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Objetivo: Analizar la utilidad de la resonancia magnética para el diagnóstico morfológico en las cardiopatías congénitas complejas comparada con el ecocardiograma transtorácico. Método: Se incluyeron 45 pacientes con sospecha clínica de cardiopatía congénita grave, se les realizó una resonancia magnética y ecocardiograma transtorácico y se comparó anatomía y tamaño de cavidades cardíacas. Para el análisis se empleó estadística descriptiva y análisis de Bland-AItman para la concordancia entre ambos métodos. Resultados: No hubo diferencias significativas en mediciones obtenidas por ambos métodos, excepto en la fracción de expulsión del ventrículo izquierdo (66.56 ± 9.47 por ECO vs 52.32 ± 13.85 por RM, p = 0.0001) y la medición del diámetro de la rama izquierda de la arteria pulmonar (9.77 ± 6.80 por ECO vs 13.83 ± 8.46 por RM, p = 0.05). La sensibilidad, especificidad y los valores predictivos de la RM fueron elevados para analizar la conexión AV y VA, no así para el situs atrial y los drenajes venosos (pulmonar y sistémico) donde el ECO tiene baja resolución. La concordancia de las mediciones de la FE, índice de McGoon y tamaño del VD mostró pocos valores extremos. Conclusiones: La RM y el ECO son similares para el diagnóstico morfológico de las cardiopatías congénitas graves, pero la RM puede superar al ECO en la visualización de estructuras extracardíacas.

Objective: To analyze the usefulness of magnetic resonance for the morphological diagnosis in complex congenital cardiopathies and compare it with the transthoracic echocardiogram. Method: We included 45 patients with clinical suspicion of severe congenital cardiopathy A magnetic resonance and a transthoracic echocardiogram were performed and the anatomy and size of the cardiac cavities were compared. Descriptive statistics were used and Bland-Altman test was used to analyze concordance between both methods. Results: There were no significant differences in the measures obtained with either method, except for the left ventricle ejection fraction (66.56 ± 9.47 with ECO vs 52.32 ± 13.85 with MR, p = 0.0001) and for diameter of the left branch of the pulmonary artery (9.77 ± 6.80 with ECO vs 12.83 ± 8.46 with MR, p = 0.05). The sensitivity, specificity, and predictive values of MR were high to analyze the AV and VA connection, but not for the atrial sinus and the venous drainages (pulmonary and systemic). Concordance of ejection fraction measures, McGoon index, and size of VD revealed few extreme values. Conclusions: MR and ECO are similar for the morphological diagnosis of severe congenital cardiopathies, but MR can be better than ECO for the visualization of extracardiac structures.

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OBJECTIVE: To analyze the usefulness of magnetic resonance for the morphological diagnosis in complex congenital cardiopathies and compare it with the transthoracic echocardiogram. METHOD: We included 45 patients with clinical suspicion of severe congenital cardiopathy. A magnetic resonance and a transthoracic echocardiogram were performed and the anatomy and size of the cardiac cavities were compared. Descriptive statistics were used and Bland-Altman test was used to analyze concordance between both methods. RESULTS: There were no significant differences in the measures obtained with either method, except for the left ventricle ejection fraction (66.56 +/- 9.47 with ECO vs 52.32 +/- 13.85 with MR, p = 0.0001) and for diameter of the left branch of the pulmonary artery (9.77 +/- 6.80 with ECO vs 12.83 +/- 8.46 with MR, p = 0.05). The sensitivity, specificity, and predictive values of MR were high to analyze the AV and VA connection, but not for the atrial sinus and the venous drainages (pulmonary and systemic). Concordance of ejection fraction measures, McGoon index, and size of VD revealed few extreme values. CONCLUSIONS: MR and ECO are similar for the morphological diagnosis of severe congenital cardiopathies, but MR can be better than ECO for the visualization of extracardiac structures.

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OBJECTIVE: To compare results of dilatation of native coarctation of the aorta with and without stent implantation. DESIGN: Open, observational, non-randomised study. PATIENTS: 54 consecutive adult patients: 32 with balloon angioplasty alone (group 1) and 22 with stent placement (group 2). INTERVENTIONS: Balloon dilatation from 1995 to 1997; dilatation with Palmaz stent placement from 1997 to 1999. MAIN OUTCOME MEASURES: The primary end point was a composite index of failure including heart related death, a residual gradient of > 20 mm Hg, the need of reintervention, and aneurysm formation. RESULTS: Peak systolic gradient (mean (SD)) was reduced both in group 1 (from 63.3 (22.8) to 10.7 (10.8) mm Hg, p < 0.001) and group 2 (from 63.9 (20.8) to 2.7 (4.3) mm Hg, p < 0.001), but Delta change was significantly greater in group 2. A residual gradient of > 10 mm Hg was shown to be the best cut off point to separate risk groups, representing a hazard ratio (HR) of 9.59 compared with a residual gradient of < or = 10 mm Hg (95% confidence interval (CI) 1.92 to 47.8). From multivariate Cox regression analysis, the only risk marker was the residual gradient (HR 8.9, 95% CI 1.2 to 63.0). The type of the coarctation and the use of stent were the factors associated with a residual gradient of < or = 10 mm Hg. CONCLUSIONS: Mid term outcome in adult patients with native aortic coarctation receiving percutaneous treatment is strongly related to the immediate residual gradient. When treating these cases, efforts should be made to obtain gradients under 10 mm Hg, either by angioplasty alone or by placing a stent. Patients with discrete aortic coarctation have similar mid term results when the immediate residual gradient is < or = 10 mm Hg despite the implantation of a stent. To achieve these gradients, patients with hypoplastic isthmus or tubular coarctation should be treated with primary stenting. Further studies including exercise tests and non-invasive imaging are still needed before definitive conclusions can be drawn.

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Diagnosis in congenital heart disease in the early days was based on the electrocardiogram, fluoroscopy, and RX images, their accuracy was improved by catheterization and angiography. The next step was surgery, with the closure of the ductus arteriosus and the Blalock-Taussig shunt that changed the natural history of congenital heart defects with diminished pulmonary flow. Echocardiography enabled noninvasive, painless studies, and nowadays it is our main tool for diagnostic purposes including the fetal period with corrective surgical possibilities at an earlier age. Finally the most recent advances have been performed in the area of cardiac development and the knowledge of the etiology of genetic syndromes associated with heart disease, through molecular biology. As a result, many children with such diseases now survive to adulthood creating the need of new medical facilities for their specialized care.

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Important advances in the diagnosis and treatment of congenital heart disease (CHD) have been made in the past 50 years. Nowadays echocardiogram plays an important role in the diagnosis. This procedure is able to identify a wide range of malformations. Cardiac catheterization is mainly a therapeutic tool, surgery is now performed much earlier because CHDS are diagnosed sometimes before birth or very early in life. All this advances in the diagnosis and treatment of this group of patients, allows them not only a better quality of life but also the possibility of reaching adulthood and having children. The study of the etiology of CHD is a field that has not evolved as fast as the assessment and treatment. Nowadays we have a larger population of adults with CHD. The discovery of a microdeletion of chromosome 22 q11.2 associated with conotruncal cardiac defects, proves a common etiology for clinical phenotypes and conotruncal malformations. In order to identify, which of these patients share the same etiology and presented with this syndrome, we collected a group that shared not only heart defects of the conotruncal type but also specific phenotypic alterations such as broad nasal bridge, nasal dimple, high palate, and digitalization of the first finger among others. The first two patients studied with FISH technique were positive to monosomy of a locus on chromosome 22. Those patients with CHD of conotruncal type should undergo microdeletion testing so genetic counseling can be offered as well as appropriate treatment in areas such as cardiology and developmental psychology.

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OBJECTIVE: To know the frequency and type of nutritional alterations of children with congenital heart disease. MATERIAL AND METHODS: Sixty six children with congenital heart disease were studied. MEASUREMENTS were: weight, size, mid arm circumference, tricipital and subscapular skinfold thickness. Blood samples for white blood cells count, and albumin were taken and urine was collected for determination of creatinine. Muscular and fat areas of the arm and creatinine/height index were calculated. In order to compare the children with both cyanotic and non-cyanotic types of heart diseases chi 2 test or Fisher's exact test, ware used. RESULTS: 41 girls and 25 boys, 42 with acyanotic and 24 cyanotic heart disease. 50 children were malnourished (26 compensated, 23 non-compensated and one acute); 16 were normal. 85% of the children presented diminished muscular area and 97% diminished fatty area. The creatinine/height index was diminished in 94% of the cases. No significant differences were demonstrated between both groups. CONCLUSION: chronic malnutrition in children with congenital heart disease is frequent. Most of the cases with malnutrition are compensated. This poor nutritional status is at the expense of fatty and muscular tissues.

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We report the results of radiofrequency catheter ablation in 203 patients under 18 years of age with supraventricular tachyarrhythmias between April 1992 and June 1997. The presence of an accessory pathway caused the tachyarrhythmia in 181 patients (89.1%) with a total of 187 accessory pathways; atrioventricular nodal reentry caused the arrhythmia in 18 patients (8.8%) and atrial flutter in only 4 patients (1.9%). We eliminated the accessory pathway in 171 patients (91.4%), 23 patients showed recurrence of the tachycardia and we had complications in 4 patients (2.1%). The procedure was successful in the treatment of the atrioventricular nodal reentry in the 18 cases, with ablation of the slow pathway in 17 cases and in only one patient of the fast pathway, one patient showed total A-V block, and recurrence of the arrhythmia in 3 cases (16.6%). Finally the procedure was successful in the 4 cases of atrial flutter with one recurrence (25%). In the total of the series, the radiofrequency catheter ablation was successful in 193 patients (95%), with recurrence of the arrhythmia in 27 cases (13.3%) and with complications in only 5 patients (2.6%). Radiofrequency catheter ablation is a safe and effective procedure for the definitive treatment of supraventricular tachyarrhythmias in children.

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We present the clinical and hemodynamic profile of 33 patients, older than the 18 year with tetralogy of Fallot. Cardiac catheterization and selective angiocardiography were performed in all cases. We excluded patients with valvular pulmonary atresia associated with tetralogy of Fallot. Most of the patients were in functional class II or IV of the New York Heart Association and all presented with cyanosis. In the electrocardiogram of 23 patients we found right bundle branch block. None had significant cardiomegaly, 19 of 20 cases had reduced pulmonary blood flow and reticular pattern in the lung fields as observed in the chest X-ray. We did not find correlation between the age and the degree of cyanosis, but the level of arterial desaturation correlated with the functional class. The right ventricular systolic pressure was elevated in all patients. In all cases but one the pulmonary arterial systolic pressure were normal of slightly increased. One case with mild pulmonary infundibular stenosis had pulmonary systolic pressure similar to the systemic pressure. Multivariate analysis for functional class showed significant value for cyanosis. Systemic-pulmonary shunt was performed in 10 patients with hypoplastic pulmonary arteries plus reduced pulmonary blood flow, with one postoperative death. We did not find postoperative mortality in patients who underwent corrective surgery. The anatomic and functional behavior of patients who underwent surgery, allowed better tolerance to their heart defects.

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OBJECTIVE: To present our experience in 58 children (37 female, 21 male) aged 4 to 18 years (mean +/- SD = 14.9 +/- 2.7) with rheumatic mitral valve disease who underwent surgical reconstruction. METHODS: They were divided in four groups according to the abnormalities of the mitral apparatus: Group I had mitral regurgitation due to lesions located in the valvar structures with normal valvar movement (n = 1, 2%), group II mitral regurgitation with lesions located mainly in subvalvular structures with valve prolapse (n = 11, 19%), group III with mitral regurgitation due to lesion located both in valvular and subvalvular structures and restricted valvar motion (n = 38, 65%), and group IV included patients with stenosis (n = 8, 14%). RESULTS: Surgical mortality was 5% (3/58) and three patients (5%) were reoperated immediately. The 52 remaining patients were followed from 6 to 108 months (mean 45.8 +/- 30.1 months, 199 patient-years). Six cases were reoperated in the follow-up (3.0% per patient-year). The functional class using the criteria of the New York Heart Association was evaluated before and after the procedure: class I raised from two to 42 patients whereas it decreased in the others (class II from 13 to 7, class III from 38 to 3, class IV from 5 to none; p < 0.001). The cardiothoracic index decreased from 0.61 +/- 0.064 before surgery to 0.55 +/- 0.069 after surgery (p < 0.001). Thirty patients (57%) were evaluated with echo-Doppler in the follow-up period. There were no deaths in the follow-up period. CONCLUSIONS: Our data shows that reconstructive surgery of the mitral valve with rheumatic involvement offers good immediate and late results, with a low reoperation rate, avoiding the use of prosthetic valves as a first option.

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Percutaneous closure of the patent ductus arteriosus (PDA) has been recently reported to be an effective alternative in the treatment of patients with ductal shunting. We report the initial experience and results during follow-up of percutaneous ductal occlusion with the Rashkind occluder (USCI) in six patients with isolated PDA. Ages ranged from 3 to 23 years. Diagnosis was corroborated with two dimensional and Doppler echocardiography in all patients. During cardiac catheterization systolic pulmonary artery pressure oscillated between 22 and 64 mmHg and Qp/Qs ratio between 1.3 and 4.1. In two patients prosthesis of 12 mm were used and in the remaining prosthesis of 17 mm. Only one patient demonstrated total occlusion during immediate control aortography, the other patients presented central residual shunting over the occluder. In the three patients occlusion with balloon-catheter was added to the procedure, resulting in total occlusion in two and significant reduction of the shunt magnitude in one. Two technical problems were resolved satisfactorily. None of the cases presented device embolization. Mean follow-up was 23.8 months with control echocardiograms at 24 hours, 1, 4, 12 and 24 months. In all patients immediate reduction of the left atrial dimension was demonstrated. Three patients presented residual shunts in the first 24 hours. In two of them total occlusion had occurred after one month and the other patient persisted with a small residual shunt until one year after the procedure. In conclusion, in this small study group good results were obtained with percutaneous ductal closure.

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The percutaneous balloon valvulotomy is nowadays the treatment of choice for isolated pulmonary stenosis. Forty two patients with congenital pulmonary stenosis underwent balloon valvulotomy at the Instituto Nacional de Cardiología "Ignacio Chávez". Ages 1 month to 24 years, mean 7.6 +/- 5.9 years, with a follow-up of 28.3 +/- 14.8 months. With an infundibular gradient of 50 mm Hg or more a treatment with propranolol was given. After valvulotomy the total basal pressure gradient decrease from 82.9 +/- 40.6 mm Hg to 31.2 +/- 27.1 mm Hg (p < 0.00001). The patient's results were divided in two groups: with and without reactive infundibular obstruction. The 31 patients without infundibular obstruction had a total initial gradient of 71.6 +/- 33.5 mm Hg, and after valvulotomy the gradient was reduced to 18.9 +/- 11.2 mm Hg (p < 0.00001). The 11 patients with infundibular obstruction had a total initial gradient of 114.8 +/- 43.2 mm Hg, and after valvulotomy the valvular gradient was 12.4 +/- 8.5, the infundibular gradient 53.4 +/- 22.9 and the total gradient 65.9 +/- 29.1 mm Hg (p = 0.002 in relation to the basal gradient). All patients with infundibular obstruction (8 treated with Propranolol) and a follow-up of 8.5 +/- 9.8 months underwent Doppler examination, showing a progressive reduction of the total gradient. By six months no one had a total gradient greater than 50 mm Hg. These data confirm that balloon valvulotomy in valvular pulmonary stenosis is safe and effective, and that restenosis is very low. Good results relay on the use of proper balloon diameters (balloon diameter/pulmonary annulus relation of 1.2 to 1.5).(ABSTRACT TRUNCATED AT 250 WORDS)

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Three brothers 9, 12, and 14 years of age were studied. The three of them fulfilled the diagnostic criteria of Marfan Syndrome that was made based on physical and ophthalmologic examination, these criteria included musculoskeletal abnormalities, subluxation of lens, myopia and aortic aneurysm. The three siblings died due to complications of the aortic aneurysm, which had an early onset as well as the other clinical manifestations of the disease. They were 2 brothers and one sister. There was neither mental or psychomotor retardation, nor metabolic disease in any of the patients. Family history did not show any clinical manifestation of the disease in parents or grandparents. We propose that the hereditary pattern is recessive autosomic instead of dominant autosomic given the features of these cases and their history.

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Sixty-nine patients with Corrected Transposition of the Great Arteries were studied, with a mean age of 8.3 years. Forty-nine had atrial situs solitus (SS) and 20 atrial situs inversus (SI). The diagnosis was proved by echocardiography in 28 cases and in all by angiocardiography. In cases with SS, 29 presented ventricular septal defect (VSD), 20 had subvalvular pulmonary stenosis (SVPS), 13 tricuspid regurgitation (TR), 13 atrio-ventricular block (AVB), and seven without associated defects. In cases with SI, 13 had VSD, 14 SVPS, five IT, three AVB, and two without associated defects. The long-term follow-up of the atrioventricular (AV) conduction in 53 cases, ranging between 2 and 37 years (mean 8.3) showed at the beginning of the evaluation 37 cases with normal AV conduction, 14 with first degree AVB, and two with complete AVB. At the end of the follow-up 24 cases had normal AV conduction, 13 first degree, two second degree, four intermittent and 10 complete AVB. The contingency table showed that the possibility of developing changes of the AV conduction was greater in those cases who had an abnormality at the beginning with a relative risk of 2.27 (p = 0.03). Twenty-three cases had SVPS associated with VSD without significant symptoms. During a follow-up ranging from 1 to 20 years (mean 6.5 +/- 5.9), five of these patients were surgically corrected at ages from 6 to 11 years. The remaining cases have had a stable course. At the beginning of the study, 18 cases had TR, and it developed in five more patients during the same follow-up period.(ABSTRACT TRUNCATED AT 250 WORDS)

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We describe a 9-year old patient with arrhythmogenic right ventricular dysplasia. The patient presented with sustained ventricular tachycardia in an apparently normal heart. The diagnosis was suspected by the electrocardiographic morphology of the tachycardia and definitive diagnosis was made by right ventricular angiogram, echocardiogram and the ventricular endomyocardial biopsy findings (replacement of myocardial by adipose tissue and fibrosis). We believe that this condition may be a common etiology of ventricular tachycardia with left bundle branch block conduction pattern in children with otherwise normal hearts.

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UNLABELLED: Most of the published papers on Rheumatic Fever (RF) have not included the younger population. We selected 211 cases of children with RF younger than 6 years of age from 9,471 clinical files from 1944 to 1982. These were followed retrospectively to identify the presence of rheumatic activity, subsequent attacks and penicillin profilaxis. From de 211 cases, 209 had carditis; 57% of them were girls and 43% boys. There were no previous infections of the upper respiratory tract in 36% of the patients. The number of cases with RF increased abruptly after 3 years of age and continued increasing until 5 years of age when 70.5% of the population had there first clinically recognized attack. Lesions were present in the mitral valve in 80% of the cases, in the aortic valve in 12%, in the tricuspid in 5% and in the pulmonary valve in 3%. The death rate during the first attack was 20% being refractory heart failure the main cause of death. Thirteen cases suffered rheumatic pneumonia, 9 of whom died (69.2%). CONCLUSIONS: 1) The incidence of acute rheumatic fever in children under 6 years of age has decreased with time. 2) The death rate as well as the valvular damage decreased with the parents cooperation with the treatment. 3) The changes in the clinical picture and the severity of valve sequelea may be due to penicillin profilaxis and the better understanding of the disease.

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Two-dimensional echocardiography has proved its utility in the diagnosis of cor triatriatum sinister by visualizing the defect and its relation to the other structures. Recently pulsed and continuous wave Doppler have been used to determine the degree of obstruction of the membrane. In this study we present three patients with ages six, eight and fourteen months respectively, all with a clinical history of growth retardation, respiratory infections and dyspnea. The patients were studied with two-dimensional and color flow Doppler echocardiography. In all, a membrane was visualized, which divided the left atrium in two chambers, a superior one which received the pulmonary veins and an inferior in continuity with the mitral valve and the left atrial appendage and with color flow Doppler the absence of obstruction at this level was demonstrated. In one patient besides the anomaly of cor triatriatum complete transposition of great arteries with pulmonary stenosis was diagnosed and in another one, patent ductus arteriosus and ventricular septal defect. Diagnosis were confirmed by hemodynamic studies. It is concluded that the color flow Doppler is a diagnostic method of great utility because it permits to obtain more information which complements conventional echocardiography.

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Due to the frequent association of congenital heart disease and urinary tract malformations we studied 434 patients undergoing angiocardiograms for diagnosis of their congenital heart disease. In every patient a plain abdominal X-ray film was made in order to observe urinary elimination of the contrast material. We found 70 patients with urinary tract malformations, the most frequent of which was pyelo-chaliceal dilatation, accounting for 47.1% of all the malformations found. A double pyelo-chaliceal system was found in 25.7%, kidney hypoplasia in 10% and in lower frequencies double urethers, kidney agenesia, pelvic kidneys, horseshoe-shaped kidney and others. Patients were usually urologically asymptomatic. In view of the frequent association of congenital heart disease and urinary tract malformations a plain abdominal X-ray film should always be performed postangiographically. In some cases a contrast urography is recommended.

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Two cases of double outlet of the right ventricle with an anterior aorta to the left of the pulmonary artery are described. Both in situs solitus. One had an anterior interventricular communication, and other had a middle posterior communication. The importance of the aortic component of the second sound is analyzed with regard to its localization and intensity. An analysis is also made of the morphology of the cardiac silhouette and the peculiarities of the electrocardiogram as diagnostic aides in the malformation. Considerations are made of the distinct theories which explain the malformation. It is clearly shown that a correct diagnosis is necessary since this cardiopathy may be treated surgically.

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