Echocardiography: conotruncal anomaly: a case of common arterial trunk with intact ventricular septum and hypoplastic left heart complex with unbalanced pulmonary stenoses.

We describe the echocardiographic findings of a common arterial trunk with intact ventricular septum, mitral and left ventricular hypoplasia, atretic left ventricular outlet and bilateral, and unbalanced pulmonary artery stenoses.

Open field stress testing: finally an optimal method in young children? Reference values for mobile cardiopulmonary exercise testing in healthy children aged 4-8 years.

INTRODUCTION: Cardiopulmonary exercise testing represents the diagnostic tool for determining cardiopulmonary function. Especially in small children, exercise testing is extremely challenging. To address this problem, field testing has been implemented using small mobile devices. This study aims at using this protocol for developing normal values for cardiopulmonary exercise testing in very young children. MATERIAL AND METHODS: Healthy children aged 4-8 years were recruited. All children were tested according to an outdoor protocol, in which they were instructed to walk, then run slowly, then a little harder and at last run at full speed. Each step lasted for 2 minutes, except the last step, in which the children were instructed to maintain as long as possible. RESULTS: A total of 104 children (64 female/35 male, mean age 6.6 years) performed outdoor cardiopulmonary exercise testing using a mobile device. Almost all tests were completed successfully (95%). Despite a predominance of female study subjects, anthropometric values did not differ between boys and girls. V̇O2peak/kg, respiratory exchange ratio, VT1, heart rate at VT1, and time of exercise were also comparable between sexes. Generally, a tendency of higher maximal oxygen uptake could be observed in older children. CONCLUSION: Open field mobile cardiopulmonary exercise testing represents a novel approach in very young children. In this study, we were able to determine normal values of maximal oxygen uptake and OUES/kg for 4-8-year-old children. The method is easy to achieve and safe.

Retrospective study of complete atrioventricular canal defects: Anesthetic and perioperative challenges.

OBJECTIVE: The objective of this study was to highlight anesthetic and perioperative management and the outcomes of infants with complete atrioventricular (AV) canal defects. DESIGN: This retrospective descriptive study included children who underwent staged and primary biventricular repair for complete AV canal defects from 1999 to 2013. SETTING: A single-center study at a university affiliated heart center. PARTICIPANTS: One hundred and fifty-seven patients with a mean age at surgery of 125 ± 56.9 days were included in the study. About 63.6% of them were diagnosed as Down syndrome. Mean body weight at surgery was 5.6 ± 6.3 kg. METHODS: Primary and staged biventricular repair of complete AV canal defects. MEASUREMENTS AND MAIN RESULTS: A predefined protocol including timing of surgery, management of induction and maintenance of anesthesia, cardiopulmonary bypass, and perioperative intensive care treatment was used throughout the study. Demographic data as well as intraoperative and perioperative Intensive Care Unit (ICU) data, such as length of stay in ICU, total duration of ventilation including reintubations, and total length of stay in hospital and in hospital mortality, were collected from the clinical information system. Pulmonary hypertension was noted in 60% of patients from which 30% needed nitric oxide therapy. Nearly 2.5% of patients needed permanent pacemaker implantation. Thorax was closed secondarily in 7% of patients. In 3.8% of patients, reoperations due to residual defects were undertaken. Duration of hospital stay was 14.5 ± 4.7 days. The in-hospital mortality was 0%. CONCLUSION: Protocolized perioperative management leads to excellent outcome in AV canal defect repair surgery.

Shear-stress induced acquired von Willebrand syndrome in children with congenital heart disease.

OBJECTIVES: To determine the frequency and severity of acquired von Willebrand syndrome (AVWS) in children with stenotic congenital heart disease (CHD) before and after intervention. METHODS: In this single-centre prospective observational case-control study, 50 children [median age: 26 (range, 0-175) months, bodyweight: 9.5 (2.2-7.5) kg] underwent catheter interventions or cardiac surgery. A total of 26 children with high stenosis [mean gradient: 80 (range, 52-130) mmHg] represented the stenosis group and 24 without relevant stenosis (<20 mmHg) served as the control group. von Willebrand factor (VWF) was analysed with respect to quantity and function before and after corrective or palliative intervention. RESULTS: Demographic data were comparable. The stenosis group had more surgical and the control group more interventional procedures (P = 0.025). Before intervention, 13 patients from the stenosis group (50%) showed a significant reduction in VWF-multimers compared with no patients in the control group (P <0.001). Collagen binding capacity (VWF:CB) was lower in the stenosis group [0.5 (0.2-2.6) U/ml vs 0.8 (0.4-2.1) U/ml, P <0.05), as was the collagen binding capacity to antigen ratio (VWF:CB/Ag) [0.8 (0.4-1.4) U/ml vs 1 (0.4-1.7) U/ml, P <0.001). After intervention, VWF parameters normalized rapidly within the first 24 h after the procedure and showed no group differences. {VWF:CB [1.7 (0.6-3.7) vs 1.7 (0.6-4.2) U/ml, P = n.s.], VWF:CB/Ag [1.1 (0.5-2.9) vs 1.2 (0.7-2.2), P = n.s} Time in the intensive care unit, respirator time, duration of stay and bleeding before and after intervention were not significantly different. CONCLUSIONS: AVWS is detected in half of the children with high intra- or extracardiac stenoses and resolves completely after surgical or interventional repair. Even when undergoing surgery on cardiopulmonary bypass, excessive surgical site bleeding was not detected in our study patients.

Aortic flow patterns in patients with Marfan syndrome assessed by flow-sensitive four-dimensional MRI.

PURPOSE: To apply time-resolved three-dimensional (3D) phase contrast MRI with three-directional velocity encoding (flow-sensitive 4D MRI) for the characterization of flow pattern changes in patients with Marfan syndrome (MFS) compared with normal controls. MATERIALS AND METHODS: Flow-sensitive 4D MRI of the thoracic aorta (temporal resolution ∼45 ms, spatial resolution ∼2.4 × 2.1 × 2.8 mm(3) ) was performed in 24 MFS patients and 10 volunteers. Aortic flow patterns were visualized by 3D particle traces and streamlines. Global (affecting the complete lumen) and local (parts of the vessel lumen) helix and vortex flow in the ascending aorta (AAo), aortic arch, and descending aorta (DAo) were graded in 3 categories (blinded reading, two observers): none = 0, moderate = 1, pronounced = 2. RESULTS: Flow grading revealed similar global helix and vortex flow in the AAo and arch for MFS patients and controls. Local helix flow in the AAo was significantly (P = 0.011) increased in patients and was associated with aortic sinus dilatation. The incidence of global helix and vortex flow in the DAo was increased in patients (77% and 50% of subjects) compared with controls (none and 10%). CONCLUSION: The 4D flow analysis revealed marked differences of the aortic flow patterns between Marfan patients and controls: Local helix flow in the patients' AAo may be associated with the increased incidence of aortic root dilatation. The flow alterations in the proximal DAo could explain the occurrence of Type-B dissection originating from this site.

High colloid oncotic pressure priming of cardiopulmonary bypass in neonates and infants: implications on haemofiltration, weight gain and renal function.

OBJECTIVE: To evaluate the influence of high colloid oncotic pressure (COP) priming of cardiopulmonary bypass (CPB) on fluid balances, haemofiltration, capillary leakage and renal function in neonates and infants. METHODS: Twenty neonates or infants underwent heart surgery using CPB and were randomised in two groups. For group 1 (FFP-group) a blood priming with fresh frozen plasma (FFP, low oncotic pressure) was chosen, for group 2 (HA-group) a blood priming containing FFP and human albumin 20% (HA) to realise higher oncotic pressures was substituted. All patients were monitored before, during and 6h after CPB. We measured weights, fluid balances, transfusion volumes, colloid oncotic pressures, inflammatory parameters (c-reactive protein, interleukin-6, interleukin-8, thrombocytes, leucocytes) and renal function (creatinine clearances, renal protein losses). RESULTS: Patient's demographics and operational procedures were comparable in both groups with no further differences in operation procedures regarding palliation or correction. Colloid oncotic pressures of the priming solutions were higher in the HA-group (28 mmHg+/-4.9) than in the FFP-group (6 mmHg+/-1.3, p<0.001). Relative weight gain as a marker of capillary leakage in the HA-group (2%+/-4.5) was significantly lower 6h post CPB than in the FFP-group (8%+/-8.0, p=0.015). Haemofiltration rates were higher in the HA-group (569 ml+/-197 vs 282 ml+/-157, p=0.002) on CPB. There were no differences of creatinine clearances 6h after the end of CPB. Renal protein losses were elevated in both groups without any inter-group differences during and 6h after CPB. CONCLUSION: Addition of concentrated human albumin to priming fluids in paediatric cardiac surgery leads to less weight gain even after CPB. Supplementing paediatric patients undergoing cardiac surgery with concentrated human albumin does not affect renal function more severely than in paediatric patients undergoing cardiac surgery on CPB with blood priming.

Penetration of left and right atrial wall and aortic root by an Amplatzer atrial septal occluder in a nine year old boy with Marfan syndrome: Case report.

BACKGROUND: To describe complications associated with Amplatzer septal occluders in a patient with Marfan syndrome CASE PRESENTATION: A nine-year-old boy with Marfan syndrome and a 22 mm atrial septal defect (ASD) was treated successfully by interventional closure of his ASD by placing a 24 mm Amplatzer septal occluder. Follow up examinations showed a good result but an increasing enlargement of aortic root, so the patient was scheduled for operation. Intraoperative findings showed a perforation of the left atrial roof and the non-coronary sinus by penetration of the occluder device as well as penetration into the right atrial wall. The occluder was resected, the ASD was closed and the aortic sinus was reconstructed using a Dacron patch. CONCLUSION: We describe the first case of a patient with Marfan syndrome and an interventional closure of an ASD. Due to alterations of the connective tissue, as it is typical for patients with Marfan syndrome, the Amplatzer occluder probably perforated adjacent structures more easily as in non-affected individuals. Amplatzer occluders should be used with caution and follow up examinations should be performed in short intervals.

Successful interventional closure of a patent foramen ovale in a pediatric patient supported with a biventricular assist device.

We report on a 16-year-old boy after an event of cardiac arrest and initial treatment with a veno-arterial extracorporeal membrane oxygenator (ECMO). After a short stabilisation period a biventricular assist device (BVAD, Thoratec) was implanted. Although the BVAD was functioning well, the patient showed persisting hypoxemia. Transthoracic echocardiography revealed a patent foramen ovale with a high right-to-left shunt due to low aspiration pressures of the BVAD. The patient was successfully treated by interventional closure of the PFO with a 27-mm Amplatzer septal occluder and could easily be weaned from the respirator. Meanwhile the boy has successfully undergone heart transplantation. PFO has to be considered as a cause of arterial hypoxemia in patients supported with ventricular assist devices. The diagnosis of a PFO may be missed under ECMO-treatment. Interventional closure of a PFO can successfully be performed even if the patient is supported with a BVAD.