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1.
Medicine (Baltimore) ; 98(42): e17615, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31626139

RESUMO

BACKGROUND: The Fontan circulation is fragile, which is easily broken down. For now, there is no consensus on the drug treatment for the prevention of failure of the Fontan circulation. METHODS: Studies comparing pulmonary vasodilator and control in Fontan patients were identified by searching the PubMed, EMBASE, Clinical Trials, and the Cochrane Library databases until March 20, 2019. The assessed variables included the change of pulmonary resistance, heart function, exercise capacity, life of quality, mortality, and serials of adverse events before and after drug administration. A random-effect/fixed-effect model was used to summarize the estimates of the mean difference (MD)/risk ratio (RR) with 95% confidence interval (CI). Subgroup analysis stratified by drug was performed. RESULTS: This study will be submitted to a peer-reviewed journal for publication. CONCLUSION: This study will assess the efficacy and safety of pulmonary vasodilators for patients after Fontan procedure, and provide more evidence-based guidance in clinical practice. PROSPERO REGISTRATION NUMBER: CRD42019132135.


Assuntos
Técnica de Fontan/métodos , Cardiopatias Congênitas/cirurgia , Hipertensão Pulmonar/tratamento farmacológico , Circulação Pulmonar/efeitos dos fármacos , Pressão Propulsora Pulmonar/efeitos dos fármacos , Vasodilatadores/uso terapêutico , Cardiopatias Congênitas/fisiopatologia , Humanos , Hipertensão Pulmonar/fisiopatologia
2.
Methodist Debakey Cardiovasc J ; 15(2): 99-104, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31384372

RESUMO

The population of patients with adult congenital heart disease has grown and is currently estimated to include approximately 1 million people in the United States. Cardiologists and imagers frequently encounter complex patients who have undergone multiple prior operations and interventions. A myriad of imaging tests are currently available, including echocardiography, cardiovascular magnetic resonance imaging, and computed tomography, all of which collectively provide invaluable information on cardiac anatomy and hemodynamics. Advanced imaging plays a role in diagnosis and preprocedural planning and also determines the need and frequency of follow-up. This article provides a contemporary review of the current role of cardiac imaging in patients with complex congenital heart disease.


Assuntos
Técnicas de Imagem Cardíaca , Cardiopatias Congênitas/diagnóstico por imagem , Adulto , Fatores Etários , Ecocardiografia , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/terapia , Humanos , Imagem por Ressonância Magnética , Valor Preditivo dos Testes , Prognóstico , Tomografia Computadorizada por Raios X
3.
Methodist Debakey Cardiovasc J ; 15(2): 105-110, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31384373

RESUMO

Patients with a functionally univentricular heart who have had an atriopulmonary Fontan are at risk for atrial dilatation, atrial arrhythmias, and progressive circulatory failure. Between 1994 and 2018, we performed 149 Fontan conversions with arrhythmia surgery and epicardial pacemaker placement at Ann & Robert H. Lurie Children's Hospital of Chicago. This operation converts the atriopulmonary Fontan to an extracardiac Fontan that improves hemodynamics and controls the atrial arrhythmias. Operative mortality during that time was 2%, and freedom from death or heart transplant at 10 years is 84%. For properly selected patients, Fontan conversion improves both survival and quality of life. Patients with an atriopulmonary Fontan constitute an eroding population, as they face many comorbidities and have a decreased life expectancy without treatment; therefore, all patients with an atriopulmonary Fontan should be evaluated for this procedure.


Assuntos
Arritmias Cardíacas/cirurgia , Cardiomegalia/cirurgia , Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Adolescente , Adulto , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/mortalidade , Arritmias Cardíacas/fisiopatologia , Cardiomegalia/etiologia , Cardiomegalia/mortalidade , Cardiomegalia/fisiopatologia , Criança , Pré-Escolar , Tomada de Decisão Clínica , Feminino , Técnica de Fontan/efeitos adversos , Técnica de Fontan/mortalidade , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Seleção de Pacientes , Intervalo Livre de Progressão , Reoperação , Medição de Risco , Fatores de Risco , Fatores de Tempo , Adulto Jovem
4.
Methodist Debakey Cardiovasc J ; 15(2): 145-148, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31384378

RESUMO

Steady advances in the diagnosis and management of congenital heart disease over the last few decades has resulted in a growing population of adults with congenital heart disease (ACHD). Consequently, there has been a parallel increase in the number of ACHD patients plagued with end-stage heart failure. Even so, the transplantation rate for these patients has remained low, at about 3% of all adult heart transplants. This review discusses the scope of transplantation for ACHD, including indications and contraindications, specific challenges and nuances, and post-transplant outcomes.


Assuntos
Cardiopatias Congênitas/cirurgia , Insuficiência Cardíaca/cirurgia , Transplante de Coração , Adulto , Fatores Etários , Tomada de Decisão Clínica , Progressão da Doença , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/fisiopatologia , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/mortalidade , Insuficiência Cardíaca/fisiopatologia , Transplante de Coração/efeitos adversos , Transplante de Coração/mortalidade , Humanos , Seleção de Pacientes , Medição de Risco , Fatores de Risco , Resultado do Tratamento , Listas de Espera
5.
Methodist Debakey Cardiovasc J ; 15(2): 158-159, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31384382

RESUMO

This column is supplied by Benjamin J. Lee, MD, MAS, an assistant professor of clinical medicine at both the Houston Methodist Institute for Academic Medicine and Weill Cornell Medical College. After earning his medical degree at Harvard Medical School, Dr. Lee completed a residency in internal medicine at the University of California, San Francisco (UCSF). He subsequently completed a nephrology fellowship at UCSF while simultaneously obtaining a Master of Advanced Study in clinical research from the UCSF Department of Epidemiology and Biostatistics. Dr. Lee is a Fellow of the American Society of Nephrology, a Certified Hypertension Specialist through the American Hypertension Specialist Certification Program, and a member of the American Society of Transplantation. He maintains his clinical practice with the Houston Kidney Consultants.


Assuntos
Cianose/etiologia , Cardiopatias Congênitas/complicações , Nefropatias/etiologia , Rim/fisiopatologia , Cianose/diagnóstico , Cianose/fisiopatologia , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/tratamento farmacológico , Cardiopatias Congênitas/fisiopatologia , Humanos , Rim/efeitos dos fármacos , Rim/patologia , Nefropatias/diagnóstico , Nefropatias/tratamento farmacológico , Nefropatias/fisiopatologia , Prognóstico , Fatores de Risco
6.
Med Clin North Am ; 103(5): 945-956, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31378336

RESUMO

Cardiac defects are the most common congenital defects, accounting for approximately 9 per 1000 births. Patients with structural heart disease related to congenital diseases are prone to develop intrinsic rhythm abnormalities as a result of altered physiology. In addition, they are at an increased risk of developing acquired arrhythmias secondary to the nature of surgical interventions done to improve physiologic function in the setting of these defects. Arrhythmia management and risk stratification pose particularly complex challenges to clinicians managing this population.


Assuntos
Fibrilação Atrial/diagnóstico , Fibrilação Atrial/terapia , Cardiopatias Congênitas/epidemiologia , Fibrilação Atrial/etiologia , Ablação por Cateter , Gerenciamento Clínico , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/fisiopatologia , Humanos , Marca-Passo Artificial
7.
Braz J Cardiovasc Surg ; 34(3): 271-278, 2019 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-31310464

RESUMO

OBJECTIVE: The goal of the present study was to compare the myocardial protection obtained with histidine-tryptophan-ketoglutarate (HTK) cardioplegic solution (Custodiol®) and with intermittent hypothermic blood solution. METHODS: Two homogenous groups of 25 children with acyanotic congenital heart disease who underwent total correction with mean aortic clamping time of 60 minutes were evaluated in this randomized study. Troponin and creatine kinase-MB curves, vasoactive-inotropic score, and left ventricular function were obtained by echocardiogram in each group. The values were correlated and presented through graphs and tables after adequate statistical treatment. RESULTS: It was observed that values of all the studied variables varied over time, but there was no difference between the groups. CONCLUSION: We conclude that in patients with acyanotic congenital cardiopathies submitted to total surgical correction, mean aortic clamping time around one hour, and cardiopulmonary bypass with moderate hypothermia, the HTK crystalloid cardioplegic solution offers the same myocardial protection as the cold-blood hyperkalemic cardioplegic solution analyzed, according to the variables considered in our study model.


Assuntos
Soluções Cardioplégicas/uso terapêutico , Cardiopatias Congênitas/cirurgia , Análise de Variância , Creatina Quinase Forma MB/análise , Método Duplo-Cego , Ecocardiografia , Feminino , Glucose/uso terapêutico , Cardiopatias Congênitas/fisiopatologia , Humanos , Lactente , Recém-Nascido , Masculino , Manitol/uso terapêutico , Duração da Cirurgia , Cloreto de Potássio/uso terapêutico , Procaína/uso terapêutico , Estudos Prospectivos , Substâncias Protetoras/uso terapêutico , Valores de Referência , Reprodutibilidade dos Testes , Estatísticas não Paramétricas , Fatores de Tempo , Resultado do Tratamento , Troponina/análise , Função Ventricular Esquerda
8.
Life Sci ; 232: 116546, 2019 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-31176777

RESUMO

Pulmonary arterial hypertension (PAH) is reported to contribute to right ventricular failure and death. PAH of variable degrees is often related to congenital heart disease (CHD). Galectin-3 (Gal-3) has been proven to be of great importance in PAH and CHD. Therefore, we investigated the specific mechanism of Gal-3 in CHD-PAH. Patients with CHD-PAH were enrolled to detect the changes of T-cell subsets, cytokine levels, and other related inflammatory cells in the plasma and to assess the Gal-3 levels in the serum. Next, CHD-PAH mouse models were established and treated with restored or depleted Gal-3 to evaluate the systolic pulmonary artery pressure (sPAP) and right ventricular hypertrophy index (RVHI), to determine levels of IL-4, IL-5, IL-13, AKT and p-AKT along with proliferation of pulmonary artery smooth muscle cells (PASMCs). Finally, we explored the effects of adoptive transfer of CD4+T cells on CHD-PAH in mice with Gal-3 knockdown to further investigate the role of Gal-3 in vivo. Initially, Gal-3 was up-regulated in patients with CHD-PAH. Subsequently, it was demonstrated that restored Gal-3 increased sPAP and RVHI, and promoted proliferation of PASMCs by activating the immune response with elevated levels of IL-4, IL-5, IL-13 and p-AKT. Finally, adoptive transfer of CD4+T cells promoted CD4+T cell perivascular infiltration and the progression of CHD-PAH in mice with Gal-3 knockdown. Collectively, the current study suggests a facilitating role of Gal-3 in pulmonary artery remodeling and progression of CHD-PAH via activation of Th2.


Assuntos
Galectina 3/metabolismo , Cardiopatias Congênitas/metabolismo , Hipertensão Pulmonar/metabolismo , Adulto , Idoso , Animais , Modelos Animais de Doenças , Feminino , Galectina 3/sangue , Galectina 3/imunologia , Cardiopatias Congênitas/sangue , Cardiopatias Congênitas/imunologia , Cardiopatias Congênitas/fisiopatologia , Humanos , Hipertensão Pulmonar/imunologia , Hipertensão Pulmonar/fisiopatologia , Imunomodulação/fisiologia , Interleucina-13/metabolismo , Interleucina-4/metabolismo , Interleucina-5/metabolismo , Masculino , Camundongos , Camundongos Endogâmicos BALB C , Pessoa de Meia-Idade , Miócitos de Músculo Liso/metabolismo , Proteínas Proto-Oncogênicas c-akt/metabolismo , Artéria Pulmonar/fisiopatologia , Remodelação Vascular/fisiologia
9.
Klin Padiatr ; 231(4): 183-190, 2019 Jul.
Artigo em Alemão | MEDLINE | ID: mdl-31216577

RESUMO

Children with congenital heart disease (CHD) are at significant risk of psychomotor and cognitive developmental disorders, with negative effects on psychosocial and academic performance and quality of life. Innate characteristics such as genetic abnormalities, type and severity of CHD and socioeconomic factors are the most important risk factors. Prenatally, hypoperfusion or hypoxia can cause developmental and maturation disorders of the brain, thus promoting postpartum lesions of the more vulnerable brain substance and neurodevelopmental disorders. The high-risk group includes primarily infants requiring open heart surgery. Specific phenotypic characteristics of development emerge at different ages: in early childhood motor and linguistic articulation deficits; at school age impaired speech and language, attention, memory, visual-spatial, executive, and motor skills, often associated with academic deficits, and emotional or social issues; in adolescence executive, psychosocial and psychiatric disorders and impaired quality of life; in adulthood, neurocognitive, psychosocial and problems of self-management and professional perspective. Physical and social functioning, psychomotor development and mental health are key factors for quality of life. The German Society of Pediatric Cardiology demands detailed serial neuropsychological examinations at the age of 2 and 5 years, before puberty and before adulthood, for the high-risk group of children with CHD operated on in infant age in order to detect and to treat partial performance disorders at an early stage.


Assuntos
Cognição/fisiologia , Deficiências do Desenvolvimento , Função Executiva/fisiologia , Cardiopatias Congênitas , Qualidade de Vida , Adolescente , Adulto , Criança , Pré-Escolar , Transtornos Cognitivos/etiologia , Feminino , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/psicologia , Humanos , Lactente , Testes Neuropsicológicos
10.
Int J Cardiovasc Imaging ; 35(8): 1535-1548, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31175525

RESUMO

Congenital pulmonary artery (PA) anomalies comprise a rare and heterogeneous spectrum of disease, ranging from abnormal origins to complete atresia. They may present in early infancy or more insidiously in adulthood, often in association with congenital heart disease such as tetralogy of Fallot or other syndromes. In recent years, cross-sectional imaging, including computed tomography (CT) and magnetic resonance imaging (MRI), has become widely utilized for the noninvasive assessment of congenital PA diseases, supplementing echocardiography and at times supplanting invasive angiography. In this article, modern CT and MRI techniques for imaging congenital PA disorders are summarized. The key clinical features, cross-sectional imaging findings, and treatment options for the most commonly encountered entities are then reviewed. Emphasis is placed on the ever-growing role of cross-sectional imaging options in facilitating early and accurate diagnosis and tailored treatment.


Assuntos
Angiografia por Tomografia Computadorizada/métodos , Cardiopatias Congênitas/diagnóstico por imagem , Imagem por Ressonância Magnética/métodos , Artéria Pulmonar/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/terapia , Hemodinâmica , Humanos , Angiografia por Ressonância Magnética , Imagem Cinética por Ressonância Magnética , Tomografia Computadorizada Multidetectores , Valor Preditivo dos Testes , Prognóstico , Artéria Pulmonar/fisiopatologia , Circulação Pulmonar , Reprodutibilidade dos Testes
11.
Angiology ; 70(10): 969-977, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31064194

RESUMO

Aortic diameter and progression to thoracic aortic aneurysm are influenced by several factors. In this study, we investigated the association of alcohol consumption with aortic root and ascending aorta dilatation. In the context of the Corinthia study, we examined 1751 patients with echocardiography. Several demographic and clinical characteristics were recorded. Alcohol consumption was assessed based on a questionnaire of frequency, type, and quantity. Accordingly, patients were categorized as everyday alcohol consumers (EDACs) and as social drinkers (SoD). Everyday alcohol consumers were further categorized to group 1: 0 to 1 drink/d; group 2: 1 to 2 drinks/d; and group 3: ≥3 drinks/d. From the study population, 40% were categorized as EDAC and had an increased aortic root diameter (AoRD) and an elevated AoRD index compared with SoD. Interestingly, there was a stepwise increase in aortic root and ascending aorta diameter according to daily alcohol consumption. Specifically, patients consuming ≥3 drinks of alcohol/d had increased indexed aortic by 1.4 mm/m2 compared with SoD even after adjustment for possible confounders. Daily alcohol consumption is associated with increased aortic root diameter. These findings may have important clinical implications, especially in patients with borderline or dilated aortic root, and merit further investigation.


Assuntos
Consumo de Bebidas Alcoólicas/efeitos adversos , Aorta Torácica/patologia , Doenças da Aorta/patologia , Cardiopatias Congênitas/patologia , Doenças das Valvas Cardíacas/patologia , Adulto , Idoso , Consumo de Bebidas Alcoólicas/metabolismo , Aorta Torácica/diagnóstico por imagem , Doenças da Aorta/fisiopatologia , Valva Aórtica/patologia , Valva Aórtica/fisiopatologia , Dilatação Patológica/patologia , Dilatação Patológica/fisiopatologia , Ecocardiografia Transesofagiana/métodos , Feminino , Cardiopatias Congênitas/fisiopatologia , Doenças das Valvas Cardíacas/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Medição de Risco , Fatores de Risco
12.
Middle East Afr J Ophthalmol ; 26(1): 37-39, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31114123

RESUMO

Marcus Gunn jaw-winking syndrome (MGJWS) is a rare form of congenital blepharoptosis and one of the congenital cranial dysinnervation disorders (CCDD). In addition, morning glory disc anomaly (MGDA) is a congenital optic disc anomaly of unknown etiology. The present report is the first to describe an association between MGJWS and MGDA in an otherwise healthy 7-year-old boy. He also had counting finger vision, anisometropia, esotropia, and monocular elevation deficiency in the same eye. In the literature, both MGJWS and MGDA have been reported to be associated with Duane retraction syndrome, a form of CCDD.


Assuntos
Blefaroptose/etiologia , Anormalidades do Olho/complicações , Cardiopatias Congênitas/etiologia , Anormalidades Maxilomandibulares/etiologia , Doenças do Sistema Nervoso/etiologia , Disco Óptico/anormalidades , Anisometropia/etiologia , Anisometropia/fisiopatologia , Blefaroptose/diagnóstico , Blefaroptose/fisiopatologia , Criança , Esotropia/etiologia , Esotropia/fisiopatologia , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/fisiopatologia , Humanos , Anormalidades Maxilomandibulares/diagnóstico , Anormalidades Maxilomandibulares/fisiopatologia , Masculino , Doenças do Sistema Nervoso/diagnóstico , Doenças do Sistema Nervoso/fisiopatologia , Reflexo Anormal
13.
Expert Rev Cardiovasc Ther ; 17(6): 449-459, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31120797

RESUMO

Introduction: Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) in patients with congenital heart disease (CHD). It is characterised by a severe rise in pulmonary vascular resistance resulting in shunt reversal and cyanosis. Areas covered: In this paper, an overview of ES and other types of PAH related to CHD (PAH-CHD) in adults is provided. The modern management of PAH-CHD in tertiary centers is described, with an emphasis on co-morbidities and complications. Expert opinion: PAH-CHD describes a wide spectrum of conditions, of which ES is the archetype. The size and location of the shunt, the degree of adaptation of the right ventricle to the increased afterload and other compensatory mechanisms, such as secondary erythrocytosis, define the clinical presentation and natural history of these patients. PAH therapies have improved the quality of life and outcome of many patients with PAH-CHD, but expert multidisciplinary management remains essential in optimising the care of this rare and complex group of patients.


Assuntos
Complexo de Eisenmenger/fisiopatologia , Cardiopatias Congênitas/fisiopatologia , Hipertensão Pulmonar/fisiopatologia , Adulto , Humanos , Qualidade de Vida
14.
J Ayub Med Coll Abbottabad ; 31(2): 276-278, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31094131

RESUMO

Accessory mitral valve tissue is a rare congenital anomaly that is commonly incidentally diagnosed. When symptomatic, it tends to present with features of left ventricular outflow (LVOT) obstruction in about two thirds of cases. It is also commonly associated with other congenital anomalies, notably ventricular septal defects. Transthoracic echocardiography is a very useful diagnostic test to make a diagnosis and its widespread use will increase detection of this condition. We are presenting a case of a 29-year-old lady who presented with breathlessness in the third trimester of pregnancy and was subsequently found to have evidence of accessory mitral valve tissue on echocardiography.


Assuntos
Cardiopatias Congênitas , Valva Mitral , Complicações Cardiovasculares na Gravidez , Adulto , Dispneia/etiologia , Ecocardiografia , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Humanos , Valva Mitral/anormalidades , Valva Mitral/diagnóstico por imagem , Gravidez , Complicações Cardiovasculares na Gravidez/diagnóstico por imagem , Complicações Cardiovasculares na Gravidez/fisiopatologia
15.
World J Pediatr Congenit Heart Surg ; 10(3): 286-291, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30832541

RESUMO

INTRODUCTION: Treatment of patients with adult congenital heart disease (ACHD) with advanced therapies including heart transplant (HT) is often delayed due to paucity of objective prognostic markers for the severity of heart failure (HF). While the utility of Cardiopulmonary Exercise Testing (CPET) in non-ACHD patients has been well-defined as it relates to prognosis, CPET for this purpose in ACHD is still under investigation. METHODS: We performed a retrospective cohort study of 20 consecutive patients with ACHD who underwent HT between March 2010 and February 2016. Only 12 of 20 patients underwent CPET prior to transplantation. Demographics, standard measures of CPET interpretation, and 30-day and 1-year post transplantation outcomes were collected. RESULTS: Patient Characteristics. Twenty patients with ACHD were transplanted at a median of 40 years of age (range: 23-57 years). Of the 12 patients who underwent CPET, 4 had undergone Fontan procedures, 4 had tetralogy of Fallot, 3 had d-transposition of the great arteries, and 1 had Ebstein anomaly. Thirty-day and one-year survival was 100%. All tests included in the analysis had a peak respiratory quotient _1.0. The median peak oxygen consumption per unit time (_VO2) for all diagnoses was 18.2 mL/kg/min (46% predicted), ranging from 12.2 to 22.6. CONCLUSION: There is a paucity of data to support best practices for patients with ACHD requiring transplantation. While it cannot be proven based on available data, it could be inferred that outcomes would have been worse or perhaps life sustaining options unavailable if providers delayed referral because of the lack of attainment of CPET-specific thresholds.


Assuntos
Teste de Esforço , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/cirurgia , Insuficiência Cardíaca/cirurgia , Transplante de Coração , Adulto , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/fisiopatologia , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Encaminhamento e Consulta , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Adulto Jovem
16.
Cardiorenal Med ; 9(3): 180-189, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30844805

RESUMO

BACKGROUND: No previous study has defined the prevalence of cardiac geometric and mechanical function abnormalities through the analysis of advanced echocardiographic parameters in children with autosomal recessive polycystic kidney disease (ARPKD). AIM: The purpose of this study was to evaluate cardiac geometry and function through advanced echocardiography in a well-characterized sample of pediatric patients with ARPKD. METHODS: Standard echocardiograms were obtained in 27 children with ARPKD (0-18 years) and in 88 healthy children of similar age, gender distribution, and body build. Left ventricular (LV) hypertrophy was defined as LV mass > 45g/(m2.16 + 0.09) and cardiac remodeling was defined by age-adjusted relative wall thickness (RWT). Systolic function was assessed by ejection fraction, midwall fractional shortening (mFS), and global longitudinal (GLS) and circumferential strain (GCS). RESULTS: Patients with ARPKD exhibited a higher LV mass index as compared to controls, and a more concentric LV geometry (both p < 0.001). Accordingly, the prevalence of abnormal LV geometry was significantly higher in ARPKD (33 vs. 0%; p < 0.005). No differences could be observed in the two groups for ejection fraction or GLS (both p = n.s.), while a significantly lower mFS (p < 0.05) as well as GCS (p < 0.001) could be observed. In the analysis of covariance, both LV mass index and RWT remained significantly higher in the ARPKD group, while mFS and GCS remained significantly lower (all p < 0.05). The prevalence of subclinical systolic dysfunction was significantly higher in patients with ARPKD as compared with control subjects (33 vs. 0%; p < 0.001). CONCLUSIONS: Children with ARPKD show significantly impaired cardiac phenotype, characterized by high rates of LV abnormal geometry paired with systolic mechanical dysfunction.


Assuntos
Ecocardiografia Doppler/métodos , Cardiopatias Congênitas/diagnóstico , Ventrículos do Coração/diagnóstico por imagem , Rim Policístico Autossômico Recessivo/diagnóstico , Volume Sistólico/fisiologia , Função Ventricular Esquerda/fisiologia , Adolescente , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/fisiopatologia , Ventrículos do Coração/fisiopatologia , Humanos , Lactente , Recém-Nascido , Itália/epidemiologia , Masculino , Prevalência
17.
Semin Pediatr Surg ; 28(1): 11-17, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30824128

RESUMO

More children with congenital heart disease are surviving and require noncardiac surgery. A high-yield summary of congenital heart anatomy and pathophysiology is presented to contextualize these patients for surgeons. Preoperative planning including risk stratification, anesthetic management and timing of elective surgery are discussed. Specific intraoperative considerations for particular cases, such as the use of laparoscopy and thoracic surgery, are reviewed. Postoperative outcomes and recommended management required to mitigate complications are summarized.


Assuntos
Cardiopatias Congênitas/complicações , Assistência Perioperatória/métodos , Procedimentos Cirúrgicos Operatórios/métodos , Criança , Procedimentos Cirúrgicos Eletivos/métodos , Cardiopatias Congênitas/patologia , Cardiopatias Congênitas/fisiopatologia , Humanos , Avaliação de Resultados (Cuidados de Saúde) , Pediatria , Complicações Pós-Operatórias/prevenção & controle , Complicações Pós-Operatórias/terapia , Medição de Risco
18.
Codas ; 31(2): e20180153, 2019 Mar 07.
Artigo em Português, Inglês | MEDLINE | ID: mdl-30843926

RESUMO

PURPOSE: To evaluate the variation of cardiorespiratory parameters in the feeding of cardiopathy infants. METHODS: A cross-sectional, controlled study was performed on infants from 0 to 6 months of age, both genders, at a referral hospital for pediatric cardiac surgery. They were divided into a group study of infants with a medical diagnosis of congenital heart disease, post-cardiac surgery and in a control group composed of infants with no co-morbidities pre-established as risk factors for dysphagia. Vital signs monitoring and pulse oximetry were performed before, during and after clinical evaluation of swallowing in both groups. RESULTS: There was a significant difference in heart rate between the two groups prior to the evaluation, however, a marked increase was observed in the study group during the oral route. The respiratory rate variation was significant during the maternal breast offer in the study group, which also showed a greater decrease in saturation when compared to the control group. CONCLUSION: There is variation of cardiorespiratory parameters during feeding of cardiopathy infants.


Assuntos
Ingestão de Alimentos/fisiologia , Cardiopatias Congênitas/fisiopatologia , Frequência Cardíaca/fisiologia , Taxa Respiratória/fisiologia , Estudos de Casos e Controles , Estudos Transversais , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Oximetria
19.
Pediatr Cardiol ; 40(4): 871-877, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30850878

RESUMO

Congenital heart disease (CHD) is a common chronic disease. This study aimed to verify the relationship between spirometry and exercise capacity in children, considering the CHD severity. All cardiopulmonary exercise testing (CPET) and Spirometry from CHD children (5-18 years) were retrospectively reviewed during three years. CPET and Spirometry were analyzed and correlated based on the CHD severity[modified Ross classification (mR)]. Patients (n = 321) were analyzed and subdivided for CHD severity (n = 49, n = 149, n = 80, n = 43, from mR1 to mR4, respectively). The maximal workload (Wmax) in mR1 and mR2 was higher than in patients from mR3 and mR4. Peak oxygen uptake (peak VO2) was reduced in mR3 and mR4 compared to mR1 and mR2. Carbon dioxide output was only significantly lower in mR4. Although spirometric parameters were globally in the normal range, forced expiratory volume and forced vital capacity were different between subgroups (p < 0.001 and p = 0.002, respectively). Wmax and peakVO2 were weakly or moderately but significantly correlated with spirometry. Respiratory exchange ratio and final blood oxygen saturation were only significantly and weakly correlated to obstruction in small airways. The most severe CHD patients had lower exercise capacity and lung function parameters. A weak to moderate correlation between CPET and spirometry was found. However, the lung function reported in our study was normal, but with a negative correlation with the age. It reinforces the benefits of precocious and regularly spirometry and CPET assessment in CHD children.


Assuntos
Teste de Esforço/métodos , Tolerância ao Exercício/fisiologia , Cardiopatias Congênitas/fisiopatologia , Espirometria/métodos , Adolescente , Criança , Pré-Escolar , Exercício/fisiologia , Feminino , Humanos , Pulmão/fisiopatologia , Masculino , Estudos Retrospectivos
20.
World J Pediatr Congenit Heart Surg ; 10(1): 81-89, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30799726

RESUMO

BACKGROUND: Long-term survival of patients with a single ventricle palliated with a Fontan procedure is still limited. No curative treatment options are available. To investigate the pathophysiology and potential treatment options, such as mechanical circulatory support (MCS), appropriate large animal models are required. The aim of this review was to analyze all full-text manuscripts presenting approaches for an extracardiac total cavopulmonary connection (TCPC) animal model to identify the feasibility and limitations in the acute and chronic setting. METHODS: A literature search was performed for full-text publications presenting large animal models with extracardiac TCPCs on Pubmed and Embase. Out of 454 reviewed papers, 23 manuscripts fulfilled the inclusion criteria. Surgical procedures were categorized and hemodynamic changes at the transition from the biventricular to the univentricular condition analyzed. RESULTS: Surgical procedures varied especially regarding coronary venous flow handling and anatomic shape of the TCPC. In most studies (n = 14), the main pulmonary artery was clamped and the coronary venous flow redirected by additional surgical interventions. Only in five reports, the caval veins were connected to the right pulmonary artery to create a true TCPC shape, whereas in all others (n = 18), the veins were connected to the main pulmonary artery. An elevated pulmonary vascular resistance was identified as a limiting hemodynamic factor for TCPC completion in healthy animals. CONCLUSIONS: A variety of acute TCPC animal models were successfully established with and without MCS, reflecting the most important hemodynamic features of a Fontan circulation; however, chronic animal models were not reported.


Assuntos
Técnica de Fontan/métodos , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/anormalidades , Modelos Animais , Artéria Pulmonar/cirurgia , Veia Cava Superior/cirurgia , Animais , Cardiopatias Congênitas/fisiopatologia , Ventrículos do Coração/cirurgia , Hemodinâmica
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