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1/95. Familial hypertrophic cariomyopathy and lentiginosis.

    Members of three generations of a family studied, manifest profuse lentiginosis and hypertrophic cardiomyopathy. The autosomal dominant inheritance of this syndrome is established. Lentiginosis should alert the physician to possible underlying heart disease. ( info)

2/95. 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. ( info)

3/95. 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. ( info)

4/95. 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. ( info)

5/95. 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. ( info)

6/95. The functional defect in amyloid heart disease. The "stiff heart" syndrome.

    Left ventricular performance was studied in three patients with heart failure due to amyloid deposits. The diagnosis of amyloidosis was proved by cardiac biopsy in two patients and by rectal biopsy in the third. One patient had myelomatosis, but the other two had no other identifiable disease. The investigative technique allowed simultaneous measurements of pressure and volume in the left ventricle. The functional defect with slow cardiac filling at high pressure and greatly reduced left ventricular contraction differed from that of constrictive pericarditis and other heart muscle disease. These features of a "stiff heart" are probably unique to amyloidosis and should make possible positive recognition of the condition on the basis of echocardiographic, angiographic and hemodynamic findings. ( info)

7/95. 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. ( info)

8/95. 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. ( info)

9/95. 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. ( info)

10/95. 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. ( info)
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