[Primary stent implantation in aortic coarctation: mid-term follow-up]. / Implantación primaria de stent en la coartación de aorta: seguimiento a medio plazo.

BACKGROUND: The use of balloon-expandable stents provides an effective alternative therapy in patients with stenotic lesions in congenital heart disease. Stents implantation has served to improve the results and to reduce complications of balloon angioplasty for coarctation and recoarctation of the aorta. OBJECTIVE: We report our results after primary stents implantation for coarctation and recoarctation of the aorta. PATIENTS AND METHODS: Balloon-expandable stents were implanted in 14 patients (mean age 20 +/- 12 years) with coarctation of the aorta (11 native and 3 postoperative); 2 patients had associated malformations. The morphology varied: 10 resembled a located-diaphragm (one of them with moderate arch hypoplasia); 2 had distorted coarctation and 2 had a complete aortal obstruction. Five patients were hypertensive and 1 had cardiogenic shock and severe arrhythmias which did not respond to intensive medical therapy. In all cases 14 Palmaz stents (7 P308 and 7 P4014) were implanted with the primary technique through a Mullin's sheath. The balloon-to-descending aorta diameter ratio, measured at the level of the diaphragm, was 1. A special technique was carried out in the 2 cases with complete aortal obstruction. RESULTS: The procedure was effective in all 14 cases. The coarctation diameter increased from 4 +/- 2 to 15 +/- 2 mm (p < 0.0001) and transcoarctation systolic pressure gradient decreased from 43 +/- 19 to 2 +/- 2 mmHg (p < 0.0001). The ratio of the coarctation to descending aorta diameter measured at the level of the diaphragma increased from 0.3 +/- 0.1 to 0.95 +/- 0.05 (p < 0.001). At 19 +/- 8 months follow up, all patients showed sustained clinical improvement. The patient with complete aortal obstruction experienced a dramatic improvement, but she died from a sudden cardiac event 22 months after the procedure. At angiographic follow up in 7 patients, 1 year after implantation, no recoarctation was observed with secondary vessels patent, and absence of restenosis. CONCLUSIONS: a) Percutaneous endovascular stents implantation in coarctation and recoarctation of the aorta may become an effective treatment modality in the older child, adolescent and adults; b) stents are particularly attractive in those patients with a more complex anatomy and higher surgical risk; c) primary stenting is expected to have a lower rate of complications, and d) we describe a special technique with a right femoral-left humeral arterial circuit that is successfully applied to patients with complete aortal obstruction.

Reversible cardiomyopathy secondary to alpha-interferon in an infant.

Interferon-alpha (IFN-alpha) is a biological response modifier with antiviral and tumoral effect that is used in the treatment of chronic myelogenous leukemias. Adverse effects are well documented and cardiovascular disturbances mostly include hypotension and tachycardia and rarely cardiomyopathy. We report on an infant with chronic myelomonocytic leukemia (CML) diagnosed at 3 months of age who was treated with increasing IFN-alpha dosage (2.5-5.5 million U/m2/day) given subcutaneously for 7.5 months. At that age, he presented anorexia, general malaise, and nocturnal sweating for about a week, followed by respiratory distress and tachycardia. Diagnosis of congestive heart failure was suspected and documented by cardiomegaly and echographic changes of left ventricular dilated cardiomyopathy, with a 40% left ventricular ejection fraction (EF) and 20% fractional shortening (FS). He was treated with digoxin, furosemide, and angiotensin converting inhibitors, and IFN-alpha was discontinued. Progressive improvement of cardiac function was observed within 7 months of the events with normalization of the echocardiographic findings (EF 60%, FS 31%). We should emphasize the possibility of severe and reversible cardiac toxicity of IFN-alpha in infancy.

[Cor triatriatum with interventricular communication: Doppler color ultrasonography and magnetic resonance diagnosis. Repair in the first months]. / Cor triatrial con comunicación interventricular: diagnóstico por eco-Doppler color y resonancia magnética. Corrección en los primeros meses.

We report a case of left cor-triatrial with ventricular septal defect and patent ductus arteriosus in a lactant of six months of age. It was diagnosed using color-coded Doppler echocardiography and magnetic resonance imaging. The intracardiac defects were successfully corrected through right atriotomy.

[Unilateral agenesis of the pulmonary branch. Its diagnosis by color echo-Doppler and nuclear magnetic resonance]. / Agenesia unilateral de rama pulmonar. Diagnóstico con eco-Doppler color y resonancia nuclear magnética.

We present two symptomatic patients of five and six months old with unilateral agenesis of main pulmonary branch (right and left) without associated anomalies. The diagnostic was made with magnetic resonance imaging and two-dimensional echocardiographic with Doppler color flow. We think that the magnetic resonance, must be used with priority in this pathology for being a non invasive diagnostic method, that is able to diagnostic the basic cardiopathy and the intracardiac and extracardiac associated anomalies. His association with the Doppler-echocardiography allow to ignore the arteriography upon to get a accurate diagnostic.

Double-chambered right ventricle.

UNLABELLED: Between May 1974 and December 1993, 37 patients (0.75%) with a double-chambered right ventricle underwent surgical repair. The patients ranged in age from 11 months to 12 years (mean 4 +/- 1.1 years). Cardiac catheterization was performed in 36 patients. The proximal right chamber pressure was 118 +/- 10 mmHg and the mean ventricular gradient pressure was 75 +/- 10 mmHg. A ventricular septal defect was present in 36 cases and fixed subaortic stenosis in eight. Longitudinal right ventriculotomy, group I, was performed in 19 patients (51.3%): 11 had a perimembranous ventricular septal defect and eight an infundibular ventricular septal defect. Combined pulmonary arteriotomy and right atriotomy, group II, was performed in 18 patients (48.7%): 17 patients had a perimembranous ventricular septal defect. The ventricular septal defect was closed using a double velour patch in 26 patients, continuous suture in four and a Gore-Tex patch in six. In the ventriculotomy group one patient died shortly after the operation (following pulmonary complication), and ten patients required inotropic support. Two patients developed patch dehiscence and underwent reoperation. There were no complications in group II patients who underwent right atriotomy. Associated cardiac anomalies were corrected in all patients. Follow-up of 6.5 +/- 3.1 years after operation showed that 36 patients were alive and asymptomatic. CONCLUSION: the transatrial approach with pulmonary arteriotomy is an appropriate and effective double-chambered right ventricle correction even if it is associated with a perimembranous ventricular septal defect.

[Univentricular heart with left morphology with permeable atrio-ventricular valves. Study of the bulbo-ventricular foramen with color echo-Doppler. Importance for the surgical repair]. / Corazón univentricular de morfología izquierda con válvulas aurículo-ventriculares permeables. Estudio del foramen bulbo-ventricular con eco-Doppler color. Importancia para la corrección quirúrgica.

INTRODUCTION AND OBJECTIVES: Bidimensional and color-coded Doppler echocardiography were used to study 19 patients with univentricular heart, permeable atrioventricular valves, muscular bulbo-ventricular foramen and an anterior rudimentary chamber. We evaluated the influence of the foramen upon the size of the great vessels, and in consequence the most appropriated surgical techniques. MATERIAL AND METHODS: The foramen was measured in two projections at the end of the systolic period, and its area was calculated, according to the body surface, with the formula S = pi (D1/2 x D2/2). Indexes lower than 2 cm2/m2 were considered as restrictive. We studied 19 patients that presented a mean age of 11 +/- 12 years (range 1 day-39 years). The patients were classified in two groups. Group A: patients who had transposition of the great vessels (13 cases). Group B: patients who had normal connection of the great vessels (6 cases). Each group was classified in two subgroups: subgroup 1, patients who presented a restrictive foramen, and subgroup 2, patients who presented a normal foramen. In this study, we evaluated the associated cardiac anomalies. RESULTS: Group A. Thirteen cases. The index value for the whole group was 2.47 +/- 1.18 cm2/m2. The first subgroup included 6 cases with a restrictive foramen, 2 patients presented pathology of the arch. Four patients had a pressure gradient between the ventricle and the rudimentary chamber beyond 20 mmHg. The index of this subgroup was 1.68 +/- 0.39 cm2/m2, the median was 1.7. Seven cases, with a normal foramen, were included in the second subgroup, six of them had pulmonary valve stenosis, and the other one had undergone a pulmonary artery bandage 2 months before. Only one patient had a gradient pressure at the foramen beyond 20 mmHg. The index for this second subgroup was 3.34 +/- 0.83 cm2/m2. GROUP B: Six patients with normal connection of the great vessels were included. The index was 1.56 +/- 1.17 cm2/m2. The first subgroup included 3 cases with a restrictive foramen. The index was 0.72 +/- 0.29 cm2/m2 (range 0.41-1) and the median was 0.75. Pressure gradient between the ventricle and the rudimentary chamber was greater than 20 mmHg. Three patients with a normal foramen were in the second subgroup and here, the index was under normal limits 2.4 +/- 0.52 cm2/m2 (range 2.1-3), the median was 2.1. One patient underwent a pulmonary artery bandage two months before. CONCLUSIONS: Bulbo-ventricular foramen size/body surface index may exert an influence on the size of the vessel connected to the rudimentary chamber. The index vary in a negative way with the evolution time, or in those cases with transposition of the great vessels after a pulmonary artery bandage. Whenever the foramen is restrictive, the pulmonary artery or the aorta have a smaller diameter and the pressure gradient between the ventricle and the rudimentary chamber rises.

[Angioplasty of the stenotic Blalock-Taussig]. / Angioplastia del Blalock-Taussig estenótico.

BACKGROUND: Balloon angioplasty is a useful therapeutic method in some congenital heart diseases. We show our experience of stenotic Blalock-Taussig angioplasty, which was effective in six cases. PATIENTS AND METHODS: Six symptomatic patients with different congenital heart diseases, reduced pulmonary blood flow and a previous Blalock-Taussig shunt (it was classic in three patients and modified in another three) underwent the procedure. Conventional angioplasty was performed in four cases and coronary angioplasty technique, under monorail system, was applied in the other two. RESULTS: After angioplasty, we observed an increase in the minimum luminal diameter from 1 +/- 0.7 to 4 +/- 0.5 mm (p < 0.01), a reduction in the percentage of the stenosis from 77 +/- 15 to 13 +/- 13 (p < 0.01) and an increase in oxygen saturation from 63 +/- 10% to 82 +/- 5% (p < 0.05). The effectiveness of the technique in an extreme case of a thrombosed Blalock-Taussig, hours later the surgery, is reported. In the follow-up, we observed a symptomatic improvement, stable oxygenation levels, and a tendency in the hematocrit reduction. CONCLUSION: We consider that angioplasty of Blalock-Taussig shunt is indicated in those symptomatic patients, in whom a complete surgical correction is contraindicated. We demonstrate the effectiveness of the procedure, the coronary angioplasty, under monorail system is very useful for the stenotic modified Blalock-Taussig. It can be an effective technique when there is a postoperative thrombosis of the fistula.

[Pulmonary artery sling. A nuclear magnetic resonance study]. / Sling de la arteria pulmonar. Estudio con resonancia nuclear magnética.

We present a symptomatic newborn infant with anomalous pulmonary artery (pulmonary artery sling) studied by magnetic resonance imaging. Of the ones diagnosed by this method, is the first case published in our country and the eight of all that have been reported until this moment. The different methods used for the diagnostic, are discussed. We conclude that, the magnetic resonance imaging is at the moment, the elective diagnostic method.

[Propafenone efficacy in preventing supraventricular tachycardia in childhood]. / Eficacia de la propafenona en la prevención de la taquicardia supraventricular en la infancia.

OBJECTIVES: In order to study the efficiency of oral dose of propafenone in preventing childhood supraventricular tachycardias, we have treated 38 children without left ventricular dysfunction. METHODS: The mean age has been 8 +/- 4.7 years old. All of them had suffered at least two episodes of supraventricular tachycardia, and eleven had undergone a previous antiarrhythmic treatment. The initial dose of propafenone has been 6.5 +/- 2.1 mg/kg/24 h (in 3 doses), which has been increased in case of inefficacy. RESULTS: We have not advised neither side-effects, nor proarrhythmic effects, during a follow-up of 13 +/- 6 months. Propafenone has been efficient in preventing supraventricular tachycardias in all patients. The initial dose has been efficient in 17 patients, and we have increased the initial dose up to 11.1 +/- 3.7 mg/kg/24 h in 21 patients. The mean effective dose has been 8.9 +/- 3.6 mg/kg/24 h. We stopped treatment in 14 patients after been 6 months without supraventricular tachycardias. CONCLUSION: From our study, we conclude that propafenone has been efficient in preventing supraventricular tachycardias in childhood with a dose of 8.9 mg/kg/24 h. We have not found side-effects.

[An imaging and color echo-Doppler study of interventricular defects]. / Estudio de los defectos interventriculares por imagen y ECO-Doppler color.

OBJECTIVE: Evaluation of ventricular septal defect with two-dimensional echocardiography and color flow Doppler. PATIENTS AND METHODS: We had studied by this method 180 patients; 97 males and 83 (mean age 1.5 +/- 1 years) diagnosed of a VSD. Cases with a complex cardiopathy were excluded from the study. The VSD was classified according to its location and relation to the tricuspid anulus and semilunar valves in perimembranous VSD, muscular VSD and subarterial double committed defect. The colour Doppler identified one or two areas of low through the ventricular septum with a zona of proximal acceleration throughout systole. Every group can be evaluated in different views but they all have a selective echocardiographic view except for muscular trabecular VSD. RESULTS: Seventy-six patients had perimembranous VSD (30 inlet, 26 trabecular and 20 infundibular-outlet), 102 had muscular VSD (3 inlet, 97 trabecular and 2 infundibular-outlet) and 2 had a subarterial double committed defect. A ventricular septal aneurysm was associated in 20 patients with muscular or perimembranous defects. Six patients, 2 of the perimembranous trabecular VSD, 2 muscular-trabecular VSD and 2 subarterial were initially misclassified. In this last 2 cases the turbulence created in the pulmonary valve was wrongly interpreted as pulmonary valvular stenosis; subcostal view for both tracts is necessary to avoid such mistake. In conclusion, color Doppler flow mapping is very useful to differentiate the various types as VSD and aids identification of multiples ventricular septal defects.

[Left coronary artery-right ventricle fistula secondary to heart surgery. Non-invasive diagnosis with Doppler-color bidimensional echocardiography]. / Fístula de arteria coronaria izquierda-ventrículo derecho secundaria a intervención quirúrgica cardíaca. Diagnóstico no invasivo con ecocardiografía bidimensional-Doppler color.

A case of 20 year-old man with a left coronary artery fistula to right ventricle secondary to open heart surgery is reported; he was asymptomatic and a continuous precordial murmur was found on physical examination. Two dimensional echocardiography-Doppler color flow imaging visualized a dilated left coronary artery with flow into the right ventricle. The discovery was confirmed by coronary angiography. We conclude that two dimensional echocardiography Doppler color flow imaging may be a method included in evaluation of pediatric patients with coronary artery fistula suspicion.

[Aneurysm of the atrial septum associated with interatrial communication: a study of 12 cases by 2-dimensional color echocardiography]. / Aneurisma del septo auricular asociado a comunicación interauricular. Estudio de 12 casos con ecocardiografía bidimensional-color.

Between 1983-1991 we have studied 5,221 two-dimensional echocardiography in patients suffering cardiopathies. Twelve (0.22%) had an aneurysm of the atrial septum associated with atrial septal defect. Eight patients had other cardiac anomalies. The criteria used to diagnosis the aneurysm were: 1) The atrial septum central part protrudes in the atrium. 2) 6 mm aneurysm base. 3) Minimum distance from the base to the protrusion top 6 mm. The aneurysm was located in the oval fossa in 6 patients (50%) and in the other 6 patients the aneurysm was situated in the ostium secundum. The aneurysm base measured 7.79 +/- 1 mm and the left or right septum displacement was of 7.37 +/- 1.82 mm. The left displacement was observed in 10 patients (83.3%) and the right in 2 patients (16.6%). The spontaneous closure of atrial septal defect by the aneurysm was showed in 2 (16.6%) patients and in one (8%) was necessary the surgical closure. The other 9 patients: five rested along the follow-up without any change and other 4 patients died because of cardiac malformation. To conclude the atrial septal aneurysm associated with atrial septal defect in an unusual cardiopathy. Two dimensional echocardiography and Doppler color was diagnostic in all infants.

[The usefulness of continuous Doppler in the diagnosis of aortic coarctation]. / Utilidad del Doppler continuo en el diagnóstico de la coartación de aorta.

Were studied 32 patients with coarctation of the aorta by continuous wave Doppler ultrasound in order to assess the usefulness of this diagnostic method. Nineteen (59%) had native coarctation and 13 (41%) recoarctation. Seventeen (53%) were male and 15 (47%) female. The mean age when diagnosis was performed was 56 +/- 54 months. We found associated anomalies in 15 patients (46%). We studied 5 variables of the continuous Doppler curve at the descending aorta that were compared with those of a control group of 20 patients without cardiac anomalies. The variables were: peak systolic gradient (50.1 +/- 18 mmHg; VS 7.6 +/- 3 mmHg; p = 0.001); peak diastolic gradient 18.5 +/- 10 mmHg; VS 0.49 +/- 0.5 mmHg; p = 0.001; systolic velocity half time 151.5 +/- 40 msec; VS 102 +/- 17 msec; p = 0.01; diastolic velocity half time 132.2 +/- 82 msec; VS 7 +/- 14 msec; p = 0.001 corrected acceleration time (169 +/- 26 mmHg; VS 138 +/- 22 mmHg; p = 0.01). Systolic gradient was highly sensitive (100%) and specific (100%). Diastolic gradient and time to half peak diastolic velocity were very specific (100%) and less sensitive (93% and 62%). There were 6 false positive and 4 false negative when we used the time to half peak systolic velocity (sensitivity 81%, specificity 72%). We conclude that continuous Doppler is a useful method in the diagnosis of coarctation of the aorta. The most important variables of Doppler curve are systolic gradient, diastolic gradient and time to half peak diastolic velocity. All patients with coarctation of aorta had at least 2 variables altered.

[Biventricular rhabdomyoma or a case of spontaneous regression. A two-dimensional echocardiographic study]. / Rabdomioma biventricular o un caso de regresión espontánea. Estudio con ecocardiografía bidimensional.

We present the case of an infant diagnosed by prenatal echocardiography as having a biventricular rhabdomyoma which was confirmed after birth. In the series of two-dimensional echocardiographies this tumor was seen to decrease in size until its complete disappearance.

[Application of bidimensional echocardiography in the diagnosis of ventricular septal defect]. / Aplicación de la ecocardiografía bidimensional en el diagnóstico de la comunicación interventricular.

Between 1985-1990 we studied by means of two-dimensional echocardiography 286 patients with isolated ventricular septal defect (VSD). A hundred and sixty three being male and 123 female, as a mean age of 5 +/- 3 months. Sixty two cases (22.3%) reveales associated anomalies and in other 63 (22.4%) there was in the evolution an aneurysm of the septum membranous. The projections used were apical 4-chamber, with or without aortic root; parasternal long-short axis of great arterias or at ventricular level; subcostal 4-chamber; right oblique outlet right outflow tract and left oblique outlet left and right outflow tracts. Patients with VSD smaller than 3 mm were excluded. Perimembranous defects, 189 cases (66%), were more frequent than muscular, 91 31.8%), and subarterial defects, 6 (2.1%). Only 19 (9 apical muscular, 6 perimembranous outlet and 4 perimembranous trabecular) were initially misclassified. The diameter of the VSD was large in subarterial (0.85 +/- 0.1 cm) and perimembranous outlet VSD (0.75 +/- 0.5 cm) compared with the rest. Each group of defects was more easily shown by one particular projection except trabecular muscular defect. In conclusion, we are able to state that two-dimensional echocardiography enables us to discover the size and situation of isolated ventricular defects.

[Atrioventricular and ventriculoarterial discordance (corrected transposition of the great vessels). Diagnosis with bidimensional echocardiography]. / Discordancia atrioventricular y ventriculoarterial (transposición corregida de los grandes vasos). Diagnóstico con ecocardiografía bidimensional.

We present 15 patients with atrioventricular and ventriculoarterial discordance diagnosed with two-dimensional echocardiography from January 1973 to June 1991. The 15 had situs solitus and six dextrocardia. A different level of insertion of the atrioventricular valves worth from 0.77 +/- 0.3 cm, was observed in all the cases which didn't perimembranous inlet ventricular septal defect. Ten patients, presented septal insertion of one of the chords of the left atrioventricular valve, while, this insertion wasn't observed in the right atrioventricular valve. The ventricular morphology and trabeculation, permitted the identification of both ventricles in all the cases. The subxifoidea view resulted in being fundamental, for the examination of the ventriculoarterial connections. Ten cases (66.6%) had an obstruction in the pulmonary outflow tract (6 in the valve and 4 in subvalvular area) and nine (60%) ventricular septal defect (7 perimembranous and 2 muscular). We believe that the two-dimensional echocardiography gives enough information to arrive at the correct diagnosis of this cardiac malformation and of the associated anomalies. This information can be completed with the use of the color-flow Doppler.

[Aortic atresia with situs inversus]. / Atresia aórtica con situs inversus.

We report a case of mitro-aortic atresia with situs inversus. The patient was studied with two-dimensional echocardiography and the diagnosis was confirmed in the necropsy. Our newborn infant is the second case reported with this abnormality and the first in our country.

[Diverticulum of the right ventricle with dilated myocardiopathy]. / Divertículo de ventrículo derecho con miocardiopatía dilatada.

We report on a newborn infant with right ventricular diverticulum associated to dilated myocardiopathy, who was diagnosed by two-dimensional echocardiography within 24 hours after vaginal delivery. We believe this case represents the youngest one ever diagnosed with this disease.

[Angioplasty of a stenosed Blalock-Taussig shunt. Its long-term assessment]. / Angioplastia del Blalock-Taussig estenótico. Valoración a largo plazo.

A Blalock-Taussig shunt was performed in a child of 14 months diagnosed with tetralogy of Fallot associated with a complete atrioventricular defect. Seven months later, a balloon dilatation was performed because the shunt was stenosed in the site of the distal anastomosis. Repeat angiography we proved the patency of the shunt without restenosis 4 year later.

[The double-chambered right ventricle: the surgical results in 28 cases]. / Ventrículo derecho bicameral: resultados quirúrgicos de 28 casos.

Between May 1974 and June 1990, 28 double chambered right ventricle underwent surgical repair. Twenty-one (75%) had high obstructive anomalous muscle bundles and in seven (25%) the anomalous muscle bundles were in low position. A ventricular septal defect (VSD) was present in all cases and a subaortic stenosis in six. Longitudinal right ventriculotomy was performed in 15 (53.57%) patients and a combined pulmonary arteriotomy and right atriotomy was used in 13 (46.43%). In the group treated by ventriculotomy 2 patients died shortly after operation (one as a consequence of disseminated infection and one after a lung lobectomy); another patient developed patch dehiscence and reoperation was performed via right atrium a year after. In other 3 patients the Doppler revealed a residual gradient of 20 to 30 mmHg. There were no complications in the group of patients that underwent right atriotomy. In addition to resection of the anomalous muscle bundles, the associated cardiac anomalies were corrected in all patients. Follow-up of 5.5 +/- 3.8 years after operation showed that 26 (92.86%) patients were alive and asymptomatic. We conclude that double chambered right ventricle can be early repaired with good results using a combined procedure with right atriotomy and pulmonary arteriotomy.