Normal values of the sagittal diameter of the lumbar spine (vertebral body and dural sac) in children measured by MRI

Background: The definition of normal values is a prerequisite for the reliable evaluation of abnormality in the lumbar spine, such as spinal canal stenosis or dural ectasia in patients with Marfan syndrome. Values for vertebral body diameter (VBD) and dural sac diameter (DSD) for the lumbar spine have been published in adults. In children, normal values have been established using conventional radiography or myelography, but not by MRI. Objective: To define normal values for the sagittal diameter of the vertebral body and dural sac, and to calculate a dural sac ratio (DSR) in the lumbosacral spine (L1-S1) in healthy children using MRI. Materials and methods: A total of 75 healthy children between 6years and 17years of age were examined using a sagittal T2-weighted sequence. Sagittal VBD and DSD were measured and a DSR was calculated. This was a retrospective and cross-sectional study. Results: With increasing age there is a significant increase of VBD, a slight increase of DSD, and a slight decrease of DSR. There is no significant sex difference. DSR in healthy children is higher than in healthy adults. Conclusions: MRI is a reliable method demonstrating the natural shape of the lumbosacral spine and its absolute values. These normal values compare well with those established by conventional radiological techniques. Our data may serve as a reference for defining dural ectasia in children with Marfan syndrom

Assessment of Myocardial Function in Pediatric Patients with Operated Tetralogy of Fallot: Preliminary Results with 2D Strain Echocardiography

The global myocardial function in patients after repair of tetralogy of Fallot (TOF) can be assessed by cardiovascular magnetic resonance (CMR) and measurement of B-type natriuretic peptides. Two-dimensional echocardiography-derived strain and strain rate (2D strain) facilitate the assessment of regional myocardial function. We evaluated myocardial function in 16 children with residual severe pulmonary valve regurgitation and right ventricular (RV) volume overload after TOF repair before, 1month after, and 6months after pulmonary valve replacement (PVR). In 2D strain echocardiography preoperatively, the longitudinal systolic RV strain was reduced (p<0.05). One month after PVR, longitudinal systolic RV strain decreased further (p<0.05), while systolic and early diastolic radial left ventricular strain and strain rate increased (each p<0.05), followed by a return toward preoperative values after 6months. Six months after PVR, preoperatively elevated RV end-diastolic volume (p<0.01) assessed by CMR and N-terminal pro-B-type natriuretic peptide (p<0.05) decreased. In conclusion, the impairment of the regional myocardial after TOF repair and transient changes after PVR can be subtly analyzed by 2D strain echocardiography in addition to the established assessment of myocardial function with CMR and measurement of B-type natriuretic peptide

High proportion of genetic cases in patients with advanced cardiomyopathy including a novel homozygous Plakophilin 2-gene mutation

Cardiomyopathies might lead to end-stage heart disease with the requirement of drastic treatments like bridging up to transplant or heart transplantation. A not precisely known proportion of these diseases are genetically determined. We genotyped 43 index-patients (30 DCM, 10 ARVC, 3 RCM) with advanced or end stage cardiomyopathy using a gene panel which covered 46 known cardiomyopathy disease genes. Fifty-three variants with possible impact on disease in 33 patients were identified. Of these 27 (51%) were classified as likely pathogenic or pathogenic in the MYH7, MYL2, MYL3, NEXN, TNNC1, TNNI3, DES, LMNA, PKP2, PLN, RBM20, TTN, and CRYAB genes. Fifty-six percent (n = 24) of index-patients carried a likely pathogenic or pathogenic mutation. Of these 75% (n = 18) were familial and 25% (n = 6) sporadic cases. However, severe cardiomyopathy seemed to be not characterized by a specific mutation profile. Remarkably, we identified a novel homozygous PKP2-missense variant in a large consanguineous family with sudden death in early childhood and several members with heart transplantation in adolescent age

Extracorporeal life support in pediatric cardiac dysfunction

<p>Abstract</p> <p>Background</p> <p>Low cardiac output (LCO) after corrective surgery remains a serious complication in pediatric congenital heart diseases (CHD). In the case of refractory LCO, extra corporeal life support (ECLS) extra corporeal membrane oxygenation (ECMO) or ventricle assist devices (VAD) is the final therapeutic option. In the present study we have reviewed the outcomes of pediatric patients after corrective surgery necessitating ECLS and compared outcomes with pediatric patients necessitating ECLS because of dilatated cardiomyopathy (DCM).</p> <p>Methods</p> <p>A retrospective single-centre cohort study was evaluated in pediatric patients, between 1991 and 2008, that required ECLS. A total of 48 patients received ECLS, of which 23 were male and 25 female. The indications for ECLS included CHD in 32 patients and DCM in 16 patients.</p> <p>Results</p> <p>The mean age was 1.2 ± 3.9 years for CHD patients and 10.4 ± 5.8 years for DCM patients. Twenty-six patients received ECMO and 22 patients received VAD. A total of 15 patients out of 48 survived, 8 were discharged after myocardial recovery and 7 were discharged after successful heart transplantation. The overall mortality in patients with extracorporeal life support was 68%.</p> <p>Conclusion</p> <p>Although the use of ECLS shows a significantly high mortality rate it remains the ultimate chance for children. For better results, ECLS should be initiated in the operating room or shortly thereafter. Bridge to heart transplantation should be considered if there is no improvement in cardiac function to avoid irreversible multiorgan failure (MFO).</p

Substantial radiation reduction in pediatric and adult congenital heart disease interventions with a novel X-ray imaging technology

Pediatric catheterization exposes patients to varying radiation doses. Concerns over the effects of X-ray radiation dose on the patient population have increased in recent years. This study aims at quantifying the patient radiation dose reduction after the introduction of an X-ray imaging technology using advanced real time image noise reduction algorithms and optimized acquisition chain for fluoroscopy and exposure in a pediatric and adult population with congenital heart disease. Patient and radiation dose data was retrospectively collected (July 2012–February 2013) for 338 consecutive patients treated with a system using state of the art image processing and reference acquisition chain (referred as “reference system”). The same data was collected (March–October 2013) for 329 consecutive patients treated with the new imaging technology (Philips AlluraClarity, referred as “new system”). Patients were divided into three weight groups: A) below 10 kg, B) 10–40 kg, and C) over 40 kg. Radiation dose was quantified using dose area product (DAP), while procedure complexity using fluoroscopy time, procedure duration and volume of contrast medium. The new system provides significant patient dose reduction compared to the reference system. Median DAP values were reduced in group A) from 140.6 cGy·cm2 to 60.7 cGy·cm2, in group B) from 700.0 cGy·cm2 to 202.2 cGy·cm2 and in group C) from 4490.4 cGy·cm2 to 1979.8 cGy·cm2 with reduction of 57%, 71% and 56% respectively (p < 0.0001 for all groups). Despite no other changes in procedural approach, the novel X-ray imaging technology provided substantial radiation dose reduction of 56% or higher

Normal values of the sagittal diameter of the lumbar spine (vertebral body and dural sac) in children measured by MRI

Background: The definition of normal values is a prerequisite for the reliable evaluation of abnormality in the lumbar spine, such as spinal canal stenosis or dural ectasia in patients with Marfan syndrome. Values for vertebral body diameter (VBD) and dural sac diameter (DSD) for the lumbar spine have been published in adults. In children, normal values have been established using conventional radiography or myelography, but not by MRI. Objective: To define normal values for the sagittal diameter of the vertebral body and dural sac, and to calculate a dural sac ratio (DSR) in the lumbosacral spine (L1-S1) in healthy children using MRI. Materials and methods: A total of 75 healthy children between 6years and 17years of age were examined using a sagittal T2-weighted sequence. Sagittal VBD and DSD were measured and a DSR was calculated. This was a retrospective and cross-sectional study. Results: With increasing age there is a significant increase of VBD, a slight increase of DSD, and a slight decrease of DSR. There is no significant sex difference. DSR in healthy children is higher than in healthy adults. Conclusions: MRI is a reliable method demonstrating the natural shape of the lumbosacral spine and its absolute values. These normal values compare well with those established by conventional radiological techniques. Our data may serve as a reference for defining dural ectasia in children with Marfan syndrom