Anomalía de Ebstein y aneurisma del seno coronario / Ebstein’s anomaly and coronary sinus aneurysm

Resumen La anomalía de Ebstein es una cardiopatía congénita poco común que se asocia a la presencia de vías de conducción anómalas y episodios de taquicardia supraventricular frecuentes, algunos inestables. La asociación con alteraciones anatómicas del seno coronario es rara y no ha sido reportada. Se presenta el caso de una paciente de 58 años con enfermedad coronaria, anomalía de Ebstein, episodios de taquicardia ortodrómica y aneurisma del seno coronario, a quien se realizó ablación.

Abstract Ebstein’s disease is a congenital cardiomyopathy, with a low prevalence in the general population. This abnormality has been associated with abnormal cardiac conduction problems, one of the most important being the accessory pathways. In the presence of an accessory pathway, frequent supraventricular tachycardias may occur, some of which are poorly tolerated. The association with the anomalies of the coronary sinus is not currently reported. The case of a 58-year-old woman with Ebstein’s disease, episodes of supraventricular tachycardia, and coronary sinus aneurysm undergoing ablation therapy is presented.

Anomalía de Ebstein en un paciente con síndrome de Down. Reporte de un caso / Ebstein's anomaly in a patient with Down syndrome. Case report

Resumen Paciente con síndrome de Down referido a nuestro departamento con cianosis y soplo cardíaco. Un ecocardiograma transtorácico mostró anomalía de Ebstein. Esta asociación es extremadamente rara. Se inició manejo médico ya que la lesión en la válvula tricúspide era leve. De acuerdo con nuestra revisión, se han descrito únicamente 12 casos en literatura médica.

Abstract An infant with Down's syndrome was referred to our department with cyanosis and heart murmur. A transthoracic echocardiogram demonstrated the presence of Ebstein's anomaly. This association is extremely unusual. Medical management was initiated since the tricuspid valve lesion was mild. Only twelve cases, to our knowledge, have been previously reported.

Tricuspid Valve Replacement in an Adult with Destroyed Tricuspid Valve and Refractory Right Heart Failure.

22 year old woman with no previous heart disease history, presented with progressive worsening of right heart failure symptoms due to severe tricuspid valve regurgitation which had become refractory to medical management. Echocardiogram revealed probable rare case of dysplastic tricuspid valve with large calcified mobile masses attached to leaflets. Calcified masses were thought to be due to healed vegetations from silent infective endocarditis of abnormal tricuspid valve which she had suffered in the past. There were no known acquired causes of tricuspid valve endocarditis. She had successfully undergone tricuspid valve replacement with bio-prosthetic valve along with a right atrial reduction surgery after which her heart failure symptoms improved markedly.

Associação da Anomalia de Ebstein com a Síndrome de Wolff-Parkinson-White: relato de caso / Association of Ebstein´s Anomaly with Wolff-Parkinson-White Syndrome: case report

Primeiramente relatada por Wilhelm Ebstein em 1866,a anomalia de Ebstein corresponde a uma doença cardíacacongênita incomum, levando a uma má formaçãoda valva tricúspide. Pode se apresentar com dispnéia,cianose, arritmias, cardiomegalia e insuficiência ventriculardireita. O ecocardiograma confirma o diagnóstico.Descrevemos o caso de uma paciente feminina, 38 anos,com hipertensão arterial sistêmica e fibromialgia, apresentandoSíndrome de Wolff-Parkinson-White associadaà anomalia de Ebstein.

First reported by Wilhelm Ebstein in 1866, Ebstein´sAnomaly is an unusual congenital cardiac disease, leadingto a tricuspid valve bad formation. It can presentsdyspnea, cyanosis, arrythmia, cardiomegaly and rightventricular failure. Echocardiography firms the diagnosis.We describe a female patient, 38 years old, withsystemic arterial hypertension and fibromyalgia, presentingWolff-Parkinson-White Syndrome associated withEbstein´s Anomaly.

Cardiopatía y embarazo: serie de casos / Heart disease and pregnancy: case series

El objetivo de este reporte es presentar nuestra experiencia en el manejo de pacientes con cardiopatías y embarazo en el Hospital Simón Bolívar de Bogotá. Se presentan tres pacientes con enfermedad cardiaca y embarazo, atendidas entre enero y diciembre de 2009. El caso 1 es una paciente de 28 años, con tetralogía de Fallot y embarazo de 38,3 semanas. El caso 2 es una paciente de 20 años, con anomalía de Ebstein y 38,6 semanas de embarazo, en trabajo de parto. El caso 3 es una paciente de 21 años, con estenosis aórtica severa y embarazo de 39 semanas. En los tres casos el desenlace fue favorable. El conocimiento de las diferentes cardiopatías permite un manejo anestésico seguro. Para establecer la técnica anestésica se debe evaluar: reserva cardiaca, función ventricular, hipertensión pulmonar,estenosis pulmonar o de conductos, defectos de conducción y arritmias.

We report our experience managing pregnant patients with heart disease at the Simón Bolívar Hospital in Bogotá. The report includes three patients seen between January and December,2009. The first case is a 28-year old patient with tetralogy of Fallot and 38.3 weeks of gestation. The second case is a 20-year old patient in labor withan Ebstein’s anomaly and 38.6 weeks of gestation. The third case is a 21-year old patient with severe aortic stenosis in week 39 of pregnancy. The outcome in all three cases was favorable.Knowledge of the different heart diseases enables safe anesthetic management. The choice of the anesthetic technique requires assessment of the following parameters: cardiac reserve, ventricularfunction, pulmonary hypertension, pulmonary or duct stenosis, conduction defects and arrhythmias.

Anomalía de Ebstein “tipo B”: caso clínico / Ebstein’s anomaly “type B”: case report

La anomalía de Ebstein es una rara enfermedad cardíacacongénita, con incidencia 1/200.000 nacidos vivos, presenta un espectro morfológico amplio caracterizado por diferentes grados de desplazamiento y adherencia de la valvas septal y posterior de la válvula tricúspide hacia el ventrículo derecho, comunicación interauricular o foramen oval permeable, asociada a degeneración del miocardio subyacente a las valvas posterior y septal incriminadas. El reservorio venoso auricular aumenta su volumen al desplazarse el orificio tricuspídeo efectivo dentro de la cámara de entrada del ventrículo derecho, afectándose su función sistolo-diastólica y aparece cianosis secundaria a cortocircuito a nivel auricular. En 20% - 30% se asocia a síndrome de Wolff-Parkinson-White (WPW). Presentamos el caso clínico de un adolescente de 16 años con anomalía de Ebstein y taquicardia supraventricular, quién presentó evento cerebrovascular isquémico a los 7 años, la evaluación clínica y ecocardiográfica resultó en anomalía de Ebstein tipo B, se realizó ablación por radiofrecuencia eficaz de vía accesoria posterolateral derecha guiada por sistema de navegación, sin embargo, 7 meses después reingresa por disnea progresiva y acentuación de la cianosis, se realiza resonancia magnética y cateterismo cardíaco; para calcular volumen ventricular funcional del ventrículo derecho y medir presiones pulmonares, realizándole corrección bi-ventricular con técnica de Carpentier y reemplazo valvular tricuspídeo (Prótesis Medtronic Advantage N°23), con resultado satisfactorio y evolución clínica a clase funcional I-II NYHA.

Ebstein’s anomaly is a rare congenital heart disease, with incidence 1/200.000 live births, has a broad morphologics pectrum characterized by different degrees of displacement and adherence of the septal and posterior leaflets of the tricuspid valve into the right ventricle atrial septal defect or patent foramen ovale associated with myocardial degeneratio nunderlying the posterior and septal leaflets incriminated. The atrial venous reservoir increases in volume by moving the effective tricuspid orifice in the right ventricle inlet chamber, affecting systolic-diastolic function and appears cyanosis secondary to shunt at the atrial level. In 20% - 30% is associated with Wolff-Parkinson-White (WPW). We report the case of a 16-year Ebstein’s anomaly and supraventricular tachycardia, who presented ischemic stroke event at age 7-year, the clinical and echocardiographic assessment of Ebstein’s anomaly resulted in type B, was performed radiofrequency ablation of accessory pathway effective right postero-lateral guided navigation system, but seven months later readmitted because of progressive dyspnoea and cyanosis accentuation is performed cardiac MRI and catheterization to calculate volume right ventricle and measure functional pulmonary pressures, performing bi-ventricular correction technique Carpentier tricuspid valve replacement (Medtronic Advantage prosthesis No. 23), with satisfactory results and clinical functional class NYHA I-II.