Resultados a medio plazo del cierre percutáneo de la comunicación interauricular en niños / Medium-term results of percutaneous closure of atrial septal defect

Objetivo: Evaluar los resultados a medio plazo del cierre percutáneo de la comunicación interauricular (CIA). Métodos: Bajo anestesia general se procedió por vía anterógrada al intento de cierre percutáneo de la CIA tipo ostium secundum en 22 niños. La edad fue de 5,7 ± 2, 4 años y el peso 23 ± 11kg. El procedimiento se monitorizó mediante ecocardiografía transesofágica (ETE). Los dispositivos utilizados fueron DAS-Angel Wings(r) y Amplatzer(r). Resultados: Hemodinámicos: presión arterial pulmonar 13 ± 2,8 mmHg, resistencias pulmonares, 1,3 ± 0,3U/m2 y Qp/Qs 2,2 ± 0,6. El diámetro del defecto por ETE fue de 14,5 ± 6,3 mm y por oclusión con balón de 15,9m2 5,3mm. Se emplearon 31 dispositivos, 27 Amplatzer(r) y 4DAS-Angel Wings(r). Se retiraron sin dificultad 12 dispositivos Amplatzer(r)a través de su introductor, cinco por discrepancia con el tamaño de la aurícula izquierda, cuatro por no estabilizarse en el tabique al ser demasiado pequeños y tres por apertura defectuosa en la aurícula izquierda. En 19 pacientes el dispositivo se implantó de forma correcta. La ecocardiografía transtorácica con Doppler color realizada a las 24h demostró ausencia de cortocircuito 17 y en todos al mes de la implantación. Los niños fueron dados de alta a las 38 ± 12h y en el seguimiento de 15 ± 6meses no se han producido complicaciones, fracturas de la estructura, ni se han demostrado cortocircuitos residuales. Conclusión: Mediante cierre percutáneo se consiguió una elevada tasa de cierre sin cortocircuito residual cuando se realizó una adecuada selección de los pacientes (AU)

[Mid-term results of percutaneous closure of atrial septal defect in children]. / Resultados a medio plazo del cierre percutáneo de la comunicación interauricular en niños.

OBJECTIVE: To evaluate the medium-term results of percutaneous closure of atrial septal defect. METHODS: Twenty-two children (mean weight, 23 11kg; mean age, 5.7 2.4 years) underwent percutaneous atrial septal defect closure under general anesthesia. The procedure was monitored by transesophageal echocardiography. DAS-Angel Wings (n4) and the Amplatzer device (n18) were used. RESULTS: Mean pulmonary artery pressure was 13 2.8mmHg, mean pulmonary vascular resistance was 1.50.5U/m2 and mean Qp/Qs flow ratio was 2.2 0.6. The mean diameter of the defects was 14.5 6.3mm by transesophageal echocardiography OmniPlane measurement and 15.95.3mm using balloon occlusion reference. A total of 31 devices were used: 4 Angel Wings and 27Amplatzer devices. Twelve Amplatzer devices were withdrawn through the introducer without complications, 5 due to a discrepancy in the size of the left auricle, 4 because they were too small to stabilize in the septum and 3 due to defective opening in the left auricle. In 19 patients implantation was successful. In 17 patients transthoracic color Doppler echocardiography carried out 24 hours after the procedure showed a minimal shunt which was no longer present 1 month later. The mean time of discharge was 38 12 hours after the procedure. After a mean follow up 15 6 months the patients remain asymptomatic with no clinical or technical problems. CONCLUSION: The success rate of percutaneous closure of atrial septal defects in well-selected patients was high and presented no complications.

[Percutaneous closure of atrial septal defects: midterm results of a new therapeutic strategy]. / Cierre percutáneo de la comunicación interauricular: resultados a medio plazo de esta nueva opción terapéutica.

OBJECTIVE: To evaluate the midterm results of percutaneous closure of the atrial septal defect using two new devices. PATIENTS AND METHODS: Nine children (weight 19.7 +/- 7 kg, age 5.1 +/- 1.9 years) underwent percutaneous type II atrial septal defect closure through the antegrade pathway under general anaesthesia, and monitored by transesophageal echocardiography. The closing devices used were DAS-Angel Wings and Ampaltzer. RESULTS: The hemodynamic results were: mean diameter of the defects was 11.4 +/- 2 mm by TEE measurement and 12.3 +/- 2.6 mm using balloon occlusion reference. Mean pulmonary artery pressure was 12.7 +/- 2 mmHg and mean pulmonary vascular resistance 1.5 +/- 0.5 U/m2. A total of 13 devices were used: 9 Amplatzer and 4 DAS-Angel Wings. Four Amplatzer through the introducer were retrieved without complications. Two of which because of lack of sufficient stability in the atrial septum because they were too small inappropriate and the other two because of inappropriate expansion of distal disk of the device. Finally in all patients the device was a successfully deployed. The angiographic evaluation immediate post-procedure showed a minimal shunt in five patients that was no longer present by color Doppler echocardiography 24 hours later. The children were discharged 38 +/- 12 hours after the procedure and at a mean follow up of 9.6 +/- 2.2 months they remain asymptomatic without any clinical or technical problems. CONCLUSION: With the right selection of patients percutaneous closure of atrial septal defects can obtain a very high success rate without complications.

Estado actual del cateterismo cardiaco en pediatría / Pediatric cardiac catheterization: state of the art

En los últimos años se han producido importantes avances en el cateterismo cardiaco en niños. El desarrollo de otras técnicas de imagen como la ecocardiografía y la resonancia magnética nuclear han sustituido al cateterismo diagnóstico en muchos casos, mientras que las técnicas invasivos son cada vez más frecuentes. En el HGU 'Gregorio Marañón' se han practicado en los últimos diez años 1.436 cateterismos cardiacos en niños, de los que 338 fueron terapéuticos, y 243 biopsias endomiocárdicas. Se presentan las indicaciones, contraindicaciones y complicaciones del cateterismo cardiaco diagnóstico. Asimismo se exponen los diferentes procedimientos intervencionistas, incluyendo las últimas técnicas como el cierre percutáneo de la comunicación interauricular. Es de esperar que materiales y técnicas mejoren en los próximos años permitiendo extender sus aplicaciones a numerosos pacientes (AU)

Diagnóstico y tratamiento de la estenosis aórtica valvular en niños / Pediatric aortic valve stenosis: diagnosis and treatment

La estenosis aórtica congénita es una cardiopatía frecuente que condiciona la actividad cotidiana del niño y del adolescente. Se discuten las técnicas diagnósticas necesarias en el seguimiento de esta enfermedad en los distintos grupos de edad pediátricos, así como la historia natural. La mayoría de estos pacientes precisa siempre cierto grado de restricción de su actividad física y de su práctica deportiva debido al riesgo de muerte súbita y síncope. El tratamiento actual de la estenosis aórtica valvular en la edad Pediátrica es la valvuloplastia con catéter balón, que ha demostrado resultados superiores a las técnicas quirúrgicas. Siempre que sea posible debe evitarse el recambio valvular en niños, debido a la duración limitada de las prótesis y la necesidad de anticoagulación (AU)

Alternativas terapéuticas de la miocardiopatía dilatada idiopática en la edad pediátrica / Therapeutic alternatives for the management of idiopathic dilated cardiomyopathy

La miocardiopatía dilatada idiopática es la afectación cardiaca con disfunción sistólica más frecuente en la infancia. Aunque su causa es desconocida, la mayoría de los autores acepta que se trata del estadio final evolutivo de una miocarditis vírica aguda o subaguda. En este artículo se recogen las alternativas terapéuticas de este proceso: tratamiento de la insuficiencia cardiaca, inmunoterapia y trasplante cardiaco, señalando la experiencia de la Sección de Cardiología Pediátrica del HGU 'Gregorio Marañón' en algunas de estas opciones terapéuticas (AU)

[Radiofrequency catheter ablation for atrial tachycardia]. / Ablación con catéter mediante radiofrecuencia de taquicardias auriculares.

INTRODUCTION: Radiofrequency catheter ablation of atrial tachycardia guided by bipolar activation mapping has been reported in the last years. This article reports the use of radiofrequency catheter ablation for the treatment of atrial tachycardia using simultaneous bipolar and unipolar activation mapping at our institution. METHODS: Nine patients (7 male and 2 female, mean age 37.2 +/- 24.1 years), were selected for radiofrequency catheter ablation of drug refractory atrial tachycardia. Mapping procedure included an investigation of the local earliest bipolar and unipolar activity and unipolar morphology analysis. RESULTS: Atrial tachycardia was successfully ablated in 7 patients (78%) with an average number of 6.8 +/- 3.1 RF pulses. Procedure related complications and tachycardia follow-up recurrences were not observed in any patient. Bipolar local activation time was significantly shorter at successful than at unsuccessful ablation sites (-30 +/- 21.1 ms vs -18.3 +/- 20.6 ms; p = 0.01). No difference was observed in unipolar local activation time between successful and unsuccessful sites (-22.5 +/- 26.2 ms vs -19.8 +/- 21.5 ms; p = 0.56). Accurate localization of the successful ablation site by unipolar electrogram analysis was not feasible because a "QS" pattern was found at both 21 unsuccessful and 2 successful ablation sites. Finally, a fast slope of the negative deflection of the unipolar electrogram was found at 2 out of 45 unsuccessful and 3 out of 6 successful ablation sites. CONCLUSIONS: Radiofrequency catheter ablation of atrial tachycardia is feasible without complications in most patients. Bipolar activation mapping accurately localizes the successful ablation site. A "QS" pattern is not predictive of successful radiofrequency application.

[The midterm evolution in the percutaneous closure of ductus arteriosus with the Rashkind prosthesis]. / Evolución a medio plazo del cierre percutáneo del ductus arterioso mediante la prótesis de Rashkind.

Background percutaneous PDA occlusion has been proposed as a valid therapeutic alternative to surgical closure. Between April 1990 and July 1994, 44 patients underwent percutaneous PDA occlusion. Thirteen patients were less than 18 months old and weighed 8.7 +/- 3.2 Kg (Group I). Thirty-one patients, older than 18 months of age, had a mean weight of 36.8 +/- 20.6 Kg (Group II). Four patients in group I and two in group II had associated cardiac anomalies Residual shunt after percutaneous occlusion was studied by color Doppler. Fifty-one occluding devices were used. Forty-nine were implanted and there were two device embolizations. Twelve devices were implanted in group I. One device needed surgical removal from the right ventricle where it had embolized. In group II, 31 devices were implanted and one embolized to the descending aorta and was percutaneously removed through the femoral artery sheath. In two patients of group I and four of group II, a second device was implanted. The total occlusion rate immediately after implantation at 24 hours and at 6 months was 75%, 83% and 83% in group I and 64.5%, 77% and 84% in group II. After implantation of a second device the total occlusion rate without residual shunt reached 100% in group I (after 16 +/- 12 months follow-up) and 97% in group II (after 23 +/- 16 months of follow-up). We conclude that percutaneous ductal occlusion with a Rashkind device is a valid therapeutic alternative to surgical closure in all age groups.

[Outcome of anatomical correction of transposition of the great vessels in the neonatal period]. / Resultados de la corrección anatómica en la transposición de las grandes arterias en el periodo neonatal.

Between february 1992 to January 1994, anatomic correction was performed on 15 patients with transposition of the great arteries and intact ventricular septum. The mean age was 8.3 +/- 2.9 days and the mean weight 3.39 +/- 0.39 Kg. Before the operation, 13 patients (86.6%) received prostaglandin El infusion and 13 patients (86.6%) underwent Rashkind septostomy. Mean aortic cross-clamps was 56 +/- 11 minutes and mean cardiopulmonary bypass was 108 +/- 91 minutes. Hospital mortality rate was 13%. Mean extubation period was mean discharge from ICU was 13.1 +/- 12.3 days and discharge of hospital was 17.8 +/- 7.5 days. The mean followup period was 11.4 months. All the patients remained asymptomatic with adequate psychomotor and ponderal development. All patients remained in sinus rhythm. Pulmonary suture gradient was over 60 mm Hg in five patients (33%). One patient needed reintervention and four pulmonary artery angioplasty (PAA) that was successful. In the midterm follow-up pulmonary stenosis suture was the most common complication. If the stenosis is severe and PAA should be the initial approach.

[The myxoma syndrome: "cardiac myxoma, cutaneous pigmented lesions and peripheral and endocrine neoplasms". Apropos 2 cases]. / Síndrome mixoma: "Mixoma cardíaco, lesiones pigmentadas cutáneas y tumores periféricos y endocrinos". A propósito de dos casos.

We present two cases, mother and son, with "syndrome myxoma". Both had cardiac myxoma and cutaneous pigmented lesions. The son had a cutaneous myxoma and the mother had been diagnosed as having Cushing's syndrome caused by adrenal adenoma. The "syndrome myxoma" is a systemic disease which causes cardiac, cutaneous, and breast myxomas, adrenal disease, testicle and pituitary tumours. Whenever it is diagnosed all first relatives should be tested for the syndrome. The patient should be re-examined every six-twelve months, due to frequent recurrence of cardiac myxoma.

[Rheumatic fever: clinical and therapeutic aspects]. / Fiebre reumática: aspectos clínicos y terapéuticos.

From 1974 to 1987 we have diagnosed thirty three patients with rheumatic fever. The age of onset ranged from three to sixteen years with a mean age of nine years and six months. Carditis was the most frequent major criteria (27/33) followed by arthritis (14/33) and Sydenham's chorea (6/33). All the patients with carditis had the mitral valve affected. The usual treatment in the acute phase was penicillin and aspirin. The prophylaxis recommended to the patients was penicillin G benzatine every 21 days if they had carditis and every 28 days if they didn't. Two patients allergic to penicillin are receiving erythromycin. We have observed that the incidence of rheumatic fever has remained unchanged that the age of onset increased and that the antiinflammatory drug of choice in most patients with carditis is aspirin.

[Congenital unilateral pulmonary hypoplasia]. / Hipoplasia pulmonar unilateral congénita.

Authors report twelve patients who presented unilateral diminished lung volume on chest x-ray and analyze ECG, bronchography, lung scan and angiography performed in this patients. Based on these data and following classification proposed by CURRARINO they divide patients in groups: 1) Simple lung hypoplasia, seven cases, four of these were of the left side. 2) Absence of one pulmonary artery, two cases. 3) Scimitar syndrome, three cases. Eleven patients have remained free of significant symptoms. Authors believe that diagnostic work-up of a patient with unilateral diminished lung volume on chest x-ray should depend on clinical symptoms and possibility of surgical treatment. Therapeutic approach will depend on clinical and anatomic findings.

[Clinical evolution of rheumatic carditis treated with aspirin]. / Evolución clínica de las carditis reumáticas tratadas con aspirina.

We have analyzed the clinical, laboratory, electrocardiographic and echocardiographic findings of six patients with rheumatic carditis whose antiinflammatory treatment was aspirin. We include one case who was initially treated with steroids. She relapsed when the steroid treatment was discontinued and she was then treated with aspirin. There were 2 males and four females. The age range was 9 to 14 years. This was the first rheumatic attack for all the patients. They were also treated with penicillin G during ten days. The prophylaxis was accomplished with benzatine penicillin 1.200.000 IU at 28 days intervals. The aspirin was given at doses ranging from 60 to 100 mg/Kg/day for eight to twelve weeks. All the patients had auscultatory findings consistent with mitral insufficiency accompanied in two cases with findings of aortic regurgitation. In three cases, there was a transient mild diastolic murmur during the first five days. In one case the rheumatic carditis was complicated with acute renal failure due to acute rheumatic nephritis that required peritoneal dialysis. The follow-up period ranged from nine to twenty six months. There have been no relapses. All the patients remain asymptomatic and leading a normal life. At the last visit three patients had mild mitral insufficiency and in three patients the clinical findings, electrocardiogram and echocardiogram were normal. If we accept that most of the cases of rheumatic carditis are mild or moderate, is our believe that the aspirin is the antiinflammatory treatment of choice at the present time.

[Acute lymphoblastic leukemia an pericarditis (author's transl)]. / Un caso de leucemia linfoblástica aguda revelada por una pleuropericarditis

In a brief communication a case of acute lymphoblastic leukemia is presented, of which the principal characteristics were: 1) An exceptional onset due to its pleuropericardial manifestations while the hemogram was normal. 2) A quick terminal evolution after a short and total remission. As far as authors know in Spain, this localization has never been described primarily and in consulted bibliography the appeared cases were always found in older patients. We call attention to the pericardial effusions of a large volume in children in which the diagnosis of classical pericarditis can't be quickly detected.