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1.
J Am Heart Assoc ; 13(14): e033068, 2024 Jul 16.
Artículo en Inglés | MEDLINE | ID: mdl-38958142

RESUMEN

BACKGROUND: Reinterventions may influence the outcomes of children with functionally single-ventricle (f-SV) congenital heart disease. METHODS AND RESULTS: We undertook a retrospective cohort study of children starting treatment for f-SV between 2000 and 2018 in England, using the national procedure registry. Patients were categorized based on whether they survived free of transplant beyond 1 year of age. Among patients who had transplant-free survival beyond 1 year of age, we explored the relationship between reinterventions in infancy and the outcomes of survival and Fontan completion, adjusting for complexity. Of 3307 patients with f-SV, 909 (27.5%), had no follow-up beyond 1 year of age, among whom 323 (35.3%) had ≥1 reinterventions in infancy. A total of 2398 (72.5%) patients with f-SV had transplant-free survival beyond 1 year of age, among whom 756 (31.5%) had ≥1 reinterventions in infancy. The 5-year transplant-free survival and cumulative incidence of Fontan, among those who survived infancy, were 93.4% (95% CI, 92.4%-94.4%) and 79.3% (95% CI, 77.4%-81.2%), respectively. Both survival and Fontan completion were similar for those with a single reintervention and those who had no reinterventions. Patients who had >1 additional surgery (adjusted hazard ratio, 3.93 [95% CI, 1.87-8.27] P<0.001) had higher adjusted risk of mortality. Patients who had >1 additional interventional catheter (adjusted subdistribution hazard ratio, 0.71 [95% CI, 0.52-0.96] P=0.03) had a lower likelihood of achieving Fontan. CONCLUSIONS: Among children with f-SV, the occurrence of >1 reintervention in the first year of life, especially surgical reinterventions, was associated with poorer prognosis later in childhood.


Asunto(s)
Cuidados Paliativos , Reoperación , Humanos , Masculino , Inglaterra/epidemiología , Femenino , Estudios Retrospectivos , Gales/epidemiología , Lactante , Preescolar , Reoperación/estadística & datos numéricos , Trasplante de Corazón/estadística & datos numéricos , Sistema de Registros , Procedimiento de Fontan/mortalidad , Corazón Univentricular/cirugía , Corazón Univentricular/mortalidad , Corazón Univentricular/fisiopatología , Ventrículos Cardíacos/anomalías , Ventrículos Cardíacos/cirugía , Ventrículos Cardíacos/fisiopatología , Recién Nacido , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/mortalidad , Factores de Tiempo , Resultado del Tratamiento
3.
J Child Health Care ; 27(2): 197-211, 2023 06.
Artículo en Inglés | MEDLINE | ID: mdl-36165269

RESUMEN

Mainstream law and ethics literature on consent to children's surgery contrasts with moral experiences of children and adults observed in two heart surgery centres. Research interviews were conducted with 45 practitioners and related experts, and with 16 families of children aged 6 to 15, admitted for non-urgent surgery, as well as an online survey. Thematic data analysis was informed by critical realism and childhood studies.Impersonal adult-centric mainstream literature assumes young children cannot consent. It is based on dichotomies: adult/child, competent/incompetent, respect or protect children, inform or distract them, use time swiftly or flexibly, verbal/non-verbal communication, respect or control children and reason/emotion.Through their moral experiences, adults and children resolve these seeming dichotomies. Through understanding young children's reasoning and emotions about complex distressing decisions related to heart surgery, adults share knowledge, control, trust and respect with them. They see children's consent or refusal before non-urgent surgery as a shared personal moral experience within the child's life course, beyond mere legal compliance. Adults help children to understand and 'want' the surgery that offers things they value: better health or to 'be more like their friends'. If children are not convinced, sometimes surgery is postponed or occasionally cancelled.


Asunto(s)
Procedimientos Quirúrgicos Cardíacos , Principios Morales , Adulto , Niño , Humanos , Preescolar
4.
J Thorac Cardiovasc Surg ; 166(2): 306-316.e3, 2023 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-36535820

RESUMEN

OBJECTIVES: Long-term survival is an important metric for health care evaluation, especially in functionally single-ventricle (f-SV) congenital heart disease (CHD). This study's aim was to evaluate the relationship between center volume and long-term survival in f-SV CHD within the centralized health care service of England and Wales. METHODS: This was a retrospective cohort study of children born with f-SV CHD between 2000 and 2018, using the national CHD procedure registry, with survival ascertained in 2020. RESULTS: Of 56,039 patients, 3293 (5.9%) had f-SV CHD. Median age at first intervention was 7 days (interquartile range [IQR], 4, 27), and median follow-up time was 7.6 years (IQR, 1.0, 13.3). The largest diagnostic subcategories were hypoplastic left heart syndrome, 1276 (38.8%); tricuspid atresia, 440 (13.4%); and double-inlet left ventricle, 322 (9.8%). The survival rate at 1 year and 5 years was 76.8% (95% confidence interval [CI], 75.3%-78.2%) and 72.1% (95% CI, 70.6%-73.7%), respectively. The unadjusted hazard ratio for each 5 additional patients with f-SV starting treatment per center per year was 1.04 (95% CI, 1.02-1.06), P < .001. However, after adjustment for significant risk factors (diagnostic subcategory; antenatal diagnosis; younger age, low weight, acquired comorbidity, increased severity of illness at first procedure), the hazard ratio for f-SV center volume was 1.01 (95% CI, 0.99-1.04) P = .28. There was strong evidence that patients with more complex f-SV (hypoplastic left heart syndrome, Norwood pathway) were treated at centers with greater f-SV case volume (P < .001). CONCLUSIONS: After adjustment for case mix, there was no evidence that f-SV center volume was linked to longer-term survival in the centralized health service provided by the 10 children's cardiac centers in England and Wales.


Asunto(s)
Cardiopatías Congénitas , Síndrome del Corazón Izquierdo Hipoplásico , Atresia Tricúspide , Embarazo , Niño , Humanos , Femenino , Recién Nacido , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Gales/epidemiología , Estudios Retrospectivos , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/cirugía , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/cirugía , Ventrículos Cardíacos/anomalías , Inglaterra/epidemiología , Resultado del Tratamiento
5.
Eur J Cardiothorac Surg ; 62(2)2022 07 11.
Artículo en Inglés | MEDLINE | ID: mdl-35640135

RESUMEN

OBJECTIVES: There is increasing evidence that performing superior cavopulmonary connection (SCPC) at 3 months reduces mortality: reducing the risky 'interstage' period, volume off-loading the ventricle and reducing coronary steal, hopefully preserving ventricular function. Our objective was to describe our experience of early SCPC with preoperative computer tomography (CT) assessment compared to later connection at 6 months. METHODS: Patients undergoing Norwood procedure from 2005 to 2020 were divided into 2 eras were described and compared. Era 1 from 2005 to 2016 when SCPC was undertaken at 6 months: and era 2 (2017-2020) when an earlier operation was performed. Demographics, mortality (interstage, early and late following SCPC) and data on postoperative course and complications were recorded. RESULTS: In era 1, 191 patients underwent Norwood (120 survivors to SCPC) and 28 patients (23 survivors) in era 2. There were no significant differences in the demographics. Interstage mortality was 17.8% in era 1 and 8.0% in era 2 but not significantly significant (P = 0.22). The median (interquartile range) age at pre-imaging and SCPC was significantly lower: 99 (81-120) vs 77 (47-102) days and 175 (117-208) vs 106 (102-122) days in era 1 vs era 2 (P < 0.005). Weight was lower at SCPC in era 2 [mean (standard deviation) 6.2 kg (1.2) vs 5.1 kg (0.8), P < 0.05]. Intubation time and total length of stay were not statistically different. Median intensive care unit stay was statistically significantly longer, but not clinically significant: 2.5 (2-4) vs 3 (3-5) days, respectively (P < 0.05). There was no significant difference in early or late mortality, rates of diagnostic or interventional catheter, postoperative magnetic resonance imaging/CT or stroke. Logistic regression analysis demonstrates increasing age at SCPC was associated with increased chance of stroke or early death (P = 0.043). CONCLUSIONS: Early SCPC with CT assessment is feasible and although intensive care unit length of stay was slightly longer there was no change in the overall length of stay and no change in postoperative mortality or complications.


Asunto(s)
Síndrome del Corazón Izquierdo Hipoplásico , Procedimientos de Norwood , Accidente Cerebrovascular , Computadores , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/diagnóstico por imagen , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Lactante , Procedimientos de Norwood/efectos adversos , Procedimientos de Norwood/métodos , Cuidados Paliativos/métodos , Estudios Retrospectivos , Tomografía , Tomografía Computarizada por Rayos X , Resultado del Tratamiento
6.
Nurs Ethics ; 29(4): 1078-1090, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35212562

RESUMEN

BACKGROUND: The law and literature about children's consent generally assume that patients aged under-18 cannot consent until around 12 years, and cannot refuse recommended surgery. Children deemed pre-competent do not have automatic rights to information or to protection from unwanted interventions. However, the observed practitioners tend to inform young children s, respect their consent or refusal, and help them to "want" to have the surgery. Refusal of heart transplantation by 6-year-olds is accepted. RESEARCH QUESTION: What are possible reasons to explain the differences between theories and practices about the ages when children begin to be informed about elective heart surgery, and when their consent or refusal begins to be respected? RESEARCH DESIGN, PARTICIPANTS AND CONTEXT: Research methods included reviews of related healthcare, law and ethics literature; observations and conversations with staff and families in two London hospitals; audio-recorded semi-structured interviews with a purposive sample of 45 healthcare professionals and related experts; interviews and a survey with parents and children aged 6- to 15-years having elective surgery (not reported in this paper); meetings with an interdisciplinary advisory group; thematic analysis of qualitative data and co-authorship of papers with participants. ETHICAL CONSIDERATIONS: Approval was granted by four research ethics committees/authorities. All interviewees gave their informed written consent. FINDINGS: Interviewees explained their views and experiences about children's ages of competence to understand and consent or refuse, analysed by their differing emphases on informed, signified or voluntary consent. DISCUSSION: Differing views about children's competence to understand and consent are associated with emphases on consent as an intellectual, practical and/or emotional process. Conclusion: Greater respect for children's practical signified, emotional voluntary and intellectual informed consent can increase respectful understanding of children's consent. Nurses play a vital part in children's practitioner-patient relationships and physical care and therefore in all three elements of consent.


Asunto(s)
Procedimientos Quirúrgicos Cardíacos , Consentimiento Informado , Adolescente , Niño , Comunicación , Humanos , Padres/psicología , Encuestas y Cuestionarios
10.
Echo Res Pract ; 6(3): 63-70, 2019 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-31413860

RESUMEN

BACKGROUND: The Technical Performance Score (TPS) developed by Boston Children's Hospital showed surgical outcomes correlate with adequacy of technical repair when implemented on pre-discharge echocardiograms. We applied this scoring system to intraoperative imaging in a tertiary UK congenital heart surgical centre. METHODS: After a period of training, intraoperative TPS (epicardial and/or transesophageal echocardiography) was instituted. TPS was used to inform intraoperative discussions and recorded on a custom-made database using the previously published scoring system. After a year, we reviewed the feasibility, results and relationship between the TPS and mortality, extubation time and length of stay. RESULTS: From 01 September 2015 to 04 July 2016, there were 272 TPS procedures in 251 operations with 208 TPS recorded. Seven patients had surgery with no documented TPS, three had operations with no current TPS score template available. Patients left the operating theatre with TPS optimal in 156 (75%), adequate 34 (16%) and inadequate 18 (9%). Of those with an optimal score on leaving theatre, ten had more than one period of cardiopulmonary bypass. All four deaths <30 days after surgery (1.9%) had optimal TPS. There was a statistically significant difference in extubation times in the RACHS category 4 patients (3 days vs 5 days, P < 0.05) and in PICU and total length of stay in the RACHS category three patients (2 and 8 days vs 12.5 and 21.5 days respectively) if leaving theatre with an inadequate result. CONCLUSIONS: Application of intraoperative TPS is feasible and provides a way of objectively recording intraoperative imaging assessment of surgery. An 'inadequate' TPS did not predict mortality but correlated with a longer ventilation time and longer length of stay compared to those with optimal or adequate scores.

11.
Heart ; 104(3): 244-252, 2018 02.
Artículo en Inglés | MEDLINE | ID: mdl-28754809

RESUMEN

OBJECTIVE: Norwood and hybrid procedure are two options available for initial palliation of patients with hypoplastic left heart syndrome (HLHS). Our study aimed to assess potential differences in right ventricular (RV) function and pulmonary artery dimensions using cardiac magnetic resonance (CMR) in survivors with HLHS. METHODS: 42 Norwood (mean age 2.4±0.8) and 44 hybrid (mean age 2.0±1.0 years) patients were evaluated by CMR after stage II palliation prior to planned Fontan completion. Initial stage I Norwood procedure was performed using a modified Blalock-Taussig shunt, while the hybrid procedure consisted of bilateral pulmonary artery banding and arterial duct stenting. Need for reinterventions and subsequent outcomes were also assessed. RESULTS: Norwood patients had larger RV end-diastolic dimensions (91±23 vs 80±31 mL/m2, p=0.004) and lower heart rate (90±15 vs 102±13, p<0.001) than hybrid patients. Both Norwood and hybrid patients showed preserved global RV pump function (59±9 vs 59%±10%, p=0.91), while RV strain, strain rate and intraventricular synchrony were superior in the Norwood group. Pulmonary artery size was reduced (lower lobe index 135±74 vs 161±62 mm2/m2, p=0.02), and reintervention rate was significantly higher in the hybrid group whereas subsequent outcome did not differ significantly (p=0.24). CONCLUSIONS: Norwood and hybrid strategy were associated with equivalent and preserved global RV pump function while development of the pulmonary arteries and reintervention rate were superior using the Norwood approach. Impaired RV myocardial deformation as a potential marker of early RV dysfunction in the hybrid group may have a negative long-term impact in this population.


Asunto(s)
Procedimiento de Blalock-Taussing/métodos , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Procedimientos de Norwood/métodos , Arteria Pulmonar/patología , Función Ventricular Derecha/fisiología , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/patología , Preescolar , Femenino , Procedimiento de Fontan , Ventrículos Cardíacos/patología , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/diagnóstico por imagen , Síndrome del Corazón Izquierdo Hipoplásico/patología , Síndrome del Corazón Izquierdo Hipoplásico/fisiopatología , Lactante , Estimación de Kaplan-Meier , Angiografía por Resonancia Magnética/métodos , Masculino , Cuidados Paliativos/métodos , Arteria Pulmonar/diagnóstico por imagen , Reoperación/métodos
12.
Cardiol Young ; 27(1): 131-138, 2017 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-27055807

RESUMEN

Introduction The hybrid procedure is one mode of initial palliation for hypoplastic left heart syndrome. Subsequently, patients proceed with either the "three-stage" pathway - comprehensive second stage followed by Fontan completion - or the "four-stage" pathway - Norwood procedure, hemi-Fontan, or Fontan completion. In this study, we describe somatic growth patterns observed in the hybrid groups and a comparison primary Norwood group. METHODS: A retrospective analysis of patients who have undergone hybrid procedure and Fontan completion was performed. Weight-for-age and height-for-age z-scores were recorded at each operation. RESULTS: We identified 13 hybrid patients - eight in the three-stage pathway and five in the four-stage pathway - and 49 Norwood patients. Weight: three stage: weight decreased from hybrid procedure to comprehensive second stage (-0.4±1.3 versus -2.3±1.4, p<0.01) and then increased to Fontan completion (-0.4±1.5 versus -0.6±1.4, p<0.01); four stage: weight decreased from hybrid procedure to Norwood (-2.0±1.4 versus -3.3±0.9, p=0.06), then stabilised to hemi-Fontan. Weight increased from hemi-Fontan to Fontan completion (-2.7±0.6 versus -1.0±0.7, p=0.01); primary Norwood group: weight decreased from Norwood to hemi-Fontan (p<0.001) and then increased to Fontan completion (p<0.001). Height: height declined from hybrid procedure to Fontan completion in the three-stage group. In the four-stage group, height decreased from hybrid to hemi-Fontan, and then increased to Fontan completion. The Norwood group decreased in height from Norwood to hemi-Fontan, followed by an increase to Fontan completion. CONCLUSION: In this study, we show that patients undergoing the hybrid procedure have poor weight gain before superior cavopulmonary connection, before returning to baseline by Fontan completion. This study identifies key periods to target poor somatic growth, a risk factor of morbidity and worse neurodevelopmental outcomes.


Asunto(s)
Desarrollo Infantil/fisiología , Procedimiento de Fontan/métodos , Síndrome del Corazón Izquierdo Hipoplásico/fisiopatología , Procedimientos de Norwood/métodos , Cuidados Paliativos , Femenino , Estudios de Seguimiento , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Lactante , Masculino , Periodo Posoperatorio , Estudios Retrospectivos , Resultado del Tratamiento
13.
Eur J Cardiothorac Surg ; 51(1): 50-57, 2017 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-27422888

RESUMEN

OBJECTIVES: The Norwood procedure for hypoplastic left heart syndrome (HLHS) is performed either via a right ventricle-to-pulmonary artery (RVPA) conduit or a modified Blalock-Taussig (MBT) shunt. Cardiac magnetic resonance (CMR) data was used to assess the effects of the RVPA conduit on ventricular shape and function through a computational analysis of anatomy and assessment of indices of strain. METHODS: A retrospective analysis of 93 CMR scans of subjects with HLHS was performed (59 with MBT shunt, 34 with RVPA conduit), incorporating data at varying stages of surgery from two congenital centres. Longitudinal and short-axis cine images were used to create a computational cardiac atlas and assess global strain. RESULTS: Those receiving an RVPA conduit had significant differences (P< 0.0001) in the shape of the RV corresponding to increased ventricular dilatation (P = 0.001) and increased sphericity (P = 0.006). Differences were evident only following completion of stage II surgery. Despite preserved ejection fraction in both groups, functional strain in the RVPA conduit group compared with that in the MBT shunt group was reduced across multiple ventricular axes, including a reduced systolic longitudinal strain rate (P< 0.0001), reduced diastolic longitudinal strain rate (P = 0.0001) and reduced midventricular systolic circumferential strain (P < 0.0001). CONCLUSIONS: Computational modelling analysis reveals differences in ventricular remodelling in patients with HLHS undergoing an RVPA conduit insertion with focal scarring and volume loading leading to decreased functional markers of strain. The need for continued surveillance is warranted, as deleterious effects may not become apparent until later years.


Asunto(s)
Procedimiento de Blalock-Taussing/métodos , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/cirugía , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Arteria Pulmonar/cirugía , Función Ventricular Derecha/fisiología , Remodelación Ventricular/fisiología , Anastomosis Quirúrgica/métodos , Femenino , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/diagnóstico , Síndrome del Corazón Izquierdo Hipoplásico/fisiopatología , Recién Nacido , Imagen por Resonancia Cinemagnética , Masculino , Procedimientos de Norwood/métodos , Estudios Retrospectivos , Resultado del Tratamiento
14.
Int J Pediatr Otorhinolaryngol ; 83: 57-62, 2016 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-26968054

RESUMEN

INTRODUCTION: Paediatric tracheobronchomalacia is a rare but potentially serious condition. Severe tracheobronchomalacia requires intervention or operation. This is an evaluation of a ten-year experience at an institution. METHODS: In this retrospective study all patients were included that required an intervention for severe tracheobronchomalacia from 2003 to 2012. Symptoms, aetiology, comorbidities, localisation of the malacia, age at diagnosis, therapeutic measures and associated complications were evaluated. RESULTS: Forty-four patients with severe tracheobronchomalacia underwent intervention/operation. The predominant aetiology was vascular compression in 48%. The majority of patients had complex comorbidities, most importantly cardiac pathology in 66%. The median age at diagnosis was 3 months. A total of 17 aortopexies, 21 tracheostomies and 25 stent placements were performed. The mean follow-up was 2.6 years. Severe complications occurred in 12 patients. The most common complications were stent obstruction/fracture and tracheostomy tube obstruction. CONCLUSION: The management of severe tracheobronchomalacia is complex and the population of patients is very heterogeneous. Therefore the treatment has to be adapted for each patient individually. The decision strategies are discussed in this article. The surgical techniques for placement and safe removal of expandable bare metallic stents employed in our institution are presented. A multidisciplinary team of ENT surgeons, Intensivists, Cardiologists and Cardiac surgeons is of great importance.


Asunto(s)
Stents/efectos adversos , Traqueobroncomalacia/cirugía , Traqueostomía/métodos , Adolescente , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Masculino , Complicaciones Posoperatorias/epidemiología , Estudios Retrospectivos , Traqueobroncomalacia/complicaciones , Traqueostomía/efectos adversos
15.
Eur Heart J Cardiovasc Imaging ; 17(6): 687-92, 2016 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-26323279

RESUMEN

AIMS: Velocity vector imaging (VVI) is an echocardiographic technique based on speckle tracking, which has been validated for the left ventricle (LV). It has not been validated to assess the systemic right ventricle (RV) in patients with hypoplastic left heart syndrome (HLHS). The aim of this study was to evaluate whether VVI measurements reliably reflect RV systolic function in patients with HLHS when compared with RV ejection fraction (EF) calculated using magnetic resonance imaging (MRI). METHODS AND RESULTS: In this prospective study, 49 children with HLHS underwent transthoracic echocardiography and cardiac MRI under the same general anaesthetic as a part of routine assessment between the different stages of palliative surgery. Global RV fractional area change (FAC-VVI), strain (S), strain rate (SR), and peak systolic velocity (V) were analysed from the apical four-chamber view using the VVI technique. MRI-derived EF was calculated from a short-axis cine stack of images. Intra- and interobserver reproducibility was excellent for all VVI parameters (intraclass correlation coefficient >0.9). All VVI-derived parameters, except myocardial velocity, correlated with MRI-derived EF (FAC-VVI: R = 0.7, P < 0.001; S: R = -0.5, P < 0.001; SR: R = 0.5, P = 0.001, and V: R = 0.1, P = 0.4). CONCLUSIONS: All VVI-derived parameters, except V, correlate with MRI-derived EF, with FAC being the best predictor of it. Reproducibility of all VVI parameters is excellent. VVI provides a useful tool for the follow-up of RV function during the staged treatment protocol for HLHS.


Asunto(s)
Ecocardiografía Doppler/métodos , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Procesamiento de Imagen Asistido por Computador , Imagen por Resonancia Cinemagnética/métodos , Disfunción Ventricular Derecha/diagnóstico por imagen , Función Ventricular Derecha/fisiología , Velocidad del Flujo Sanguíneo , Niño , Preescolar , Intervalos de Confianza , Estudios Transversales , Femenino , Procedimiento de Fontan/métodos , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/diagnóstico por imagen , Lactante , Masculino , Variaciones Dependientes del Observador , Pronóstico , Estudios Prospectivos , Volumen Sistólico/fisiología
16.
Ann Thorac Surg ; 100(6): 2286-90; discussion 2291-2, 2015 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-26433522

RESUMEN

BACKGROUND: Hybrid procedure offers patients with severe congenital heart disease an alternative initial procedure to conventional surgical reconstruction. We report the midterm outcomes of a cohort of neonates who had a hybrid procedure for variants of hypoplastic left heart syndrome because they were at high risk for the Norwood procedure. METHODS: Between December 2005 and January 2013, 41 neonates underwent bilateral pulmonary artery banding followed by ductal stenting by means of a sternotomy at a median age of 6 days (range, 2 to 18 days) and weight of 2.6 kg (range, 1.5 to 3.7 kg). Thirty-five patients had hypoplastic left heart syndrome, and 6 patients had critical aortic stenosis with hypoplastic left ventricle. Primary indications for the hybrid procedure were low birth weight in 17 patients, hypoplastic left ventricle with the possibility of later biventricular repair in 6 patients, intact or near-intact atrial septum in 5 patients, and poor patient condition in 13 patients. Echocardiographic, angiographic, operative, and clinical data were reviewed. Outcomes were summarized with descriptive statistics and risk factors for mortality identified. RESULTS: All but 6 patients had an antenatal diagnosis, and 24 patients were from other congenital cardiac centers. Nine patients had perioperative balloon aortic valvuloplasty, 1 patient had fetal balloon aortic valvuloplasty, and 17 patients had intervention to their atrial septum (41.4%). There were 9 inpatient deaths (21.9%) and 4 interstage deaths (9.8%) after the hybrid procedure. Twenty-eight patients subsequently underwent either the Norwood procedure (11 patients), combined stage I and II (14 patients), or biventricular repair (3 patients). No patient had heart transplantation. Among the patients who had combined stage I and II as a second procedure after the hybrid procedure, there were 2 early deaths, 1 late death before the Fontan, and 1 late death after the Fontan completion after combined stage I and II. All patients who had subsequent Norwood procedure were midterm survivors. Three of the 4 patients who had biventricular repair were midterm survivors. Overall survival was 56.1% at a median follow-up of 32.0 months. By univariate analysis, patient factors, intact or near-intact atrial septum, and aortic atresia were associated with nonsurvival. CONCLUSIONS: Hybrid procedure as an alternative to the Norwood procedure offers good midterm survival in patients deemed at high risk for neonatal reconstruction.


Asunto(s)
Procedimientos Quirúrgicos Cardíacos/métodos , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Cateterismo Cardíaco , Contraindicaciones , Femenino , Estudios de Seguimiento , Humanos , Recién Nacido , Masculino , Procedimientos de Norwood , Estudios Retrospectivos , Factores de Riesgo , Factores de Tiempo , Resultado del Tratamiento
17.
J Cardiovasc Magn Reson ; 17: 28, 2015 Apr 14.
Artículo en Inglés | MEDLINE | ID: mdl-25890289

RESUMEN

BACKGROUND: Selection of patients with congenital heart disease for surgical septation in biventricular repair or surgical palliation in functionally single ventricles requires low pulmonary vascular resistance (PVR). Where there is uncertainty, PVR can be assessed using hybrid cardiovascular magnetic resonance (CMR) and fluoroscopic (X-Ray) guided cardiac catheterizations (XMR). CMR/XMR catheterization is a validated technique for accurate assessment of pulmonary vascular resistance. However, data concerning its application in clinical practice is lacking. METHODS: PVR assessments were performed in 167 studies in 149 congenital heart disease patients by CMR/XMR catheterization. Data was collated on patient demographics, procedural data, complications and outcomes. Institutional ethics approval was obtained. RESULTS: Median age was 3.6 years (6 days-67 years) and weight 13.8 kg (2.3-122 kg). One hundred and eight studies were in biventricular circulations and 59 in functionally single ventricles. Median radiation dose was 0.72 mSv. A baseline Qp:Qs ≤2.75 in biventricular circulations with left-to-right shunts predicted a PVR ≥6 WU x m(2) with 100% sensitivity and 48% specificity. Median follow up until death or last review was 4.2 years (4 days-11 years). Eighty-four patients had a surgical or catheter intervention based on CMR/XMR catheterization findings at a median of 94 days after the study. This included successful biventricular repair at resting PVR values ≤6 WU x m(2) and Fontan completion at ≤4 WU x m(2). CONCLUSION: PVR measured by CMR/XMR catheterization allows accurate stratification for intervention in patients with congenital heart disease in both, biventricular and univentricular circulations.


Asunto(s)
Cateterismo de Swan-Ganz/métodos , Cardiopatías Congénitas/diagnóstico , Imagen por Resonancia Magnética Intervencional , Arteria Pulmonar/fisiopatología , Circulación Pulmonar , Resistencia Vascular , Adolescente , Adulto , Anciano , Presión Arterial , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Niño , Preescolar , Femenino , Cardiopatías Congénitas/fisiopatología , Cardiopatías Congénitas/cirugía , Humanos , Lactante , Recién Nacido , Masculino , Persona de Mediana Edad , Selección de Paciente , Valor Predictivo de las Pruebas , Pronóstico , Curva ROC , Radiografía Intervencional , Factores de Riesgo , Adulto Joven
18.
Heart ; 100(10): 775-80, 2014 May.
Artículo en Inglés | MEDLINE | ID: mdl-24415666

RESUMEN

OBJECTIVE: The 'hybrid procedure', consisting of surgical banding of the pulmonary arteries with intraoperative stenting of the arterial duct, was developed as primary palliation in hypoplastic left heart syndrome (HLHS), avoiding the risks of cardiopulmonary bypass. In many centres, it is reserved for low birth weight, premature or unstable neonates; however, its role in such high risk cases of HLHS has yet to be defined. METHODS: The preoperative condition of all patients with HLHS who underwent either the hybrid or the Norwood procedure for HLHS between 2005-2011 was analysed retrospectively, using a modified comprehensive Aristotle score. We then compared operative, interstage and 1 year mortalities between the groups after Aristotle adjustment via Cox proportional hazards analyses. RESULTS: Of 138 patients with HLHS, 27 had hybrid and 111 Norwood procedures. The hybrid group had significantly higher Aristotle scores (mean 4.1 vs 1.8; p<0.001); however, there was no significant difference in mortality at any stage. At 1 year, the overall unadjusted survival among Norwood and hybrid patients was 58.6% and 51.9%, respectively, yielding an Aristotle adjusted hazard ratio for mortality among hybrid patients of 1.09 (95% CI 0.56 to 2.11, p=0.80). CONCLUSIONS: Applying a hybrid approach to high risk patients with HLHS produces a comparable early and interstage mortality risk to lower risk patients undergoing the Norwood procedure. Prospective studies are needed to establish whether the hybrid procedure is a viable alternative to the Norwood procedure in all HLHS patients in terms of both mortality and long term morbidity.


Asunto(s)
Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Procedimientos de Norwood , Cuidados Paliativos/métodos , Arteria Pulmonar/cirugía , Ecocardiografía , Femenino , Estudios de Seguimiento , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/diagnóstico , Síndrome del Corazón Izquierdo Hipoplásico/mortalidad , Mortalidad Infantil/tendencias , Recién Nacido , Masculino , Periodo Preoperatorio , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Factores de Riesgo , Índice de Severidad de la Enfermedad , Tasa de Supervivencia/tendencias , Resultado del Tratamiento , Reino Unido/epidemiología
19.
Ann Thorac Surg ; 94(5): 1562-9, 2012 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-22858280

RESUMEN

BACKGROUND: Pediatric patients with hypoplastic left heart syndrome rely solely on the right ventricle, resulting in anatomic maladaptations that can significantly compromise diastolic efficiency and lead to heart failure. Clinical indices to evaluate diastole are generally derived from the adult left ventricle, limiting their relevance to patients with hypoplastic left heart syndrome. We investigated the effect of the ventricular cavity shape and tricuspid inflow typology on the filling dynamics to provide new directions of investigation for assessing diastolic function in these patients. METHODS: Magnetic resonance imaging data were used to generate personalized mathematic models of 4 patients with different prognoses after stage I of the Norwood procedures. Two of these patients were also modeled after stage II. Numeric simulations were performed to analyze the interaction between blood flow and the myocardium during diastole. RESULTS: The filling dynamics were characterized by the formation of an organized structure of swirling blood (vortex ring). This was strongly influenced by the ventricular shape and the timing of the E and A wave. Biphasic rather than fused inflows and more elliptical than spherical cavities were found to increase the intraventricular pressure gradients and the filling capacity by optimizing the energy transfer between blood flow and the myocardium. This resulted in a better flow propagation and higher tissue velocities and displacements. CONCLUSIONS: The variations in the kinetic energy associated with the blood motion reflected the base-to-apex pressure difference and can therefore be used to quantify the efficiency of filling, providing a potential new metric to assess diastolic function in these patients.


Asunto(s)
Ventrículos Cardíacos/patología , Ventrículos Cardíacos/fisiopatología , Síndrome del Corazón Izquierdo Hipoplásico/fisiopatología , Humanos , Imagen por Resonancia Magnética , Modelos Cardiovasculares , Flujo Sanguíneo Regional
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