Dimpna C. Albert

Embarazo y cardiopatías congénitas

Introduccion y objetivos Desde la creacion de las Unidades de Cardiopatias Congenitas (CC) del Adulto y las Unidades Obstetricas de Alto Riesgo Cardiologico, ha habido creciente interes por la evolucion hemodinamica y obstetrica de embarazadas con CC. Metodos Estudio descriptivo retrospectivo de 56 mujeres con CC y media de edad de 25 (18-40) anos, que iniciaron 84 gestaciones entre enero de 1992 y agosto de 2006. Se las distribuyo en 3 grupos de riesgo gestacional: A, bajo; B, moderado y C, alto. Resultados Las incidencias de complicaciones durante la gestacion fueron del 1,6, el 15 y el 20%, y durante el puerperio, el 2, el 23 y el 50%; la mortalidad materna fue 0, del 7,6 y del 25% de los grupos A, B y C respectivamente. Nacieron 69 ninos y las tasas de prematuridad fueron del 11, el 15 y el 100% respectivamente. Los factores de riesgo principales fueron: la hipertension pulmonar (HTP), la cianosis, la arritmia, la obstruccion del tracto de salida del ventriculo izquierdo, el ventriculo derecho (VD) dilatado, el VD sistemico necesidad de y la anticoagulacion. La HTP fue el factor mas importante asociado a morbimortalidad maternofetal. Conclusiones La estratificacion por riesgo en las gestantes con CC ofrece informacion pronostica que permite adecuar la atencion de equipos multidisciplinarios para conseguir resultados exitosos.

Actualización en cardiología pediátrica y cardiopatías congénitas: técnicas de imagen, hipertensión arterial pulmonar, tratamientos híbridos y quirúrgicos

This article contains a review of the most significant contributions to pediatric cardiology and congenital heart disease reported in publications between September 2009 and August 2010. The review focuses on imaging techniques, new treatment for pulmonary arterial hypertension in pediatric patients, and therapy in general (e.g. hybrid treatment and surgical treatment). With regard to imaging techniques, the review highlights the increasing application of congenital heart disease diagnosis during fetal life, the introduction of new echocardiographic techniques (e.g. tissue Doppler imaging, two-dimensional speckle-tracking imaging and three-dimensional echocardiography) into routine clinical practice, and the growing use of cardiac CT and magnetic resonance imaging in diagnosis and the assessment of cardiac function, respectively. The role played by cardiac interventions continues to increase and cardiac surgery is becoming more advanced and has, in some cases, been combined with hybrid techniques. However, there are still a number of controversial issues in cardiac surgery that have not yet been resolved, such as whether or not fenestration should be used with Fontan surgery, the optimum type of correction for hypoplastic left heart syndrome, and the best conduit for pulmonary artery replacement.

Recurrent congenital heart block in neonatal lupus

Congenital heart block (CHB) is the main complication of neonatal lupus (NL) and is strongly associated with the presence of anti-SSA/Ro and anti-SSB/La antibodies. The recurrence of CHB in subsequent pregnancies in mothers with these antibodies is uncommon, occurring in approximately 15% of cases. We describe here a case of recurrent CHB in a previously asymptomatic mother with Sjögren syndrome and discuss the current strategies for the prevention and treatment of CHB in NL.

Fibrosis endomiocárdica tropical e hipertensión pulmonar secundaria a esquistosomiasis

An 11-year-old black girl from Equatorial Guinea was admitted to our hospital to investigate fatigue, hepatomegaly, and ascites. Echocardiography showed severe left atrial dilation, normal systolic function, and severe dilation of the right chambers. The pulmonary pressure, estimated by the tricuspid regurgitation, was 100 mmHg. A hemodynamic study was performed and confirmed systemic pulmonary pressure with pulmonary resistance of 23 UW/m, which did not change with oxygen and nitric oxide therapy. Additional testing showed a normal hemogram and positive stool culture for Schistosoma intercalatum and S. haematobium. The diagnosis was restrictive cardiomyopathy and pulmonary hypertension due to schistosomiasis. Medical treatment was started with antiparasitic therapy, diuretics, and systemic and pulmonary vasodilators, but there was no apparent response. During the evolution of her condition, the patient presented an episode of left hemiparesis secondary to cerebral thromboembolism during an episode of atrial fibrillation. Four years after the diagnosis, she underwent successful cardiopulmonary transplantation. The figures show a macroscopic section of the ventricle at the level of the papillary muscles in which the ventricular endocardium has a pearly appearance, corresponding to endomyocardial fibrosis (EMF) (Fig. 1), a microscopy view with Masson trichrome stain showing an area of acellular fibrosis (Fig. 2), and a section of the right atrium (Fig. 3). Rev Esp Cardiol. 2011;64(8):713

Retorno venoso pulmonar total anómalo en pediatría: importancia del diagnóstico ecocardiográfico y de la cirugía precoz

Introduccion y objetivos Estudiamos los pacientes afectados de retorno venoso pulmonar total anomalo intervenidos en nuestro centro. Presentamos la importancia del diagnostico ecocardiografico exclusivo previo a la cirugia. Metodos Desde 1990 hasta 1999, 14 pacientes fueron intervenidos de dicha cardiopatia en nuestro centro. El sitio de drenaje fue en 6 pacientes supracardiaco, 4 infracardiaco, 1 cardiaco (seno coronario) y en 3 pacientes mixto. En 11 casos el diagnostico se realizo por estudio ecocardiografico, siendo los hallazgos corroborados durante la cirugia. Resultados Solo hubo una muerte durante la intervencion quirurgica, relacionada con un ventriculo izquierdo pequeno, y a los 35 dias de la intervencion se produjo otro fallecimiento por sepsis. Durante el postoperatorio inmediato, el control y tratamiento de la hipertension pulmonar fueron los objetivos primordiales. Tras un seguimiento medio de 50 meses, solo un paciente preciso reintervencion; el resto de pacientes se encuentra asintomatico. Conclusiones El diagnostico ecocardiografico de esta cardiopatia puede ser suficiente para indicar la cirugia. Asi mismo la correccion quirurgica precoz del retorno venoso pulmonar anomalo total puede ser realizada con bajo riesgo y buenos resultados a medio plazo.

Fibrilación auricular familiar

Introduccion y objetivo Se presenta el hallazgode dos familias afectadas de fibrilacion auricularen 20 de los 50 de sus miembros con ritmo cardiacoconocido, a lo largo de tres generaciones, con la finalidadde dar a conocer su excepcional existenciay sus datos clinicos mas relevantes. Metodo Se examinan el estado clinico, la evolucion,los hallazgos ECG y ECO-2D de los pacientes,los tratamientos efectuados y las complicacionesatribuibles a la enfermedad. Resultados Se confirma la presencia de 20miembros con fibrilacion auricular, aunque uno estabaen ritmo sinusal en el momento del estudio;tres pacientes presentaban dilatacion ventricularizquierda en el ECO-2D; el estado clinico es satisfactorioexcepto en dos pacientes que fallecieronpor causa relacionable con la arritmia y en un terceroque padecio un accidente vascular cerebral.La diversidad de medicos que atendia a los pacientesse relaciona con una amplia gama de tratamientos,debiendo destacarse la posible muerte por proarritmiaen un caso. Conclusion Se trata de una entidad muy pocofrecuente, en general bien tolerada, que sigue unpatron de herencia autosomica dominante. Debidoal riesgo de embolismo es aconsejable el uso de antiagregantesplaquetarios o de anticoagulantes enpresencia de factores de riesgo asociados. La cardioversionelectrica se ha demostrado ineficaz, debiendovalorarse el efecto proarritmico de los farmacosusados para el control de la frecuenciacardiaca.

Prueba de esfuerzo con función cardiopulmonar en niños operados de cardiopatía congénita. Recomendaciones de ejercicio físico en el ámbito escolar

INTRODUCTION AND OBJECTIVES To analyze and discover if stress testing with exhaled gases in children who have had congenital heart surgery is useful so we could make physical exercise recommendations according to heart disease, type of surgery performed, present hemodynamic state and level of exercise practiced. METHODS Prospective study of 108 children, who performed stress testing with exhaled gases, electrocardiogram monitoring and blood pressure. A questionnaire was used to obtain variables concerning heart disease, surgery, present functional condition and level of exercise practiced. Exercise recommendations were given after stress testing, and after a year 35 patients answered a questionnaire. RESULTS There were significant differences between lesion severity and heart rate at rest and during effort, systolic pressure at rest and during effort, oxygen uptake, oxygen pulse, carbon dioxide production and test duration. A relationship was observed between level of weekly exercise and greater oxygen uptake and test duration, but this was not observed with the underlying heart disease. We observed that best performance occurred with fast repairing for 59 children with cyanotic heart disease. Increased exercise level was recommended for 48 children. CONCLUSIONS The cardiopulmonary function study allows us to examine the physical performance of children who have had congenital heart surgery and provides us with important data so that we can recommend better physical exercise planning.

Switch arterial: bypass aortocoronario con interposición de injerto vascular de politetrafluoroetileno (Gore-tex)

Presentamos un caso de una lactante afectada de una transposicion de grandes arterias cuyo inusual patron coronario hizo necesaria la utilizacion de un injerto vascular de politetrafluoroetileno (Gore-tex) en la arteria coronaria derecha, para su resolucion quirurgica (correccion anatomica). El periodo de seguimiento postoperatorio es de 8 meses.

Immunosuppressive Therapy and Interferon-1β in Acute Myocarditis.

Acute myocarditis is an inflammatory disease of the myocardium with a variable presentation and clinical course. Although the symptoms spontaneously resolve in between 50% and 60% of patients, 20% to 40% die or require heart transplantation. Most patients with a favorable course improve during the first 2 to 4 weeks. A worse clinical course has been associated with the presence of ventricular dysfunction. From July 2008 to March 2016, 32 infants and children (016 years) with acute myocarditis were admitted to our center. Of these, 53% (17 of 32) had a left ventricular ejection fraction (LVEF)

Pregnancy and Congenital Heart Disease

INTRODUCTION AND OBJECTIVES Since the creation of the Adult Congenital Heart Disease Units and of the High Obstetric Risk Units, there has been increasing interest in hemodynamic and obstetric outcomes in pregnant woman with congenital heart disease. METHODS Retrospective descriptive study of 56 women with congenital heart disease aged (mean [range]) 25 (18-40) years, who experienced a total of 84 pregnancies between January 1992 and August 2006. The women were divided into three pregnancy risk groups: A, low-risk; B, moderate-risk, and C, high-risk. RESULTS The incidence of complications during pregnancy was 1.6%, 15%, and 20% in groups A, B, and C, respectively; the incidence during the puerperium was 2%, 23%, and 50%, respectively; and maternal mortality was 0%, 7.6%, and 25%, respectively. Overall, 69 children were born, and the prematurity rates in the three groups were 11%, 15%, and 100%, respectively. The following risk factors were studied: pulmonary hypertension, cyanosis, arrhythmia, left ventricular outflow tract obstruction, right ventricular dilatation, systemic right ventricle, and anticoagulation therapy. The risk factor most significantly associated with maternal or fetal morbidity or mortality was found to be pulmonary hypertension. CONCLUSIONS Risk stratification in pregnant women with congenital heart disease provides prognostic information that can help multidisciplinary teams to target care to achieve the best results.