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We sought to develop and validate a new risk stratification score for mortality for children supported with a ventricular assist device (VAD). This retrospective, multicenter study used data from patients undergoing VAD implantation between April 2018 and February 2023 at 44 participating institutions in the Advanced Cardiac Therapies Improving Outcomes (ACTION) network. Multivariable Cox proportional-hazards modeled mortality after VAD implantation. A total of 1,022 patients were enrolled. The 1 year mortality was 19% (95% confidence interval [CI]: 16-23). The multivariable model was used to build the ACTION VADs risk stratification score with four components: ventilation, advanced organ support (dialysis or ECMO), diagnosis, and size (weight ≤5 kg). One point is added for each risk factor. Based on the sum of the risk factors, patients were classified into four classes: class 0-green (4% mortality at 1 year), class 1-yellow (16% mortality at 1 year), class 2-orange (21% mortality at 1 year), and class 3 or higher-red (42% mortality at 1 year). The score performed well, with area under the curve (AUC) of 0.72 and excellent calibration. The ACTION VADs score for mortality can be calculated easily and offers risk stratification and prognostic information for pediatric VAD candidates. This is the first validated risk assessment tool for pediatric mechanical circulatory support.
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Arrhythmogenic cardiomyopathy (ACM) is an inherited cardiomyopathy associated with fibrofatty tissue replacement of the ventricular tissue. The disease can cause ventricular dysfunction and arrhythmias and can increase the risk of sudden cardiac death. This cardiomyopathy can have variable clinical presentations, especially in the pediatric and young adult populations. In this report, we describe the case of an 18-year-old female with myocarditis as the initial presentation of ACM. She presented following a resuscitated cardiac arrest due to ventricular arrhythmia. On arrival, myocardial edema and delayed gadolinium enhancement were present on cardiac magnetic resonance imaging, with no ventricular changes observed, making the diagnosis consistent with myocarditis. Genetic testing revealed a pathogenic mutation in the desmoplakin gene consistent with ACM. Given the unconventional initial presentation of this patient's disease, early consideration of genetic testing may be beneficial to aid in the early diagnosis and management of ACM in young patients.
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As the number of patients with congenital heart disease (CHD) continues to increase, the burden of heart failure (HF) in this population requires innovative strategies to individualize management. Given the success of implanted invasive hemodynamic monitoring (IHM) with the CardioMEMSTM HF system in adults with acquired HF, this is often suggested for use in patients with CHD, though published data are limited to case reports and case series. Therefore, this review summarizes the available published reports on the use of IHM in patients with complex CHD, describes novel applications, and highlights future directions for study. In patients with CHD, IHM has been used across the lifespan, from age 3 years to adulthood, with minimal device-related complications reported. IHM uses include (1) prevention of HF hospitalizations; (2) reassessment of hemodynamics after titration of medical therapy without repeated cardiac catheterization; (3) serial monitoring of at-risk patients for pulmonary hypertension to optimize timing of heart transplant referral; (4) and hemodynamic assessment with exercise (5) or after ventricular assist device placement. IHM has the potential to reduce the number of cardiac catheterizations in anatomically complex patients and, in patients with Fontan circulation, IHM pressures may have prognostic implications. In conclusion, though further studies are needed, as patients with CHD age and HF is more prevalent, this tool may assist CHD physicians in caring for this complex patient population.
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Cardiopatias Congênitas , Monitorização Hemodinâmica , Humanos , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/terapia , Cardiopatias Congênitas/cirurgia , Monitorização Hemodinâmica/métodos , Hemodinâmica/fisiologia , Insuficiência Cardíaca/fisiopatologia , Insuficiência Cardíaca/terapia , Cateterismo Cardíaco/métodosRESUMO
BACKGROUND: Children awaiting heart transplant (Tx) have a high risk of death due to donor organ scarcity. Historically, ventricular assist devices (VADs) reduced waitlist mortality, prompting increased VAD use. We sought to determine whether the VAD survival benefit persists in the current era. METHODS: Using the Scientific Registry of Transplant Recipients, we identified patients listed for Tx between 3/22/2016 and 9/1/2020. We compared characteristics of VAD and non-VAD groups at Tx listing. Cox proportional hazards models were used to identify risk factors for 1-year waitlist mortality. RESULTS: Among 5054 patients, 764 (15%) had a VAD at Tx listing. The VAD group was older with more mechanical ventilation and renal impairment. Unadjusted waitlist mortality was similar between groups; the curves crossed ~90 days after listing (p = .55). In multivariable analysis, infant age (HR 2.77, 95%CI 2.13-3.60), Black race (HR 1.57, 95%CI 1.31-1.88), congenital heart disease (HR 1.23, 95%CI 1.04-1.46), renal impairment (HR 2.67, 95%CI 2.19-3.26), inotropes (HR 1.28, 95%CI 1.09-1.52), and mechanical ventilation (HR 2.23, 95%CI 1.84-2.70) were associated with 1-year waitlist mortality. VADs were not associated with mortality in the first 90 waitlist days but were protective for those waiting ≥90 days (HR 0.43, 95%CI 0.26-0.71). CONCLUSIONS: In the current era, VADs reduce waitlist mortality, but only for those waitlisted ≥90 days. The differential effect by race, size, and VAD type is less clear. These findings suggest that Tx listing without VAD may be reasonable if a short waitlist time is anticipated, but VADs may benefit those expected to wait >90 days.
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Transplante de Coração , Coração Auxiliar , Sistema de Registros , Listas de Espera , Humanos , Listas de Espera/mortalidade , Masculino , Feminino , Lactente , Criança , Pré-Escolar , Adolescente , Fatores de Risco , Bases de Dados Factuais , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Insuficiência Cardíaca/mortalidade , Insuficiência Cardíaca/cirurgia , Insuficiência Cardíaca/terapia , Estados Unidos/epidemiologiaRESUMO
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.
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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 , TransplantadosRESUMO
BACKGROUND: Pulmonary hypertension (PH) is a common complication in patients with complete dextro-transposition of the great arteries (TGA) after atrial switch (D-TGA/AS) and congenitally corrected TGA (ccTGA). In this population with subaortic right ventricles (sRVs), echocardiography is a poor screening tool for PH; implantable invasive haemodynamic monitoring (IHM) could be used for this purpose, but data are limited. The aim of this study is to report on novel uses of IHM in patients with sRV. METHODS: This retrospective study describes the uses of IHM, impact of IHM on heart failure hospitalisation (HFH) and device-related complications in adults with sRV from a single centre (2015-2022). RESULTS: IHM was placed in 18 patients with sRV (median age 43 (range 30-54) years, 8 female, 16 with D-TGA/AS, 2 with ccTGA); 16 had moderate or severe sRV systolic dysfunction, 13 had PH on catheterisation. IHM was used for (1) Medical therapy titration, (2) Medical management after ventricular assist device in patients with transplant-limiting PH and (3) Serial monitoring of pulmonary artery pressures without repeat catheterisations to help identify the optimal time for heart transplant referral. In follow-up (median 23 months), HFHs/year were similar to the year prior to IHM (median 0 (IQR 0-1.0) before vs 0 (0-0.8) after, p=0.984). Device migration occurred in one, without long-term sequelae. CONCLUSIONS: Uses of IHM in patients with sRV are described which may minimise the need for serial catheterisations in a population where PH is prevalent. HFHs were low overall but not impacted by IHM. One device-related complication occurred without long-term consequence.
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Monitorização Hemodinâmica , Transposição dos Grandes Vasos , Adulto , Humanos , Feminino , Pessoa de Meia-Idade , Estudos Retrospectivos , Ventrículos do Coração , Transposição das Grandes Artérias Corrigida CongenitamenteRESUMO
NEONATE score > 17 has been proposed as a risk factor for interstage mortality/cardiac transplant (IM/T) for patients with single ventricle physiology. Hybrid procedure is assigned 6 points, the highest possible score for that surgical variable. Most centers reserve the hybrid procedure for high-risk patients. Goal of this study was to evaluate the NEONATE score at a center that routinely performs the hybrid procedure. Retrospective chart review of patients undergoing the hybrid procedure was performed (2008-2021). Demographics and variables used for the NEONATE score were collected. Maximization of Youden's J Statistic used to determine cohort-specific optimal threshold for patients undergoing comprehensive Stage II procedure (H-CSII) versus those with IM/T (H-IM/T). Total of 120 patients met inclusion criteria (H-CSII = 105, H-IM/T = 15). Gestational age was median 39 weeks (IQR 38, 39) and birth weight was 3.18 kg (2.91, 3.57). No patient was discharged with opiates or required post-operative extracorporeal circulatory support. Optimal threshold, as selected by maximizing Youden's J Statistic, was 22. Score > 22 had a positive predictive value of 0.33 (95% CI 0.12-0.62), negative predictive values of 0.90 (95% CI 0.83-0.95), and accuracy of 0.83 (95% CI 0.75-0.90) for IM/T. At a center that routinely performs the hybrid procedure, value of > 22 had the highest accuracy. This suggests that the hybrid procedure is not necessarily intrinsically a risk-factor for IM/T, but rather patient selection for the hybrid procedure may play a larger role at centers that do not routinely perform this procedure.
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Síndrome do Coração Esquerdo Hipoplásico , Procedimentos de Norwood , Recém-Nascido , Humanos , Lactente , Estudos Retrospectivos , Resultado do Tratamento , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Procedimentos de Norwood/métodos , Peso ao Nascer , Fatores de Risco , Cuidados PaliativosRESUMO
BACKGROUND: We report current outcomes in patients supported with the HeartMate 3 (HM3) ventricular assist device in a multicenter learning network. METHODS: The Advanced Cardiac Therapies Improving Outcomes Network database was queried for HM3 implants between 12/2017 and 5/2022. Clinical characteristics, postimplant course, and adverse events were collected. Patients were stratified according to body surface area (BSA) (<1.4 m2, 1.4-1.8 m2, and >1.8 m2) at device implantation. RESULTS: During the study period, 170 patients were implanted with the HM3 at participating network centers, with median age 15.3years; 27.1% were female. Median BSA was 1.68 m2; the smallest patient was 0.73 m2 (17.7 kg). Most (71.8%) had a diagnosis of dilated cardiomyopathy. With a median support time of 102.5days, 61.2% underwent transplantation, 22.9% remained supported on device, 7.6% died, and 2.4% underwent device explantation for recovery; the remainder had transferred to another institution or transitioned to a different device type. The most common adverse events included major bleeding (20.8%) and driveline infection (12.9%); ischemic and hemorrhagic stroke were encountered in 6.5% and 1.2% of patients, respectively. Patients with BSA <1.4 m2 had a higher incidence of infection, renal dysfunction, and ischemic stroke. CONCLUSIONS: In this updated cohort of predominantly pediatric patients supported with the HM3 ventricular assist device, outcomes are excellent with <8% mortality on device. Device-related adverse events including stroke, infection, and renal dysfunction were more commonly seen in smaller patients, highlighting opportunities for improvements in care.
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OBJECTIVE: To evaluate the hypothesis that childhood survival for individuals with Down syndrome (DS) and congenital heart defects (CHDs) has improved in recent years, approaching the survival of those with DS without CHDs. STUDY DESIGN: Individuals with DS born from 1979 to 2018 were identified through the Metropolitan Atlanta Congenital Defects Program, a population-based birth defects surveillance system administered by the Centers for Disease Control and Prevention. Survival analysis was performed to evaluate predictors of mortality for those with DS. RESULTS: The cohort included 1671 individuals with DS; 764 had associated CHDs. The 5-year survival in those with DS with CHD improved steadily among individuals born in the 1980s through the 2010s (from 85% to 93%; P = .01), but remained stable (96% to 95%; P = .97) in those with DS without CHDs. The presence of a CHD was not associated with mortality through 5 years of age for those born 2010 or later (hazard ratio, 2.63; 95% CI, 0.95-8.37). In multivariable analyses, atrioventricular septal defects were associated with early (<1 year) and late (>5 year) mortality, whereas ventricular septal defects were associated with intermediate (1-5 years) mortality and atrial septal defects with late mortality, when adjusting for other risk factors. CONCLUSIONS: The gap in 5-year survival between children with DS with and without CHDs has improved over the last 4 decades. Survival after 5 years remains lower for those with CHDs, although longer follow-up is needed to determine if this difference lessens for those born in the more recent years.
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Síndrome de Down , Cardiopatias Congênitas , Comunicação Interatrial , Comunicação Interventricular , Defeitos dos Septos Cardíacos , Criança , Humanos , Síndrome de Down/epidemiologia , Cardiopatias Congênitas/epidemiologia , Defeitos dos Septos Cardíacos/complicaçõesRESUMO
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.
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Transplante de Coração , Obtenção de Tecidos e Órgãos , Humanos , Criança , Disparidades Socioeconômicas em Saúde , Etnicidade , Políticas , Listas de Espera , Estudos RetrospectivosAssuntos
Transplante de Rim , Transplantes , Humanos , Fatores de Risco , Imunidade , Rejeição de EnxertoRESUMO
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.
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Cardiomiopatias , Transplante de Coração , Humanos , Criança , Listas de Espera , Políticas , Estudos RetrospectivosRESUMO
We aimed to describe the longitudinal risk of advanced heart failure (HF) leading to death, heart transplantation, or ventricular assist device (VAD) placement after congenital heart surgery (CHS) and how it varies across the spectrum of congenital heart disease. We linked the records of patients who underwent first CHS in the Pediatric Cardiac Care Consortium between 1982 and 2003 with the United States National Death Index and Organ Procurement and Transplantation Network databases. Primary outcome was time from CHS discharge to HF-related death, heart transplant, or VAD placement, analyzed with proportional hazards models accounting for competing mortality. In 35,610 patients who survived a first CHS, there were 799 HF deaths, transplants, or VADs over a median of 23 years (interquartile range, 19 to 27). Cumulative incidence at 25 years was 2.3% (95% confidence interval [CI] 2.1% to 2.4%). Compared to mild 2-ventricle defects, the adjusted subhazard ratio for moderate and severe 2-ventricle defects was 3.21 (95% CI 2.28 to 4.52) and 9.46 (95% CI 6.71 to 13.3), respectively, and for single-ventricle defects 31.8 (95% CI 22.2 to 45.6). Systemic right ventricle carried the highest risk 2 years after CHS (subhazard ratio 2.76 [95% CI 2.08 to 3.68]). All groups had higher rates of HF-related death compared with the general population (cause-specific standardized mortality ratio 56.1 [95% CI 51.0 to 61.2]). In conclusion, the risk of advanced HF leading to death, transplantation, or VAD was high across the spectrum of congenital heart disease. While severe defects carry the highest risk, those with mild disease are still at greater risk than the general population.
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Cardiopatias Congênitas , Insuficiência Cardíaca , Transplante de Coração , Coração Auxiliar , Criança , Humanos , Estudos Retrospectivos , Resultado do Tratamento , Estados Unidos/epidemiologiaAssuntos
COVID-19/complicações , COVID-19/virologia , SARS-CoV-2 , Síndrome de Resposta Inflamatória Sistêmica/diagnóstico , Síndrome de Resposta Inflamatória Sistêmica/etiologia , Adolescente , Biomarcadores , COVID-19/diagnóstico , COVID-19/etiologia , COVID-19/terapia , Gerenciamento Clínico , Suscetibilidade a Doenças , Humanos , Masculino , Imagem Multimodal/métodos , Avaliação de Sintomas , Síndrome de Resposta Inflamatória Sistêmica/terapiaRESUMO
BACKGROUND: There is wide variability in the timing of heart transplant (HTx) after pediatric VAD implant. While some centers wait months before listing for HTx, others accept donor heart offers within days of VAD surgery. We sought to determine if HTx within 30 days versus ≥ 30 after VAD impacts post-HTx outcomes. METHODS: Children on VAD pre-HTx were extracted from the Pediatric Heart Transplant Study database. The primary endpoints were post-HTx length of hospital stay (LOS) and one-year survival. Confounding was addressed by propensity score weighting using inverse probability of treatment. Propensity scores were calculated based on age, blood type, primary cardiac diagnosis, decade, VAD type, and allosensitization status. RESULTS: A total of 1064 children underwent VAD prior to HTx between 2000 to 2018. Most underwent HTx ≥ 30 days post-VAD (70%). Infants made up 22% of both groups. Patients ≥ 12 years old were 42% of the < 30 days group and children 1 to 11 years comprised 47% of the ≥ 30 days group (p < 0.001). There was no difference in the prevalence of congenital heart disease vs. cardiomyopathy (p = 0.8) or high allosensitization status (p = 0.9) between groups. Post-HTx LOS was similar between groups (p = 0.11). One-year survival was lower in the < 30 days group (adjusted mortality HR 1.76, 95% CI 1.11-2.78, p = 0.016). CONCLUSIONS: A longer duration of VAD support prior to HTx is associated with a one-year survival benefit in children, although questions of patient complexity, post-VAD complications and the impact on causality remain. Additional studies using linked databases to understand these factors will be needed to fully assess the optimal timing for post-VAD HTx.
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Cardiomiopatias/terapia , Cardiopatias Congênitas/terapia , Transplante de Coração , Coração Auxiliar , Criança , Pré-Escolar , Duração da Terapia , Feminino , Humanos , Lactente , Estudos Longitudinais , Masculino , Fatores de Tempo , Resultado do TratamentoRESUMO
Background With advances in the treatment of congenital heart disease (CHD), more women with CHD survive childhood to reach reproductive age. The objective of this study was to evaluate the maternal and neonatal outcomes of pregnancies among women with CHD in the modern era. Methods and Results We conducted a meta-analysis of peer-reviewed literature published January 2007 through June 2019. Studies were included if they reported on maternal or fetal mortality and provided data by CHD lesion. Meta-analysis was performed using random effect regression modeling using Comprehensive Meta-Analysis (v3). CHD lesions were categorized as mild, moderate, and severe to allow for pooling of data across studies. Of 2200 articles returned by our search, 32 met inclusion criteria for this study. Overall, the rate of neonatal mortality was 1%, 3.1%, and 3.5% in mild, moderate, and severe lesions, respectively. There were too few maternal deaths in any group to pool data. The rates of maternal and neonatal morbidity among women with CHD increase with severity of lesion. Specifically, rates of maternal arrhythmia and heart failure, cesarean section, preterm birth, and small for gestational age neonate are all markedly increased as severity of maternal CHD increases. Conclusions In the modern era, pregnancy in women with CHD typically has a successful outcome in both mother and child. However, as maternal CHD severity increases, so too does the risk of numerous morbidities and neonatal mortality. These findings may help in counseling women with CHD who plan to become pregnant, especially women with severe lesions.
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Cardiopatias Congênitas/mortalidade , Complicações Cardiovasculares na Gravidez/mortalidade , Feminino , Idade Gestacional , Humanos , Lactente , Mortalidade Infantil/tendências , Recém-Nascido , Mortalidade Materna/tendências , Gravidez , Resultado da GravidezRESUMO
Educational development is an important component of quality of life for children with heart transplant. Aims include determining prevalence of and risk factors for modified education placement in a large representative sample of pediatric heart transplant recipients. Participants included 1495 patients (age 6-18 years) from the PHTS database. Data on education placement and clinical predictors were collected at listing and at 1 and 3 years post-transplant. At listing, 88% of patients were in typical education placement, while 12% were in modified education. Males (P = .02), those with CHD (P < .0001), those with non-private insurance (P < .0001), and those with longer hospital stay (P = .001) were more likely to be in a modified education placement at time of listing. Age, race, listing status, mechanical support, and waitlist time were not significantly associated with placement. The prevalence of typical education placement was similar (87% at 1-year and 86% at 3-year) post-transplant. Predictors of modified education placement at 3-year follow-up included placement at listing (OR = 12.9 [95% CI 7.6-21.9], P < .0001), non-private insurance (OR = 2.0 [95% CI 1.3-3.2], P = .001), CHD (OR = 1.8 [95% CI 1.1-2.7, P = .01), history of post-transplant infection (OR = 1.9 [95% CI 1.2-2.9, P = .007), and number of post-transplant infections (OR = 1.3 [95% CI 1.1-1.5, P = .002). Among pediatric heart transplant recipients, males, those with non-private insurance, those with CHD, and those who experience post-transplant infections are at greatest risk for modified academic placement, which persists for several years post-transplant and deserves targeted intervention.
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Escolaridade , Transplante de Coração , Deficiências da Aprendizagem/epidemiologia , Complicações Pós-Operatórias/epidemiologia , Adolescente , Criança , Estudos de Coortes , Feminino , Humanos , Masculino , Fatores de RiscoRESUMO
TDI-MPI has been shown to predict cardiovascular mortality in adults; there are a paucity of data on its use in children. We sought to determine the prognostic significance of TDI-MPI at time of DCM diagnosis in children. Patients aged ≤18 years diagnosed with DCM were included along with age- and sex-matched controls. Echo at diagnosis was analyzed to obtain standard measures of LV function, PW-MPI, and septal and LV free wall TDI-MPI. Survival analysis was used to assess the time to composite outcome of death, VAD, or transplant, stratified by TDI-MPI z-score. The study included 79 patients with DCM and 79 controls. During a median follow-up of 182 days (IQR 41-815 days), 16 underwent VAD placement, 21 underwent cardiac transplant, 6 died, and 36 had event-free survival. The median septal TDI-MPI for cases was 0.70 for patients with DCM vs 0.45 for controls (P < .001). Those with septal TDI-MPI z-scores ≥2 develop events significantly earlier than those with z-score <2 (P = .014). In multivariable analysis, TDI-MPI z-score ≥2 was significantly associated with poor outcomes (HR 2.12, 95% CI 1.06-4.23). TDI-MPI can be reliably performed in pediatric patients with DCM. A TDI-MPI z-score ≥2 at diagnosis may be associated with earlier poor outcome. Further studies evaluating the use of TDI-MPI in longitudinal follow-up of patients with DCM may be helpful in refining its clinical use.
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Cardiomiopatia Dilatada/diagnóstico por imagem , Ecocardiografia Doppler/métodos , Adolescente , Cardiomiopatia Dilatada/mortalidade , Cardiomiopatia Dilatada/fisiopatologia , Cardiomiopatia Dilatada/cirurgia , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Seguimentos , Transplante de Coração , Coração Auxiliar , Humanos , Lactente , Recém-Nascido , Masculino , Variações Dependentes do Observador , Prognóstico , Estudos Retrospectivos , Análise de SobrevidaRESUMO
OBJECTIVE: Pulmonary atresia with intact ventricular septum (PA/IVS) can be treated by various operative and catheter-based interventions. We aim to understand the long-term transplant-free survival of patients with PA/IVS by treatment strategy. METHODS: Cohort study from the Pediatric Cardiac Care Consortium, a multi-institutional registry with prospectively acquired outcome data after linkage with the National Death Index and the Organ Procurement and Transplantation Network. RESULTS: Eligible patients underwent neonatal surgery or catheter-based intervention for PA/IVS between 1982 and 2003 (median follow-up of 16.7 years, IQR: 12.6-22.7). Over the study period, 616 patients with PA/IVS underwent one of three initial interventions: aortopulmonary shunt, right ventricular decompression or both. Risk factors for death at initial intervention included earlier birth era (1982-1992), chromosomal abnormality and atresia of one or both coronary ostia. Among survivors of neonatal hospitalisation (n=491), there were 99 deaths (4 post-transplant) and 10 transplants (median age of death or transplant 0.7 years, IQR: 0.3-1.8 years). Definite repair or last-stage palliation was achieved in the form of completed two-ventricle repair (n=201), one-and-a-half ventricle (n=39) or Fontan (n=96). Overall 20-year survival was 66%, but for patients discharged alive after definitive repair, it reached 97.6% for single-ventricle patients, 90.9% for those with one-and-a-half ventricle and 98.0% for those with complete two-ventricle repair (log-rank p=0.052). CONCLUSIONS: Transplant-free survival in PA/IVS is poor due to significant infantile and interstage mortality. Survival into early adulthood is excellent for patients reaching completion of their intended path independent of type of repair.