Velocardiofacial syndrome in Mexican patients: Unusually high prevalence of congenital heart disease.

INTRODUCTION: Velocardiofacial syndrome (VCFS) is the most common microdeletion syndrome with an incidence of 1:4000 live births. Its phenotype is highly variable with facial, velopharyngeal, cardiac, endocrine, immunologic and psychiatric abnormalities. It is caused by a microdeletion in chromosome 22q11.2. OBJECTIVES: We present 7 years of experience evaluating patients with VCFS regarding their main clinical characteristics. MATERIAL AND METHODS: The patients included were multidisciplinary evaluated and had a positive FISH analysis for del22q11.2. RESULTS: A total of 62 patients were assessed, a 34 female/28 male ratio was observed with ages ranging from 9 days to 16 years, all but one patient had typical facial features. A diagnosis of congenital heart disease was established in 97% of the patients; other clinical characteristics were identified with different percentages such as cleft palate, and hypocalcaemia. Three cases had a familial presentation. DISCUSSION: While the clinical findings of this study were in general terms in keeping with the literature, it is interesting the unexpectedly high percentage of congenital heart disease identified in Mexican children with VCFS that also was the main cause for clinical referral.

Mexican registry of pediatric cardiac surgery: First report

Background: Currently, there is a spreading worldwide tendency to characterize health issues and to propose alternative solutions via the creation of computerized databases. The aim of this study was to present the results in a computerized database of pediatric cardiac surgeries developed under the auspices of the Mexican Association of Specialists in Congenital Heart Diseases (Asociación Mexicana de Especialistas en Cardiopatías Congénitas A.C) and coordinated by the collegiate group of Pediatric Cardiology and Surgery as petitioned by the National Institutes of Health and High Specialty Hospitals Coordinating Commission. Methods: We analyzed all cases registered in the database during a 1-year observation period (August 1, 2011 to July 31, 2012) by all major Health Ministry-dependent institutes and hospitals offering surgical services related to pediatric cardiopathies to the non-insured population. Results: Seven institutions participated voluntarily in completing the database. During the analyzed period, 943 surgeries in 880 patients with 7% reoperations (n = 63) were registered. Thirty-eight percent of the surgeries were performed in children <1 year of age. The five most common cardiopathies were patent ductus arteriosus (n = 96), ventricular septal defect (n = 86), tetralogy of Fallot (n = 72), atrial septal defect (n = 68), and aortic coarctation (n = 54). Ninety percent of surgeries were elective and extracorporeal circulation was used in 62% of surgeries. Global mortality rate was 7.5% with the following distribution in the RACHS-1 score categories: 1 (n = 4, 2%), 2 (n = 19, 6%), 3 (n = 22, 8%), 4 (n = 12, 19%), 5 (n = 1, 25%), 6 (n = 6, 44%), and non-classifiable (n = 2, 9%). Conclusions: This analysis provides a representative view of the surgical practices in cardiovascular diseases in the pediatric population at the national non-insured population level. However, incorporating other health institutions to the national registry database will render a more accurate panorama of the national reality in surgical practices in the population <18 years of age.

Mexican registry of pediatric cardiac surgery. First report.

BACKGROUND: Currently, there is a spreading worldwide tendency to characterize health issues and to propose alternative solutions via the creation of computerized databases. The aim of this study was to present the results in a computerized database of pediatric cardiac surgeries developed under the auspices of the Mexican Association of Specialists in Congenital Heart Diseases (Asociación Mexicana de Especialistas en Cardiopatías Congénitas A.C) and coordinated by the collegiate group of Pediatric Cardiology and Surgery as petitioned by the National Institutes of Health and High Specialty Hospitals Coordinating Commission. METHODS: We analyzed all cases registered in the database during a 1-year observation period (August 1, 2011 to July 31, 2012) by all major Health Ministry-dependent institutes and hospitals offering surgical services related to pediatric cardiopathies to the non-insured population. RESULTS: Seven institutions participated voluntarily in completing the database. During the analyzed period, 943 surgeries in 880 patients with 7% reoperations (n=63) were registered. Thirty-eight percent of the surgeries were performed in children <1 year of age. The five most common cardiopathies were patent ductus arteriosus (n=96), ventricular septal defect (n=86), tetralogy of Fallot (n=72), atrial septal defect (n=68), and aortic coarctation (n=54). Ninety percent of surgeries were elective and extracorporeal circulation was used in 62% of surgeries. Global mortality rate was 7.5% with the following distribution in the RACHS-1 score categories: 1 (n=4, 2%), 2 (n=19, 6%), 3 (n=22, 8%), 4 (n=12, 19%), 5 (n=1, 25%), 6 (n=6, 44%), and non-classifiable (n=2, 9%). CONCLUSIONS: This analysis provides a representative view of the surgical practices in cardiovascular diseases in the pediatric population at the national non-insured population level. However, incorporating other health institutions to the national registry database will render a more accurate panorama of the national reality in surgical practices in the population <18 years of age.

El ecocardiograma y su papel en niños con cáncer / Role of echocardiogram in children with cancer

Introducción. Actualmente las antraciclinas se han incorporado en más de 50% de los protocolos de tratamiento para cáncer en la población infantil. La utilidad clínica de estos agentes queda limitada por la aparición de cardiomiopatías, cuya presencia depende de la dosis total del fármaco y suele ser irreversible. El ecocardiograma es el estudio más utilizado para la detección de cardiotoxicidad por antraciclinas. El objetivo de este trabajo fue describir la evolución de los índices de funcionamiento cardiaco reportados por ecografía durante el tratamiento de niños con cáncer con esquemas que contenían antraciclinas. Métodos. Se incluyeron pacientes pediátricos con diagnóstico de osteosarcoma y leucemia mieloide aguda tratados entre enero del 2006 y mayo del 2011 en el Hospital Infantil de México Federico Gómez. En el expediente clínico se documentaron los valores de las fracciones de eyección y acortamiento reportadas antes de cada ciclo. Se utilizó estadística descriptiva para el reporte de los resultados. Resultados. La fracción de eyección prácticamente no sufrió variaciones hasta el sexto ciclo de tratamiento a una dosis acumulada de 332.5 mg/m²SC, para posteriormente decaer de forma acelerada. En la fracción de acortamiento el mayor cambio se produjo después del séptimo ciclo, a una dosis acumulada de 450 mg/m²SC. Conclusiones. Reconocer el daño que se produce en etapas tempranas es un paso fundamental en la prevención de complicaciones. El reto es implementar nuevas herramientas que nos permitan alcanzar el objetivo de prevención o de diagnóstico de enfermedad subclínica.

Background. Currently, anthracyclines have been used in >50% of cancer treatment protocols in children. The clinical usefulness of these agents is limited by the onset of cardiomyopathy whose presence depends on the total dose of drug and usually is irreversible. Echocardiography is used to study anthracycline cardiotoxicity detection. The aim of this study was to describe the evolution of cardiac function reported by echocardiography for treatment of children with cancer whose medications contain anthracyclines. Methods. We included pediatric patients diagnosed with osteosarcoma and acute myeloid leukemia treated between January 2006 and May 2011 in the Hospital Infantil de México Federico Gómez. In the clinical files, values were documented for ejection fraction and shortening reported before each cycle, using descriptive statistics for reporting results. Results. The ejection fraction experiences virtually no changes until the 6th cycle of treatment to a cumulative dose of 332.5 mg/m²/sc, afterwards it demonstrates an accelerated decline. According to the shortening fraction, the most significant change occurs after the seventh cycle to a cumulative dose of 450 mg/m²/sc. Conclusions. Recognizing the damage that occurs during the early stages is a critical step in preventing complications. The challenge is to implement new tools that will allow us to achieve the objective of preventing or diagnosing subclinical disease.

Polymorphism 677C → T MTHFR gene in Mexican mothers of children with complex congenital heart disease.

Congenital heart defects (CHD) are the third leading cause of death in children <1 year of age in Mexico where there is a high prevalence of the 677C → T polymorphism of the MTHFR gene. This is important because the homozygous 677T/T MTHFR gene and deficiency of folic acid (FA) intake have been associated with CHD. Our objective was to analyze the possible association between the genotype 677T/T of the MTHFR gene and supplementation of FA in Mexican women with the presence of complex CHD in their children. We analyzed genotypes of 31 mothers of children with complex CHD (group I) and 62 mothers of healthy children (group II) and investigated FA supplementation during pregnancy in both study groups. Allele frequencies in group I were 41.9 % for C and 58.1 % for T and 22.6 % for genotype frequencies CC, 38.7 % for CT, and 38.7 % for TT. Allele frequencies in group II were 63.7 % for C and 36.3 % for T and 38.7 % for genotype frequencies CC, 50 % for CT and 11.3 % for TT. Both populations are in Hardy-Weinberg equilibrium. Odds ratio for having a child with a complex CHD was 5.9, p = 0.008 (95 % CI 1.67; 20.63) for the TT genotype. FA supplementation at any time during pregnancy was 90.3 and 87.9 % in groups II and I respectively (p > 0.05). Association was found between the maternal genotype (677/TT MTHFR) with the presence of complex CHD in their offspring. No differences in FA supplementation during any stage were found between groups.

[Pediatric cardiovascular surgical data base registry in México. First report]. / El Registro Mexicano de Cirurgía Cardiaca Pediátrica. Primer informe.

INTRODUCTION: Current world tendency is the detection of health problems in order to offer solution alternatives by means of the development of computarized data bases. OBJECTIVE: To present the results of a computerized data base developed for the registry of pediatric cardiac surgery with the support of Asociación Mexicana de Especialistas en Cardiopatías Congénitas (AMECC, A.C.). MATERIAL AND METHODS: A one-year analysis (from August 1, 2011 to July 31, 2012) of a computerized data base was performed with the support of AMECC and the participation of the most important Mexican institutions for pediatric surgical heart disease health care, particularly for the uninsured population. RESULTS: There were 7 health institutions voluntarily incorporated to the national data base registry, and in the first year of observation, 943 surgical procedures in 880 patients and 7% re-operations (n = 63), were reported. Patients up to one-year old accounted for 38%. The most frequent types of operated congenital heart diseases were: patent ductus arteriosus (n = 96), ventricular septal defect (n = 86), tetralogy of Fallot (n = 72), atrial septal defect (n = 68), and aortic coarctation (n = 54). Elective procedures were 90%, and 62% of them were performed with the use of cardiopulmonary bypass. Overall mortality was 7.5% with the following RACHS-1 score risk distribution: 1 (n = 4.2%), 2 (n = 19.6%), 3 (n = 22.8%), 4 (n = 12.19%), 5 (n = 1.25%), 6 (n = 6.44%) and not classifiable (n = 2.9%). CONCLUSIONS: Although this analysis gives a representative vision of the cardiovascular surgical health care for the uninsured national pediatric population, the incorporation of other health institutions to this data base may lead us to have a most realistic overview in relation to the surgical cardiovascular health care for the up to 18 year-old population.

Pentalogy of Cantrell: Forty-two Years of Experience in the Hospital Infantil de Mexico Federico Gomez.

Pentalogy of Cantrell is a rare disease. Approximately 185 cases have been reported around the world. The authors performed a retrospective study that reviewed the clinical files and pathological samples of 22 cases of pentalogy of Cantrell treated at the Hospital Infantil de México Federico Gómez. Thirteen patients had ectopia cordis associated with pentalogy of Cantrell (group I), and there were 9 cases without ectopia cordis (group II). In group I, the following types of congenital heart disease were found: single ventricle (4), double-outlet right ventricle (4), ventricular septal defect (3), aortic coarctation (1), and atrial septal defect (1). In group II, the following types of congenital heart disease were found: double-outlet right ventricle (3), double-inlet left ventricle (2), ventricular septal defect (2), tetralogy of Fallot (1), and hypoplastic right ventricle syndrome (1). Nine cases had a ventricular diverticulum (40%). Ten patients (45%) had some other congenital anomaly associated with pentalogy of Cantrell. Thirteen patients underwent surgery (59%), which included cardiac surgery in 10 cases (45%). Sixteen patients died (73%): 11 from group I and 5 from group II (P < .05). Little more than 50 years since it was first described, pentalogy of Cantrell remains a disease with high mortality, especially in patients with associated ectopia cordis.

Pericarditis purulenta y neoplásica en un paciente con leucemia aguda linfoblástica. Reporte de un caso y revisión de la literatura / Purulent and neoplasic pericarditis in a patient with acute lymphoblastic leukemia: case report and literature

Introducción. La leucemia aguda linfoblástica (LAL) es la neoplasia maligna más frecuente en los niños y constituye el 25% de todos los tipos de cáncer. Actualmente, la supervivencia en estos niños es superior al 70% debido al mejor entendimiento de su fsiopatología, al desarrollo de nuevos agentes de quimioterapia y a la mejoría de los cuidados de soporte y manejo de las complicaciones propias de la enfermedad y secundarias al tratamiento. Caso clínico. Reportamos un caso de septicemia y pericarditis purulenta por estafilococo dorado en el cuadro inicial de una LAL. La pericarditis en el curso de una leucemia en niños es una manifestación inusual y puede representar la muerte del paciente. Hay 18 casos reportados en el mundo de niños con leucemia y pericarditis. En 6 de ellos se encuentra una etiología infecciosa incluyendo S. aureus, Phytum insidiosum, Candida sp., S. pneumoniae y dos por Aspergillus. Conclusiones. Éste es el segundo caso de pericarditis purulenta por estafilococo reportado en la literatura. Representa el tercer caso de infltración pericárdica publicado en nuestro hospital. Es importante resaltar que la vía de entrada del estafilococo pudo ser mediante un procedimiento de acupuntura previo al diagnóstico. Estos recursos de medicina alternativa son ampliamente utilizados por nuestra población.

Background. Acute lymphoblastic leukemia (ALL) represents the most common cause of childhood cancer, accounting for 25% of all malignancies. Overall survival has improved remarkably due to the better understanding of its pathophysiology, the creation of new chemotherapy regimes and the improvement of the supportive care and the management of the disease complications as well as to complications secondary to treatment. Case report. We report a case of septicemia and purulent pericarditis secondary to Staphylococcus aureus in the initial presentation of ALL. Pericarditis in the course of leukemia in children is an unusual presentation. There are 18 cases reported worldwide of children with leukemia and pericarditis. In six cases an infectious etiology was found including S. aureus, Candida sp, Phytum insidiosum, S. pneumonia and two by Aspergillus. Conclusions. This is the second case of purulent pericarditis secondary to S. aureus reported in the literature. It represents the third case of pericardial infltration of leukemia reported by our hospital. It is important to note that S. aureus may have entered the bloodstream through an acupuncture procedure performed prior to diagnosis. These alternative resources are widely used in the Mexican population.

Morphometric study of the ventricular segment of the human fetal heart between 13 and 20 weeks' gestation.

UNLABELLED: Our objective was to determine the normal dimensions of the ventricular segment of the human fetal heart between 13 and 20 weeks' gestation. STUDY DESIGN: 103 hearts obtained by necropsy were dissected and measurements of different portions of ventricles were determined under stereoscopic magnification. In each ventricle were measured anteroposterior and lateral diameters, inlet and outlet length, and thickness of walls at different levels. Our results showed the cardiac apex was constituted by the left ventricle in 68.9% of the hearts. Both ventricles showed linear growth during this period of fetal development. Ranges in median values of external and internal ventricular measurements were determined. The left ventricular wall was thicker than the right, and the right ventricular cavity was larger. This study provides morphometric reference information concerning the dimensions and growth of both ventricles of the fetal heart, which may be useful in pediatric cardiac surgery and echocardiography.

[Left ventricular function in children after successful repair of aortic coarctation]. / Función ventricular izquierda en niños después de la reparación exitosa de la coartación aórtica.

INTRODUCTION AND OBJECTIVES: Lifetime prognosis following successful repair of aortic coarctation can be affected by a number of late complications. The objective of this study was to assess left ventricular function in these patients and to identify factors that predispose to functional abnormalities. METHODS: The study involved patients who had undergone repair of aortic coarctation and who had a pressure gradient pound 15 mmHg after repair and normal systemic blood pressure. Echocardiographic data collected before repair and the results of the most recent postoperative left ventricular function studies were analyzed. RESULTS: The study involved 40 patients and 31 controls. Their mean age at repair was 6.9 years and the mean follow-up period was 4.25 years. During follow-up, the ejection fraction and the shortening fraction were observed to increase in 82.5% and 67.5% of patients, respectively. The myocardial performance index was abnormal in 47.5% of patients. The highest myocardial performance indices were observed in patients with arterial hypertension at diagnosis, in those who were aged over 4 years when they underwent repair, and in those with the most abnormal left ventricles before repair. CONCLUSIONS: Measurement of the myocardial performance index showed that global left ventricular function was abnormal in 47.5% of patients after successful repair of aortic coarctation despite functional parameters being normal or elevated.

Función ventricular izquierda en niños después de la reparación exitosa de la coartación aórtica / Left ventricular function in children after successful repair of aortic coarctation

Introducción y objetivos. El pronóstico para la vida después de una corrección exitosa de coartación aórtica puede verse afectado por varias complicaciones tardías. Nuestro objetivo fue evaluar la función del ventrículo izquierdo en este grupo de pacientes y tratar de identificar factores que predisponen a anomalías circulatorias. Métodos. Se incluyó a pacientes con reparación de coartación aórtica que tuvieron un gradiente en la zona de reparación £ 15 mmHg y presión arterial normal. Se analizaron sus ecocardiogramas antes de la corrección y se estudió la función ventricular izquierda al postoperatorio actual. Resultados. Se estudió a 40 pacientes y 31 controles. La edad de la reparación fue 6,9 años, con 4,25 años de seguimiento. La fracción de eyección y la fracción de acortamiento se encontraron aumentadas en el 82,5 y el 67,5% de los casos, respectivamente, durante el seguimiento. El índice de rendimiento miocárdico fue anormal en el 47,5% de los pacientes. Los pacientes que tenían hipertensión arterial al momento del diagnóstico, los que se corrigieron después de los 4 años y los que tenían un ventrículo izquierdo más anormal antes de la corrección tuvieron índices de rendimiento miocárdico más elevados. Conclusiones. La función ventricular izquierda general evaluada con el índice de rendimiento miocárdico fue anormal en el 47,5% de los casos después de una corrección de coartación aórtica exitosa, pese a tener índices endocárdicos normales o aumentados (AU)

Introduction and objectives. Lifetime prognosis following successful repair of aortic coarctation can be affected by a number of late complications. The objective of this study was to assess left ventricular function in these patients and to identify factors that predispose to functional abnormalities. Methods. The study involved patients who had undergone repair of aortic coarctation and who had a pressure gradient £15 mm Hg after repair and normal systemic blood pressure. Echocardiographic data collected before repair and the results of the most recent postoperative left ventricular function studies were analyzed. Results. The study involved 40 patients and 31 controls. Their mean age at repair was 6.9 years and the mean follow-up period was 4.25 years. During follow-up, the ejection fraction and the shortening fraction were observed to increase in 82.5% and 67.5% of patients, respectively. The myocardial performance index was abnormal in 47.5% of patients. The highest myocardial performance indices were observed in patients with arterial hypertension at diagnosis, in those who were aged over 4 years when they underwent repair, and in those with the most abnormal left ventricles before repair. Conclusions. Measurement of the myocardial performance index showed that global left ventricular function was abnormal in 47.5% of patients after successful repair of aortic coarctation despite functional parameters being normal or elevated (AU)

Resultados y complicaciones del cateterismo cardiaco en el recién nacido / Outcomes and complications of cardiac catheterization in neonates

Introducción. Objetivos: determinar el riesgo de complicaciones del cateterismo cardiaco diagnóstico y terapéutico en el período neonatal, y evaluar los resultados obtenidos. Métodos. Se revisaron 95 cateterismos cardiacos consecutivos llevados a cabo en pacientes menores de 28 días que representaron 3.2% de 2 879 cateterismos practicados de 1991 a 2006 en el Hospital Infantil de México Federico Gómez. Resultados. La edad media fue 17.5 ± 0.7 días, peso 2.9 ± 0.98 kg, 59% del género masculino. En 73 (77%) se llevó a cabo un cateterismo terapéutico (Cat-T) y en 22 (23%) un cateterismo diagnóstico (Cat-D). Las técnicas de Cat-T incluyeron: septostomía atrial en 54 pacientes, valvuloplastia aórtica en 8, valvuloplastia pulmonar en 5, angioplastia de coartación aórtica en 3, implantación de marcapasos transvenosos en 2 y férula al conducto arterioso en 1. Se consideraron 24 (25.2%) de complicaciones mayores y 14 (14-7%) menores; en las primeras, las arritmias y las alteraciones vasculares fueron las más comunes; sin embargo, sólo 5 pacientes con complicaciones (5.2%) necesitó de algún procedimiento quirúrgico de urgencia. La muerte atribuible directamente al cateterismo cardiaco ocurrió en 2 casos (2.1%). En los 22 casos de Cat-D la correlación del diagnóstico anatómico con los hallazgos quirúrgicos fue excelente. En los 73 Cat-T, se logró una paliación o corrección satisfactoria en 94.6%. Conclusiones. Estos resultados confirman que el cateterismo cardiaco puede llevarse a cabo en el neonato con buenos resultados, independientemente de la edad y estado crítico; aunque el riesgo de complicaciones es alto, la posibilidad de muerte es baja. En este grupo de pacientes sumamente graves los resultados son alentadores.

Introduction. The aim of this study was to determine the relative risks of diagnostic and interventional catheterization in the neonate and to evaluate outcomes. Methods. A study of 95 consecutive cardiac catheterizations in neonates was undertaken (3.2% of 2 879 paediatric procedures done from 1991 to 2006) at the Hospital Infantil de Mexico Federico Gomez. Results. Mean age was 17.5 ± 0.7 days, mean weight of 2.9 ± 0.98 kg, 59% were males. A therapeutic catheterization (Cat-T) was performed in 73 patients (77%), and a diagnostic procedure (Cat-D) in the remaining 22 (23%). The Cat-T procedures included: atrial septostomy in 54, aortic valvuloplasty in 8, pulmonary valvuloplasty in 5, aortic angioplasty in 3, transvenous pacemaker in 2 and stenting of ductus arteriosus in 1. In the 22 Cat-D procedures, the results correlated well with surgical findings, while a satisfactory palliation or correction was achieved in 94.6% of the 73 Cat-T. One or more complications occurred in 32/73 (40%) Cat-T, and in 6/22 (27.2%) Cat-D procedures. Considering the whole experience, 24 (25.2%) complications were qualified as major and 14 (14-7%) as minor. Of the former, arrhythmias and vascular lesions were the most common; however, only 5 patients (5.2%) required urgent surgical intervention. Death as a result of catheterization occurred in 2 cases (2.1%). An excellent correlation with surgical findings was obtained in the 22 Cat-D cases, whereas a satisfactory palliation was achieved in 69 of 73 Cat-T procedures (94-6%). Conclusions. These results confirm that cardiac catheterization in the neonate can be performed with good results and should not be withheld purely on the basis of age or critical clinical status.

Evaluación del flujo de las venas pulmonares con ecocardiografía transtorácica en niños con cardiomiopatía dilatada / Pulmonary venous flow in children with dilated cardiomyopathy using transthoracic echocardiography

OBJECTIVE: To evaluate the pulmonary venous flow in children with dilated cardiomyopathy and to establish its correlation with mitral Doppler flow and with the functional class. METHODS: It is a descriptive and transversal study in which we evaluated the pulmonary venous flow and its correlation with mitral Doppler flow and the functional class in 14 children with dilated cardiomyopathy. RESULTS: Nine patients were female. The mean age was 5.58 years and the mean follow up was 17 months. The functional class was I in 7 patients, II in 4 and III in 3. When diastolic function was evaluated using mitral Doppler flow it was abnormal in 9 of 14 patients, while the pulmonary venous flow was altered in all 14 patients since S/D ratio was abnormal in all. S wave was reduced in 12 patients; D wave was elevated in 8 patients, A reverse wave was elevated in 4 patients and in 8 the A reverse wave length was higher than mitral A wave length. CONCLUSIONS: Systolic function of the left ventricle is reduced in children with dilated cardiomyopathy. Diastolic function is also frequently abnormal in this group of patients. Pulmonary venous flow identifies better the abnormal diastolic function and correlates better with functional class than mitral Doppler flow.

[Pulmonary venous flow in children with dilated cardiomyopathy using transthoracic echocardiography]. / Evaluación del flujo de las venas pulmonares con ecocardiografía transtorácica en niños con cardiomiopatía dilatada.

OBJECTIVE: To evaluate the pulmonary venous flow in children with dilated cardiomyopathy and to establish its correlation with mitral Doppler flow and with the functional class. METHODS: It is a descriptive and transversal study in which we evaluated the pulmonary venous flow and its correlation with mitral Doppler flow and the functional class in 14 children with dilated cardiomyopathy. RESULTS: Nine patients were female. The mean age was 5.58 years and the mean follow up was 17 months. The functional class was I in 7 patients, II in 4 and III in 3. When diastolic function was evaluated using mitral Doppler flow it was abnormal in 9 of 14 patients, while the pulmonary venous flow was altered in all 14 patients since S/D ratio was abnormal in all. S wave was reduced in 12 patients; D wave was elevated in 8 patients, A reverse wave was elevated in 4 patients and in 8 the A reverse wave length was higher than mitral A wave length. CONCLUSIONS: Systolic function of the left ventricle is reduced in children with dilated cardiomyopathy. Diastolic function is also frequently abnormal in this group of patients. Pulmonary venous flow identifies better the abnormal diastolic function and correlates better with functional class than mitral Doppler flow.

Comparación de las medidas obtenidas por resonancia magnética con reconstrucción tridimensional y las obtenidas por angiografía digital para el diagnóstico de coartación de aorta / Comparison between the measurements obtained by magnetic resonance and digital angiography in patients with aortic coarctation

Introducción. La angiografía digital (AD) transcateterismo es el estándar de oro en el diagnóstico de la coartación de aorta (CoAo) aunque la resonancia magnética (RM) ha mostrado utilidad en la evaluación del sistema cardiovascular. La medición de los segmentos del arco aórtico facilita la selección de los candidatos para angioplastia transcateterismo evitando una cirugía torácica. No hay reportes de estudios comparativos de las medidas de los diferentes segmentos aórticos, obtenidas por RM y AD. Material y métodos. Se estudiaron 49 pacientes con RM y AD que ingresaron con CoAo entre enero de 2002 y diciembre de 2003. Se determinó el tipo de CoAo. Se midieron los segmentos aórticos por RM y AD y se compararon por medio de correlación lineal (Pearson). En 43 pacientes se realizó angioplastia con globo. Resultados. La variabilidad en la medición de los diferentes segmentos entre ambos métodos fueron: aorta ascendente 1.99-2.10 mm (desviación estándar (DE) 2.7-2.8), arco aórtico 1.79-2 mm (DE 2.55-2.99), istmo 1.53-1.56 mm (DE 2-2.17), aorta descendente 1.75-1.78 mm (DE 2.54-2.55). Las medidas comparadas por correlación lineal muestran r entre 0.80 y 0.999, excepto en arco aórtico por RM que obtuvo r de 0.57. Conclusión. La evaluación con RM de niños con CoAo puede sustituir a la realizada mediante AD ya que una variación de 1.5 a 2.1 mm en las medidas obtenidas por ambos métodos no interfiere con la decisión terapéutica. Sólo debe realizarse la medición del arco aórtico en 2 proyecciones.

Introduction. Quantitative aortic arch analysis may improve the selection of candidates for angioplasty who are most likely to benefit from the procedure. Digital angiography (DA) has traditionally been the definitive preoperative diagnostic procedure for aortic coarctation (AoCo), but magnetic resonance (MR) imaging affords good spatial resolution and excellent contrast between blood vessels and soft tissues and offers great potential for delineating thoracic cardiovascular structures. Material and methods. Forty nine patients with AoCo were examined with MR and DA between June 2002 and December 2003. The site and type of AoCo were determined and the measurements of aortic arch segments were obtained. We realized balloon angioplasty in 43 patients. Results. We compared the measurements using Pearson's linear correlation. The variability of the measurements was: ascending aorta 1.99-2.1 mm (standard deviation [SD] 2.7-2.8), aortic arch 1.79-2 mm (SD 2.55-2.99), aortic isthmus 1.53-1.56 mm (SD 2-2.17), and descending aorta 1.75-1.78 mm (SD 2.54-2.55). The lineal correlation of Pearson for measurements were; r =0.80-0.999 but the aortic arch was r =0.57 by MR. Conclusion. We conclude that quantitative MR measurements of aorta may be used to diagnose of AoCo and avoid using DA. To obtain precise measurements of aortic arch in patients with AoCo, MR should include 2 projections.

[Echocardiographic diagnosis of anomalous origin of one pulmonary artery from the ascending aorta]. / Diagnóstico ecocardiográfico del origen anómalo de una rama de la arteria pulmonar de la aorta ascendente.

OBJECTIVE: To present our experience in the echocardiographic diagnosis of anomalous origin of one pulmonary artery from the ascending aorta (AOPA). To analyze its clinical presentation, treatment and outcomes with special emphasis in the echocardiographic data. METHOD: We reviewed restrospectively the clinical, hemodynamic data, and surgical findings of patients with an echocardiographic diagnosis of AOPA studied in the Hospital Infantil of Mexico "Federico Gomez" from 1991 to 2002. RESULTS: The study includes 12 children with AOPA; Seven were males. The average age at diagnosis was two months; 4 in neonatal period, 3 under 1 year and 5 older than 1 year. The diagnosis was established prospectively by echocardiography in all patients and it was confirmed by angiography in 8 and at surgery in 9. Ten had anomalous origin of right pulmonary artery. The associated anomalies were patent ductus arteriosus in 6, ventricular septal defect in 2 and aortopulmonary window, atrioventricular discordance, double outlet right ventricle and tetralogy of Fallot in one case each. Nine underwent corrective surgery of all the anomalies. One patient died on the sixth postoperative day; the remaining are in good condition without stenosis at the site of the anastomosis. Surgery was refused in one. One patient was not candidate to surgery due to advanced obstructive pulmonary vascular disease (OPVD) and one case is awaiting surgery. CONCLUSION: The diagnosis of AOPA may be established with precision through echocardiography. Cardiac catheterization is seldom needed to confirm morphology but is mandatory in older children with suspected of OPVD.