Valvuloplastia aórtica durante la edad pediátrica: experiencia de 15 años / Aortic valvoloplasty in childhood: a fifteen year experience

Introducción: Previamente al inicio de la intervención quirúrgica, los pacientes con estenosis aórtica grave fallecían de manera precoz. En la década de los ochenta, se introduce el tratamiento no quirúrgico: la valvuloplastia percutánea. En general, la valvuloplastia aórtica con balón reduce el gradiente un 60 por ciento desde valores previos, sin incremento importante de la insuficiencia aórtica. Pocos estudios consiguen reunir en un mismo trabajo un número importante de casos y un seguimiento a largo plazo (15 años) para establecer variables pronósticas. Objetivos: Valorar los resultados inmediatos obtenidos mediante la valvuloplastia aórtica con balón en un grupo de edad pediátrica; la evolución ecocardiográfica a largo plazo, identificando variables pronósticas dela evolución; e identificar el grado de concordancia entre los estudios hemodinámicos y ecocardiográficos. Pacientes y métodos: Analizamos los resultados obtenidos en 75 procedimientos realizados en una población compuesta por 55 varones y 20: mujeres (2,75:1): con edades comprendidas entre 2,8 y 210 meses (90,93 ñ 64,25 meses). Se excluyó el grupo neonatal por tener una entidad propia diferencial. Se estudiaron e intervinieron todos ellos en el Servicio de Cardiología Pediátrica del Hospital "Ramón y Cajal" de Madrid, entre julio de 1986 y junio de 2001. El tiempo de seguimiento varió entre 0 y 180 meses (41,10 ñ 32,96 meses). Resultados: Los efectos inmediatos observados mediante estudio ecocardiográfico fueron un descenso significativo en el gradiente Doppler máximo (de 83,67 a 48,59 mmHg, p <0,0001) y en el gradiente Doppler medio (de 43,49 a 32,18 mmHg, p <0,0014), con incremento de la insuficiencia aórtica por encima de dos grados en sólo un 8 por ciento de los casos. Los estudios hemodinámicos, por su parte, reflejaron un descenso significativo de la presión sistólica del ventrículo izquierdo (de 153,89 a 123,90 mmHg, p <0,0001) y de la telediastólica (de 13,30 a 11,70 mmHg, p 4,039), con un aumento significativo también de las presiones aórticas sistólica, diastólica y media. El gradiente pico hemodinámico descendió de 71,93 a 30,37 mmHg, p <0,0001. La insuficiencia aórtica sólo se incrementó de manera significativa en un 5,3 por ciento. Durante el seguimiento ecocardiográfico, se demostró un aumento significativo del tamaño telediastólico del ventrículo izquierdo (VIDD), así como una reducción del gradiente Doppler máximo y medio. Las comparaciones entre los resultados ecocardiográficos y hemodinámicos fueron perfectamente equiparables en la valoración del tamaño del anillo aórtico y en la reducción del gradiente, pero no en el estudio del gradiente transvalvular. El análisis de supervivencia libre de intervención sobre la válvula fue del 90,88 por ciento a los 12 meses y del 78,65 por ciento a los 63 meses. El análisis de riesgos proporcionales mostró que el gradiente posvalvuloplastia resultante de la intervención se manifestaba como un factor pronóstico de posteriores intervenciones sobre la válvula. Conclusiones: La valvuloplastia aórtica con balón es un procedimiento efectivo tanto inmediatamente después de la intervención como a largo plazo, manteniéndose los resultados durante el tiempo de seguimiento. El porcentaje de éxito inicial se cifra en un 70 por ciento y, en la evolución, más del 90 por ciento de los niños queda libre de reintervención sobre la válvula a los 12 meses y casi un 80 por ciento a los 5 años. El gradiente posvalvuloplastia puede ser un buen factor pronóstico de futuras reintervenciones necesarias sobre la válvula. Las complicaciones obtenidas con este procedimiento han sido escasas, con tendencia a disminuir con la mayor experiencia técnica de los grupos y la mejor elección de los catéteres (AU)

[Non-surgical treatment of aortic coarctation and recoarctation]. / Tratamiento no quirúrgico de la coartación y recoartación de aorta.

OBJECTIVES: To analyze the results of nonsurgical treatment of aortic coarctation and recoarctation by evaluating the results of each technique, and its complications and outcome. PATIENTS AND METHODS: The results were as follows: 51 children underwent balloon dilatation due to recoarctation (86.5 %); two underwent dilation of a native coarctation (3.3 %) and six underwent stent implantation for recoarctation (10 %). Age ranged from 2 to 236 months (109 +/- 63.45 months) with a follow-up of between 1 and 156 months (38.87 +/-32.96 months). RESULTS: The mean predilatation gradient in children with recoarctation was 34 +/- 11.62 mmHg, which decreased to 11 +/- 5.38 mm Hg (p < 0.0001). In 12 patients (20.3 %) effective dilatation was not achieved. The size of the stenosis was 6.7 +/- 2.35 mm predilatation, which increased to 9.3 +/- 3.10 mm (p < 0.0001) after dilatation with a percentage increase of 50.97. There were very few complications. Six children required subsequent redilatation. The experience with stent showed a mean gradient of 32.83 +/- 10.62 mm Hg, which decreased to 7.3 +/- 3.8 mm Hg (p < 0.0001) with a balloon/stenosis ratio of 1.94. CONCLUSIONS: We conclude that the interventionist technique is highly effective in both native coarctation and recoarctation in the short term, as well as subsequently, with very few complications. The stent technique produces equally good results in older children, which is promising for the future.

Corazones univentriculares. Treinta años después* / Univentricular hearts. Thirty years later

No disponible

[Balloon valvoplasty for critical aortic valve stenosis in neonates]. / La valvuloplastia en la estenosis valvular aórtica crítica del neonato.

OBJECTIVES: To evaluate the immediate results obtained with balloon aortic valvuloplasty in neonates and long-term echocardiographic outcome as well as to identify variables predictive for outcome. To identify the degree of agreement between hemodynamic and echocardiographic study. PATIENTS AND METHODS: We analyzed the results obtained in 26 procedures performed in 18 boys and 8 girls (2.25:1), aged 2 to 49 days (16.1 12.9 days). All procedures were performed in the Pediatric Cardiology Unit of Ramón y Cajal Hospital in Madrid between June 1989 and June 2001. Follow-up was from 0 to 144 months (39.5 39.7 months). RESULTS: The immediate effects observed through echocardiographic study were a significant decrease in the maximum Doppler gradient (from 77.8 to 32.4 mm Hg; p < 0.0001) and in the medium Doppler gradient (from 41.7 to 18.5 mm Hg; p < 0.05). Hemodynamic studies showed a significant decrease in left ventricular systolic pressure (from 119.8 to 82.8 mm Hg; p < 0.0001) and an increase in aortic systolic pressure (from 56.8 to 66.6 mm Hg; p < 0.007). The hemodynamic peak gradient decreased from 63.1 to 17.7 mm Hg; p < 0.0001. In 23 % of the patients, aortic insufficiency significantly increased. Echocardiographic follow-up showed a significant increase in the telediastolic size of the left ventricle and a decrease in the maximum and medium Doppler gradient. The procedure showed initial success in 68.7 % and analysis of survival free of valvular surgery was 65.8 % at 45 months. Proportional risk analysis revealed that the post-valvuloplasty gradient was a predictive factor for future valvular surgery.

La valvuloplastia en la estenosis valvular aórtica crítica del neonato / Balloon valvoplasty for critical aortic valve stenosis in neonates

Objetivos: Evaluar los resultados inmediatos obtenidos mediante valvuloplastia aórtica con balón en el período neonatal, así como la evolución ecocardiográfica a largo plazo, identificando variables predictivas en relación a la evolución. Identificar el grado de concordancia entre los estudios hemodinámicos y ecocardiográficos. Pacientes y métodos: Se analizaron los resultados obtenidos en 26 procedimientos realizados en una población compuesta por 18 varones y 8 mujeres (2,25:1) cuyas edades fueron de 2 a 49 días (16,1 12,9 días). Todos fueron estudiados e intervenidos en el Servicio de Cardiología Pediátrica del Hospital Ramón y Cajal de Madrid, entre junio de 1989 y junio de 2001. El tiempo de seguimiento osciló entre 0 y 144 meses (39,5 39,7 meses). Resultados: Los efectos inmediatos observados mediante el estudio ecocardiográfico fueron un descenso significativo en el gradiente Doppler máximo (de 77,8 a 32,4 mmHg; p < 0,0001) y del gradiente Doppler medio (de 41,7 a 18,5 mmHg, p < 0,05). Los estudios hemodinámicos reflejaron un descenso significativo de la presión sistólica del ventrículo izquierdo (de 119,8 a 82,8 mmHg, p < 0,0001) y un aumento de la presión sistólica aórtica (de 56,8 a 66,6 mmHg, p < 0,007). El gradiente pico hemodinámico descendió de 63,1 a 17,7 mmHg, p < 0,0001). La insuficiencia aórtica se incrementó de manera significativa en el 23% de los casos. El seguimiento ecocardiográfico demostró un aumento significativo del tamaño telediastólico del ventrículo izquierdo (VIDD) y una reducción del gradiente Doppler máximo y medio. El porcentaje de éxito inicial fue del 68,7% y el análisis de supervivencia libre de intervención sobre la válvula se mostró del 65,8% a los 45 meses. El análisis de riesgos proporcionales demostró que el gradiente posvalvuloplastia resultante de la intervención era un factor predictivo de posteriores intervenciones sobre la válvula (AU)

Contribution of Doppler atrioventricular flow waves to ventricular filling in the human fetus.

The normal fetal flow velocity profile across the atrioventricular valves is characterized by an early peak (E), which is related to preload and to active ventricular muscle relaxation, and a higher late peak (A), which is caused by the atrial contraction and also influenced by ventricular compliance. The purpose of this study was to determine how these two elements of ventricular filling change during gestation in both ventricles. A total of 485 normal fetuses from 17 weeks to term were examined by Doppler echocardiography. We measured E and A peak velocities and E/A ratio for both mitral and tricuspid valves. Simple regression analysis was applied to assess possible correlation between Doppler variables and gestational age. Moreover, E and A peak velocities were compared using paired Student's t-test. With the advance of gestation a significant linear increase in the E wave and E/A ratio was found for both mitral and tricuspid valves. The A wave shows little change throughout pregnancy. We found significantly higher Doppler velocities for the tricuspid valve than for the mitral valve. The relationship between the E/A ratios for the two valves and gestational age diverge slightly, with higher values for the mitral E/A ratio. This study shows that the A wave velocity remains constant throughout gestation, suggesting little or no change in ventricular compliance. The E wave is mainly responsible for the change in E/A ratio for both atrioventricular valves during gestation. These findings suggest progressive enhancement of relaxation and elastic recoil, an increase in preload, or both, throughout gestation, rather than a change in myocardial compliance as an explanation for the observed increase in the E/A ratio.

[Aortic-left ventricular tunnel. Long-term surgical results]. / Túnel aorta-ventrículo izquierdo. Resultados quirúrgicos a largo plazo.

OBJECTIVE: The aim of this work is to describe the different aspects of diagnosis and postoperative follow-up in four patients after surgery for aortic-left ventricular tunnel (ALVT). The surgical techniques and results have been published, in the literature but there are no reports about the progression of aortic regurgitation. METHOD: We reviewed the clinical features of four patients operated with ALVT. The clinical information (electrocar-diograms, echocardiograms and catheterilution) was studied in the preoperative period. Surgical technique and postoperative evolution were analysed. The follow-up period ranged between 4.7 and 13 years. RESULTS: In a 14 years period, four children (2 boys and 2 girls), were operated for ALVT. The mean age at the time of diagnosis was 8.5 years (ranged between 1 month and 14 years). A continuous murmurs was present in all cases, associated to cardiac failure in the youngest patient (1 month old). The clinical diagnosis was ALVT in the four cases but only in two the echocardiographic study showed correctly the anatomic aspects of the malformation. The angiohemodynamic study was correct in three patients and the differential diagnosis was made in last one with rupture of aortic sinuses of Valsalva to the left ventricle. All cases were operated; with direct closure of the aortic orifice (two cases), or with dacron patch (two cases). In one case aortic valve replacement was also performed. There was not mortality and the mean age at the time of surgery was 9 years (1.5 months and 14 years). The mean follow-up was 8.5 years (ranged between 4.7 and 13 years), three patients showed clinically and in the echocardiogram study an aortic valvular regurgitation without ALVT deshiscence. All cases are asymptomatic. No patient was reoperated. CONCLUSION: The surgical treatment of ALVT must be done as soon as the diagnosis is made to prevent left ventricular enlargement, ventriculo-aortic floor distortion and aortic valvular lesion. A precise echocardiographic study is necessary to avoid catheterization. This study provided a clear knowledge of anatomic structure of the ALVT for both clinician and surgeons. This information is important in order to obtain an optimal surgical success, particularly to prevent the valvar aortic regurgitation.

[Heart growth in the normal human fetus. A two-dimensional echocardiographic study]. / Crecimiento cardíaco en el feto humano normal. Estudio mediante ecocardiografía bidimensional.

INTRODUCTION: Fetal cardiac growth was studied by two-dimensional echocardiography. The size of various cardiac structures were compared with gestational age. Normal values for each measurement were determined, with special attention being paid to the differences between the right and left chambers. PATIENTS AND METHODS: Ninety human fetuses with a gestational ages between 17 and 39 weeks were studied. Any structural abnormality was excluded at prenatal and postnatal examination. Aortic, pulmonary artery, tricuspid and mitral valve annulus diameters were measured with classical echocardiographic views. These variables were compared between them and plotted against gestational age. The correlations were determined by Student's T-test and regression analysis using 95% confidence limits. RESULTS: A good correlation (r > 0,7) between all the measurements and gestational age was obtained. In absolute values, tricuspid and pulmonary artery valve diameters were significantly greater (p < 0,01) than mitral and aortic valve diameters, respectively. Nevertheless, the diastolic diameter of the left ventricle (LV) was slightly greater than the diastolic diameter of the right ventricle (RV). Separating the data of the second trimester from of those of the third, the initial dominance of LV (RV/LV ratio = 0,93) tends to disappear and a slight dominance of RV (RV/LV ratio = 1,03) was observed at term. CONCLUSION: This study establishes normal values based on two-dimensional echocardiographic cardiac measurements throughout gestational stages. The data obtained will be very useful for the prenatal diagnosis and perinatal management of certain cardiopathies like the hypoplastic left heart syndrome. The relative dominance of RV in utero was also demonstrated. The different morphology of both ventricular outflow tracts and their volume changes throughout gestation, secondary to placental resistance variation, could justify the changes in the RV/LV ratio during the intrauterine period.

[Redundancy of the interatrial septum without associated congenital cardiopathy. Its prenatal echocardiographic diagnosis and follow-up]. / Redundancia del septo interatrial sin cardiopatía congénita asociada. Diagnóstico ecocardiográfico prenatal y seguimiento.

INTRODUCTION AND OBJECTIVE: Aneurysm of the atrial septum at the level of the foramen ovale is reported commonly at the routine fetal echocardiographic examination. Cases with entire involvement of the atrial septum have been exceptionally reported. We described the data concerning the prenatal detection of a total redundancy of the atrial septum in 6 cases without congenital heart disease. METHODS: The gestational age was between 28 and 38 weeks, and none of the fetuses had evidence of heart defect. A routine fetal echocardiography was made in all the cases. Rhythm disturbances were studied by M mode. During the first three months of life, a cardiologic control was made in all the cases. RESULTS: The large displacement of the atrial septum towards the left atrium was clearly visualized in four-chambers view; by using Pulsed Doppler and Color flow mapping, the pulmonary venous return and mitral orifice flow were not impaired. Premature atrial beats were found in two fetuses and no hemodynamic disfunction was observed in all entire study group. Postnatal echocardiographic control was normal in all patients. The atrial septum becomes completely rectified with normal patency of the foramen ovale membrane in the newborn studies. The supraventricular extrasystoles were confirmed in the neonatal period. In both, the arrhythmia disappeared in a few days after delivery without treatment. CONCLUSIONS: Despite the benign follow-up in our cases, the unexpected presence of an extremely redundant atrial septum, leads to a close prenatal attention particularly in the presence of rhythm disturbance. The pathologic substrate of this anomaly might be an intrinsic alteration of the myocardial structure of the septum like seems demonstrated in the aneurysm circumscribed to area of the fossa ovalis [correction of fosa ovale]. The increase in the left atrial pressure after birth will produce a normal 2-D echo patterns in the newborn period.

[Aortic hypoplasia in a case of single ventricle with normally related vessels]. / Hipoplasia aórtica en un caso de ventrículo único con vasos normalmente relacionados.

In cases with double inlet left ventricle when there exists a hypoplasia of one of the great arteries, the affected vessel arises from the accessory right ventricular chamber. This is due in general to the presence of a restrictive bulbo-ventricular foramen that results in hypoplasic vessel. The case we present in this paper has unusual anatomic morphology: the hypoplasic vessel (the aorta) arises from the main ventricular chamber. We analyse the electrocardiographic findings useful for the differential diagnosis from cases of isolated aortic coarctation of hypoplasia. We review also the intraventricular anatomic malformations that may cause the presence of a hypoplasic vessel. Even if, our patient died in the neonatal period, the adequate surgical procedure for this case is reviewed.

[Congenital familial cardiopathies. Prenatal diagnosis]. / Presentación familiar de las cardiopatías congénitas. Diagnóstico prenatal.

The diagnosis of congenital heart disease by means of echocardiography is already a real fact. The use of this technique for diagnosis in utero currently allows the detection of these malformations during the early stages of pregnancy and to determine the prognosis of the fetal cardiopathies, as well as to establish a medical or surgical treatment of the neonate. The prenatal echocardiographic study of 126 pregnant women, previously having had children with heart disease, has allowed s to prove the greater incidence of cardiopathy in this population (6.4%), compared to the results obtained from a group of pregnant women previously having normal children and without a high risk pregnancy (0.8%). These findings support the theory that genetic factors are the primary cause of congenital cardiac malformations, although the existence of teratogen factors (rubella, diabetes, etc.) in some cases cannot be excluded.

[Two-dimensional and color-coded Doppler echocardiography in the diagnosis of infradiaphragmatic total anomalous pulmonary venous connection (ITAPVC) to the portal venous system]. / La ecocardiografía bidimensional y Doppler codificado en color en el diagnóstico del drenaje venoso pulmonar anómalo total infradiafragmático (DVPATI) al sistema venoso porta.

We have studied by means of 2D echocardiogram and pulsed, continuous and codified colour Doppler, 3 newborns with a total anomalous infradiaphragmatic pulmonary venous connection to the portal vein. In the right subcostal projection we have observed the abnormal venous conduit crossing the diaphragm going to an enlarged portal system. The codified colour and the pulsed Doppler showed the venous characteristics and the abnormal direction of the flow. With these techniques it was also possible to detect the anatomic and functional intracardiac abnormalities. We consider these techniques a reliable method to make a surgical correction without the practise of any other invasive diagnostic method.

Discrete subaortic stenosis (DSS) in childhood: a congenital or acquired disease? Follow-up in 65 patients.

Discrete subaortic stenosis (DSS) is a frequent malformation easily diagnosed by echocardiography; surgical resection of the membrane is the most suitable treatment. However, some evolutive aspects of the lesion remain unexplained (the presence of aortic dysfunction, associated malformations, etc.). With a view to analyse these aspects, we have studied 65 patients with DSS and divided them into two groups: Group I, 37 patients without surgery, and Group II, 28 patients treated by resection of the membrane. During follow-up we observed: (a) development of aortic regurgitation in operated and non-operated cases; (b) frequent association of DSS with closed or closing ventricular septal defect; (c) the absence of DSS in neonates and (d) the progressive growth of the membrane. These aspects give this malformation an evolutive character that requires observation and management even after resection of the DSS.

[Aortico-left ventricular tunnel. Presentation of 2 new cases. Usefulness of non-invasive study]. / Túnel aorta-ventrículo izquierdo. Presentación de dos nuevos casos. Utilidad del estudio no invasivo.

In the last years the anatomic characteristics of the left aorto-ventricular tunnel and the clinical aspects of this uncommon malformation have been well described. Its evolution usually fatal without surgical procedure is in great contrast with the slight symptoms encountered by us in two new cases. This particular finding joined to the specific aspects found in the noninvasive color Doppler study, have lead us to present two new cases of left aorto-ventricular tunnel successfully operated.