Assessing pulmonary hypertensive vascular disease in childhood. Data from the Spanish registry.

RATIONALE: There is a lack of knowledge regarding the epidemiology, clinical characterization, and survival in pediatric pulmonary hypertension. OBJECTIVES: To describe the epidemiology, outcomes, and risk factors for mortality in pediatric pulmonary hypertension in Spain. METHODS: We analyzed data from the Spanish Registry for Pediatric Pulmonary Hypertension. From January 2009 to June 2012, a total of 225 patients diagnosed with pulmonary hypertension in 1998 or after were collected from 21 referral and nonreferral centers. We included all Nice etiologies, estimated incidence and prevalence of pulmonary hypertension in the Spanish pediatric population, and analyzed risk factors for mortality (Nice etiologic group, clinical and hemodynamic variables). Patients were classified as follows: group I, pulmonary arterial hypertension (n = 142; 61%); group II, left heart disease (n = 31; 14%); group III, respiratory disease (n = 41; 18%); group IV, thromboembolic pulmonary hypertension (n = 2; 1%); or group V, mostly inherited metabolic diseases (n = 10; 4.5%). Of the patients studied, 31% had multifactorial pulmonary hypertension. MEASUREMENTS AND MAIN RESULTS: Mean age at diagnosis was 4.3 ± 4.9 years (50% < 2 yr). Survival rates at 1 and 3 years were 80 and 74% for the whole cohort, and 89 and 85% for patients with pulmonary arterial hypertension. Independent risk factors for mortality included an etiologic group other than pulmonary arterial hypertension (P < 0.001), age at diagnosis younger than 2 years old (P < 0.001), advanced functional class at diagnosis (P < 0.001), and high right atrial pressure at diagnosis (P = 0.002). CONCLUSIONS: In moderate to severe pediatric pulmonary hypertension, the prognosis is better in pulmonary arterial hypertension than in other Nice categories. In pediatric pulmonary hypertension age at diagnosis younger than 2 years is a risk factor for mortality, in addition to the previously established risk factors.

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.

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

Objetivos: Analizar los resultados del intervencionismo no quirúrgico sobre la recoartación y coartación de aorta, valorando los resultados de cada técnica, las complicaciones y su evolución. Pacientes y métodos: Los tratamientos fueron: 51 dilatados con balón por recoartación (86,5 por ciento), dos en los que se dilató una coartación nativa (3,3 por ciento) y seis en los que se ha implantado un tubo expansible (stent) por recoartación (10 por ciento). Las edades oscilan entre los 2 y los 236 meses (109 +/- 63,45 meses), con un tiempo de seguimiento entre 1 y 156 meses (38,87 +/- 32,96 meses). Resultados: El gradiente predilatación en los niños recoartados fue de 34 +/- 11,62 mmHg, que descendió a 11 +/- 5,38 mmHg (p < 0,0001). Doce casos (20,3 por ciento) no obtuvieron una dilatación eficaz. El tamaño de la estenosis fue de 6,7 +/- 2,35 mm predilatación y aumentó a 9,3 +/- 3,10 mm (p < 0,0001) con un incremento porcentual del 50,97. Las complicaciones fueron escasas. Seis niños precisaron redilatación posterior. La experiencia con stent muestra un gradiente medio de 32,83 +/- 10,62 mmHg que descendió a 7,3 3,8 mmHg (p < 0,0001) con una relación globo-estenosis de 1,94. Conclusiones: La técnica intervencionista es muy eficaz, tanto en coartación nativa como en recoartación de aorta, en resultados inmediatos y durante la evolución, y con escasas complicaciones. Los stents en niños mayores obtienen igualmente excelentes resultados, abriendo un futuro esperanzador (AU)

[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.

[Intravascular hemolysis following percutaneous occlusion of the ductus arteriosus]. / Hemólisis intravascular tras el cierre percutáneo del conducto arterioso.

Transcatheter occlusion of patent ductus arteriosus has become a safe and successful technique, but it's not free of complications. We present the case of a two-year-old boy who underwent routine transcatheter closure of his patent ductus arteriosus, using a "coil" device. Twenty hours later he developed severe persistent hemolysis in association with residual ductal flow. Patient's clinical situation became stable when the device was removed. Pulmonary embolization of the device and hemolysis are the main complications of percutaneous closure of the patent ductus arteriosus. Hemolysis occurs rarely (0.5%) and is always associated with the presence of residual ductal flow. Several approaches to this problem have been described. Mild cases may require no intervention; however, when severe hemolysis is present, removal of the device may be needed, proceeding with surgical repair of the patent ductus arteriosus.

[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.

[Aortico-left ventricular tunnel associated with pulmonary valve stenosis]. / Túnel aorta-ventrículo izquierdo asociado con estenosis valvular pulmonar.

Aortico-left ventricular tunnel is an unusual cardiac anomaly. The main clinical feature is early, severe aortic regurgitation, and surgical management is mandatory. Exceptionally this defect is associated with pulmonary valve stenosis. A case of a newborn with aortico-left ventricular tunnel plus pulmonary valve stenosis is reported. Initially she underwent percutaneous pulmonary valvuloplasty during diagnostic cardiac catheterization and with surgical closure of the tunnel later.

[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.

[Non-surgical extraction of intravascular foreign bodies in children. Experience with 8 cases]. / Extracción no quirúrgica de cuerpos extraños intravasculares en niños. Experiencia en 8 casos.

BACKGROUND AND OBJECTIVES: Foreign body retrieval in the catheter room is a useful procedure at any age, but, although its interest, few reports of such technique have been reported in children. METHODS: We review and present our experience in 8 children, aged 5 days to 11 years, five of them having congenital hearts defects. RESULTS: We retrieved 4 catheter fragments, 2 endocardial electrode catheter tips, 1 Rashkind 12 mm. PDA umbrella, and 1 detachable Jackson coil. All of them were placed in systemic veins, right heart chambers or pulmonary arteries. We used biplane fluoroscopy and percutaneous right femoral vein puncture in all cases. Goose-Neck (Microvena Corporation) snares were used in 5 patients, hand made snares in 2 and a Swan-Ganz catheter in one. In 4 cases, the snare was introduced trough a Mullins long sheath and the foreign body pulled into its distal end, in order to bring it out of the femoral vein. Six foreign bodies came off the femoral vein: 4 trough the puncture site and 2 needing a venous cut-down. The two remaining foreign bodies, stopped while pulling at the common iliac vein and a minor surgical procedure was needed for final extraction. CONCLUSIONS: Therapeutic catheterization is the technique of choice for intravascular foreign body retrieval in children.

[The ghost intraventricular obstruction. Its detection in the right ventricle by Doppler color]. / Obstrucción intraventricular fantasma. Su detección con Doppler color en ventrículo derecho.

INTRODUCTION: The Color Doppler echocardiography is a method of choice to detect the regional acceleration of flow in the left ventricle. The color study confirm it also in the right ventricle of infants with diseases other than hypertrophic cardiomyopathy. MATERIAL: We centered our attention in this finding observed in 16 infants with a mean age of 2.5 months (3 of whom were neonates) with several congenital heart malformations: 9 immediately after surgical ventricular septal defect closure (A-V canal type in 6, and isolated in 3); 2 with pulmonary atresia+intact septum+reconstruction of right ventricular-pulmonary artery connection; 2 with severe aortic stenosis; 1 with a double outlet right ventricle+pulmonary band; 1 with Jatene operation; finally 1 after balloon pulmonary valvuloplasty. RESULTS: The phenomenon consisted in a late Doppler systolic signal in the right ventricular apex whose velocity ranged between 1.5 and 2.9 m/s. The jet was directed towards the tricuspid orifice, with a normal pulmonary flow. The turbulence was easily viewed in the apical four chambers and subcostal sagittal plane of the right ventricle, the spectral signal showed a morphology with left side concavity. An erroneous diagnosis of apical ventricular septal defect was suggested in 2 cases in whom the left ventricular angiogram performed before treatment excluded the lesion. CONCLUSION: The dynamic nature of the jet suggest that an asynchronous order of myocardial contraction in hypertrophic right ventricule play a role in its genesis. With the increasing use of Doppler color technique, this finding can be observed with greater frequency in neonates and infants with operated or unoperated heart diseases and right ventricular hypertrophy. Apical ventricular septal defect will be included in the differential diagnosis.

[Value of two-dimensional Doppler color echocardiography in mitral-ventricular-aortic obstructive disease in the newborn]. / Valor de la ecocardiografía bidimensional Doppler color en la patología obstructiva mitroventriculoaórtica del neonato.

Over the last years, the bidimensional echocardiography has assumed an increasingly impact in the management of heart disease in neonates. With the addition of Doppler technique and color flow mapping this non invasive method provide the most accurate definition of cardiac anatomy and physiology. It is particularly true in newborns with left inflow or outflow obstruction disease such as: pulmonary vein stenosis, divided left atrium, mitral and aortic stenosis or atresia, subvalvular narrowing, and hypoplastic left ventricle and aortic tract. The combination of these two methods contribute to recognize a specific left cardiac abnormalities and allow us to evaluate the therapeutical success in the follow up 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.

[Unilateral agenesis of the pulmonary artery. Experience with 4 cases]. / Agenesia unilateral de la arteria pulmonar. Experiencia en 4 casos.

We present our experience with four cases of unilateral pulmonary agenesis, 3 of the right artery and the other one of the left. Two of the patients had pulmonary hypertension and one of them died at two years of age. All cases were diagnosed by using pulmonary angiography. Aortography demonstrated the absence of circulatory supply to the lung and a contralateral aortic arch to the absent pulmonary artery. We have performed a review of the literature with an emphasis on the diagnostic aspects of this condition, and discuss the considerations for possible surgery in symptomatic patients.