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1/178. Fatal neonatal hypertrophic cardiomyopathy with severe stenosis of the right ventricular outflow tract: echocardiographic diagnosis and potential therapy.

    We discuss the clinical course of aneonate with idiopathic hypertrophic cardiomyopathy who showed rapid progression of stenosis of the right ventricular outflow tract and reduction in size of the right ventricular cavity due to thicken ing of the ventricular septum. Medical treatment proved unsuccessful. We suggest that balloon atrial septostomy combined with construction of a Blalock-Taussig shunt may be effective in view of the hemodynamics, which mimic pulmonary atresia with intact interventricular septum.
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ranking = 1
keywords = stenosis
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2/178. Subaortic obstruction after the use of an intracardiac baffle to tunnel the left ventricle to the aorta.

    Postoperative hemodynamic studies in five patients document subaortic obstruction after surgical repair utilizing an intracardiac baffle to establish continuity between the left ventricle and the aorta. Four of the patients had a Rastelli procedure for D-transposition of the great arteries with a ventricular septal defect and pulmonary stenosis; one patient had repair of double outlet right ventricle with a ventricular septal defect and pulmonary stenosis. The left ventricular outflow was shown to be a long narrow tunnel by angiography in four of five patients and by echocardiography in one patient. Resting aortic peak systolic pressure gradient ranged from 10 to 42 mm Hg (mean 24). The obstruction was localized to the proximal end of the left ventricule to aorta tunnel (i.e., at the site of ventricular septal defect) in five patients. One patient with a gradient of 42 mm Hg has angina and decreased exercise tolerance. Subaortic obstruction is a newly described sequelae after the Rastelli procedure for transposition or repair of double outlet right ventricle. The obstruction may be hemodynamically significant and should be searched for at postoperative cardiac catheterization.
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ranking = 6.8282093624762
keywords = subaortic, stenosis
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3/178. Pre- and postoperative echocardiographic features of discrete subaortic stenosis.

    In two patients with discrete membranous subaortic stenosis, partial early systolic closure of the aortic valve was noted on the preoperative record. Postoperatively, this abnormality was found to be less pronounced. Narrowing of the left ventricular outflow tract was seen in the preoperative tracing in each patient. Echocardiograms taken after resection of the subaortic membrane showed widening of the left ventricular outflow tract as compared with the preoperative tracing. Thus, echocardiography may be of value in distinguishing between discrete subaortic stenosis and other forms of left ventricular outflow tract obstruction.
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ranking = 46.197465537333
keywords = subaortic, stenosis
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4/178. Fixed left ventricular outflow tract obstruction in presumed hypertrophic obstructive cardiomyopathy: implications for therapy.

    BACKGROUND: A subset of patients presenting with a presumed diagnosis of hypertrophic obstructive cardiomyopathy (HOCM) have a fixed left ventricular outflow tract (LVOT) obstruction. Recognition of this pathophysiologic abnormality is important in choosing therapy. methods: Of patients referred for treatment of HOCM, 4 had fixed LVOT obstruction. Clinical and echocardiographic data and surgical findings were reviewed. RESULTS: In the 4 patients with clinical features consistent with HOCM or HOCM-like conditions, echocardiography showed fixed LVOT obstruction with an early-peaking LVOT Doppler signal or absence of severe systolic anterior motion of the mitral valve. The causes of fixed obstruction included accessory mitral tissue with associated fibrous ring (1 patient), fixed subaortic tunnel stenosis (2 patients), and a discreet subaortic ridge (1 patient). After surgical relief of the fixed LVOT obstruction, all patients had relief of the ventricular outflow tract gradient. CONCLUSIONS: Not all patients with a presumed diagnosis of HOCM have isolated dynamic LVOT obstruction but may have isolated or additional fixed obstruction. Careful two-dimensional and Doppler echocardiography are needed to identify this subset of patients who are best treated surgically.
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ranking = 13.056418724952
keywords = subaortic, stenosis
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5/178. Diffuse angiodysplasia of the upper gastrointestinal tract in a patient with hypertrophic obstructive cardiomyopathy.

    A 64-year-old woman with a known history of hypertrophic obstructive cardiomyopathy presented with severe anemia of unknown origin. She had also suffered from repeated episodes of upper gastrointestinal bleeding for the previous 3 years. Despite bone marrow examination and panendoscopic and angiographic studies, the origin of anemia remained undefined until a small bleeding site was found during a duodenoscopic examination. The lesion proved to be angiodysplasia. This case report is interesting in that angiodysplasia elicited gastrointestinal bleeding and was the cause of anemia. In the international literature, there are very few reported cases of bleeding from gastrointestinal angiodysplasia in association with subvalvular aortic obstruction.
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ranking = 1.4255059529482
keywords = subvalvular
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6/178. Long-term results of apico-aortic valved conduit for severe idiopathic hypertrophic subaortic stenosis.

    We report our long-term results of apico-aortic conduit implantation in patients with isolated idiopathic hypertrophic subaortic stenosis. Between December 1977 and July 1983, apico-aortic prosthetic-valved conduits were implanted in 4 such patients (age range, 24-65 years) who had severe left ventricular hypertrophy and small left ventricular chambers. In this procedure, the distal end of the conduit was anastomosed to the ascending aorta in 3 patients and to the upper abdominal aorta in 1. Postoperative echocardiography showed relief of the left ventricle-aortic gradient and enlargement of the left ventricular chamber in all cases. One patient died of perioperative wound infection. One patient died of unnatural causes 13 years after the initial operation; in his case, the conduit was known to be occluded. Two patients are alive 15 and 19 years, respectively, after the initial operation. Three instances of conduit obstruction due to bioprosthetic calcification were observed. Despite the high incidence of reoperation due to conduit valve failure, apicoaortic conduit implantation has produced good hemodynamic outcome and has improved the quality of life in patients who have idiopathic hypertrophic subaortic stenosis and anatomic features unsuitable for Morrow's operation. Improvements in bioprostheses and in apical implantation techniques may allow a revival of apico-aortic conduit implantation in selected patients with idiopathic hypertrophic subaortic stenosis.
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ranking = 46.397465537333
keywords = subaortic, stenosis
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7/178. angina pectoris after aortic valve replacement.

    angina pectoris after aortic valve replacement may be due to reduced myocardial blood flow (coronary artery stenosis or valvular dysfunction) or to increased myocardial oxygen demand (idiopathic hypertrophic subaortic stenosis or valvular dysfunction). If a patient does not do well after an aortic valve replacement, causes other than dysfunction of the prosthesis should be sought.
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ranking = 6.8282093624762
keywords = subaortic, stenosis
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8/178. Biventricular hypertrophic cardiomyopathy with right ventricular outflow tract obstruction associated with noonan syndrome in an adult.

    This report describes an adult patient with noonan syndrome accompanied by biventricular hypertrophic cardiomyopathy causing isolated right ventricular outflow tract obstruction. Biventricular hypertrophic cardiomyopathy causing right- and/or left-side outflow tract obstruction, as well as valvular pulmonary stenosis, is relatively common in infants with noonan syndrome. However, this condition without a dysplastic pulmonary valve, or indeed any polyvalvular dysplasia, is rare in adults with noonan syndrome. Treatment with a beta-adrenergic receptor blocking agent improved the patient's symptoms. Because neither the etiologic and prognostic relationship nor the genetic linkage between hypertrophic cardiomyopathy associated with noonan syndrome and non-syndromic hypertrophic cardiomyopathy is clearly defined, clinicopathological findings and further follow-up may provide important evidence for the pathogenesis of hypertrophic cardiomyopathy.
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ranking = 0.2
keywords = stenosis
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9/178. Hypertrophic cardiomyopathy with midventricular obstruction and apical aneurysm: a case report.

    A 71-year-old woman had hypertrophic cardiomyopathy associated with midventricular obstruction and an apical aneurysm in the left ventricle. She had had abnormal electrocardiograms for more than 30 years and for the past year had been suffering from occasional attacks of dizziness and low systemic blood pressure. Holter 24-h electrocardiographic monitoring revealed ventricular paroxysmal contractions (676/day) with nonsustained ventricular tachycardia. Doppler echocardiography revealed paradoxical jet flow from the apical aneurysm to the left ventricular outflow during early diastole. magnetic resonance imaging depicted midventricular hypertrophy and a dyskinetic thin apical wall, which were confirmed by angiography. Coronary angiograms showed no narrowing of the major extramural coronary arteries, but there was compression of aberrant coronary arteries apparently feeding the hypertrophic portion of the left ventricular wall. Stress thallium-201 myocardial imaging showed a persistent severe defect in the left ventricular apex. A hemodynamic study revealed low cardiac output and an intraventricular pressure gradient (approximately 90 mmHg) between the left ventricular apical high-pressure chamber and the subaortic low-pressure chamber. The present case represents a rare combination of hypertrophic cardiomyopathy, midventricular obstruction, and an apical aneurysm in an elderly woman. myocardial ischemia may have played an important role in the genesis of the apical aneurysm.
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ranking = 6.4282093624762
keywords = subaortic
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10/178. 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 = 33.141046812381
keywords = subaortic, stenosis
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