To describe presentation, hospital course, and predictors of bad outcome in multisystem inflammatory syndrome in children (MIS-C).
Retrospective data review of a case series of children meeting the ...published definition for MIS-C who were discharged or died between March 1, 2020, and June 15, 2020, from 33 participating European, Asian, and American hospitals. Data were collected through a Web-based survey and included clinical, laboratory, electrocardiographic, and echocardiographic findings and treatment management.
We included 183 patients with MIS-C: male sex, 109 (59.6%); mean age 7.0 ± 4.7 years; Black race, 56 (30.6%); obesity, 48 (26.2%). Overall, 114 of 183 (62.3%) had evidence of severe acute respiratory syndrome coronavirus 2 infection. All presented with fever, 117 of 183 (63.9%) with gastrointestinal symptoms, and 79 of 183 (43.2%) with shock, which was associated with Black race, higher inflammation, and imaging abnormalities. Twenty-seven patients (14.7%) fulfilled criteria for Kawasaki disease. These patients were younger and had no shock and fewer gastrointestinal, cardiorespiratory, and neurologic symptoms. The remaining 77 patients (49.3%) had mainly fever and inflammation. Inotropic support, mechanical ventilation, and extracorporeal membrane oxygenation were indicated in 72 (39.3%), 43 (23.5%), and 4 (2.2%) patients, respectively. A shorter duration of symptoms before admission was found to be associated with poor patient outcome and for extracorporeal membrane oxygenation and/or death, with 72.3% (95% confidence interval: 0.56-0.90;
= .006) increased risk per day reduction and 63.3% (95% confidence interval: 0.47-0.82;
< .0001) increased risk per day reduction respectively.
In this case series, children with MIS-C presented with a wide clinical spectrum, including Kawasaki disease-like, life-threatening shock and milder forms with mainly fever and inflammation. A shorter duration of symptoms before admission was associated with a worse outcome.
Persistent Patent ductus arteriosus (PDA) is a common finding in extremely low gestational age newborn infants and its prevalence is inversely proportional to the gestational age. The presence of a ...persistent PDA is associated with increased mortality and several significant morbidities including intraventricular hemorrhage, pulmonary hemorrhage, necrotizing enterocolitis, and chronic lung disease or bronchopulmonary dysplasia. However, treating PDA has not been demonstrated to have beneficial impact on the long term outcomes. Currently there is no consensus on whether to treat the PDA or not, and if treat, when to treat and how to treat. The echocardiography is the investigation of choice to diagnose PDA, estimating the magnitude of shunt volume and assessing its hemodynamic significance, and to exclude/diagnose any associated congenital heart defect before any intervention. Various echocardiographic parameters and staging/scoring systems have been described to help the clincians making the clinical decisions and some of theses scoring systems are quite complex to apply in a busy day to day clinical practice. This concised review paper is focused to help the clinicians in making a clinical decision based upon clincial and echocardiography parameters. Hence, only the parameters which are commonly used and helpful in making the clinical decisions in day to day clincial practice have been described in this paper.
Activin A receptor type II-like kinase-1 (ACVRL1, also known as ALK1) mutation is a cause of hereditary hemorrhagic telangiectasia (HHT) and/or heritable pulmonary arterial hypertension (PAH).
To ...describe the characteristics of patients with PAH carrying an ACVRL1 mutation.
We reviewed clinical, functional, and hemodynamic characteristics of 32 patients with PAH carrying an ACVRL1 mutation, corresponding to 9 patients from the French PAH Network and 23 from literature analysis. These cases were compared with 370 patients from the French PAH Network (93 with a bone morphogenetic protein receptor type 2 BMPR2 mutation and 277 considered as idiopathic cases without identified mutation). Distribution of mutations in the ACVRL1 gene in patients with PAH was compared with the HHT Mutation Database.
At diagnosis, ACVRL1 mutation carriers were significantly younger (21.8 +/- 16.7 yr) than BMPR2 mutation carriers and noncarriers (35.7 +/- 14.9 and 47.6 +/- 16.3 yr, respectively; P < 0.0001). In seven of the nine patients from the French PAH Network, PAH diagnosis preceded manifestations of HHT. ACVRL1 mutation carriers had better hemodynamic status at diagnosis, but none responded to acute vasodilator challenge and they had shorter survival when compared with other patients with PAH despite similar use of specific therapies. ACVRL1 mutations in exon 10 were more frequently observed in patients with PAH, as compared with what was observed in the HHT Mutation Database (33.3 vs. 5%; P < 0.0001).
ACVRL1 mutation carriers were characterized by a younger age at PAH diagnosis. Despite less severe initial hemodynamics and similar management, these patients had worse prognosis compared with other patients with PAH, suggesting more rapid disease progression.
Background Right aortic arch with aberrant left subclavian artery is the most frequent cause of vascular ring. Ligamentum arteriosus division opens the ring but leaves the Kommerell diverticulum in ...place, with a risk of residual compression, aneurysmal dilation, dissection, or even rupture. A procedure consisting of translocation of the aberrant left subclavian artery to the left carotid artery with removal of the Kommerell diverticulum and division of the ligamentum is currently advocated. Methods Between September /2009 and August 2011, 12 consecutive patients underwent the foregoing procedure. Clinical findings, surgical procedure, complications, histopathologic findings, and follow-up data were retrospectively analyzed. Results Mean age at the time of operation was 7.7 years (median, 4.3 years, range, 0.9 to 18.9 years), and mean weight was 26 kg (median, 18 kg; range, 8.4 to 59 kg). All patients had symptoms. Computed tomography scan or magnetic resonance imaging, or both, had confirmed the diagnosis in all patients. Postoperative events, all transient, included chylothorax (1 patient), phrenic palsy (1 patient), pneumonia (1 patient), and the need for prolonged chest drainage (3 patients). Mean follow-up reached 19 months (median, 13 months; range, 12 to 41 months). Mild residual respiratory symptoms were noted in 3 patients. Echo-Doppler analysis showed a patent left subclavian–to–carotid artery anastomosis. Histopathologic analysis of the resected diverticulum (n = 6) showed cystic medial necrosis in four diverticula (focal in one). Conclusions Translocation of the aberrant left subclavian artery with diverticulum resection and ligamentum division is an efficient procedure for symptom relief. Profound wall abnormalities such as medial necrosis in at least 50% of the analyzed diverticula encourage us to maintain this strategy, to reduce the risk of aneurysm formation and dissection.
Kawasaki disease (KD) can be associated with high morbidity and mortality due to coronary artery aneurysms formation and myocardial dysfunction. Aim of this study was to evaluate the diagnostic ...performance of non-invasive myocardial work in predicting subtle myocardial abnormalities in Kawasaki disease (KD) children with coronary dilatation (CADL). A total of 100 patients (age 8.7 ± 5 years) were included: 45 children with KD and CADL (KD/CADL) (Z-score > 2.5), 45 age-matched controls (CTRL) and, finally, an additional group of 10 children with KD in absence of coronary dilatation (KD group). Left ventricular (LV) systolic function and global longitudinal strain (GLS) were assessed. Global myocardial work index (MWI) was calculated as the area of the LV pressure-strain loops. From MWI, global Constructive Work (MCW), Wasted Work (MWW) and Work Efficiency (MWE) were estimated. Despite normal LV systolic function by routine echocardiography, KD/CADL patients had lower MWI (1433.2 ± 375.8 mmHg% vs 1752.2 ± 265.7 mmHg%, p < 0.001), MCW (1885.5 ± 384.2 mmHg% vs 2175.9 ± 292.4 mmHg%, p = 0.001) and MWE (994.0 ± 4.8% vs 95.9 ± 2.0%, p = 0.030) compared to CTRL. Furthermore, MWI was significantly reduced in children belonging to the KD group in comparison with controls (KD: 1498.3 ± 361.7 mmHg%; KD vs CTRL p = 0.028) and was comparable between KD/CADL and KD groups (KD/CADL vs KD p = 0.896). Moreover, KD/CADL patients with normal GLS (n = 38) preserved significant differences in MWI and MCW in comparison with CTRL. MWI, MCW and MWE were significantly reduced in KD children despite normal LVEF and normal GLS. These abnormalities seems independent from CADL. Thus, in KD with normal LVEF and normal GLS, estimation of MWI may be a more sensitive indicator of myocardial dysfunction.
Persistent patent ductus arteriosus is a very common condition in preterm infants. Although there is no management agreed by consensus, despite numerous randomized controlled trials, hemodynamically ...significant patent ductus arteriosus increases morbidity and mortality in these vulnerable patients. Medical treatment is usually offered as first-line therapy, although it carries a limited success rate and potential severe adverse events. In recent years, transcatheter patent ductus arteriosus closure has fast developed and become widely accepted as a safe and efficient alternative to surgical ductal ligation in extremely low birth weight infants >700 g, using most often the dedicated Amplatzer Piccolo Occluder device. This article aims to provide an appraisal of the patients' selection process, and a step-by-step description of the procedure as well as a comprehensive review of its outcomes.
Persistent patent ductus arteriosus (PDA) is very common in preterm infants, especially in extremely preterm infants. Despite significant advances in management of these vulnerable infants, there has ...been no consensus on management of PDA—when should we treat, who should we treat, how should we treat and in fact there is no agreement on how we should define a hemodynamically significant PDA. Medical management with non-steroidal anti-inflammatory drugs (NSAIDs) remains the first line of therapy with moderate success rate in closing the PDA. Paracetamol has been reported to be a safe and equally effective medical therapy for closure of PDA. However, additional studies on its long-term safety and efficacy in extremely low birth weight infants are needed before paracetamol can be recommended as standard treatment for a PDA in preterm infants. Surgical ligation of PDA is not without an increased risk of mortality and co-morbidities. Recently, there has been a significant interest in percutaneous transcatheter closure of PDA in preterm infants, including extremely low birth weight infants. Transcatheter PDA closure in preterm ELBW infants is technically feasible with high PDA occlusion success rates and acceptable complication rates as compared to surgical ligation. Many centers have reported promising early- and mid-term follow-up results. However, they need to be further tested in the prospective well-designed studies and randomized controlled trials comparing the results and outcomes of this technique with current treatment strategies including medical treatment before they can be used as the new standard of care for PDA closure in extremely low birth weight infants.
Conduit rupture is a serious complication encountered during percutaneous pulmonary valve implantation (PPVI). We sought to evaluate the incidence and predictors of conduit rupture during right ...ventricular outflow tract (RVOT) transcatheter treatment.
All consecutive patients who underwent transcatheter RVOT treatment from May 2008 to December 2011 were prospectively studied. Baseline demographics along with incidence, predictors and outcomes of conduit rupture with various transcatheter therapies were reviewed. Conduit rupture occurred in nine out of 99 patients (9.09%). All conduit ruptures occurred during balloon dilatation. Significant risk factors included heavy calcification (p<0.05, OR=16 1.87-357), and conduit type (homograft/others; p<0.05, OR=4.37 1.1-17.8). Other factors such as prolonged time interval between prior surgical RVOT repair and interventions, use of high-pressure balloons, balloon diameter, and overexpansion of conduit statistically failed to show any association. All patients were managed in the cardiac catheterisation laboratory. There were no delayed complications during a mean follow-up period of 2.3±0.95 years.
Conduit rupture is a serious complication. Heavy calcification and homograft conduit were significant predictors. Immediate diagnosis with the use of targeted interventional therapies should be attempted before proceeding with PPVI.
2D-transesophageal echocardiography (TEE) is routinely performed to guide percutaneous ASD closure in children. We aimed to assess whether two-dimensional (2D)-transthoracic echocardiography (TTE) is ...a safe alternative for assessment and guidance of atrial septal defect (ASD) closure in unselected children.
We performed a retrospective single-center study including 389 consecutive children aged less than 15-year-old who underwent percutaneous ASD closure under 2D-TEE (1998-2005, n=133) or 2D-TTE (2005-2014, n=256). A balloon calibration was performed in all cases for the Amplatz Septal Occluder choice.
ASDs were larger and rims deficiencies were more frequent in the TTE-guided group. The procedure was successful in 376 patients 96.7%; 95% confidence interval (CI), 94.4-98.2%. The success rate tended to be higher in the TTE- versus TEE-guided group (98.0% versus 94.0%, P=0.069). Device migration occurred in 4 patients (1.0%; 95% CI: 0.3-1.6%), all after TEE-guided procedure (P=0.013). Early major adverse events were observed in 5 patients (1.3%; 95% CI: 0.4-3.0%), all in the TEE group (P=0.004). Fluroroscopic time and irradiation dose were not different among the 2 groups (P=0.450 and P=0.130 respectively). After a median follow-up of 7 years (range, 1-16 years), no adverse events was reported. One (0.3%, 95% CI: 0-1.4%) 12-year-old patient developed atrial fibrillation 5 years after the procedure. Pregnancies were uneventful in 72 cases.
When a balloon sizing is performed, 2D-TTE imaging is as efficient as 2D-TEE to guide percutaneous ASD closure in children. The procedure can safely be done in spontaneously breathing children under TTE guidance alone in experienced centers.
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