[Congenital sternal cleft]. / Hendidura esternal congenita.

INTRODUCTION: Congenital sternal cleft is a rare malformation. Little more than one hundred cases have been published, and rarely more than two cases are published in a single publication. We present five new cases that show the entire spectrum of defects. METHODS: They were 3 males and 2 females. Age range between 1 day and 5 years of age. There were 2 supraxiphoid clefts (SC), 2 gladiolar defects and 1 xiphoid cleft. We evaluate the demographics, associated malformations, mode of treatments and results. RESULTS: The SC were not associated to congenital herat malformations, but with a many other malformative spectrum (anterior cervical web, hemangiomatosis, CNS malformations, coloboma and pectus excavatum). The two females had SC and were operated on the 24th and 30th days of age. The first one developed a superior vena cava síndrome for a few days after surgery and a moderate pectus excavatum that did not required surgery. A midline cervical web was also surgically repaired at 10 years of age. The second patient with SC was treated with interferon alfa-2a and local infiltration of corticosteroid for treatment of hemangiomatosis. Both patients had excellent final results (follow up 11 and 5 years respectively). Gladiolar clefts were observed durign surgical repair of congenital Herat malformations (ventricular septal defect and partial anomalous of pulmonary venous draninage). They were repaired by direct closure of the defect. The patient with the xiphoid cleft had associaed a Cantrell pentalogy. He died 4 days after operation because of extreme prematurity and pulmonary vascular hypertenion. CONCLUSIONS: 1) We have observed five new cases of sternal cleft. 2) Each anatomical variety had their own mode of presentation, a different malformative spectrum and clinical evolution that are based on a distinct embryonic origin. 3) Excluding the xiphoid clefts, the long term surviving is 100%, but they are associated with a great variety of other congenital defects.

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.

[Extracorporeal membrane oxygenation, ECMO. Experience with the first 22 cases]. / Oxigenación por membrana extracorpórea, ECMO. Experiencia de los primeros 22 casos.

OBJECTIVE: Extracorporal membrane oxygenation (ECMO) is an alternative to cases of respiratory or cardiopulmonary insufficiency when conventional therapy has failed. We present the first 22 patients treated with ECMO at the neonatology unit of the "Gregorio Marañon" Hospital. PATIENTS AND METHODS: From October 1997 until September 1999, 22 patients were treated with ECMO. In 8 of them ECMO was necessary because of respiratory insufficiency without response to conventional treatment (r-ECMO) and a veno-venous tidal flow system was used. In 14 patients, cardiac ECMO was necessary in the veno-arterial modality because of ventricular failure after extracorporal circulatory assistance during cardiovascular surgery. RESULTS: The 8 patients of the respiratory ECMO group had a mean previous oxygenation index of 89 +/- 36.6 (50-150). The mean duration of ECMO was 7.8 +/- 6.5 (1-16) days and the age at the beginning of ECMO ranged between 1 and 151 days. The most frequent indications in this group were congenital diaphragmatic hernia in three cases, meconium aspiration syndrome in 2 cases and 1 case each of septic shock, idiopathic pulmonary hypertension and air leak. The cannula was removed in 5/8 patients due to recovery and 5/8 survived. The 14 patients of the cardiac ECMO group needed veno-arterial ECMO because of severe ventricular failure. Eight out of fourteen were decannulated after improvement. In 5/14 there was multiorgan failure or bad neurological prognosis resulting in death while on ECMO. In 1 out of 14 patients removal of the cannula was impossible because of cardiac insufficiency. The mean age at the beginning of ECMO was 54 (3-178) days. The mean weight at ECMO was 3209 +/- 739 (2700-5000) gr and the mean duration of ECMO was 6 (1-15) days. CONCLUSIONS: ECMO is an effective treatment in rescuing critical patients when conventional treatment fails. Meconium aspiration syndrome is the pathology with the best prognosis on ECMO. Cardiac ECMO represents a complex group of patients in which ECMO is the only treatment and which may result in recovery in 40% of the patients.

[Surgical treatment of Fallot's tetralogy with hyperplasia or agenesis of the conal septum]. / Tratamiento quirúrgico de la tetralogía de Fallot con hipoplasia o agenesia del septo conal.

INTRODUCTION: The clinical evolution, anatomosurgical aspects, and postoperative evolution of a specific group of Fallot's tetralogy with hipoplasic or agenesic of the conal septum (representing a 7% of our Fallot's tetralogy cases) is presented. METHODS: A total of 41 patients surgically corrected in our Service between February 1973 and December 1995 has been retrospectively studied. Ages ranged between 13 months and 13 year. Clinically all cases developed moderate or severe hypoxemia. In 43.9% of cases it was necessary to perform a palliative surgery at an age between 15 days and 4 years. RESULTS: There was no hypoxemics spells. In the ECG we have found a QS in the aVR in 40% of cases. There was 7 deaths (17% of mortality rate). Four cases required reoperation. The clinical evolution, ECG, EKO, and hemodynamics findings are commented. CONCLUSIONS: In this type of Fallot's tetralogy, specifically in those cases with a pulmonary artery ring of normal size, it is necessary to have a good preoperative diagnoses in order to perform a longitudinal ventriculotomy with right ventricular outflow tract enlargement. Also, it is important to be meticulous with the stitches in the closure of the ventricular septal defect with a patch in order to avoid the potential lesion of the aortic valve.

[The perioperative management of aortic coarctation in the neonatal period]. / Manejo perioperatorio de la coartación de aorta en el período neonatal.

Clinical records of 30 neonates with aortic coarctation admitted to neonate ICU from January 1985 to June 1990 are reviewed. We analyzed perioperative data to search for adverse prognostic signs. Patients weights were 2,970 +/- 500 grams and gestational age 38.5 +/- 1.7 weeks. Mean age at admission was 10.5 +/- 10 and mean age of surgery 13 +/- 11 days. 70% had associated congenital heart defects. Surgical technique was patch angioplasty in 86% and subclavian flap in 14%. Early or late mortality among patients with isolated aortic coarctation was nonexistent, and it was 28.5% in patients with other congenital heart defects. 13% has postoperative hypertension and 3.3% recoarctation. Adverse prognosis signs were preoperative (associated congenital heart defects, especially ventricular septal defect and interrupted aortic arch, greater dose of catecholamines and mechanical ventilation), intraoperative (pulmonary artery banding), and postoperative (hypotension, cardiac failure, arrhythmia, oligoanuria, metabolic acidosis, greater need of mechanical ventilation, bleeding and thrombopenia). Date are compared with other neonatal series.

[Cardiac surgery in the neonatal period. 17 years' experience]. / Cirugía cardiaca en el período neonatal. Diecisiete años de experiencia.

Although the mortality rate with cardiac surgery in the neonatal period has decreased in the last years, due to the development of the neonatal intensive care units and the routinary use of prostaglandins, it remains higher than in whatever other ages. Since october 1967 to may 1984, 321 consecutive cases were operated on. Ages ranged between 12 hours and 31 days. In 301 cases closed cardiac surgery was performed, and cardiopulmonary bypass was employed in the remaining 20 cases. Taking off the mortality rate by periods of time, first (1968-1974), second (1975-1979), third (1980-1984), and by surgical groups (closed and open heart surgery) we found a statistically significant decrease in the mortality rate comparing the results over the time with the current period (1980-1984) having 114 cases of closed surgery and a 27% of mortality rate and 11 cases of open heart surgery with 2 deaths, 18% of mortality rate. Follow up was possible in 184 out of the 203 survivors, representing a 91% with a range between 3 month a 15 years. Medical and surgical management and long term follow up is described.