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1.
Pediatr Transplant ; 28(3): e14710, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38553813

RESUMEN

As caregivers for critically ill children and their families, there are moments when we find ourselves unsure of how best to offer support. The magnitude of the medical experience can cloud our communication with patients and their families. With years of counseling families through some of their most challenging and darkest hours, I aim to emphasize the profound impact of thoughtful and well-informed reassurance; how we can provide hope in hopeless situations.


Asunto(s)
Cuidadores , Comunicación , Niño , Humanos
2.
Pediatr Transplant ; 28(1): e14632, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-37897124

RESUMEN

BACKGROUND: The use of apixaban in the pediatric cardiac population is expanding. We describe our apixaban dosing and monitoring strategy in children and young adults awaiting heart transplantation, along with outcomes related to bleeding and thrombosis during wait-list and early post-transplant periods. METHODS: This study is a retrospective, single-center analysis of all patients receiving apixaban while awaiting cardiac transplantation. Weight-based dosing was monitored with peak drug-specific anti-Xa chromogenic analysis. Significant post-operative bleeding defined by chest tube output or need for surgical intervention. RESULTS: From September 2020 to December 2022, 19 patients, median age 13.5 years (6.1, 15.8 years), weighing 48.9 kg (15.4, 67.6) received apixaban while awaiting transplant. Indication for apixaban was prophylaxis (n = 18, 3 with ventricular assist devices) and treatment of thrombus (n = 1). There were no clinically relevant non-major or major bleeding, nor thrombotic events while awaiting transplant. The median time from last apixaban dose to arrival in the operating room was 23.2 h (15.6-33.8), with median random apixaban level of 37 ng/mL (28.3, 59), 6.3 h (4.8, 8.4) prior to arrival in the operating room. In this study, 32% of patients had significant post-operative bleeding based on chest tube output post-transplant or need for intervention. No patients meeting criteria for significant post-operative bleeding were thought to be attributable to apixaban. CONCLUSIONS: Careful use of apixaban can be safe and effective while awaiting heart transplant. There was no appreciable increase in peri-operative bleeding. The use of apixaban is promising in providing safe, predictable and efficacious anticoagulation while avoiding additional patient stressors.


Asunto(s)
Inhibidores del Factor Xa , Trasplante de Corazón , Pirazoles , Piridonas , Niño , Adulto Joven , Humanos , Adolescente , Estudios Retrospectivos , Inhibidores del Factor Xa/uso terapéutico , Inhibidores del Factor Xa/análisis , Hemorragia/epidemiología , Anticoagulantes/uso terapéutico
3.
Pediatr Crit Care Med ; 25(7): e318-e327, 2024 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-38619330

RESUMEN

OBJECTIVES: Placement of a ventricular assist device (VAD) improves outcomes in children with advanced heart failure, but adverse events remain important consequences. Preoperative mechanical ventilation (MV) increases mortality, but it is unknown what impact prolonged postoperative MV has. DESIGN: Advanced Cardiac Therapies Improving Outcomes Network (ACTION) and Pediatric Cardiac Critical Care Consortium (PC 4 ) registries were used to identify and link children with initial VAD placement admitted to the cardiac ICU (CICU) from August 2014 to July 2020. Demographics, cardiac diagnosis, preoperative and postoperative CICU courses, and outcomes were compiled. Univariable and multivariable statistics assessed association of patient factors with prolonged postoperative MV. Multivariable logistic regression sought independent associations with outcomes. SETTING: Thirty-five pediatric CICUs across the United States and Canada. PATIENTS: Children on VADs included in both registries. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Two hundred forty-eight ACTION subjects were linked to a matching patient in PC 4 . Median (interquartile) age 7.7 years (1.5-15.5 yr), weight 21.3 kg (9.1-58 kg), and 56% male. Primary diagnosis was congenital heart disease (CHD) in 35%. Pre-VAD explanatory variables independently associated with prolonged postoperative MV included: age (incidence rate ratio [IRR], 0.95; 95% CI, 0.93-0.96; p < 0.01); preoperative MV within 48 hours (IRR, 2.76; 95% CI, 1.59-4.79; p < 0.01), 2-7 days (IRR, 1.82; 95% CI, 1.15-2.89; p = 0.011), and greater than 7 days before VAD implant (IRR, 2.35; 95% CI, 1.62-3.4; p < 0.01); and CHD (IRR, 1.96; 95% CI, 1.48-2.59; p < 0.01). Each additional day of postoperative MV was associated with greater odds of mortality (odds ratio [OR], 1.09 per day; p < 0.01) in the full cohort. We identified an associated greater odds of mortality in the 102 patients with intracorporeal devices (OR, 1.24; 95% CI, 1.04-1.48; p = 0.014), but not paracorporeal devices (77 patients; OR, 1.04; 95% CI, 0.99-1.09; p = 0.115). CONCLUSIONS: Prolonged MV after VAD placement is associated with greater odds of mortality in intracorporeal devices, which may indicate inadequacy of cardiopulmonary support in this group. This linkage provides a platform for future analyses in this population.


Asunto(s)
Insuficiencia Cardíaca , Corazón Auxiliar , Sistema de Registros , Respiración Artificial , Humanos , Niño , Masculino , Femenino , Respiración Artificial/estadística & datos numéricos , Preescolar , Lactante , Adolescente , Insuficiencia Cardíaca/terapia , Insuficiencia Cardíaca/mortalidad , Estados Unidos/epidemiología , Canadá/epidemiología , Unidades de Cuidado Intensivo Pediátrico , Resultado del Tratamiento , Complicaciones Posoperatorias/epidemiología
4.
Cardiol Young ; 34(3): 531-534, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-37518866

RESUMEN

The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) and Pediatric Heart Transplant Society (PHTS) convened a working group at the beginning of 2020 during the COVID-19 pandemic, with the aim of using telehealth as an alternative medium to provide quality care to a high-acuity paediatric population receiving advanced cardiac therapies. An algorithm was developed to determine appropriateness, educational handouts were developed for both patients and providers, and post-visit surveys were collected. Telehealth was found to be a viable modality for health care delivery in the paediatric heart failure and transplant population and has promising application in the continuity of follow-up, medication titration, and patient education/counselling domains.


Asunto(s)
Insuficiencia Cardíaca , Trasplante de Corazón , Telemedicina , Humanos , Niño , Pandemias , Insuficiencia Cardíaca/cirugía , Algoritmos
5.
Pediatr Emerg Care ; 39(12): 907-912, 2023 Dec 01.
Artículo en Inglés | MEDLINE | ID: mdl-37246140

RESUMEN

PURPOSE: We sought to establish whether nucleated red blood cells (NRBCs) are predictive of disposition, morbidity, and mortality for pediatric patients presenting to the emergency department (ED). METHODS: A single-center retrospective cohort study examining all ED encounters from patients aged younger than 19 years between January 2016 and March 2020, during which a complete blood count was obtained. Univariate analysis and multivariable logistic regression were used to test the presence of NRBCs as an independent predictor of patient-related outcomes. RESULTS: The prevalence of NRBCs was 8.9% (4195/46,991 patient encounters). Patient with NRBCs were younger (median age 4.58 vs 8.23 years; P < 0.001). Those with NRBCs had higher rates of in-hospital mortality (30/2465 [1.22%] vs 65/21,741 [0.30%]; P < 0.001), sepsis (19% vs 12%; P < 0.001), shock (7% vs 4%; P < 0.001), and cardiopulmonary resuscitation (CPR) (0.62% vs 0.09%; P < 0.001). They were more likely to be admitted (59% vs 51%; P < 0.001), have longer median hospital length of stay {1.3 (interquartile range [IQR], 0.22-4.14) vs 0.8 days (IQR, 0.23-2.64); P < 0.001}, and median intensive care unit (ICU) length of stay (3.9 [IQR, 1.87-8.72] vs 2.6 days [IQR, 1.27-5.83]; P < 0.001). Multivariable regression revealed presence of NRBCs as an independent predictor for in-hospital mortality (adjusted odds ratio [aOR], 2.21; 95% confidence interval [CI], 1.38-3.53; P < 0.001), ICU admission (aOR, 1.30; 95% CI, 1.11-1.51; P < 0.001), CPR (aOR, 3.83; 95% CI, 2.33-6.30; P < 0.001), and 30-day return to the ED (aOR, 1.15; 95% CI, 1.15-1.26; P < 0.001). CONCLUSIONS: The presence of NRBCs is an independent predictor for mortality, including in-hospital mortality, ICU admission, CPR, and readmission within 30 days for children presenting to the ED.


Asunto(s)
Eritroblastos , Unidades de Cuidados Intensivos , Humanos , Niño , Anciano , Preescolar , Estudios Retrospectivos , Mortalidad Hospitalaria , Recuento de Células Sanguíneas
6.
Am J Transplant ; 22(3): 833-842, 2022 03.
Artículo en Inglés | MEDLINE | ID: mdl-34897984

RESUMEN

We describe waiting times for pediatric heart transplant (HT) candidates after the 2016 revision to the US allocation policy. The OPTN database was queried for pediatric HT candidates listed between 7/2016 and 4/2019. Of the 1789 included candidates, 65% underwent HT, 14% died/deteriorated, 8% were removed for improvement, and 13% were still waiting at the end of follow-up. Most candidates were status 1A at HT (81%). Median wait times differ substantially by listing status, blood type, and recipient weight. The likelihood of HT was lower in candidates <25 kg and in those with blood type O; The <25 kg, blood type O subgroup experiences longer wait times and higher wait list mortality. For status 1A candidates, median wait times were 108 days (≤25 kg, blood type O), 80 days (≤25 kg, non-O), 47 days (>25 kg, O), and 24 days (>25 kg, non-O). We found that centers with more selective organ acceptance practices, based on a lower median Pediatric Heart Donor Assessment Tool (PH-DAT) score for completed transplants, experience longer status 1A wait times for their listed patients. These data can be used to counsel families and to select appropriate advanced heart failure therapies to support patients to transplant.


Asunto(s)
Cardiopatías Congénitas , Trasplante de Corazón , Obtención de Tejidos y Órganos , Niño , Humanos , Políticas , Donantes de Tejidos , Estados Unidos , Listas de Espera
7.
Artif Organs ; 46(7): 1389-1398, 2022 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-35132634

RESUMEN

OBJECTIVE: Ventricular assist devices (VADs) increase waitlist survival, yet the risk of stroke remains notable. The purpose of this study was to analyze how strokes on VAD support impact post-transplant (post-Tx) outcomes in children. METHODS: About 520 pediatric (<18 years) heart transplant candidates listed from January 2011 to April 2018 with a VAD implant date were matched between the United Network of Organ Sharing and Pediatric Health Information System databases. Patients were divided into pre-Tx Stroke and No Stroke cohorts. RESULTS: About 81% of the 520 patients were transplanted; 28% (n = 146) had a pre-Tx Stroke; and 59% (n = 89) of the Stroke patients were transplanted at a median of 57 (IQR 17-102) days from stroke. Significantly more No Stroke cohort (90%) were transplanted (p < 0.001). There was no difference in post-Tx survival between the Stroke and No Stroke cohorts (p = 0.440). Time between stroke and transplant for patients who died within 1 year of transplant was 32.0 days (median) compared to 60.5 days for those alive >1 year (p = 0.18). Regarding patients in whom time from stroke to transplant was more than 60 days, one-year survival of Stroke vs. No Stroke patients was 96% vs. 95% (p = 0.811), respectively. CONCLUSION: Patients with stroke during VAD support, once transplanted, enjoy similar survival compared to No Stroke patients. We hypothesize that allowing Stroke patients more time to recover could improve post-Tx outcomes. Unfortunately, the ideal duration of time between stroke and safe transplantation could not be determined and will require more detailed and larger studies in the future.


Asunto(s)
Insuficiencia Cardíaca , Trasplante de Corazón , Corazón Auxiliar , Accidente Cerebrovascular , Niño , Trasplante de Corazón/efectos adversos , Corazón Auxiliar/efectos adversos , Humanos , Estudios Retrospectivos , Accidente Cerebrovascular/etiología , Resultado del Tratamiento , Listas de Espera
8.
Pediatr Cardiol ; 43(6): 1379-1382, 2022 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-35247057

RESUMEN

We sought to characterize strokes in children with ventricular assist devices. Of 407 patients in the ACTION registry (4/1/18-5/3/2021), 45 (11%) experienced 52 strokes (45 ischemic and 7 hemorrhagic). Median time to stroke was 23.5 days and 19/52 (37%) occurred ≤ 10 days. Stroke rate was 0.09 and 0.63 strokes per patient-year for implantable continuous and paracorporeal devices, respectively. Patients with stroke were younger, more likely to have congenital heart disease and have been on extracorporeal membrane oxygenation at time of VAD. Based on these data, ACTION is now focused on decreasing strokes in these higher-risk patients with particular attention to the peri-implant period.


Asunto(s)
Oxigenación por Membrana Extracorpórea , Insuficiencia Cardíaca , Corazón Auxiliar , Accidente Cerebrovascular , Niño , Insuficiencia Cardíaca/etiología , Corazón Auxiliar/efectos adversos , Humanos , Sistema de Registros , Estudios Retrospectivos , Accidente Cerebrovascular/epidemiología , Accidente Cerebrovascular/etiología , Accidente Cerebrovascular/prevención & control , Resultado del Tratamiento
9.
Pediatr Blood Cancer ; 68(12): e29355, 2021 12.
Artículo en Inglés | MEDLINE | ID: mdl-34532964

RESUMEN

OBJECTIVE: To characterize viscoelastic testing profiles of children with multisystem inflammatory syndrome in children (MIS-C). METHODS: This single-center retrospective review included 30 patients diagnosed with MIS-C from March 1 to September 1, 2020. Thromboelastography (TEG) with platelet mapping was performed in 19 (63%) patients and compared to age- and sex-matched controls prior to cardiac surgery. Relationships between TEG parameters and inflammatory markers were assessed using correlation. RESULTS: Patients with MIS-C had abnormal TEG results compared to controls, including decreased kinetic (K) time (1.1 vs. 1.7 minutes, p < .01), increased alpha angle (75.0° vs. 65.7°, p < .01), increased maximum amplitude (70.8 vs. 58.3 mm, p < .01), and decreased lysis in 30 minutes (Ly30) (1.1% vs. 3.7%, p = .03); consistent with increased clot formation rate and strength, and reduced fibrinolysis. TEG maximum amplitude was moderately correlated with erythrocyte sedimentation rate (ESR) (r = 0.60, p = .02), initial platelet count (r = 0.67, p < .01), and peak platelet count (r = 0.51, p = .03). TEG alpha angle was moderately correlated with peak platelet count (r = 0.54, p = .02). Seventeen (57%) patients received aspirin (ASA) and anticoagulation, five (17%) received only ASA, and three (10%) received only anticoagulation. No patients had a symptomatic thrombotic event. Six (20%) patients had a bleeding event, none of which was major. CONCLUSIONS: Patients with MIS-C had evidence of hypercoagulability on TEG. Increased ESR and platelets were associated with higher clot strength. Patients were prophylactically treated with ASA or anticoagulation with no symptomatic thrombosis or major bleeding. Further multicenter study is required to characterize the rate of thrombosis and optimal thromboprophylaxis algorithm in this patient population.


Asunto(s)
Coagulación Sanguínea , COVID-19/complicaciones , Síndrome de Respuesta Inflamatoria Sistémica/sangre , Trombofilia/sangre , Adolescente , Anticoagulantes/uso terapéutico , Aspirina/uso terapéutico , Coagulación Sanguínea/efectos de los fármacos , Plaquetas/efectos de los fármacos , COVID-19/sangre , Niño , Preescolar , Femenino , Humanos , Masculino , Estudios Retrospectivos , Síndrome de Respuesta Inflamatoria Sistémica/tratamiento farmacológico , Tromboelastografía , Trombofilia/tratamiento farmacológico , Tratamiento Farmacológico de COVID-19
10.
Eur J Pediatr ; 180(2): 307-322, 2021 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-32803422

RESUMEN

Initial reports on COVID-19 described children as largely spared from severe manifestations, with only 2-6% of children requiring intensive care treatment. However, since mid-April 2020, clusters of pediatric cases of severe systemic hyperinflammation and shock epidemiologically linked with COVID-19 have been reported. This condition was named as SARS-Cov-2-associated multisystem inflammatory syndrome in children and showed similarities to Kawasaki disease. Here, we present a narrative review of cases reported in literature and we discuss the clinical acute and follow-up management of these patients. Patients with SARS-Cov-2-associated multisystem inflammatory syndrome frequently presented with persistent fever, gastrointestinal symptoms, polymorphic rash, conjunctivitis, and mucosal changes. Elevated inflammatory markers and evidence of cytokine storm were frequently observed. A subset of these patients also presented with hypotension and shock (20-100%) from either acute myocardial dysfunction or systemic hyperinflammation/vasodilation. Coronary artery dilation or aneurysms have been described in 6-24%, and arrhythmias in 7-60%. Cardiac support, immunomodulation, and anticoagulation are the key aspects for the management of the acute phase. Long-term structured follow-up of these patients is required due to the unclear prognosis and risk of progression of cardiac manifestations.Conclusion: Multisystem inflammatory syndrome is a novel syndrome related to SARS-CoV-2 infection. Evidence is still scarce but rapidly emerging in the literature. Cardiac manifestations are frequent, including myocardial and coronary involvement, and need to be carefully identified and monitored over time. What is Known: • Multisystem inflammatory syndrome in children (MIS-C) has been described associated with SARS-CoV-2. What is New: • Patients with MIS-C often present with fever, gastrointestinal symptoms, and shock. • Cardiac involvement is found in a high proportion of these patients, including ventricular dysfunction, coronary artery dilation or aneurysm, and arrhythmias. • Management is based on expert consensus and includes cardiac support, immunomodulatory agents, and anticoagulation. • Long-term follow-up is required due to the unclear prognosis and risk of progression of cardiac manifestation.


Asunto(s)
COVID-19/complicaciones , Cardiopatías/etiología , SARS-CoV-2 , Síndrome de Respuesta Inflamatoria Sistémica/complicaciones , Niño , Cuidados Críticos/estadística & datos numéricos , Humanos
11.
Pediatr Cardiol ; 42(7): 1635-1639, 2021 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-34076713

RESUMEN

With growing number of pediatric cases of COVID-19, a unique hyper-inflammatory syndrome, linked to SARS-CoV-2 infection, has emerged in children referred to as multisystem inflammatory syndrome in children (MIS-C). This Kawasaki Disease (KD)-like illness has been described across the world. This syndrome shares features of KD, toxic shock syndrome, and macrophage activation syndrome and is associated with significantly elevated inflammatory markers. Everyday there are new data emerging improving the care of these patients. The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) is a collaborative network designed to improve the outcomes of pediatric patients with end-stage heart failure and involves centers from across North America. The committee gathered information concerning COVID-19 anticoagulation practices at various centers and harmonized the data to formulate a set of recommendations.


Asunto(s)
COVID-19 , Síndrome Mucocutáneo Linfonodular , Niño , Humanos , Síndrome Mucocutáneo Linfonodular/complicaciones , Síndrome Mucocutáneo Linfonodular/tratamiento farmacológico , SARS-CoV-2 , Síndrome de Respuesta Inflamatoria Sistémica
12.
Mol Genet Metab ; 131(1-2): 147-154, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32828637

RESUMEN

Inborn errors of metabolism (IEM) involving the non-oxidative pentose phosphate pathway (PPP) include the two relatively rare conditions, transketolase deficiency and transaldolase deficiency, both of which can be difficult to diagnosis given their non-specific clinical presentations. Current biochemical testing approaches require an index of suspicion to consider targeted urine polyol testing. To determine whether a broad-spectrum biochemical test could accurately identify a specific metabolic pattern defining IEMs of the non-oxidative PPP, we employed the use of clinical metabolomic profiling as an unbiased novel approach to diagnosis. Subjects with molecularly confirmed IEMs of the PPP were included in this study. Targeted quantitative analysis of polyols in urine and plasma samples was accomplished with chromatography and mass spectrometry. Semi-quantitative unbiased metabolomic analysis of urine and plasma samples was achieved by assessing small molecules via liquid chromatography and high-resolution mass spectrometry. Results from untargeted and targeted analyses were then compared and analyzed for diagnostic acuity. Two siblings with transketolase (TKT) deficiency and three unrelated individuals with transaldolase (TALDO) deficiency were identified for inclusion in the study. For both IEMs, targeted polyol testing and untargeted metabolomic testing on urine and/or plasma samples identified typical perturbations of the respective disorder. Additionally, untargeted metabolomic testing revealed elevations in other PPP metabolites not typically measured with targeted polyol testing, including ribonate, ribose, and erythronate for TKT deficiency and ribonate, erythronate, and sedoheptulose 7-phosphate in TALDO deficiency. Non-PPP alternations were also noted involving tryptophan, purine, and pyrimidine metabolism for both TKT and TALDO deficient patients. Targeted polyol testing and untargeted metabolomic testing methods were both able to identify specific biochemical patterns indicative of TKT and TALDO deficiency in both plasma and urine samples. In addition, untargeted metabolomics was able to identify novel biomarkers, thereby expanding the current knowledge of both conditions and providing further insight into potential underlying pathophysiological mechanisms. Furthermore, untargeted metabolomic testing offers the advantage of having a single effective biochemical screening test for identification of rare IEMs, like TKT and TALDO deficiencies, that may otherwise go undiagnosed due to their generally non-specific clinical presentations.


Asunto(s)
Errores Innatos del Metabolismo de los Carbohidratos/genética , Errores Innatos del Metabolismo/genética , Transaldolasa/deficiencia , Transaldolasa/genética , Transcetolasa/genética , Adulto , Biomarcadores/sangre , Errores Innatos del Metabolismo de los Carbohidratos/sangre , Errores Innatos del Metabolismo de los Carbohidratos/metabolismo , Errores Innatos del Metabolismo de los Carbohidratos/patología , Niño , Preescolar , Cromatografía Liquida , Femenino , Humanos , Lactante , Masculino , Espectrometría de Masas , Errores Innatos del Metabolismo/sangre , Errores Innatos del Metabolismo/metabolismo , Errores Innatos del Metabolismo/patología , Metabolómica , Vía de Pentosa Fosfato/genética , Transaldolasa/sangre , Transaldolasa/metabolismo , Transcetolasa/sangre , Transcetolasa/deficiencia , Adulto Joven
13.
J Cardiovasc Electrophysiol ; 31(12): 3243-3250, 2020 12.
Artículo en Inglés | MEDLINE | ID: mdl-33112018

RESUMEN

INTRODUCTION: Guidelines recommend trans-esophageal echocardiography (TEE) for patients with atrial fibrillation (AF) or atrial flutter (AFL) for >48 h, due to risk of intracardiac thrombus formation. With growing evidence that AFL in adults with structurally normal hearts has less thrombogenic potential compared to AF, and the need for TEE questioned, we compared prevalence of intracardiac thrombus detected by TEE in pediatric and congenital heart disease (CHD) patients presenting in AF and AFL. METHODS/RESULTS: Single-center, cross-sectional analysis for unique first-time presentations of patients for either AF, AFL, or intra-atrial reentrant tachycardia (IART) between 2000 and 2019. Patients were categorized by presenting arrhythmia (AF vs. AFL/IART), with the exclusion of other forms of atrial tachycardia, hemodynamic instability, chronic anti-coagulation before TEE, and presentation for a reason other than TEE examination for thrombus. A total of 201 patients had TEE with co-diagnosis of AF or AFL. Of these, 105 patients (29 AF, 76 AFL) met inclusion criteria, with no difference in age between AF (median 24.9 years; IQR 18.6-38.3 years) and AFL/IART (23.3 years; 15.4-38.4 years). The prevalence of thrombus in the entire cohort was 9.5%, with no difference between AF (13.8%) and AFL groups (7.9%), p = .46. Patients with thrombus demonstrated no difference in age, systemic ventricular function, cardiac complexity, or CHADS2/CHA2DS2VASc score at presentation. CONCLUSIONS: The risk for intracardiac thrombus is high in the pediatric and CHD population, with no apparent distinguishing factors to warrant a change in the recommendations for TEE, with all levels of cardiac complexity being at risk for clot.


Asunto(s)
Fibrilación Atrial , Aleteo Atrial , Cardiopatías Congénitas , Trombosis , Adolescente , Adulto , Fibrilación Atrial/diagnóstico por imagen , Fibrilación Atrial/epidemiología , Aleteo Atrial/diagnóstico por imagen , Aleteo Atrial/epidemiología , Niño , Estudios Transversales , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/epidemiología , Humanos , Prevalencia , Trombosis/diagnóstico por imagen , Trombosis/epidemiología , Adulto Joven
14.
Pediatr Cardiol ; 39(8): 1514-1522, 2018 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-29948029

RESUMEN

Infants with a single ventricle can develop systemic ventricular dysfunction (SVD) after stage 1 operation, but available information is sparse. We reviewed our patients having Norwood, Sano, or hybrid procedures to better understand this problem. We conducted a retrospective, case-controlled cohort study of 267 patients having stage1 operation, examining outcomes between stages 1 and 2 (survival and subsequent cardiac surgeries), predictor variables, and histology of hearts explanted at transplantation. SVD developed in 32 (12%) patients and resolved in 13 (41%); mean age of onset was 3.0 ± 1.63 months; median = 2.79. SVD was not associated with cardiac anatomy, type of stage 1 procedure, weight, coronary abnormality, or atrioventricular valve regurgitation. The mean age of resolution = 12.1 ± 9.6 months; median = 6.3, and resolution may have been more likely with a systemic LV than RV (p = 0.067). Outcomes for the entire SVD group were less favorable than for those without, but patients with resolution of SVD had outcomes at least as good those without SVD. Myocardial histology (n = 4) suggested chronic ischemia. The risk of SVD after stage 1, while low, may be a fundamental feature of this patient population. SVD occurs with either a systemic RV or LV, although patients with a systemic LV may be more likely to have resolution than those with an RV. We identified no predictor variables, but histologic findings suggest chronic ischemia may be involved. Given the low incidence of SVD, multi-center studies will be required to better define predictors of onset and resolution.


Asunto(s)
Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Procedimientos de Norwood , Cuidados Paliativos/métodos , Disfunción Ventricular/etiología , Estudios de Casos y Controles , Estudios de Cohortes , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Procedimientos de Norwood/efectos adversos , Estudios Retrospectivos , Disfunción Ventricular/fisiopatología
15.
J Pediatr ; 189: 61-65, 2017 10.
Artículo en Inglés | MEDLINE | ID: mdl-28552449

RESUMEN

OBJECTIVE: To describe the safety and efficacy of warfarin for patients with Kawasaki disease and giant coronary artery aneurysms (CAAs, ≥8 mm). Giant aneurysms are managed with combined anticoagulation and antiplatelet therapies, heightening risk of bleeding complications. STUDY DESIGN: We reviewed the time in therapeutic range; percentage of international normalization ratios (INRs) in range (%); bleeding events, clotting events; INRs ≥6; INRs ≥5 and <6; and INRs <1.5. RESULTS: In 9 patients (5 male), median age 14.4 years (range 7.1-22.8 years), INR testing was prescribed weekly to monthly and was done by home monitor (n = 5) or laboratory (n = 3) or combined (1). Median length of warfarin therapy was 7.2 years (2.3-13.3 years). Goal INR was 2.0-3.0 (n = 6) or 2.5-3.5 (n = 3), based on CAA size and history of CAA thrombosis. All patients were treated with aspirin; 1 was on dual antiplatelet therapy and warfarin. The median time in therapeutic range was 59% (37%-85%), and median percentage of INRs in range was 68% (52%-87%). INR >6 occurred in 3 patients (4 events); INRs ≥5 <6 in 7 patients (12 events); and INR <1.5 in 5 patients (28 events). The incidence of major bleeding events and clinically relevant nonmajor bleeding events were each 4.3 per 100 patient-years (95% CI 0.9-12.6). New asymptomatic coronary thrombosis was detected by imaging in 2 patients. CONCLUSIONS: Bleeding and clotting complications are common in patients with Kawasaki disease on warfarin and aspirin, with INRs in range only two-thirds of the time. Future studies should evaluate the use of direct oral anticoagulants in children as an alternative to warfarin.


Asunto(s)
Anticoagulantes/uso terapéutico , Síndrome Mucocutáneo Linfonodular/tratamiento farmacológico , Warfarina/uso terapéutico , Adolescente , Anticoagulantes/efectos adversos , Niño , Femenino , Hemorragia/inducido químicamente , Hemorragia/epidemiología , Humanos , Incidencia , Relación Normalizada Internacional , Masculino , Estudios Retrospectivos , Trombosis/inducido químicamente , Trombosis/epidemiología , Warfarina/efectos adversos , Adulto Joven
16.
Pediatr Cardiol ; 38(5): 1032-1041, 2017 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-28456829

RESUMEN

Mechanical circulatory support in the form of ventricular assist devices (VADs) in children has undergone rapid growth in the last decade. With expansion of device options available for larger children and adolescents, the field of outpatient VAD support has flourished, with many programs unprepared for the clinical, programmatic, and administrative responsibilities. From preimplantation VAD evaluation and patient education to postimplant VAD management, the VAD program, staffed with an interdisciplinary team, is essential to providing safe, effective, and sustainable care for a new technology in an exceedingly complex patient population. Herein, this paper describes the Boston Children's Hospital VAD experience over a decade and important lessons learned from developing a pediatric program focusing on a high-risk but low-volume population. We highlight the paramount role of the VAD coordinator, clinical infrastructure requirements, as well as innovation in care spanning inpatient and outpatient VAD supports at Boston Children's Hospital.


Asunto(s)
Insuficiencia Cardíaca/cirugía , Corazón Auxiliar , Hospitales Pediátricos , Desarrollo de Programa , Adolescente , Boston , Niño , Preescolar , Continuidad de la Atención al Paciente , Femenino , Hospitales Pediátricos/organización & administración , Humanos , Lactante , Recién Nacido , Masculino , Grupo de Atención al Paciente , Desarrollo de Programa/normas , Evaluación de Programas y Proyectos de Salud/normas , Adulto Joven
17.
Pediatr Transplant ; 20(2): 194-202, 2016 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-26841274

RESUMEN

The last five yr have been monumental for the pediatric heart failure community. In the US, the most notable has been the FDA approval of the first pediatric specific device (Berlin Heart EXCOR(®) ; Berlin Heart, Inc., Berlin, Germany). Subsequently, the field of heart failure has gained a great deal of knowledge regarding the nuances of MCS in children. Despite FDA approval in the US, the Berlin EXCOR(®) is only currently indicated for in-hospital use. Due to the limitations with discharge and the positive in- hospital experiences with the Berlin EXCOR(®) , there has been an increased interest in the implantation of adult durable devices into children. While many institutions have focused their intial efforts on the first phase of care within the hospital, they are now ready to tackle the challenge of how to safely transition children to the community setting.


Asunto(s)
Insuficiencia Cardíaca/cirugía , Trasplante de Corazón , Corazón Auxiliar , Adolescente , Conducta del Adolescente , Conducción de Automóvil , Cuidadores , Niño , Continuidad de la Atención al Paciente , Diseño de Equipo , Femenino , Insuficiencia Cardíaca/psicología , Humanos , Masculino , Pacientes Ambulatorios , Alta del Paciente , Educación del Paciente como Asunto , Pruebas en el Punto de Atención , Periodo Posoperatorio , Calidad de Vida , Riesgo
18.
Pediatr Transplant ; 19(1): 27-34, 2015 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-25377124

RESUMEN

Children with non-renal solid organ transplants are surviving longer, but outcome is complicated by CKD. Accurate and frequent renal function monitoring is imperative to recognize and institute measures early to reverse, prevent, or arrest progression. This study of 59 children determined the accuracy (P30), bias, sensitivity and specificity between measured renal function by NM-GFR, and estimated GFR by three formulas: Filler (serum cystatin C), mSchwartz (serum creatinine), and CKiD (serum cystatin C, creatinine, urea, and height). Mean GFR by all formulas differed significantly from NM-GFR. Filler and mSchwartz formulas significantly increased the proportion of patients with GFR ≥ 90 mL/min/1.73 m(2) (CKD stage 1) while decreasing those with GFR 60-89 mL/min/1.73 m(2) (CKD stage 2). All formulas overestimated GFR. CKiD showed the highest P30 and lowest bias (79.7%; 6.9 mL/min/1.73 m(2) ) followed by Filler (67.7%; 19.9 mL/min/1.73 m(2) ) and Schwartz (57.6%; 26.8 mL/min/1.73 m(2) ) for all GFR values. All formulas performed best with GFR ≥ 90 mL/min/1.73 m(2) , but CKiD was the only formula to achieve 91.1% accuracy. All formulas showed high sensitivities, but low specificities at NM-GFR cutoff at 90. Thus, GFR estimated by CKiD followed by Filler formula is an adequate method to monitor renal function closely and frequently in these children.


Asunto(s)
Cistatina C/sangre , Trasplante de Corazón , Riñón/fisiopatología , Trasplante de Hígado , Adolescente , Niño , Preescolar , Femenino , Tasa de Filtración Glomerular , Humanos , Pruebas de Función Renal/métodos , Masculino , Insuficiencia Renal Crónica/sangre , Insuficiencia Renal Crónica/diagnóstico , Insuficiencia Renal Crónica/fisiopatología
19.
Pediatr Cardiol ; 36(4): 842-50, 2015 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-25573076

RESUMEN

Congenital heart disease is the leading cause of stroke in children. Warfarin therapy can be difficult to manage safely in this population because of its narrow therapeutic index, multiple drug and dietary interactions, small patient size, high-risk cardiac indications, and lack of data to support anticoagulation recommendations. We sought to describe our institution's effort to develop a dedicated cardiac anticoagulation service to address the special needs of this population and to review the literature. In 2009, in response to Joint Commission National Patient Safety Goals for Anticoagulation, Boston Children's Hospital created a dedicated pediatric Cardiac Anticoagulation Monitoring Program (CAMP). The primary purpose was to provide centralized management of outpatient anticoagulation to cardiac patients, to serve as a disease-specific resource to families and providers, and to devise strategies to evolve clinical care with rapidly emerging trends in anticoagulation care. Over 5 years the CAMP Service, staffed by a primary pediatric cardiology attending, a full-time nurse practitioner, and administrative assistant with dedicated support from pharmacy and nutrition, has enrolled over 240 patients ranging in age from 5 months to 55 years. The most common indications include a prosthetic valve (34 %), Fontan prophylaxis (20 %), atrial arrhythmias (11 %), cardiomyopathy (10 %), Kawasaki disease (7 %), and a ventricular assist device (2 %). A patient-centered multi-disciplinary cardiac anticoagulation clinic was created in 2012. Overall program international normalized ratio (INR) time in therapeutic range (TTR) is favorable at 67 % (81 % with a 0.2 margin) and has improved steadily over 5 years. Pediatric-specific guidelines for VKOR1 and CYP2C9 pharmacogenomics testing, procedural bridging with enoxaparin, novel anticoagulant use, and quality metrics have been developed. Program satisfaction is rated highly among families and providers. A dedicated pediatric cardiac anticoagulation program offers a safe and effective strategy to standardize anticoagulation care for pediatric cardiology patients, is associated with high patient and provider satisfaction, and is capable of evolving care strategies with emerging trends in anticoagulation.


Asunto(s)
Anticoagulantes/uso terapéutico , Coagulación Sanguínea/efectos de los fármacos , Cardiopatías Congénitas/complicaciones , Atención Primaria de Salud/métodos , Atención Primaria de Salud/estadística & datos numéricos , Warfarina/uso terapéutico , Adolescente , Adulto , Anticoagulantes/administración & dosificación , Boston , Niño , Preescolar , Femenino , Cardiopatías Congénitas/fisiopatología , Humanos , Lactante , Relación Normalizada Internacional , Masculino , Persona de Mediana Edad , Atención Primaria de Salud/organización & administración , Warfarina/administración & dosificación , Adulto Joven
20.
Res Pract Thromb Haemost ; 8(1): 102312, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-38327612

RESUMEN

Background: Direct oral anticoagulants are commonly prescribed for adults and increasingly also for children requiring anticoagulation therapy. While household medications should not be accessible to children, accidental, and intentional overdoses occur. Key Clinical Question: How should apixaban overdose in children be managed?. Clinical Approach: We present a case of an accidental overdose with the factor Xa antagonist apixaban in a young child and propose an approach to the management of cases of apixaban overdose in children. Conclusion: Given the increasing use of direct oral anticoagulants, it is important to have an approach to the management of overdose of these medications.

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