Monitoring unfractionated heparin in children: a parallel-cohort randomized controlled trial comparing 2 dose protocols.

Monitoring unfractionated heparin (UFH) is crucial to prevent over- or under-anticoagulation. However, the optimal parameters for monitoring UFH in children are not well established. The study objectives were to investigate (1) the relationship between UFH dose and its anticoagulant effect as assessed by anti-Xa, activated partial thromboplastin time (aPTT) and activated clotting time (ACT); (2) other factors influencing UFH effect; (3) the agreement between the assays; and (4) the association between UFH effect and clinical outcome. HEARTCAT was a parallel-cohort randomized controlled trial comparing high-dose (100 U/kg bolus followed by age-based continuous infusion in randomized children) vs low-dose UFH (50 U/kg bolus) during cardiac catheterization in children. Blood samples were drawn before and after UFH administration at 30, 60, and 90 minutes. Four-hundred and two samples of 149 patients were evaluable. Anti-Xa, aPTT, and ACT all showed good discrimination between UFH doses. Regression models demonstrated the following determinants of UFH effect: UFH dose, age, baseline antithrombin (for anti-Xa), and baseline levels of aPTT and ACT, respectively. UFH effects were lower in infants compared with older children, which was more pronounced at low-dose than at high-dose UFH. Agreement between the 3 assays was poor. Most aPTT values were above therapeutic range or beyond measuring limit and thus of limited value for UFH monitoring. No association of UFH dose or effect with clinical outcome could be observed. In conclusion, all assays reflected a significant UFH dose-effect relationship, however, with poor agreement between the respective tests. The age-dependency of UFH effect was confirmed. Notably, the influence of age on UFH effect was dose-dependent.

The "Polar Light Sign" is a useful tool to detect discrete membranous supravalvular mitral stenosis.

Mitral valve stenosis caused by a discrete supravalvular membrane is a rare congenital malformation haemodynamically leading to significant mitral valve stenosis. When the supravalvular mitral stenosis consists of a discrete supravalvular membrane adherent to the mitral valve, it is usually not clearly detectable by routine echocardiography. We report about the typical echocardiographic finding in three young patients with this rare form of a discrete membranous supravalvular stenosis caused by a membrane adherent to the mitral valve. These cases present a typical echocardiographic feature in colour Doppler generated by the pathognomonic supramitral flow acceleration. Whereas typical supravalvular mitral stenosis caused by cor triatriatum or a clearly visible supravalvular ring is easily detectable by echocardiography, a discrete supravalvular membrane adjacent to the mitral valve leaflets resembling valvular mitral stenosis is difficult to differentiate by routine echocardiography. In our opinion, this colour phenomenon does resemble the visual impression of polar lights in the northern hemisphere; owing to its typical appearance, it may therefore be named as "Polar Light Sign". This phenomenon may help to detect this anatomical entity by echocardiography in time and therefore improve the prognosis for repair.

Paediatric patients with inflammatory bowel disease who received infliximab experienced improved growth and bone health.

AIM: Children with inflammatory bowel disease (IBD) have a high prevalence of growth retardation and low bone mineral density (BMD). This retrospective study investigated whether the start of infliximab treatment (IFX) was associated with improvement of growth and bone health. METHODS: Anthropometry, BMD and bone markers were measured 1 year before and after the start of IFX treatment in 33 patients (51% males), with a median age of 13.5 years at baseline. Outcomes were growth with treatment and indicators of improved bone health. RESULTS: Twenty-one children (64%) experienced a positive catch-up growth after IFX. Height standard deviation scores (SDS) were significantly higher in children in remission. Treatment with IFX was associated with a statistically significant increase in 25-hydroxycholecalciferol (25-OHD, p = 0.01). IFX had no influence on BMD. Children with low BMD < -2 had significantly higher inflammation scores, lower body mass index, weight, height SDS and 25-ODH after IFX. CONCLUSION: After treatment with IFX, children with IBD improved significantly in weight, with the majority achieving positive catch-up growth. Bone mass tended to remain static with time of treatment with IFX, despite a significant increase in 25-OHD. Improved nutritional status positively predicts improved bone mineralisation.

Radiofrequency catheter ablation can be performed with high success rates and very low complication rates in children and adolescents.

AIM: Radiofrequency catheter ablation is a standard treatment for tachyarrhythmia in children. Recently, several centres using cryoenergy for ablation have reported high success and low complication rates, but an increased risk of recurrence of arrhythmia. The aim of this study was to report success, complications and recurrence rates for radiofrequency catheter ablation in children under current conditions. METHODS: A retrospective cohort study of 333 consecutive children undergoing radiofrequency catheter ablation over the last two decades. RESULTS: Radiofrequency catheter ablation was performed successfully in 96.7% of patients, but was significantly less successful in patients with a right anterior or right anterolateral accessory pathway (81.8%). Overall mortality was 0%; there was only one (0.3%) major complication, a pericardial haemorrhage, and only 2.8% of the patients developed minor vascular complications at the puncture site. None of the patients developed a persistent atrioventricular block. Over a median observation time of 4.3 years (0.1; 17.8), recurrence of arrhythmia occurred in 9.3% of all patients, but 50% of children with right lateral accessory pathways. CONCLUSION: Radiofrequency catheter ablation can be performed with high success and very low complication rates in children. Recurrence rates are substantially lower than those reported for cryoablation in the literature.

Percutaneous closure of atrial septal defects in spontaneously breathing children under deep sedation: a feasible and safe concept.

Interventional cardiac catheterization in children and adolescents is traditionally performed with the patient under general anesthesia and endotracheal intubation. However, percutaneous closure of atrial septum defect (ASD) without general anaesthesia is currently being attempted in a growing number of children. The study objective was to evaluate the success and complication rate of percutaneous ASD closure in spontaneously breathing children under deep sedation. Retrospective single centre cohort study of consecutive children undergoing percutaneous ASD closure at a tertiary care pediatric cardiology centre. Transesophageal echocardiography (TEE) and percutaneous ASD closure were performed with the patient under deep sedation with intravenous bolus of midazolam and ketamine for induction and propofol continuous infusion for maintenance of sedation in spontaneously breathing children. One hundred and ninety-seven patients (median age 6.1 years [minimum 0.5; maximum 18.8]) underwent TEE and ASD balloon sizing. Percutaneous ASD closure was attempted in 174 patients (88 %), and device implantation was performed successfully in 92 %. To achieve sufficient deep sedation, patients received a median ketamine dose of 2.7 mg/kg (0.3; 7) followed by a median propofol continuous infusion rate of 5 mg/kg/h (1.1; 10.7). There were no major cardiorespiratory complications associated with deep sedation, and only two patients (1 %) required endotracheal intubation due to bronchial obstruction immediately after induction of sedation. Seventeen patients (8 %) had minor respiratory complications and required frequent oral suctioning or temporary bag-mask ventilation. TEE and percutaneous ASD closure can be performed safely and successfully under deep sedation in spontaneously breathing children of all ages.

Anticoagulation in children undergoing cardiac surgery.

Advances in medical and surgical care have resulted in improved survival of patients with congenital heart disease (CHD). Parallel to these progresses, an increasing number of immediate and long-term complications have been recognized. One important complication in CHD is the development of thrombosis. Children with a single functional cardiac ventricle usually require sequential steps of surgery: the initial Blalock-Taussig shunts (BTS) during the neonatal period, followed by the Glenn shunt, and finally, the Fontan shunt, the "definitive palliative" procedure. Surgery mostly involves cardiopulmonary bypass (CPB), which also affects the coagulation system and causes an inflammatory response. This article will review surgical procedures, such as BTS, Glenn shunt, and Fontan shunt, prosthetic mechanical valves, and CPB, and their risk of thrombotic complications. There is insufficient evidence and no consensus for optimal anticoagulant prophylaxis or treatment in children with CHD. Current recommendations are mostly based on adult data.

A Cyanotic Newborn with a Pink Right Upper Extremity.

Aberrant origin of the subclavian artery (SCA) is a well-known vascular anomaly as part of congenital heart diseases with the left subclavian artery (LSCA) being more frequently affected than the right subclavian artery (RSCA). Complete isolation of the SCA is an even more infrequent aortic arch anomaly, occurring in less than 1% for the LSCA and even less for the RSCA. Isolation of the RSCA in patients with d-transposition of the great arteries (D-TGA) is even scanter with only a hand full of cases being reported in the literature. However, isolation of the RSCA has important implications on hemodynamics and surgical strategies. In this case report, we present a newborn patient with D-TGA which presented with distinct differential cyanosis. While the right upper extremity appeared pink with an oxygen saturation of 100%, the rest of the body was cyanotic. At first, this appearance was interpreted as the Harlequin phenomenon during primary care. However, detailed echocardiography revealed an aberrant origin of the RSCA from the right pulmonary artery, which led to the differential cyanosis. The patient underwent arterial switch operation on day of life two including dissection and reimplantation of the RSCA. The special hemodynamic situation of this is discussed in terms of pathophysiology and as well as its impact on perioperative and surgical management.

Special schooling in children with congenital heart disease: a risk factor for being disadvantaged in the world of employment.

The objective of this study was to evaluate type of schooling in children with congenital heart disease (CHD) who were inpatients at a tertiary pediatric cardiology center. This retrospective cohort study included 227 consecutive children with CHD (male, 125; female, 102) who had been inpatients from 1996 to 2005. Data on type of schooling had been documented by the in-hospital teacher at the time of admission. Medical data were obtained by reviewing medical charts. The primary endpoint was the percentage of children requiring special schooling, which was related to the respective percentage in the Austrian pediatric background population. Furthermore, the influence of clinical and demographic covariables was assessed. Fifteen percent (vs. 3.6% in the background population) of the study cohort required special schooling; 86% of them had a history of cardiac surgery. Cardiopulmonary bypass surgery in the first year of life showed a trend for an association with an increased frequency of special schooling. There were no significant associations with the Aristotle Basic Score (a measure for procedure complexity in CHD), gender, or first language. In conclusion, the need for special schooling is increased in children with CHD.

Prediction of spontaneous preterm delivery in twin pregnancies by cervical length at mid-gestation.

The objective of our study was to evaluate the correlation of the cervical length at 20-25 weeks of gestation with the incidence of spontaneous preterm delivery in twins in a country with a high incidence of preterm delivery compared to other European countries. Cervical length was measured in 262 consecutive patients. Previous preterm delivery before 34 weeks of gestation, chorionicity, maternal age, body-mass-index, smoking habit and parity were recorded as risk factors for preterm delivery. Women who were symptomatic at 20-25 weeks and who delivered because of other reasons than spontaneous labour and preterm rupture of membranes or at term were excluded. The primary outcome was incidence of preterm birth before 34 weeks. Two hundred and twenty-three patients were analyzed. Thirty-two (14%) delivered before 34 weeks. There was a significant correlation between cervical length of less than 25 mm and spontaneous delivery before 34 weeks (50% vs. 13%, p = .007). In addition, logistic regression analysis found cervical length to be the only significant predictor of spontaneous delivery before 34 weeks (OR 1.084; 95% CI 1.015; 1.159; p = .017). We conclude that the risk of severe preterm delivery in twins is high. Cervical length at mid-gestation was the only predictor of delivery before 34 weeks.

Optimal septum alignment of the Figulla Flex occluder to the atrial septum in patients with secundum atrial septal defects.

AIMS: To investigate the implantation safety, anatomic performance and septal alignment of the Occlutech Figulla Flex occluder (FFO) device, an atrial septal defect (ASD) closure device with specific left-sided deployment characteristics and right-sided septal alignment properties. METHODS AND RESULTS: Between January 2011 and December 2013 we prospectively collected the change of orientation of the device to the septum during the release process and the feasibility of implantation of the FFO in 122 patients. The mean age was 10.7 years (±10.2), weight 32.9 kg (±20.3), and height 129.4 cm (±30). Devices used were 9 (n=13), 10.5 (n=16), 12 (n=16), 15 (n=39), 18 (n=17), 21 (n=8), 24 (n=5), 27 (n=7) and 30 mm (n=3) in size. No additional implantation techniques were required. Before release, the mean angles of the left and right-sided discs were 29.2° (±9.9°) and 43.4° (±9.2°) to the body axis, and 18.7° (±8.7°) and 27.0° (±10°) immediately thereafter. Thus, there was only a slight change in orientation of the left-sided (10.6°±7.5°) and right-sided (16.3°±7.9°) discs. CONCLUSIONS: The design of this occluder system results in an ideal septum alignment which increases its feasibility as well as patient safety during implantation.

Compression, distortion and dislodgement of large caliber stents in congenital heart defects caused by cardiopulmonary resuscitation: a case series and review of the literature.

Stenting of vascular, extracardiac or lately intracardiac stenosis has become an established interventional treatment for a variety of problems in congenital or acquired heart disease. Most stent procedures are completed successfully and the long-term outcome is favorable in the majority of cases. Stent collapse or deformation is a well recognized entity in peripheral stents and can be attributed to insufficient radial force; it can also be attributed to excessive external forces, like deformation of stents in the right ventricular outflow tract, where external compression is combined with continuous movement caused by the beating heart. The protection of the thoracic cage may prove to be insufficient in extraordinary circumstances, such as chest compression in trauma or cardiopulmonary resuscitation (CPR). In this case series, we describe three patients in whom large endovascular stents were placed to treat significant stenosis of the aorta, the aortic arch or the venous system of the inferior vena cava close to the atrium. In all patients, CPR was necessary during their clinical course for various reasons; after adequate CPR, including appropriate chest compression all patients survived the initial resuscitation phase. Clinical, echocardiographic as well as radiologic re-evaluation after resuscitation revealed significant stent distortion, compression, displacement or additional vascular injury. The possibility of mechanical deformation of large endovascular stents needs to be considered and recognized when performing CPR; if CPR is successful, immediate re-evaluation of the implanted stents--if possible by biplane fluoroscopy--seems mandatory.

Incidence and diagnosis of thrombosis in children with short-term central venous lines of the upper venous system.

OBJECTIVES: Deep venous thrombosis in children is frequently related to central venous lines. Study objectives were to determine objectively the incidence of deep venous thrombosis in children with short-term central venous lines and to assess the diagnostic value of venography, venous ultrasonography, and echocardiography, in a prospective cohort study. METHODS: Consecutive children with congenital heart disease requiring short-term central venous lines in the upper venous system were screened systematically for deep venous thrombosis by using venography, venous ultrasonography, and echocardiography, according to standardized protocols. RESULTS: The study population consisted of 90 children (median age: 2.7 years; range: birth to 18 years). Most central venous lines (97%) were located in the jugular veins. The overall incidence of deep venous thrombosis was 25 cases (28%) among 90 children. Venography identified deep venous thrombosis located in the subclavian and central veins but missed most deep venous thrombosis in the jugular veins. Venous ultrasonography had good sensitivity in the jugular veins but did not detect deep venous thrombosis in central veins. Echocardiography detected only 1 case of central deep venous thrombosis. CONCLUSIONS: The incidence of central venous line-related deep venous thrombosis in children with short-term central venous lines is high and comparable to reports for children with long-term central venous lines. Sensitivities of venography, venous ultrasonography, and echocardiography in children vary depending on the affected venous segment. A combination of diagnostic tests is required for sensitive detection of central venous line-related deep venous thrombosis in the upper venous system.

Predictors of spontaneous closure of isolated secundum atrial septal defect in children: a longitudinal study.

OBJECTIVES: The goals were to assess the frequency of spontaneous closure of isolated secundum atrial septal defect in children and to identify predictors of spontaneous atrial septal defect closure. METHODS: A retrospective cohort study was performed in a tertiary care pediatric cardiology center. Consecutive patients (n = 200) diagnosed as having isolated atrial septal defects (no multiple or fenestrated atrial septal defects, no additional congenital heart disease, and no syndromes) were monitored for > 6 months with serial 2-dimensional echocardiography, according to a standardized protocol. RESULTS: The median age at diagnosis was 5 months (minimum: 0 months; maximum: 13.9 years). The atrial septal defect diameter at diagnosis was 4 to 5 mm in 40% of cases, 6 to 7 mm in 28% of cases, 8 to 10 mm in 21% of cases, and > 10 mm in 11% of cases. The median age at the final follow-up evaluation was 4.5 years (range: 6.8 months to 16.2 years). Thirty-four percent of atrial septal defects showed spontaneous closure, and 28% decreased to a diameter of < or = 3 mm. Logistic regression analysis revealed atrial septal defect diameter and age at diagnosis as independent predictors of spontaneous closure or regression to < or = 3-mm defect size. Of atrial septal defects with a diameter of 4 to 5 mm at diagnosis, 56% showed spontaneous closure, 30% regressed to a diameter of < or = 3 mm, and none required surgical closure. Of atrial septal defects with a diameter of > 10 mm at diagnosis, none closed spontaneously, whereas 77% required surgical or device closure. Gender and observation time were not associated with spontaneous atrial septal defect closure or regression to < or = 3 mm. CONCLUSIONS: In the present study population of children with atrial septal defects, 62% showed spontaneous closure (34%) or regression to < or = 3 mm (28%). Initial atrial septal defect diameter was the main predictor of spontaneous closure.