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A. Vista interior de las cámaras derechas de un corazón con hipoplasia ventricular derecha, atresia pulmonar y septum ventricular íntegro. Obsérvese el anillo tricuspídeo estrecho (línea discontinua), el adosamiento de las valvas septal y posterior (flecha con doble cabeza) y la gran hipertrofia de la pared libre del VD. Las abreviaturas iguales a las anteriores.
Source publication
In hypoplastic right ventricle the dysplasia of the tricuspid valve (TV) has repercussions on the development of the right ventricle. This paper documents the results of an anatomopathologic and morphometric studies of the tricuspid valve and the right ventricle in 25 hearts with this cardiopathy, which were morphologically analyzed using the segme...
Citations
... Until the preparation of this article, no similar case had been published, with a controversial clinical presentation, with significant pulmonary hypoflow that required emergency intervention. The case of a patient with situs inversus, tetralogy of Fallot, and total anomalous pulmonary venous connection have been described, in which clinical findings were partially similar to those in our patient, with pulmonary hypoflow due to the right ventricular outflow tract obstruction being found 6 ; however, our patient had situs solitus with tricuspid valve hypoplasia, which also determined a Type 1 hypoplastic right ventricle, with generalized hypoplasia at its three portions 7 . ...
... E: right ventricular outflow tract balloon angioplasty. F: X-ray showing ductus arteriosus stent (6) and collector-coronary sinus anastomosis stent (7). ...
... Ambos tipos constituyen los extremos del espectro que incluye formas intermedias. 3,8,12,14,17,18 Se debe señalar la asociación excepcional de la anomalía de Uhl (VD papiláceo de paredes muy delgadas) a la entidad que nos ocupa. 8 Existe en el mundo científico de nuestros tiempos una gran controversia acerca de la capacidad o no de desarrollo posquirúrgico del VD. 11,[13][14][15][16][17][18] -Arco aórtico siempre a la izquierda. ...
... 3,8,12,14,17,18 Se debe señalar la asociación excepcional de la anomalía de Uhl (VD papiláceo de paredes muy delgadas) a la entidad que nos ocupa. 8 Existe en el mundo científico de nuestros tiempos una gran controversia acerca de la capacidad o no de desarrollo posquirúrgico del VD. 11,[13][14][15][16][17][18] -Arco aórtico siempre a la izquierda. ...
Pulmonary atresia with intact ventricular septum is an apparently simple cyanotic congenital cardiopathy, characterized by several morphological variants and associated lesions leading to a complex diagnosis and treatment. The high mortality rate recorded in under 6 months-old infants demands an early aggressive management to face this situation. This review was intended to make a comprehensive assessment of the disease from its basic conceptual elements to the therapeutic variants to be adopted. To this end, the most important elements of morphology, physiopathology, diagnosis and treatment were addressed, in addition to discussing the roles of echocardiography, surgery and interventional cardiology techniques. The fundamental strategy of treatment is to separate the systemic circulation from the pulmonary circulation, causing neither reduction of the cardiac output nor increase of the central venous pressure. It is also aimed to return the right ventricle to the pulmonary circuit, provided that the coronary circulation does not depend on it. The algorithm presented in this paper underlined the importance of the integration of the surgical, interventional and hybrid modalities for the therapeutic management of this cardiopathy.
El espectro del ventrículo derecho hipoplásico es amplio y desafiante, ya que dependiendo de su variabilidad anatómica y funcionalidad se planteará qué tratamiento es el adecuado. Presentamos el caso de un lactante de sexo femenino, de 15 meses de edad, quien cursó asintomática, valorada en consulta de seguimiento por cianosis con saturación de oxígeno del 70%, motivo por el cual es referida a nuestra institución.
Pulmonary atresia with intact ventricular septum is an apparently simple cyanotic congenital cardiopathy, characterized by several morphological variants and associated lesions leading to a complex diagnosis and treatment. The high mortality rate recorded in under 6 months-old infants demands an early aggressive management to face this situation. This review was intended to make a comprehensive assessment of the disease from its basic conceptual elements to the therapeutic variants to be adopted. To this end, the most important elements of morphology, physiopathology, diagnosis and treatment were addressed, in addition to discussing the roles of echocardiography, surgery and interventional cardiology techniques. The fundamental strategy of treatment is to separate the systemic circulation from the pulmonary circulation, causing neither reduction of the cardiac output nor increase of the central venous pressure. It is also aimed to return the right ventricle to the pulmonary circuit, provided that the coronary circulation does not depend on it. The algorithm presented in this paper underlined the importance of the integration of the surgical, interventional and hybrid modalities for the therapeutic management of this cardiopathy.
Introduction: pulmonary atresia with intact ventricular system is a cardiovascular malformation accounting for 1% of congenital cardiopathies seen in the extrauterine life; due to its unfavorable outcomes, this disease is a true challenge for the contemporary medicine. Objectives: the research study comprised the application of the classification guidelines, the characterization of the atresia outlet track, the morphological study of the right ventricle, the assessment of the tricuspid ring and the detection of anomalies in the coronary circulation. Methods: forty three patients diagnosed with this disease and referred to "William Soler" pediatric cardiocenter from January 1992 to November 2011 were studied. Each case was performed bidimensional echocardiography and color-coding Doppler. Results and conclusions: the study confirmed the predominance of the valvular morphological variant of the disease and the presence of moderate and severe levels of right ventricular hypoplexia associated with limited volumetric capacitance of this chamber, with tricuspid valvular hypodevelopment and with anomalous sinusoids-dependent coronary circulation. The permeable oral foramen was the interatrial septal defect that was most associated to this disease and several structural anomalies of the tricuspid system was evidenced in conjunction or not with the prevailing annular hypodevelopment.
