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1/16. Considerations in biventricular repair after the Norwood procedure.

    OBJECTIVE: The Norwood procedure can be applicable as a first stage palliation in children who can eventually undergo a biventricular repair. Although usual management of these patients is a primary neonatal repair, in selected patients staged approach with a Norwood procedure in the neonatal period followed by a Rastelli procedure in the infancy for conversion to two-ventricle physiology has been used alternatively. methods: We report our experiences on two infants who underwent a previous palliation with the Norwood procedure for lesions other than hypoplastic left heart syndrome and converted to two-ventricle physiology by the use of a Rastelli-type procedure. This report examines considerations in biventricular repair after the Norwood procedure especially need for ventricular septal defect enlargement and approach to placement of the right ventricle to pulmonary artery conduit. RESULTS: Both of the infants who underwent staged approach with an initial Norwood procedure for lesions other than hypoplastic left heart syndrome survived the operations and were clinically well at mid-term follow-up. CONCLUSION: In selected patients, the staged approach is an alternative in management of malformations other than hypoplastic left heart syndrome which share the important physiologic features of aortic outlet obstruction and ductal dependency of systemic circulation. We recommend routine enlargement of ventricular septal defect and proper positioning of the conduit at the time of subsequent biventricular repair. ( info)

2/16. Accelerated ventricular rhythm in children: a review and report of a case with congenital heart disease.

    We report a child with accelerated ventricular rhythm (AVR) and congenital heart disease. Three children with congenital heart defect associated with AVR were previously reported, but in each AVR occurred only postoperatively. Because our patient's 24-hour electrocardiograph recording showed AVR rates, and differences between sinus and AVR rates, exceeding published childhood limits, we reviewed the topic. On the basis of our review, we suggest guidelines for diagnosing AVR and differentiating it from ventricular tachycardia. ( info)

3/16. Marfan's syndrome, dextrocardia and situs inversus associated with discrete subaortic stenosis and aortic insufficiency in an adult female: case report.

    Marfan's syndrome is an inherited connective tissue defect that affects many organs, especially of the musculoskeletal, ophthalmic and cardiovascular systems, and may be associated with some rare conditions. Here, we report the first known case of Marfan's syndrome, combined with situs inversus totalis with dextrocardia and discrete subaortic stenosis and aortic insufficiency in a 22-year-old woman. ( info)

4/16. Proximal isovelocity surface area (PISA) in the evaluation of fixed membranous subaortic stenosis.

    The evaluation of the severity of subaortic stenosis is usually expressed by the magnitude of the subvalvular gradient. Calculation of the membrane orifice area noninvasively is difficult by the standard means. We present a patient in whom the area was calculated using the proximal isovelocity surface area (PISA) method. This method should have clinical applicability because it is not flow dependent and can be used in patients with normal, reduced, or increased stroke volume. ( info)

5/16. Mitral annular aneurysm resulting from subaortic muscle resection.

    We present a case of mitral annular aneurysm as a very rare complication developing soon after the surgical relief of subaortic stenosis. The cause of the aneurysm was considered to be the disruption of aorto-mitral intervalvular fibrosa at the initial operation. The surgical repair was successfully performed without compromising either aortic or mitral valvular functions. The preoperative transesophageal echocardiography was useful in delineating the precise anatomic features of this rare complication. ( info)

6/16. Staged biventricular repair of Taussig-Bing anomaly with subaortic stenosis and coarctation of aorta.

    We present successful procedures for 2 infants who had the Taussig-Bing anomaly with subaortic stenosis and coarctation of the aorta. The initial procedure was coarctoplasty and the Damus-Kaye-Stansel procedure with modified Blalock-Taussig shunt. The second procedure was intraventricular repair (Kawashima procedure), Damus-Kaye-Stansel take-down and the reuse of native aortic and pulmonary valves 19 and 25 months later. Both patients survived the operations and postoperative hemodynamics were excellent at both 28 and 59 months follow-ups. ( info)

7/16. Live three-dimensional transthoracic echocardiographic identification of discrete subaortic membranous stenosis.

    We describe an adult patient in whom live three-dimensional transthoracic echocardiography illustrated the exact site and full extent of the subaortic membrane as well as the narrow opening within the membrane, indicative of severe stenosis. To our knowledge this has not been reported previously. ( info)

8/16. Transnasal placement of biplane transesophageal echocardiography probe intraoperatively in an adolescent with congenital heart disease.

    Intraoperative transesophageal echocardiography (TEE) is frequently used in children with congenital heart disease (CHD). Although transnasal TEE is being used in various settings in the adult population, there are no descriptions of its use intraoperatively in patients with CHD. This report describes the successful use of transnasal TEE after multiple unsuccessful transoral attempts in an adolescent male undergoing subaortic stenosis repair. IMPLICATIONS: Transnasal transesophageal echocardiography (TEE) is being used in various settings in the adult population. The author describes its use intraoperatively in an adolescent undergoing surgery for congenital heart disease after unsuccessful transoral attempts. ( info)

9/16. Cystic subaortic discrete membrane in an elderly woman.

    A discrete subaortic membrane is an uncommon cause of left ventricular outflow tract (LVOT) obstruction in patients of advanced age. Here, a significant LVOT obstruction caused by a discrete membrane is reported in a 64-year-old woman. On echocardiographic examination the membrane had a cystic appearance. To the present authors' knowledge, this is the first reported case of a cystic subaortic membrane. ( info)

10/16. Subaortic stenosis produced by an accessory mitral valve: the role of echocardiography.

    Subaortic stenosis caused by an accessory mitral valve is an exceedingly rare finding. We report the case of an asymptomatic 14-year-old patient, in whom transthoracic echocardiography revealed an accessory mitral valve in the left ventricular outflow tract, producing mild subaortic stenosis. Except for an aneurysm of the interventricular septum, with no shunt, there were no other anomalies. Transesophageal echocardiography provided details about the morphology and location of the accessory valve. Being asymptomatic and having only a mild gradient, antibiotic prophylaxis for infective endocarditis and follow-up were recommended. After 2 years the patient is asymptomatic, with a similar echocardiographic gradient. ( info)
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