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1.
Pediatr Cardiol ; 42(1): 36-41, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33394107

RESUMO

In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address (1) developing a risk score to predict adverse outcomes in patients with hypoplastic left heart syndrome using the national pediatric cardiology quality improvement collaborative, (2) outcomes of the Ross procedure in patients with a previous aortic valve surgery showing better outcomes in these patients compared to patients with the primary Ross procedure, (3) the initial experience with continuous flow ventricular assist devices in pediatric patients showing low mortality post implantation, (4) lower socioeconomic status is associated with worse long-term neurodevelopmental outcomes in patients with hypoplastic left heart syndrome, (5) QRS fragmentation is associated with higher incidence of appropriate shock after implantable cardioverter defibrillator implantation in repaired tetralogy of Fallot, (6) atrioventricular valve repair may have better outcomes if performed before the Fontan operation compared to after the Fontan operation.


Assuntos
Cardiologia , Procedimentos Cirúrgicos Cardiovasculares/métodos , Cardiopatias/terapia , Pediatria , Valva Aórtica/cirurgia , Criança , Feminino , Técnica de Fontan/métodos , Cardiopatias/cirurgia , Implante de Prótese de Valva Cardíaca/métodos , Coração Auxiliar , Humanos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Masculino , Transtornos do Neurodesenvolvimento/epidemiologia , Publicações Periódicas como Assunto , Fatores de Risco , Fatores Socioeconômicos , Tetralogia de Fallot/cirurgia
2.
Pediatr Clin North Am ; 67(5): 945-962, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32888691

RESUMO

Hypoplastic left heart syndrome (HLHS) is a complex form of congenital heart disease defined by anatomic and functional inadequacy of the left side of the heart with nonviability of the left ventricle to perform systemic perfusion. Lethal if not treated, a strategy for survival currently is well established, with continuing improvement in outcomes over the past 30 years. Prenatal diagnosis, good newborn care, improved surgical skills, specialized postoperative care, and unique strategies for interstage monitoring all have contributed to increasing likelihood of survival. The unique life with a single right ventricle and a Fontan circulation is a focused area of investigation.


Assuntos
Técnica de Fontan/métodos , Ventrículos do Coração/anormalidades , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Cuidados Paliativos/métodos , Diagnóstico Pré-Natal/métodos , Feminino , Ventrículos do Coração/fisiopatologia , Humanos , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Recém-Nascido , Gravidez
3.
Pediatrics ; 146(4)2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32973120

RESUMO

BACKGROUND: Low socioeconomic status (SES) has emerged as an important risk factor for higher short-term mortality and neurodevelopmental outcomes in children with hypoplastic left heart syndrome and related anomalies; yet little is known about how SES affects these outcomes over the long-term. METHODS: We linked data from the Single Ventricle Reconstruction trial to US Census Bureau data to analyze the relationship of neighborhood SES tertiles with mortality and transplantation, neurodevelopment, quality of life, and functional status at 5 and 6 years post-Norwood procedure (N = 525). Cox proportional hazards regression and linear regression were used to assess the association of SES with mortality and neurodevelopmental outcomes, respectively. RESULTS: Patients in the lowest SES tertile were more likely to be racial minorities, older at stage 2 and Fontan procedures, and to have more complications and fewer cardiac catheterizations over follow-up (all P < .05) compared with patients in higher SES tertiles. Unadjusted mortality was highest for patients in the lowest SES tertile and lowest in the highest tertile (41% vs 29%, respectively; log-rank P = .027). Adjustment for patient birth and Norwood factors attenuated these differences slightly (P = .055). Patients in the lowest SES tertile reported lower functional status and lower fine motor, problem-solving, adaptive behavior, and communication skills at 6 years (all P < .05). These differences persisted after adjustment for baseline and post-Norwood factors. Quality of life did not differ by SES. CONCLUSIONS: Among patients with hypoplastic left heart syndrome, those with low SES have worse neurodevelopmental and functional status outcomes at 6 years. These differences were not explained by other patient or clinical characteristics.


Assuntos
Técnica de Fontan/métodos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Classe Social , Cateterismo Cardíaco/estatística & dados numéricos , Criança , Desenvolvimento Infantil , Pré-Escolar , Deficiências do Desenvolvimento/epidemiologia , Escolaridade , Feminino , Técnica de Fontan/mortalidade , Técnica de Fontan/estatística & dados numéricos , Transplante de Coração/estatística & dados numéricos , Humanos , Síndrome do Coração Esquerdo Hipoplásico/complicações , Síndrome do Coração Esquerdo Hipoplásico/etnologia , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Renda , Recém-Nascido , Masculino , Ocupações , Complicações Pós-Operatórias/epidemiologia , Modelos de Riscos Proporcionais , Qualidade de Vida , Características de Residência , Resultado do Tratamento , Coração Univentricular/mortalidade , Coração Univentricular/cirurgia
4.
Int Heart J ; 61(4): 851-855, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32728004

RESUMO

Protein-losing enteropathy (PLE) is one of the major complications after a Fontan operation. Some PLE patients suffer from concurrent gastrointestinal bleeding. An effective treatment regimen for such patients has not been established yet. Further, it remains unknown whether PLE and gastrointestinal bleeding coexist independently, or protein losing is associated with gastrointestinal bleeding. We report a 7-year-old steroid-refractory post-Fontan PLE case suggesting the latter pathogenesis together with a literature review.


Assuntos
Técnica de Fontan/efeitos adversos , Hemorragia Gastrointestinal/complicações , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Enteropatias Perdedoras de Proteínas/etiologia , Administração Oral , Anemia Refratária/etiologia , Cateterismo Cardíaco/métodos , Criança , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Humanos , Hipoalbuminemia/etiologia , Masculino , Prednisolona/administração & dosagem , Prednisolona/uso terapêutico , Enteropatias Perdedoras de Proteínas/tratamento farmacológico , Remissão Espontânea , Resultado do Tratamento
5.
Artigo em Inglês | MEDLINE | ID: mdl-32520450

RESUMO

The Melody valve (Medtronic, Minneapolis, MN, USA) is a stented bovine jugular vein graft that was primarily approved for transcatheter implantation in a pulmonary valve position. The prosthetic valve can also be implanted in an atrioventricular position in infants and young children, and in these cases it must be modified appropriately.  In this tutorial we demonstrate the surgical preparation of a stented transcatheter Melody valve for implantation in the atrioventricular position. Additionally, we present a safe and effective method for surgical valve-in-valve implantation in a 3-year-old patient with hypoplastic left heart syndrome.


Assuntos
Bioprótese , Implante de Prótese de Valva Cardíaca/métodos , Próteses Valvulares Cardíacas , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Stents , Animais , Bovinos , Humanos , Lactente , Veias Jugulares/cirurgia , Valva Tricúspide/cirurgia
6.
Pediatr. aten. prim ; 22(86): 185-188, abr.-jun. 2020. ilus, tab
Artigo em Espanhol | IBECS | ID: ibc-198536

RESUMO

La enfermedad hepática relacionada con cirugía de Fontan es la afectación estructural y funcional del hígado tras realizar una cirugía de Fontan. Como en otras hepatopatías, el curso puede ser progresivo, y abocar potencialmente a la aparición de complicaciones, algunas de ellas importantes, como cirrosis, ascitis, varices esofágicas o encefalopatía. Su origen no es inflamatorio, sino congestivo. Entre las pruebas complementarias, destacan la ecografía Doppler, la elastografía y la biopsia hepática. Su tratamiento es multidisciplinar e incluye optimizar la función cardiaca, prevención y manejo de las complicaciones hepáticas y prevención del hígado graso


Liver disease related to Fontan's surgery is a structural and functional liver disease after performing the Fontan procedure. As in other liver diseases, the course can be progressive, potentially leading to the appearance of complications, such as cirrhosis, ascites, esophageal varices or encephalopathy. Its origin is not inflammatory, but congestive. Among the complementary tests, the Doppler ultrasound, elastography and liver biopsy stand out. Its treatment is multidisciplinary, and includes optimizing cardiac function, prevention and management of liver complications, and prevention of fatty liver disease


Assuntos
Humanos , Feminino , Adolescente , Técnica de Fontan/efeitos adversos , Hepatopatias/etiologia , Síndrome do Coração Esquerdo Hipoplásico/complicações , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Hepatopatias/diagnóstico por imagem , Ultrassonografia/métodos
7.
Ann Thorac Surg ; 110(2): 622-629, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32380059

RESUMO

BACKGROUND: Contradictory data exist regarding timing of stage 2 palliation (S2P). Prolonged interstage hospitalizations and home surveillance programs have contributed to a more rapid progression to S2P. Our goal is to describe the S2P population and explore the relationships of clinical outcomes and S2P timing at our institution over the last 14 years. METHODS: S2P procedures from 2004 to 2018 at a single institution were included in a retrospective analysis. The analysis was stratified by S2P timing and clinical variables. The primary outcome was Fontan completion, and secondary outcomes included mortality and orthotopic heart transplantation rate, as well as perioperative clinical variables. RESULTS: A total of 114 patients who underwent S2P were included. The median age and weight at S2P were 100 days (interquartile range [IQR], 87-119) and 5.1 (IQR, 4.6-5.5) kg, respectively. The median age in the early group was 79 (IQR, 73-87) days and in the nonearly group was 107 (IQR, 100-124) days. Ninety percent of cavopulmonary anastomoses were augmented with an ePTFE (expanded polytetrafluoroethylene) patch. The overall Fontan completion rate was 76%, without differences in Kaplan-Meier estimates. There were no operative mortalities and no differences in late mortality rate (P = .30). CONCLUSIONS: The interstage period continues to be high risk for those undergoing single-ventricle palliation. In our experience, S2P performed at less than 90 days seems to be a viable and safe procedure when indicated, resulting in comparable Fontan completion rates.


Assuntos
Técnica de Fontan/métodos , Previsões , Ventrículos do Coração/cirurgia , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Cuidados Paliativos , Feminino , Seguimentos , Humanos , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Lactente , Masculino , Pennsylvania/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida/tendências , Resultado do Tratamento
8.
Int J Cardiovasc Imaging ; 36(9): 1667-1677, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32363447

RESUMO

The principal aim of this study was to evaluate changes in systolic function in the single right ventricle (SRV), during progression of the same patient through the three stages of surgical repair for hypoplastic left heart syndrome and during a 5-year follow-up. We hypothesize that, SRV global longitudinal strain (GLS) will be low during 3 stages of repair even in stable patients. We retrospectively evaluated 140 echocardiograms in 20 patients with HLHS (ages 0-11.3 years), before and after 3 stages of surgical palliation. Five-year follow-up data were available in all 20 patients. Controls with structurally normal hearts and in the same age group were used for comparison. We utilized speckle-tracking imaging for assessment of SRV segmental and global longitudinal and circumferential strains, from previously acquired 4-chamber and mid-cavity short-axis views prior to and within 1-3 months of each surgical stage. Longitudinal strain (LS) remained low through all 3 stages of repair and during follow-up. The pre-Fontan stage demonstrated significant interstage improvement compared to the post-Glenn stage despite similar volume status. Global LS was (- 15.6 ± 4.5% after Fontan surgery and remained similar (- 15.32 ± 3.2%) 5 years later. The SRV also showed increased dominance of circumferential strain compared to the normal RV, where the longitudinal deformation was dominant. In SRV, longitudinal strain may be a useful clinical index for evaluating both segmental and global function in an objective manner. Due to lack of significant clinical deterioration over a 10-year period, we speculate that a "lower-than-normal" longitudinal strain may be used as an objective measure of SRV function in clinically stable patients, particularly after the Fontan operation. Compensatory mechanisms where the longitudinal pattern of contraction switches to a more circumferential pattern, may play a role in asymptomatic patients with HLHS.


Assuntos
Ecocardiografia , Técnica de Fontan , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Contração Miocárdica , Cuidados Paliativos , Função Ventricular Direita , Criança , Pré-Escolar , Feminino , Técnica de Fontan/efeitos adversos , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Lactente , Recém-Nascido , Masculino , Variações Dependentes do Observador , Projetos Piloto , Valor Preditivo dos Testes , Recuperação de Função Fisiológica , Reprodutibilidade dos Testes , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
9.
J Pediatr ; 222: 186-192.e1, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32417078

RESUMO

OBJECTIVE: Within the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), a learning health network developed to improve outcomes for patients with hypoplastic left heart syndrome and variants, we assessed which centers contributed to reductions in mortality and growth failure. STUDY DESIGN: Centers within the NPC-QIC were divided into tertiles based on early performance for mortality and separately for growth failure. These groups were evaluated for improvement from the early to late time period and compared with the other groups in the late time period. RESULTS: Mortality was 3.8% for the high-performing, 7.6% for the medium-performing, and 14.4% for the low-performing groups in the early time period. Only the low-performing group had a significant change (P < .001) from the early to late period. In the late period, there was no difference in mortality between the high- (5.7%), medium- (7%), and low- (4.6%) performing centers (P = .5). Growth failure occurred in 13.9% for the high-performing, 21.9% for the medium-performing, and 32.8% for the low-performing groups in the early time period. Only the low-performing group had a significant change (P < .001) over time. In the late period, there was no significant difference in growth failure between the high- (19.8%), medium- (21.5%), and low- (13.5%) performing groups (P = .054). CONCLUSIONS: Improvements in the NPC-QIC mortality and growth measures are primarily driven by improvement in those performing the worst in these areas initially without compromising the success of high-performing centers. Focus for improvement may vary by center based on performance.


Assuntos
Educação em Saúde , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Procedimentos de Norwood/métodos , Cuidados Paliativos/normas , Melhoria de Qualidade , Sistema de Registros , Feminino , Humanos , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Lactente , Masculino , Estudos Retrospectivos
10.
Pediatr Cardiol ; 41(5): 996-1011, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32337623

RESUMO

The objective of this analysis was to update trends in LOS and costs by survivorship and ECMO use among neonates with hypoplastic left heart syndrome (HLHS) undergoing stage 1 palliation surgery using 2016 data from the Healthcare Cost and Utilization Project Kids' Inpatient Database. We identified neonates ≤ 28 days old with HLHS undergoing Stage 1 surgery, defined as a Norwood procedure with modified Blalock-Taussig (BT) shunt, Sano modification, or both. Multivariable regression with year random effects was used to compare LOS and costs by hospital region, case volume, survivorship, and ECMO vs. no ECMO. An E-value analysis, an approach for conducting sensitivity analysis for unmeasured confounding, was performed to determine if unmeasured confounding contributed to the observed effects. Significant differences in total costs, LOS, and mortality were noted by hospital region, ECMO use, and sub-analyses of case volume. However, other than ECMO use and mortality, the maximum E-value confidence interval bound was 1.71, suggesting that these differences would disappear with an unmeasured confounder 1.71 times more associated with both the outcome and exposure (e.g., socioeconomic factors, environment, etc.) Our findings confirm previous literature demonstrating significant resource utilization among Norwood patients, particularly those undergoing ECMO use. Based on our E-value analysis, differences by hospital region and case volume can be explained by moderate unobserved confounding, rather than a reflection of the quality of care provided. Future analyses on surgical quality must account for unobserved factors to provide meaningful information for quality improvement.


Assuntos
Custos Hospitalares/estatística & dados numéricos , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Tempo de Internação/estatística & dados numéricos , Procedimentos de Norwood/mortalidade , Bases de Dados Factuais , Oxigenação por Membrana Extracorpórea/economia , Oxigenação por Membrana Extracorpórea/mortalidade , Feminino , Humanos , Síndrome do Coração Esquerdo Hipoplásico/economia , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Recém-Nascido , Masculino , Procedimentos de Norwood/economia , Cuidados Paliativos/economia , Cuidados Paliativos/estatística & dados numéricos , Melhoria de Qualidade , Estudos Retrospectivos , Estados Unidos/epidemiologia
11.
Am J Cardiol ; 125(8): 1263-1269, 2020 04 15.
Artigo em Inglês | MEDLINE | ID: mdl-32081367

RESUMO

Implantation of a permanent pacemaker is a negative prognostic marker in patients with Fontan palliation; however, data delineating outcomes in adult patients with pacemaker requirements are lacking. We hypothesize that high ventricular pacing burden is associated with adverse outcomes in adult Fontan patients. We performed a retrospective review comprising adult patients with history of Fontan repair. A high burden of ventricular pacing was defined as ≥40% pacing. Major adverse clinical events (MACE) were defined as all-cause mortality or need for advanced cardiac therapies (ventricular assist device or heart transplant). A total of 145 adult patients with Fontan were studied for a median of 3.1 years. Twenty (14%) patients had implanted pacemakers with ≥40% ventricular pacing. Twelve events occurred in those with ≥40% ventricular pacing (incidence 60.0%) versus 11 in those without (incidence 8.8%). In multivariable analysis, ≥40% ventricular-pacing (odds ratio 12.51, confidence interval [CI] 3.56 to 43.83, p <0.001) was associated with MACE independent of initial Fontan type, New York Heart Association functional class at baseline, or history of atrial tachyarrythmia. In survival analysis, patients with ≥40% ventricular pacing had nearly 8 times the risk of MACE compared with those with a lower ventricular pacing burden (hazard ratio 7.79, 95% CI 2.56 to 23.66, p <0.001), whereas patients with atrial-only or <40% ventricular pacing burden had a trend toward higher hazard of MACE compared with those without permanent pacemaker (hazard ratio 3.38, 95% CI 0.92 to 12.47, p = 0.07) that did not meet statistical significance. These findings suggest that high ventricular pacing burden contributes to poor outcomes in the adult Fontan patients and bear consideration when determining optimal treatment of tachyarrhythmias in this population.


Assuntos
Estimulação Cardíaca Artificial/estatística & dados numéricos , Técnica de Fontan , Bloqueio Cardíaco/terapia , Cardiopatias Congênitas/cirurgia , Transplante de Coração/estatística & dados numéricos , Coração Auxiliar/estatística & dados numéricos , Mortalidade , Complicações Pós-Operatórias/terapia , Adulto , Feminino , Bloqueio Cardíaco/fisiopatologia , Defeitos dos Septos Cardíacos/cirurgia , Humanos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Masculino , Razão de Chances , Marca-Passo Artificial , Complicações Pós-Operatórias/fisiopatologia , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Síndrome do Nó Sinusal/fisiopatologia , Síndrome do Nó Sinusal/terapia , Atresia Tricúspide/cirurgia , Adulto Jovem
12.
Circ J ; 84(2): 235-244, 2020 01 24.
Artigo em Inglês | MEDLINE | ID: mdl-31932561

RESUMO

BACKGROUND: Right ventricular (RV) volumetric and functional assessments are both crucial for the management of patients with hypoplastic left heart syndrome (HLHS). 3-dimensional echocardiography (3DE) for volume measurements and 2D speckle-tracking echocardiography (2D-STE) for strain analysis are performed separately. 3D-STE is capable of evaluating those parameters simultaneously and providing principal strain (PS), unifying the concepts of myofiber orientation and contraction into a single, maximal contractile direction. However, the application of 3D-STE to HLHS has not been studied and so became the aim of our study.Methods and Results:64 HLHS patients after Fontan palliation underwent 3D-STE analysis measuring RV end-diastolic volume index (EDVi), ejection fraction (EF), global PS (GPS), global circumferential strain (GCS), and global longitudinal strain (GLS). Volume measurements were compared between 3D-STE and 3DE, and strains were compared between 3D- and 2D-STE. EDVi and EF showed strong correlations between 3D-STE and 3DE (r=0.93 and 0.87, respectively). GCS and GLS showed moderate correlations between 3D- and 2D-STE (r=0.65 and 0.61, respectively). GPS showed highest magnitude and excellent correlation with EF (r=-0.95). CONCLUSIONS: Simultaneous volumetric and functional assessment by 3D-STE was a useful method in this HLHS cohort. PS is a promising parameter for evaluating the RV function of HLHS, which could be useful during longitudinal follow-up.


Assuntos
Ecocardiografia Tridimensional , Técnica de Fontan , Ventrículos do Coração/cirurgia , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Cuidados Paliativos , Função Ventricular Direita , Adolescente , Criança , Pré-Escolar , Estudos de Viabilidade , Feminino , Ventrículos do Coração/anormalidades , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Masculino , Variações Dependentes do Observador , Valor Preditivo dos Testes , Recuperação de Função Fisiológica , Reprodutibilidade dos Testes , Fatores de Tempo , Resultado do Tratamento
13.
J Thorac Cardiovasc Surg ; 159(6): 2459-2466.e5, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-31866080

RESUMO

OBJECTIVES: The purpose of this article is to provide thoracic and cardiovascular surgeons with a practical step-by-step strategy to use in collaboration with a biostatistician for implementation of competing risks analysis when analyzing time-to-event data. Patients may have an outside event that precludes the event of interest. Traditional time-to-event analysis incorrectly assumes noninformative censoring in this scenario, which will lead to invalid results and conclusions. METHODS: The steps are (1) to determine whether competing risks analysis is needed, (2) to perform a nonparametric analysis, (3) to perform a model-based analysis, (4) to interpret the results, and (5) to compare to traditional survival analysis methods. We apply our approach to a hypothetical cardiovascular surgery example in determining the hazard of mortality after the stage 3 Fontan operation associated with prematurity among patients with hypoplastic left heart syndrome who had successful completion of Norwood stage 1 while incorporating mortality during the stage 2 bidirectional Glenn procedure as a competing risk. We apply nonparametric, semiparametric, and parametric methods. RESULTS: Although Cox regression establishes prematurity as a significant risk factor of mortality after stage 3 (hazard ratio, 1.26; 95% confidence interval, 1.06-1.50; P = .009), the competing risks analysis with the Fine-Gray model accounting for mortality after stage 2 determines that prematurity is not a significant predictor (hazard ratio, 1.07; 95% confidence interval, 0.90-1.27; P = .467). CONCLUSIONS: This article provides a practical step-by-step approach for making competing risks more accessible for cardiac surgeons collaborating with a biostatistician in analyzing and interpreting time-to-event data.


Assuntos
Bioestatística , Técnica de Fontan/mortalidade , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Procedimentos de Norwood/mortalidade , Nascimento Prematuro/mortalidade , Cirurgiões , Técnica de Fontan/efeitos adversos , Técnica de Fontan/estatística & dados numéricos , Idade Gestacional , Humanos , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Lactente , Recém-Nascido , Recém-Nascido Prematuro , Procedimentos de Norwood/efeitos adversos , Procedimentos de Norwood/estatística & dados numéricos , Intervalo Livre de Progressão , Medição de Risco , Fatores de Risco , Fatores de Tempo
14.
World Neurosurg ; 134: 302-307, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31715418

RESUMO

BACKGROUND: Patients with single-ventricle congenital heart disease may present for scoliosis correction. These patients undergo a series of cardiac operations that create a novel circulation that has a significant impact on the management of their spinal surgery. CASE DESCRIPTION: A 21-year-old man with severe scoliosis presented for posterior T4-L3 spinal fusion. He was born with complex congenital heart disease that resulted in his having a single functioning ventricle. He underwent a series of operations culminating in a Fontan procedure to palliate his heart disease. Both the surgical procedure and the anesthetic plan were modified based on his abnormal physiology, which led to a successful correction with no complications. CONCLUSIONS: Patients who have undergone a Fontan procedure can successfully undergo a lengthy scoliosis correction, but it requires multidisciplinary planning.


Assuntos
Síndrome do Coração Esquerdo Hipoplásico/complicações , Escoliose/complicações , Escoliose/cirurgia , Fusão Vertebral/métodos , Técnica de Fontan , Humanos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Masculino , Adulto Jovem
15.
Pediatr Cardiol ; 41(1): 141-148, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31722042

RESUMO

Patients with hypoplastic left heart syndrome (HLHS) are now surviving through to Fontan palliation and beyond, however, with increased morbidity and mortality. Prolonged QRSd has become one of the predictors of morbidity and mortality in certain congenital heart diseases. There is limited data characterizing the QRSd in patients with HLHS. We aimed to describe the changes in QRSd at various times during the lifetime and to evaluate whether QRSd correlates with a higher risk of developing a composite endpoint of heart failure, heart transplant, or death. We conducted a retrospective chart review of patients with HLHS who survived Fontan palliation. QRSd was measured on ECGs at various stages pre- and postsurgical palliations and subsequently at 5 year intervals. Patients with a composite endpoint were compared to those without. A total of 89 patients were included in the final analysis. The QRSd increased significantly with time from 68.7 ± 9.0 ms prior to Norwood to 91.0 ± 14.0 ms immediately following Fontan and 104.7 ± 13.6 ms 15 years after Fontan (p < 0.001). The composite endpoint was observed in 28 patients (31.4%). The time trends of QRSd differ so that the patients having the composite endpoint experienced a greater increase in QRSd over time (p = 0.009). Ever having a QRSd of 120 ms or more predicted the composite endpoint with 93% specificity. The area under the curve of the receiver operator curve analysis was 0.596. A Cox regression analysis demonstrated that QRS duration > 120 ms was independently related to a greater frequency of composite endpoint and this was confirmed by a Kaplan-Meier analysis (p = 0.011). This study unveils a novel relationship between QRSd of 120 ms or more with the composite endpoint. Despite the low sensitivity, this finding on a routine surveillance ECG could help identify HLHS Fontan patients at risk for heart failure, heart transplant, or death.


Assuntos
Eletrocardiografia/métodos , Técnica de Fontan/mortalidade , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Feminino , Seguimentos , Humanos , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Cuidados Paliativos/métodos , Modelos de Riscos Proporcionais , Curva ROC , Estudos Retrospectivos , Resultado do Tratamento
16.
Eur J Pediatr ; 179(2): 309-316, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31741094

RESUMO

Neonates with hypoplastic left heart syndrome (HLHS) were identified from the National Inpatient Sample dataset for the years 1998-2014. These patients were stratified into two chronological groups, past group (1998-2005) and recent group (2006-2014). A total of 20,649 neonates with HLHS were identified. Of them, 9179 (44.5%) were born in the past group and 11,470 (55.5%) in the recent group. Median birth weight was significantly less in the recent group (2967 g vs. 3110 g, p = 0.005). The patients in the recent group had more patients with low birth weight ( < 2.5 kg) and prematurity (8.7% vs 7.6% and 12.7% vs. 4.3%., respectively). In addition, recent group had more comorbidities including chromosomal anomalies, total anomalous pulmonary venous return, and kidney anomalies (5.6% vs. 3.6%, 2.3% vs. 1.7%, and 5.6% vs. 3.6%, respectively, p < 0.001); these were associated with a higher rate of extracorporeal membrane oxygenation utilization (9.2% vs. 4.5%, p < 0.001). Consequently, median length of stay was longer in the recent group (8 vs. 6 days, p < 0.001).Conclusion: Despite the higher frequency of comorbidities in recent group, the mortality rates decreased by 20% (from 25.3% to 20.6%, p < 0.001). Balloon atrial septostomy was performed less frequently in the recent group (23.3% vs. 16.1%, p < 0.001).What is known:• Hypoplastic left heart syndrome has the highest mortality among congenital cardiac defects during the first year of life.• Limited studies on patients' comorbidities and mortality rates trends over last two decades.What is new:• The study utilized a national database to compare in-hospital mortality and length of stay between the two time periods 1998-2005 and 2006-2014.• The recent group had more comorbidities (prematurity, chromosomal anomalies, total anomalous pulmonary venous return, and kidney anomalies), and there was higher rate of ECMO and longer length of stay, while mortality rates decreased by 20%.


Assuntos
Anormalidades Múltiplas/mortalidade , Causas de Morte , Mortalidade Hospitalar/tendências , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Recém-Nascido Prematuro , Anormalidades Múltiplas/diagnóstico , Anormalidades Múltiplas/cirurgia , Distribuição de Qui-Quadrado , Bases de Dados Factuais , Feminino , Humanos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Recém-Nascido de Baixo Peso , Recém-Nascido , Tempo de Internação , Masculino , Estudos Retrospectivos , Medição de Risco , Estatísticas não Paramétricas , Análise de Sobrevida , Fatores de Tempo , Resultado do Tratamento , Estados Unidos
17.
J Card Surg ; 35(1): 174-179, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31705822

RESUMO

BACKGROUND: Congenital heart disease (CHD) continues to be among the most common birth defects, affecting an estimated 40 000 births annually in the United States. The most common complication of CHD is heart failure. With improved medical management and surgical outcomes, survival for complex congenital heart defects has dramatically improved, but consequentially there are more adults with CHD than children with CHD. Due to longer-term sequelae of CHD, surgical and medical treatment previously thought to be curative is now realized at best to be palliative, and there is a considerable burden of CHD-related heart failure. Stem cell therapy as an adjunct to current surgical and medical strategies is being explored in an effort to ameliorate CHD-related heart failure. This review aims to explore the current literature with regard to stem cell therapy for CHD as well as ongoing trials. METHODS: A MEDLINE (Ovid), MEDLINE (Pubmed), and clinicaltrials.gov search were performed using the medical subject headings congenital heart defects combined with hematopoietic stem cells, stem cell transplantation, mesenchymal stem cells (MSC), cell- or tissue-based therapy, or MSC transplantation. Articles must have been published after 2010. RESULTS: Twenty three articles and 9 ongoing trials met all inclusion criteria. CONCLUSIONS: Areas of interest include myocardiocyte regeneration, tissue graft development to minimize reoperations, and methods of stem cell delivery. While several small trials are showing promise, it is too soon to make definitive statements about the future of stem cell therapies in this field.


Assuntos
Cardiopatias Congênitas/cirurgia , Transplante de Células-Tronco/métodos , Transplante de Células-Tronco Hematopoéticas , Humanos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Transplante de Células-Tronco Mesenquimais
18.
J Thorac Cardiovasc Surg ; 159(2): 637-646, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31668539

RESUMO

OBJECTIVES: Endothelial-to-mesenchymal transition (EndMT) has been identified as the underlying mechanism of endocardial fibroelastosis (EFE) formation. The purpose of this study was to determine whether hemodynamic alterations due to valvar defects promote EndMT and whether age-specific structural changes affect ventricular diastolic compliance despite extensive surgical resection of EFE tissue. MATERIAL AND METHODS: We analyzed EFE tissue from 24 patients with hypoplastic left heart syndrome (HLHS) who underwent left ventricular (LV) rehabilitation surgery at Boston Children's Hospital between December 2011 and March 2018. Six patients with flow disturbances across the aortic valve and/or mitral valve but no HLHS diagnosis and macroscopic appearance of "EFE-like tissue" in the LV were included for comparison. All samples were examined for amount of collagen/elastin production and degradation, and presence of active EndMT by histologic analysis. RESULTS: EFE tissue from patients with and without HLHS consisted predominantly of elastin and collagen fibers. There was no alteration in degradation activity for collagen or elastin as shown by in situ zymography. Active EndMT was found in all patients with and without HLHS with flow disturbances ("EFE-like"). In patients with HLHS, EFE infiltrated into the underlying myocardium with increasing age. CONCLUSIONS: Patients with and without HLHS with flow disturbances due to stenotic or incompetent valves develop EndMT-derived fibrotic tissue covering the LV. When EFE recurs, it is directly associated with flow disturbances and switches to an infiltrative growth pattern with increasing age, leading to increased diastolic stiffness of the LV.


Assuntos
Fibroelastose Endocárdica , Síndrome do Coração Esquerdo Hipoplásico , Procedimentos Cirúrgicos Cardíacos , Criança , Pré-Escolar , Estudos de Coortes , Colágeno/metabolismo , Elastina/metabolismo , Fibroelastose Endocárdica/etiologia , Fibroelastose Endocárdica/patologia , Fibroelastose Endocárdica/fisiopatologia , Ventrículos do Coração/química , Ventrículos do Coração/patologia , Ventrículos do Coração/fisiopatologia , Ventrículos do Coração/cirurgia , Hemodinâmica/fisiologia , Humanos , Síndrome do Coração Esquerdo Hipoplásico/complicações , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Lactente
19.
Ann Thorac Surg ; 109(1): 155-162, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31404548

RESUMO

BACKGROUND: Mortality after stage 1 palliation of hypoplastic left heart syndrome remains significant. Hemodynamic changes result from interaction of cardiac output (CO) and systemic vascular resistance (SVR). We sought to identify time-dependent changes in postoperative hemodynamic states and their associations with mortality. METHODS: Perioperative data were prospectively collected in an institutional review board-approved database. Hemodynamic state was classified as high CO, high SVR, low SVR, and low CO using bivariate analysis of mean arterial pressure and somatic regional near-infrared spectroscopic oximetry saturation. State classifications over 48 postoperative hours were modelled using multinomial logistic regressions for association with mortality. RESULTS: Data from 9614 of 10,272 hours in 214 patients were analyzed. Operative survival was 91%. The predominant state was high CO (46% time). Low CO state without extracorporeal membrane oxygenation (ECMO) was found in 52% of patients for 9.7% time. ECMO was employed in 24 (11.2%) patients for 10.4% time. State stability was 33%, with high SVR the least stable (17%) and high CO the most stable (53%). Transition from high CO increased in hours 1 to 12, mainly to low SVR. Transition to low CO was 18.4%, increasing in hours 1 to 12, mainly from high SVR. The transition risk to ECMO was 0.32%, and 0.74% during hours 1 to 12, only from low CO. Both low CO and ECMO had increased mortality risk. CONCLUSIONS: Bivariate classification defines hemodynamic states with distinct physiologic, transition, and mortality risk profiles. High SVR state was unstable. Transition to ECMO occurred only from low CO, while the low SVR and high CO states were safest.


Assuntos
Débito Cardíaco , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Procedimentos de Norwood , Resistência Vascular , Oxigenação por Membrana Extracorpórea , Feminino , Hemodinâmica , Humanos , Recém-Nascido , Masculino , Procedimentos de Norwood/métodos , Cuidados Paliativos , Medição de Risco , Fatores de Risco , Fatores de Tempo
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