[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.

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

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[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)

[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.

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)