Abstract The updated American Heart Association/American Stroke Association guidelines include recommendation for thrombectomy in certain adult stroke cases. The safety and efficacy of thrombectomy ...in children are unknown. An 8-year-old girl experienced acute stroke symptoms on two occasions while therapeutically anticoagulated on Novalung. Computed tomography scans showed proximal vessel thrombi, which were retrieved using a Trevo device without hemorrhagic complications. Postprocedural assessment found respective decreases in the National Institutes of Health Stroke Scale score from 10 to 4 and 12 to 7. The indications for treatment and early benefits observed in our case are consistent with other pediatric thrombectomy cases reported. However, publication bias and the heterogeneity of reported cases prevent drawing conclusions about the safety and efficacy of thrombectomy in children. Anticipating that updates to adult stroke guidelines would likely incite stroke providers to consider thrombectomy in children, our institution developed guidelines for thrombectomy before the index patient. Establishing institutional guidelines before considering thrombectomy in children may optimize patient safety.
Abstract Background Cardiac disease is a leading cause of stroke in children, yet limited data support the current stroke prevention and treatment recommendations. A multidisciplinary panel of ...clinicians was convened in February 2014 by the International Pediatric Stroke Study group to identify knowledge gaps and prioritize clinical research efforts for children with cardiac disease and stroke. Results Significant knowledge gaps exist, including a lack of data on stroke incidence, predictors, primary and secondary stroke prevention, hyperacute treatment, and outcome in children with cardiac disease. Commonly used diagnostic techniques including brain computed tomography and ultrasound have low rates of stroke detection, and diagnosis is frequently delayed. The challenges of research studies in this population include epidemiologic barriers to research such as small patient numbers, heterogeneity of cardiac disease, and coexistence of multiple risk factors. Based on stroke burden and study feasibility, studies involving mechanical circulatory support, single ventricle patients, early stroke detection strategies, and understanding secondary stroke risk factors and prevention are the highest research priorities over the next 5-10 years. The development of large-scale multicenter and multispecialty collaborative research is a critical next step. The designation of centers of expertise will assist in clinical care and research. Conclusions There is an urgent need for additional research to improve the quality of evidence in guideline recommendations for cardiogenic stroke in children. Although significant barriers to clinical research exist, multicenter and multispecialty collaboration is an important step toward advancing clinical care and research for children with cardiac disease and stroke.
Progression of pulmonary hypertension is associated with increased serine elastase activity and the proteinase-dependent deposition of the extracellular matrix smooth muscle cell survival factor ...tenascin-C (refs. 1,2). Tenascin-C amplifies the response of smooth muscle cells to growth factors, which are also liberated through matrix proteolysis. Recent organ culture studies using hypertrophied rat pulmonary arteries have shown that elastase inhibitors suppress tenascin-C and induce smooth muscle cell apoptosis. This initiates complete regression of the hypertrophied vessel wall by a coordinated loss of cellularity and extracellular matrix. We now report that elastase inhibitors can reverse advanced pulmonary vascular disease produced in rats by injecting monocrotaline, an endothelial toxin. We began oral administration of the peptidyl trifluoromethylketone serine elastase inhibitors M249314 or ZD0892 21 days after injection of monocrotaline. A 1-week treatment resulted in 92% survival, compared with 39% survival in untreated or vehicle-treated rats. Pulmonary artery pressure and muscularization were reduced by myocyte apoptosis and loss of extracellular matrix, specifically elastin and tenascin-C. After 2 weeks, pulmonary artery pressure and structure normalized, and survival was 86%, compared with 0% in untreated or vehicle-treated rats. Although concomitant treatment with various agents can reduce pulmonary hypertension, we have documented complete regression after establishment of malignant monocrotaline-induced disease.
To “Cath” or Not in Pediatric Pulmonary Hypertension? Beghetti, Maurice, MD; Berger, Rolf M.F., MD, PhD; Ivy, Dunbar D., MD ...
Journal of the American College of Cardiology,
03/2016, Letnik:
67, Številka:
8
Journal Article
Recenzirano
Odprti dostop
Instead of optimizing an accurate estimate of serious complications of HC, identifying its risk factors and balancing clinical decision making, the current paper will cause a drift away from HC ...procedures, possibly withholding optimal care.
Abstract Aim The aim of this study was to investigate the association of observed/expected (O/E) lung-to-head ratio (LHR) with long-term morbidity for isolated fetal congenital diaphragmatic hernia ...(CDH) patients in a single institution. Methods We performed a retrospective study of prenatally diagnosed CDH from 18 to 38 weeks of gestation (01/2002–04/2010). Two cohorts of O/E LHR were defined (22.6–45%, 45.1–78.3%) based upon previous studies. Survivors with at least 1-year follow-up of prospectively collected long-term morbidity assessments were included. Results O/E LHR was available in 43 survivors (median 40%, range 22.8–78.3%). Follow-up data were available in 41 survivors (M:F = 24:17, left CDH = 39/41). Median follow-up was 6.5 years (1–11 years). Height/weight trajectories were similar between the two cohorts, with the majority below the 50th centile. There were no differences between the two cohorts by age 3 years for Bayley scales (developmental domains) and/or REEL-3 (language development). In addition, V/Q scans in the two cohorts demonstrated similar degrees of mismatch (mean delta V/Q = 35.4 versus 31.3). Conclusions In fetuses with isolated CDH, a reduction in O/E LHR does not predict a worse outcome in long-term follow-up. There is no association between a lower O/E LHR and a reduction in REEL-3 or Bayley score nor V/Q mismatch.
Adverse Effects of Dopamine on Systemic Hemodynamic Status and Oxygen Transport in Neonates After the Norwood Procedure
Jia Li, Gencheng Zhang, Helen Holtby, Tilman Humpl, Christopher A. Caldarone, ...Glen S. Van Arsdell, Andrew N. Redington
Dopamine induces a significant increase in systemic oxygen consumption (Vo2) in neonates after the Norwood procedure. Terminating dopamine was not associated with significant changes in systemic blood flow and systemic oxygen delivery (Do2) but was associated with a significant decrease in Vo2, resulting in an improved balance of Vo2–Do2.
The purpose of this study was to evaluate the effects of dopamine on hemodynamic status and oxygen transport in neonates after the Norwood procedure.
Dopamine is widely used to augment cardiac performance and increase oxygen delivery (Do2) in patients after cardiopulmonary bypass (CPB). This might be at the expense of increased myocardial and systemic oxygen consumption (Vo2), thus offsetting the improved Do2. This balance is particularly fragile in critically ill neonates.
Systemic oxygen consumption was continuously measured with respiratory mass spectrometry in 13 sedated, paralyzed, and mechanically ventilated neonates for 72 h after the Norwood procedure. Arterial, superior vena caval, and pulmonary venous blood gases were measured to calculate pulmonary blood flow (Qp) and systemic blood flow (Qs), Do2, and oxygen extraction ratio (ERo2). Rate-pressure product was calculated. Dopamine at a dose of 5 μg/kg/min was routinely administered at cessation of CPB and terminated within the first 48 h. Hemodynamic and oxygen transport measures were obtained before and at 100 min after the termination of dopamine.
Terminating dopamine was not associated with significant changes in arterial pressure, Qp, Qs, or Do2but was associated with a significant decrease in heart rate (p = 0.003), rate-pressure product (p = 0.03), and Vo2(−20 ± 11%, p < 0.0001), resulting in a significant decrease in ERo2(p = 0.01).
Dopamine induces a significant increase in Vo2in neonates after the Norwood procedure, and termination is associated with an improved balance of Vo2–Do2. These data further emphasize the importance of understanding changes in Vo2as well as Do2in infants after cardiac surgery.
To enable adequate interpretation of growth measurements in the management of children with pulmonary arterial hypertension (PAH), we assessed growth and its associated determinants in children with ...PAH.
We did a retrospective longitudinal study of height and body-mass index in reference to WHO growth standards by pooling data from four contemporary prospective registries of paediatric PAH representing 53 centres in 19 countries. The main outcome measures were median height for age and body-mass index for age percentiles and longitudinal deviation of height for age and body-mass index for age Z scores from WHO standards.
601 children were followed up for a median of 2·9 years (IQR 1·5-4·4). Baseline median height for age percentile was 26 (IQR 4-54) and baseline median body-mass index for age percentile was 41 (IQR 12-79). Mean height for age Z score was significantly lower than the reference (-0·81, 95% CI -0·93 to -0·69; p<0·0001), as was body-mass index for age Z score (-0·12, -0·25 to -0·01; p=0·047). Height for age Z score was particularly decreased in young patients (aged ≤5 years) with idiopathic or hereditary PAH and in all patients with PAH associated with congenital heart disease. Although Z scores increased in some patients and decreased in others, we detected no significant trend in height for age Z score (p=0·57) or body-mass index for age Z score (p=0·48) before taking account of covariates. Multivariable linear mixed effects modelling showed that age, cause of PAH, ex-prematurity, WHO functional class, trisomy 21, and time since diagnosis were associated with height for age Z score, whereas age, ethnicity, and trisomy 21 were associated with body-mass index for age Z score. A favourable WHO functional class course was independently associated with increases in height for age Z score.
PAH is associated with impaired growth, especially in younger children and those with pulmonary arterial hypertension associated with congenital heart disease. The degree of impairment is independently associated with cause of PAH and comorbidities, but also with disease severity and duration. Because a favourable clinical course was associated with catch-up growth, height for age could serve as an additional and globally available clinical parameter to monitor patients' clinical condition.
Actelion Pharmaceuticals.
Background: Maximal cardiopulmonary exercise testing (CPX) is valuable to quantifying functional capacity in patients with pulmonary
hypertension (PH), but information on CPX in children is limited ...possibly because of safety concerns. The purpose of this
study was to examine the safety of CPX in pediatric patients with PH.
Methods: Data were obtained retrospectively from patients referred for CPX at our institution between January 2001 and September 2007.
Patients with a 6-min walk distance < 275 m were excluded. Exercise test complications were grouped according to ischemic
ECG changes, presence of arrhythmia, and oxygen desaturation at peak exercise and were graded as mild, moderate, or severe.
Results: Twenty-seven patients (4 with idiopathic PH, 23 with secondary PH), 12.5 years of age (range, 6.9 to 18 years), participated
in 64 CPX sessions. The mean (± SD) peak oxygen uptake was 23.3 ± 5.4 mL/kg/min, with a mean decrease in arterial oxygen saturation
to 85% ± 15.7% at peak exercise. Mild arrhythmia was detected in 30% of the patients. ST-segment depression was graded as
mild (19%) or moderate (1.5%). There were no significant adverse events, such as syncope, chest pain, or dizziness. CPX was
stopped for fatigue in 53% of patients, leg fatigue in 23%, dyspnea in 21%, and miscellaneous reasons in 3%.
Conclusions: This study suggests that CPX can be performed safely in pediatric patients with PH, with the exception of patients with severe
limitation who were excluded from exercise testing. Although the number of patients in the sample is small, the data imply
that the absence of significant patient symptoms and low incidence of arrhythmia or significant ST-segment depression make
CPX a safe choice for measuring functional capacity in this patient population.
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