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1/82. Critical subaortic stenosis in a newborn caused by accessory mitral valve tissue.

    A 2-week-old newborn girl underwent successful surgery in our clinic for critical subaortic stenosis caused by accessory mitral valve tissue, which, because of excessive growth, protruded into the left ventricular outflow tract. The preoperative pressure gradient below the aortic valve was 80 mm Hg. The operation consisted of resection of the accessory tissue through a combined aortotomy and atriotomy approach without residual pressure gradient and mitral valve incompetence. This approach is recommended to ensure that accessory tissue is removed without damaging the mitral valve.
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ranking = 1
keywords = subaortic, stenosis
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2/82. Discrete subvalvular aortic stenosis in adults.

    Discrete subvalvular aortic stenosis is a relatively rare condition in adults. It is often diagnosed during first decade of life especially in association with other congenital malformations. Isolated form of discrete subvalvular aortic stenosis may however silently progress from innocent murmurs of childhood and adolescence to symptomatic left ventricular outflow tract obstruction in adults. Certain overt and subtle morphological abnormalities may underlie the initial expression as well as high recurrence rates after surgical resection of sub aortic membrane. Though surgical resection is the only treatment available, debate on the surgical technique and appropriate timing of surgery continues. Close followup with serial echocardiographic examinations in patients detected to have functional murmurs during childhood may be helpful in early detection of subvalvular aortic stertosis.
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ranking = 6.0937907034913
keywords = subvalvular, stenosis
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3/82. Discrete subaortic membranous stenosis--a case report.

    Isolated subaortic stenosis is a rare type of cardiac anomaly which has been characterized as having two types: the discrete type, including membranous or fibromuscular, and the tunnel type. In the discrete type, a crescent-shaped, fibrous curtain is attached to the ventricular septum or completely encircles the left ventricular outflow tract and can be located anywhere from immediately below the aortic valve to 10 mm or more into the body of ventricle. A 22-year-old female presented at our hospital with a divided PDA, a murmur that was found by incident and progressive exertional dyspnea. echocardiography revealed left ventricular hypertrophy, moderate aortic insufficiency, and severe aortic stenosis characterized by a thickened aortic valve and membranous type subaortic stenosis with a transmembranous high pressure gradient, 121 mmHg. An operation to replace the aortic valve and excise the membranous collar was performed with cardiopulmonary bypass support. The patient did well during the postoperative follow-up period. If the preoperative LVOT pressure gradient had been higher than 45 mmHg, the incidence of recurrent stenosis, progression of aortic regurgitation and the need for reoperation would have been higher. In order to prevent this from happening, we chose to replace the defective valve with a mechanical valve and enucleate the discrete lesion.
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ranking = 1.2394841047827
keywords = subaortic, stenosis
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4/82. Hypertrophic subaortic stenosis complicated by high degree heart block: successful treatment with an atrial synchronous ventricular pacemaker.

    The development of high degree atrioventricular block in a patient with hypertrophic subaortic stenosis underscores the importance of a properly timed atrial contraction in this disorder. Atrial synchronous ventricular pacing therapy, by providing, reliably timed atrial systole and increased left ventricular end-diastolic volume, has an important role in this patient and in similar cases.
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ranking = 1
keywords = subaortic, stenosis
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5/82. Accessory mitral valve tissue in combination with subaortic membrane and significant impairment of left ventricular function.

    A 6-year-old girl was found to have a combination of accessory mitral valve tissue and subaortic membrane. The association caused left ventricular outflow tract obstruction leading to severe left ventricular dysfunction. Surgical relief of the obstruction resulted in immediate improvement of ventricular function.
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ranking = 0.90128973804325
keywords = subaortic
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6/82. Subaortic stenosis diagnosed in the course of a twins pregnancy: a controversial management.

    Fixed subaortic stenosis, commonly associated with other congenital cardiac defects, is the cause of 10 per cent of cases of congenital obstruction of the left ventricular outflow. Corrective surgery is frequently a successful treatment, recommendations being based on the transaortic gradient in europe while in the USA the most prevalent opinion is surgical repair independently of the gradient. We present a case of adult clinical onset of a fixed subaortic stenosis during pregnancy, in which hemodynamic changes are significant, that was medically treated and followed in the outpatient clinic of our hospital, and review the state of the art of the management and surgical indications of this condition.
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ranking = 0.4789682095654
keywords = subaortic, stenosis
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7/82. Idiopathic hypertrophic subaortic stenosis in a patient with mirror-image dextrocardia.

    A 42-year-old man with mirror-image dextrocardia was found to have idiopathic hypertrophic subaortic stenosis. The coexistence of these uncommon congenital cardiovascular anomalies was previously reported in only one patient.
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ranking = 1
keywords = subaortic, stenosis
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8/82. Retroaortic innominate vein with coarctation of the aorta: surgical repair and embryology review.

    A newborn girl with a retroaortic innominate vein, coarctation of the aorta, ventricular septal defect, and subaortic stenosis underwent a complete repair at 8 days of age. The ascending aorta was transected and the innominate vein was brought in front of it. We review the anatomy and embryology of this rare entity and describe the surgical technique to avoid recoarctation and innomante vein compression.
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ranking = 0.2
keywords = subaortic, stenosis
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9/82. Aortoventriculoplasty: a new technique for the treatment of left ventricular outflow tract obstruction.

    Aortoventriculoplasty is a new method of treatment for left ventricular outflow tract obstructions. The concept is based on creating a surgical defect which is patched in such a way as to provide the largest possible outflow to the left ventricle. The incision of the aorta continues down as far as necessary, with the right ventricular wall, the aortic ring, and the septum being cut. Reconstruction with an inner Dacron patch on the septum is completed by replacing the aortic valve with an adequate prosthesis, covering the aortic incision with the same patch, and patching the right venticular opening with an outer patch. This method was used in 4 children with tunnel-like subaortic stenosis, 3 of whom had had unsuccessful previous surgical attempts. Other associated lesions including parachute mitral valve were also corrected during aortoventriculoplasty. Hemodynamic results were excellent following this operation. Two patients died postoperatively, one from advanced myocardial damage and progressive failure and the other from cerebral ischemia caused by insufficient retrograde perfusion through an aortic coarctation that was not repaired earlier. No arrhythmias were observed following the procedure. The other 2 patients are well 7 and 5 months postoperatively with excellent hemodynamic function.
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ranking = 0.2
keywords = subaortic, stenosis
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10/82. Hypercontractile cardiac states simulating hypertrophic cardiomyopathy.

    Hypertrophic cardiomyopathy (HCM) or idiopathic hypertrophic subaortic stenosis (IHSS) has been defined as an autosomal dominant myocardial disease characterized by specific physical findings, echocardiographic features, asymmetric septal hypertrophy and disordered myocardial architecture. Echocardiographic and scintiphotographic studies failed to reveal evidence of asymmetric septal hypertrophy in four patients with systolic anterior movement of the mitral valve and the typical ausculatory and peripheral pulse abnormalities characteristic of HCM. Postmortem examimination in one patient demonstrated mild concentric left ventricular hypertrophy and a normal arrangement of myocardial muscle fibers. These observations in four patients demonstrate that both systolic anterior movement of the mitral valve and the physical findings characteristic of HCM are not specific for the autosomal dominant myocardial disease characterized by asymmetric septal hypertrophy and abnormal septal histology.
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ranking = 0.2
keywords = subaortic, stenosis
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