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1.
J Heart Lung Transplant ; 43(5): 816-825, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38232791

RESUMO

BACKGROUND: Presence of donor-specific antibodies (DSAs), particularly to class II antigens, remains a major challenge in pediatric heart transplantation. Donor-recipient human leukocyte antigen (HLA) matching is a potential strategy to mitigate poor outcomes associated with DSAs. We evaluated the hypothesis that antigen mismatching at the DQB1 locus is associated with worse rejection-free survival. METHODS: Data were collected from Scientific Registry of Transplant Recipients for all pediatric heart transplant recipients 2010-2021. Only transplants with complete HLA typing at the DQB1 locus for recipient and donor were included. Primary outcome was rejection-free graft survival through 5 years. RESULTS: Of 5,115 children, 4,135 had complete DQB1 typing and were included. Of those, 503 (12%) had 0 DQB1 donor-recipient mismatches, 2,203 (53%) had 1, and 1,429 (35%) had 2. Rejection-free survival through 5 years trended higher for children with 0 DQB1 mismatches (68%), compared to those with 1 (62%) or 2 (63%) mismatches (pairwise p = 0.08 for both). In multivariable analysis, 0 DQB1 mismatches remained significantly associated with improved rejection-free graft survival compared to 2 mismatches, while 1 DQB1 mismatch was not. Subgroup analysis showed the strongest effect in non-Hispanic Black children and those undergoing retransplant. CONCLUSIONS: Matching at the DQB1 locus is associated with improved rejection-free survival after pediatric heart transplant, particularly in Black children, and those undergoing retransplant. Assessing high-resolution donor typing at the time of allocation may further corroborate and refine this association. DQB1 matching may improve long-term outcomes in children stabilized either with optimal pharmacotherapy or supported with durable devices able to await ideal donors.


Assuntos
Rejeição de Enxerto , Sobrevivência de Enxerto , Cadeias beta de HLA-DQ , Transplante de Coração , Humanos , Masculino , Criança , Feminino , Rejeição de Enxerto/imunologia , Sobrevivência de Enxerto/imunologia , Pré-Escolar , Cadeias beta de HLA-DQ/genética , Lactente , Teste de Histocompatibilidade/métodos , Adolescente , Estudos Retrospectivos , Doadores de Tecidos , Sistema de Registros , Transplantados
2.
J Extra Corpor Technol ; 55(4): 189-193, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-38099633

RESUMO

BACKGROUND: Highly sensitized pediatric patients awaiting heart transplantation experience longer wait times and thus higher waitlist mortality. Similarly, children less than 2 years of age have increased waitlist times and mortality when compared to their older peers. To improve the likelihood of successful transplantation in these patients, various strategies have been utilized, including peri-operative plasmapheresis. However, limited data exists comparing plasmapheresis techniques for antibody reduction. This study's aim was to compare the in vitro magnitude of isohemagglutinin titers (IT) and human leukocyte antigen (HLA) antibody removal and the time required between membrane-based plasmapheresis (MP) and centrifuge-based plasmapheresis (CP) incorporated into the extracorporeal (EC) circuit. METHODS: Two MP (Prismaflex) and two CP (Spectra Optia, Terumo BCT) circuits were incorporated into four separate EC circuits primed with high titer, highly sensitized type O donor whole blood. Assays were performed to determine baseline IT and anti-HLA antibodies and then at 30-minute increments until completion of the run (two plasma volume exchanges) at two hours. RESULTS: There was a decrease in anti-A and anti-B IgM and IgG titers with both MP and CP. Mean anti-A and anti-B titer reduction was by 4.625 titers (93.7% change) and 4.375 titers (93.8% change) using MP and CP, respectively. At 2 h of apheresis, CP reduced 62.5% of all ITs to ≤ 1:4, while MP reduced 50% of ITs to ≤ 1:4. Additionally, reduction of anti-HLA class II antibody to mean fluorescence intensity (MFI) <3000 was achieved with both MP and CP. At 2 h of apheresis, CP reduced MFI by 2-3.5 fold and MP reduced MFI by 1.7-2.5 fold. Both demonstrated similar hemolytic and thrombotic profiles. CONCLUSIONS: In this in vitro plasmapheresis model of IT and anti-HLA antibody reduction, both MP and CP incorporated into the EC circuit can be used quickly and effectively to reduce circulating antibodies. While CP may have some greater efficiency, further study is necessary to verify this in vivo.


Assuntos
Transplante de Coração , Hemaglutininas , Humanos , Criança , Antígenos HLA , Plasmaferese , Transplante de Coração/métodos , Rejeição de Enxerto/prevenção & controle
3.
Pediatr Qual Saf ; 8(5): e630, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37780603

RESUMO

Introduction: Failure to recognize and mitigate critical patient deterioration remains a source of serious preventable harm to hospitalized pediatric cardiac patients. Emergency transfers (ETs) occur 10-20 times more often than code events outside the intensive care unit (ICU) and are associated with morbidity and mortality. This quality improvement project aimed to increase days between ETs and code events on an acute care cardiology unit (ACCU) from a baseline median of 17 and 32 days to ≥70 and 90 days within 12 months. Methods: Institutional leaders, cardiology-trained physicians and nurses, and trainees convened, utilizing the Institution for Healthcare Improvement model to achieve the project aims. Interventions implemented focused on improving situational awareness (SA), including a "Must Call List," evening rounds, a visual management board, and daily huddles. Outcome measures included calendar days between ETs and code events in the ACCU. Process measures tracked the utilization of interventions, and cardiac ICU length of stay was a balancing measure. Statistical process control chart methodology was utilized to analyze the impact of interventions. Results: Within the study period, we observed a centerline shift in primary outcome measures with an increase from 17 to 56 days between ETs and 32 to 62 days between code events in the ACCU, with sustained improvement. Intervention utilization ranged from 87% to 100%, and there was no observed special cause variation in our balancing measure. Conclusions: Interventions focused on improving SA in a particularly vulnerable patient population led to sustained improvement with reduced ETs and code events outside the ICU.

4.
JTCVS Open ; 13: 307-319, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37063131

RESUMO

Objective: The prevalence of postoperative cardiac arrest (CA) increases with cardiothoracic surgical case complexity and is associated with a 40% to 50% mortality. Despite having a low overall surgical mortality rate at our center, our postoperative CA rates were higher than expected, with an observed-to-expected ratio of 2.6. Utilizing quality improvement methodology, we evaluated the influence of proactive risk mitigation on postprocedure CA in a high-risk cohort of pediatric cardiac patients. Methods: This single-center study utilized the Institute for Healthcare Improvement model. We created and implemented our Proactive Mitigation to Decrease Serious Adverse Events program in July 2020, prospectively enrolling preidentified high-risk patients. Enrolled patients underwent scheduled multidisciplinary reviews via virtual platform at 2 periprocedural time points with discussion of patient-specific risks and the subsequent development of proactive risk mitigation plans. Primary outcome measures were derived from the Pediatric Cardiac Critical Care Consortium national registry and included rate of postprocedure CA within 7 days and an institution-specific observed-to-expected ratio for postoperative CA. Results: Our baseline median number of high-risk cases between postprocedure CAs was 3. Following project initiation, median high-risk cases between events increased to 7. Our observed-to-expected ratio for postoperative CA decreased from 2.56 during the 12 months before Proactive Mitigation to Decrease Serious Adverse Events program implementation to 1.01 during the 12 months after Proactive Mitigation to Decrease Serious Adverse Events program implementation, and hospital length of stay decreased by ∼10 days. Conclusions: Implementation of periprocedure-related proactive risk mitigation strategies in high-risk pediatric cardiac patients led to improvement in postprocedure CA with a 133% increase in high-risk cases between events.

5.
J Heart Lung Transplant ; 42(9): 1233-1241, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-37088341

RESUMO

BACKGROUND: The 2016 revision of the US Pediatric Heart Allocation Policy developed stringent rules for priority status creating impetus for clinicians to seek status exceptions. We hypothesized there may be differential status exceptions based on race and socioeconomic status (SES) contributing to disparities in waitlist outcomes. METHODS: The Scientific Registry for Transplant Recipients was queried for children listed for heart transplant from 2012 to 2020. Waitlist status & mortality with regards to race and neighborhood SES were stratified by listing before (Era 1) or after (Era 2) the policy change. RESULTS: The use of both 1A and 1B exceptions (E) increased in Era 2. In Era 1, there was no association between patient race or neighborhood SES on use of 1A(E) or 1B(E) when controlling for age and diagnosis. In Era 2, neither race nor neighborhood SES were associated with 1A(E), but both were associated with 1B(E): non-Hispanic (NH) Black children and those from low- and middle-SES neighborhoods were significantly less likely to be listed 1B(E). In Era 1, there were no significant differences in waitlist mortality based on race at any waitlist status; in Era 2, NH Black children had higher waitlist mortality when initially listed 1B or 2. CONCLUSIONS: Since the 2016 policy change, racial disparities in waitlist mortality have worsened among children initially listed with lower priority status. Unequal use of 1B exceptions, which lower waitlist mortality, may explain some of these disparities. Recently implemented standardized pediatric exception guidance has the potential to improve equity.


Assuntos
Transplante de Coração , Obtenção de Tecidos e Órgãos , Humanos , Criança , Disparidades Socioeconômicas em Saúde , Etnicidade , Políticas , Listas de Espera , Estudos Retrospectivos
6.
Circ Heart Fail ; 16(2): e010123, 2023 02.
Artigo em Inglês | MEDLINE | ID: mdl-36786204

RESUMO

Individuals with Fontan circulation are at risk of late mortality from both cardiac and noncardiac causes. Despite the known risk of mortality, referral indications for advanced heart failure care vary between centers, and many individuals die from Fontan circulation-related complications either after late consideration for advanced heart failure therapies or having never seen a heart failure specialist. There is a critical need for guidelines to direct appropriately timed referral for advanced heart failure consultation. The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) Fontan Committee has developed recommended thresholds for advanced heart failure referral to guide primary cardiologists. These recommendations are divided into 4 categories of clinical Fontan circulatory dysfunction including (1) cardiac/systemic ventricular dysfunction, (2) Fontan pathway dysfunction, (3) lymphatic dysfunction, and (4) extracardiac dysfunction.


Assuntos
Técnica de Fontan , Cardiopatias Congênitas , Insuficiência Cardíaca , Disfunção Ventricular , Humanos , Cardiopatias Congênitas/cirurgia , Disfunção Ventricular/complicações , Ventrículos do Coração
8.
Pediatr Transplant ; 27(3): e14412, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36329630

RESUMO

BACKGROUND: The US Pediatric Heart Allocation Policy (PHAP) was revised in March 2016, with the goal of reducing waitlist mortality. We evaluated the hypothesis that these changes, which increased status exceptions, have worsened racial disparities in waitlist outcomes. METHODS: Children in the Pediatric Heart Transplant Study database listed for first heart transplant from January 2012 - June 2020 were included and stratified by listing before (Era 1) or after (Era 2) the PHAP revision. RESULTS: A total of 4,089 children were listed during the study period. Compared with white children (n = 2648), non-white children (n = 1441) were more likely to have an underlying diagnosis of cardiomyopathy in both eras. Waitlist mortality was similar in white and non-white children in Era 1, but comparatively worse for non-white children in Era 2. In multivariable analysis controlling for diagnosis, age, and severity markers, non-white children had a significantly higher waitlist mortality only in Era 2 (Era 1: sHR 1.22 [95%CI 0.90 - 1.66] vs. Era 2: sHR 1.57 [95%CI 1.17 - 2.10]). CONCLUSIONS: Widening racial disparities in waitlist mortality may be an unintended consequence of the 2016 PHAP revision. Additional analyses may inform the degree to which this policy vs. unrelated changes in care differentially contribute to these disparities.


Assuntos
Cardiomiopatias , Transplante de Coração , Humanos , Criança , Listas de Espera , Políticas , Estudos Retrospectivos
9.
Am J Perinatol ; 40(14): 1495-1508, 2023 10.
Artigo em Inglês | MEDLINE | ID: mdl-34852367

RESUMO

Persistent pulmonary hypertension of the newborn, or PPHN, represents a challenging condition associated with high morbidity and mortality. Management is complicated by complex pathophysiology and limited neonatal specific evidence-based literature, leading to a lack of universal contemporary clinical guidelines for the care of these patients. To address this need and to provide consistent high-quality clinical care for this challenging population in our neonatal intensive care unit, we sought to develop a comprehensive clinical guideline for the acute stabilization and management of neonates with PPHN. Utilizing cross-disciplinary expertise and incorporating an extensive literature search to guide best practice, we present an approachable, pragmatic, and clinically relevant guide for the bedside management of acute PPHN. KEY POINTS: · PPHN is associated with several unique diagnoses; the associated pathophysiology is different for each unique diagnosis.. · PPHN is a challenging, dynamic, and labile process for which optimal care requires frequent reassessment.. · Key management goals are adequate tissue oxygen delivery, avoiding harm..


Assuntos
Hipertensão Pulmonar , Síndrome da Persistência do Padrão de Circulação Fetal , Recém-Nascido , Humanos , Síndrome da Persistência do Padrão de Circulação Fetal/diagnóstico , Síndrome da Persistência do Padrão de Circulação Fetal/terapia , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/terapia , Unidades de Terapia Intensiva Neonatal
10.
Front Physiol ; 13: 958734, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36160862

RESUMO

Pulmonary arterial hypertension (PAH) is a complex disease involving increased resistance in the pulmonary arteries and subsequent right ventricular (RV) remodeling. Ventricular-arterial interactions are fundamental to PAH pathophysiology but are rarely captured in computational models. It is important to identify metrics that capture and quantify these interactions to inform our understanding of this disease as well as potentially facilitate patient stratification. Towards this end, we developed and calibrated two multi-scale high-resolution closed-loop computational models using open-source software: a high-resolution arterial model implemented using CRIMSON, and a high-resolution ventricular model implemented using FEniCS. Models were constructed with clinical data including non-invasive imaging and invasive hemodynamic measurements from a cohort of pediatric PAH patients. A contribution of this work is the discussion of inconsistencies in anatomical and hemodynamic data routinely acquired in PAH patients. We proposed and implemented strategies to mitigate these inconsistencies, and subsequently use this data to inform and calibrate computational models of the ventricles and large arteries. Computational models based on adjusted clinical data were calibrated until the simulated results for the high-resolution arterial models matched within 10% of adjusted data consisting of pressure and flow, whereas the high-resolution ventricular models were calibrated until simulation results matched adjusted data of volume and pressure waveforms within 10%. A statistical analysis was performed to correlate numerous data-derived and model-derived metrics with clinically assessed disease severity. Several model-derived metrics were strongly correlated with clinically assessed disease severity, suggesting that computational models may aid in assessing PAH severity.

11.
Pediatr Transplant ; 26(7): e14338, 2022 11.
Artigo em Inglês | MEDLINE | ID: mdl-35768886

RESUMO

BACKGROUND: Management of infants with pulmonary atresia/intact ventricular septum (PA/IVS) is variable. Because of higher mortality in more severe forms, heart transplant (HT) is an acceptable approach, but waitlist and post-transplant outcomes are unclear. This study compared outcomes of infants with PA/IVS vs. other single ventricle (SV) anatomies listed for HT. METHODS: Data from the Pediatric Heart Transplant Society (1993-2018) were analyzed for survival and risk factors for mortality. RESULTS: Of 1617 SV infants, 300 had PA/IVS (19%) and 1317 had other SV (81%). Overall, 1-, 5-, and 10-year survival was higher among PA/IVS (74%, 65%, 61%) versus other SV infants (62%, 54%, 50%, p = .004). While waitlist mortality was similar between groups (p = .09), PA/IVS was an independent predictor of improved waitlist survival (HR 0.68, p = .03), and PA/IVS infants had higher incidence of waitlist removal (8% vs. 5.5%, p = .03), most commonly for being "too well." Post-transplant survival was superior among PA/IVS versus other SV infants (1- and 5-year survival 93% and 81% vs. 80% and 71%, p < .0001). Risk factors for PA/IVS waitlist mortality (2008-2018) included extracorporeal membrane oxygenation and mechanical ventilation. Prior aortopulmonary (AP) shunt among PA/IVS infants was associated with improved waitlist survival. CONCLUSIONS: Overall survival among PA/IVS infants listed for HT exceeds that of other SV infants with PA/IVS identified as an independent predictor of improved waitlist and post-transplant survival. Prior AP shunt among listed PA/IVS infants was associated with improved waitlist outcomes, though, which may reflect a listing selection bias.


Assuntos
Cardiopatias Congênitas , Transplante de Coração , Atresia Pulmonar , Septo Interventricular , Criança , Humanos , Lactente , Atresia Pulmonar/cirurgia , Estudos Retrospectivos , Resultado do Tratamento
12.
Pediatr Cardiol ; 43(6): 1379-1382, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-35247057

RESUMO

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.


Assuntos
Oxigenação por Membrana Extracorpórea , Insuficiência Cardíaca , Coração Auxiliar , Acidente Vascular Cerebral , Criança , Insuficiência Cardíaca/etiologia , Coração Auxiliar/efeitos adversos , Humanos , Sistema de Registros , Estudos Retrospectivos , Acidente Vascular Cerebral/epidemiologia , Acidente Vascular Cerebral/etiologia , Acidente Vascular Cerebral/prevenção & controle , Resultado do Tratamento
13.
Pediatr Qual Saf ; 6(6): e493, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34934877

RESUMO

Congenital heart disease (CHD), the most common congenital malformation, often requires surgical correction. As surgical mortality rates are low, a common quality marker linked with surgical outcomes is hospital length of stay (LOS). Reduced LOS is associated with better long-term outcomes, reduced hospital-acquired complications, and improved patient-family satisfaction. This project aimed to reduce aggregate median postoperative LOS for four CHD lesions from a baseline of 6.2 days by 10%. METHODS: This single-center study utilized the Institute for Healthcare Improvement model to achieve the project aim. A diuretic wean protocol implemented in April 2018 entailed weaning to a homegoing diuretic regimen upon transfer from the cardiac intensive care unit to the inpatient step-down unit. A discharge milestone checklist implemented in September 2018 contained milestones necessary for discharge and an anticipated date of discharge. Outcome measures included aggregate median postoperative LOS and ∆LOS. Balancing measures included cardiac intensive care unit bounce back, pleural chest tube replacement, and readmission rates. RESULTS: Our baseline aggregate median postoperative LOS for the lesions studied was 6.2 days. Following diuretic protocol implementation, the aggregate median LOS decreased to 4.4 days. Baseline ∆LOS decreased from 5.5 to 0.42 days. Postoperative cost fell by an average of $11,874. Balancing measures demonstrated no unintended consequences. CONCLUSIONS: Implementation of a diuretic wean protocol led to sustained improvement in postoperative LOS, and ∆LOS in a subset of CHD patients with no unintended consequences supporting that standardization of postoperative care is effective for improvement efforts and can reduce overall practice variation.

14.
J Pediatr ; 232: 257-263, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33301784

RESUMO

OBJECTIVE: To develop a diagnostic error index (DEI) aimed at providing a practical method to identify and measure serious diagnostic errors. STUDY DESIGN: A quality improvement (QI) study at a quaternary pediatric medical center. Five well-defined domains identified cases of potential diagnostic errors. Identified cases underwent an adjudication process by a multidisciplinary QI team to determine if a diagnostic error occurred. Confirmed diagnostic errors were then aggregated on the DEI. The primary outcome measure was the number of monthly diagnostic errors. RESULTS: From January 2017 through June 2019, 105 cases of diagnostic error were identified. Morbidity and mortality conferences, institutional root cause analyses, and an abdominal pain trigger tool were the most frequent domains for detecting diagnostic errors. Appendicitis, fractures, and nonaccidental trauma were the 3 most common diagnoses that were missed or had delayed identification. CONCLUSIONS: A QI initiative successfully created a pragmatic approach to identify and measure diagnostic errors by utilizing a DEI. The DEI established a framework to help guide future initiatives to reduce diagnostic errors.


Assuntos
Erros de Diagnóstico/prevenção & controle , Hospitais Pediátricos/normas , Melhoria de Qualidade/organização & administração , Indicadores de Qualidade em Assistência à Saúde/estatística & dados numéricos , Diagnóstico Tardio/prevenção & controle , Diagnóstico Tardio/estatística & dados numéricos , Erros de Diagnóstico/estatística & dados numéricos , Hospitais Pediátricos/estatística & dados numéricos , Humanos , Ohio , Melhoria de Qualidade/estatística & dados numéricos , Indicadores de Qualidade em Assistência à Saúde/normas , Estudos Retrospectivos
15.
ASAIO J ; 66(4): 441-446, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32224822

RESUMO

Improving the outcomes of pediatric patients with congenital heart disease with end-stage heart failure depends on the collaboration of all stakeholders; this includes providers, patients and families, and industry representatives. Because of the rarity of this condition and the heterogeneity of heart failure etiologies that occur at pediatric centers, learnings must be shared between institutions and all disciplines to move the field forward. To foster collaboration, excel discovery, and bring data to the bedside, a new, collaborative quality improvement science network-ACTION (Advanced Cardiac Therapies Improving Outcomes Network)-was developed to meet the needs of the field. Existing gaps in care and the methods of improvement that will be used are described, along with the mission and vision, utility of real-world data for regulatory purposes, and the organizational structure of ACTION is described.


Assuntos
Atenção à Saúde/organização & administração , Colaboração Intersetorial , Aprendizagem , Melhoria de Qualidade , Criança , Humanos
16.
Pediatr Transplant ; 22(5): e13216, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29774622

RESUMO

Pediatric patients awaiting heart transplant face high mortality rates due to donor organ shortages, including non-use of marginal donor hearts. We examined national trends in pediatric marginal donor heart use over time. UNOS data were queried for heart donors <18 years from 2005 to 2014. The proportion of donor hearts considered marginal was determined using previously cited marginal characteristics: left ventricular ejection fraction (LVEF) <50%, use of ≥2 inotropes, cerebrovascular death, CDC high-risk status, and eGFR < 30 mL/min/1.73 m2 . Disposition of donor hearts was determined and stratified by marginal donor status. Of 6778 pediatric hearts offered from 2005 to 2014, 2373 (35.0%) were considered marginal. Non-use of marginal donor hearts was significantly higher than that of donor hearts without any marginal characteristics (59.5% vs 20.3%, P < .001). In particular, LVEF < 50% and donor inotropes were associated with high rates of organ non-use among pediatric donors. Yet, non-use of marginal donor organs decreased from 67% to 48% from 2005 to 2014 (P < .001). Although the proportion of pediatric donor hearts used for pediatric patients has increased, more than half of donor hearts are declined for use in pediatric recipients due, in part, to perceived marginal status.


Assuntos
Seleção do Doador/tendências , Transplante de Coração , Doadores de Tecidos/provisão & distribuição , Adolescente , Criança , Pré-Escolar , Seleção do Doador/normas , Seleção do Doador/estatística & dados numéricos , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Sistema de Registros , Estudos Retrospectivos , Estados Unidos
17.
Eur J Pediatr Surg ; 28(6): 508-514, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-29036736

RESUMO

PURPOSE: The relationship between pulmonary hypoplasia and pulmonary arterial hypertension (PHTN) in patients with congenital diaphragmatic hernia (CDH) remains ill-defined. We hypothesized that prenatal estimates of lung size would directly correlate with PHTN severity. METHODS: Infants with isolated CDH (born 2004-2015) at a single institution were included. Estimates of lung size included observed-to-expected LHR (o:eLHR) and %-predicted lung volumes (PPLV = observed/predicted volumes). The primary outcome was severity of PHTN (grade 0-3) on echocardiography performed between day of life 3 and 30. RESULTS: Among 62 patients included, there was 32% mortality and 65% ECMO utilization. PPLV (odds ratio [OR] = 0.94 per 1 grade in PHTN severity, 95% confidence interval [CI] = 0.89-0.98, p < 0.01) and o:eLHR (OR = 0.97, 95% CI = 0.94-0.99, p < 0.01) were significantly associated with PHTN grade. Among patients on ECMO, PPLV (OR = 0.92, 95% CI = 0.84-0.99, p = 0.03) and o:eLHR (OR = 0.95, 95% CI = 0.92-0.99, p = 0.01) were more strongly associated with PHTN grade. PPLV and o:eLHR were significantly associated with the use of inhaled nitric oxide (iNO) (OR = 0.90, 95% CI = 0.83-0.98, p = 0.01 and OR = 0.94, 95% CI = 0.91-0.98, p < 0.01, respectively) and epoprostenol (OR = 0.91, 95% CI = 0.84-0.99, p = 0.02 and OR = 0.93, 95% CI = 0.89-0.98, p < 0.01, respectively). CONCLUSION: Among infants with isolated CDH, PPLV, and o:eLHR were significantly associated with PHTN severity, especially among patients requiring ECMO. Prenatal lung size may help predict postnatal PHTN and associated therapies.


Assuntos
Hérnias Diafragmáticas Congênitas/complicações , Hipertensão Pulmonar/etiologia , Pneumopatias/complicações , Pulmão/anormalidades , Índice de Gravidade de Doença , Anormalidades Múltiplas/diagnóstico por imagem , Ecocardiografia , Feminino , Seguimentos , Hérnias Diafragmáticas Congênitas/diagnóstico por imagem , Humanos , Hipertensão Pulmonar/diagnóstico , Recém-Nascido , Modelos Logísticos , Pulmão/diagnóstico por imagem , Pneumopatias/diagnóstico por imagem , Masculino , Gravidez , Estudos Retrospectivos , Ultrassonografia Pré-Natal
18.
Eur Heart J ; 38(38): 2900-2909, 2017 Oct 07.
Artigo em Inglês | MEDLINE | ID: mdl-29019615

RESUMO

AIMS: To evaluate associations between haemodynamic profiles and symptoms, end-organ function and outcome in children listed for heart transplantation. METHODS AND RESULTS: Children <18 years listed for heart transplant between 1993 and 2013 with cardiac catheterization data [pulmonary capillary wedge pressure (PCWP), right atrial pressure (RAP), and cardiac index (CI)] in the Pediatric Heart Transplant Study database were included. Outcomes were New York Heart Association (NYHA)/Ross classification, renal and hepatic dysfunction, and death or clinical deterioration while on waitlist. Among 1059 children analysed, median age was 6.9 years and 46% had dilated cardiomyopathy. Overall, 58% had congestion (PCWP >15 mmHg), 28% had severe congestion (PCWP >22 mmHg), and 22% low cardiac output (CI < 2.2 L/min/m2). Twenty-one per cent met the primary outcome of death (9%) or clinical deterioration (12%). In multivariable analysis, worse NYHA/Ross classification was associated with increased PCWP [odds ratio (OR) 1.03, 95% confidence interval (95% CI) 1.01-1.07, P = 0.01], renal dysfunction with increased RAP (OR 1.04, 95% CI 1.01-1.08, P = 0.007), and hepatic dysfunction with both increased PCWP (OR 1.03, 95% CI 1.01-1.06, P < 0.001) and increased RAP (OR 1.09, 95% CI 1.06-1.12, P < 0.001). There were no associations with low output. Death or clinical deterioration was associated with severe congestion (OR 1.6, 95% CI 1.2-2.2, P = 0.002), but not with CI alone. However, children with both low output and severe congestion were at highest risk (OR 1.9, 95% CI 1.1-3.5, P = 0.03). CONCLUSION: Congestion is more common than low cardiac output in children with end-stage heart failure and correlates with NYHA/Ross classification and end-organ dysfunction. Children with both congestion and low output have the highest risk of death or clinical deterioration.


Assuntos
Insuficiência Cardíaca/fisiopatologia , Hemodinâmica/fisiologia , Adolescente , Baixo Débito Cardíaco/mortalidade , Baixo Débito Cardíaco/fisiopatologia , Cardiomiopatias/complicações , Cardiomiopatias/mortalidade , Cardiomiopatias/fisiopatologia , Criança , Pré-Escolar , Doença Crônica , Deterioração Clínica , Feminino , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/mortalidade , Ventrículos do Coração/anormalidades , Humanos , Lactente , Recém-Nascido , Masculino
19.
Pediatr Transplant ; 21(8)2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28913983

RESUMO

Prolonged graft ischemia may be a risk factor for early rejection post-HTx, but this has not been well studied in children. Furthermore, factors moderating the association between IT and early rejection have not been investigated. From 2004 to 2012, pediatric HTx recipients (n = 2381) were identified from the UNOS database. A ROC curve determined the optimal IT discriminating patients by the presence of early rejection. Separate univariate analyses identified factors associated with: (i) early (prior to hospital discharge) rejection, and (ii) IT. A multivariable logistic regression assessed independent risk factors for early rejection. We included interaction terms to evaluate whether IT's independent risk effect on early rejection is moderated via interaction with associated factors found in univariate analysis. Longer IT was associated with an increased risk of early rejection. In multivariable analysis, IT > 3.1 hours was an independent risk factor for early rejection (AOR 1.44, P = .01). No interaction terms between IT and any associated factors were significant. Longer IT is an independent risk for early rejection in pediatric HTx recipients. Better understanding the association between IT and early rejection may identify interventions to mitigate this risk.


Assuntos
Rejeição de Enxerto/etiologia , Transplante de Coração/métodos , Isquemia/complicações , Adolescente , Criança , Pré-Escolar , Bases de Dados Factuais , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Modelos Logísticos , Masculino , Curva ROC , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo
20.
J Pediatr ; 188: 82-86, 2017 09.
Artigo em Inglês | MEDLINE | ID: mdl-28690004

RESUMO

OBJECTIVE: To describe the change in health insurance after heart transplantation among adolescents, and characterize the implications of this change for long-term transplant outcomes. STUDY DESIGN: Patients age 15-18 years receiving first-time heart transplantation between 1999 and 2011 were identified in the United Network for Organ Sharing registry and included in the analysis if they survived at least 5 years. The primary exposure was change or continuity of health insurance coverage between the time of transplant and the 5-year follow-up. Cox proportional hazards models were used to determine the association between insurance status change and long-term (>5 years) patient and graft survival. RESULTS: The analysis included 366 patients (age 16 ± 1 years at transplant), of whom 205 (56%) had continuous private insurance; 96 (26%) had continuous public insurance; and 65 (18%) had a change in insurance status. In stepwise multivariable Cox regression, change in insurance status was associated with greater mortality hazard, compared with continuous private insurance (hazard ratio = 1.9; 95% CI: 1.1, 3.2; P = .016), whereas long-term patient and graft survival did not differ between patients with continuous public and continuous private insurance. CONCLUSIONS: Continuity of insurance coverage is associated with improved long-term clinical outcomes among adolescent heart transplant recipients who survive into adulthood.


Assuntos
Transplante de Coração/economia , Cobertura do Seguro/estatística & dados numéricos , Seguro Saúde/estatística & dados numéricos , Adolescente , Criança , Feminino , Seguimentos , Transplante de Coração/estatística & dados numéricos , Humanos , Masculino , Modelos de Riscos Proporcionais , Sistema de Registros , Análise de Sobrevida , Sobreviventes , Adulto Jovem
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