RESUMEN

BACKGROUND: Coumel tachycardia is an infrequent form of supraventricular tachycardia (SVT) that usually occurs in infants and children. It is a tachycardia mediated by an accessory pathway with retrograde slow conduction that explains the classic ECG pattern with long RP' interval and negative P waves in leads II, III, and aVF. In this study, we describe the clinical course and management of Coumel tachycardia in children. CASE REPORT: We conducted a retrospective review of five consecutive pediatric patients, mean age 11 ± 3 years (range 6 to 14). The first episode of SVT was at a mean age of 10.4 ± 4.8 years (range 2 to 14) with a mean evolution of 7.4 ± 9.4 months (range 1 to 24). Pharmacological therapy was unsuccessful despite the combination of antiarrhythmic drugs. The tachycardia was incessant with a density > 85% by 24-hour Holter monitoring; one patient developed tachycardia-induced cardiomyopathy. All children underwent successful radiofrequency catheter ablation, mean 5 ± 3 applications (range 1 to 8) with a single session and with no complications. After a mean follow-up of 24 ± 16 months, all patients were asymptomatic and recurrence-free without antiarrhythmic treatment. CONCLUSIONS: Coumel tachycardia is clinically persistent and usually refractory to antiarrhythmic treatment with substantial risk of tachycardia-mediated cardiomyopathy. Catheter ablation is effective and safe in children; thus, it should be indicated promptly and based on individual selection.

INTRODUCCIÓN: La taquicardia de Coumel es una forma poco frecuente de taquicardia supraventricular que suele presentarse en lactantes. Es una taquicardia mediada por una vía accesoria de conducción lenta retrógrada que explica el patrón ECG clásico con intervalo RP' largo y ondas P negativas en las derivaciones II, III y aVF. En este trabajo se describe el curso clínico y el manejo de la taquicardia de Coumel en niños. CASO CLÍNICO: Se llevó a cabo una revisión retrospectiva de cinco pacientes pediátricos consecutivos, con una media de edad de 11 ± 3 años (intervalos 6 a 14). El primer episodio de taquicardia 10.4 ± 4.8 años con evolución de 7.4 ± 9.4 meses. El tratamiento farmacológico fue ineficaz a pesar de la combinación de antiarrítmicos. La taquicardia era incesante con una densidad > 85% por Holter-24h; un paciente desarrolló miocardiopatía inducida por taquicardia. Todos los niños fueron sometidos a ablación con catéter y radiofrecuencia con éxito, y un promedio de 5 ± 3 aplicaciones en una sola sesión y sin complicaciones. Después de un seguimiento de 24 ± 16 meses, todos los pacientes fueron asintomáticos y libres de recurrencia sin tratamiento antiarrítmico. CONCLUSIONES: La taquicardia de Coumel es clínicamente persistente y generalmente refractaria al tratamiento antiarrítmico con un riesgo sustancial de miocardiopatía mediada por taquicardia. La ablación con catéter es eficaz y segura en niños, por lo que debe indicarse de forma temprana y en lactantes de una selección individual.

Asunto(s)

RESUMEN

Abstract Background: Coumel tachycardia is an infrequent form of supraventricular tachycardia (SVT) that usually occurs in infants and children. It is a tachycardia mediated by an accessory pathway with retrograde slow conduction that explains the classic ECG pattern with long RP' interval and negative P waves in leads II, III, and aVF. In this study, we describe the clinical course and management of Coumel tachycardia in children. Case report: We conducted a retrospective review of five consecutive pediatric patients, mean age 11 ± 3 years (range 6 to 14). The first episode of SVT was at a mean age of 10.4 ± 4.8 years (range 2 to 14) with a mean evolution of 7.4 ± 9.4 months (range 1 to 24). Pharmacological therapy was unsuccessful despite the combination of antiarrhythmic drugs. The tachycardia was incessant with a density > 85% by 24-hour Holter monitoring; one patient developed tachycardia-induced cardiomyopathy. All children underwent successful radiofrequency catheter ablation, mean 5 ± 3 applications (range 1 to 8) with a single session and with no complications. After a mean follow-up of 24 ± 16 months, all patients were asymptomatic and recurrence-free without antiarrhythmic treatment. Conclusions: Coumel tachycardia is clinically persistent and usually refractory to antiarrhythmic treatment with substantial risk of tachycardia-mediated cardiomyopathy. Catheter ablation is effective and safe in children; thus, it should be indicated promptly and based on individual selection.

Resumen Introducción: La taquicardia de Coumel es una forma poco frecuente de taquicardia supraventricular que suele presentarse en lactantes. Es una taquicardia mediada por una vía accesoria de conducción lenta retrógrada que explica el patrón ECG clásico con intervalo RP' largo y ondas P negativas en las derivaciones II, III y aVF. En este trabajo se describe el curso clínico y el manejo de la taquicardia de Coumel en niños. Caso clínico: Se llevó a cabo una revisión retrospectiva de cinco pacientes pediátricos consecutivos, con una media de edad de 11 ± 3 años (intervalos 6 a 14). El primer episodio de taquicardia 10.4 ± 4.8 años con evolución de 7.4 ± 9.4 meses. El tratamiento farmacológico fue ineficaz a pesar de la combinación de antiarrítmicos. La taquicardia era incesante con una densidad > 85% por Holter-24h; un paciente desarrolló miocardiopatía inducida por taquicardia. Todos los niños fueron sometidos a ablación con catéter y radiofrecuencia con éxito, y un promedio de 5 ± 3 aplicaciones en una sola sesión y sin complicaciones. Después de un seguimiento de 24 ± 16 meses, todos los pacientes fueron asintomáticos y libres de recurrencia sin tratamiento antiarrítmico. Conclusiones: La taquicardia de Coumel es clínicamente persistente y generalmente refractaria al tratamiento antiarrítmico con un riesgo sustancial de miocardiopatía mediada por taquicardia. La ablación con catéter es eficaz y segura en niños, por lo que debe indicarse de forma temprana y en lactantes de una selección individual.

RESUMEN

BACKGROUND: Idiopathic ventricular tachycardia (VT) in children with structurally normal hearts is generally unrelated to the risk of sudden arrhythmic death. Still, it may be associated with deterioration in the quality of life. VT involving the fascicular conduction system is the most typical form of idiopathic left VT. In this retrospective study, we describe the experience of the clinical presentation, catheter ablation, and long-term follow-up of left fascicular VT in children. METHODS: An electrophysiological study was performed on consecutive children at a single tertiary center. Clinical fascicular left VT was induced by programmed stimulation, and catheter ablation was guided searching for Purkinje potentials. RESULTS: We included 18 patients (0.8 patients/year): 14 (77.8%) males and four females. The mean age of the first VT episode was 8.5 ± 5 years. Intravenous verapamil administration was effective for paroxysmal fascicular VT but not for prevention of recurrences. The mean age at the time of catheter ablation was 11.1 ± 3.8 years (8 months-16 years). The mean weight was 36.8 ± 16.4 kg (8.7-58 kg). A 100% success rate was observed with catheter ablation after repeated procedures without major complications. Mean follow-up was 2.0 ± 1.2 years (1.0-4.0 years, median 1.5), with permanent success in all patients and no antiarrhythmic drug administration. CONCLUSIONS: Fascicular VT has an adverse clinical course in children. In most cases, this condition is drug refractory. Catheter ablation is successful and safe treatment and should represent the first-line approach in symptomatic children.

INTRODUCCIÓN: La taquicardia ventricular (TV) idiopática en niños con corazón estructuralmente normal generalmente no se relaciona con el riesgo de muerte súbita arrítmica, pero puede asociarse con deterioro de la calidad de vida. La TV que involucra el sistema de conducción fascicular es la forma más común de TV izquierda idiopática. En este estudio retrospectivo se describe la experiencia de presentación clínica, ablación con catéter y seguimiento a largo plazo de TV fascicular en niños. MÉTODOS: Se llevó a cabo un estudio electrofisiológico en niños consecutivos en un centro terciario. La TV fascicular clínica se indujo mediante la estimulación programada y la ablación con catéter fue guiada buscando el registro de potenciales de Purkinje. RESULTADOS: Se incluyeron 18 pacientes (0.8 pacientes/año): 14 (77.8%) de sexo masculino y cuatro de sexo femenino. La media de edad a la cual ocurrió el primer episodio fue de 8.5 ± 5 años. La administración intravenosa de verapamilo fue eficaz para la TV fascicular paroxística, pero no para prevención de recurrencias. La media de edad de la ablación con catéter fue de 11.1 ± 3.8 años (8 meses-16 años). La media del peso fue 36.8 ± 16.4 kg (8.7-58 kg). Se observó el 100% de éxito con la ablación con catéter después de procedimientos repetidos sin complicaciones mayores. La media de seguimiento fue de 2.0 ± 1.2 años (1.0-4.0, mediana de 1.5 años) con éxito permanente en todos los pacientes y sin administración de fármacos antiarrítmicos. CONCLUSIONES: En niños, el curso clínico de la TV fascicular es adverso. Además, en la mayoría de los casos, esta condición es refractaria a fármacos. La ablación con catéter resulta exitosa y segura y debe representar el abordaje de primera línea en niños sintomáticos.

Asunto(s)

RESUMEN

Abstract Background: Idiopathic ventricular tachycardia (VT) in children with structurally normal hearts is generally unrelated to the risk of sudden arrhythmic death. Still, it may be associated with deterioration in the quality of life. VT involving the fascicular conduction system is the most typical form of idiopathic left VT. In this retrospective study, we describe the experience of the clinical presentation, catheter ablation, and long-term follow-up of left fascicular VT in children. Methods: An electrophysiological study was performed on consecutive children at a single tertiary center. Clinical fascicular left VT was induced by programmed stimulation, and catheter ablation was guided searching for Purkinje potentials. Results: We included 18 patients (0.8 patients/year): 14 (77.8%) males and four females. The mean age of the first VT episode was 8.5 ± 5 years. Intravenous verapamil administration was effective for paroxysmal fascicular VT but not for prevention of recurrences. The mean age at the time of catheter ablation was 11.1 ± 3.8 years (8 months-16 years). The mean weight was 36.8 ± 16.4 kg (8.7-58 kg). A 100% success rate was observed with catheter ablation after repeated procedures without major complications. Mean follow-up was 2.0 ± 1.2 years (1.0-4.0 years, median 1.5), with permanent success in all patients and no antiarrhythmic drug administration. Conclusions: Fascicular VT has an adverse clinical course in children. In most cases, this condition is drug refractory. Catheter ablation is successful and safe treatment and should represent the first-line approach in symptomatic children.

Resumen Introducción: La taquicardia ventricular (TV) idiopática en niños con corazón estructuralmente normal generalmente no se relaciona con el riesgo de muerte súbita arrítmica, pero puede asociarse con deterioro de la calidad de vida. La TV que involucra el sistema de conducción fascicular es la forma más común de TV izquierda idiopática. En este estudio retrospectivo se describe la experiencia de presentación clínica, ablación con catéter y seguimiento a largo plazo de TV fascicular en niños. Métodos: Se llevó a cabo un estudio electrofisiológico en niños consecutivos en un centro terciario. La TV fascicular clínica se indujo mediante la estimulación programada y la ablación con catéter fue guiada buscando el registro de potenciales de Purkinje. Resultados: Se incluyeron 18 pacientes (0.8 pacientes/año): 14 (77.8%) de sexo masculino y cuatro de sexo femenino. La media de edad a la cual ocurrió el primer episodio fue de 8.5 ± 5 años. La administración intravenosa de verapamilo fue eficaz para la TV fascicular paroxística, pero no para prevención de recurrencias. La media de edad de la ablación con catéter fue de 11.1 ± 3.8 años (8 meses-16 años). La media del peso fue 36.8 ± 16.4 kg (8.7-58 kg). Se observó el 100% de éxito con la ablación con catéter después de procedimientos repetidos sin complicaciones mayores. La media de seguimiento fue de 2.0 ± 1.2 años (1.0-4.0, mediana de 1.5 años) con éxito permanente en todos los pacientes y sin administración de fármacos antiarrítmicos. Conclusiones: En niños, el curso clínico de la TV fascicular es adverso. Además, en la mayoría de los casos, esta condición es refractaria a fármacos. La ablación con catéter resulta exitosa y segura y debe representar el abordaje de primera línea en niños sintomáticos.

RESUMEN

Introducción. La cardiomiopatía dilatada idiopática es la forma más común de cardiomiopatía en niños; sin embargo, ocasionalmente son identificadas causas potencialmente reversibles. Entre estas existe un grupo de pacientes con manifestaciones de insuficiencia cardiaca y taquicardia persistente que representan una forma de cardiomiopatía no familiar adquirida conocida como cardiomiopatía inducida por taquicardia o taquicardiomiopatía, que es reversible con el tratamiento efectivo de la taquicardia. Estos pacientes pueden ser mal diagnosticados y tratados de forma inapropiada. El diagnóstico frecuentemente es tardío, por lo que debe sospecharse en pacientes con insuficiencia cardiaca y taquicardia persistente sin causa aparente. Casos clínicos. Se describen seis casos de pacientes pediátricos de 6 a 16 años de edad (media 12 ± 4 años) que se presentaron con manifestaciones clínicas de insuficiencia cardiaca. Tuvieron seguimiento por una forma de cardiomiopatía dilatada en los que se documentaron diferentes mecanismos de taquiarritmia persistente como la causa. El tratamiento apropiado -mediante ablación con catéter- condujo a la recuperación de la función ventricular. Conclusiones. Es importante el reconocimiento de la cardiomiopatía inducida por taquiarritmia en pediatría. También es necesario un diagnóstico oportuno así como un tratamiento eficaz, ya que la cardiomiopatía inducida por taquicardia es una causa reversible de insuficiencia cardiaca.

Background. Idiopathic dilated cardiomyopathy is the most common form of cardiomyopathy in children; however, potentially reversible causes may occasionally be identified. Among these a group of patients with symptoms of congestive heart failure and persistent tachycardia representing a form of nonfamilial acquired cardiomyopathy known as tachycardia-induced cardiomyopathy or tachycardiomyopathy. This is a reversible condition with effective treatment of tachycardia. These patients may be misdiagnosed, potentially leading to inappropriate treatment. Diagnosis is often late and always should be suspected in patients with congestive heart failure and unexplained persistent tachycardia. Case reports. We describe six pediatric patients (mean age 12 ±4 years old, range 6-16 years). Patients presented with clinical manifestations of heart failure followed by dilated cardiomyopathy. Different mechanisms of persistent tachycardia were documented as the cause and total recovery was achieved of ventricular function after successful treatment of tachycardia by catheter ablation. Conclusions. The recognition of tachyarrhythmia-induced cardiomyopathy in pediatric patients is important. Opportune diagnosis and effective treatment are necessary because tachycardia-induced cardiomyopathy is a reversible cause of heart failure.

RESUMEN

Two pairs of twins with specific concordance in congenital heart lesions are presented. We reviewed all the monozygotic twins with specific concordance in congenital heart lesions in the last 18 years. Two pairs were found. First, a pair of 13 years-old monozygotic female twins; a severe infundibular and pulmonary valve stenosis was discovered in both girls. In the second pair of twins (8 years-old boys), Tetralogy of Fallot was diagnosed by echocardiogram, cardiac catheterization and angicardiography. In fluorescence hybridization test in situ all patients were negative for microdeletion in chromosome 22q11. Anatomic differences between twins are not enough explained on genetic bases; it's necessary to consider the role of other factors, probably acquired during the first stages of embryonic development. These are the two first pairs of twins with specific concordance in congenital cardiac lesions reported in Mexico.

Asunto(s)

RESUMEN

BACKGROUND: antiphospholipid antibody syndrome (APS) is characterized by the association among arterial or venous thrombosis, recurrent pregnancy loss, thrombocytopenia and the presence of circulating antiphospholipid antibodies. Antiphospholipid antibody-related thrombosis seems to constitute a significant proportion of childhood thrombosis. We present the case of an adolescent female with non-ST-elevation acute myocardial infarction secondary to APS. CLINICAL CASE: we present the case of a 14-year old female with a history of idiopathic thrombocytopenic purpura whose symptomatology initiated with oppressive chest pain, dyspnea, diaphoresis and nausea. During the physical exam she had tachycardia, accentuation of the second heart sound, a mesosystolic murmur in the pulmonary focus and pulmonary rales. Electrocardiogram showed ST-segment depression in anterior and lateral wall and positive enzymatic curve for infarction. Clinical study reported anterolateral and apical infarction, ostium secundum atrial septal defect and APS. CONCLUSIONS: many studies have demonstrated the diversity of clinical features associated with APS in children and teenagers including deep venous thrombosis, stroke, digital ischemia, pulmonary vasoocclusive disease and, more rarely, acute myocardial infarction. In pediatric patients with acute myocardial infarction we must consider the possibility of APS even though coronary thrombotic occlusion is one of the rarest manifestations.

Asunto(s)

RESUMEN

The experience with echocardiographic diagnosis of five cases of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) is reviewed. MATERIAL AND METHODS: all cases with a diagnosis of ALCAPA during a 10 year period were included. RESULTS: two age groups were clearly identified: infants and older patients. In the former, the echocardiographic findings included a dilated left ventricle with low ejection fraction (19% and 26%), mitral regurgitation, dilated right coronary artery and difficulties in identifying the origin of the left coronary artery. An 11-year-old asymptomatic boy, a 17-year-old young woman with dyspnea on effort and a 55-year-old woman with angina formed the older group. In these three cases, an abnormal upward flow was detected within the ventricular septum related to the collaterals and the inverse flow of the anterior descending artery. A reverse flow within the origin of the left coronary artery, probably related to an origin in the pulmonary artery, was observed. In all five cases the diagnosis was corroborated using selective right coronary artery angiography. CONCLUSIONS: In the infants, the dilated left ventricle with impaired systolic function, mitral regurgitation and dilated right coronary artery make it necessary to discard the ALCAPA diagnosis. In the older group, even in asymptomatic patients, an abnormal ascending flow within the ventricular septum, combined with a dilated coronary artery, was the most reliable echocardiographic evidence for a diagnosis of ALCAPA.

Asunto(s)

RESUMEN

UNLABELLED: Congenital coronary artery fistulas are uncommon anomalies, however themselves may resemble the whole spectrum of cardiac manifestations. Clinical presentations vary considerably from adults to children. MATERIAL AND METHODS: patients with coronary artery fistula diagnosed by selective coronariography in the period from 2000 to 2007 were included. RESULTS: 7 cases were found, 4 children, mean age 6.5 years, and 3 adults, mean age 25.6 years. The fistulae originate from the left coronary artery in 3 children, 2 connect to the pulmonary artery an one to the right atrium, in one child the fistula originated from the right coronary artery and terminated at the right ventricle. In the 3 adult patients, the fistula originated from the right ventricle, two connected to the right ventricle and one to the coronary sinus. Two children were operated on successfully and are asymptomatic 1 and 4 years later. In an adult patient with surgical ligation was performed, 4 weeks later developed an uncomplicated myocardial infarction. We reviewed the literature available and were compared with our cases. CONCLUSIONS: The clinical manifestations and hemodynamic features differ in childhood and adulthood. Coronary angiography is the primary diagnostic tool. Surgical treatment has low mortality and morbidity.

Asunto(s)

RESUMEN

Congenital coronary artery fistulas are uncommon anomalies, however themselves may resemble the whole spectrum of cardiac manifestations. Clinical presentations vary considerably from adults to children. MATERIAL AND METHODS: patients with coronary artery fistula diagnosed by selective coronariography in the period from 2000 to 2007 were included. RESULTS: 7 cases were found, 4 children, mean age 6.5 years, and 3 adults, mean age 25.6 years. The fistulae originate from the left coronary artery in 3 children, 2 connect to the pulmonary artery an one to the right atrium, in one child the fistula originated from the right coronary artery and terminated at the right ventricle. In the 3 adult patients, the fistula originated from the right ventricle, two connected to the right ventricle and one to the coronary sinus. Two children were operated on successfully and are asymptomatic 1 and 4 years later. In an adult patient with surgical ligation was performed, 4 weeks later developed an uncomplicated myocardial infarction. We reviewed the literature available and were compared with our cases. CONCLUSIONS: The clinical manifestations and hemodynamic features differ in childhood and adulthood. Coronary angiography is the primary diagnostic tool. Surgical treatment has low mortality and morbidity.

Asunto(s)

RESUMEN

UNLABELLED: The experience with echocardiographic diagnosis of five cases of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) is reviewed. MATERIAL AND METHODS: all cases with a diagnosis of ALCAPA during a 10 year period were included. RESULTS: two age groups were clearly identified: infants and older patients. In the former, the echocardiographic findings included a dilated left ventricle with low ejection fraction (19% and 26%), mitral regurgitation, dilated right coronary artery and difficulties in identifying the origin of the left coronary artery. An 11-year-old asymptomatic boy, a 17-year-old young woman with dyspnea on effort and a 55-year-old woman with angina formed the older group. In these three cases, an abnormal upward flow was detected within the ventricular septum related to the collaterals and the inverse flow of the anterior descending artery. A reverse flow within the origin of the left coronary artery, probably related to an origin in the pulmonary artery, was observed. In all five cases the diagnosis was corroborated using selective right coronary artery angiography. CONCLUSIONS: In the infants, the dilated left ventricle with impaired systolic function, mitral regurgitation and dilated right coronary artery make it necessary to discard the ALCAPA diagnosis. In the older group, even in asymptomatic patients, an abnormal ascending flow within the ventricular septum, combined with a dilated coronary artery, was the most reliable echocardiographic evidence for a diagnosis of ALCAPA.

Asunto(s)

RESUMEN

Ectopia cordis is an extremely rare cardiac anomaly. The heart is localized partially or totally outside the thorax cavity. This anomaly occurs as an isolated defect or combined with others midline defects. Cantrell and colleagues described, in 1958, a syndrome including defects of the abdominal wall, sternum, diaphragm, pericardium and heart. There are few successful surgical cases with this pentalogy. We describe a case with this Cantrell's pentalogy. The cardiac malformation was a univentricular heart with pulmonary stenosis. The patient underwent successful surgical palliation with a systemic-to-pulmonary anastomosis and uneventful recovering.

Asunto(s)

RESUMEN

Ectopia cordis is an extremely rare cardiac anomaly. The heart is localized partially or totally outside the thorax cavity. This anomaly occurs as an isolated defect or combined with others midline defects. Cantrell and colleagues described, in 1958, a syndrome including defects of the abdominal wall, sternum, diaphragm, pericardium and heart. There are few successful surgical cases with this pentalogy. We describe a case with this Cantrell's pentalogy. The cardiac malformation was a univentricular heart with pulmonary stenosis. The patient underwent successful surgical palliation with a systemic-to-pulmonary anastomosis and uneventful recovering.

Asunto(s)

RESUMEN

The primary cardiac tumors are inusual, the incidence varies in all the ages between 0.005 to 0.05%. In pediatrics patients the incidence is 0.27%. The more frequent tumors during the childhood are the cardiac rhabdomyomas. These tumors are considered benigns. The clinical expression is wide, in the most the cases, the patients are asymptomatic and are detected by murmurs. In the prenatal age are manifested by arrhythmias or hydrops fetalis. The neonates and children may be show cardiac arrhythmias, low cardiac index and sudden cardiac death. The association with tuberous sclerosis had been reported in 81%. We present a neonate with cardiac rhabdomyoma diagnosed in the newborn period when he was asymptomatic, however in the follow-up he developed cardiac failure by obstruction in the out flow tract of the right ventricle. He underwent open cardiac surgery to resect the obstruction. Five months after surgery, the patient remain asymptomatic.

Asunto(s)

RESUMEN

Los tumores cardiacos primarios son raros, con incidencia variable en todas las edades del 0.005 al 0.05%. En pacientes pediátricos, la incidencia es del 0.27%. Los tumores más frecuentes durante la infancia son los rabdomiomas cardiacos, considerados como benignos. Aunque la expresión clínica es amplia, en la mayoría de los casos son asintomáticos y se detectan por la presencia de soplos. En la etapa prenatal se manifiestan con arritmias o hydrops fetalis. En algunos neonatos y lactantes se encuentran arritmias, datos de bajo gasto cardíaco o muerte súbita. La asociación con esclerosis tuberosa se ha observado hasta en un 81%. Se presenta el caso de paciente masculino neonato, con diagnóstico de rabdomioma cardiaco que inicialmente estaba asintomático, sin embargo en el seguimiento requirió de tratamiento quirúrgico a los 5 meses de edad, por datos de insuficiencia cardiaca secundaria a obstrucción del tracto de salida del ventrículo derecho. A 5 meses de la cirugía, el paciente está asintomático.

The primary cardiac tumors are inusual, the incidence varies in all the ages between 0.005 to 0.05%. In pediatrics patients the incidence is 0.27%. The more frequent tumors during the childhood are the cardiac rhabdomyomas. These tumors are considered benigns. The clinical expression is wide, in the most the cases, the patients are asymptomatic and are detected by murmurs. In the prenatal age are manifested by arrhythmias or hydrops fetalis. The neonates and children may be show cardiac arrhythmias, low cardiac index and sudden cardiac death. The association with tuberous sclerosis had been reported in 81%.We presenta neonate with cardiac rhabdomyoma diagnosed in the newborn period when he was asymptomatic, however in the follow-up he developed cardiac failure by obstruction in the out flow tract of the right ventricle. He underwent open cardiac surgery to resect the obstruction. Five months after surgery, the patient remain asymptomatic.

Asunto(s)

RESUMEN

OBJECTIVE: To evaluate 15 years of experience with balloon pulmonary valvuloplasty in a single third level health care center. MATERIAL AND METHODS: Hundred-fifty patients underwent the procedure, 73 (48%) men and 77 (52%) women, mean age 10.5 +/- 11.3 years. RESULTS: The initial systolic gradient decreased from 86 +/- 35 to 21.67 +/- 12.20 mm Hg, p < 0.001, whereas the initial right ventricular systolic pressure decreased from 106 +/- 34.8 to 53 +/- 27 mm Hg, p < 0.0001. At the end of the follow-up, 48 +/- 44 months, the systolic gradient was 13.43 +/- 8.73 mm Hg, p < 0.001. Major complications occurred in 9 (6.4%) patients. Immediate technical success was achieved in 111 (74%) patients and failure in 39 (26%). At the end of the follow-up period, successful outcomes were achieved in 104 (89.6%); in contrast, failures were present in 12 (10.4%) patients, p < 0.001. Death occurred in 2 (1.33%) patients. The predictors for failure were age < 1.5 +/- 1-33 years (p < 0.004), dysplastic valve (p < 0.001), high initial systolic gradient (p < 0.002), and high initial systolic right ventricular pressure (p < 0.0001). CONCLUSION: Balloon pulmonary valvuloplasty is an effective, safe, and first choice treatment for congenital pulmonary valve stenosis.

Asunto(s)

RESUMEN

Objetivo: Evaluar la experiencia de 15 años, de la valvuloplastía pulmonar con balón en el Centro Médico Nacional Siglo XXI. Material y métodos: Se estudiaron 150 pacientes 73 (48.7%) masculino y 77 (51.7%) femenino. La edad promedio de los 141 en quienes se realizó la valvuloplastía pulmonar con balón fue de 10.5 ± 11.3 años. Resultados: El gradiente promedio prevalvuloplastía de 86 ± 35 descendió a 21.67 ± 12.20 mm Hg p < .001. La presión sistólica del ventrículo derecho de 106 ± 34.8 mm Hg disminuyó a 53 ± 27 mm Hg p < 0.0001. El gradiente valvular pulmonar en el seguimiento promedio de 48 ± 44 meses fue de 13.43 ± 8.73 mm Hg p < 0.001. Se presentaron complicaciones en 9 (6%) pacientes. Se obtuvo éxito técnico inmediato post en 111 (74%) y fracaso inmediato en 39 (26%). Al final del seguimiento se obtuvo éxito en 104 (89.6%) y fracaso en 12 (10.4%) pacientes, p < 0.0001, con una mortalidad global de 2 (1.33 %) pacientes. Las variables significativas para fracaso fueron: la edad de, 1.5 ± 1.33 años para falla de procedimiento, p < .004; morfología valvular displásica p < 0.001. gradiente valvular pulmonar prevalvuloplastía elevado con p < 0.002 y presión sistólica del ventrículo derecho pre valvuloplastía elevados. p < 0.0001, no descendiendo ambos en el post inmediato. Conclusión: La valvuloplastía pulmonar con balón es el tratamiento de elección, efectivo y seguro de la estenosis valvular pulmonar congénita.

Objective: To evaluate 15 years of experience with balloon pulmonary valvuloplasty in a single third level health care center. Material and methods: Hundred-fifty patients underwent the procedure, 73 (48%) men and 77 (52%) women, mean age 10.5 ± 11.3 years. Results: The initial systolic gradient decreased from 86 ± 35 to 21.67 ± 12.20 mm Hg, p < 0.001, whereas the initial right ventricular systolic pressure decreased from 106 ± 34-8 to 53 ± 27 mm Hg, p < 0.0001. At the end of the follow-up, 48 ± 44 months, the systolic gradient was 13.43 ± 8.73 mm Hg, p < 0.001. Major complications occurred in 9 (6.4%) patients. Immediate technical success was achieved in 111 (74%) patients and failure in 39 (26%). At the end of the follow-up period, successful outcomes were achieved in 104 (89.6%); in contrast, failures were present in 12 (10.4%) patients, p < 0.001. Death occurred in 2 (1.33%) patients. The predictors for failure were age < 1.5 ± 1-33 years (p < 0.004), dysplastic valve (p < 0.001), high initial systolic gradient (p < 0.002), and high initial systolic right ventricular pressure (p < 0.0001). Conclusion: Balloon pulmonary valvuloplasty is an effective, safe, and first choice treatment for congenital pulmonary valve stenosis. (Arch Cardiol Mex 2003; 73:190-196).

Asunto(s)

RESUMEN

OBJECTIVES: To analyze immediate and long-term results of balloon dilation for aortic coarctation in a three-center experience in Mexico, and to determine factors associated with increased risk. BACKGROUND: Results demonstrated that the procedure is effective and safe, however its use in some groups is still controversial, specially in neonates and infants. METHODS: In a ten-year period, 333 patients with aortic coarctation on underwent balloon dilation with an immediate success rate of 93.7% and a major complication incidence of 1.8%. Of the total cohort, 272 patients were followed for a period of 24.3 +/- 20 months. Demographic and procedural data were analyzed to determine factors related to a poor outcome or to sustained high blood pressure. RESULTS: Cox regression analysis found age (risk ratio 3.42 p = 0.0001), isthmic hypoplasia (risk ratio 4.64, p < 0.0001), and post-dilation gradient (risk ratio 2.19, p = 0.0113) as independent risk factors for a follow-up event, mainly restenosis. Age at dilation was the only independent factor related to sustained hypertension with a seven-fold increase in the risk. CONCLUSIONS: Balloon dilation is an effective and safe alternative to treat aortic coarctation. Patients younger than one year of age, with severe isthmic hypoplasia and a post-dilation gradient > 20 mmHg have the highest risk to develop an event in the follow-up period. When the dilation procedure is performed in patients older than 10 years of age, and specially those older than 20 years, the probability that they remain or develop high blood pressure is increase.

Asunto(s)

RESUMEN

Presentamos un caso de comunicación directa entre la arteria pulmonar derecha y la aurícula izquierda. El diagnóstico se hizo antes de la operación. La ligadura quirúrgica de la fístula, resolvió la cianosis de la enferma. El angiocardiograma selectivo en la arteria pulmonar derecha, realizado 4 meses después, descartó cortocircuito residual. Esta malformación muy rara debe considerarse en presencia de cianosis inexplicable. Es éste el caso número 50 en la literatura.

Asunto(s)

RESUMEN

De septiembre de 1996 a marzo de 1997 se colocaron stents tipo Palmaz (P-308 Johnson & Johnson) a 6 pacientes con el diagnóstico de coartación aórtica: 3 del sexo femenino y 3 del sexo masculino con edades entre 13 y 30 años (m=20). En ningún caso fue necesaria la dilatación del sitio coartado antes de colocar el stent. El globo utilizado para el implante fue de 12 mm en dos casos y de 15 mm en 4 casos y para la implantación final varió de 14 a 20 mm en 5 casos. En un paciente fue necesario la dilatación final con un globo dual de 15 + 15 mm. El gradiente pre-stent varió de 30 a 65 mmHg (m=44) y disminuyó a 0 en 5 casos y en uno fue de 4 mmHg(m=1). La presión sistólica de la aorta ascendente disminuyó de 135 a 117 mmHg en promedio post colocación del stent. No se presentó ninguna complicación significativa o mortalidad. En ningún caso se observó imagen de disección o formación de aneurisma. En conclusión, pensamos que la colocación de stents en coartación aórtica en este grupo de pacientes parece ser una alternativa excelente al tratamiento quirúrgico convencional e incluso a la angioplastía con globo, al ofrecer una corrección anatómica muy favorable del sitio coartado, eliminar el gradiente, mínimo morbimortalidad, la incidencia de disección y formación de aneurismas pensamos será menor, y como todos los procedimientos vía percutánea, elimina la necesidad de una toracotomía. La reestenosis tardía no puede ser determinada por el momento pero se espera mínima principalmente en vista del gran diámetro luminal obtenido y posiblemente a que la velocidad del flujo a este nivel es alta

Asunto(s)