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1.
Congenit Heart Dis ; 14(5): 846-853, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31489769

RESUMO

OBJECTIVE: A significant body of patients who have undergone Mustard or Senning procedure require lifelong follow up. In this retrospective review, we examined the cohort of such patients currently attending our center. DESIGN: Patients who had undergone either Mustard or Senning procedure were identified. We retrospectively reviewed medical records, recorded demographic information and data regarding the clinical state, NHYA class, cardiopulmonary exercise testing, NT-proBNP measurement, and recent cardiac MRI findings. RESULTS: Forty-six patients were identified, the mean age was 32.2 years (± 6.1 years), 67.4% were male. Thirty-two patients (69.6%) had undergone a Senning procedure. The median length of the follow-up was 32 years. Thirty-two patients (69.6%) were NHYA class 1. The mean VO2max achieved was 24.2 ± 5.8 mL/min/kg. The mean NT-proBNP was 266.4 pg/mL (± 259.9 pg/mL). The mean right ventricular end-diastolic volume (RVEDV) was 212.4 mL ± 73.1 mL (indexed 114.2 mL/m2  ± 34.4 mL/m2 ). The mean right ventricular ejection fraction (RVEF) was 53.7% ± 7.9%. The mean left ventricular end-diastolic volume (LVEDV) was 161.5 mL ± 73.7 mL (indexed 87.8 mL/m2  ± 41.1 mL/m2 ). The mean left ventricular ejection fraction (LVEF) was 59.8% ± 5.7%. There was a significant correlation between right ventricular (RV) size on MRI and NT-proBNP level. CONCLUSIONS: We present a relatively well cohort of patients with overall favorable long-term outcome. The majority of patients are NHYA class 1 and the systemic right ventricular function appears to be well preserved as assessed by MRI. The exercise tolerance is reduced, with the majority of patients achieving around 60% of the estimated VO2max . Regular specialist follow-up and assessment with advanced imaging at regular intervals remain important for this group.


Assuntos
Transposição das Grandes Artérias/métodos , Tolerância ao Exercício/fisiologia , Previsões , Volume Sistólico/fisiologia , Transposição dos Grandes Vasos/cirurgia , Função Ventricular Esquerda/fisiologia , Função Ventricular Direita/fisiologia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Imagem Cinética por Ressonância Magnética , Masculino , Estudos Retrospectivos , Transposição dos Grandes Vasos/diagnóstico , Transposição dos Grandes Vasos/fisiopatologia , Resultado do Tratamento , Adulto Jovem
2.
J Radiol Prot ; 37(3): 742-760, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-28721947

RESUMO

OBJECTIVE: This study sought to determine if DNA integrity was compromised by ionising radiation from paediatric cardiac catheterisations and if dose optimisation techniques allowed DNA integrity to be maintained. MATERIALS AND METHODS: Children were imaged using either: (i) an anti-scatter grid (current departmental protocol), (ii) no anti-scatter grid or, (iii) no anti-scatter grid and a 15 cm air-gap between the child and the x-ray detector. Dose area product and image quality were assessed, lifetime attributable cancer risk estimates were calculated and DNA double-strand breakages quantified using the γH2AX assay. RESULTS: Consent was obtained from 70 parents/guardians/children. Image quality was sufficient for each procedure performed. Removal of the anti-scatter grid resulted in dose reductions of 20% (no anti-scatter grid) and 30% (15 cm air-gap), DNA double-strand break reductions of 30% (no anti-scatter grid) and 20% (15 cm air-gap) and a reduction of radiation-induced cancer mortality risk of up to 45%. CONCLUSION: Radiation doses received during paediatric cardiac catheterisation procedures resulted in a significant increase in DNA damage while maintaining acceptable image quality and diagnostic efficacy. It is feasible to remove the anti-scatter grid resulting in a reduction in DNA damage to the patient. The γH2AX assay may be used for assessment of dose optimisation strategies in children.


Assuntos
Cateterismo Cardíaco , Dano ao DNA/efeitos da radiação , Doses de Radiação , Proteção Radiológica/métodos , Espalhamento de Radiação , Adolescente , Criança , Pré-Escolar , Inglaterra , Feminino , Humanos , Lactente , Recém-Nascido , Masculino
3.
Cardiol Young ; 27(7): 1430-1433, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-28382884

RESUMO

We report the case of a young boy with a history of total occlusion of the superior caval vein, diagnosed early after complex neonatal cardiac surgery, who developed severe protein-losing enteropathy. Protein-losing enteropathy was precipitated by a relatively mild stenosis at the junction of the inferior caval vein with the right atrium. Percutaneous stent dilation of the veno-atrial junction definitively relieved the stenosis, and the protein-losing enteropathy subsequently resolved.


Assuntos
Enteropatias Perdedoras de Proteínas/etiologia , Stents , Síndrome da Veia Cava Superior/complicações , Síndrome da Veia Cava Superior/diagnóstico por imagem , Criança , Constrição Patológica/diagnóstico por imagem , Humanos , Masculino , Enteropatias Perdedoras de Proteínas/fisiopatologia , Síndrome da Veia Cava Superior/cirurgia , Tomografia Computadorizada por Raios X
5.
J Paediatr Child Health ; 50(6): 482-6, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-24528530

RESUMO

AIM: The aim of this study is to evaluate consultant general paediatricians' opinions of a UK paediatric telecardiology service. METHODS: A structured questionnaire was developed and sent to all consultant paediatricians working in a district general hospital in Northern Ireland. RESULTS: Paediatricians (n = 35) regarded the regional paediatric telecardiology service as very useful and of good value for money. Paediatricans in hospitals without access to telecardiology expressed a desire to join the network (86%, 12/14). More frequent use of the paediatric telecardiology service was associated with increased confidence in performing echocardiography and using the telemedicine equipment and a special interest in neonatology. The vast majority of paediatricians (32/35, 91%) believed that there should be a shared clinical responsibility for the patient following a teleconsultation. A total of 33/35 (94%) stated that the telephone costs of the consultation should be paid by the paediatrician but that the professional time of the cardiologist should be paid by the tertiary centre (29/35, 83%). CONCLUSIONS: Paediatricians have consistently positive experiences of a regional paediatric telecardiology service. They believe that clinical responsibility is shared, and there should not be any professional fee for telemedicine activities.


Assuntos
Atitude do Pessoal de Saúde , Cardiologia/métodos , Cardiopatias Congênitas/terapia , Inquéritos e Questionários , Telemedicina/organização & administração , Adulto , Criança , Pré-Escolar , Estudos Transversais , Feminino , Cardiopatias Congênitas/diagnóstico , Humanos , Lactente , Recém-Nascido , Masculino , Monitorização Fisiológica , Pediatria , Avaliação de Programas e Projetos de Saúde , Encaminhamento e Consulta/estatística & dados numéricos , Reino Unido
7.
Prenat Diagn ; 32(9): 883-7, 2012 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-22718083

RESUMO

OBJECTIVE: The aims of this study were to evaluate patients' opinions on a fetal cardiology telemedicine service compared with usual outpatient care, the effect of the telemedicine consultation on maternal anxiety and its impact on travel times and time absent from work. METHODS: Prospective study over 20 months. Eligible patients attended for routine anomaly scan followed by fetal echocardiogram transmitted to the regional centre with live guidance by a fetal cardiologist, followed by parental counselling. All patients were offered a fetal cardiology appointment at the regional centre. Structured questionnaires assessing maternal satisfaction, travel times/days off and anxiety scores completed at time of both fetal echocardiograms. RESULTS: Sixty-seven patients were recruited and 66 completed the study. Participants expressed very high satisfaction rates with fetal telecardiology, equivalent to face-to-face consultation. The telecardiology appointments were associated with significantly reduced travel times and days off work (p < 0.01). Expectant mothers expressed a clear inclination for a fetal cardiology appointment at the local hospital facilitated by telemedicine (p < 0.01). CONCLUSIONS: Fetal telecardiology is highly acceptable to patients and is even preferred compared with travelling to a regional centre. There are additional socio-economic benefits that should encourage the development of remote fetal cardiology services.


Assuntos
Serviço Hospitalar de Cardiologia , Doenças Fetais/diagnóstico , Cardiopatias/diagnóstico , Preferência do Paciente , Perinatologia/métodos , Telemedicina , Adolescente , Adulto , Assistência Ambulatorial/psicologia , Assistência Ambulatorial/estatística & dados numéricos , Ansiedade/epidemiologia , Ansiedade/etiologia , Serviço Hospitalar de Cardiologia/organização & administração , Serviço Hospitalar de Cardiologia/estatística & dados numéricos , Ecocardiografia/economia , Ecocardiografia/métodos , Feminino , Doenças Fetais/economia , Doenças Fetais/terapia , Cardiopatias/congênito , Cardiopatias/economia , Humanos , Preferência do Paciente/economia , Preferência do Paciente/estatística & dados numéricos , Satisfação do Paciente/estatística & dados numéricos , Perinatologia/organização & administração , Gravidez , Diagnóstico Pré-Natal/economia , Diagnóstico Pré-Natal/métodos , Encaminhamento e Consulta/organização & administração , Fatores Socioeconômicos , Telemedicina/economia , Telemedicina/métodos , Telemedicina/estatística & dados numéricos , Adulto Jovem
8.
Arch Dis Child Fetal Neonatal Ed ; 96(6): F394-7, 2011 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-21586481

RESUMO

OBJECTIVES: This study aimed to evaluate the feasibility, accuracy and user acceptability of performing remote fetal echocardiograms (FEs). SETTING: A regional fetal cardiology unit and a district general hospital (DGH). DESIGN: A prospective study over 20 months. An initial FE was performed by a radiographer in the DGH (D1) followed by a second FE transmitted to the regional centre, in real time, via a telemedicine link with live guidance by a fetal cardiologist (D2). A FE was performed later at the regional centre (D3, reference standard). Structured questionnaires were employed to evaluate the technical quality of each tele-link and the radiographers' confidence at performing FE. RESULTS: 69 remote FEs were performed and showed 58 normal hearts and 11 with congenital heart disease (CHD). D2 was accurate in 97% of cases compared with D3 (κ score=0.89) indicating excellent agreement. All tele-links connected at first attempt with a mean study time = 13.9 min. Overall tele-link quality was rated highly (median=4/5). In 94% of tele-links, at least 11/12 components of the FE were confidently assessed. The mean composite radiographer's questionnaire score increased significantly during the study period (p<0.05). CONCLUSIONS: To date this is the largest study of its kind. CHD can be confidently diagnosed and excluded by remote FE. Radiographers report increased confidence and proficiency following involvement in real-time telemedicine. This application of telemedicine could improve access to fetal cardiology and support radiographers screening for CHD.


Assuntos
Doenças Fetais/diagnóstico por imagem , Cardiopatias Congênitas/diagnóstico por imagem , Telerradiologia/métodos , Ultrassonografia Pré-Natal , Adolescente , Adulto , Atitude do Pessoal de Saúde , Serviço Hospitalar de Cardiologia/organização & administração , Sistemas Computacionais , Estudos de Viabilidade , Feminino , Hospitais de Distrito/organização & administração , Hospitais Gerais/organização & administração , Humanos , Irlanda do Norte , Gravidez , Estudos Prospectivos , Ultrassonografia Pré-Natal/métodos , Adulto Jovem
9.
Arch Dis Child ; 95(4): 276-80, 2010 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-19948507

RESUMO

OBJECTIVES: To determine the accuracy of remote diagnosis of congenital heart disease (CHD) by real-time transmission of echocardiographic images via integrated services digital network (ISDN) lines, to assess the impact on patient management and examine cost implications. DESIGN: Prospective comparison of echocardiograms on infants with suspected significant CHD performed as follows: (1) hands-on evaluation and echocardiogram by a paediatrician at a district general hospital (DGH) followed by (2) transmission of the echocardiogram via ISDN 6 with guidance from a paediatric cardiologist and finally (3) hands-on evaluation and echocardiogram by a paediatric cardiologist. The economic analysis compares the cost of patient care associated with the telemedicine service with a hypothetical control group. SETTING: Neonatal units of three DGH and a UK regional paediatric cardiology unit. RESULTS: Echocardiograms were transmitted on 124 infants. In five cases scans were inadequate for diagnosis. Of the remaining 119 tele-echocardiograms, a follow-up echocardiogram was performed on 109/119 (92%). Major CHD was diagnosed in 39/109 infants (36%) and minor CHD in 45 (41%). The tele-echo diagnosis was accurate in 96% of cases (kappa=0.89). Unnecessary transfer to the regional unit was avoided in 93/124 patients (75%). Despite relatively high implementation costs, telemedicine care was substantially cheaper than standard care. Each DGH potentially saved money by utilising the telemedicine service (mean saving: pound728/patient). CONCLUSIONS: CHD is accurately diagnosed by realtime transmission of echocardiograms performed by paediatricians under live guidance and interpretation by a paediatric cardiologist. Remote diagnosis and exclusion of CHD affects patient management and may be cost saving.


Assuntos
Cardiopatias Congênitas/diagnóstico por imagem , Telerradiologia/métodos , Serviço Hospitalar de Cardiologia/economia , Serviço Hospitalar de Cardiologia/organização & administração , Custos de Cuidados de Saúde/estatística & dados numéricos , Cardiopatias Congênitas/economia , Hospitais de Distrito/economia , Hospitais de Distrito/organização & administração , Hospitais Gerais/economia , Hospitais Gerais/organização & administração , Humanos , Lactente , Recém-Nascido , Internet/economia , Irlanda do Norte , Transferência de Pacientes/estatística & dados numéricos , Estudos Prospectivos , Telerradiologia/economia , Ultrassonografia , Procedimentos Desnecessários/estatística & dados numéricos
10.
J Telemed Telecare ; 14(3): 137-40, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-18430281

RESUMO

Over an eight-year period, echocardiograms were transmitted by ISDN at 384 kbit/s for a total of 132 patients suspected of having congenital heart disease (CHD). Five transmitted scans were inadequate. Hands-on echocardiograms were performed subsequently on 116 of the remaining 127 cases (91%). Major CHD was diagnosed in 42 of the 116 infants (36%) and minor CHD in 49 (42%). The telemedicine diagnosis was accurate in 97% of the cases (kappa = 0.90). There were four diagnostic errors. Transfer to the regional unit was avoided in 95 patients (72%). The present study shows that high diagnostic accuracy is possible using a telemedicine link to transmit images obtained with the assistance of real-time guidance by a paediatric cardiologist. The results also demonstrate the importance of an expert interpreting the echocardiographic images, since the accuracy of diagnosis was considerably improved (the kappa coefficient increased from 0.14 to 0.90).


Assuntos
Ecocardiografia/métodos , Cardiopatias Congênitas/diagnóstico , Interpretação de Imagem Assistida por Computador/normas , Triagem Neonatal/métodos , Telemedicina/normas , Humanos , Recém-Nascido , Telemedicina/estatística & dados numéricos , Telemetria/normas , Comunicação por Videoconferência
11.
J Telemed Telecare ; 14(3): 140-2, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-18430282

RESUMO

We originally developed a home support service for babies with complex congenital heart disease, in which videoconferencing was delivered via three ISDN lines. We have now investigated the feasibility of using broadband (Internet protocol) transmission instead of ISDN lines. Five patients were enrolled (age range 14-58 days) and 78 videoconferences were conducted over a six-month period. In 70 videoconferences (90%), a successful connection was established at the first attempt. In the last 56 videoconferences a connection bandwidth of 256 kbit/s was consistently achieved. The clinician's opinions of the videoconferences were good. Parental opinions on the videoconferences were very high. There was also a significant reduction in parental anxiety following the video consultations: the median reduction in the STAI score was 6 points (P < 0.05) (n = 78). Home support for infants or children with complex congenital heart disease can be provided successfully by video consultations utilizing home broadband links.


Assuntos
Continuidade da Assistência ao Paciente/normas , Cardiopatias Congênitas/terapia , Telemedicina/organização & administração , Comunicação por Videoconferência/organização & administração , Estudos de Viabilidade , Serviços de Assistência Domiciliar , Humanos , Lactente , Recém-Nascido , Satisfação do Paciente , Telemetria
12.
Neonatology ; 94(1): 68-70, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-18196933

RESUMO

This paper describes a female infant with achondroplasia, Down syndrome and tetralogy of Fallot. Down syndrome and achondroplasia were confirmed by karyotyping and presence of a common fibroblast growth factor receptor 3 mutation (Gly380Arg), respectively. The clinical course was complicated by pulmonary hypoplasia and subsequent intractable respiratory failure secondary to the combination of congenital conditions, which resulted in the patient's death at 5 months.


Assuntos
Acondroplasia/diagnóstico , Síndrome de Down/diagnóstico , Tetralogia de Fallot/diagnóstico , Acondroplasia/complicações , Acondroplasia/genética , Síndrome de Down/complicações , Síndrome de Down/genética , Evolução Fatal , Feminino , Humanos , Lactente , Cariotipagem , Mutação/genética , Receptores de Fatores de Crescimento de Fibroblastos/genética , Insuficiência Respiratória/etiologia , Tetralogia de Fallot/complicações , Tetralogia de Fallot/genética
13.
Arch Dis Child Fetal Neonatal Ed ; 92(6): F454-8, 2007 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-17460023

RESUMO

OBJECTIVES: To evaluate the impact of selective fluconazole prophylaxis on incidence of invasive fungal infection and emergence of fluconazole resistance in neonatal intensive care. DESIGN: Retrospective study of very low birthweight (VLBW) babies (<1500 g birth weight) admitted to a neonatal intensive care unit (NICU) in the period 1 year before and after the implementation of an antifungal prophylaxis guideline. PATIENTS: VLBW babies with an additional risk factor: colonisation of Candida species from surface sites with a central venous catheter; third generation cephalosporin treatment; or total duration of antibiotic treatment >10 days. Fluconazole protocol: Fluconazole 6 mg/kg for 3 weeks. Dose interval is every 72 h during the first 2 weeks of life. Thereafter, dose interval is reduced to every 48 h until 3 weeks old when daily fluconazole is given. Fluconazole is administered orally when enteral feeding achieved. RESULTS: 121 and 107 VLBW babies were admitted to the NICU in the year before and after the guideline was implemented, respectively. Data were available in 110 and 102 charts. 33/110 and 31/102 babies were eligible for fluconazole prophylaxis in the period before and after guideline implementation. 6/33 babies eligible for prophylaxis developed culture proven Candida sepsis before compared with no (0/31) babies after the guideline was implemented (p = 0.03). One baby (1/31) did develop probable Candida sepsis in the post guideline implementation period. During both study periods all Candida isolates remained fully susceptible to fluconazole. CONCLUSIONS: Selective antifungal prophylaxis has reduced invasive fungal sepsis in one NICU without evidence of fluconazole resistance emerging.


Assuntos
Antifúngicos/uso terapêutico , Fluconazol/uso terapêutico , Fungemia/prevenção & controle , Recém-Nascido de muito Baixo Peso , Candidíase/prevenção & controle , Quimioprevenção , Farmacorresistência Fúngica , Humanos , Recém-Nascido , Unidades de Terapia Intensiva Neonatal , Seleção de Pacientes , Estudos Retrospectivos , Análise de Sobrevida
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