[Magnetic resonance imaging and lung perfusion scintigraphy in tetralogy of Fallot following surgery]. / Resonancia magnética y gammagrafía de perfusión pulmonar en casos de tetralogía de Fallot operados.

OBJECTIVE: To evaluate pulmonary arteries in patients with tetralogy of Fallot following surgery with quantified lung perfusion scintigraphy and magnetic resonance imaging. MATERIAL AND METHODS: From January 1985 to December 1999, 47 patients who underwent surgery between 1985 and 1999 were studied. To obtain values of normality, 45 infants with no pulmonary artery disease were assessed with lung perfusion scintigraphy (right lung flow: 54-61%, left lung flow: 38.7-46%) and magnetic resonance imaging axial view (right/left pulmonary artery branch diameter ratio: 1-1.1). Patients with stenosis underwent catheterization. RESULTS: Group 1: 27 patients with normal parameters. Group 2: nine patients with left pulmonary branch stenosis and irregularities in all parameters; left/right branch diameter ratio 0.51 and left lung perfusion 26.3 +/- 7.9%, r correlation ratio 65%, p < 0.005, left branch pressure gradient 34.4 +/- 17.9 mm Hg, rate-gradient r 89%, p < 0.001. Group 3: five patients with right pulmonary branch stenosis and irregularities in all parameters; right/left branch diameter ratio 0.52, reduced right lung perfusion 32 +/- 11%, with a stenotic branch gradient of 40 +/- 19 mm Hg., rate/gradient ratio r 72%, p < 0.005; gradient/perfusion ratio r 82%, p < 0.003. Group 4: Six patients with bilateral stenosis, reduced diameter in the stenotic area with normal perfusion in both lungs and bilateral gradient. Eighteen patients with stenosis underwent re-operation, with favorable outcome in 14. CONCLUSION: Magnetic resonance imaging and quantified lung perfusion scintigraphy provide useful information in the follow-up of tetralogy of Fallot.

[Tetralogy of Fallot with absent pulmonary valve in a newborn and infant. Complete surgical correction]. / Tetralogía de Fallot con agenesia de válvula pulmonar en el recién nacido y lactante. Corrección completa.

We report two patients, a newborn and a 7-month old infant, with tetralogy of Fallot and absent pulmonary valve syndrome. Both had severe obstruction at the level of the ring with aneurysmal pulmonary artery branches, which compressed and displaced the trachea and main bronchial tubes. The neonate required mechanical ventilation from birth. Treatment was aggressive in both patients with interventricular septum defect closure, arterioplasty of the branches and homograft in the infant, and resection of the truncus and pulmonary branches with posterior face suture of both branches associated with a valved conduit in orthotopic position in the neonate. We believe that early treatment avoids airway degeneration and right ventricle volume overload.

Tetralogía de Fallot con agenesia de válvula pulmonar en el recién nacido y lactante. Corrección completa / Tetralogy of Fallot with absent pulmonary valve in a newborn and infant. Complete surgical correction

Se presentan 2 pacientes, un recién nacido y un lactante de 7 meses, con tetralogía de Fallot y agenesia de la válvula pulmonar. Ambos tenían obstrucción grave del anillo con ramas pulmonares aneurismáticas que comprimían y desplazaban la tráquea y los bronquios principales. El paciente recién nacido necesitó ventilación mecánica desde el nacimiento. El tratamiento fue agresivo en ambos, con cierre de la comunicación interventricular, arterioplastia de las ramas y homoinjerto en el lactante y resección del tronco y ramas pulmonares con sutura de la cara posterior de ambas ramas, asociado a un conducto valvulado en posición ortotópica en el neonato. Creemos que el tratamiento precoz evita la degeneración de la vía respiratoria y la sobrecarga de volumen del ventrículo derecho (AU)

Resonancia magnética y gammagrafía de perfusión pulmonar en casos de tetralogía de Fallot operados / Magnetic resonance imaging and lung perfusion scintigraphy in the tetralogy of fallot following surgery

Objetivo: Estudio de las ramas pulmonares de los pacientes intervenidos de tetralogía de Fallot con gammagrafía de perfusión cuantificada y resonancia magnética (RM).Material y métodos Desde enero de 1995 a diciembre de 1999 se estudiaron 47 pacientes intervenidos durante el período 1985-1999. Para obtener valores de normalidad, se estudiaron 45 jóvenes sin enfermedad pulmonar con gammagrafía (flujo pulmón derecho, 54-61 por ciento; flujo pulmón izquierdo, 38,7-46 por ciento) y RM en proyección axial (índice diámetro rama derecha/diámetro rama izquierda, 1-1,1). Los pacientes con estenosis fueron cateterizados. Resultados: Grupo 1: 27 pacientes con parámetros normales. Grupo 2: pacientes con estenosis de la rama pulmonar izquierda (9 casos con todos los parámetros alterados). Índice diámetro rama izquierda/rama derecha, 0,51, y perfusión pulmón izquierdo, 26,3+/-7,9 por ciento; coeficiente correlación r 65 por ciento; p < 0,005. Con un gradiente de presión en la rama izquierda de 34,4+/-17,9 mmHg índice/gradiente r 89 por ciento; p < 0,001. Grupo 3: 5 pacientes con estenosis rama pulmonar derecha con todos los parámetros alterados. Índice diámetro rama derecha/diámetro rama izquierda 0,52, disminución de la perfusión del pulmón derecho 32+/-11 por ciento con gradiente en esta rama de 40+/-19 mmHg con coeficiente índice/gradiente r 72 por ciento; p < 0,005; gradiente/perfusión r 82 por ciento; p < 0,003. Grupo 4: 6 pacientes con estenosis bilateral. Perfusión normal con diámetro reducido en la zona de estenosis. Dieciocho pacientes con obstrucción fueron reintervenidos y reevaluados con buenos resultados en 14 pacientes. Conclusión: La RM y la gammagrafía de perfusión cuantificada aportan información en el seguimiento de la tetralogía de Fallot (AU)

Lactante con tetralogía de Fallot y estenosis grave del ostium de la coronaria izquierda / Tetralogy of Fallot and severe coronary ostium stenoses in an infant

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[The treatment of postoperative junctional ectopic tachycardia]. / Tratamiento de la taquicardia ectópica de la unión tras la cirugía.

OBJECTIVE: To evaluate treatment of junctional ectopic tachycardia after cardiac surgery. MATERIAL AND METHODS: Twenty-seven patients (5.5 % of 488 patients who underwent surgery) were treated for junctional ectopic tachycardia between 1994 and 1998. There were 14 boys and 13 girls with a mean age of 11 11 months. Seven suffered from tetralogy of Fallot, seven from ventricular septal defect, six from atrioventricular septal defect, three from transposition of the great vessels and the remaining four had other complex heart diseases. The mean initial frequency was 186 27 beats/min. Crystalloid cardioplegia was applied in 274 patients (1994-1996) and 20 patients (7.4 %) showed junctional ectopic tachycardia. Hematic cardioplegia was performed in 214 patients (1997-1998) and seven patients (3.2 %) developed junctional ectopic tachycardia. Of the 33 patients who were treated during the surgical procedure with high mean doses of sympathomimetic catecholamine agents, 27 (81 %) developed tachycardia. Tachycardia developed 8.24 7 hours after surgery (range: 1-24 hours) in 25 patients and after 4 and 5 days in the remaining two patients. The mean duration of tachycardia was 4 days. RESULTS: In all patients rectal temperature was reduced to 32-34 C. Nineteen patients (70 %) showed a quick response (1-2 hours), although the technique was effective as an isolated procedure in only one patient. Sympathomimetic catecholamine level was reduced to 2-5 g/kg/min in 20 patients but this was effective in 14 (70 %). In 15 patients intravenous amiodarone was also administered and was effective in 11 patients (73 %). Finally, intravenous propafenone was administered to 5 patients. The most effective treatments were hypothermia with reduction of sympathomimetic catecholamine levels in 7 patients (100 %) or intravenous amiodarone in 4 (80 %). Tachycardia led to low cardiac output in 10 patients and only four recovered normal sinus rhythm. Eight patients died. Of these, hemorrhage in the junction area was confirmed in six patients. CONCLUSIONS: Junctional ectopic tachycardia is favored by high levels of sympathomimetic catecholamines after surgery. On the other hand, myocardial protection with hematic cardioplegia reduces tachycardia. Moderate hypothermia with reduction of sympathomimetic agents or intravenous amiodarone reverses ectopic tachycardia.

Tratamiento de la taquicardia ectópica de la unión tras la cirugía / Management of postoperative junctional ectopic tachyca

Objetivo: Tratamiento de la taquicardia ectópica de la unión tras la cirugía. Material y métodos: Durante el período 1994-1998 se trataron 27 pacientes (5,5 % de los 488 intervenidos) con edad de 11 ± 11 meses, 14 varones y 13 mujeres. Siete tenían tetralogía de Fallot; 7, comunicación interventricular; 6, canal auriculoventricular complejo; 3, transposición de grandes vasos, y 4, cardiopatía compleja. La frecuencia media al inicio fue de 186 27 lat./min. En 274 (1994-1996) se aplicó cardioplejía de cristaloides, en 20 con taquicardia (7,4%), y en 214 (1997-1998) con cardioplejía hemática, 7 con taquicardia (3,2%). De los 33 pacientes que se trataron a la salida de extracorpórea con dosis media elevada de catecolaminas, 27 (81%) presentaron taquicardia. En 25 pacientes la taquicardia apareció a las 8,24 7 h de la intervención (límites, 1-24 h), en 1 a los 4 días y en otro a los 5 días. La duración máxima de la taquicardia fue de 4 días. Resultados: A todos los pacientes se le redujo la temperatura rectal a 32-34 °C con efecto precoz 1-2 h en 19 (70 por ciento) pero sólo efectiva como tratamiento aislado en 1 caso. A 20 se les redujeron las catecolaminas hasta 2-5 g/kg/min con efectividad en 14 (70%) y en 15 se asoció amiodarona por vía intravenosa efectiva en 11 (73%). Finalmente, a 5 pacientes se le añadió propafenona también por vía intravenosa. La asociación de hipotermia con reducción de catecolamina (7 [100%]) o de hipotermia con amiodarona (4 [80%]) fueron los tratamientos más efectivos. En 10 pacientes la taquicardia generó un bajo gasto con recuperación del ritmo sinusal sólo en cuatro. Fallecieron 8 pacientes, en seis de los cuales se confirmó la presencia de hemorragia en la zona de la unión. Conclusión: El nivel elevado de catecolaminas tras la extracorpórea favorece la aparición de la taquicardia. Por el contrario, la protección del miocardio con cardioplejía hemática la disminuye. La hipotermia moderada con reducción de catecolaminas o asociada a amiodarona intravenosa eliminan la taquicardia (AU)

[Surgical treatment of supravalvular aortic stenosis]. / Tratamiento quirúrgico de la estenosis aórtica supravalvular.

OBJECTIVE: Surgical correction for 10 supravalvular aortic stenosis since 1988. MATERIALS AND METHODS: Diagnosis was carried out by means of echocardiography and magnetic resonance. Seven patients showed features of Williams-Beuren syndrome, 5 patients showed in their genotype a delection of 7-chromosome. 5 showed membranous-localized type obstruction and the other 5 with hourglass type. The patients in the first group underwent circumferential resection of the stenosis ring followed by a diamond- shaped patch and the second group were treated with resection of the stenosing ring associated with an inverted Y- shaped patch with releasing of the coronary ostia in two of them. RESULTS: There was no hospital death. The gradient was substantially reduced from 60 +/- 8 to 5 +/- 1 mm Hg (range 0-14). The average staying was under 7 +/- 1 days. The average follow-up was 58 +/- 8 months (2-120). CONCLUSIONS: Postoperatively, all the patients were in NYHA class I-II. The residual gradient was less than 15 mmHg in the echocardiography study during the follow-up and none of them required an additional operation.

La resonancia magnética en un recién nacido con agenesia de válvula pulmonar y deleción del cromosoma 22q 11,2 / Microdeletion of chromosomal region 22q 11 in newborn with congenital absence of pulmonary valve. Evaluation with magnetic resonance imaging

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Tratamiento quirúrgico de la estenosis aórtica supravalvular / Supravalvular aortic stenosis surgical treatment

OBJETIVO: Corrección desde 1998 de 10 pacientes afectados de una estenosis aórtica supravalvular. MATERIAL Y MÉTODOS: El diagnóstico se realizó con ecocardiografía y resonancia magnética. Siete tenían un síndrome de Williams-Beuren, confirmándose en 5 una deleción del cromosoma 7. Cinco tenían obstrucción de tipo membranosa y otros 5 "en reloj de arena". Para el primer grupo se aplicó una resección y aortoplastia con parche en lágrima, y en el segundo grupo aortoplastia con parche en Y invertida con liberación de ostium coronario en 2 casos. RESULTADOS: No existió mortalidad en ningún grupo, reduciéndose el gradiente de 60 ñ 8 a 5 ñ 1 mmHg (rango 0-14). La estancia no fue superior a 7 ñ 1 días. El seguimiento medio fue de 58 ñ 8 meses (2-120). CONCLUSIÓN: Todos los pacientes se encuentran en grado funcional I-II de la NYHA. En los controles ecocardiográficos ningún paciente superó los 15 mmHg, no precisando reintervención (AU)

[Surgical correction of symptomatic ventricular septal defects in patients less than 6 months of age]. / Corrección de la comunicación interventricular aislada sintomática en pacientes menores de 6 meses.

OBJECTIVE: Our objective was to evaluate the efficiency of a single surgical intervention in patients with symptomatic interventricular septal defects during the first six months of life. PATIENTS AND METHODS: Between 1989 and 1997, 42 patients, 20 males and 22 females with an average age of 3.9 +/- 0.3 months and an average weight of 4 +/- 0.4 kg, were operated. Seven suffered from Down's syndrome. All of the patients became symptomatic during the first two months of life. The defect was localized by using Echo-Doppler in all of the cases. Thirty-six had perimembranous ventricular septal defects, 2 were muscular, 3 multiple and 1 was infundibular. The average defect size was 8 +/- 1.2 mm. A catheter was placed in 34 patient with the following results: Left to right shunt with 2.2 +/- 1.2, right ventricle systolic pressure of 57 +/- 20 mmHg (16 with systemic pulmonary pressure) and an average pulmonary pressure of 38 +/- 1.8 mmHg. The average pulmonary vascular resistance was 28 +/- 1.8 U/m2. Deep hypothermia (18 degrees C was applied during the surgery and the average cardiac arrest time was 31 +/- 4 minutes. RESULTS: None of the patients died during or after the surgical procedure. Patients required minimum ionotropic support during the first hours. The average time in the intensive care unit was 3.5 +/- 0.6 days, with an average hospitalization time of 11.2 +/- 2.1 days. Immediate complications included one hypertensive crisis, four junctional ectopic tachycardias, two atrio-ventricular blocks, 1 transient arrhythmia, two atelectasia-pneumonias, two patients with stridor and two sternal infections. During the follow-up period, two patients required a second intervention to repair the patch. CONCLUSIONS: We believe that one-time surgery is adequate to correct symptomatic ventricular septal defects.

[Aortic valve stenosis. Surgical treatment in children]. / Estenosis aórtica valvular. Tratamiento quirúrgico en la infancia.

OBJECTIVES: The results of aortic commissurotomy, as the first step in the treatment of aortic valve stenosis in children, have been estimated after a medium-term follow-up. MATERIAL AND METHODS: Twenty-two patients were operated at a mean age of 7.3 +/- 3.6 years of age. Surgery was performed under cardiopulmonary bypass and moderate hypothermia. Twenty patients underwent cardiac catheterization (the systolic gradient pressure was 67.9 +/- 24.7 mmHg and the end diastolic pressure was 17 +/- 7.1 mmHg). We evaluated 10 patients with Doppler flow echocardiography (the valvular area was 0.8 +/- 0.2 cm2/m2). After surgery, we made a follow-up of 8.6 +/- 5.4 years. RESULTS: The aortic valve was bicuspid in 13 cases and tricuspid in 9 cases. There was no mortality. Before the age of 7, 7 patients developed restenosis. The mean pressure gradient was 92.5 +/- 16.6 mmHg, so six of them underwent valvuloplasty and the other one was operated with an aortic valvulated homograft. The pressure gradient, which was estimated in 20 patients, was lowered to 43.5 +/- 17.5 mmHg and the aortic area was increased to 2.4 cm2/m2. Six patients had a pressure gradient above 40 mmHg. Six patients had previous aortic regurgitation and this reappeared in 3 patients after valvulotomy. At this time, 12 patients have aortic regurgitation, being mild in 9 patients, mild to moderate in 1 and moderate in 2 patients. CONCLUSION: Valvulotomy is a palliative therapeutic method. We had no mortality. Restenosis appeared in 7 patients, before the age of seven years, and it can be easily corrected. The aortic regurgitation is usually mild. Valve replacement can be avoided in childhood.

[Systemic-pulmonary shunt with autologous internal jugular vein]. / Fístula sistémico-pulmonar con vena yugular interna autóloga.

We report a variant of systemic-pulmonary shunt for newborns or low-weight neonates affected with hypoplasia or atresia of pulmonary artery. An autologous internal jugular vein was used. Six months after surgery all patients referred no symptoms and a normal systemic-pulmonary shunt with an arterial oxygenation of 82-90% was present.