[Magnetic resonance imaging in long-term assessment after surgery for aortic coarctation]. / Valoración a largo plazo con resonancia magnética en pacientes corregidos de coartación de aorta.

OBJECTIVES: Spin echo or gradient echo magnetic resonance imaging provides enough information to plan patient management and can be completed with the use of three-dimensional magnetic resonance angiography to evaluate the need for reintervention, assess follow-up, or discharge the patient. PATIENTS AND METHODS: From 1990-2003, we evaluated 101 patients after initial corrective surgery for aortic coarctation at the age of 3.1 +/- 3 years, with subsequent postoperative assessment at 1 year and again 12.4 +/- 4.2 years later. No mortality was registered during the follow-up. Corrective surgery was performed in 32 patients (31.6 %) before the first month of life. All the patients were evaluated with spin-echo and echo gradient and 34 were evaluated with magnetic resonance angiography. The patients were classified into two groups: group A consisted of 68 patients (11 < 1 month old) with no complications. End-to-end anastomosis was performed in 55, the Alvarez technique in five, the Waldhausen technique in six, and conduit in two. Group B consisted of 33 patients complicated with early aortic recoarctation (21 corrected before the age of 1 month). End-to-end anastomosis was performed in 29, the Alvarez technique in two, and grafting in two. During the follow-up, 43 reinterventions were performed. Isthmus diameter/descending aorta diameter at the diaphragmatic level and repaired site diameter/descending aorta diameter at the diaphragmatic level were measured. RESULTS: Group A: the isthmus diameter/descending aorta diameter at the diaphragmatic level index was 0.92 +/- 0.08 and the repaired site diameter/ descending aorta diameter at diaphragmatic level index was 0.90 +/- 0.05. Twenty-three patients were assessed by means of three-dimensional magnetic resonance angiography, which revealed two late stenosis (one in a 10-year-old patient with an end-to-end anastomosis performed previously, and another in an 18-year-old patient with a proximal and distal obstructed conduit). Group B: the isthmus diameter/descending aorta diameter index was 0.84 +/- 0.1 and the repaired site diameter/ descending aorta diameter index was 0.82 +/- 0.11. Eleven patients were assessed by means of three-dimensional magnetic resonance angiography, which revealed five stenosis indexes (0.53-0.73) surgically corrected before the age of 2 months, four with an initial technique based on end-to-end anastomosis and one with a graft. CONCLUSIONS: Our results support the influence of young age, the use of end-to-end anastomosis and grafts in recoarctation and their late influence on recurrent recoarctation. The patients in group A were discharged in childhood or adulthood after periodic follow-up every 5 years with magnetic resonance angiography for 18 years with results within the normal range, while patients in group B required longer follow-up.

Valoración a largo plazo con resonancia magnética en pacientes corregidos de coartación de aorta / Magnetic resonance imaging in long-term assessment after surgery for aortic coarctation

Objetivos La resonancia con spin-eco o ecogradiente da una información suficiente que puede completarse con la angiorresonancia tridimensional para decidir una reintervención, controlar un seguimiento o dar el alta. Pacientes y métodos Durante el período 1990-2003 evaluamos 101 pacientes intervenidos inicialmente a los 3,1 ± 3 años, con estudio posterior al año y a los 12,4 ± 4,2 años de su intervención. No presentaron mortalidad en el seguimiento. Un total de 32 (31,6 %) fueron corregidos antes del mes de vida. Todos se estudiaron con spin-eco y ecogradiente y 34 con angiorresonancia. Los pacientes se dividieron en dos grupos: grupo A, con 68 pacientes, sin complicaciones (11 menores de un mes) con cirugía término-terminal 55, Álvarez 5, Waldhausen 6 y conducto en 2 casos; grupo B, con 33 pacientes complicados con recoartación precoz (21 corregidos antes de un mes), cirugía término-terminal 29, Álvarez 2 y parche en 2 casos. Durante el tiempo de seguimiento se realizaron 43 reintervenciones. Se midieron índices diámetro istmo/diámetro aorta descendente a la altura del diafragma e índice diámetro zona intervenida/diámetro aorta descendente en el diafragma. Resultados En el grupo A, el índice istmo/aorta descendente a la altura del diafragma era 0,92 ± 0,08 y el índice zona intervenida/aorta descendente en el diafragma, 0,9 ± 0,05. Un total de 23 fueron revisados con angiografía tridimensional. Se vieron dos estenosis tardías en un paciente de 10 años operado de término-terminal y en otro paciente de 18 años, un conducto con obstrucción distal y proximal. En el grupo B: el índice istmo/aorta descendente era 0,84 ± 0,1 y el índice zona intervenida/aorta descendente, 0,82 ± 0,11. De estos pacientes, 11 fueron revisados con angiorresonancia, 5 con reestenosis índice (0,53-0,73) intervenidos antes de los 2 meses, 4 con técnica inicial término-terminal y otro con parche. Conclusiones Nuestros resultados apoyan la influencia de la edad precoz, la técnica término-terminal y el parche en la recoartación y su influencia tardía en la rerrecoartaciones. Los pacientes del grupo A pueden ser dados de alta en edad juvenil-adulta, tras seguimientos periódicos cada cinco años con resonancia magnética durante 18 años si los resultados están dentro de la normalidad, mientras que los del grupo B necesitan controles más prolongados

Objectives Spin echo or gradient echo magnetic resonance imaging provides enough information to plan patient management and can be completed with the use of three-dimensional magnetic resonance angiography to evaluate the need for reintervention, assess follow-up, or discharge the patient. Patients and methods From 1990-2003, we evaluated 101 patients after initial corrective surgery for aortic coarctation at the age of 3.1 ± 3 years, with subsequent postoperative assessment at 1 year and again 12.4 ± 4.2 years later. No mortality was registered during the follow-up. Corrective surgery was performed in 32 patients (31.6 %) before the first month of life. All the patients were evaluated with spin-echo and echo gradient and 34 were evaluated with magnetic resonance angiography. The patients were classified into two groups: group A consisted of 68 patients (11 < 1 month old) with no complications. End-to-end anastomosis was performed in 55, the Alvarez technique in five, the Waldhausen technique in six, and conduit in two. Group B consisted of 33 patients complicated with early aortic recoarctation (21 corrected before the age of 1 month). End-to-end anastomosis was performed in 29, the Alvarez technique in two, and grafting in two. During the follow-up, 43 reinterventions were performed. Isthmus diameter/descending aorta diameter at the diaphragmatic level and repaired site diameter/descending aorta diameter at the diaphragmatic level were measured. Results Group A: the isthmus diameter/descending aorta diameter at the diaphragmatic level index was 0.92 ± 0.08 and the repaired site diameter/ descending aorta diameter at diaphragmatic level index was 0.90 ± 0.05. Twenty-three patients were assessed by means of three-dimensional magnetic resonance angiography, which revealed two late stenosis (one in a 10-year-old patient with an end-to-end anastomosis performed previously, and another in an 18-year-old patient with a proximal and distal obstructed conduit). Group B: the isthmus diameter/descending aorta diameter index was 0.84 ± 0.1 and the repaired site diameter/ descending aorta diameter index was 0.82 ± 0.11. Eleven patients were assessed by means of three-dimensional magnetic resonance angiography, which revealed five stenosis indexes (0.53-0.73) surgically corrected before the age of 2 months, four with an initial technique based on end-to-end anastomosis and one with a graft. Conclusions Our results support the influence of young age, the use of end-to-end anastomosis and grafts in recoarctation and their late influence on recurrent recoarctation. The patients in group A were discharged in childhood or adulthood after periodic follow-up every 5 years with magnetic resonance angiography for 18 years with results within the normal range, while patients in group B required longer follow-up

Tetralogía de Fallot con ausencia de rama pulmonar izquierda. Corrección quirúrgica / Tetralogy of Fallot with absence of the left pulmonary branch. Surgical correction

No disponible

[Tuberous sclerosis associated with rhabdomyoma in the left ventricular outlet tract]. / Esclerosis tuberosa con rabdomioma del tracto de salida del ventrículo izquierdo.

We report a 2-month old newborn with a family history of adenoma sebaceum, achromic spots and renal lithiasis. Echocardiography-magnetic resonance imaging showed a solitary pediculated rhabdomyoma in the left ventricular outlet tract causing a severe systolic aortic valve obstruction. Surgical excision was carried out through the aortic valve with no valvular lesions. The patient presented multiple seizures 24 hours after surgery with good response to medical therapy. Ten days later, computed tomography showed a spot lesion in the subependimary area in the posterior position of the parietal horn, compatible with a hamartoma associated with tuberous sclerosis.

Esclerosis tuberosa con rabdomioma del tracto de salida del ventrículo izquierdo / Tuberous sclerosis associated with rhabdomyoma in the left ventricular outlet tract

Paciente de 2 meses de edad con antecedentes familiares de adenomas sebáceos, manchas acrómicas y litiasis renal. Fue diagnosticado por ecocardiografía y resonancia de rabdomioma solitario pediculado en el tracto de salida del ventrículo izquierdo, con obstrucción grave en sístole de la válvula aórtica. Se realizó resección por vía aórtica sin afectación valvular. El paciente presentó crisis convulsivas múltiples a partir de las 24 h de la intervención con buena respuesta al tratamiento. A los 10 días, en un estudio con tomografía computarizada se observa en zona subependimaria en la porción posterior del asta parietal del ventrículo lateral derecho una lesión puntiforme que puede corresponder a un hamartoma con esclerosis tuberosa (AU)

We report a 2-month old newborn with a family history of adenoma sebaceum, achromic spots and renal lithiasis. Echocardiography-magnetic resonance imaging showed a solitary pediculated rhabdomyoma in the left ventricular outlet tract causing a severe systolic aortic valve obstruction. Surgical excision was carried out through the aortic valve with no valvular lesions. The patient presented multiple seizures 24 hours after surgery with good response to medical therapy. Ten days later, computed tomography showed a spot lesion in the subependimary area in the posterior position of the parietal horn, compatible with ahamartoma associated with tuberous sclerosis (AU)

[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)

[Criss-cross heart with atrioventricular discordance, straddling A-V valve, double outlet right ventricle and preexcitation]. / Criss-cross con discordancia auriculoventricular, acabalgamiento de la válvula auriculoventricular derecha, doble salida del ventrículo derecho y preexcitación.

We report the case of a newborn with cyanosis, dyspnea, and supraventricular tachycardia due to reentry with good response to medical therapy. The diagnosis was made by echocardiography. The patient died suddenly at 6 days of follow-up. Necropsy confirmed the following anomaly: criss-cross heart with straddling right atrioventricular valve, and left atrioventricular valve stenosis connected to the anterior trabeculated ventricle giving rise to the pulmonary artery and aorta.Precocious rotation when septal closure is not concluded favors atrioventricular valve anomalies and rhythm disorders.

Criss-cross con discordanciaauriculoventricular, acabalgamiento de la válvula auriculoventricular derecha, doble salida del ventrículo derecho y preexcitación / Criss-cross heart with atrioventricular discordance, straddling A-V valve, double outlet right ventricle and preexcitation

Se presenta un recién nacido con cianosis, disnea y taquicardia supraventricular por reentrada con buena respuesta al tratamiento. El diagnóstico de la cardiopatía se realizó por ecocardiografía. El paciente falleció súbitamente a los 6 días. El estudio anatómico confirmó la anomalía: corazón en criss-cross con discordancia auriculoventricular (AV), válvula derecha acabalgante, válvula AV izquierda estenótica en conexión con el ventrículo trabeculado anterior del que emergían los dos vasos, aorta y pulmonar. La rotación precoz cuando la septación no ha concluido favorece las anomalías de las válvulas AV y las alteraciones del ritmo (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

No disponible

[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.