Giant coronary aneurysms in incomplete Kawasaki disease Marimón Blanch, Cristina; Hernández, and, Rosa Collell; Betrián Blascob, Pedro
REC, Interventional cardiology (Internet. English ed.),
07/2022, Letnik:
4, Številka:
3
Journal Article
Recenzirano
Odprti dostop
The association of Kawasaki disease with the formation of aneurysms and coronary stenoses is well established, especially in gammaglobulinnaïve patients. This is the case of a female patient ...monitored due to patent ductus arteriosus. The control echocardiography performed at the age of 2 years revealed the presence of giant coronary aneurysms in both coronary arteries. The coronary computed tomography angiography (CCTA) and following cardiac catheterization performed confirmed this finding. The patient's past medical history revealed she had been admitted at the age of 9 months due to fever compatible with pyelonephritis with good response to antibiotic therapy that could have been consistent with incomplete Kawasaki disease due to fever and further desquamation. Antiplatelet and anticoagulant therapies were administered that, to this date, have been maintained indefinitely. When the patient was 6 years old, another echocardiography (figure 1: short axis, Ao, aorta, 1A: right arrow: left coronary aneurysm; left arrow: right coronary aneurysm; arrow 1B: right coronary aneurysm), CCTA (figure 2, right arrows: left coronary aneurysm; left arrows: right coronary aneurysm), and cardiac catheterization were performed that revealed the presence of 2 17 mm x 8.8 mm and 7.3 mm × 5.3 mm calcified aneurysms in the right coronary artery (figure 3A,B...
An asymptomatic 1-month-old girl was studied in another institution because of the presence of a cardiac murmur, and referred to our center for further evaluation as a result of tricuspid valve ...abnormalities detected in the echocardiographic study. Echocardiography revealed a very redundant, thin, freely mobile structure in the right atrium, moving rapidly in (systole) and out (diastole) of the right ventricle through the tricuspid orifice. It arose from near the border of the inferior vena cava and attached to the atrial wall close to the coronary sinus ostium, suggesting an unusually prominent Chiari's network. Three-dimensional imaging allowed definition of the structure in all the planes and dimensions, and the relationship of the structure with right atrium and ventricle. Chiari's network is an embryonic remnant present in 2% to 3% of the population. The identification of a Chiari's network is important because the widely mobile structure within the right atrium can be confused with other entities, such as right heart vegetation, flail tricuspid leaflet, ruptured chordae tendinae, or a thrombus.
Resumen: Se presenta el caso de una niña de diez años que sufrió un síncope durante el esfuerzo y que fue diagnosticada de taquicardia ventricular polimórfica catecolaminérgica. Es una canalopatía ...arritmógena que puede desencadenar arritmias ventriculares graves y alto riesgo de muerte súbita en pacientes jóvenes con un corazón de estructura normal. El síncope es un problema médico común, con una prevalencia estimada del 40% en la población general. Suele tener una evolución benigna, aunque en un 2-3% también puede relacionarse con eventos cardiacos y ser un síntoma de posibilidad de muerte súbita. La anamnesis es esencial para identificar las causas y mecanismos desencadenantes y orientar las pruebas diagnósticas a realizar. En este caso la ergometría fue la prueba diagnóstica, ya que puso en evidencia la arritmia. La decisión sobre la aptitud deportiva será individualizada y dependerá de la etiología del síncope. Se debe establecer el pronóstico y valorar la posibilidad de recurrencias y de muerte súbita y evitar los diagnósticos erróneos tanto de contraindicación como de aptitud deportiva.
Kawasaki disease is a self-limiting acute vasculitis that affects small and medium-sized vessels, and is the most common cause of acquired heart disease in children in our environment. Up to 25% of ...untreated patients develop coronary aneurysms. It is suspected that an infectious agent may be the trigger of the disease, but the causative agent is still unknown. Based on the previous evidence, recommendations are proposed for the diagnosis, treatment of acute disease, and the long-term management of these patients, in order to unify criteria. The diagnosis must be quick, based on easy-to-use algorithms and with the support of complementary tests. This document includes the indication of available imaging techniques, as well as the planning of cardiological examinations based on the initial involvement. Intravenous immunoglobulin is the basis of the initial treatment. The role of corticosteroids is still controversial, but there are studies that support its use as adjuvant treatment. A multidisciplinary working group has developed a scheme with different treatment guidelines depending on the risk factors at diagnosis, the patient's clinical situation, and response to previous treatment, including indications for thromboprophylaxis in patients with coronary involvement. The stratification of risk for long-term treatment is essential, as well as the recommendations on the procedures based on the initial cardiological involvement and its progression. Patients with coronary aneurysms require continuous and uninterrupted cardiological monitoring for life.
La enfermedad de Kawasaki es una vasculitis aguda autolimitada que afecta a vasos de pequeño y mediano calibre y es la causa más común de enfermedad cardiaca adquirida en niños en nuestro medio. Hasta un 25% de pacientes no tratados desarrollan aneurismas coronarios. Se sospecha que un agente infeccioso puede ser el desencadenante de la enfermedad, pero aún se desconoce el agente causal. En base a la evidencia previa, se proponen recomendaciones para el diagnóstico, tratamiento de la enfermedad aguda y manejo a largo plazo de estos pacientes, con el fin de unificar criterios. El diagnóstico debe ser rápido, basado en algoritmos de fácil manejo y con el apoyo de pruebas complementarias. Este documento recoge la indicación de las técnicas de imagen disponibles, así como la planificación de las revisiones cardiológicas en función de la afectación inicial. La inmunoglobulina intravenosa es la base del tratamiento inicial. El papel de los corticoides aún es controvertido, pero cada vez hay más estudios que avalan su uso como tratamiento adyuvante. Un equipo multidisciplinar ha elaborado un esquema con diferentes pautas de tratamiento en función de los factores de riesgo al diagnóstico, situación clínica del paciente y respuesta al tratamiento previo, incluyendo indicaciones sobre tromboprofilaxis en pacientes con afectación coronaria. La estratificación del riesgo para el tratamiento a largo plazo es esencial, así como las recomendaciones acerca del proceder en función de la afectación cardiológica inicial y su evolución. Los pacientes con aneurismas coronarios requieren un seguimiento cardiológico continuo e ininterrumpido de por vida.
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