[Fetal diagnosis and prognosis of Ebstein's anomaly]. / Diagnóstico y pronóstico fetal de la anomalía de Ebstein.

BACKGROUND: Currently fetal echocardiography may confirm the diagnosis in utero of Ebstein anomaly, as well as determine the perinatal outcome with high certainty. OBJECTIVE: To review 16 cases diagnosed with Ebstein anomaly, by fetal echocardiography, analyzing prognostic echocardiographic parameters set by Pavlova and colleagues. MATERIAL AND METHOD: A descriptive, observational, retrospective study was done in patients with fetal diagnosis of Ebstein anomaly, during January 2001 to December 2011. Echocardiographic parameters are analyzed and its correlation to perinatal evolution of 16 cases of Ebstein anomaly. RESULTS: Maternal age was of 27.94 + 5.7 years, gestational age was 31.3 +/- 3.6 weeks. In utero mortality represented 37.5% (n = 6), and neonatal mortality 50% (n = 8), two patients (12.5%) survived. Cardiothoracic index was of 0.61 +/- 0.074, ratio foramen ovale-atrial septal was of 0.6 +/- 0.015, obstruction of the outflow tract of the right ventricle was seen in 14 (87.5%) patients; 81% of the deceased had a degree of displacement valve > 2.5; ratio right ventricle-left ventricle 2.24 +/- 0.37. The umbilical vein was throbbing in 64% of the deceased, the tricuspid insufficiency was severe in 15 cases (94%, 21.62 +/- 2.82 mmHg), hydrops affected 18.7% of patients. CONCLUSIONS: The following factors are of bad prognosis in fetal stage of Ebstein anomaly: cardiothoracic index > 0.55, relative foramen ovale-atrial septal <0.3, the obstruction to the outflow tract, a degree of valve displacement > 2.5, absence of reverse flow in the duct arteriosus, ratio right ventricle-left ventricle > 2. The Ebstein anomaly diagnosed in utero has a perinatal mortality of 87.5%.

[Clinical guide practice. Patent ductus arteriosus]. / Guía de práctica clínica. Persistencia del conducto arterioso.

Patent ductus arteriosus (PDA) is the most common congenital heart disease in Mexico. The clinical manifestations of the PCA are from asymptomatic patients to the presence of heart failure. Its management should be individualized based on clinical, hemodynamic data and presence of pulmonary hypertension. Our objective was to provide current medical recommendations based on the best, available scientific evidence for the diagnosis, study and therapeutic decisions of the PCA. Established a standardized sequence to search for Practice Guidelines, based on the clinical questions about PCA diagnosis and treatment. Most of the recommendations were taken from selected guidelines and supplemented with the remaining material. The information is expressed in levels of evidence (E) and grade of recommendation (R) according to the characteristics of the study design and type of publications. Currently produces large amounts of medical information in a relatively short period of time which is necessary to have evidence-based CPG to facilitate and standardize the diagnostic decision-treatment to provide better care for children and adults with PCA.

[Fetal echocardiography efficiency. Clinical experience]. / Eficacia de la ecocardiografía fetal. Experiencia clínica.

BACKGROUND: Congenital heart disease diagnostic has a high diagnostic precision with fetal echocardiography. This study has been reported in populations with high risk and with a sensibility of 86 to 99% and specificity of 91 to 100%. OBJECTIVE: To know sensibility and specificity of fetal echocardiography in high-risk pregnancies, and to describe types and frequency of congenital heart disease in utero. MATERIAL AND METHOD: 229 files of pregnant women with high-risk factors, more than 15 weeks of gestation, and at birth cardiovascular exam were analyzed. This analysis was made by means of simple frequencies, sensibility, specificity, positive and negative predictive value, and truth index calculation. RESULTS: We found 62 (27%) cases with fetal heart disease. Mean of maternal age was 27 +/- 5.5 years, and of gestational age 31 +/- 5 weeks. Risk factors that require study were: four-chamber abnormality in routine ultrasound, dysmorphy, fetal bradicardia, and poll and oligohydramnios. There were 55 (88.7%) high-risk heart diseases, and most frequent were Ebstein's anomaly, unique ventricle, hypoplastic left ventricle syndrome, and tumors. Sensibility was 98.41%, specificity was 97.59%, positive prognostic value was 97.59%, and negative prognostic value was 99.39%. CONCLUSIONS: Fetal echocardiography has a high diagnosis certainty in our hospital unit, thus, it has to be a normal prenatal exam in pregnant women with high-risk factors.

[Endomyocardial biopsy. Revision and experience of 176 procedures]. / Biopsia endomiocárdica. Revisión y experiencia de 176 procedimientos.

Endomyocardial biopsy (EB) is often used in the clinical evaluation of several cardiac disease. Hundred-seventy-six consecutive procedures were performed in 65 patients, 43 men and 22 women mean age was 29.86 +/- 11.53 (range 4 days to 66 years). Group A, 26 postcardiac transplantation patients in whom 137 EB were performed (39 heterotopic and 98 orthotopic), average sample 5.2 biopsy for each patient. Group B (n = 39) was studied for several cardiac diseases during cardiac diagnostic catheterism. The jugular venous approach was performed in 3 patients (1.7%), femoral in 173 patients. Major complications were found in 3 (1.7%) cases consisting in cerebrovascular accident (stroke) and coronary fistula into the right ventricle. Endomyocardial biopsy provides a low incidence of adverse reactions, mortality was 0%.

Biopsia endomiocárdica: revisión y experiencia de 176 procedimientos / Endomyocardial biopsy: revision and experience of 176 procedures

Endomyocardial biopsy (EB) is often used in the clinical evaluation of several cardiac disease. Hundred-seventy-six consecutive procedures were performed in 65 patients, 43 men and 22 women mean age was 29.86 +/- 11.53 (range 4 days to 66 years). Group A, 26 postcardiac transplantation patients in whom 137 EB were performed (39 heterotopic and 98 orthotopic), average sample 5.2 biopsy for each patient. Group B (n = 39) was studied for several cardiac diseases during cardiac diagnostic catheterism. The jugular venous approach was performed in 3 patients (1.7%), femoral in 173 patients. Major complications were found in 3 (1.7%) cases consisting in cerebrovascular accident (stroke) and coronary fistula into the right ventricle. Endomyocardial biopsy provides a low incidence of adverse reactions, mortality was 0%.

[Right ventricular thrombous and pulmonary artery aneurysms in Behçet's disease. Report of one case]. / Trombo en ventriculo derecho y aneurismas de arterias pulmonares en enfermedad de Behçet. Reporte de un caso.

UNLABELLED: Behçet's disease uncommon in the pediatric population. Intracardiac thrombus and bilateral pulmonary artery aneurysms are uncommon manifestations. We are reporting one case. A 14 years old patient was admitted, with high fever, dyspnea and hemoptysis since 4 months ago. Two years ago, fever, oral ulcers, aphthae (gingival, palate, tonsils), nose ulceration in and arthralgias-arthritis were noted. Chest radiograph showed round mass in the right lower lung field. The chest tomographic computed scan and pulmonary centellography were done to investigate malformations arterial. An Intracardiac thrombus of 27 x 12 mm was identified in the right ventricle by transthoracic echocardiography. Catheterization and pulmonary angiography showed an aneurism located in the right and left lobares arteries. Medical management with immunosuppressive and anticoagulation therapy resulted in complete remission of the clinical manifestations. Due to heamodynamic compromise surgical removal of the intracardiac thrombus was done. IN SUMMARY: The Behçet's disease is rare disease in children. Intracardiac thrombus and bilateral pulmonary artery aneurysms are rare complications. Medical treatment (immunosupressive and anticoagulation) is the first line therapy with resolution of the mucous, skin, cardiac and pulmonary manifestations.

Trombo en ventriculo derecho y aneurismas de arterias pulmonares en enfermedad de Behçet: reporte de un caso / Right ventricular thrombous and pulmonary artery aneurysms in Behçet's disease: report of one case

Behçet's disease uncommon in the pediatric population. Intracardiac thrombus and bilateral pulmonary artery aneurysms are uncommon manifestations. We are reporting one case. A 14 years old patient was admitted, with high fever, dyspnea and hemoptysis since 4 months ago. Two years ago, fever, oral ulcers, aphthae (gingival, palate, tonsils), nose ulceration in and arthralgias-arthritis were noted. Chest radiograph showed round mass in the right lower lung field. The chest tomographic computed scan and pulmonary centellography were done to investigate malformations arterial. An Intracardiac thrombus of 27 x 12 mm was identified in the right ventricle by transthoracic echocardiography. Catheterization and pulmonary angiography showed an aneurism located in the right and left lobares arteries. Medical management with immunosuppressive and anticoagulation therapy resulted in complete remission of the clinical manifestations. Due to heamodynamic compromise surgical removal of the intracardiac thrombus was done. IN SUMMARY: The Behçet's disease is rare disease in children. Intracardiac thrombus and bilateral pulmonary artery aneurysms are rare complications. Medical treatment (immunosupressive and anticoagulation) is the first line therapy with resolution of the mucous, skin, cardiac and pulmonary manifestations.

[Diagnostic and frequency of fetal heart disease by echocardiography in pregnancies with high-risk factors]. / Diagnóstico y frecuencia de cardiopatía fetal mediante ecocardiografía en embarazos con factores de alto riesgo.

BACKGROUND: Fetal heart diseases are increasingly frequent. These are part of birth defects and, therefore, appear in early phases of the gestation. Diagnosis or early detection of functional and structural abnormalities of the heart allows to the obstetrician and the pediatric cardiologist to plan, in a timely manner, the treatment and prenatal and postnatal monitoring with the purpose of preventing complications. OBJECTIVE: To determine the types and frequencies of heart diseases in pregnancies with high-risk factors. PATIENTS AND METHOD: It was analized an observational, descriptive and retrospective series of cases. From January 2003 to June 2006, fetal echocardiograms were made in women with pregnancies longer than 15 weeks and with high-risk factors. At childbirth was made a cardiovascular evaluation. Analysis was carried out through descriptive statistics. RESULTS: maternal age average was of 27.3 +/- 6.8 years; gestational age was of 31 +/- 5 weeks. In total, there were carried out 275 fetal echocardiograms (FE) in 208 patients; in 56 of them, was made one fetal ecocardiogram, two in 37 and three in 15. Risk factors of reference to the specialized medical unit were: abnormalities detected through obstetrical ultrasound (35.1%), tachycardia-bradycardia-arrhythmia (29.8%), probable chromosomic anomalies (13.9%), oligohydramnios or polyhydramnios (4.8%), familiar antecedents of congenital heart diseases (3.8%), others (12.6%). Heart disease was diagnosed by fetal echocardiogram in 55 patient (26.4%): unique ventricle (n = 5), hypoplastic right ventricle (n = 3), hypoplastic left ventricle (n = 4), congenital complete heart block (n = 1), Ebstein and dysplasia of the mitral and tricuspid valves (n = 12), tumor (n = 5), ectopia cordis (n = 2), auriculoventricular channel (= 1), supraventricular tachycardia (n = 4), supraventricular extrasystole (n = 3) and other. Diagnosis was confirmed at childbirth in 99.5% of the cases. The treatment was initiated in uterus for arrhythmia and cardiac insufficiency in 19 patients. The patients with high-risk cardiopathy (at birth) were evaluated at beginning through specialized cardiological treatment and then they were transferred to the service of pediatric cardiology. CONCLUSIONS: Frequency of fetal heart diseases in patients with high-risk factors is of 25.4%. In these patients, the fetal echocardiogram (prenatal and postnatal) allows planning the optimal cardiological and obstetric treatment as well as to inform to the relatives.

Oclusión percutánea de conducto arterioso permeable con dispositivo de Rashkind. seguimiento a 4 años / Percutaneous occlusion of batent ductus arteriosus with Rashkind device

Se valoraron resultados y seguimiento del cierre de conducto arterioso con dispositivo de Rashkind. Se colocaron 66 dispositivos en 63 pacientes: 41 mujeres y 22 hombres, con edad promedio de 8.2 + 7.5 años. Se determinó el diámetro del conducto, la presencia o ausencia de fuga residual inmediata y evolución mínimo a 1 año bajo control ecocardiográfico. Las determinaciones hemodinámicas mostraron: morfología tipo A en 49, C en 7 y E en 7 pacientes, con diámetro de 4.2 ñ 1.4 mm (extremos de 2.3 y 8.7 mm) y un Qp/Qs de 2.2 + 1.5 (extremos de 0.7 y 8.6). Se colocaron 45 dispositivos n§ 17 y 18 del n§ 12. La fuga residual inmediata fue del 65 por ciento (n-41); a las 24 hrs. del 31.7 por ciento (n-20) y al año del 7.9 por ciento (n-5). A 3 pacientes, con fuga persistente, se les colocó una 2a. sombrilla, en otro se instaló además del dispositivo de Rashkind una espiral endovascular (coil) y un paciente se encuentra en espera de un segundo oclusor. Las complicaciones menores se presentaron en el 15.8 por ciento (n-10). Los resultados indican un índice de oclusión a las 24 hrs. del 68.3 por ciento y al año del 92.0 por ciento; ningún paciente presentó embolia del dispositivo, hemólisis, ni obstrucción de las ramas pulmonares. Por lo anterior consideramos que la oclusión del conducto arterioso con dispositivo de Rashkind es una opción segura y efectiva para su tratamiento.

Oclusión de conducto arterioso permeable con dispositivo de Gianturco-Grifka. primer caso en el IMSS / Transcatheter occlusión of patent ductus arteriosus with Gianturco-Grifka device

Nuestro objetivo es presentar la experiencia inicial de cierre de conducto arterioso, con un nuevo dispositivo de Gianturco-Grifka, en el Hospital General del Centro Médico "La Raza". Se trata de un paciente femenino de 4 años de edad, asintomática, a quien se le detectó soplo continuo a los 3 años 7 meses de edad. El estudio ecocardiográfico demostró la persistencia de conducto arterioso cónico largo, de 4.9 mm de diámetro, presión sistólica de la arteria pulmonar de 35 mm Hg, con QP/QS 1.6:1. El estudio hemodinámico diagnóstico encontró un conducto arterioso cónico largo de 5 mm de diámetro tipo A1 de Krichenko. Se procedió al cierre del conducto arterioso permeable con dispositivo de Gianturco-Grifka de 7 mm, hasta obtener la oclusión del 100 por ciento de dicho conducto, lo cual se verificó por angiografía. No se presentaron complicaciones ni accidentes durante el procedimiento. Es necesario el empleo de este dispositivo en un mayor número de pacientes para determinar a largo plazo sus beneficios y sus limitaciones, sin embargo podemos concluir que técnicamente es fácil de utilizar y se obtiene una mayor disminución de la fuga residual que se presenta con otros dispositivos.

Estudio comparativo entre dispositivos de Rashkind, Grifka y Coil para el cierre percutáneo del conducto arterioso / Comparative study between Rashkind, Grifka and Coil devices in the closure of patent ductus arteriosus

Evaluamos la eficacia de los dispositivos Grifka y Coil, comparándolos con el dispositivo de Rashkind, utilizados para el cierre percutáneo del conducto arterioso. Estudiamos 97 pacientes (36 hombres, 61 mujeres), analizando edad, sexo, diámetro del conducto, morfología, tipo de dispositivo, tiempo de oclusión, fuga persistente, requerimiento de 2o dispositivo y complicaciones. Se realizó análisis estadístico con t de Student y c2. La edad fue 7.82 ñ 6.89. A 45 pacientes se colocó Rashkind -17, a 19 Rashkind-12, a 18 Coil y a l3 bolsas de Grifka; en 2 pacientes no se colocó dispositivo. Hubo diferencias entre morfología y dispositivo empleado (p = 0.008), entre diámetro y dispositivo utilizado (p < 0.001). La oclusión fue inmediata en 26.7 por ciento con Rashkind-17, 57.9 por ciento Rashkind-12, 83.3 por ciento Coil y de 91.7 por ciento Grifka. A las 24 h, fue 60 por ciento Rashkind-17, 78.9 por ciento Rashkind-12, 94.4 por ciento Coil y 100 por ciento Grifka. Persistieron con fuga por más de un año 7 Rashkind-17 y 1 Rashkind 12, (p = 0.001), 4 pacientes tienen 2 dispositivos. Las complicaciones fueron: 15.5 por ciento para Rashkind-17, 26.3 por ciento Rashkind-12, 5.2 por ciento para Coil y de 30 por ciento para Grifka, (p = 0.004); embolizaron 1 Coil y 1 Grifka. El Coil y Grifka han mostrado mejor oclusión inmediata. Las indicaciones de cada dispositivo deberán ser establecidas de acuerdo a la morfología y tamaño del conducto arterioso.

Valvuloplastía pulmonar: Resultados a largo plazo. Experiencia en el Centro Médico La Raza / Pulmonary valvuloplasty: Long term results at the Centro Medico La Raza

El objetivo fue valorar la evolución a corto y largo plazo en pacientes sometidos a valvuloplastía pulmonar (VP), e identificar los factores pronóstico para el fracaso. Se estudiaron 109 pacientes, 60 mujeres y 49 hombres con edad promedio de 7.04 ñ 8.4 años. El gradiente pulmonar previo fue de 89.53 ñ 37.23 mmHg y post-valvulopastía de 20.8 ñ 19.41 mmHg. (p < 0.0001), 43 pacientes presentaron gradiente infundibular post-valvular en 8 años de seguimiento fue 20.75 ñ 14.32 (p< 0.001). El porcentaje de éxito fue 86.2 por ciento, con una mortalidad global de 1.9 por ciento, complicaciones menores en 15.2 por ciento, falla del procedimiento en 13.8 por ciento. El 66.1 por ciento tenía estenosis pulmonar aislada y el 33.9 por ciento, presentaba lesiones asociadas. La reestenosis fue del 6.7 por ciento. Separando el análisis entre el grupo de éxito y de fracaso, las variables significativas que se encontraron en el grupo de fracaso fueron edad menor de 3.5 años y la morfología valvular displásica (p< 0.05) o combinada (p< 0.05), teniendo mayor número de complicaciones (p< 0.05). Los resultados inmediatos en el grupo de fracaso fueron un gradiente alto post-valvuloplastía (p< 0.05). La valvuloplastía pulmonar es procedimiento electivo tanto por sus resultados inmediatos como a largo plazo, siendo en nuestro medio un método efectivo y seguro

Angioplastía transluminal percutánea en coartación aórtica : resultados a corto y mediano plazo / Percutaneous transluminal angioplasty in aortic coarctation :

Se realizó la angioplastía transluminal percutánea (ATP) en 34 pacientes con el diagnóstico de coartación aórtica (CoAo). Uno con reestenosis postquirúrgica y el resto con CoAo nativa. Se utilizó un solo globo en 28 pacientes y dos simultáneamente en 6. Se dividieron en 3 grupos según el grado de hipoplasia del arco aórtico. Grupo I (Hipoplasia leve a moderada N=9) durante el seguimiento (m=13.1 meses) se obtuvo una caída del gradiente de 39 por ciento y mejoría angiográfica del 48 por ciento Tres casos se recoartaron, 2 fueron redilatados satisfactoriamente y uno fue enviado a cirugía. En el grupo II (Hipoplasia severa N=4) con un seguimiento de 16.3 meses, el gradiente descendió 31 por ciento con mejoría angiográfica del 30 por ciento Dos casos se recoartaron y fueron enviados a cirugía. En el grupo III (sin hipoplasia N=21) se obtuvo una caída del gradiente de 71 por ciento con incremento de 60 por ciento en el diámetro de segmento coartado en un seguimiento de 18.5 meses. Dos casos fueron redilatados con éxito. Las complicaciones fueron: Hemorragia cerebral por crisis hipertensiva falleció 1 sujeto, embolia cerebral (1), trombosis en el sitio de punción (1) y aneurisma en el sitio dilatado (1). En resumen, pensamos que la TP es una buena alternativa a la cirugía convencional, con baja morbimortalidad; los resultados dependen fundamentalmente del tipo anatómico de coartación y el grado de hipoplasia del arco aórtico