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1.
Cardiol Young ; 29(3): 303-309, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30572973

RESUMO

OBJECTIVE: To bring together stakeholders in the United Kingdom to establish national priorities for research in single-ventricle heart conditions. METHODS: This study comprised two surveys and a workshop. The initial public online survey asked respondents up to three questions they would like answered for research. Responses were classified as unanswered, already answered, or unable to be answered by scientific research. In the follow-up survey, unanswered questions were divided into categories and respondents were asked to rank categories and questions by priority. A stakeholder workshop attended by patients, parents, healthcare professionals, researchers, and charities was held to determine the final list of research priorities. RESULTS: A total of 128 respondents posed 344 research questions, of which 271 were classified as unanswered, and after removing duplicates, 204 questions remained, which were divided into 20 categories. In the second survey, 56 (49.1%) respondents successfully ranked categories and questions. A total of 39 participants attended the workshop, drawing up a list of 30 research priorities across nine priority categories. The nine priority categories are: Associated co-morbidities; Brain & neurodevelopment; Exercise; Fontan failure; Heart function; Living with a single ventricle heart condition; Management of the well-functioning Fontan circulation; Surgery & perioperative care; and Transplantation, mechanical support & novel therapies. CONCLUSIONS: Through a multi-stage process, we engaged a wide range of interested parties to establish a list of research priorities in single-ventricle heart conditions. This provides a platform for clinicians, researchers, and funders in the United Kingdom and elsewhere to address the most important questions and improve outcomes in these rare but high-impact CHDs.


Assuntos
Pesquisa Biomédica/organização & administração , Procedimentos Cirúrgicos Cardíacos , Atenção à Saúde/métodos , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/anormalidades , Cuidados Pré-Operatórios , Inquéritos e Questionários , Adolescente , Adulto , Idoso , Feminino , Cardiopatias Congênitas/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Morbidade/tendências , Estudos Retrospectivos , Reino Unido/epidemiologia , Adulto Jovem
2.
BMC Cardiovasc Disord ; 17(1): 131, 2017 05 22.
Artigo em Inglês | MEDLINE | ID: mdl-28532389

RESUMO

BACKGROUND: The Fontan circulation, a result of a palliative procedure in patients with single systemic ventricles, is defined by chronically elevated pulmonary vascular resistance. When traditional heart failure therapies fail, pharmacological agents that reduce pulmonary artery pressures may be used. These include endothelial-receptor antagonists, prostanoids and phosphodiesterase type 5 inhibitors. We report the first use of macitentan, an endothelin-receptor antagonist, in a patient with a Fontan circulation. CASE PRESENTATION: We describe the case of a 50 year old female with tricuspid atresia and transposition of the great arteries. Following complex surgery as a child, she subsequently underwent a fenestrated modified atrial pulmonary Fontan operation which was later converted to a total cavopulmonary anastomosis Fontan circulation. Due to failure of various medications to relieve her worsening symptoms, she was commenced on macitentan in April 2016. Few months later, she demonstrated a significant symptomatic improvement and associated increase in her incremental shuttle walking test distance. CONCLUSIONS: Macitentan has slower receptor dissociation kinetics compared to other endothelin-receptor antagonists, leading to enhanced pharmacological activity with promising effects in patients with pulmonary arterial hypertension. The patient we report has shown considerable improvement in exercise capacity following introduction of this medication and thus we suggest further randomised trials to establish the role of different endothelin-receptor antagonists in the management of the Fontan circulation.


Assuntos
Antagonistas dos Receptores de Endotelina/uso terapêutico , Técnica de Fontan/efeitos adversos , Hipertensão Pulmonar/tratamento farmacológico , Artéria Pulmonar/efeitos dos fármacos , Pirimidinas/uso terapêutico , Sulfonamidas/uso terapêutico , Transposição dos Grandes Vasos/cirurgia , Atresia Tricúspide/cirurgia , Pressão Arterial/efeitos dos fármacos , Tolerância ao Exercício/efeitos dos fármacos , Feminino , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Pessoa de Meia-Idade , Cuidados Paliativos , Artéria Pulmonar/fisiopatologia , Circulação Pulmonar/efeitos dos fármacos , Recuperação de Função Fisiológica , Transposição dos Grandes Vasos/complicações , Resultado do Tratamento , Atresia Tricúspide/complicações
3.
Europace ; 18(2): 304-7, 2016 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-25995386

RESUMO

AIMS: In patients with an extra-cardiac Fontan circulation, there is no direct access to the heart. The insertion of a permanent pacemaker requires surgery to insert epicardial pacing wires. We present the implantation of a permanent endocardial pacing lead from the superior vena cava (SVC) into the atrium via direct passage from the right pulmonary artery (RPA). METHODS AND RESULTS: A permanent pacing lead was passed directly from the SVC to the RPA and then into the atrial mass. Direct passage from the RPA (attached directly to the right SVC) into the atrial mass was achieved using a trans-septal puncture needle. CONCLUSION: This novel technique is an alternative to epicardial pacing in patients with an extra-cardiac Fontan circulation, thus avoiding the need for surgical intervention. It may also be applied to gain access to the atrial mass for arrhythmia ablation therapy.


Assuntos
Arritmias Cardíacas/terapia , Cateterismo Cardíaco , Estimulação Cardíaca Artificial/métodos , Técnica de Fontan , Artéria Pulmonar , Veia Cava Superior , Adulto , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/fisiopatologia , Septos Cardíacos , Humanos , Masculino , Marca-Passo Artificial , Flebografia/métodos , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Punções , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Veia Cava Superior/diagnóstico por imagem , Veia Cava Superior/cirurgia
4.
Open Heart ; 10(2)2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37827809

RESUMO

In this viewpoint, we respond to the recently published national priorities for research in congenital heart disease (CHD) among adults, established through the James Lind Alliance Priority Setting Partnership, with specific attention to priority 3 (mental health) and priority 5 (maternal health). Our recent policy impact project explored how maternal mental health is currently addressed in adult congenital heart disease (ACHD) services in the National Health Service, identified gaps and discussed possible ways forward. Our multidisciplinary discussion groups, which included women with lived experience of CHD and pregnancy, cardiology and obstetrics clinicians and medical anthropologists, found that while pregnancy and the postnatal period increase the mental health challenges faced by women with CHD, current services are not yet equipped to address them. Based on this work, we welcome the prioritisation of both mental health and maternal health in ACHD, and suggest that future research should focus on the overlaps between these two priority areas.


Assuntos
Cardiologia , Cardiopatias Congênitas , Gravidez , Humanos , Adulto , Feminino , Saúde Mental , Saúde Materna , Medicina Estatal , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/terapia
5.
Open Heart ; 10(2)2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-37460271

RESUMO

BACKGROUND: COVID-19 has caused significant worldwide morbidity and mortality. Congenital heart disease (CHD) is likely to increase vulnerability and understanding the predictors of adverse outcomes is key to optimising care. OBJECTIVE: Ascertain the impact of COVID-19 on people with CHD and define risk factors for adverse outcomes. METHODS: Multicentre UK study undertaken 1 March 2020-30 June 2021 during the COVID-19 pandemic. Data were collected on CHD diagnoses, clinical presentation and outcomes. Multivariable logistic regression with multiple imputation was performed to explore predictors of death and hospitalisation. RESULTS: There were 405 reported cases (127 paediatric/278 adult). In children (age <16 years), there were 5 (3.9%) deaths. Adjusted ORs (AORs) for hospitalisation in children were significantly lower with each ascending year of age (OR 0.85, 95% CI 0.75 to 0.96 (p<0.01)). In adults, there were 24 (8.6%) deaths (19 with comorbidities) and 74 (26.6%) hospital admissions. AORs for death in adults were significantly increased with each year of age (OR 1.05, 95% CI 1.01 to 1.10 (p<0.01)) and with pulmonary arterial hypertension (PAH; OR 5.99, 95% CI 1.34 to 26.91 (p=0.02)). AORs for hospitalisation in adults were significantly higher with each additional year of age (OR 1.03, 95% CI 1.00 to 1.05 (p=0.04)), additional comorbidities (OR 3.23, 95% CI 1.31 to 7.97 (p=0.01)) and genetic disease (OR 2.87, 95% CI 1.04 to 7.94 (p=0.04)). CONCLUSIONS: Children were at low risk of death and hospitalisation secondary to COVID-19 even with severe CHD, but hospital admission rates were higher in younger children, independent of comorbidity. In adults, higher likelihood of death was associated with increasing age and PAH, and of hospitalisation with age, comorbidities and genetic disease. An individualised approach, based on age and comorbidities, should be taken to COVID-19 management in patients with CHD.


Assuntos
COVID-19 , Cardiopatias Congênitas , Hipertensão Arterial Pulmonar , Adulto , Humanos , Criança , Adolescente , COVID-19/terapia , COVID-19/complicações , Pandemias , Hospitalização , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/terapia , Hipertensão Pulmonar Primária Familiar
6.
Open Heart ; 10(2)2023 Dec 14.
Artigo em Inglês | MEDLINE | ID: mdl-38097363

RESUMO

OBJECTIVES: This study aimed to determine the status of training of adult congenital heart disease (ACHD) cardiologists in Europe. METHODS: A questionnaire was sent to ACHD cardiologists from 34 European countries. RESULTS: Representatives from 31 of 34 countries (91%) responded. ACHD cardiology was recognised by the respective ministry of Health in two countries (7%) as a subspecialty. Two countries (7%) have formally recognised ACHD training programmes, 15 (48%) have informal (neither accredited nor certified) training and 14 (45%) have very limited or no programme. Twenty-five countries (81%) described training ACHD doctors 'on the job'. The median number of ACHD centres per country was 4 (range 0-28), median number of ACHD surgical centres was 3 (0-26) and the median number of ACHD training centres was 2 (range 0-28). An established exit examination in ACHD was conducted in only one country (3%) and formal certification provided by two countries (7%). ACHD cardiologist number versus gross domestic product Pearson correlation coefficient=0.789 (p<0.001). CONCLUSION: Formal or accredited training in ACHD is rare among European countries. Many countries have very limited or no training and resort to 'train people on the job'. Few countries provide either an exit examination or certification. Efforts to harmonise training and establish standards in exit examination and certification may improve training and consequently promote the alignment of high-quality patient care.


Assuntos
Cardiologistas , Cardiologia , Cardiopatias Congênitas , Humanos , Adulto , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/terapia , Cardiologia/educação , Qualidade da Assistência à Saúde , Europa (Continente)/epidemiologia
7.
Eur J Echocardiogr ; 11(10): i27-34, 2010 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-21078836

RESUMO

Patent foramen ovale (PFO) is common, with a probe-patent PFO present in 15-35% of the general population. Patent foramen ovale has been implicated in the aetiology of a number of different pathologies, including cryptogenic stroke, decompression sickness in divers, platypnea orthodeoxia, and migraine with aura. Cardiac ultrasound has a major role not only in the diagnosis of PFO but also in monitoring subsequent therapeutic intervention and in the post-procedural assessment of patients following percutaneous closure. The aim of this review was to outline the data regarding the role of echocardiography in diagnosis, during monitoring and post-procedural assessment so as to provide practical advice to minimize error and optimize patient outcomes. The review will seek to outline the limitations of the available techniques and factors that should be taken into account during percutaneous device closure.


Assuntos
Ecocardiografia/métodos , Forame Oval Patente/diagnóstico por imagem , Forame Oval Patente/cirurgia , Humanos , Ultrassonografia de Intervenção/métodos
8.
Echo Res Pract ; 7(1): R1-R10, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32015897

RESUMO

Aortic diseases may be diagnosed after a long period of subclinical development or they may have an acute presentation. Acute aortic syndrome is often the first sign of the disease, which needs rapid diagnosis and decision making to reduce the extremely poor prognosis. Aortic dilatation is a well-recognised risk factor for acute events and can occur as a result of trauma, infection, or, most commonly, from an intrinsic abnormality in the elastin and collagen components of the aortic wall. Over the years it has become clear that a few monogenic syndromes are strongly associated with aneurysms and often dictate a severe presentation in younger patients while the vast majority have a multifactorial pathogenesis. Conventional cardiovascular risk factors and ageing play an important role. Management strategy is based on prevention consisting of regular follow-up with cross-sectional imaging, chemoprophylaxis of further dilatation with drugs proved to slow down the disease progression and preventative surgery when dimension exceeds internationally recognised cut-off values for aortic diameters and the risk of rupture/dissection is therefore deemed very high.

10.
Heart ; 105(14): 1103-1108, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-30772822

RESUMO

OBJECTIVE: To evaluate time trends in the use of catheter and surgical procedures, and associated survival in isolated congenital shunt lesions. METHODS: Nationwide, retrospective observational study of the UK National Congenital Heart Disease Audit database from 2000 to 2016. Patients undergoing surgical or catheter procedures for atrial septal defect (including sinus venosus defect), patent foramen ovale, ventricular septal defect and patent arterial duct were included. Temporal changes in the frequency of procedures, and survival at 30 days and 1 year were determined. RESULTS: 40 911 procedures were performed, 16 604 surgical operations and 24 307 catheter-based interventions. Transcatheter procedures increased over time, overtaking surgical repair in 2003-2004, while the number of operations remained stable. Trends in interventions differed according to defect type and patient age. Catheter closure of atrial septal defects is now more common in children and adults, although surgical interventions have also increased. Patent foramen ovale closure in adults peaked in 2009-2010 before falling significantly since. Surgery remains the mainstay for ventricular septal defect in infants and children. Duct ligation is most common in neonates and infants, while transcatheter intervention is predominant in older children. Excluding duct ligation, survival following surgery was 99.4% and ≈98.7%, and following catheter interventions was 99.7% and ≈99.2%, at 30 days and 1 year, respectively. CONCLUSIONS: Trends in catheter and surgical techniques for isolated congenital shunt lesions plot the evolution of the specialty over the last 16 years, reflecting changes in clinical guidelines, technology, expertise and reimbursement, with distinct patterns according to lesion and patient age.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Permeabilidade do Canal Arterial , Procedimentos Endovasculares , Forame Oval Patente , Comunicação Interatrial , Comunicação Interventricular , Adulto , Fatores Etários , Cateterismo Cardíaco/métodos , Procedimentos Cirúrgicos Cardíacos/métodos , Procedimentos Cirúrgicos Cardíacos/estatística & dados numéricos , Permeabilidade do Canal Arterial/epidemiologia , Permeabilidade do Canal Arterial/terapia , Procedimentos Endovasculares/métodos , Procedimentos Endovasculares/estatística & dados numéricos , Feminino , Forame Oval Patente/epidemiologia , Forame Oval Patente/terapia , Comunicação Interatrial/epidemiologia , Comunicação Interatrial/terapia , Comunicação Interventricular/epidemiologia , Comunicação Interventricular/terapia , Humanos , Lactente , Irlanda/epidemiologia , Masculino , Avaliação de Processos e Resultados em Cuidados de Saúde , Administração dos Cuidados ao Paciente/métodos , Administração dos Cuidados ao Paciente/estatística & dados numéricos , Estudos Retrospectivos , Análise de Sobrevida , Reino Unido/epidemiologia
11.
Int J Cardiol ; 244: 354-357, 2017 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-28622940

RESUMO

BACKGROUND: Pregnancy is associated with an increased risk of aortic pathology. We sought to assess the feasibility of performing non-contrast 3D steady-state free-precession (SSFP) magnetic resonance angiography (MRA) in pregnant subjects with inherited aortopathy. METHODS: Fifteen pregnant subjects (age 27±4yr) with positive genotyping for aortopathy (Marfan, Loeys-Dietz, Ehlers-Danlos) and/or a family history of aortic dissection underwent non-contrast 3D-SSFP MRA at 1.5T (Avanto, Siemens Healthcare, Erlangen, Germany) using a modified ECG-triggered orientated in a sagittal-oblique plane with a respiratory navigator at the diaphragmatic level (mean acquisition time 4.1±1.9min). Imaging was performed during the mid-trimester (21±5weeks). Image analysis was performed off-line using Cvi42 software (Circle Cardiovascular Imaging, Calgary, Canada). An assessment of image quality (score 0-3) was made before performing inner edge to inner edge measurements of the thoracic aorta at 7 levels from the multiplanar reconstructions by two independent blinded observers. RESULTS: Non-contrast 3D-MRA was successfully acquired in all 15 subjects. Image quality was deemed excellent in 87% (13/15) of cases after a mean acquisition time of 4.1±1.9min. There was a high level of agreement for aortic measurements, with low intra- and inter-observer variability (ICC ranges; 0.95-0.99 and 0.92-0.98, respectively). All pregnancies reached term (≥37/40) with a mean gestation at delivery of 38.0±0.5weeks. The mode of delivery was vaginal in 9 out of 15 subjects (60%). CONCLUSIONS: Non-contrast SSFP MRA imaging provides a quick and reproducible method of assessing the thoracic aorta in pregnancy.


Assuntos
Aorta Torácica/diagnóstico por imagem , Imageamento Tridimensional/métodos , Angiografia por Ressonância Magnética/métodos , Complicações Cardiovasculares na Gravidez/diagnóstico por imagem , Adulto , Aorta Torácica/fisiopatologia , Síndrome de Ehlers-Danlos/diagnóstico por imagem , Síndrome de Ehlers-Danlos/fisiopatologia , Estudos de Viabilidade , Feminino , Humanos , Síndrome de Marfan/diagnóstico por imagem , Síndrome de Marfan/fisiopatologia , Projetos Piloto , Gravidez , Complicações Cardiovasculares na Gravidez/fisiopatologia , Estudos Prospectivos
12.
Int J Cardiol ; 257: 76-77, 2018 04 15.
Artigo em Inglês | MEDLINE | ID: mdl-29506742
13.
Congenit Heart Dis ; 8(4): 302-7, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-22967110

RESUMO

INTRODUCTION: The right ventricle (RV) supports the systemic circulation in patients who have had an intraatrial repair of transposition of the great arteries or have congenitally corrected transposition. There is concern about the ability of a systemic RV to support the additional volume load of pregnancy, and previous studies have reported deterioration in RV function following pregnancy. However, conditions with a systemic RV are also associated with progressive RV dysfunction over time. To date, no study has examined whether the deterioration associated with pregnancy is due to the physiological changes of pregnancy itself, or is part of the known deterioration that occurs with time in these patients. METHODS: Women who had undergone pregnancy under the care of the Adult Congenital Heart Disease Unit at the Queen Elizabeth Hospital were retrospectively identified and matched to separate male and nulliparous female controls. Functional status (New York Health Association [NYHA]), RV function, and systemic atrioventricular valve regurgitation were recorded for each group at baseline, postpregnancy (or at 1 year for control groups) and at latest follow-up. RESULTS: Eighteen women had 31 pregnancies (range 1-4) resulting in 32 live births. There were no maternal but one neonatal death. At baseline, there was no significant difference in NYHA class or RV function between pregnancy and control groups. In postpregnancy, there was a significant deterioration in the pregnant group alone for both NYHA class (P = 0.004) and RV function (P = 0.02). At latest follow-up, there was a significant deterioration in RV function in all three groups. There was still a reduction from baseline in NYHA of women who had undergone pregnancy (P = 0.014), which again was not seen in the controls groups. CONCLUSION: This study suggests that pregnancy is associated with a premature deterioration in RV function in women with a systemic RV. These women are also more symptomatic, with a greater reduction in functional class compared with patients with a systemic RV who do not undergo pregnancy. This study will allow this cohort of women to be more accurately counseled as to the potential long-term risks of pregnancy.


Assuntos
Ventrículos do Coração/fisiopatologia , Paridade , Complicações Cardiovasculares na Gravidez/etiologia , Transposição dos Grandes Vasos/complicações , Disfunção Ventricular Direita/etiologia , Função Ventricular Direita , Adolescente , Adulto , Distribuição de Qui-Quadrado , Transposição das Grandes Artérias Corrigida Congenitamente , Progressão da Doença , Inglaterra , Feminino , Ventrículos do Coração/anormalidades , Ventrículos do Coração/cirurgia , Humanos , Gravidez , Complicações Cardiovasculares na Gravidez/fisiopatologia , Resultado da Gravidez , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Transposição dos Grandes Vasos/fisiopatologia , Transposição dos Grandes Vasos/cirurgia , Disfunção Ventricular Direita/fisiopatologia , Adulto Jovem
14.
Congenit Heart Dis ; 7(3): 243-9, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22348734

RESUMO

OBJECTIVE: Adult patients with a Fontan circulation tend to have diminished exercise capacity. The principal objective of this study was to investigate the safety of the endothelin receptor antagonist bosentan in Fontan patients, and, secondarily, to assess effects on cardiovascular performance, New York Heart Association functional classification (NYHA FC), and ventricular function. DESIGN: A 6-month prospective, single-center, pilot, safety study of bosentan in Fontan patients. Setting. Adult Congenital Heart Disease referral center. PATIENTS: All patients ≥18 years old with a Fontan circulation and in NYHA FC ≥II were invited to enroll. Interventions. Patients started on 62.5 mg bid of bosentan, uptitrating to 125 mg bid after 2 weeks. OUTCOME MEASURES: Safety was assessed by the incidence of anticipated and unanticipated adverse events during the 6-month study period; specifically those relating to hepatic, renal, or hematological dysfunction as measured by monthly blood tests. Other outcome measures included cardiopulmonary exercise test, 6-minute walk distance test, Borg dyspnea index, NYHA FC, and ventricular function parameters using transthoracic echocardiography. RESULTS: Of the eight patients enrolled, six completed the study. Two patients withdrew from the study (one for non-trial related reasons, one due to adverse events). No clinically significant adverse events relating to bosentan therapy occurred during this study and, in particular, no significant abnormalities in hepatic function tests were observed. Three patients reported transient adverse events. Improvements in NYHA FC and systolic ventricular function were observed after 6 months of bosentan treatment. CONCLUSIONS: The small number of patients with a Fontan circulation in our study was able to tolerate bosentan for 6 months. The safety and tolerability of bosentan in a larger patient population remains unknown. The results presented here justify further investigation in larger studies.


Assuntos
Fármacos Cardiovasculares/uso terapêutico , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Complicações Pós-Operatórias/tratamento farmacológico , Artéria Pulmonar/efeitos dos fármacos , Sulfonamidas/uso terapêutico , Adulto , Bosentana , Fármacos Cardiovasculares/efeitos adversos , Ecocardiografia Doppler , Antagonistas dos Receptores de Endotelina , Inglaterra , Teste de Esforço , Tolerância ao Exercício/efeitos dos fármacos , Feminino , Humanos , Masculino , Projetos Piloto , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/metabolismo , Complicações Pós-Operatórias/fisiopatologia , Valor Preditivo dos Testes , Estudos Prospectivos , Artéria Pulmonar/metabolismo , Artéria Pulmonar/fisiopatologia , Receptores de Endotelina/metabolismo , Recuperação de Função Fisiológica , Sulfonamidas/efeitos adversos , Fatores de Tempo , Resultado do Tratamento , Resistência Vascular/efeitos dos fármacos , Função Ventricular/efeitos dos fármacos
15.
Congenit Heart Dis ; 6(3): 253-6, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-21418528

RESUMO

We present the case of a 22-year-old man with a Fontan circulation who suffered from intractable protein losing enteropathy for an 8-year period necessitating several hospital admissions for recurrent pleural effusions and edema. Despite trying several recognized medical therapies to alleviate his protein losing enteropathy, his condition did not improve until the introduction of loperamide for troublesome diarrhea. Following this, his symptoms and serum albumin improved dramatically and he has not required any further hospital admissions at 22 months follow up.


Assuntos
Antidiarreicos/uso terapêutico , Diarreia/tratamento farmacológico , Técnica de Fontan/efeitos adversos , Loperamida/uso terapêutico , Enteropatias Perdedoras de Proteínas/tratamento farmacológico , Albumina Sérica/metabolismo , Biomarcadores/sangue , Diarreia/etiologia , Humanos , Masculino , Enteropatias Perdedoras de Proteínas/sangue , Enteropatias Perdedoras de Proteínas/etiologia , Resultado do Tratamento , Adulto Jovem
17.
Congenit Heart Dis ; 4(1): 42-5, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-19207403

RESUMO

Two adult patients with isolated, aortic interruption were successfully treated by percutaneous insertion of graft stents. Prior to the intervention, both patients were hypertensive and on medication. In both cases, an ascending aortogram demonstrated a blind ending of the thoracic aorta distal to the left subclavian artery with a large gradient across the interruption and with multiple collaterals. A graft stent was successfully deployed across the interrupted segment in both cases. We believe that this is one of the first reported cases of percutaneous stenting of aortic interruption and represents a promising new therapeutic option for these adult patients.


Assuntos
Angioplastia/métodos , Aneurisma da Aorta Torácica/cirurgia , Dissecção Aórtica/cirurgia , Stents , Dissecção Aórtica/diagnóstico por imagem , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Aneurisma da Aorta Torácica/diagnóstico por imagem , Aortografia , Cateterismo Cardíaco/métodos , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Medição de Risco , Resultado do Tratamento
18.
Congenit Heart Dis ; 4(6): 494-8, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-19925548

RESUMO

Percutaneous techniques are being increasingly used in adult congenital heart disease but there is limited experience in the treatment of native nonvalvar right ventricular outflow tract obstruction. We describe two cases of percutaneous stenting of the subpulmonary region where surgery was not an option.


Assuntos
Cardiopatias Congênitas/terapia , Estenose da Valva Pulmonar/terapia , Stents , Obstrução do Fluxo Ventricular Externo/terapia , Adulto , Idoso , Síndrome de Down , Fluoroscopia , Cardiopatias Congênitas/diagnóstico por imagem , Humanos , Masculino , Estenose da Valva Pulmonar/congênito , Estenose da Valva Pulmonar/diagnóstico por imagem , Obstrução do Fluxo Ventricular Externo/diagnóstico por imagem
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