Fernando Benito

Discrete subaortic stenosis in adults: increased prevalence and slow rate of progression of the obstruction and aortic regurgitation☆

OBJECTIVES We sought to determine the prevalence and rate of progression of left ventricular outflow tract obstruction (LVOTO) and aortic regurgitation (AR) in adults with discrete subaortic stenosis (DSS). BACKGROUND Discrete subaortic stenosis is an uncommon form of LVOTO, with rapid hemodynamic progression in children, but the prevalence and rate of progression in adults have not been studied so far. METHODS The prevalence of DSS was determined in 2,057 consecutive adults diagnosed with congenital heart disease (CHD). The relationship between LVOTO on Doppler echocardiography and patient age was analyzed. Sequential changes in LVOTO and AR were determined for patients with two or more Doppler echocardiograms obtained with at least a two-year interval. RESULTS A total of 134 adults (mean age 31 +/- 17 years) were diagnosed with DSS. The prevalence was 6.5% for all adults with CHD. Sixty patients (44%) had other associated CHD. The mean age of 29 patients who had undergone an operation for DSS during their adult life (56 +/- 15 years) was significantly higher than that of 64 patients (27 +/- 13 years) who had not required a surgical intervention (p < 0.0001). A significant relationship between LVOTO and patient age (r = 0.61, p < 0.0001) was found: 21 +/- 16 mm Hg in patients <25 years old, 51 +/- 47 mm Hg for those between 25 and 50 years old, and 78 +/- 36 mm Hg for those >50 years old. The LVOTO increased from 39.2 +/- 28 to 46.8 +/- 34 mm Hg (p = 0.01) during a mean follow-up of 4.8 +/- 1.8 years in 25 patients. The slope of the change in LVOTO was 2.25 +/- 4.7 mm Hg per year of follow-up. Aortic regurgitation was detected by color Doppler imaging in 109 patients (81%), but it was hemodynamically significant in <20%. An increase in the mean degree of AR over time was not significant (baseline: 1.3 +/- 0.8; follow-up: 1.5 +/- 0.9; p = 0.096). CONCLUSIONS The prevalence of DSS is increasing in adults due to the greater number of repaired CHDs that develop into evolutive DSS. In contrast to infants and children, adults with DSS show a slow rate of LVOTO progression. Aortic regurgitation is a common but usually mild and nonprogressive consequence. The current indications for surgical intervention should be revised.

Predisposing conditions for atrial fibrillation in atrial septal defect with and without operative closure

The aims of this study were to determine the prevalence and predisposing conditions for atrial fibrillation (AF) in adults with atrial septal defect (ASD) and to evaluate the influence of age at surgical repair. The study population consisted of 286 adults with ASD (mean age 39.5 +/- 19 years). All patients had >or = 1 follow-up visit and a Doppler echocardiographic study. One hundred ninety-two of the patients underwent surgical closure 1 to 34 years before the study. Analyzed variables were entered into univariate (Mann-Whitney U) and multivariate (stepwise logistic regression) models to assess independent predictors for AF. The prevalence of AF was similar in surgically treated patients (15.6%) and in the nonsurgical group (13.8%) (p = 0.69). Multivariate analysis showed that current age (RR 1.9 per each decade of age, 95% confidence interval [CI] 1.3 to 2.7, p = 0.001), mitral regurgitation (RR 3.0 per each degree of regurgitation, 95% CI 1.6 to 5.8, p = 0.001), left atrial enlargement (RR 2.8 per each 10 mm increase in size, 95% CI 1.5 to 5.2, p = 0.001), and tricuspid regurgitation (RR 1.9 per each degree of regurgitation, 95% CI 1.0 to 3.7, p = 0.04) were independent predictors of AF; however, gender, anatomic type, defect size, Qp:Qs, pulmonary artery pressure, right ventricular dimension, left ventricular shortening fraction, and prior surgical repair were not related to late AF development. In the surgical group, age >25 years at the time of surgery was the only predictor for AF independent of age at the time of the study (p = 0.02).

Rapid progression of midventricular obstruction in adults with double-chambered right ventricle.

OBJECTIVE The purpose of this study was to determine the rate of progression of midventricular obstruction in adolescents and adults with double-chambered right ventricle. METHODS Clinical and echocardiographic findings in 45 patients (mean age 26 +/- 6 years, range 15-44) diagnosed with double-chambered right ventricle were retrospectively analyzed. Twenty patients underwent surgical repair before the age of 15 years. The relationship between Doppler midventricular pressure gradient and patient age was analyzed in 25 patients without previous repair. Sequential change in midventricular obstruction was determined for patients with 2 or more Doppler echocardiographic examinations performed within at least a 2-year interval. RESULTS Right midventricular pressure gradient in nonrepaired patients was 70 +/- 38 mm Hg (range 25-150). A significant relationship between midventricular obstruction and patient age (r = 0.64, P <.001) was found. Midventricular pressure gradient at initial evaluation was 32 +/- 27 mm Hg in 16 patients < 25 years and 73 +/- 45 mm Hg in 9 patients >/= 25 years (P <.03). After the initial study, 5 patients underwent surgical repair and 13 patients without repair were followed up for a period of 6.1 +/- 2.7 years (range 2-9), in which midventricular pressure gradient increased from 32 +/- 26 mm Hg to 67 +/- 35 mm Hg (P <.001). The slope of the change in midventricular pressure gradient was 6.2 +/- 3 mm Hg per year of follow-up. Seven more patients underwent surgical repair during follow-up due to progression of the obstruction. There was no mortality nor residual midventricular obstruction in surgically repaired patients. CONCLUSIONS Mild right midventricular obstruction shows a fast rate of progression in adolescents and young adults. Thus, close clinical and echocardiographic follow-up is advised, and surgical repair should be considered if significant progression of obstruction is detected.

Novedades en cardiología pediátrica, cardiopatías congénitas del adulto y cirugía cardiaca de cardiopatías congénitas

La cardiologia pediatrica es una disciplina en continua evolucion, que abarca no solo las cardiopatias congenitas y adquiridas en la infancia, sino tambien al adulto afecto de cardiopatias congenitas y el diagnostico y la prevencion de las cardiopatias en la epoca prenatal. Destacamos novedades en el campo de la genetica y resaltamos algunos articulos de diagnostico por resonancia magnetica o tomografia multicorte, asi como nuevas publicaciones en el campo de la electrofisiologia y en el tratamiento quirurgico en el nino y en el adulto con cardiopatia congenita. Tambien abordamos el campo cada vez mas avanzado de la asistencia ventricular mecanica como puente al trasplante en ninos

Taquicardia ventricular inducible en un paciente con síncope recurrente tras operación de Senning

Paciente de 17 anos con transposicion de las grandes arterias operado con tecnica de Senning, que presento, 2 meses antes de su ingreso, dos episodios sincopales, uno de los cuales preciso reanimacion cardiopulmonar basica. En el Holter presentaba extrasistoles ventriculares politopicos aislados y en parejas, induciendose en el estudio electrofisiologico una taquicardia ventricular polimorfica que, tras procainamida intravenosa, paso a taquicardia ventricular monomorfica sostenida. Se implanto un desfibrilador automatico por via endovenosa y, tras 12 meses de seguimiento, ha presentado un episodio de taquicardia ventricular polimorfica tratada mediante una descarga del desfibrilador.

Cierre quirúrgico de la comunicación interauricular antes o después de los 25 años de edad. Comparación con la evolución natural en pacientes no operados

Introduccion El cierre quirurgico de la comunicacion interauricular (CIA) antes de los 25 anos de edad disminuye las complicaciones durante la vida adulta, pero las consecuencias de la intervencion en pacientes mayores de 25 anos siguen siendo motivo de controversia. Metodos Para comparar los efectos de la cirugia precoz y/o tardia con la evolucion natural se ha estudiado, de forma retrospectiva, a 280 adultos (edad media 40 ± 18 anos) con CIA no restrictiva. Ciento dos pacientes (grupo 1) habian sido operados antes de los 25 anos, 90 (grupo 2) habian sido operados despues de los 25 anos y 88 (grupo 3) no habian sido operados previamente. Se comparo la presion pulmonar sistolica, el tamano del ventriculo derecho y la auricula izquierda, el grado de insuficiencia mitral y tricuspide, la funcion sistolica del ventriculo izquierdo y la prevalencia de fibrilacion auricular. Resultados No habia diferencia en la funcion sistolica del ventriculo izquierdo o en el grado de insuficiencia mitral entre los tres grupos. Comparado con el grupo 2, el grupo 1 tenia menores presion sistolica pulmonar (p Conclusiones El cierre quirurgico de la CIA despues de los 25 anos no previene el deterioro hemodinamico o el desarrollo de arritmias auriculares, por lo que se deberia concentrar esfuerzos en corregir el defecto antes de la edad adulta.

Implantación de stent en la obstrucción al drenaje venoso sistémico tras la cirugía tipo Mustard en el adulto

La obstruccion tardia al drenaje venoso sistemicoen pacientes con transposicion de las grandesarterias intervenidos mediante tecnica de Mustardes una complicacion bien conocida. Los stents intravascularesexpandibles mediante cateter-balonhan sido ya utilizados para aliviar dicha estenosisen ninos. Describimos la implantacion de un stent en una paciente adulta intervenida mediante dichatecnica, con obstruccion sintomatica de la venacava superior y dehiscencia del parche. La pacientehabia sido rechazada para cirugia debido a disfuncionventricular derecha. Dieciocho meses despuesdel procedimiento la mejoria clinica persiste.

Dilatación con stent de la estenosis de la arteria pulmonar en el adulto con cardiopatía congénita

Las endoprotesis tipo stent han sido eficazmente utilizadas para resolver estenosis de arterias pulmonares en ninos En 3 pacientes con una edad media de 22,7 + 4,7 anos, con estenosis de las arterias pulmonares secundarias a cirugia paliativa se implantaron 7 stents, en 4 procedimientos; seis fueron del tipo P308 de Palmaz y se colocaron por via venosa femoral, cuatro en el primer caso, 2 solapados en serie en cada rama y otros dos, tambien en serie, en el paciente 3, en la arteria pulmonar izquierda. En el caso 2 se implanto un stent del tipo NIR en la arteria pulmonar derecha por la arteria femoral. El diametro de la estenosis se incremento de 5,3 ± 2,3 a 14,4 ± 4,2 mm y el gradiente a su traves se redujo de 40,6 ± 15,3 a 6,5 ± 5 mmHg. No hubo complicaciones. Tras un seguimiento de 30,6 ± 6,1 meses, los stents se mantienen permeables y dos pacientes previamente inoperables estan pendientes de correccion total. Stents have been previously used to resolve stenoses of branch pulmonary arteries in children We report 3 patients, with mean age of 22.7 ± 4.7 years and pulmonary artery stenosis after palliative surgery in whom we implanted seven stents in four procedures. Six P308 Palmaz, overlapped two by two, were implanted by venous femoral approach in two patients, receiving four in the first case and the other two in the third case. In the second case, a NIR type stent was implanted through femoral artery in the right pulmonary artery. Stenosis diameter enlarged from 5.3 ± 2.3 to 14.4 ± 4.2 mm and the pressure gradient through stenosis fell from 40.6 ± 15.3 to 6.5 ± 5 mmHg. All stents are well deployed and there are two patients waiting for total correction (previously not feasible) during a follow-up of 30.6 ± 6.1 months.

Tratamiento de las taquicardias pediátricas mediante ablación con catéter y radiofrecuencia

La practica del EEF diagnostico requiere la introduccion de 3 a 4 electrocateteres a traves de las venas femorales y la vena braquial izquierda o yugular derecha, que se situan, bajo control radiologico, en la auricula derecha alta (ADA), haz de His (HIS), apex del ventriculo derecho (AVD) y seno coronario (SC). Para la ablacion de las vias accesorias localizadas en el surco auriculoventricular (AV) izquierdo se utiliza un abordaje retrogrado a traves de la arteria femoral o transeptal por puncion del tabique interauricular. La ablacion de las vias derechas y la mayoria de las septales se realiza por via venosa, desde la vena cava inferior o superior.

Dilatación con balón de una estenosis adquirida del seno coronario

A un nino de 4 anos de edad con taquicardia suprave n t ricular incesante tipo C o u m e l re f ra c t a ria al trat amiento antiarritmico se le practico ablacion mediante ra d i o f recuencia. Se progra m a ron 60 °C durante 60 s. Se pre c i s a ron 3 aplicaciones alrededor del o s t i u m d e l seno coro n a rio para conseguir la ablacion de una via a c c e s o ria postero s eptal dere cha. Ocho meses despues se practico un segundo procedimiento por re c u rre n c i a de la taquicardia. Ante la imposibilidad de intro d u c i r el cateter de ablacion en el interior del seno coro n aFig. 1. A: Angiografia retrograda del seno coronario (CS) en la proyeccion oblicua anterior izquierda en la que se observa estenosis severa del ostium (flecha) con dilatacion postestenotica. B: tras la dilatacion con balon se consiguio una buena apertura del ostium, con paso amplio a la auricula derecha (RA) y desaparicion de la dilatacion del CS. IM A G E N E S E N C A R D I O L O G I A