Pilar Arnaiz G

Perfil de presión arterial e historia familiar de hipertensión en niños escolares sanos de Santiago de Chile

Data of 112 children aged between 6-12 years was analyzed. Hypertension (BP>percentile 95) was detected in 2.7% and pre hypertension (BP in percentiles 90-95) in 3.6% of thesample. Children with abnormal BP had higher levels of usCRP (p <0.05) and a non significanttendency towards a higher body mass index. All hypertensive and one pre hypertensive children hadFH of AHT. Eleven percent of parents, had high blood pressure. In no children, both parents werehypertensive. Children with a family history of hypertension had higher concentrations of totalserum cholesterol (p <0.05).

Comportamiento clínico de los tumores cardíacos desde el feto hasta el adulto: serie multicéntrica de 38 pacientes

BACKGROUND Cardiac tumors are very uncommon at all ages. There are important clinical differences between children and adults in the behavior of these tumors. AIM To compare the behavior of primary and secondary cardiac tumors, from fetal age to adults. PATIENTS AND METHOD Multicentric retrospective analysis of 38 children and adults with cardiac tumors, evaluated with echocardiography between January 1995 and August 2001. Medical records, echocardiographic and radiological examinations, surgical protocols and pathologic examinations were reviewed. Follow-up was obtained through data on medical records or calling patients by telephone. RESULTS Tumors were diagnosed in 38 patients (13 children and 25 adults), from a total of 31.800 echocardiograms. In children the diagnosis was made by fetal, transthoracic or transesophageal echocardiography in 23.6% and 8% of cases, respectively. Eighty five percent were primary (10 benign and 1 malignant) and 15%, secondary tumors. Fifty four percent were rhabdomyomas and 75% regressed spontaneously. Seventy seven percent were symptomatic and 31% were treated with surgery. During a follow up of 44+/-35 months, 31% of patients died. In adults, 76% of tumors were diagnosed by transthoracic and 20% by transesophageal echocardiography. Seventy six percent were primary (18 benign and 1 malignant) and 24% secondary tumors. Fifty six percent were myxomas. Ninety two percent were symptomatic and 84% were treated surgically. Twenty percent of patients died in the early postoperative period. No adult patients had a follow-up. CONCLUSIONS Rhabdomyomas were solely found in children. In adults, myxomas are the predominant cardiac tumors. Primary and metastasic malignant tumors are observed both in children and in adults.Multicentric retrospective analysis of 38 children andadults with cardiac tumors, evaluated with echocardiography between January 1995 andAugust 2001. Medical records, echocardiographic and radiological examinations, surgicalprotocols and pathologic examinations were reviewed. Follow-up was obtained through data onmedical records or calling patients by telephone.

Mixoma cardiaco: Caracterización clínica, métodos diagnósticos y resultados alejados del tratamiento quirúrgico durante tres décadas de experiencia

Background: Cardiac myxoma is the most common primary cardiac tumor. Aim: To evaluate clinical aspects, diagnostic methods and surgical outcomes in patients with cardiac myxoma. Patients and Methods: All patients who underwent surgical resection of a cardiac myxoma between January 1973 and December 2004 at our institution, were identified and their medical records and diagnostic data reviewed. Results: Thirty seven patients identified (24 women), with a median age of 54 years (range 2-74). The most common symptom was dyspnea (51%), followed by embolism (24%) and cardiac murmurs (16%). The diagnosis was made by echocardiogram in 84% of patients. The mean tumor size was 4.9 ± 1.3 cm. The tumor was located in the left atrium in 83% of the patients, right atrium in 8% and left ventricle in 3%. Two patients had multiple tumors. Surgical approach was transeptal in 51%, left atriotomy in 22%, combined transeptal and roof of the left atrium in 19% and right atriotomy in 8%. The tumor and its attachment base were excised in one piece. There were two operative deaths and five late deaths. The mean follow-up was 11.5 years. The 5, 10, 15 and 20 years survival was 89%, 83%, 75% and 64%, respectively. Two patients had recurrence (5.4%), at 2 and 25 years after surgery, respectively, and the recurrence free survival at 10 and 25 years was 97% and 73%, respectively. Conclusions: Dyspnea was the most common symptom and echocardiogram the main diagnostic method. Surgical treatment was highly effective, with low mortality and recurrence rate, with good long term survival (Rev Med Chile 2008; 136: 287-95). (Key words: Echocardiography; Heart neoplasms; Myxoma)

Heterogeneidad de la presentación clínica del síndrome de microdeleción del cromosoma 22 región q11

Background: DiGeorge anomaly, velocardiofacial syndrome and conotruncal anomaly face syndrome are part of a group of congenital malformations of the chromosome 22q11 microdeletion syndrome, since they share certain phenotypic features as well as a common genetic abnormality. The malformations include mild facial dysmorphic features, conotruncal heart defects, thymic and parathyroid hypoplasia or aplasia and cleft palate. Aim: To describe the initial clinical presentation of children with clinical and molecular diagnosis of 22q11 microdeletion. Patients and methods: Ten children (seven male) with the phenotypic features of 22q11 microdeletion syndrome are reported. Microdeletion was detected in peripheral Iymphocytes by fluorescent in situ hybridisation (FISH) with the TUPLE-1 DNA probe. Results: Two children had abnormal karyotypes, one of them had a visible deletion and another child had an unbalanced translocation inherited from his mother who had a balanced translocation between chromosomes 14 and 22. Two of the 10 patients had an anterior laryngeal web, a malformation infrequently described in this syndrome. Five patients had the diagnosis of DiGeorge anomaly, had a more serious clinical presentation and a higher early mortality. Conclusions: The high frequency of the 22q11 microdeletion syndrome, estimated at 1:5.000 newborns, and its variable presentations requires a high level of awareness for its early diagnosis and appropriate management of associated complications. (Rev Med Chile 2001; 129: 515-21)

Operación de Fontan: resultados inmediatos y a mediano plazo con anastomosis cavopulmonar total

Background: During the last five years, 65 patients with univentricular heart have been treated surgically in our institution, according to a protocol of staged operations that have been previously reported. Aim: To evaluate the early and mid-term outcome of those patients that have completed their staging protocol by means of a Fontan procedure. Patients and Methods: Between April 1996 and June 2001, 23 patients (age 16 to 223 months) underwent a Fontan procedure, 15 with an intracardiac lateral tunnel technique and 8 with an extracardiac conduit. A retrospective review of their clinical, surgical, echocardiographic, angiographic and hemodynamic data was performed, trying to identify risk factors for both mortality and functional capacity (FC). Follow up was complete in all survivors. Results: Three patients died early after surgery (13.04%). Excessive pulmonary blood flow was a risk factor for early death (p= 0.03). One patient died at 14 months. Follow up was 29.9 months (1-63). For those who survived the operation, five years survival was 93.3%. The majority of patients are in FC I or II, with no related risk factors. Conclusions: Our current results are comparable with those of larger series. Patients reach good FC and mid-term survival, irrespective of type of single ventricle or the surgical strategy (Rev Med Chile 2002; 130: 1217-26).

Arterioesclerosis subclínica, factores de riesgo cardiovascular clásicos y emergentes en niños obesos chilenos

La arteriosclerosis puede comenzar en la ninez y desarrollarse cronicamente dependiendo de la carga de factores de riesgo (FR) cardiovascular. Objetivo: Comparar ninos obesos con eutroficos en cuanto a FR clasicos, emergentes (Proteina C Reactiva ultrasensible: PCRus) y arteriosclerosis subclinica, mediante dos nuevas tecnicas no invasivas: dilatacion mediada por flujo de la arteria braquial (DMF) y grosor de la intima-media carotidea (IMT). Metodo: Se estudiaron 26 ninos obesos (IMC ³ Pc95) y 57 eutroficos (IMC: Pc10 - Pc85). Se evaluo antropometria, presion arterial (PA), DMF, IMT, y se determino de PCRus, perfil lipidico y glicemia de ayunas. Resultados: El 50% fueron mujeres y 41% prepuberes. Con edad de 9,9 ± 1,6 y 9,8 ± 1,8 anos (ns), zIMC: 2,0 ± 0 2 y 1,7 ± 0,6, perimetro de cintura (% Media): 133,5 ± 16 y 100,5 ±1 0% en obesos y eutroficos respectivamente. Los obesos tuvieron mayor Colesterol Total, CLDL, Trigliceridos, PCRus y menor CHDL (p < 0,005). No hubo diferencia significativa en DMF: 9,03 ± 5,2% vs 9,3 ± 4,2%, IMT: 0,49 ± 0,03 vs 0,50 ± 0,03 mm, glicemia ni PA. Conclusion: Este grupo de ninos obesos chilenos presenta mayor carga de FR clasicos y nivel de PCRus que los eutroficos, pero no se encontro diferencia significativa en marcadores sustitutos de arteriosclerosis subclinica

Cirugía paliativa mediante operación de Norwood en distintas formas de corazón univentricular: resultados quirúrgicos inmediatos

: Norwood procedure is used as the first stage in thepalliative treatment of the hypoplastic heart syndrome and can be used, with some technicalmodifications, in other forms of univentricular heart with aortic stenosis or hypoplasia. Thesepatients have a high mortality (50%), derived from the procedure itself and from their abnor-mal physiological status.

Mixoma auricular izquierdo recurrente en niño de 2 años

Primary cardiac tumors are uncommon in pediatric patients. We report a two year-old boy, who presented a recurrent left atrial myxoma. He was referred for cardiologic evaluation because of cardiomegaly on a chest X-ray. An echocardiography showed a big left atrial tumor attached to the atrial septum. The tumor was uneventfully removed and the pathological examination confirmed a myxoma. He remained asymptomatic and during a follow up echocardiography two years later, a recurrent tumor was noted. He was successfully operated again, remaining free of a new recurrence after 3 years of follow up.

Resonancia magnética en el estudio de cardiopatías congénitas: rol en la evaluación de las estructuras vasculares extracardiacas

En la actualidad la resonancia magnetica ha adquirido cada vez mas importancia en el diagnostico y seguimiento de las cardiopatias congenitas. Entrega informacion anatomica y mas recientemente informacion funcional. Se presenta la experiencia acumulada entre enero y agosto del ano 2002, en cinco casos pediatricos en que la RM cardiaca fue utilizado como metodo complementario a la ecocardiografia y angiografia. En todos los casos aporto informacion anatomica precisa, en especial de la anatomia vascular extracardiaca y fue determinante en la conducta terapeutica. Se discute ademas las caracteristicas de metodo e indicaciones

Cierre transcatéter de ductus arterioso persistente con espirales de Gianturco

Objetivo: evaluar retrospectivamente el rendimiento del cierre del ductus arterioso persistente (DAP) con tecnica transcateter, utilizando espirales (coils) de Gianturco. Pacientes y Metodo: entre octubre de 1996 y septiembre de 2000, 22 pacientes de peso mayor de 10 kg (10,5 a 59), portadores de DAP de hasta 4 mm en su diametro menor, fueron sometidos a esta tecnica de cierre. Bajo sedacion con midazolam y ketamina, se efectuo sondeo cardiaco via arteria y vena femoral derechas, y aortografia. Por medio de un cateter multiproposito se avanzo espiral de Gianturco a traves del ductus desde su extremo aortico hasta posicionarlo en el DAP. Resultados: se logro cierre angiografico ductal en 22 pacientes (100%), observandose minima filtracion residual por ecocardiograma con Doppler color en 3 pacientes (14%), consignandose ausencia de filtracion a los 15 dias en dos de ellos. Todos los pacientes fueron dados de alta a las 24 horas. Cuatro pacientes presentaron embolizacion del espiral, los que fueron recuperados durante el procedimiento. Conclusion: el cierre transcateterismo de DAP con espirales de Gianturco es una tecnica segura y efectiva, constituyendo una muy buena alternativa al cierre quirurgico, con el beneficio adicional de no requerir anestesia general ni toracotomia.