[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

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[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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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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[Guidelines of clinical practice of the Spanish Society of Cardiology. Requirements and equipment of invasive techniques in pediatric cardiology: clinical application]. / Guías de actuación clínica de la Sociedad Española de Cardiología. Requerimientos y equipamiento de las técnicas invasivas en cardiología pediátrica: aplicación clínica.

Invasive techniques in pediatric cardiology have experienced a big change since the 80's. The growth of non-invasive methods for diagnosing congenital heart defects has made the number of diagnostic catheterizations decrease remarkably. On the other hand, the notable development of pediatric interventional catheterization techniques will allow that, in the near future, the number of therapeutic catheterizations overcomes the diagnostic ones in our country. The former are more difficult and dangerous, so they require experienced and skilled hands and more economic resources. This chapter is divided in three main sections: I) Requirements and equipment needed for pediatric invasive techniques; II) Current indications, contraindications and complications of the diagnostic catheterization, and III) Techniques, indications and results of pediatric therapeutic catheterization: current state. Likewise, we state the suitability or not for these therapeutic procedures in different cardiac anomalies.

[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.

[Interventricular communication in the neonatal period]. / Comunicación interventricular en la edad neonatal.

OBJECTIVE: The aim of this study was to evaluate the prevalence and the follow-up in the long run of ventricular septal defect in the neonatal period. MATERIAL AND METHODS: 222 patients with ventricular septal defect from a total amount of 30,840 newborns admitted to our Hospital were evaluated between January 1991 and December 1996 (The incidence accounts for 7.19 per 1,000 newborns). 123 of these patients were males (55.4%) and 99 females (44.6%). The mean age at the time of examination was 3.09 +/- 1.67 days (range from 1 to 11 days). Diagnosis was suspected because of a harsh systolic murmur in 207 cases (93.2%), a malformative syndrome in 10 (4.5%) or polypnea in 5 (2.3%). The study was performed with a Sonos 1000 Echocardiograph device. The mean follow-up period ranged from 1 to 72 months. 10 patients underwent cardiac catheterization (4.5%). RESULTS: A muscular defect was the common finding in 177 patients (79.7%) whereas 41 patients (18.5%) exhibited a perimembranous defect. The mean size defect was 2.9 +/- 1.2 mm (range 1-10 mm), being smaller in the muscular defects (2.56 +/- 0.68) than in the perimembranous defects (4.33 +/- 1.56) p < 0.001. Spontaneous closure occurred in 176 ventricular septal defects (79.3%), 155 of them corresponding to muscular defects (87.6%) and 18 to perimembranous defects (43.9%). The mean age at the time of spontaneous closure was 9.5 +/- 8.9 months (9.4 +/- 9 months in the muscular defects and 10.3 +/- 4 months in the perimembranous defects). Spontaneous closure occurred by age 6 months in 52% of the patients with ventricular septal defect (54.5% muscular defects, 33.3% perimembranous defects), 12 months in 72.7% (71.2% muscular defects and 83.3% perimembranous defects) and 24 months in 96% (96.1% muscular defects and 94.4% perimembranous defects). 10 patients underwent surgical repair by age 11 +/- 8 months. All of them exhibited perimembranous defects with a mean size 6 +/- 1.5 mm which were tantamount to 24.4% of this group. 3 patients died (1.4%), 2 suffering from trisomy 18 and 1 after cardiac surgical repair. CONCLUSION: The most common ventricular septal defects in the neonatal period occur in the region of the muscular septum. The prognosis is favourable. Most of the patients experience spontaneous closure and show a very low mortality rate which is basically related to extracardiac anomalies.

[Magnetic resonance of congenital cardiopathies]. / Resonancia magnética en las cardiopatías congénitas.

OBJECTIVE: The objective of this study was to determine the usefulness of magnetic resonance in the diagnosis and follow-up in congenital heart disease. PATIENTS AND METHODS: Between 1992 and 1994, we have evaluated 250 patients (between 1 day-22 years of age) with congenital heart disease. We used a Philips-Gyroscan 0.5 Tesla set-up and the studies were made with ECG-gated multislice sequences with the time of repetition adapted to the cardiac frequency. The slice thicknesses were 3-10 mm and the field of view was 100-400 mm. Three axes were used: axial, oblique coronal and 30-60 degrees oblique sagittal. Three groups were made: A) Patients in whom MR imaging was the elected technique for establishment of the diagnosis; B) Patients were MR imaging was an alternative technique; and C) Patients were MR imaging was complementary to other techniques. All patients underwent a Doppler echocardiographic study or an angiocardiogram. RESULTS: Group A) One hundred and fifty-one patients were included with the following conditions: Eighty-two with coarctation of the aorta or aortic arch interruption, 12 with vascular ring, 5 with truncus arteriosus in order to evaluate the pulmonary branches, 28 cases operated for Fallot or transposition of the great vessels for the evaluation of the pulmonary branches, 16 with single ventricle for the evaluation of the bulboventricular foramen, 5 with complex congenital heart disease and 3 with pericardial agenesia-herniation. Group B) Sixty-five patients were included in this group with the following diagnoses: Ten with aorto-pulmonary colateral arteries, 13 with anomalous pulmonary drainage of the systemic veins, 38 with tronco-conal anomalies and 4 with supravalvular aortic stenosis. Group C) Thirty-four patients with the following conditions were included in this group: Ten with common atrioventricular septal defects, 6 with tricuspid atresia, 6 with atrioventricular and ventriculoarterial discordance and 12 with other anomalies. CONCLUSIONS: MR imaging can replace other techniques in the diagnosis and follow-up of some congenital heart diseases and in a non-invasive way.

[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.