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.
Objectives The lack of accurate measurement of hemodynamics and oxygen transport has limited our understanding of Norwood physiology and postoperative management. We used measured oxygen consumption ...to characterize hemodynamics and oxygen transport after the classic Norwood procedure. Methods Fourteen neonates had continuous respiratory mass spectrometry to measure oxygen consumption (VO2 ). Arterial, superior vena caval, and pulmonary venous saturations were measured at 2- to 4-hour intervals for 72 hours postoperatively. Systemic (Qs) and pulmonary (Qp) blood flows, systemic vascular resistance (SVR) and pulmonary vascular resistance inclusive of the Blalock–Taussig shunt (BT-PVR), systemic oxygen delivery (DO2 ), and the oxygen extraction ratio (ERO2 ) were calculated. Results Qs and DO2 were low during the first 12 hours (1.8 ± 0.6 L · min−1 · m−2 and 281 ± 86 mL · min−1 · m−2 at the 12th hour, respectively) and increased over the study period ( P < .05 for both). VO2 decreased markedly during the first 24 hours (101 ± 26 to 86 ± 16 mL · min−1 · m−2 , P < .0001). Consequently, ERO2 decreased significantly over the study, most rapidly during the first 24 hours (0.44 ± 0.11 to 0.28 ± 0.09, P < .0001). There was a close correlation of DO2 to SVR and to Qs ( P < .0001 for both). There was no correlation of DO2 to BT-PVR ( P = .14) or to Qp ( P = .67). DO2 was closely correlated with hemoglobin value ( P < .0001), weakly correlated with Pa o2 ( P = .0002), and not correlated with arterial oxygen saturation ( P = .32). Conclusions There is wide variability of hemodynamics and oxygen transport after the Norwood procedure. The decrease in VO2 during the first 24 hours is the main contributor to improving the balance of oxygen transport. DO2 is most closely correlated to SVR and hemoglobin and weakly correlated to Pa o2 . It is not correlated to Qp. Postoperative management strategies to decrease VO2 and maintain a high hemoglobin level and a low SVR appear to be rational.
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 Background In pulmonary arterial hypertension (PAH), acute vasodilator response testing (AVT) is considered important to identify adult patients with favorable prognosis using ...calcium-channel blocker (CCB) therapy. However, in pediatric PAH, criteria used to identify acute responders and CCB use are insufficiently studied. Objectives This study sought to describe current clinical practice of AVT and subsequent treatment decisions in pediatric PAH. Methods From January 2008 to May 2013, 529 consecutive children with confirmed pulmonary hypertension were enrolled in an international registry. We analyzed those children with evaluable AVT. Results Of 382 children with evaluable AVT, 212 had idiopathic/familial PAH (IPAH/FPAH) and 105 had PAH associated with congenital heart disease (PAH-CHD). In 70% of the patients, AVT was performed using inhaled nitric oxide; other agents were used in the remaining patients. In IPAH/FPAH patients, 78 (37%) patients were acute responders according to their physician, 62 (30%) according to REVEAL (Registry-to-Evaluate-Early-And-Long-term PAH disease management)-pediatric criteria, and 32 (15%) according to Sitbon criteria. For PAH-CHD patients, the numbers of AVT responders were 38 (36%), 14 (13%), and 7 (7%) respectively. Correlation between AVT responder status as judged by the treating physician and by published response criteria was poor. Moreover, of the IPAH/FPAH patients judged by the treating physician as acute responders, only 23% were treated with CCB without additional PAH-targeted therapy. The Sitbon criteria selected patients with better prognosis who had excellent outcome when treated with CCB. Conclusions The current practice of identifying responders to AVT and subsequent treatment with CCB therapy demonstrated large discrepancies with current international guidelines. Also, in pediatric IPAH, the Sitbon criteria are the criteria of choice to identify patients with excellent survival when treated with CCB therapy.
Objectives The purpose of our study was to characterize the hemodynamic and corresponding pharmacokinetic responses to a single dose of oral sildenafil by children with pulmonary arterial ...hypertension (PAH) undergoing invasive testing. Background Although used frequently for the treatment of children with PAH, data regarding the acute responses to sildenafil are limited. Methods Thirty-six patients (mean age 7.5 ± 5.9 years; 24 females) were studied during cardiac catheterization with general anesthesia. Eight of 36 (22%) had idiopathic PAH; the remainder had associated congenital heart disease. Hemodynamics and serum cyclic-guanosine monophosphate levels (cGMP) were evaluated at baseline and after inhaled nitric oxide (NO) (40 ppm). In addition, cGMP and sildenafil levels were measured 30 min after administration of sildenafil (0.5 mg/kg, suspended in 5 ml sterile water) through a nasogastric tube. Results For the 36 patients, the pulmonary vasodilating capability of oral sildenafil was lower than that of inhaled NO (2.8% vs. 11.6% reduction in pulmonary vascular resistance indexed to body surface area PVRI, respectively; p = 0.01). However, only 21 of 36 (58%) patients had a detectable sildenafil level. In those with detectable sildenafil levels, the fall in PVRI was greater (−11.6% vs. −19.1%, p = NS). Mean cGMP levels at baseline and after NO were 41.8 ± 20.0 pmol/ml and 83.8 ± 35.5 pmol/ml, respectively (p < 0.0001). Surprisingly, there was no significant increase in cGMP in patients with either undetectable (37.5 ± 29.8 pmol/ml) or detectable (44.4 ± 31.7 pmol/ml) sildenafil levels (p = NS compared with baseline) with sildenafil. Conclusions Our study demonstrates suboptimal absorption of sildenafil in almost half the children undergoing acute hemodynamic testing. When detectable, there was no statistically significant difference between the fall in PVRI associated with sildenafil and NO despite lower circulating cGMP levels in the sildenafil group. These data should be taken into account when designing acute testing protocols, and assessing the acute response to sildenafil in patients with PAH.
Background Pediatric pulmonary arterial hypertension (PAH), whether idiopathic PAH (iPAH) or PAH associated with congenital heart disease (aPAH), carries high morbidity and mortality. Low pulmonary ...arterial capacitance (PAC), defined as right ventricular stroke volume/pulmonary artery pulse pressure, is a risk factor for mortality in adults with PAH. However, the relation of PAC to pulmonary vascular resistance (PVR), exercise endurance, and survival is poorly defined in children. Methods Catheterization and clinical data of children with PAH (mean pulmonary artery pressure >25 mm Hg) were reviewed. Children with pulmonary shunts, stents, collaterals, or pulmonary venous hypertension were excluded. Primary outcomes were 6-minute walk distance and freedom from death/lung transplant. Results Forty-seven patients were studied. Nineteen (43%) had iPAH, and 28 (57%) had aPAH (7.1 ± 6.2 vs 8.4 ± 5.5 years, P = .45). Patients with iPAH had higher PVR indexed for body surface area (PVRi), lower indexed PAC (PACi), lower exercise tolerance, and lower freedom from death/lung transplant than patients with aPAH. Both higher PVRi ( P < .0001) and lower PACi ( P = .02) were associated with shorter 6-minute walk distance. A PACi <0.70 mL/mm Hg per square meter or >1.25 mL/mm Hg per square meter and a PVRi >13 Wood units × m2 were associated with decreased freedom from death or lung transplant. The relationships between PVRi and PACi and survival were independent of each other and not confounded by etiologic group. Conclusions Low PACi and high PVRi are independently associated with low 6-minute walk distance and survival in children with PAH. Therefore, both should be assessed for better prognostication and management in this high-risk population.
Introduction Cor triatriatum sinistrum (CTS) is a rare congenital cardiac defect that can present with a wide range of symptoms and may be associated with other structural cardiac defects. Very ...limited data are available for pediatric patients. Objective To analyze the experience with CTS at a single pediatric centre, highlighting symptoms on presentation, mode of diagnosis and outcome. Methods Hospital databases were searched to identify patients with CTS who were diagnosed between 1954 and 2005. Medical records with demographic data, clinical evaluation, diagnostic approach, interventions and autopsy results were reviewed. Results Between 1954 and 2005, 82 patients (43 female children, 52%) with CTS were diagnosed at the institution. Patients were born between 1951 and 2004, and the median age at presentation was eight months (range one day to 16.1 years). The majority of patients (77%) presented with associated cardiac lesions. Of 82 patients, 57 (70%) underwent resection of the fibromuscular diaphragm, 14 (17%) did not require surgery and 11 (13%) did not survive the time to intervention. Nineteen patients (23%) died a median of two months (range one day to 5.5 years) after presentation. Nine of these patients (11%) died before surgery was attempted. Most patients were completely asymptomatic at the time of the last follow-up at a median of three years (range one day to 18 years). Echocardiography significantly facilitated the diagnosis and subsequent management of patients with CTS. Conclusions Despite the diversity in presentation and complexity of associated lesions, the outcome for patients with CTS is favourable.
Objectives Ischemic brain injury is an important morbidity in neonates after the Norwood procedure. Its relationship to systemic hemodynamic oxygen transport is poorly understood. Methods Sixteen ...neonates undergoing the Norwood procedure were studied. Continuous cerebral oxygen saturation was measured by near-infrared spectroscopy. Continuous oxygen consumption was measured by respiratory mass spectrometry. Pulmonary and systemic blood flow, systemic vascular resistance, oxygen delivery, and oxygen extraction ratio were derived with measurements of arterial, and superior vena cava and pulmonary venous gases and pressures at 2- to 4-hour intervals during the first 72 hours in the intensive care unit. Results Mean cerebral oxygen saturation was 66% ± 12% before the operation, reduced to 51% ± 13% on arrival in the intensive care unit, and remained low during the first 8 hours; it increased to 56% ± 9% at 72 hours, still significantly lower than the preoperative level ( P < .05). Postoperatively, cerebral oxygen saturation was closely and positively correlated with systemic arterial pressure, arterial oxygen saturation, and arterial oxygen tension and negatively with oxygen extraction ratio ( P < .0001 for all). Cerebral oxygen saturation was moderately and positively correlated with systemic blood flow and oxygen delivery ( P < .0001 for both). It was weakly and positively correlated with pulmonary blood flow ( P = .001) and hemoglobin ( P = .02) and negatively correlated with systemic vascular resistance ( P = .003). It was not correlated with oxygen consumption ( P > .05). Conclusions Cerebral oxygen saturation decreased significantly in neonates during the early postoperative period after the Norwood procedure and was significantly influenced by systemic hemodynamic and metabolic events. As such, hemodynamic interventions to modify systemic oxygen transport may provide further opportunities to reduce the risk of cerebral ischemia and improve neurodevelopmental outcomes.
Right ventricular (RV) function is an important determinant of mortality in patients with idiopathic pulmonary arterial hypertension (iPAH). The aim of this study was to serially evaluate global and ...regional RV two-dimensional strain and their relation to transplantation-free survival in children with iPAH.
RV regional and global longitudinal strain was retrospectively assessed in children with iPAH. Serial echocardiograms at 3 to 6 months from presentation and then at yearly intervals were analyzed. Results were compared with those from controls and between iPAH survivors (group 1) and those who died or needed transplantation (group 2). Survival stratified by RV global longitudinal strain at presentation was analyzed.
Seventeen patients with iPAH (mean age, 8.4 ± 4.8 years; seven male patients), of whom 11 were alive (group 1) and six had died or undergone transplantation (group 2), and 17 age-matched controls were studied. The median follow-up period was 1.5 years (range, 0.04-7.8 years). RV global longitudinal strain was significantly reduced in patients with iPAH compared with controls (-13.5 ± 5.9% vs -24.4 ± 3.9%, P < .001) and in group 2 compared with group 1 at presentation (-9 ± 2.8% vs -16 ± 5.7%, P < .05) and throughout follow-up. During follow-up, RV global and regional longitudinal strain worsened in group 2, especially in RV apical segments (-6.3 ± 5% vs -1.9 ± 1.6% at presentation compared with the last echocardiographic assessment in group 2, P < .05), but was unchanged in group 1. RV global longitudinal strain > -14% predicted transplantation-free survival with 100% sensitivity and 54.5% specificity.
RV strain imaging may be useful for serial follow-up and prognostication in children with iPAH.
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