Concordance of congenital heart defects in two pairs of monozygotic twins: pulmonary stenosis and tetralogy of Fallot.

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.

Anomalous origin of the left coronary artery from the pulmonary artery: echocardiographic diagnosis / Origen anómalo de la arteria coronaria izquierda de la arteria pulmonar: diagnóstico ecocardiográfico

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.

Anomalous origin of the left coronary artery from the pulmonary artery. Echocardiographic diagnosis.

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.

[The Fick method underestimates pulmonary flow calculation in patients subjected to bidirectional Glenn procedure]. / El método de Fick subestima el cálculo deflujo pulmonar en los enfermos operados de Glenn bidireccional.

In patients with Bidirectional Glenn who have undergone surgery, the superior caval venous flow provides the only pulmonary blood supply. This is the effective pulmonary flow and at the same time its volume is not enough to overflow the single ventricle. The unsaturated, inferior vena cava flow is not oxygenated, since it goes across the interatrial septal communication and gets mixed in the left ventricle with the pulmonary venous blood. In this work, a bidirectional Glenn case is analyzed. The hemodynamic data before and after the operation are shown. It was evident from this case that the use of the Fick method to measure pulmonary flow in patients with bidirectional Glenn operation is not appropriate. Alternative methods, such as Doppler echocardiography and Magnetic Resonance Imaging, are recommended. A literature review on this subject was carefully done.

El método de Fick subestima el cálculo deflujo pulmonar en los enfermos operados de Glenn bidireccional / The Fick method underestimates pulmonary flow calculation in patients subjected to bidirectional Glenn procedure

In patients with Bidirectional Glenn who have undergone surgery, the superior caval venous flow provides the only pulmonary blood supply. This is the effective pulmonary flow and at the same time its volume is not enough to overflow the single ventricle. The unsaturated, inferior vena cava flow is not oxygenated, since it goes across the interatrial septal communication and gets mixed in the left ventricle with the pulmonary venous blood. In this work, a bidirectional Glenn case is analyzed. The hemodynamic data before and after the operation are shown. It was evident from this case that the use of the Fick method to measure pulmonary flow in patients with bidirectional Glenn operation is not appropriate. Alternative methods, such as Doppler echocardiography and Magnetic Resonance Imaging, are recommended. A literature review on this subject was carefully done.

Estenosis valvular aórtica congénita. Actualización del tratamiento / Congenital aortic valve stenosis. Current treatment

OBJECTIVE: Congenital aortic valve stenosis is a common lesion, with an approximate incidence of 5 to 7% of all cardiac malformations and occupies the first place among left heart obstructions. In recent years, many modalities of treatment have been developed. Fetal interventions has evolved in one extreme of life, on the other hand, percutaneous aortic valve replacement is now available for aged adults. In children and adolescents, percutaneous aortic valve valvuloplasty is now more effective with new techniques. The Ross procedure is the first choice treatment in children and young adults with hipoplastic aortic annulus. Considerable medical information has evolved and expanded from these techniques. A review of the indications, optimal timing, and outcomes of these procedures is pertinent.

[Congenital aortic valve stenosis. Current treatment]. / Estenosis valvular aórtica congénita. Actualización del tratamiento.

OBJECTIVE: Congenital aortic valve stenosis is a common lesion, with an approximate incidence of 5 to 7% of all cardiac malformations and occupies the first place among left heart obstructions. In recent years, many modalities of treatment have been developed. Fetal interventions has evolved in one extreme of life, on the other hand, percutaneous aortic valve replacement is now available for aged adults. In children and adolescents, percutaneous aortic valve valvuloplasty is now more effective with new techniques. The Ross procedure is the first choice treatment in children and young adults with hipoplastic aortic annulus. Considerable medical information has evolved and expanded from these techniques. A review of the indications, optimal timing, and outcomes of these procedures is pertinent.

[Cardiac rhabdomyoma surgically treated with success. Review of literature]. / Rabdomioma cardiaco tratado quirúrgicamente con exito y revisión de la literatura.

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.

Balloon angioplasty in aortic coarctation: a multicentric study in Mexico.

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.

Tratamiento por vía percutánea de múltiples defectos cardiacos / Percutaneous treatment of multiple heart defects

Se describe nuestra experiencia en 6 casos con diferentes cardiopatías asociadas, las cuales fueron tratadas por vía percutánea. La edad de los enfermos varió de 2.3 a 10 años (media 6.1) con un seguimiento de 1 a 84 meses (media 28.8). Había dos casos con CoAo y PCA, dos con EP y PCA, un caso con EAo y PCA y un enfermo con CoAo, EM y estenosis subaórtica (Síndrome de Shone). En todos se obtuvo cierre total del CA mediante "coil" de Gianturco u oclusor de Rashkind. En los casos con CoAo el gradiente disminuyó de 46 a 9 mmHg, con EP de 110 a 10 mmHg y en el caso con EAo de 40 a 14 mmHg. En el enfermo con síndrome de Shone de 8.1 años de edad, se realizó angioplastía de la CoAo, valvuloplastía mitral con catéter de Inoue y posteriormente se intervino quirúrgicamente en el diafragma subvalvular aórtico. Todos se encuentran asintomáticos hasta el periodo de seguimiento actual. En conclusión, pensamos que el tratamiento por vía percutánea, en pacientes con varias cardiopatías asociadas ofrece una alternativa segura y efectiva al tratamiento quirúrgico convencional.

Cierre transcateterismo del conducto arterioso mediante espirales (coils). Resultados inmediatos. Experiencia inicial en México / Clousure of ductus arteriosus with spring coils. Early experience inicial in Mexico

De marzo a mayo de 1996 se realizó cierre del conducto arterioso mediante espirales de Gianturco en 4 pacientes. La edad varió de 5 meses a 11 años (media 4.3 años). El diámetro del conducto arterioso fue de 2.1 a 3.0 mm (media 2.2 mm). Un caso correspondía a una paciente con 2 oclusores de Rashkind y fuga residual y una paciente tenía estenosis valvular pulmonar asociada. En este caso se realizó valvuloplastía pulmonar y posteriormente cierre del conducto arterioso. La presión media de la arteria pulmonar fue de 10 a 28 mmHg (media 18), el QP/QS de 1.2 a 5.0:1 (media 3.2). Se obtuvo oclusión inmediata en 2 casos y los dos restantes tienen fuga mínima demostrada por ecocardiografía Doppler, a 3 semanas de seguimiento. No se presentó ninguna complicación. En conclusión se considera este dispositivo como una nueva alternativa al cierre no quirúrgico del conducto arterioso. Es efectivo, de bajo costo, aplicable a cualquier edad con mínimo riesgo de complicaciones y prácticamente nula mortalidad.

Cor Triatriatum. Experiencia diagnóstica y quirúrgica / Cor Triatriatum. Diagnostic and therapeutic experiences

Se presentan cinco casos de cor triatriatum correspondientes a sujetos con edad promedio de 3.6 años (límites: 11 meses y 10 años), que fueron manejados durante un periodo de menos de 2 años, y se hace una revisión de la literatura. Tres pacientes fueron operados con resultados satisfactorios, otro enfermo falleció después del cateterismo cardiaco por edema pulmonar agudo y el último, con una obstrucción poco significativa, está en espera del tratamiento quirúrgico. Se hace énfasis en la utilidad del eco Doppler color para establecer el diagnóstico de esta cardiopatía congénita. No tenemos una explicación completa para la frecuencia del tal cardiopatía en un lapso tan corto

Cierre transcateterismo del conducto arterioso (CTCA) con oclusor de Rashkind: experiencia de 3.7 años / Closure of patent ductus arteriosus with Rashkind umbrella occluder

Se realizaron 38 procedimientos de cierres transcateterismo del conducto arterioso (CTCA) con el sistema de Rashkind en 34 pacientes, con edades de 1.5 a 31 años (8.5ñ9.0) y diámetoo del conducto arterioso (DCA) de 2 a 9 mm (4.0ñ1.5). La implantación fue exitosa en 36 procedimientos (94.7 por ciento). En el período postimplante inmediato se obtuvo oclusión total en 15 (45.4 por ciento), 12 (36.3 por ciento) quedaron con fuga residual mínima, y 6 (18.1 por ciento) con fuga importante. En 4 se colocó un segundo oclusor con éxito. Durante el seguimiento se ha logrado la oclusión total en el 87.0 por ciento (n=27), el 96.7 por ciento (n=30) no ameritarán manejo posterior. Dos pacientes fueron enviados a cirugía por fuga residual. Se presentó embolismo del oclusor en 2 casos (5.2 por ciento) a la rama izquierda de la arteria pulmonar (RIAP), uno de ellos fue enviado a cirugía y el otro caso se produjo durante el intento de colocación de un segundo dispositivo en una paciente con un oclusor previo y fuga residual, el oclusor se dejó en la RIAP y 4 meses más tarde se implantó otro con éxito. No hubo mortalidad. Estos resultados confirman la eficacia, la baja morbilida y la nula mortalidad del CTCA, especialmente cuando el conducto arterioso es pequeño (menor a 4mm)