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1.
Artigo em Inglês | MEDLINE | ID: mdl-38945282

RESUMO

BACKGROUND: In 2016, we initiated a quality improvement endeavor to increase pediatric heart offer acceptance. This study assessed the effect of these interventions at our center. METHODS: We evaluted pre- and postimplementation cohorts (January 1, 2008-December 31, 2016 vs January 1, 2017-July 1, 2023) comparing donor heart utilization. Six interventions were iterated over time to increase offer acceptance ("extended criteria"): ABO-incompatible transplant, ex vivo perfusion for distanced donors, 3-dimensional total cardiac volume (TCV) assessment, acceptance of hepatitis-C or Severe Acute Respiratory Syndrome Coronavirus 2 infected donors, and institutional culture change favoring consideration of donors previously considered unacceptable. Outcomes studied included annual HT volume, median waitlist duration, sequence number at acceptance, and post-transplant clinical outcomes. RESULTS: During the study period, annual transplant volume increased from 16/year to 25/year pre- and postimplementation. Three hundred thirteen of 389 (80%) listed patients were transplanted. Waitlist duration shortened postimplementation (p = 0.01), as did the percentage of accepted heart offers utilizing at least 1 extended criterion (p < 0.001). Institutional culture change and TCV assessment had the largest impact on donor heart utilization (p = 0.04 and p < 0.001). There was no difference in post-HT intubation or intensive care unit days (p = 0.05-0.9), though post-transplant hospitalization duration (p < 0.001) increased. Post-transplant survival was unaffected by the use of extended criteria hearts (p = 0.3). CONCLUSIONS: We report a successful longitudinal, multifaceted effort to increase organ offer utilization, with institutional culture change and TCV assessments most impactful. The use of extended criteria hearts was not associated with inferior survival.

3.
ASAIO J ; 2024 Jun 14.
Artigo em Inglês | MEDLINE | ID: mdl-38870474

RESUMO

Patients supported on ventricular assist devices (VADs) benefit from rehabilitation while awaiting heart transplantation to recover from surgery, prevent deconditioning, and, in most cases, optimize transplant candidacy. With bleeding and neurological dysfunction as the most common VAD complications, the importance of rehabilitation dramatically increases when a patient on a VAD also suffers from a neurological injury. The rehabilitation needs for cardiac conditioning and neurological reeducation are not the same. Patients with severe neurological deficits require intense rehabilitation that often includes base-of-support challenges, usage of bolsters and balls, partial weight-bearing treadmill training, and assumption of various body positions in prone, kneeling, or quadruped for neuromotor reeducation. However, some devices are more conducive to rehabilitation than others. For children supported by the CentriMag in particular, rehabilitation is challenged by short cannula tubing, an external motor, a large interface, and an intensive care unit (ICU) admission. We report a safe and successful physical therapy course of a pediatric stroke patient with a diagnosis of Ebstein's anomaly supported by a CentriMag right VAD (RVAD) while awaiting heart transplant in the ICU.

5.
J Pediatr Intensive Care ; 13(1): 37-45, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38571984

RESUMO

Acute kidney injury (AKI) is common after pediatric heart transplantation (HT) and is associated with inferior patient outcomes. Hemodynamic risk factors for pediatric heart transplant recipients who experience AKI are not well described. We performed a retrospective review of 99 pediatric heart transplant patients at Lucile Packard Children's Hospital Stanford from January 1, 2015, to December 31, 2019, in which clinical and demographic characteristics, intraoperative perfusion data, and hemodynamic measurements in the first 48 postoperative hours were analyzed as risk factors for severe AKI (Kidney Disease: Improving Global Outcomes [KDIGO] stage ≥ 2). Univariate analysis was conducted using Fisher's exact test, Chi-square test, and the Wilcoxon rank-sum test, as appropriate. Multivariable analysis was conducted using logistic regression. Thirty-five patients (35%) experienced severe AKI which was associated with lower intraoperative cardiac index ( p = 0.001), higher hematocrit ( p < 0.001), lower body temperature ( p < 0.001), lower renal near-infrared spectroscopy ( p = 0.001), lower postoperative mean arterial blood pressure (MAP: p = 0.001), and higher central venous pressure (CVP; p < 0.001). In multivariable analysis, postoperative CVP >12 mm Hg (odds ratio [OR] = 4.27; 95% confidence interval [CI]: 1.48-12.3, p = 0.007) and MAP <65 mm Hg (OR = 4.9; 95% CI: 1.07-22.5, p = 0.04) were associated with early severe AKI. Children with severe AKI experienced longer ventilator, intensive care, and posttransplant hospital days and inferior survival ( p = 0.01). Lower MAP and higher CVP are associated with severe AKI in pediatric HT recipients. Patients, who experienced AKI, experienced increased intensive care unit (ICU) morbidity and inferior survival. These data may guide the development of perioperative renal protective management strategies to reduce AKI incidence and improve patient outcomes.

6.
Pediatr Transplant ; 28(2): e14695, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38433565

RESUMO

BACKGROUND: Disparities in pediatric heart transplant outcomes based on socioeconomic status (SES) have been previously observed. However, there is a need to reevaluate these associations in contemporary settings with advancements in transplant therapies and increased awareness of health disparities. This retrospective study aims to investigate the relationship between SES and outcomes for pediatric heart transplant patients. METHODS: Data were collected through a chart review of 176 pediatric patients who underwent first orthotopic heart transplantation (OHT) at a single center from 2013 to 2021. The Area Deprivation Index (ADI), a composite score based on U.S. census data, was used to quantify SES. Cox proportional hazards models and generalized linear models were employed to analyze the association between SES and graft failure, rejection rates, and hospitalization rates. RESULTS: The analysis revealed no statistically significant differences in graft failure rates, rejection rates, or hospitalization rates between low-SES and high-SES pediatric heart transplant patients for our single-center study. CONCLUSION: There may be patient education, policies, and social resources that can help mitigate SES-based healthcare disparities. Additional multi-center research is needed to identify post-transplant care that promotes patient equity.


Assuntos
Transplante de Coração , Humanos , Criança , Estudos Retrospectivos , Classe Social , Disparidades em Assistência à Saúde , Hospitalização
7.
Pediatr Transplant ; 28(2): e14720, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38433570

RESUMO

BACKGROUND: There are conflicting data regarding the relationship between center volume and outcomes in pediatric heart transplantation. Previous studies have not fully accounted for differences in case mix, particularly in high-risk congenital heart disease (CHD) groups. We aimed to evaluate the relationship between center volume and outcomes using the Pediatric Heart Transplant Society (PHTS) Registry and explore how case mix may affect outcomes. METHODS: A retrospective cohort study of all pediatric patients in the PHTS Registry who received a heart transplant from 2009 to 2018 was performed. Centers were divided into 5 groups based on average yearly transplant volume. The primary outcome was time to death or graft loss and outcomes were compared using Kaplan-Meier analysis. RESULTS: There were 4583 cases among 55 centers included. There was no difference in time to death or graft loss by center volume in the entire cohort (p = .75), in patients with CHD (p = .79) or in patients with cardiomyopathy (p = .23). There was also no difference in time to death or graft loss by center size in patients undergoing transplant after Norwood, Glenn or Fontan (log rank p = .17, p = .31, and p = .10 respectively). There was a statistically significant difference in outcomes by center size in the positive crossmatch group (p < .0001), though no discernible pattern related to high or low center volume. CONCLUSIONS: Outcomes are similar among transplant centers of all sizes, including for high-risk patient groups with CHD. Future work is needed to understand how patient-specific risk factors may vary among centers of various sizes and whether this influences patient outcomes.


Assuntos
Transplante de Coração , Transplantes , Humanos , Criança , Estudos Retrospectivos , Estimativa de Kaplan-Meier , Sistema de Registros
8.
Pediatr Transplant ; 28(1): e14584, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37470130

RESUMO

BACKGROUND: Pediatric heart transplantation (HT) continues to be limited by the shortage of donor organs, distance constraints, and the number of potential donor offers that are declined due to the presence of multiple risk factors. METHODS: We report a case of successful pediatric HT in which multiple risk factors were mitigated through a combination of innovative donor utilization improvement strategies. RESULTS: An 11-year-old, 25-kilogram child with cardiomyopathy and pulmonary hypertension, on chronic milrinone therapy and anticoagulated with apixaban, was transplanted with a heart from a Hepatitis C virus positive donor and an increased donor-to-recipient weight ratio. Due to extended geographic distance, an extracorporeal heart preservation system (TransMedics™ OCS Heart) was used for procurement. No significant bleeding was observed post-operatively, and she was discharged by post-operative day 15 with normal biventricular systolic function. Post-transplant Hepatitis C virus seroconversion was successfully treated. CONCLUSIONS: Heart transplantation in donors with multiple risk factor can be achieved with an integrative team approach and should be taken into consideration when evaluating marginal donors in order to expand the current limited donor pool in pediatric patients.


Assuntos
Transplante de Coração , Obtenção de Tecidos e Órgãos , Feminino , Humanos , Criança , Doadores de Tecidos , Coração , Fatores de Risco
10.
Pediatr Nephrol ; 39(4): 1289-1300, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-37971519

RESUMO

BACKGROUND: Continuous-flow ventricular assist devices (CF-VADs) are used increasingly in pediatric end-stage heart failure (ESHF) patients. Alongside common risk factors like oxidant injury from hemolysis, non-pulsatile flow constitutes a unique circulatory stress on kidneys. Post-implantation recovery after acute kidney injury (AKI) is commonly reported, but long-term kidney outcomes or factors implicated in the evolution of chronic kidney disease (CKD) with prolonged CF-VAD support are unknown. METHODS: We studied ESHF patients supported > 90 days on CF-VAD from 2008 to 2018. The primary outcome was CKD (per Kidney Disease Improving Global Outcomes (KDIGO) criteria). Secondary outcomes included AKI incidence post-implantation and CKD evolution in the 6-12 months of CF-VAD support. RESULTS: We enrolled 134 patients; 84/134 (63%) were male, median age was 13 [IQR 9.9, 15.9] years, 72/134 (54%) had preexisting CKD at implantation, and 85/134 (63%) had AKI. At 3 months, of the 91/134 (68%) still on a CF-VAD, 34/91 (37%) never had CKD, 13/91 (14%) developed de novo CKD, while CKD persisted or worsened in 49% (44/91). Etiology of heart failure, extracorporeal membrane oxygenation use, duration of CF-VAD, AKI history, and kidney replacement therapy were not associated with different CKD outcomes. Mortality was higher in those with AKI or preexisting CKD. CONCLUSIONS: In the first multicenter study to focus on kidney outcomes for pediatric long-term CF-VAD patients, preimplantation CKD and peri-implantation AKI were common. Both de novo CKD and worsening CKD can happen on prolonged CF-VAD support. Proactive kidney function monitoring and targeted follow-up are important to optimize outcomes.


Assuntos
Injúria Renal Aguda , Insuficiência Cardíaca , Coração Auxiliar , Insuficiência Renal Crônica , Criança , Humanos , Masculino , Adolescente , Feminino , Coração Auxiliar/efeitos adversos , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/terapia , Injúria Renal Aguda/epidemiologia , Injúria Renal Aguda/etiologia , Injúria Renal Aguda/terapia , Insuficiência Renal Crônica/terapia , Insuficiência Renal Crônica/complicações , Rim , Estudos Retrospectivos , Resultado do Tratamento
11.
Pediatr Transplant ; 28(1): e14635, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37957127

RESUMO

PURPOSE: Although waitlist mortality is unacceptably high, nearly half of donor heart offers are rejected by pediatric heart transplant centers. The Advanced Cardiac Therapy Improving Outcome Network (ACTION) and Pediatric Heart Transplant Society (PHTS) convened a multi-institutional donor decision discussion forum (DDDF) aimed at assessing donor acceptance practices and reducing practice variation. METHODS: A 1-h-long virtual DDDF for providers across North America, the United Kingdom, and Brazil was held monthly. Each session typically included two case presentations posing a real-world donor decision challenge. Attendees were polled before the presenting center's decision was revealed. Group discussion followed, including a review of relevant literature and PHTS data. Metrics of participation, participant agreement with presenting center decisions, and impact on future decision-making were collected and analyzed. RESULTS: Over 2 years, 41 cases were discussed. Approximately 50 clinicians attended each call. Risk factors influencing decision-making included donor quality (10), size discrepancy (8), and COVID-19 (8). Donor characteristics influenced 63% of decisions, recipient factors 35%. Participants agreed with the decision made by the presenting center only 49% of the time. Post-presentation discussion resulted in 25% of participants changing their original decision. Survey conducted reported that 50% respondents changed their donor acceptance practices. CONCLUSION: DDDF identified significant variation in pediatric donor decision-making among centers. DDDF may be an effective format to reduce practice variation, provide education to decision-makers, and ultimately increase donor utilization.


Assuntos
Transplante de Coração , Doadores de Tecidos , Humanos , Criança , Fatores de Risco , América do Norte , Escolaridade
12.
Pediatr Transplant ; 27(8): e14601, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37706571

RESUMO

BACKGROUND: Tube feeds are used commonly in children listed for heart transplant; however, rates of renourishment and development of feeding disorders are not sufficiently characterized. METHODS: Retrospective review of pediatric heart transplant recipients from January 1, 2014, to January 3, 2021. Demographics, anthropometric, and nutritional data were collected from heart transplant listing through 3 years post-transplant. Renourishment rates, presence of a feeding disorder, and need for a gastric feeding tube were analyzed. Multivariable analysis was conducted to identify risks for poor nutritional outcomes. RESULTS: Of 104 patients, 35 (34%) and 36 (35%) were malnourished at heart transplant listing and transplant, respectively, persisting in 21/91 (23%) 1 year postheart transplant. Forty (38%) received tube feeds at listing, 42 (40%) at heart transplant, and 18/90 (20%) 1 year post-transplant. Rates of feeding disorders fell from 23% at transplantation to 10% 1 year post-transplant. Feeding disorders were associated with younger age at heart transplant (p < .001) and congenital heart disease (p = .03). Forty-six percent of infants required a gastric feeding tube. Renourishment occurred in 20% during listing and was associated with ventricular assist device support (p = .03) and noncalorically dense feeds (p = .03). Malnutrition at transplant was associated with inferior post-transplant survival (6/36 (17%) vs. 2/68 (3%); p = .02). CONCLUSIONS: Malnourishment requiring tube feeds is common in pediatric heart transplant candidates; however, most patients who eventually survive to transplant remain malnourished at time of transplantation and 1 year later. While some children develop feeding disorders, they generally resolve by 1 year post-transplant.


Assuntos
Transtornos da Alimentação e da Ingestão de Alimentos , Cardiopatias Congênitas , Insuficiência Cardíaca , Transplante de Coração , Desnutrição , Lactente , Criança , Humanos , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/cirurgia , Estudos Retrospectivos , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , Desnutrição/complicações , Listas de Espera
13.
Pediatr Transplant ; 27(6): e14565, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-37409513

RESUMO

BACKGROUND: The development of acute kidney injury (AKI) has been associated with worse outcomes in children after heart transplantation. Our study compares the application of a cumulative six-point Kidney Diseases Improving Global Outcomes (KDIGO) AKI scoring system, utilizing both creatinine and urine output criteria that we term as the AKI-6 criteria, to traditional AKI staging as a predictor for clinical and renal outcomes in the pediatric heart transplant recipients. METHODS: We conducted a retrospective single-center chart review on 155 pediatric patients who underwent heart transplantation from May 2014 to December 2021. The primary independent variable was the presence of severe AKI. Severe AKI by KDIGO was defined as Stage ≥2, whereas severe AKI by AKI-6 was defined as cumulative scores ≥4 or Stage 3 AKI based on either KDIGO criterion alone. Primary outcomes included actuarial survival and renal dysfunction by 1-year post-transplant, defined as an estimated glomerular filtration rate <60 mL/min/1.73 m2 . RESULTS: In total, 140 (90%) patients developed AKI; 98 (63%) patients developed severe AKI by KDIGO, and 60 (39%) by AKI-6. Severe AKI by AKI-6 was associated with worse actuarial survival following heart transplantation compared with KDIGO (p = 0.01). Of the 143 patients with 1-year creatinine data, 6 (11%) patients out of 54 with severe AKI by AKI-6 had evidence of renal dysfunction (p = 0.01), compared with 6 (7%) patients out of 88 by KDIGO (p = 0.3). CONCLUSIONS: AKI-6 scoring provides greater prognostic utility for actuarial survival and renal dysfunction by 1-year post-heart transplantation in pediatric patients than traditional KDIGO staging.


Assuntos
Injúria Renal Aguda , Transplante de Coração , Criança , Humanos , Estudos Retrospectivos , Creatinina , Rim , Injúria Renal Aguda/diagnóstico , Injúria Renal Aguda/etiologia , Fatores de Risco
14.
Am J Bioeth ; 23(6): 54-56, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-37220366
15.
Circ Arrhythm Electrophysiol ; 16(6): e011143, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-37254747

RESUMO

BACKGROUND: With the advent of more intensive rhythm monitoring strategies, ventricular arrhythmias (VAs) are increasingly detected in Fontan patients. However, the prognostic implications of VA are poorly understood. We assessed the incidence of VA in Fontan patients and the implications on transplant-free survival. METHODS: Medical records of Fontan patients seen at a single center between 2002 and 2019 were reviewed to identify post-Fontan VA (nonsustained ventricular tachycardia >4 beats or sustained >30 seconds). Patients with preFontan VA were excluded. Hemodynamically unstable VA was defined as malignant VA. The primary outcome was death and heart transplantation. Death with censoring at transplant was a secondary outcome. RESULTS: Of 431 Fontan patients, transplant-free survival was 82% at 15 years post-Fontan with 64 (15%) meeting primary outcome of either death (n=16, 3.7%), at a median 4.6 (0.4-10.2) years post-Fontan, or transplant (n=48, 11%), at a median of 11.1 (5.9-16.2) years post-Fontan. Forty-eight (11%) patients were diagnosed with VA (90% nonsustained ventricular tachycardia, 10% sustained ventricular tachycardia). Malignant VA (n=9, 2.0%) was associated with younger age, worse systolic function, and valvular regurgitation. Risk for VA increased with time from Fontan, 2.4% at 10 years to 19% at 20 years. History of Stage 1 surgery with right ventricular to pulmonary artery conduit and older age at Fontan were significant risk factors for VA. VA was strongly associated with an increased risk of transplant or death (HR, 9.2 [95% CI, 4.5-18.7]; P<0.001), with a transplant-free survival of 48% at 5-year post-VA diagnosis. CONCLUSIONS: Ventricular arrhythmias occurred in 11% of Fontan patients and was highly associated with transplant or death, with a transplant-free survival of <50% at 5-year post-VA diagnosis. Risk factors for VA included older age at Fontan and history of right ventricular to pulmonary artery conduit. A diagnosis of VA in Fontan patients should prompt increased clinical surveillance.


Assuntos
Técnica de Fontan , Cardiopatias Congênitas , Taquicardia Ventricular , Humanos , Técnica de Fontan/efeitos adversos , Estudos Retrospectivos , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/epidemiologia , Arritmias Cardíacas/etiologia , Artéria Pulmonar/cirurgia , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/epidemiologia , Taquicardia Ventricular/etiologia , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/diagnóstico , Resultado do Tratamento
16.
Am Heart J ; 260: 100-112, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-36828201

RESUMO

BACKGROUND: Currently there are no immunosuppression regimens FDA-approved to prevent rejection in pediatric heart transplantation (HT). In recent years, everolimus (EVL) has emerged as a potential alternative to standard tacrolimus (TAC) as the primary immunosuppressant to prevent rejection that may also reduce the risk of cardiac allograft vasculopathy (CAV), chronic kidney disease (CKD) and cytomegalovirus (CMV) infection. However, the 2 regimens have never been compared head-to-head in a randomized trial. The study design and rationale are reviewed in light of the challenges inherent in rare disease research. METHODS: The TEAMMATE trial (IND 127980) is the first multicenter randomized clinical trial (RCT) in pediatric HT. The primary purpose is to evaluate the safety and efficacy of EVL and low-dose TAC (LD-TAC) compared to standard-dose TAC and mycophenolate mofetil (MMF). Children aged <21 years at HT were randomized (1:1 ratio) at 6 months post-HT to either regimen, and followed for 30 months. Children with recurrent rejection, multi-organ transplant recipients, and those with an estimated glomerular filtration rate (eGFR) <30 mL/min/1.73m2 were excluded. The primary efficacy hypothesis is that, compared to TAC/MMF, EVL/LD-TAC is more effective in preventing 3 MATEs: acute cellular rejection (ACR), CKD and CAV. The primary safety hypothesis is that EVL/LD-TAC does not have a higher cumulative burden of 6 MATEs (antibody mediated rejection [AMR], infection, and post-transplant lymphoproliferative disorder [PTLD] in addition to the 3 above). The primary endpoint is the MATE score, a composite, ordinal surrogate endpoint reflecting the frequency and severity of MATEs that is validated against graft loss. The study had a target sample size of 210 patients across 25 sites and is powered to demonstrate superior efficacy of EVL/LD-TAC. Trial enrollment is complete and participant follow-up will be completed in 2023. CONCLUSION: The TEAMMATE trial is the first multicenter RCT in pediatric HT. It is anticipated that the study will provide important information about the safety and efficacy of everolimus vs tacrolimus-based regimens and will provide valuable lessons into the design and conduct of future trials in pediatric HT.


Assuntos
Cardiopatias , Transplante de Coração , Transplante de Rim , Insuficiência Renal Crônica , Humanos , Criança , Tacrolimo/uso terapêutico , Tacrolimo/farmacologia , Everolimo/farmacologia , Ácido Micofenólico/uso terapêutico , Ácido Micofenólico/farmacologia , Transplante de Rim/efeitos adversos , Imunossupressores/uso terapêutico , Imunossupressores/farmacologia , Insuficiência Renal Crônica/etiologia , Cardiopatias/etiologia , Quimioterapia Combinada , Sobrevivência de Enxerto
17.
Pediatr Transplant ; 27(2): e14461, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36593638

RESUMO

BACKGROUND: Elevated pulmonary vascular resistance (PVR) in the setting of left heart failure may contribute to poor outcomes after pediatric heart transplant (HTx), but peri-transplant management is variable. METHODS: We sought to characterize international practice by surveying physicians at pediatric HTx centers. RESULTS: We received 49 complete responses from 39 centers in 16 countries. Most respondents are pediatric cardiologists (90%), practice at centers offering heart (86%) and lung (55%) transplant, and perform pre-HTx acute vasoreactivity testing (AVT, 88%) in patients with elevated PVR. Half (51%) reported defining a PVR cutoff for HTx eligibility as ≤6 WU m2 (56%) post-AVT (84%). The highest post-AVT PVR ever accepted for HTx ranged from 3-14.4 (median 6) WU m2 . To treat elevated pre-transplant PVR, phosphodiesterase type 5 inhibitors are most common (65%) followed by oxygen (31%), nitric oxide (14%), endothelin receptor antagonists (11%), and prostacyclins (6%). Nearly a third (31%) do not routinely use pulmonary vasodilators without implantation of a left ventricular assist device (LVAD). Case scenarios highlight treatment variability: in a restrictive cardiomyopathy scenario, HTx listing with post-transplant vasodilator therapy was favored, whereas in a Shone's complex patient with fixed PVR, LVAD ± pulmonary vasodilators followed by repeat catheterization was most common. Management of dilated cardiomyopathy with reactive PVR was variable. Most continue vasodilator therapy until HTx (16%), PVR normalizes (16%) or ≤6 months. CONCLUSIONS: Management of elevated PVR in children awaiting HTx is heterogenous. Evidence-based guidelines are needed to allow for longitudinal determination of optimal outcomes and standardized care.


Assuntos
Insuficiência Cardíaca , Transplante de Coração , Coração Auxiliar , Hipertensão Pulmonar , Humanos , Criança , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/terapia , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/cirurgia , Resistência Vascular/fisiologia , Vasodilatadores , Resultado do Tratamento , Estudos Retrospectivos
18.
JPEN J Parenter Enteral Nutr ; 47(1): 59-66, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-35932247

RESUMO

BACKGROUND: Single-ventricle patients require a series of surgeries, with the final stage being the Fontan. This form of circulation results in several long-term complications, but the impact and consequences of nutrition status remain unclear. We sought to evaluate the incidence of malnutrition in Fontan patients and the impact on outcomes. METHODS: This study was a retrospective cohort study of children who underwent Fontan surgery between 1997 and 2018. Clinical, demographic, and nutrition data were collected, including weight, height, body mass index (BMI), and their respective z scores (z score for weight-for-age [WAZ], z score for height-for-age [HAZ], and z score for BMI-for-age [BMIZ]) pre-Fontan, at discharge, 6 months, and 1, 5, and 10 years post-Fontan. Malnutrition status was categorized using the American Society for Parenteral and Enteral Nutrition guidelines and the Michigan MTool. Fontan failure was defined as listing for heart transplant or death. RESULTS: Of the 69 patients, moderate-severe malnutrition occurred at any time point in 11% (n = 8) by WAZ, 16% (n = 11) by HAZ, and 6% (n = 4) by BMIZ. Moderate-severe malnutrition persisted in 6.5%-12.9% at 10 years post-Fontan. Compared with the pre-Fontan period, there was no change in these parameters over time. There was no statistically significant difference in Fontan failure between degrees of pre-Fontan malnutrition. CONCLUSION: There is a 6%-16% incidence of moderate-severe malnutrition in Fontan patients. Malnutrition is a condition that remains present in follow-up. There was no association with anthropometric parameters and transplant-free survival. A prospective multi-institutional study is needed to understand the impact of malnutrition on long-term outcomes.


Assuntos
Técnica de Fontan , Cardiopatias Congênitas , Desnutrição , Desnutrição Proteico-Calórica , Criança , Humanos , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , Incidência , Desnutrição/epidemiologia , Desnutrição/etiologia , Estado Nutricional , Estudos Retrospectivos
19.
J Pain Symptom Manage ; 65(3): 216-221, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36417945

RESUMO

CONTEXT: The symptom profile of children dying from cardiac disease, especially heart failure, differs from those with cancer and other non-cardiac conditions. Treatment with vasoactive infusions at home may be a superior therapy for symptom control for these patients, rather than traditional pain and anxiety management with morphine and benzodiazepines. OBJECTIVES: We report our experience using outpatient milrinone in children receiving hospice care for end-stage heart failure. METHODS: Retrospective review of a contemporary cohort of all patients at Lucile Packard Children's Hospital, Stanford who were discharged on intravenous milrinone and hospice care between 2008 and 2021. Clinical data, including cardiac diagnosis, milrinone dose and route of administration, total milrinone days, symptoms reported, rehospitalization rates, concurrent therapies and complications were analyzed. RESULTS: Among 8 patients, median duration of home milrinone infusion was 191 (33, 572) days with the longest support duration 1,054 days. All (100%) patients were also receiving diuretics at the time of death. Five (63%) were receiving no other pain control medications until the active phase of dying. From milrinone initiation to last outpatient assessment, a reduction in the number of patients reporting respiratory discomfort, abdominal pain, weight loss/lack of appetite, and fatigue was observed. Six (75%) died at home. CONCLUSION: We used milrinone with oral diuretics effectively for symptom control in children with heart failure on palliative care. Our experience was that this combination can be used safely in the outpatient setting for long-term use without the addition of opiates, benzodiazepines, or supplemental oxygen in most cases.


Assuntos
Insuficiência Cardíaca , Cuidados Paliativos na Terminalidade da Vida , Humanos , Criança , Milrinona/uso terapêutico , Insuficiência Cardíaca/tratamento farmacológico , Infusões Intravenosas , Dor/tratamento farmacológico , Diuréticos/uso terapêutico
20.
Ann Thorac Surg ; 115(5): 1098-1108, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36402175

RESUMO

BACKGROUND: The Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs), supported by The Society of Thoracic Surgeons, provides detailed information on pediatric patients supported with ventricular assist devices (VADs). METHODS: From September 19, 2012, to December 31, 2021, there were 1355 devices in 1109 patients (<19 years) from 42 North American Hospitals. RESULTS: Cardiomyopathy was the most common underlying cause (59%), followed by congenital heart disease (25%) and myocarditis (9%). Regarding device type, implantable continuous (IC) VADs were most common at 40%, followed by paracorporeal pulsatile (PP; 28%) and paracorporeal continuous (PC; 26%). Baseline demographics differed, with the PC cohort being younger, smaller, more complex (ie, congenital heart disease), and sicker at implantation (P < .0001). At 6 months after VAD implantation, a favorable outcome (transplantation, recovery, or alive on device) was achieved in 84% of patients, which was greatest among those on IC VADs (92%) and least for PC VADs (69%). Adverse events were not uncommon, with nongastrointestinal bleeding (incidence of 14%) and neurologic dysfunction (11% [stroke, 4%]), within 2 weeks after implantation being the most prevalent. Stroke and bleeding had negative impacts on overall survival (P = .002 and P < .001, respectively). CONCLUSIONS: This Sixth Pedimacs Report demonstrates the continued evolution of the pediatric field. The complexity of cardiac physiologies and anatomic constraint mandates the need for multiple types of devices used (PC, PP, IC). Detailed analyses of each device type in this report provide valuable information to further advance the care of this challenging and vulnerable population.


Assuntos
Cardiopatias Congênitas , Insuficiência Cardíaca , Transplante de Coração , Coração Auxiliar , Cirurgiões , Criança , Humanos , Insuficiência Cardíaca/cirurgia , Insuficiência Cardíaca/etiologia , Resultado do Tratamento , Cardiopatias Congênitas/etiologia , Sistema de Registros , Coração Auxiliar/efeitos adversos , Estudos Retrospectivos
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