA was decreased by -1.7+/-2.7 SD (P < 0.05), suggesting that bone strength was not adequately adapted to muscle force. This was even more expressed in females than in males (14 versus 3). These patients need closer follow up and potential specific therapeutic intervention.

["Real time"-3D-echocardiography: a valuable new tool in preoperative assessment of complex cardiac defects]. / "Real Time"-3D-Echokardiographie: Erfahrungen im Kindesalter. Fallbeispiel für die Einsatzmöglichkeit der dreidimensionalen Echtzeit-Echokardiographie in der präoperativen Abklärung angeborener Herzfehler.

Evaluating complex cardiac defects in small children preoperatively requires multiple diagnostic procedures including echocardiography, but also the invasive methods such as cardiac catheterisation, computer-tomography and magnetic resonance imaging. We tried to assess the complex anatomy of the atrioventricular valves in atrioventricular septal defect using bedside real-time three-dimensional echocardiography and comparing these results to the anatomic findings at the time of operation.

Real-time three-dimensional ultrasound - a valuable new tool in preoperative assessment of complex congenital cardiac disease.

Evaluating complex cardiac defects in small children preoperatively requires multiple diagnostic procedures including echocardiography, and also invasive methods such as cardiac catheterisation, computer-tomography and magnetic resonance imaging. We assessed the complex anatomy of the atrioventricular valves in atrioventricular septal defect using bedside real-time three-dimensional echocardiography and comparing these results to the anatomic findings at the time of operative intervention.