Abstract Aim: We investigated the anticoagulant effects of recombinant human activated protein C (rhAPC), unfractionated heparin (UH) and melagatran (a new direct thrombin inhibitor [DTI]), when administered individually and in combinations of rhAPC with either UH or melagatran, in umbilical cord and adult plasma. rhAPC is a promising candidate treatment to improve the outcome of severe sepsis in neonates and adults; the DTI melagatran represents a potential advance in antithrombotic therapy. Methods: The anticoagulant efficacy of these drugs was measured using the standard coagulation assays activated partial thromboplastin time (aPTT) and prothrombin time (PT). Results: Administered individually, rhAPC, UH and melagatran dose‐dependently prolonged aPTT to a significantly greater extent in umbilical cord than in adult plasma. Melagatran alone, but not rhAPC or UH alone, dose‐dependently prolonged the PT in both umbilical cord and adult plasma. Combining rhAPC with either UH or melagatran significantly augmented aPTT prolongation in both umbilical cord and adult plasma. Conclusion: Our results, which facilitate estimation of rhAPC and melagatran dose requirements in umbilical cord plasma, may be of benefit in critically sick newborns with severe sepsis.
A newborn with valvular aortic stenosis and a 5 mm atrial septal defect (ASD) underwent emergency aortic valvotomy. The small left ventricle of the newborn with signs of fibroelastosis showed good function but a reduced compliance and caused a large left to right shunt at atrial level.As the patient became respirator-dependant the ASD was interventionally closed with an 18 mm Amplatzer PFO occluder. The patient could be weaned from the ventilator within two days and had an uneventful recovery.Interventional closure of an ASD is possible even in the newborn period and should be considered as an alternative to surgery.
ransthoracic echocardiography (TTE) in children usually allows a comprehensive assessment of the cardiovascular anatomy.6][7] Although VVI is well recognized in conditions such as pressure overload or arrythmia, they have not been well studied in children with PH, including those with PH associated with congenital heart disease (PH-CHD).To date, VVI variables such as the LV endsystolic eccentricity index (LVes-EI) is not commonly assessed in patients with pediatric PH in clinical practice.The physiology of VVI is based on the pericardium, movement of the interventricular septum, and myocardial tracts running between the ventricles. 7The impact of the RV under pressure is related to underfilling and dysfunction of the compressed LV, which reduces LV output. 8As RV pressure load progresses, the enhanced VVI leads to impaired LV filling.In RV systole, the LV cavity is compressed owing to leftward bowing of the ventricular septum, causing a reduction in LV preload, and low LV output. 9,10The RV/LV end-systolic dimension ratio, taken in the parasternal shortaxis view, was derived to combine a measure of RV size with septal shift secondary to elevated RV pressure. 3The VVI variables LVesEI and RV/LV end-systolic dimension ratio are associated with lower functional class, worse hemodynamics, and higher NT-proBNP levels, 11 thus highlighting the importance of VVI in pediatric PH.In a recent study, we found the LVesEI to positively correlate with an RV end-systolic remodeling index, indicating that RV deformation acts in combination with RV/LV interaction. 12n this issue of Circulation: Cardiovascular Imaging, Burkett et al 13 evaluated LV shape measures in children and young adults with PH.TTE was performed near-simultaneously with cardiac catheterization, allowing-for the first time-a direct comparison between invasive hemodynamics and measures of LV shape.The authors present a new TTE measure, entitled the maximal eccentricity index (EIM).This measure is essentially the LVesEI and highlights the role of substantial, indeed post-systolic maximal septal flattening that is frequently found in PH. 13 The group investigated 78 patients with pediatric PH under general anesthesia in the cardiac cathterization laboratory, 70.5% of whom were group 1 PH, that is, PAH (38% idiopathic PAH, and 59% PH-CHD, and 78 well-matched control subjects with mean pulmonary arterial pressure (mPAP) <25 mm Hg.Most of the 78 patients with PH were on at least one PAH-targeted medication (92%), and all patients had systemic saturations >90%, 13 indicating either no significant or predominant left-to-right
Editor: We read with great interest the article “Magnetic Resonance Imaging of the Right Ventricle in Pediatric Pulmonary Arterial Hypertension” by Blalock et al.1 They present highly valuable cardiac magnetic resonance (CMR) data on children with idiopathic pulmonary arterial hypertension (IPAH) without a secondary cause of PAH, such as congenital heart disease (CHD). It has recently been shown that in patients with chronic pressure overload the resulting right ventricular (RV) myocardial damage can be demonstrated by an impaired RV long-axis function.2 Blalock and colleagues found an impaired RV function in children with IPAH. They describe an increased indexed RV end-diastolic volume (RVEDVi) and a reduced RV ejection fraction in comparison to healthy controls. For the convenience of the audience of Pulmonary Circulation, we want to add that their data are in agreement with results obtained in patients with PAH secondary to CHD (PAH-CHD).3,4 However, we find a higher RVEDVi in PAH-CHD patients, which might be related to their multiple surgical procedures. It has been shown that echocardiographic measures of systolic RV function, such as tricuspid annular peak systolic velocity and tricuspid annular plane systolic excursion, correlate with CMR data on patients with PAH-CHD.3,4 Considering that about half of PAH patients in childhood have underlying CHD,5 the CMR results of Blalock and colleagues and the respective echocardiographic results of Kassem et al.6 in patients with IPAH add up to our data in PAH-CHD patients and together provide a compact image of RV function in pediatric PAH patients. We want to thank the authors for addressing the need to investigate RV function parameters in children with IPAH. In the future, with increasing data in pediatric PAH patients, echocardiography, in addition to the gold standard method, CMR, may become the technique of choice for routine assessment of systolic RV function in the follow-up of patients with PAH.
End-stage renal disease (ESRD) in neonates still has a high mortality, particularly in the first year of life. We present the combination of peritoneal dialysis (PD) with intermittent hemodiafiltration (iHDF) in neonates with ESRD. Four infants younger than 28 days were treated with PD and iHDF. Renal diagnoses leading to ESRD were cortical necrosis, prune belly syndrome, neonatal hemolytic uremic syndrome, and autosomal recessive polycystic kidney disease. Initially, three patients were on iHDF until PD was started. At the time when complications occurred during PD, patients were switched back to iHDF. iHDF was used five times as a bridge to PD in case of abdominal surgery. Two of the four patients were switched to iHDF because of peritoneal ultrafiltration failure due to recurrent peritoneal leaks. Once, iHDF became necessary due to refractory peritonitis. All four patients survived the first year of life. Two patients were transplanted successfully at an age of 35 and 22 months, respectively. The others are on renal replacement therapy, one on PD at the age of 28 months and one on iHDF at the age of 25 months, respectively. In case of PD complications, iHDF may be an appropriate bridge to achieve long-term survival until kidney transplantation.
Objectives: Determination of the right ventricular outflow tract velocity time integral (RVOT VTI) is a part of the non-invasive investigation of pulmonary flow in adults with pulmonary hypertension (PH). The normal range of RVOT VTI in adults is stable, and differences serve as indicators for changes in RV stroke volume. In children, non-invasive techniques to assess pulmonary blood flow are of interest. Recently, we provided normative age-related pediatric RVOT VTI values in order to introduce RVOT VTI assessment to echocardiographic protocols for pediatric PH evaluation. The tricuspid regurgitation velocity (TRV)/RVOT VTI ratio, as a reliable measure of pulmonary blood flow in adults with PH, approximates the ratio of pulmonary artery pressure to pulmonary blood flow, with pediatric data to date missing. The aim of our study was to investigate RVOT VTI and TRV/RVOT VTI ratio in children with PH with respect to healthy controls and growth-related differences.
BACKGROUND: A 33-year-old adult with complex congenital heart disease and severe cyanosis presented to us with increasing symptoms of hyperviscosity, decreasing exercise tolerance and a thromboembolic complication. INVESTIGATION: Physical examination, laboratory investigations, ECG, transthoracic echocardiogram, cardiopulmonary exercise testing, angiography. DIAGNOSIS: Subvalvular and valvular pulmonary stenosis due to a calcified pulmonary valve. TREATMENT: Amiodarone, angiotensin-converting enzyme inhibitor, beta-blockers, percutaneous pulmonary balloon valvuloplasty, warfarin.
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