[Intravascular hemolysis following percutaneous occlusion of the ductus arteriosus]. / Hemólisis intravascular tras el cierre percutáneo del conducto arterioso.

Transcatheter occlusion of patent ductus arteriosus has become a safe and successful technique, but it's not free of complications. We present the case of a two-year-old boy who underwent routine transcatheter closure of his patent ductus arteriosus, using a "coil" device. Twenty hours later he developed severe persistent hemolysis in association with residual ductal flow. Patient's clinical situation became stable when the device was removed. Pulmonary embolization of the device and hemolysis are the main complications of percutaneous closure of the patent ductus arteriosus. Hemolysis occurs rarely (0.5%) and is always associated with the presence of residual ductal flow. Several approaches to this problem have been described. Mild cases may require no intervention; however, when severe hemolysis is present, removal of the device may be needed, proceeding with surgical repair of the patent ductus arteriosus.

[The role of the stent in non-coronary cardiopathies]. / Papel del stent en las cardiopatías no coronarias.

INTRODUCTION: The stent has demonstrated to be a useful device in the prevention of postangioplasty coronary restenosis and it is expected to have a favourable effect as an alternative or complementary treatment of stenotic lesions in arteries or veins associated with congenital defects. The aim of this study is to analyze our experience in this setting. MATERIAL AND METHODS: From February 1992 to March 1996, 28 stenting procedures were performed in 26 patients (mean age: 8.6 +/- 0.7 years; mean weight: 26.2 +/- 3 kg). In 12 patients, stenting was single, and a iliac Palmaz stents were always used. Stenting location was: pulmonary artery branches in 17 patients, right ventricular outflow in 2 patients, in the junction of right atrium with pulmonary artery in 2 patients, systemic veins in 2 patients and in post Mustard intratrial channel stenosis in 2 patients. 25 patients had previously undergone at least one surgical procedure. RESULTS: The stenotic diameter of the treated lesions increased significantly after the procedure (4.4 +/- 0.3 mm before stenting vs 11.6 +/- 0.3 mm after stenting, p < 0.0001) and the transtenotic gradient decreased from 38.1 +/- 5 to 12 +/- 3.8 mmHg. Those changes were associated with a diminution of right ventricular pressure (81.6 +/- 3 vs 56.7 +/- 6 mmHg, p < 0.0001) in patients with pulmonary branch stenosis without septal defects. There was no mortality among the percutaneously treated patients and only one patient needed surgery. Nevertheless, one patient died after bilateral intraoperative stenting. CONCLUSION: The treatment of proximal or distal stenotic lesions in the pulmonary tree, systemic veins, and obstructed intraatrial channels with stents, can replace or complement conventional balloon angioplasty. It also offers a useful and effective alternative to surgery, when it is impossible or carries a risk.

[Remnants of the embryologic sinus venosus valves. "Cor triatriatum dexter" (author's transl)]. / Persistencia de la válvula derecha del seno venoso "cor triatriatum dexter"

Remnants of the embryologic sinus venosus valves are not uncommon. However, persistent venous valves in the right atrium, which are large enough to obstruct the blood flow through the tricuspid valve (the so called cor triatriatum dexter) are very rare. There are 3 different types according to the location of the right atrial membrane. Right heart chambers hyoplasia is the most common association. Concurrent lesions of lesser degree have also been described. Although isolated cor triatriatum dexter could be symptomatic, both the clinical features and the prognosis usually are the consequence of the associated malformations. A review of the embryological aspects is offered.

[Single ventricle and related anomalies. Anatomic findings in 30 cases. Diagnostic and therapeutic implications (author's transl)]. / Ventrículo único y anomalías similares. Hallazgos anatómicos en 30 casos. Implicaciones diagnósticas y terapéuticas

Anatomic characteristics are studied in 30 cases of single ventricle and related anomalies. Cases are distributed into 6 groups according to: a) the derangement in the expansión of the atrioventricular canal towards the "bulbus cordis", which may be either defective (groups I, II and III) or excessive (groups IV and V), and b) the hypoplastic nature of the ventricular septum (group VI). The most important conclusions are: 1) An L-bulboventricular loop was found in 43.47% of the entire material; all of them pertained to groups I, II and III. 2) A normal position of the great arteries was uncommon, being encountered in 13.4% of the cases. An aorta situated anteriorly and to the left was present in 30.43% of the cases included in groups IV and V. 3) Obstructive anomalies of either outflow tract were discovered in 66.6% of the entire material, regardless of the type of bulboventricular loop. 4) The left atrioventricular orifice was found to be abnormally small in 56.66% of the total case material and in 88.23% of the cases pertaining to groups I, II and III. 5) A mitral atresia was encountered in two cases pertaining to groups I and II. The criteria which have been used to admit cases with this association of anomalies have been established in previous publications by one of the authors of the present study. 6) The changes in the spacial orientation of the leaflets of the right atrioventricular orifice in cases with an L-bulboventricular loop are attributed to the rotation of the heart around its longitudinal axis which takes place in cases with levocardia and mesocardia. 7) A preoperative study as accurate as possible is necessary in order to establish a rational approach to the surgical management of these patients.

[Fallot's tetralogy with severe symptomatology during the first year of life (author's transl)]. / Tetralogía de Fallot grave en el primer año de vida. Aspectos clínicos e implicaciones quirúrgicas

Seventeen cases of Fallot's Tetralogy with severe symptomatology during the first year of life are reviewed. In all of them, a cardiac catheterization and angiocardiography were performed. In 2 cases the pulmonary outflow tract was atretic. In general, the clinical, radiologic, electrocardiographic and hemodynamic findings were similar to those previously reported, although in 3 patients of the present series atypical electrocardiographic findings were found. Pronounced narrowing and horizontal orientation of the infundibular chamber, and accentuated hypoplasia of the pulmonary artery branches and of the left heart cavities and were all unfavourable findings for total correction frequently found in the present material. Minimal or non-existent aortic dextroposition was observed in association with cases of Fallot's Tetralogy with extreme severity. Surgery was indicated as an emergency measure in the majority of the cases. Palliatives procedures were performed in 10 patients, and corrective surgery in one. Surgical results agree with the extreme severity that this lesion has, when it gives rise to important symptomatology so early in the life.

[Balloon valvoplasty for critical aortic valve stenosis in neonates]. / La valvuloplastia en la estenosis valvular aórtica crítica del neonato.

OBJECTIVES: To evaluate the immediate results obtained with balloon aortic valvuloplasty in neonates and long-term echocardiographic outcome as well as to identify variables predictive for outcome. To identify the degree of agreement between hemodynamic and echocardiographic study. PATIENTS AND METHODS: We analyzed the results obtained in 26 procedures performed in 18 boys and 8 girls (2.25:1), aged 2 to 49 days (16.1 12.9 days). All procedures were performed in the Pediatric Cardiology Unit of Ramón y Cajal Hospital in Madrid between June 1989 and June 2001. Follow-up was from 0 to 144 months (39.5 39.7 months). RESULTS: The immediate effects observed through echocardiographic study were a significant decrease in the maximum Doppler gradient (from 77.8 to 32.4 mm Hg; p < 0.0001) and in the medium Doppler gradient (from 41.7 to 18.5 mm Hg; p < 0.05). Hemodynamic studies showed a significant decrease in left ventricular systolic pressure (from 119.8 to 82.8 mm Hg; p < 0.0001) and an increase in aortic systolic pressure (from 56.8 to 66.6 mm Hg; p < 0.007). The hemodynamic peak gradient decreased from 63.1 to 17.7 mm Hg; p < 0.0001. In 23 % of the patients, aortic insufficiency significantly increased. Echocardiographic follow-up showed a significant increase in the telediastolic size of the left ventricle and a decrease in the maximum and medium Doppler gradient. The procedure showed initial success in 68.7 % and analysis of survival free of valvular surgery was 65.8 % at 45 months. Proportional risk analysis revealed that the post-valvuloplasty gradient was a predictive factor for future valvular surgery.

[Ventricular septal defect in the first year of life (author's transl)]. / Comunicación interventricular en el primer año de la vida.

Thirty cases of CIV in the first year of life are presented. Association with prematurity and other malformations anticipate cardiac failure. Left ventricular hypertrophy is more frequent in conal defects. Repolarization disturbances were found in 73% of the cases in left precordial derivation with significative relief after surgical repair (banding). Conduction disturbances after surgical closure of ventricular septal defect was found in 15% of the cases. Surgical treatment during the first year was needed in 86% of patients. Only one case had good evolution without surgery. After four months of life total correction of septal defect as prefered. In patients with previous pulmonary artery banding, correction must be made one or two years later.

[Anomalous origin of the left coronary artery. Presentation of 5 cases]. / Origen anómalo de la arteria coronaria izquierda. Presentación de cinco casos.

Clinical findings are reported in 5 cases of anomalous left coronary artery originating from pulmonary trunk, with reference of ventricular function evaluated through echocardiographic and angio-haemodynamic methods. All patients were catheterized between 7 and 8 months of age. Left atrial and ventricular dilatation, diminished shortening of minor diameter and free wall thickness and septum normal values were obtained by echocardiography. Quantitative angiography of left ventricle demonstrated an elevation of end-diastolic volumes (mean values: 270% of predicted normal). The ejection phase indexes are diminished. The left ventricular mass/end-diastolic volume ratio was low in all cases. Myocardial imaging with thallium-201 demonstrated perfusion defect in lateral area of left ventricle in one patient. The electrocardiographic signs of myocardial necrosis support the diagnosis, and the echocardiographic and isotopic studies may contribute to it. The angio-haemodynamic method corroborate the diagnosis, and the myocardial pump function evaluation may help to decide the most appropriate treatment for this anomaly.

[Pulmonary atresia with intact interventricular septum. Study of 28 cases]. / Atresia pulmonar con tabique interventricular integro. Estudio de 28 casos

The present report is based on the study of 28 cases of pulmonary atresia with intact ventricular septum, all of them necropsically and/or angiographically verified. A microscopic study of the wall of both ventricles was performed in the 20 necropsy cases. The size of the right ventricular cavity, a fact of great surgical significance, did not correlate with the electrocardiogram and varied from diminute to very large. In two cases necropsically proved there was as associated atresia of the infundibular, in one of them existing additionally an Ebstein malformation of the tricuspid valve, which could be angiocardiographically diagnosed. The surgical mortality, in our hands, is 64.3%. All survivors have a small right ventricle.

[Unilateral agenesis of the pulmonary artery. Experience with 4 cases]. / Agenesia unilateral de la arteria pulmonar. Experiencia en 4 casos.

We present our experience with four cases of unilateral pulmonary agenesis, 3 of the right artery and the other one of the left. Two of the patients had pulmonary hypertension and one of them died at two years of age. All cases were diagnosed by using pulmonary angiography. Aortography demonstrated the absence of circulatory supply to the lung and a contralateral aortic arch to the absent pulmonary artery. We have performed a review of the literature with an emphasis on the diagnostic aspects of this condition, and discuss the considerations for possible surgery in symptomatic patients.