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      Caracterización histopatológica de la estenosis subvalvular aórtica: Estudio de 24 biopsias

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          Abstract

          La estenosis subaórtica, es la obstrucción del tracto de salida del ventrículo izquierdo, causada por un exceso de tejido o por anomalías congénitas de cualquiera de las estructuras ubicadas por debajo de la válvula aórtica. Con el objetivo de clasificar un grupo de biopsias de estenosis subaórtica se describen las características macroscópicas e histopatológicas de 24 biopsias provenientes del Hospital Universitario de Caracas, las cuales fueron analizadas con coloraciones de rutinas, coloraciones especiales e inmunohistoquímica, encontrando que la mayoría de los especimenes fueron de aspecto membranoso o fibroso, algunos mostraban tejido muscular. La fibrosis fue el hallazgo histopatológico más constante (100%), degeneración mixoide, músculo liso, fibras elásticas, neoformación vascular y células inflamatorias. Igualmente se observó músculo cardíaco correspondiente al lecho quirúrgico. Concluimos señalando la importancia de un trabajo en conjunto del patólogo con el clínico para clasificar estas lesiones, igualmente, mientras más se conozca de su estructura anatómica, macroscópica e histopatológica, más posibilidades habrá de conseguir evidencias en cuanto a su etiopatogenia.

          Translated abstract

          The subaortic stenosis is the left ventricular outflow tract obstruction caused by an excess of weaving or by congenital anomalies of any of the structures located under the valve aortica. With the objective to classify a group of biopsies, the macroscopic characteristics of subaortic stenosis are described and histopathologic of 24 biopsies proceeding from the University Hospital of Caracas, which they were analyzed with of routines and special histopathologics colorations and inmunohistochemistry. Finding that the majority of the cases they were of membranous or fibrous aspect, some they showed muscular fibers. The fibrosis was the hsitopathologic find more constant (100%), mixoide degeneration, smooth muscle, elastic fibers, neoformation vascular and inflammatory cells. Likewise corresponding heart muscle was observed a surgical bed. We conclude indicating the importance of a work as a group of the pathologist with the clinical one to classify these wounds, likewise, while more be known of its macroscopic, anatomical structure and histopathologic, more possibilities there will be of obtain evidences as for its etiopatogenia.

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          Most cited references33

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          Discrete subaortic stenosis in adults: increased prevalence and slow rate of progression of the obstruction and aortic regurgitation.

          We sought to determine the prevalence and rate of progression of left ventricular outflow tract obstruction (LVOTO) and aortic regurgitation (AR) in adults with discrete subaortic stenosis (DSS).
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            Discrete subaortic stenosis: surgical outcomes and follow-up results.

            Discrete subaortic stenosis, which is an obstructing lesion of the left ventricular outflow tract, remains a surgical challenge. The recurrence rate is high despite sufficient conventional resection. We retrospectively reviewed the results of surgery for discrete subaortic stenosis at our institution from September 1995 through March 2001. Twenty-one patients with this lesion underwent surgical treatment during this period. Excision of the fibromuscular membrane with myectomy was performed in all of the patients. Follow-up in all patients ranged from 7 to 67 months (mean follow-up period, 39.57 +/- 15.46 months). The mean systolic gradient between the left ventricle and the aorta decreased from 59.23 +/- 35.38 mmHg preoperatively to 9.47 +/- 9.91 mmHg postoperatively. There was no instance of heart block that required a permanent pacemaker, nor of bacterial endocarditis. There was no early or late postoperative death. A 22nd patient, who had 3+ aortic regurgitation, required aortic valve replacement and was excluded from the study. Two of the patients (9.5%) underwent reoperation because of recurrent gradient and residual ventricular septal defect. Our results suggest that fibromuscular membrane excision combined with myectomy in patients with discrete subaortic stenosis produces sufficient relief of obstruction with low morbidity.
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              Fixed subaortic stenosis: anatomical spectrum and nature of progression.

              Retrospective echocardiographic review identified 58 consecutive infants and children with fixed subaortic stenosis. Mean (SD) age at diagnosis was 4.8 (3.6) years (range two days to 14.7 years), and diagnosis occurred in infancy in eight. Associated cardiac abnormalities were present in 41 (71%) whereas fixed subaortic stenosis was an isolated lesion in 17 (29%). Four types of fixed subaortic stenosis were identified: short segment (47 (81%)), long segment (7 (12%)), posterior displacement of the infundibular septum with additional discrete narrowing of the left ventricular outflow tract (3 (5%)), and redundant tissue arising from the membranous septum (1 (2%)). Echocardiographic studies had been performed before the diagnosis of fixed subaortic stenosis in nine patients, all with associated abnormalities. These were performed in infancy in each and showed a "normal" left ventricular outflow tract in six and posterior deviation of the infundibular septum in three. In 16 patients serial echocardiographic studies had been performed after the diagnosis of fixed subaortic stenosis but before surgery of the left ventricular outflow tract. Rapid evolution of short segment to long segment narrowing was seen in one patient, and tethering of the aortic valve or mitral valve developed in a further four patients. Aortic valve or mitral valve involvement was not seen before the age of three years but was common thereafter (10/40 patients, 25%). Fixed subaortic stenosis may be an "acquired" lesion with the potential for changes in form as well as progression in severity of left ventricular outflow tract obstruction.
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                Author and article information

                Journal
                rfm
                Revista de la Facultad de Medicina
                RFM
                Universidad Central de Venezuela. Facultad de Medicina. Comisión de Publicaciones de la Facultad de Medicina (Caracas, Distrito Capital, Venezuela )
                0798-0469
                June 2006
                : 29
                : 1
                : 29-37
                Affiliations
                [02] orgnameUCV orgdiv1Instituto Anatomopatológico orgdiv2Sección de Patología Cardiovascular
                [01] orgnameUCV orgdiv1Instituto Anatomopatológico orgdiv2Sección de Patología Cardiovascular
                Article
                S0798-04692006000100005 S0798-0469(06)02900105
                0c049095-5c72-41d6-9d5e-f5b266949391

                http://creativecommons.org/licenses/by/4.0/

                History
                : 13 January 2006
                : 27 March 2006
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 32, Pages: 9
                Product

                SciELO Venezuela

                Categories
                Trabajos Originales

                Subaortic stenosis,Membrana subaórtica,Estenosis subvalvular aórtica

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