RESUMO
BACKGROUND: The Harmony transcatheter pulmonary valve (TPV) is the first U.S. Food and Drug Administration-approved device for severe pulmonary regurgitation (PR) in the native or surgically repaired right ventricular outflow tract (RVOT). OBJECTIVES: One-year safety and effectiveness of the Harmony TPV were evaluated in patients from the Harmony Native Outflow Tract Early Feasibility Study, Harmony TPV Pivotal Study, and Continued Access Study, representing the largest cohort to date of Harmony TPV recipients. METHODS: Eligible patients had severe PR by echocardiography or PR fraction ≥ 30% by cardiac magnetic resonance imaging and clinical indications for pulmonary valve replacement. The primary analysis included 87 patients who received a commercially available TPV22 (n = 42) or TPV25 (n = 45) device; 19 patients who received an early device iteration prior to its discontinuation were evaluated separately. RESULTS: In the primary analysis, median patient age at treatment was 26 years (IQR: 18-37 years) in the TPV22 group and 29 years (IQR: 19-42 years) in the TPV25 group. At 1 year, there were no deaths; 98% of TPV22 and 91% of TPV25 patients were free from the composite of PR, stenosis, and reintervention (moderate or worse PR, mean RVOT gradient >40 mmHg, device-related RVOT reoperation, and catheter reintervention). Nonsustained ventricular tachycardia occurred in 16% of patients. Most patients had none/trace or mild PR (98% of TPV22 patients, 97% of TPV25 patients). Outcomes with the discontinued device are reported separately. CONCLUSIONS: The Harmony TPV device demonstrated favorable clinical and hemodynamic outcomes across studies and valve types through 1 year. Further follow-up will continue to assess long-term valve performance and durability.
Assuntos
Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Insuficiência da Valva Pulmonar , Valva Pulmonar , Obstrução do Fluxo Ventricular Externo , Humanos , Cateterismo Cardíaco , Estudos Prospectivos , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/cirurgia , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/etiologia , Insuficiência da Valva Pulmonar/cirurgia , Resultado do Tratamento , Obstrução do Fluxo Ventricular Externo/etiologiaRESUMO
BACKGROUND: Genetic defects in the RAS/mitogen-activated protein kinase pathway are an important cause of hypertrophic cardiomyopathy (RAS-HCM). Unlike primary HCM (P-HCM), the risk of sudden cardiac death (SCD) and long-term survival in RAS-HCM are poorly understood. OBJECTIVES: The study's objective was to compare transplant-free survival, incidence of SCD, and implantable cardioverter-defibrillator (ICD) use between RAS-HCM and P-HCM patients. METHODS: In an international, 21-center cohort study, we analyzed phenotype-positive pediatric RAS-HCM (n = 188) and P-HCM (n = 567) patients. The between-group differences in cumulative incidence of all outcomes from first evaluation were compared using Gray's tests, and age-related hazard of all-cause mortality was determined. RESULTS: RAS-HCM patients had a lower median age at diagnosis compared to P-HCM (0.9 years [IQR: 0.2-5.0 years] vs 9.8 years [IQR: 2.0-13.9 years], respectively) (P < 0.001). The 10-year cumulative incidence of SCD from first evaluation was not different between RAS-HCM and P-HCM (4.7% vs 4.2%, respectively; P = 0.59). The 10-year cumulative incidence of nonarrhythmic deaths or transplant was higher in RAS-HCM compared with P-HCM (11.0% vs 5.4%, respectively; P = 0.011). The 10-year cumulative incidence of ICD insertions, however, was 5-fold lower in RAS-HCM compared with P-HCM (6.9% vs 36.6%; P < 0.001). Nonarrhythmic deaths occurred primarily in infancy and SCD primarily in adolescence. CONCLUSIONS: RAS-HCM was associated with a higher incidence of nonarrhythmic death or transplant but similar incidence of SCD as P-HCM. However, ICDs were used less frequently in RAS-HCM compared to P-HCM. In addition to monitoring for heart failure and timely consideration of advanced heart failure therapies, better risk stratification is needed to guide ICD practices in RAS-HCM.
Assuntos
Cardiomiopatia Hipertrófica , Desfibriladores Implantáveis , Insuficiência Cardíaca , Humanos , Estudos de Coortes , Morte Súbita Cardíaca/epidemiologia , Morte Súbita Cardíaca/etiologia , Desfibriladores Implantáveis/efeitos adversos , Cardiomiopatia Hipertrófica/complicações , Cardiomiopatia Hipertrófica/genética , Cardiomiopatia Hipertrófica/diagnóstico , Insuficiência Cardíaca/complicações , Fatores de Risco , Medição de RiscoRESUMO
PURPOSE: The purpose of this study was to compare outcomes of Melody mitral valve to mechanical mitral valve replacement (MVR) for young children. DESCRIPTION: Children who underwent Melody MVR from 2014 to 2020 were case-matched to mechanical MVR patients. Transplant-free survival and cumulative incidence of reintervention were compared. A subanalysis was performed for infants aged < 1 year (9 Melody MVRs and their matches). EVALUATION: Twelve children underwent Melody MVR. Two children (17%) salvaged from mechanical support died. Five of 10 survivors (50%) had subsequent MVR. At 1 and 3 years, transplant-free survival (Melody: 83%, 83%; mechanical: 83%, 67%; P = .180) and reintervention (Melody: 9%, 39%; mechanical: 0%, 18%; P = .18) were equivalent between groups. For children < 1 year of age, Melody MVR had a modest survival benefit (Melody: 89%, 89%; mechanical: 80%, 60%; P = .046), while rate of reintervention remained equivalent (Melody: 13%, 32%; mechanical: 0%, 22%; P = .32). CONCLUSIONS: For patients < 1 year old, Melody MVR offers a promising alternative and is a reasonable bridge to mechanical MVR, which can be performed safely at an older age. Further studies are necessary to corroborate these findings.
Assuntos
Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Insuficiência da Valva Mitral , Estenose da Valva Mitral , Lactente , Humanos , Criança , Pré-Escolar , Valva Mitral/cirurgia , Resultado do Tratamento , Insuficiência da Valva Mitral/cirurgia , Estenose da Valva Mitral/cirurgia , Estudos RetrospectivosRESUMO
BACKGROUND: Aortic valve stenosis is the most common type of congenital left ventricular (LV) outflow tract obstruction. Balloon aortic valvuloplasty (BAV) has become the first-line treatment pathway in many centers. Our aim was to assess the trajectory of LV remodeling following BAV in children and its relationship to residual aortic stenosis (AS) and insufficiency (AI). METHODS: Children <18 years of age who underwent BAV for isolated aortic stenosis from 2004 to 2012 were eligible for inclusion. Those with AI before BAV, other complex congenital heart lesions, or <2 accessible follow-up echocardiograms were excluded. Baseline and serial echocardiographic data pertaining to aortic valve and LV size and function were retrospectively collected through December 2017 or the first reintervention. Longitudinal data was assessed using per-patient time profiles with superimposed trend lines using locally estimated scatterplot smoothing. Associations with reintervention or death were also evaluated. RESULTS: Among the 98 enrolled children, the median (interquartile range) age at BAV was 2.8 months (0.2-75). The median (interquartile range) follow-up was 6.8 years (1.9-9.0). Children with predominantly residual AI (n=11) demonstrated progressive increases in their LV end-diastolic dimension Z score within the first 3 years after the BAV, followed by a plateau (P<0.001). Their mean LV circumferential and longitudinal strain values remained within the normal range but lower than in the non-AI group (P<0.001 and P=0.001, respectively). Children with predominantly residual aortic stenosis (n=44) had no changes in LV dimensions but had a rapid early increase in mean LV circumferential and longitudinal strain. The cumulative proportion (95% CI) of reintervention at 5 years following BAV was 33.7% (23.6%-42.4%). CONCLUSIONS: Our study demonstrates that LV remodeling occurs mainly during the first 3 years in children with predominantly residual AI after BAV, with no subsequent significant functional changes over the medium term. These data improve our understanding of expected patient trajectories and thus may inform decisions on the timing of reintervention.
Assuntos
Estenose da Valva Aórtica/fisiopatologia , Valva Aórtica/diagnóstico por imagem , Valvuloplastia com Balão/métodos , Remodelação Ventricular/fisiologia , Estenose da Valva Aórtica/diagnóstico , Estenose da Valva Aórtica/cirurgia , Criança , Pré-Escolar , Ecocardiografia/métodos , Feminino , Seguimentos , Humanos , Lactente , Masculino , Período Pós-Operatório , Estudos Retrospectivos , Resultado do TratamentoRESUMO
BACKGROUND: The optimal management pathway for the dysfunctional right ventricular outflow tract (RVOT) is uncertain. We evaluated the long-term outcomes and clinical impact of stent implantation for obstructed RVOTs in an era of rapidly progressing transcatheter pulmonary valve technology. METHODS: Retrospective review of 151 children with a biventricular repair who underwent stenting of obstructed RVOT between 1991 and 2017. RESULTS: RVOT stenting resulted in significant changes in peak right ventricle (RV)-to-pulmonary artery (PA) gradient (39.4 ± 17.1-14.9 ± 8.3; p < 0.001) and RV-to-aortic pressure ratio (0.78 ± 0.22-0.49 ± 0.13; p < 0.001). Subsequent percutaneous reinterventions in 51 children to palliate recurrent stenosis were similarly effective. Ninety-nine (66%) children reached the primary outcome of subsequent pulmonary valve replacement (PVR). Freedom from PVR from the time of stent implantation was 91%, 51%, and 23% at 1, 5, and 10 years, respectively. Small balloon diameters for stent deployment were associated with shorter freedom from PVR. When additional children without stent palliation (with RV-to-PA conduits) were added to the stent cohort (total 506 children), the multistate analysis showed the longest freedom from PVR in those with stent palliation and subsequent catheter reintervention. Pulmonary regurgitation was well-tolerated clinically. Indexed RV dimensions and function estimated by echocardiography remained stable at last follow up or before primary outcome. CONCLUSION: Prolongation of conduit longevity with stent implant remains an important strategy to allow for somatic growth to optimize the risk-benefit profile for subsequent surgical or transcatheter pulmonary valve replacement performed at an older age.
Assuntos
Cardiopatias Congênitas , Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Insuficiência da Valva Pulmonar , Valva Pulmonar , Obstrução do Fluxo Ventricular Externo , Cateterismo Cardíaco/efeitos adversos , Cateterismo Cardíaco/métodos , Catéteres , Criança , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/métodos , Humanos , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/cirurgia , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/etiologia , Insuficiência da Valva Pulmonar/cirurgia , Estudos Retrospectivos , Stents , Resultado do Tratamento , Obstrução do Fluxo Ventricular Externo/diagnóstico por imagem , Obstrução do Fluxo Ventricular Externo/etiologia , Obstrução do Fluxo Ventricular Externo/cirurgiaRESUMO
Background: The optimal initial treatment pathway for aortic valve stenosis remains debated. Objectives: The objective of this study was to review current outcomes of balloon aortic valvotomy (BAV) in neonates and infants. Methods: Neonates and infants with a biventricular circulation treated with BAV between 2004 and 2019 were reviewed. Results: One hundred thirty-nine infants (48% neonates) with median (Q1, Q3) age of 33(7, 84) days and weight 4.0 (3.4, 5.1) kg were followed up for 7.1 (3.3, 11.0) years. BAV reduced peak-to-peak gradient from mean (SD) 52 (16) mmHg to 18 (12) mmHg; P < 0.001. Aortic regurgitation (AI) increased with time after BAV. Three children died during follow-up. Fifty-one reinterventions (26 BAV, 19 aortic valve replacements [AVRs], and 6 surgical valvotomies) were performed on 40 children. Freedom from AVR (95% CI) was 96% (93%-99%) at 1, 91% (86%-96%) at 5, and 86% (79%-93%) at 10 years. The predictors of AVR were a unicommissural valve (hazard ratio [HR] [95% CI]: 3.7 [1.4-9.6]; P = 0.007) and moderate to severe AI after index BAV (HR [95% CI]: 3.3 [1.1-9.7]; P = 0.029). Freedom from reintervention was 84% (78%-90%) at 1, 76% (69%-83%) at 5, and 69% (60-78%) at 10 years. Main predictors of reintervention were age below 1 month (HR [95% CI]: 2.1 [1.1-4.1]; P = 0.032) and postdilation peak-to-peak gradient (per 10-mmHg increase; HR [95% CI]: 1.36 [1.02-1.79]; P = 0.032). Conclusions: BAV is a safe and effective treatment for aortic valve stenosis in neonates and infants. Outcomes are competitive with contemporary published data on aortic valve repair in relation to mortality, gradient relief, long-term AVR, and reintervention rates. In the absence of significant AI, surgery can be reserved for those with gradients resistant to valve dilation.
Assuntos
Valva Pulmonar , Estudos de Viabilidade , Humanos , Fatores de Tempo , Resultado do TratamentoRESUMO
Digital reality is an emerging platform for three-dimensional representation of medical imaging data. In this technical innovation paper, the authors evaluated the accuracy and utility of mixed-reality technology in the morphological evaluation of complex congenital heart disease. The authors converted CT datasets of 12 heart specimens with different subtypes of double-outlet right ventricle to stereoscopic images and interrogated them using a mixed-reality system. The morphological features identified on the stereoscopic models were compared with findings at macroscopic examination of the actual heart specimens. The results showed that the mixed-reality system provided highly accurate stereoscopic display of spatially complex congenital cardiac lesions, with interactive features that might enhance 3-D understanding of morphology. Additionally, the authors found that high-resolution digital reproduction of cardiac specimens using clinical CT scanners is feasible for preservation and educational purposes.
Assuntos
Cardiopatias Congênitas/diagnóstico por imagem , Imageamento Tridimensional , Interpretação de Imagem Radiográfica Assistida por Computador/métodos , Tomografia Computadorizada por Raios X , Artefatos , Humanos , Técnicas In Vitro , Estudo de Prova de ConceitoRESUMO
BACKGROUND: Recent randomized trials have confirmed the role of patent foramen ovale (PFO) closure in the secondary prevention of cryptogenic stroke. Guidelines have suggested a central role for intraprocedural imaging using intracardiac echocardiography (ICE). However, this modality may not be required to achieve safe and effective closure. We aimed to examine the periprocedural outcomes of PFO closure retrospectively, using fluoroscopic guidance in patients with cryptogenic stroke, with provisional ICE guidance driven by anatomic and procedural factors. METHODS: A retrospective chart review of consecutive patients who underwent PFO closure in a single centre using the Amplatzer PFO occluder (AGA Medical Corporation, Plymouth, Minnesota) for cryptogenic stroke was conducted. Outcomes analyzed included procedural data, periprocedural complications, length of stay, and factors contributing to the use of intraprocedural imaging. RESULTS: Between 2006 and 2017, 467 patients underwent PFO closure for cryptogenic stroke with the Amplatzer PFO occluder; 381 patients underwent closure with fluoroscopy alone, and 86 with ICE and fluoroscopic guidance. Periprocedural arrhythmic complications occurred in 1.3% in the fluoroscopy group and 1.2% in the ICE group (P = 1.000). Vascular complications occurred in 0.5% in the fluoroscopy group and 2.3% (P = 0.323) in the ICE group. One device embolized requiring surgical intervention. There was no in-hospital mortality or stroke. Same-day discharge occurred in 97.6% of patients. CONCLUSION: Our single-centre experience suggests that PFO closure can be safely conducted under fluoroscopic guidance alone with provisional adjunctive ICE use limited to specific anatomic situations.
Assuntos
Cateterismo Cardíaco , Forame Oval Patente/cirurgia , Complicações Pós-Operatórias , Dispositivo para Oclusão Septal , Acidente Vascular Cerebral , Cateterismo Cardíaco/efeitos adversos , Cateterismo Cardíaco/instrumentação , Cateterismo Cardíaco/métodos , Técnicas de Imagem Cardíaca/métodos , Ecocardiografia/métodos , Feminino , Fluoroscopia/métodos , Forame Oval Patente/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Ontário/epidemiologia , Avaliação de Processos e Resultados em Cuidados de Saúde , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/prevenção & controle , Estudos Retrospectivos , Prevenção Secundária/métodos , Prevenção Secundária/estatística & dados numéricos , Dispositivo para Oclusão Septal/efeitos adversos , Dispositivo para Oclusão Septal/estatística & dados numéricos , Acidente Vascular Cerebral/epidemiologia , Acidente Vascular Cerebral/etiologia , Acidente Vascular Cerebral/prevenção & controle , Cirurgia Assistida por Computador/efeitos adversos , Cirurgia Assistida por Computador/métodos , Cirurgia Assistida por Computador/estatística & dados numéricosRESUMO
BACKGROUND: The Harmony transcatheter pulmonary valve (TPV) was designed for treatment of postoperative pulmonary valve regurgitation in patients with repaired right ventricular outflow tracts. METHODS: The Native TPV EFS (Early Feasibility Study) is a prospective, multicenter, nonrandomized feasibility study. Three-year outcomes are reported. RESULTS: Of 20 implanted patients, 17 completed 3-year follow-up (maximum: 4.1 years). There were no deaths and 2 early explants. One patient did not complete the 3-year visit. In patients with available 3-year echocardiographic data, 1 had a mild paravalvular leak and the rest had none/trace; 1 patient had mild pulmonary valve regurgitation and the remainder had none/trace. The 3-year mean right ventricular outflow tract echocardiographic gradient was 15.7±5.5 mm Hg. Radiographically, no late frame fractures or erosions were identified. At 2 years, 2 patients presented with an increased echocardiographic outflow gradient (1 mixed lesion with moderate/severe pulmonary valve regurgitation). Computed tomography scans identified neointimal tissue ingrowth within the stent frame in both patients, and they were treated successfully with a transcatheter valve-in-valve procedure (Melody TPV). Additional follow-up computed tomography scans performed at 3.2±0.5 years after implant were obtained in 16 patients and revealed luminal tissue thickening at the inflow and outflow portion of the frame with no significant alteration of the valve housing. CONCLUSIONS: Three-year results from the Native TPV EFS revealed stable Harmony TPV device position, good valve function in most, and the absence of moderate/severe paravalvular leak and significant late frame fractures. Two patients developed significant neointimal proliferation requiring valve-in-valve treatment, while all others had no clinically significant right ventricular outflow tract obstruction. CLINICAL TRIAL REGISTRATION: URL: https://www.clinicaltrials.gov. Unique identifier: NCT01762124.
Assuntos
Bioprótese , Cateterismo Cardíaco/instrumentação , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Implante de Prótese de Valva Cardíaca/instrumentação , Próteses Valvulares Cardíacas , Insuficiência da Valva Pulmonar/cirurgia , Valva Pulmonar/cirurgia , Obstrução do Fluxo Ventricular Externo/cirurgia , Adolescente , Adulto , Canadá , Cateterismo Cardíaco/efeitos adversos , Estudos de Viabilidade , Feminino , Implante de Prótese de Valva Cardíaca/efeitos adversos , Humanos , Masculino , Estudos Prospectivos , Desenho de Prótese , Falha de Prótese , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/etiologia , Insuficiência da Valva Pulmonar/fisiopatologia , Recuperação de Função Fisiológica , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Estados Unidos , Adulto JovemRESUMO
BACKGROUND: There is little published data regarding percutaneous patent ductus arteriosus (PDA) closure in adults. We aim to describe the outcomes of adult patients undergoing PDA closure at a single tertiary referral centre. METHODS: All adults who underwent device PDA closure at our centre from 2001 to 2017 were identified and enrolled in the study. Available clinical data and imaging data were reviewed. RESULTS: At total of 141 patients were identified, with a mean age of 43 ± 15 years. Left ventricular dilation was present in 27% and pulmonary hypertension in 36% of the patients. Most ducts (74%) were of type A morphology. Mean ductal diameter at the pulmonary artery end was 4.1 ± 1.9 mm and mean length was 10.0 ± 4.7 mm. Wire passage from the pulmonary artery was achieved in 79%. Procedural success rate was 100%, and an Amplatzer duct occluder was deployed in all successful cases (ADO1 device in 97%). There was a small residual shunt in 6% at the time of closure, and only 2 patients had a residual leak on echocardiography at a median 3 months' follow-up. No major complications occurred. CONCLUSIONS: Transcatheter PDA closure with the use of an Amplatzer duct occluder is very effective in adults across all duct morphologies and associated with a very low complication rate.
Assuntos
Procedimentos Cirúrgicos Cardiovasculares , Permeabilidade do Canal Arterial , Ventrículos do Coração , Hipertensão Pulmonar , Complicações Pós-Operatórias , Implantação de Prótese , Dispositivo para Oclusão Septal , Adulto , Canadá , Cateterismo Cardíaco/métodos , Procedimentos Cirúrgicos Cardiovasculares/efeitos adversos , Procedimentos Cirúrgicos Cardiovasculares/métodos , Permeabilidade do Canal Arterial/complicações , Permeabilidade do Canal Arterial/epidemiologia , Permeabilidade do Canal Arterial/fisiopatologia , Permeabilidade do Canal Arterial/cirurgia , Ecocardiografia/métodos , Feminino , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/patologia , Ventrículos do Coração/fisiopatologia , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Masculino , Tamanho do Órgão , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/epidemiologia , Implantação de Prótese/efeitos adversos , Implantação de Prótese/instrumentação , Implantação de Prótese/métodos , Estudos Retrospectivos , Resultado do TratamentoRESUMO
BACKGROUND: Hypertrophic cardiomyopathy is the leading cause of sudden cardiac death (SCD) in children and young adults. Our objective was to develop and validate a SCD risk prediction model in pediatric hypertrophic cardiomyopathy to guide SCD prevention strategies. METHODS: In an international multicenter observational cohort study, phenotype-positive patients with isolated hypertrophic cardiomyopathy <18 years of age at diagnosis were eligible. The primary outcome variable was the time from diagnosis to a composite of SCD events at 5-year follow-up: SCD, resuscitated sudden cardiac arrest, and aborted SCD, that is, appropriate shock following primary prevention implantable cardioverter defibrillators. Competing risk models with cause-specific hazard regression were used to identify and quantify clinical and genetic factors associated with SCD. The cause-specific regression model was implemented using boosting, and tuned with 10 repeated 4-fold cross-validations. The final model was fitted using all data with the tuned hyperparameter value that maximizes the c-statistic, and its performance was characterized by using the c-statistic for competing risk models. The final model was validated in an independent external cohort (SHaRe [Sarcomeric Human Cardiomyopathy Registry], n=285). RESULTS: Overall, 572 patients met eligibility criteria with 2855 patient-years of follow-up. The 5-year cumulative proportion of SCD events was 9.1% (14 SCD, 25 resuscitated sudden cardiac arrests, and 14 aborted SCD). Risk predictors included age at diagnosis, documented nonsustained ventricular tachycardia, unexplained syncope, septal diameter z-score, left ventricular posterior wall diameter z score, left atrial diameter z score, peak left ventricular outflow tract gradient, and presence of a pathogenic variant. Unlike in adults, left ventricular outflow tract gradient had an inverse association, and family history of SCD had no association with SCD. Clinical and clinical/genetic models were developed to predict 5-year freedom from SCD. Both models adequately discriminated between patients with and without SCD events with a c-statistic of 0.75 and 0.76, respectively, and demonstrated good agreement between predicted and observed events in the primary and validation cohorts (validation c-statistic 0.71 and 0.72, respectively). CONCLUSION: Our study provides a validated SCD risk prediction model with >70% prediction accuracy and incorporates risk factors that are unique to pediatric hypertrophic cardiomyopathy. An individualized risk prediction model has the potential to improve the application of clinical practice guidelines and shared decision making for implantable cardioverter defibrillator insertion. Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT0403679.
Assuntos
Cardiomiopatia Hipertrófica/epidemiologia , Morte Súbita Cardíaca/epidemiologia , Modelos Estatísticos , Adolescente , Fatores Etários , Algoritmos , Cardiomiopatia Hipertrófica/complicações , Criança , Morte Súbita Cardíaca/etiologia , Feminino , Humanos , Masculino , Vigilância em Saúde Pública , Reprodutibilidade dos Testes , Estudos Retrospectivos , Medição de Risco , Fatores de RiscoAssuntos
Cateterismo Cardíaco , Cardiologistas/educação , Cardiologia/educação , Educação de Pós-Graduação em Medicina , Cardiopatias Congênitas/terapia , Internato e Residência , Radiologia Intervencionista/educação , Sobreviventes , Competência Clínica , Consenso , Continuidade da Assistência ao Paciente , Currículo , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/fisiopatologia , HumanosRESUMO
Computational fluid dynamics (CFD) can be used to analyze blood flow and to predict hemodynamic outcomes after interventions for coarctation of the aorta and other cardiovascular diseases. We report the first use of cardiac 3-dimensional rotational angiography for CFD and show not only feasibility but also validation of its hemodynamic computations with catheter-based measurements in three patients.
Assuntos
Angioplastia com Balão , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/terapia , Aortografia , Hemodinâmica , Imageamento Tridimensional , Modelos Cardiovasculares , Modelagem Computacional Específica para o Paciente , Adolescente , Angioplastia com Balão/instrumentação , Coartação Aórtica/fisiopatologia , Criança , Estudos de Viabilidade , Feminino , Humanos , Hidrodinâmica , Masculino , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Stents , Resultado do TratamentoRESUMO
OBJECTIVES: To investigate whether age and valve size at implant contribute to outcomes after Melody transcatheter pulmonary valve replacement (TPVR). BACKGROUND: Patient age and valve size at implant contribute to longevity of surgical pulmonary valves. METHODS: All patients discharged with a Melody valve in the pulmonary position, as part of three prospective Melody valve multicenter studies, comprised the study cohort. Acute and time-related outcomes were analyzed according to age: children (≤12 years), adolescents (13-18 years), young adults (19-29 years), and older adults (≥30 years). RESULTS: Successful Melody valve implantation occurred in 49 children, 107 adolescents, 96 young adults, and 57 older adults. Pediatric patients (≤18 years) were more likely to have TPVR for conduit stenosis than adults (62% vs. 44%); children had the smallest conduits. After TPVR, pediatric and adult patients had similar decreases in right ventricular (RV) size by MRI, but adults had improved percentage predicted peak VO2 (58% preimplant to 64% postimplant, p = .02) and FEV1 (69% pre to 71% post, p = .005). Younger age was associated with shorter freedom from RVOT dysfunction, reintervention, and explant. Children had the shortest freedom from endocarditis (p = .041), but all other groups had 5-year freedom from endocarditis of ≥90%. CONCLUSIONS: Younger age was associated with shorter time to RVOT dysfunction, reintervention, and explant after Melody TPVR. Patients ≥13 years of age were at low risk for endocarditis and explant to 5 years. A better understanding of time-related outcomes by age will aid in the comparison of therapeutic options for TPVR candidates. CLINICAL TRIAL REGISTRATION: https://clinicaltrials.gov/ct2/show/NCT00740870 (NCT00740870), https://clinicaltrials.gov/ct2/show/NCT01186692 (NCT01186692), and https://clinicaltrials.gov/ct2/show/NCT00688571 (NCT00688571).
Assuntos
Cateterismo Cardíaco/instrumentação , Implante de Prótese de Valva Cardíaca/instrumentação , Próteses Valvulares Cardíacas , Valva Pulmonar/cirurgia , Adolescente , Fatores Etários , Canadá , Cateterismo Cardíaco/efeitos adversos , Criança , Ensaios Clínicos como Assunto , Bases de Dados Factuais , Remoção de Dispositivo , Endocardite/etiologia , Endocardite/fisiopatologia , Endocardite/cirurgia , Europa (Continente) , Feminino , Implante de Prótese de Valva Cardíaca/efeitos adversos , Hemodinâmica , Humanos , Masculino , Intervalo Livre de Progressão , Desenho de Prótese , Falha de Prótese , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/fisiopatologia , Reoperação , Fatores de Risco , Fatores de Tempo , Estados Unidos , Obstrução do Fluxo Ventricular Externo/etiologia , Obstrução do Fluxo Ventricular Externo/fisiopatologia , Obstrução do Fluxo Ventricular Externo/cirurgia , Adulto JovemRESUMO
OBJECTIVES: Cardiac catheterisation is commonly used for diagnosis and therapeutic interventions in paediatric cardiology. The inherent risk of the procedure can result in unanticipated admissions to critical care. Our goals were to provide a qualitative description of characteristics and evaluation of children admitted unexpectedly to the cardiac critical care unit (CCCU). METHODS: A retrospective single centre review of cardiac catheterisation procedures was done between 1 January, 2003 and 30 April, 2013. RESULTS: Of 9336 cardiac catheterisations performed, 146 (1.6%) were admitted from the catheterisation laboratory to the CCCU and met inclusion criteria. Of these 146 patients, 117 (1.3%) met criteria for unexpected admission and 29 (0.3%) were planned admissions. The majority admitted unexpectedly were below 1 year of age without co-morbidity aside from heart disease. Patients with planned admissions were significantly more likely to have single ventricle physiology, undergoing angiography or transferred for observation. Most unplanned admissions were triggered by interventional catheterisations or procedure-related complications. Patients received mechanical ventilation as the main CCCU management. Eighteen patients needed either cardiopulmonary resuscitation and/or extracorporeal membrane oxygenation during their catheterisation. About 106/117 (90.6%) patients survived to hospital discharge with no deaths in the planned admission group. CONCLUSIONS: Admission to CCCU following cardiac catheterisation was uncommon and tended to occur in younger children undergoing interventional procedures. Outcomes did not differ between patients experiencing planned and unplanned CCCU admission. Ongoing development of risk stratification tools may help to decrease unplanned CCCU admissions. Further studies are needed to determine whether unplanned admission following paediatric cardiac catheterisation should be utilised as a quality indicator.
Assuntos
Cateterismo Cardíaco , Cuidados Críticos/métodos , Cardiopatias/diagnóstico , Unidades de Terapia Intensiva Pediátrica/tendências , Admissão do Paciente/estatística & dados numéricos , Medição de Risco/métodos , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Cardiopatias/epidemiologia , Humanos , Lactente , Recém-Nascido , Masculino , Morbidade/tendências , Ontário/epidemiologia , Estudos RetrospectivosRESUMO
BACKGROUND: Novel quantification of stroke volume according to mitral inflow and aortic outflow using automated real-time 3-dimensional volume colour flow Doppler echocardiography (3D-RT-VCFDE) is more accurate than 2-dimensional echocardiography and has excellent correlation with cardiac magnetic resonance imaging-based flows in adults. This technology is applied for the first time to the right heart and in children. METHODS: 3D-RT-VCFDE was performed in 61 image sets of flow through the aortic (AV), mitral (MV), pulmonary (PV), and tricuspid (TV) valves of 34 children. These were compared with stroke volumes of the right (RV) and left (LV) ventricles and ratio of pulmonary to systemic blood flow determined using the Fick method in 31 children with atrial shunts. RESULTS: The mean age was 8.0 ± 3.3 years, and the mean weight was 27.8 ± 10.0 kg. The mean temporal resolution for flow analyses was ≥ 22 volumes per second. In conditions with no shunt, the correlations were: AV with MV flows (r = 0.98), PV with TV flows (r = 0.96), RV stroke volume with PV flow (r = 0.95), and with TV flow (r = 0.93), LV stroke volume with AV flow (r = 0.87), and with MV flow (r = 0.89). Fick ratio of pulmonary to systemic blood flow correlations were: PV/AV ratio (r = 0.84), TV/MV ratio (r = 0.87), and RV/LV ratio (r = 0.70). CONCLUSIONS: Stroke volume determined using automated 3D-RT-VCFDE is feasible in children and in the right side of the heart. This technique potentially provides a noninvasive alternative to historically invasively acquired hemodynamic data and to cardiac magnetic resonance imaging.
Assuntos
Ecocardiografia Tridimensional/métodos , Valvas Cardíacas , Ventrículos do Coração , Hemodinâmica/fisiologia , Criança , Pré-Escolar , Precisão da Medição Dimensional , Ecocardiografia Doppler em Cores/métodos , Feminino , Valvas Cardíacas/diagnóstico por imagem , Valvas Cardíacas/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Interpretação de Imagem Assistida por Computador/métodos , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Reprodutibilidade dos TestesRESUMO
OBJECTIVES: This study sought to obtain in vivo data to confirm assumptions on device loading conditions and assess procedural feasibility, safety, and valve performance. BACKGROUND: The Harmony transcatheter pulmonary valve (Medtronic, Minneapolis, Minnesota) was designed for patients with severe pulmonary regurgitation who require pulmonary valve replacement. METHODS: Three sites participated in this first Food and Drug Administration-approved early feasibility study using an innovative device design to accommodate the complex anatomy of the right ventricular outflow tract. Potentially eligible patients underwent review by a screening committee to determine implant eligibility. Six-month outcomes are reported. RESULTS: Between May 2013 and May 2015, 66 subjects were enrolled, and 21 were approved for implant and underwent catheterization; 20 were implanted. Catheterized patients had a median age of 25 years, were predominantly diagnosed with tetralogy of Fallot (95%), had severe pulmonary regurgitation (95%), and had trivial or mild stenosis. The device was delivered in the desired location in 19 of 20 (95%) patients. Proximal migration occurred in 1 patient during delivery system removal. Two devices were surgically explanted. Premature ventricular contractions related to the procedure were reported in 3 patients; 2 were resolved without treatment. One patient had ventricular arrhythmias that required treatment and later were resolved. At 1 month, echocardiography revealed none or trivial pulmonary regurgitation in all and a mean right ventricular outflow tract gradient of 16 ± 8 mm Hg (range 6 to 31 mm Hg). CONCLUSIONS: In this feasibility study of the Harmony transcatheter pulmonary valve device, there was high procedural success and safety, and favorable acute device performance.