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OBJECTIVE: To explore the clinical value of 3D Echocardiography (3DE) in evaluating the changes of left atrial volume and pulmonary vein structure in patients with Atrial Fibrillation (AF). METHODS: Clinical data were collected from 54 AF patients. Left Atrial Anteroposterior Diameter (LADap), Left Atrial left and right Diameter (LADml), and Left Atrial upper and lower Diameter (LADsi) were measured; the maximum Left Atrial Volume (LAVmax), minimum Left Atrial Volume (LAVmin), left atrial presystolic volume (LAVpre), and Cross-Sectional Area (CSA) of each pulmonary vein were analyzed. Passive Ejection Fraction (LAPEF) was calculated. The differences in left atrial volume and pulmonary vein structure between patients with AF and healthy people were compared, and the correlation between the indexes was analyzed. The diagnostic value of the above indicators for AF patients was analyzed. RESULTS: LADap, LADml, LADsi, LAVmax, LAVmin, LAVpre, LAPEF, LSPV CSA, LIPV CSA, RSPV CSA, and RIPV CSA of AF patients were significantly higher. There was a significant positive correlation between left atrial diameter and pulmonary vein structure. There was a significant positive correlation between left atrial volume and pulmonary vein structure. There was a negative correlation between LAPEF and pulmonary vein structure. LADap, LADml, LADsi, LAVmax, LAVmin, LAVpre, LAPEF, LSPV CSA, LIPV CSA, RSPV CSA, and RIPV CSA had a diagnostic value for AF patients. CONCLUSION: 3DE is applicable for evaluating left atrial volume and pulmonary vein structure in patients with AF.

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Se presenta un caso poco frecuente de hemoptisis en un paciente adolescente con cirugía de Fontan, causada por el sangrado de una malformación arteriovenosa pulmonar (MAVP), tras la práctica de un instrumento de viento (saxofón). El paciente se estudió con angiografía y se realizó cierre percutáneo, consiguiendo posteriormente mejoría clínica y evitando la aparición de nuevos episodios de hemoptisis. Actualmente existe escasa literatura que reporte casos de hemoptisis en pacientes con cirugía de Fontan y ningún caso publicado en relación a gatillantes relacionados a las maniobras de valsalva; como la generada al practicar instrumentos de viento. Por esta razón, se decide publicar este caso clínico en pro de generar mayores conocimientos en este grupo de pacientes con cardiopatías congénitas operadas con procedimientos paliativos como es la cirugía de Fontan.

A rare case of hemoptysis is presented in an adolescent patient with Fontan surgery, caused by bleeding from a pulmonary arteriovenous malformation (PAVM), after playing a wind instrument (saxophone). The patient was studied with angiography and percutaneous closure was performed, subsequently achieving clinical improvement and preventing the appearance of new episodes of hemoptysis. Currently, there is little literature on hemoptysis in patients with Fontan surgery and no case published in relation to triggers related to valsalva maneuvers; like that generated when practicing wind instruments. For this reason, it was decided to publish this clinical case in order to generate greater knowledge in this group of patients with congenital heart disease operated on with palliative procedures such as Fontan surgery.

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Anatomic and physiologic assessment of surgical repair of scimitar syndrome can be challenging. We evaluated the first patient who underwent the Lugones procedure in 2012 using 4D flow magnetic resonance imaging. With this technology, we demonstrate that the reconstructed right pulmonary venous return drains into the left atrium with laminar flow, just as normal pulmonary veins do.

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Paradoxical embolism due to an isolated pulmonary arteriovenous malformation (PAVM) is a rare cause of ischemic stroke. PAVMs are abnormal high-flow connections between pulmonary arteries and veins, diverting deoxygenated blood into the systemic circulation and they represent a less common source of paradoxical embolisms, especially in young individuals. Endovascular embolization is the preferred treatment for clinically significant PAVMs. We present the case of a 34-year-old woman with a left thalamic ischemic stroke. Severe contrast passage was detected in cerebral arteries through transcranial Doppler. Intracardiac ultrasound did not reveal a patent foramen ovale, prompting further investigation with pulmonary CT angiography, confirming the presence of PAVM. The patient underwent successful endovascular treatment. It is essential to consider PAVM in the etiological diagnosis of ischemic stroke, especially in young patients with signs of abnormal right-to-left communication. Periodic follow-up imaging is recommended to assess potential recurrence or changes in PAVM, emphasizing the importance of appropriate management of these malformations.

La embolia paradojal debido a una malformación arteriovenosa pulmonar (MAVP) aislada es una causa infrecuente de accidente cerebrovascular (ACV) isquémico. Las MAVP son conductos anómalos de alta circulación entre arterias y venas pulmonares, desviando sangre desoxigenada hacia la circulación sistémica y representan una fuente menos común de embolias paradojales, especialmente en personas jóvenes. La embolización endovascular es el tratamiento preferido para MAVP clínicamente significativas. Presentamos el caso de una mujer de 34 años con ACV isquémico talámico izquierdo. Se detectó pasaje de burbujas "en cortina" en arterias cerebrales mediante Doppler transcraneal. En ecografía intracardíaca no se encontró foramen oval permeable, motivo por el cual se avanzó con realización de angiotomografía pulmonar, la cual confirmó la presencia de MAVP. La paciente recibió tratamiento endovascular exitoso. Es esencial considerar la MAVP en el diagnóstico etiológico del ACV isquémico, especialmente en pacientes jóvenes con signos de comunicación anormal de derecha a izquierda. Se recomienda un seguimiento periódico mediante imágenes para evaluar la posible recurrencia o cambios en la MAVP, resaltando la importancia del manejo adecuado de estas malformaciones.

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This review aimed to provide an overview of pulmonary arteriovenous malformations, including the major clinical and radiological presentations, investigation, and treatment algorithm of the condition. The primary etiology of pulmonary arteriovenous malformations is hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, with mutations in the ENG gene on chromosome 9 (HHT type 1) or in the ACVRL1/ALK1 complex (HHT type 2). Epistaxis should always be evaluated when repeated, when associated with anemia, and in some cases of hypoxemia. In the investigation, contrast echocardiography and chest CT are essential for evaluating this condition. Embolization is the best treatment choice, especially for correction in cases of hypoxemia or to avoid systemic infections. Finally, disease management was addressed in special conditions such as pregnancy. CT follow-up should be performed every 3-5 years, depending on the size of the afferent and efferent vessels, and antibiotic prophylactic care should always be oriented. Ultimately, knowledge of the disease by health professionals is a crucial point for the early diagnosis of these patients in clinical practice, which can potentially modify the natural course of the disease.

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INTRODUCTION: Anomalous pulmonary venous connection is a rare congenital variant of the pulmonary veins drained into the right atrium. The left-side partial anomalous pulmonary connections are usually detected in adulthood and occasionally drain into the left brachiocephalic vein. CASE DESCRIPTION: An asymptomatic 63-year-old woman with a known history of left carotid body paraganglioma was admitted to our institution to evaluate this tumor by computed tomography angiography of the neck and brain. As an incidental finding, CTA demonstrated a supracardiac partial anomalous pulmonary venous connection between the left upper pulmonary vein and the left innominate via the vertical vein. Additionally, dilation of the azygos vein was observed. CONCLUSION: Supracardiac left-sided partial anomalous pulmonary venous connection is a rare vascular variant, usually asymptomatic and found incidentally in adults.

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Las malformaciones arteriovenosas pulmonares (MAVP) consisten en comunicaciones directas entre el sistema arterial y el sistema venoso pulmonar, sin paso de la sangre por el lecho capilar, produciéndose un cortocircuito de derecha a izquierda extracardíaco, pueden ser congénitas o adquiridas. Algunos casos pueden ser asintomáticos, en cambio en otros pueden ocasionar diversas manifestaciones clínicas y se pueden asociar a complicaciones severas. En niños se observa una baja incidencia y son más frecuentes las formas congénitas. La MAVP se debe sospechar por las manifestaciones clínicas y las imágenes de la radiografía de tórax (RxTx) y su confirmación se realiza mediante una AngioTomografía Computada (TC) de tórax. La embolización endovascular es actualmente el tratamiento de elección, con excelentes resultados, aunque requiere de un seguimiento posterior y de un operador experimentado. Reportamos el caso de una niña que ingresó con clínica muy sugerente, incluyendo: disnea, acropaquia, cianosis periférica, e hipoxemia refractaria. Sin embargo, inicialmente el cuadro clínico fue confundido con una crisis asmática. La Angio-TC de tórax confirmó el diagnóstico y el tratamiento mediante embolización endovascular resultó exitoso.

Pulmonary arteriovenous malformations (PAVM) are communications between the arterial and the pulmonary venous system, without passage of blood through the capillary bed, causing a left to right extracardiac shunt. Some cases may be asymptomatic, while others may cause various clinical manifestations and may be associated with severe complications. In children a low incidence is observed, and congenital forms are more frequent. PAVM should be suspected by clinical manifestations and chest x-ray imaging and confirmed by chest Computed Tomography Angiography (CTA). Endovascular embolization is currently the treatment of choice, with excellent results, although it requires subsequent follow-up. We report a patient who was admitted with a very suggestive clinical history, including: dyspnea, clubbing, peripheral cyanosis, and severe hypoxemia, refractory to oxygen therapy. However, initially the clinical picture was confounded with an asthmatic crisis. CTA confirmed the diagnosis and treatment by endovascular embolization was successful.

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Accidental detachment of mechanical valve leaflet during implantation is a rare and potentially serious complication. When the lost leaflet cannot be found by direct visualisation, additional diagnostic procedures are necessary to detect it. Computer tomography is the best detection method, but the patient needs reoperation. We presented a patient in whom the detached leaflet migrated and became trapped into the left inferior pulmonary vein. The computed tomography (CT) scan was used to reveal leaflets, and successful extirpation was performed in the second operation.

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BACKGROUND: Epicardial adipose tissue (EAT) has been associated with atrial fibrillation (AF), but its pathophysiological mechanisms remain unclear. OBJECTIVES: To measure the correlation between EAT and left atrium (LA) fibrosis, and to assess their ability to predict relapse after pulmonary vein isolation (PVI). METHODS: Patients with AF enrolled for a first PVI procedure underwent both cardiac computerized tomography (CT) and cardiac magnetic resonance (CMR) imaging within less than 48 hours. EATLMwas quantified on contrast-enhanced CT images at the level of the left main. LA fibrosis was quantified on isotropic 1.5 mm 3D delayed enhancement CMR. After pulmonary vein isolation (PVI), patients were followed up for AF relapse. Statistical significance was set at p<0.05. RESULTS: Most of the 68 patients (46 men, age 61±12 years) had paroxysmal AF (71%, n=48). Patients had a median EATLMvolume of 2.4 cm3/m2(interquartile range [IQR] 1.6-3.2 cm3/m2), and a median amount of LA fibrosis of 8.9 g (IQR 5-15 g). The correlation between EATLMand LA fibrosis was statistically significant but weak (Spearman's R=0.40, p=0.001). During a median follow-up of 22 months (IQR 12-31), 31 patients (46%) had AF relapse. Multivariate analysis yielded two independent predictors of AF relapse: EATLM(HR 2.05, 95% CI 1.51-2.79, p<0.001), and non-paroxysmal AF (HR 2.36, 95% CI 1.08-5.16, p=0.031). CONCLUSION: The weak correlation between EAT and LA suggests that LA fibrosis is not the main mechanism linking EAT and AF. EAT was more strongly associated with AF relapse than LA fibrosis, supporting the existence of other more important mediators of EAT and AF.

FUNDAMENTO: O tecido adiposo epicárdico (TAE) tem sido associado à fibrilação atrial (FA), mas seus mecanismos fisiopatológicos permanecem obscuros. OBJETIVOS: Medir a correlação entre TAE e fibrose do átrio esquerdo (AE), e avaliar sua capacidade de prever recidiva após o isolamento da veia pulmonar (IVP). MÉTODOS: Pacientes com FA inscritos para um primeiro procedimento de IVP foram submetidos à tomografia computadorizada (TC) cardíaca e ressonância magnética cardíaca (RMC) em menos de 48 horas. Quantificou-se o TAECE em imagens de TC realçadas com contraste no nível do tronco da coronária esquerda. Quantificou-se a fibrose do AE em RMC tridimensional com realce tardio isotrópico de 1,5 mm. Após o isolamento da veia pulmonar (IVP), os pacientes foram submetidos a seguimento para checar a recidiva da FA. A significância estatística foi definida com p<0,05. RESULTADOS: A maioria dos 68 pacientes (46 homens, idade 61±12 anos) tinha FA paroxística (71%, n=48). Os pacientes apresentavam volume TAECE mediano de 2,4 cm3/m2 (intervalo interquartil [IIQ] 1,6­3,2 cm3/m2) e um volume médio de fibrose do AE de 8,9 g (IIQ 5­15 g). A correlação entre TAECE e fibrose do AE foi estatisticamente significativa, mas fraca (coeficiente de correlação de postos de Spearman = 0,40, p=0,001). Durante um seguimento médio de 22 meses (IIQ 12­31), 31 pacientes (46%) tiveram recidiva da FA. A análise multivariada produziu dois preditores independentes de recidiva da FA: TAECE (FC 2,05, IC de 95% 1,51­2,79, p<0,001) e FA não paroxística (FC 2,36, IC de 95% 1,08­5,16, p=0,031). CONCLUSÃO: A correlação fraca entre TAE e AE sugere que a fibrose do AE não é o principal mecanismo que liga o TAE e a FA. O TAE mostrou-se mais fortemente associado à recidiva da FA do que à fibrose do AE, corroborando a existência de outros mediadores mais importantes do TAE e da FA.

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Abstract Total anomalous pulmonary venous drainage is a rare and diverse anomaly, accounting for 1% to 3% of patients with congenital heart disease. Newborns with diagnosis of an obstructed total anomalous pulmonary venous dainage are extremely ill soon after birth and often present with severe cyanosis, pulmonary hypertension and low cardiac output requiring urgent surgical intervention. Transcatheter palliative stenting of the obstructive vertical vein can be an acceptable alternative as a bailout intervention before complete surgical correction is undertaken. This report of two cases highlights the feasibility, safety and effectiveness of the inter ventional palliative procedure and confirms that this technique can be an acceptable and attractive bridge in the algorithm of medical decisions during the evaluation of these critical patients.

Resumen El drenaje venoso pulmonar anómalo total es una enfermedad poco frecuente y de presentación diversa y se observa en el 1% a 3% de las cardiopatías congénitas. Si se asocia a obstrucción, se convierte en una afección grave en el recién nacido, mostrando cianosis intensa, hipertensión arterial pulmonar y bajo gasto cardíaco con indicación de intervención quirúrgica de urgencia. El implante de stent por cateterismo de forma paliativa para aliviar la obstrucción puede ser una alternativa aceptable de tratamiento como intervención de rescate antes de la corrección quirúrgica definitiva. Presentamos dos casos de intervención percutánea paliativa mostrando que esta técnica puede ser eficaz como puente al tratamiento quirúrgico definitivo para ser incorporado en la toma de decisiones de estos pacientes críticos.

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Total anomalous pulmonary venous drainage is a rare and diverse anomaly, accounting for 1% to 3% of patients with congenital heart disease. Newborns with diagnosis of an obstructed total anomalous pulmonary venous dainage are extremely ill soon after birth and often present with severe cyanosis, pulmonary hypertension and low cardiac output requiring urgent surgical intervention. Transcatheter palliative stenting of the obstructive vertical vein can be an acceptable alternative as a bailout intervention before complete surgical correction is undertaken. This report of two cases highlights the feasibility, safety and effectiveness of the interventional palliative procedure and confirms that this technique can be an acceptable and attractive bridge in the algorithm of medical decisions during the evaluation of these critical patients.

El drenaje venoso pulmonar anómalo total es una enfermedad poco frecuente y de presentación diversa y se observa en el 1% a 3% de las cardiopatías congénitas. Si se asocia a obstrucción, se convierte en una afección grave en el recién nacido, mostrando cianosis intensa, hipertensión arterial pulmonar y bajo gasto cardíaco con indicación de intervención quirúrgica de urgencia. El implante de stent por cateterismo de forma paliativa para aliviar la obstrucción puede ser una alternativa aceptable de tratamiento como intervención de rescate antes de la corrección quirúrgica definitiva. Presentamos dos casos de intervención percutánea paliativa mostrando que esta técnica puede ser eficaz como puente al tratamiento quirúrgico definitivo para ser incorporado en la toma de decisiones de estos pacientes críticos.

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Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.

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Abstract Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.

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Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic congenital heart disease that accounts for less than 1% of all congenital heart diseases. TAPVC is characterized by the absence of pulmonary venous drainage into the left atrium. It is underdiagnosed in utero, with prenatal detection rates of less than 1.4%. The prenatal diagnosis of TAPVC affects the postnatal outcome, particularly in obstructive forms (critical TAPVC), since planned delivery and perinatal management are mandatory. Thus, identifying the ultrasound key markers of TAPVC is important for the prenatal diagnosis and perinatal assistance. The ventricular size discrepancy (right ventricle > left ventricle) can be a useful marker. Furthermore, the increased retroatrial distance between the left atrium and the aorta could be a diagnostic marker for prenatal TAPVC, especially when the left atrium is small. Three- (3D) and four-dimensional ultrasonography may improve the prenatal diagnosis of TAPVC. This study reviews the two (2D) and 3D ultrasonographic markers used in the antenatal diagnosis of TAPVC, with a focus on the tools that can be used by sonographers, obstetricians, and fetal medicine specialists to improve the prenatal diagnosis of TAPVC, and when to refer the case to a fetal cardiac specialist.

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Tuberculosis is one of the most common infectious diseases around the world. With timely diagnosis and treatment, mortality in children is practically zero. It is usually associated with a diverse number of complications that can cause significant morbidity and mortality, such as deep and superficial vein thrombosis. This event has been associated with a procoagulant state caused by the systemic inflammatory response to infection. We report the case of a 14-year-old adolescent with pulmonary tuberculosis under the initial four-drug regimen. She presented two episodes of venous thromboembolism, the first in the kidneys and the second in the lungs. After ruling out diseases such as nephrotic and antiphospholipid antibody syndrome, chest and abdomen tomographies were performed as a fundamental tool for the diagnosis. Thereafter, treatment with low molecular weight heparin was initiated and the symptoms improved. Given the requirement for anticoagulation, further image studies could not be done. Thromboembolic complications in patients with no other risk factors, associated only with a previous pulmonary tuberculosis diagnosis, offer evidence to consider the procoagulant effect resulting from the systemic inflammatory response that, by itself, could be the cause of a serious complication, often underdiagnosed but also preventable. Therefore, it is recommended to consider the predisposition for venous thromboembolism in these patients and to establish strict surveillance so early anticoagulant therapy can be provided to prevent adverse outcomes.

La tuberculosis es una de las enfermedades infecciosas más comunes en el mundo. Aunque la mortalidad en niños es prácticamente nula cuando el diagnóstico y el tratamiento son oportunos, puede asociarse con complicaciones como la trombosis venosa profunda y la superficial a partir de la respuesta inflamatoria sistémica frente a la infección, lo que propicia la coagulación y ocasiona una significativa morbimortalidad. Se reporta el caso de una adolescente de 14 años con tuberculosis pulmonar en tratamiento combinado quien, de forma atípica, presentó dos episodios de tromboembolia venosa: el primero en el riñón y el segundo en los pulmones. Tras descartar el síndrome nefrótico y el antifosfolipídico, los estudios de tomografía de tórax y abdomen fueron una herramienta fundamental para su diagnóstico. Se inició tratamiento con heparina de bajo peso molecular con mejoría de los síntomas. Teniendo en cuenta las necesidades de anticoagulación no fue posible realizar estudios adicionales de ampliación. Las complicaciones tromboembólicas en pacientes con tuberculosis y sin otros factores de riesgo obligan a considerar el efecto coagulante que resulta de la reacción inflamatoria sistémica, la cual podría, por sí sola, ser la causa de una complicación significativa pero prevenible, aunque frecuentemente escapa al diagnóstico. En este sentido, se recomienda considerar la posibilidad de la tromboembolia venosa en estos pacientes y hacer un seguimiento estricto que permita aplicar el tratamiento anticoagulante tempranamente y prevenir, así, resultados adversos.

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A 43-year-old woman underwent radiofrequency pulmonary vein ablation for symptomatic paroxysmal atrial fibrillation. At 3 months, she developed worsening dyspnea and exercise intolerance; tests revealed severe stenosis in her right pulmonary veins at the venoatrial junction and an abnormally small left atrium.

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