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1.
Cardiol Young ; : 1-5, 2019 Sep 26.
Artigo em Inglês | MEDLINE | ID: mdl-31554525

RESUMO

Pulmonary hypertension is a complex and progressive condition that is either idiopathic or heritable, or associated with one or multiple health conditions, with or without congenital or acquired cardiovascular disease. Recent developments have tremendously increased the armamentarium of diagnostic and therapeutic approaches in children and young adults with pulmonary hypertension that is still associated with a high morbidity and mortality. These modalities include non-invasive imaging, pharmacotherapy, interventional and surgical procedures, and supportive measures. The optimal, tailored diagnostic and therapeutic strategies for pulmonary hypertension in the young are rapidly evolving but still face enormous challenges: Healthcare providers need to take the patient's age, development, disease state, and family concerns into account when initiating advanced diagnostics and treatment. Therefore, there is a need for guidance on core and advanced medical training in paediatric pulmonary hypertension. The Association for European Paediatric and Congenital Cardiology working group "pulmonary hypertension, heart failure and transplantation" has produced this document as an expert consensus statement; however, all recommendations must be considered and applied in the context of the local and national infrastructure and legal regulations.

13.
Rev. esp. cardiol. (Ed. impr.) ; 66(7): 553-555, jul. 2013.
Artigo em Espanhol | IBECS | ID: ibc-113635

RESUMO

Introducción y objetivos. Para la paliación inicial de pacientes con fisiología univentricular, discordancia ventriculoarterial y estenosis subaórtica, se utilizan diversas técnicas. La cirugía tipo Fontan depende de una correcta paliación inicial que evite la aparición de estenosis subaórtica (además de hipertrofia ventricular y disfunción diastólica). Métodos. Presentamos a 7 pacientes con fisiología univentricular, transposición y estenosis subaórtica, con bajo gasto sistémico e hiperaflujo pulmonar, de 21–383 (mediana, 75) días de edad y 3,4–9,6 (mediana, 4,2) kg de peso. Todos fueron tratados mediante switch arterial paliativo, «cambiando» su estenosis subaórtica por una estenosis subpulmonar. En 6 casos se asoció cirugía de arco aórtico; en 4, septectomía auricular y en 1, resección de membrana subaórtica. Resultados. Un paciente falleció en el postoperatorio; otro sufrió recoartación, tratada mediante angioplastia; 3 pacientes han alcanzado el estadio de Glenn y 2, el de Fontan. La función ventricular es correcta en todos ellos. Conclusiones. La fisiología de ventrículo único con transposición y estenosis subaórtica se puede tratar inicialmente mediante un switch paliativo. Esta compleja técnica inicial presenta buenos resultados y permite la progresión futura hacia Glenn y Fontan (AU)


Introduction and objectives. There are several techniques for the palliative treatment of patients with single-ventricle physiology, ventriculoarterial discordance and subaortic stenosis. The Fontan procedure relies on optimal initial palliation to avoid the development of subaortic stenosis (as well as ventricular hypertrophy and diastolic dysfunction). Methods. We present seven patients with single-ventricle physiology, transposition of the great arteries and subaortic stenosis, with low systemic output and high pulmonary flow, aged 21 to 383 days (median, 75) and weighing between 3.4 and 9.6 kg (median, 4.2). All were treated with a palliative arterial switch, thus “switching” their subaortic stenosis to subpulmonary stenosis. Six patients also underwent aortic arch surgery, 4 an atrial septectomy, and 1 a subaortic membrane resection. Results. One patient died after surgery, another developed recoarctation, which was treated with an angioplasty, 3 patients reached the Glenn stage and 2 the Fontan stage. All had good ventricular function. Conclusions. A palliative switch is an effective initial treatment for single-ventricle physiology with transposition of the great arteries and subaortic stenosis. This complex initial technique produces good results and allows the patient to progress to the Glenn or Fontan stage (AU)


Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Estenose Aórtica Subvalvar/complicações , Estenose Aórtica Subvalvar/diagnóstico , Ventrículos do Coração/fisiopatologia , Transtornos da Coagulação Sanguínea/complicações , Estenose Aórtica Subvalvar/prevenção & controle , Técnica de Fontan/métodos , Técnica de Fontan/tendências , Coagulação Intravascular Disseminada/epidemiologia , Complicações Pós-Operatórias/mortalidade , Complicações Pós-Operatórias/terapia
15.
Rev Esp Cardiol (Engl Ed) ; 66(7): 553-5, 2013 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-24776204

RESUMO

INTRODUCTION AND OBJECTIVES: There are several techniques for the palliative treatment of patients with single-ventricle physiology, ventriculoarterial discordance and subaortic stenosis. The Fontan procedure relies on optimal initial palliation to avoid the development of subaortic stenosis (as well as ventricular hypertrophy and diastolic dysfunction). METHODS: We present seven patients with single-ventricle physiology, transposition of the great arteries and subaortic stenosis, with low systemic output and high pulmonary flow, aged 21 to 383 days (median, 75) and weighing between 3.4 and 9.6kg (median, 4.2). All were treated with a palliative arterial switch, thus "switching" their subaortic stenosis to subpulmonary stenosis. Six patients also underwent aortic arch surgery, 4 an atrial septectomy, and 1 a subaortic membrane resection. RESULTS: One patient died after surgery, another developed recoarctation, which was treated with an angioplasty, 3 patients reached the Glenn stage and 2 the Fontan stage. All had good ventricular function. CONCLUSIONS: A palliative switch is an effective initial treatment for single-ventricle physiology with transposition of the great arteries and subaortic stenosis. This complex initial technique produces good results and allows the patient to progress to the Glenn or Fontan stage.


Assuntos
Estenose Aórtica Subvalvar/cirurgia , Técnica de Fontan/métodos , Cardiopatias Congênitas/cirurgia , Cuidados Paliativos/métodos , Feminino , Seguimentos , Técnica de Fontan/efeitos adversos , Humanos , Lactente , Recém-Nascido , Masculino
16.
Am J Perinatol ; 30(4): 289-95, 2013 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-22918677

RESUMO

OBJECTIVE: After the introduction of a new protocol based on the early treatment with indomethacin for patent ductus arteriosus, the objective of this study was to assess the safety and efficacy of this new practice in comparison with the safety and efficacy of the conventional treatment in a high-risk population. STUDY DESIGN: We conducted a retrospective cohort study including 154 newborns with an average gestational age of 26.4 weeks (1.37 standard deviation) and an average birth weight of 855 g (201.5 standard deviation). A statistically descriptive analysis was performed with SPSS Statistics Pack version 17.0. RESULTS AND CONCLUSIONS: We did not find any statistically significant differences in the clinical features of the two treatment groups, nor in the main efficacy, morbidity, and mortality results.


Assuntos
Fármacos Cardiovasculares/uso terapêutico , Permeabilidade do Canal Arterial/tratamento farmacológico , Indometacina/uso terapêutico , Recém-Nascido Prematuro , Distribuição de Qui-Quadrado , Estudos de Coortes , Relação Dose-Resposta a Droga , Esquema de Medicação , Permeabilidade do Canal Arterial/diagnóstico por imagem , Permeabilidade do Canal Arterial/mortalidade , Ecocardiografia Doppler , Feminino , Seguimentos , Idade Gestacional , Humanos , Recém-Nascido , Masculino , Estudos Retrospectivos , Medição de Risco , Taxa de Sobrevida , Fatores de Tempo , Resultado do Tratamento
17.
Clin Transplant ; 26(5): 755-63, 2012 Sep-Oct.
Artigo em Inglês | MEDLINE | ID: mdl-22463464

RESUMO

BACKGROUND: Congenital heart diseases (CHDs) have high infant mortality in their severe forms. When adulthood is reached, a heart transplant (HTx) may be required. Spanish adult population transplanted for CHD was analyzed and compared with the most frequent causes of HTx and between different subgroups of CHD. MATERIALS AND METHODS: A total of 6048 patients (HTx 1984-2009) were included. Pediatric transplants (<15 yr), combined transplants, reHTx, and HTx for heart diseases other than idiopathic dilated cardiomyopathy (IDCM) and ischemic heart disease (IHD) were excluded. Total patients included: 3166 (IHD = 1888; IDCM = 1223; CHD = 55). Subgroups were studied as follows: (1) single ventricle with pulmonary stenosis (n = 18), (2) single ventricle with tricuspid atresia and Glenn/Fontan surgery (n = 10), (3) congenitally corrected transposition of the great vessels (TGV) or with switch atrial surgery (n = 10), and (4) CHD with right ventricle overload (n = 17). RESULTS: Survival probability was different between groups (p = 0.0001). Post hoc analysis showed some differences between groups (CHD vs. IHD, p = 0.05; CHD vs. IDCM, p = 0.5; IHD vs. IDCM, p = 0.0001). Early mortality was different between CHD subgroups (group 1 = 19%, group 2 = 40%, group 3 = 0%, group 4 = 29%; p < 0.001); however, overall mortality did not show differences between subgroups (p = 0.5). CONCLUSIONS: The percentage of Spanish adult HTx patients for CHD is low (1%). The survival curve is better than for other HTx causes (IHD). Nevertheless, early mortality was higher, particularly in some subgroups (Fontan).


Assuntos
Cardiopatias Congênitas/mortalidade , Transplante de Coração/mortalidade , Adulto , Feminino , Seguimentos , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Recidiva , Sistema de Registros , Taxa de Sobrevida
18.
Rev. esp. cardiol. (Ed. impr.) ; 64(9): 780-787, sept. 2011. tab, ilus
Artigo em Espanhol | IBECS | ID: ibc-90867

RESUMO

Introducción y objetivos. El objetivo es analizar la utilidad de la prueba de esfuerzo con determinación de gases espirados en niños operados de cardiopatía congénita en edad escolar, para valorar la recomendación de ejercicio físico en relación con la cardiopatía de base, el tipo de cirugía realizada, la situación hemodinámica actual y el nivel de ejercicio habitual. Métodos. Estudio prospectivo de 108 niños que realizaron una prueba de esfuerzo con análisis de gases, monitorizando electrocardiograma y presión arterial. Se recogieron por cuestionario variables sobre cardiopatía de base, cirugía practicada, estado funcional actual y nivel de ejercicio habitual. Se emitió una recomendación de ejercicio después de la ergometría, y al año se controló por cuestionario a 35 de los pacientes. Resultados. Se observaron diferencias significativas entre la gravedad actual de la lesión y la frecuencia cardiaca de reposo y esfuerzo, la presión arterial sistólica en reposo y en esfuerzo, el consumo de oxígeno, el pulso de oxígeno, la producción de dióxido de carbono y la duración de la prueba. Se detectó relación entre el nivel de ejercicio semanal y mayores consumo de oxígeno y duración de la ergometría, pero no con la cardiopatía subyacente. En los 59 niños con lesiones cianóticas, se observó que la mejor capacidad funcional se correspondía con reparación de la lesión más precoz y mejor. Se pudo recomendar un incremento del nivel de ejercicio a 48 niños. Conclusiones. La prueba ergoespirométrica permite explorar la capacidad funcional de los niños operados de cardiopatía congénita y aporta datos importantes para una mejor planificación del ejercicio físico aconsejable (AU)


Introduction and objectives. To analyze and discover if stress testing with exhaled gases in children who have had congenital heart surgery is useful so we could make physical exercise recommendations according to heart disease, type of surgery performed, present hemodynamic state and level of exercise practiced. Methods. Prospective study of 108 children, who performed stress testing with exhaled gases, electrocardiogram monitoring and blood pressure. A questionnaire was used to obtain variables concerning heart disease, surgery, present functional condition and level of exercise practiced. Exercise recommendations were given after stress testing, and after a year 35 patients answered a questionnaire. Results. There were significant differences between lesion severity and heart rate at rest and during effort, systolic pressure at rest and during effort, oxygen uptake, oxygen pulse, carbon dioxide production and test duration. A relationship was observed between level of weekly exercise and greater oxygen uptake and test duration, but this was not observed with the underlying heart disease. We observed that best performance occurred with fast repairing for 59 children with cyanotic heart disease. Increased exercise level was recommended for 48 children. Conclusions. The cardiopulmonary function study allows us to examine the physical performance of children who have had congenital heart surgery and provides us with important data so that we can recommend better physical exercise planning (AU)


Assuntos
Humanos , Masculino , Feminino , Criança , Teste de Esforço/métodos , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/reabilitação , Cardiopatias Congênitas/cirurgia , Exercício , Teste de Esforço/tendências , Teste de Esforço , Estudos Prospectivos , Inquéritos e Questionários , Ergometria , Frequência Cardíaca/fisiologia , Análise Multivariada
20.
Rev Esp Cardiol ; 64(9): 780-7, 2011 Sep.
Artigo em Espanhol | MEDLINE | ID: mdl-21775042

RESUMO

INTRODUCTION AND OBJECTIVES: To analyze and discover if stress testing with exhaled gases in children who have had congenital heart surgery is useful so we could make physical exercise recommendations according to heart disease, type of surgery performed, present hemodynamic state and level of exercise practiced. METHODS: Prospective study of 108 children, who performed stress testing with exhaled gases, electrocardiogram monitoring and blood pressure. A questionnaire was used to obtain variables concerning heart disease, surgery, present functional condition and level of exercise practiced. Exercise recommendations were given after stress testing, and after a year 35 patients answered a questionnaire. RESULTS: There were significant differences between lesion severity and heart rate at rest and during effort, systolic pressure at rest and during effort, oxygen uptake, oxygen pulse, carbon dioxide production and test duration. A relationship was observed between level of weekly exercise and greater oxygen uptake and test duration, but this was not observed with the underlying heart disease. We observed that best performance occurred with fast repairing for 59 children with cyanotic heart disease. Increased exercise level was recommended for 48 children. CONCLUSIONS: The cardiopulmonary function study allows us to examine the physical performance of children who have had congenital heart surgery and provides us with important data so that we can recommend better physical exercise planning.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Teste de Esforço/métodos , Exercício , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/cirurgia , Análise de Variância , Pressão Sanguínea , Dióxido de Carbono/metabolismo , Criança , Cianose/fisiopatologia , Eletrocardiografia , Feminino , Frequência Cardíaca , Hemodinâmica , Humanos , Masculino , Consumo de Oxigênio , Estudos Prospectivos , Inquéritos e Questionários
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