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Humans , Infant , Child, Preschool , Mucocutaneous Lymph Node Syndrome/etiology , Coronary Aneurysm/diagnosis , Mucocutaneous Lymph Node Syndrome/prevention & control , Aneurysm/diagnosis , Adrenal Cortex Hormones/therapeutic use , gamma-GlobulinsSubject(s)
Coronary Aneurysm , Mucocutaneous Lymph Node Syndrome , Case-Control Studies , Humans , Infant , Risk FactorsABSTRACT
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Humans , Female , Pregnancy , Aneurysm/diagnostic imaging , Ductus Arteriosus/physiopathology , Ultrasonography, Prenatal , Fetal Diseases/diagnostic imagingABSTRACT
Introducción: La enfermedad de Kawasaki (EK) es una vasculitis aguda de causa desconocida, predominante en el sexo masculino. Los efectos de la enfermedad a largo plazo dependen de la existencia de lesiones coronarias -aneurismas-, siendo el pronóstico de los pacientes con aneurismas gigantes sombrío, ya que en su evolución natural pueden presentar trombosis coronaria o lesiones obstructivas severas. Objetivos: Describimos las características de una serie de 8 pacientes lactantes con EK y severa afectación coronaria. Se describen la epidemiología y los métodos diagnósticos, y se revisa el tratamiento de la fase aguda, así como las secuelas cardiovasculares a largo plazo. Métodos: Se realizó un análisis descriptivo de pacientes ingresados en el Servicio de Cardiología Pediátrica del Hospital Universitario La Fe (Valencia) por EK con afectación coronaria. Resultados: En todos los casos se objetivó la afectación de más de una arteria. El diagnóstico se instauró precozmente en solo 2 casos, a pesar de ello, ningún paciente presentó deterioro grave de la función ventricular durante la fase aguda. El tratamiento incluyó gammaglobulina por vía intravenosa y ácido acetilsalicílico en dosis antiinflamatorias durante la fase aguda. En los casos de trombosis coronaria se asociaron doble antiagregación y corticoides. Las lesiones aneurismáticas silentes persisten hasta el momento actual. Conclusiones: La EK es la causa más común de enfermedad cardiaca adquirida en niños. El retraso en el diagnóstico se asocia a mayor frecuencia de lesiones coronarias, que podrían incrementar el riesgo de eventos cardiovasculares en la edad adulta, precisando este subgrupo un seguimiento clínico estrecho con control de los factores de riesgo cardiovascular a largo plazo (AU)
Introduction: Kawasaki disease (KD) is an acute vasculitis of unknown origin and predominant in males. The long-term effects of the disease depend on whether there are coronary lesions, particularly aneurysms. The prognosis of patients with giant aneurysms is very poor due to their natural progression to coronary thrombosis or severe obstructive lesions. Objectives: A series of 8 cases is presented where the epidemiology and diagnostic methods are described. The treatment of the acute and long-term cardiovascular sequelae is also reviewed. Methods: A descriptive analysis was conducted on patients admitted to the Paediatric Cardiology Unit of La Fe University Hospital (Valencia) with KD and a coronary lesion. Results: More than one artery was involved in all patients. Although early diagnosis was established in only two cases, none of the patients had severe impairment of ventricular function during the acute phase. Treatment included intravenous gammaglobulin and acetylsalicylic acid at anti-inflammatory doses during the acute phase. A combination of dual antiplatelet therapy and corticosteroids was given in cases of coronary thrombosis. The silent aneurysms continue to persist. Conclusions: KD is the most common cause of acquired heart disease in children. The delay in diagnosis is associated with a greater likelihood of coronary lesions that could increase the risk of cardiovascular events in adulthood. Thus, this subgroup requires close clinical monitoring for a better control of cardiovascular risk factors over time (AU)
Subject(s)
Humans , Infant , Coronary Aneurysm/epidemiology , Mucocutaneous Lymph Node Syndrome/complications , Cardiovascular Diseases/epidemiology , Risk Factors , Myocardial Ischemia/prevention & control , gamma-Globulins/therapeutic use , Anticoagulants/therapeutic useABSTRACT
INTRODUCTION: Kawasaki disease (KD) is an acute vasculitis of unknown origin and predominant in males. The long-term effects of the disease depend on whether there are coronary lesions, particularly aneurysms. The prognosis of patients with giant aneurysms is very poor due to their natural progression to coronary thrombosis or severe obstructive lesions. OBJECTIVES: A series of 8 cases is presented where the epidemiology and diagnostic methods are described. The treatment of the acute and long-term cardiovascular sequelae is also reviewed. METHODS: A descriptive analysis was conducted on patients admitted to the Paediatric Cardiology Unit of La Fe University Hospital (Valencia) with KD and a coronary lesion. RESULTS: More than one artery was involved in all patients. Although early diagnosis was established in only two cases, none of the patients had severe impairment of ventricular function during the acute phase. Treatment included intravenous gammaglobulin and acetylsalicylic acid at anti-inflammatory doses during the acute phase. A combination of dual antiplatelet therapy and corticosteroids was given in cases of coronary thrombosis. The silent aneurysms continue to persist. CONCLUSIONS: KD is the most common cause of acquired heart disease in children. The delay in diagnosis is associated with a greater likelihood of coronary lesions that could increase the risk of cardiovascular events in adulthood. Thus, this subgroup requires close clinical monitoring for a better control of cardiovascular risk factors over time.
Subject(s)
Coronary Aneurysm/etiology , Mucocutaneous Lymph Node Syndrome/complications , Child, Preschool , Coronary Aneurysm/diagnosis , Coronary Aneurysm/pathology , Coronary Aneurysm/therapy , Female , Humans , Infant , Male , Retrospective StudiesABSTRACT
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Humans , Female , Infant, Newborn , Cardiomyopathy, Hypertrophic/complications , Cardiomyopathy, Hypertrophic/diagnosis , Noonan Syndrome/complications , Noonan Syndrome/diagnosis , Propranolol/therapeutic use , Heart Defects, Congenital/complications , Heart Defects, Congenital/diagnosis , Radiography, Thoracic/methods , Cardiomyopathy, Hypertrophic , Heart Defects, Congenital/drug therapy , Heart Defects, CongenitalSubject(s)
Cardiomyopathy, Hypertrophic/complications , Noonan Syndrome/complications , Adult , Cardiomyopathy, Hypertrophic/diagnosis , Cardiomyopathy, Hypertrophic/diagnostic imaging , Echocardiography , Electrocardiography , Female , Humans , Mutation , Noonan Syndrome/diagnosis , Noonan Syndrome/diagnostic imaging , Pregnancy , Prenatal Diagnosis , Pulmonary Valve Stenosis/complications , Pulmonary Valve Stenosis/diagnosis , Pulmonary Valve Stenosis/diagnostic imaging , RadiographyABSTRACT
Pharmacologic therapy represents the mainstay of treatment for heart failure in children. However, medical therapy for this population is not widely standardized. This is mainly due to the heterogeneity of potential etiologies, the specific challenge of patients with univentricular physiology and the lack of evidence-based prospective randomized clinical trials in pediatric patients. In fact, most current strategies are based largely on extrapolated data from adult studies. Although the classic drugs for heart failure, i.e. diuretics, angiotensin-converting enzyme inhibitors, ß -blockers and cardiac glycosides, still play a major role in the treatment of pediatric heart failure, newer alternative therapies such as levosimendan and nesiritide are increasingly utilized with promising early results. A systematic literature search of PubMed and MEDLINE databases using relevant terms was performed. All clinical trials and relevant manuscripts about the current pharmacologic treatment of heart failure in the pediatric population were reviewed. New drugs such as levosimendan and nesiritide and the treatment of single-ventricle patients were also included.
Subject(s)
Heart Defects, Congenital/complications , Heart Failure/drug therapy , Adrenergic beta-Antagonists/administration & dosage , Adrenergic beta-Antagonists/therapeutic use , Angiotensin-Converting Enzyme Inhibitors/administration & dosage , Angiotensin-Converting Enzyme Inhibitors/therapeutic use , Child , Diuretics/administration & dosage , Diuretics/therapeutic use , Heart Defects, Congenital/physiopathology , Heart Failure/diagnosis , Heart Failure/epidemiology , Heart Failure/etiology , Hemodynamics/drug effects , Humans , Hydrazones/administration & dosage , Hydrazones/therapeutic use , Incidence , Natriuretic Peptide, Brain/administration & dosage , Natriuretic Peptide, Brain/therapeutic use , Pyridazines/administration & dosage , Pyridazines/therapeutic use , SimendanSubject(s)
Endocarditis, Bacterial/complications , Endocarditis, Bacterial/diagnostic imaging , Heart Defects, Congenital/complications , Heart Defects, Congenital/diagnostic imaging , Staphylococcal Infections/complications , Staphylococcal Infections/diagnostic imaging , Humans , Infant, Newborn , Male , UltrasonographyABSTRACT
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