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1/42. aortic valve stenosis with left ventricular outflow tract pressure gradient.

    A 61-year-old man was diagnosed with severe aortic valve stenosis with left ventricular outflow tract pressure gradient due to systolic anterior movement of the mitral valve and a large poststenotic dilation of the ascending aorta. He underwent successful aortic root replacement and concomitant septal myectomy.
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2/42. Hypertrophic cardiomyopathy with mid-ventricular obstruction and splenic infarction associated with paroxysmal atrial fibrillation: a case report.

    A 54-year-old woman had been treated for hypertrophic cardiomyopathy and paroxysmal atrial fibrillation since 1992. She was admitted with paroxysmal atrial fibrillation which was resolved by medical treatment. However, on the next day, left lateral chest pain appeared. Computed tomography disclosed a low density area in the spleen. She received anticoagulant therapy under a diagnosis of splenic infarction, and the pain disappeared. echocardiography showed hypertrophic cardiomyopathy with mid-ventricular obstruction. She was treated with cibenzoline to prevent paroxysmal atrial fibrillation attack and attenuate the hemodynamic load. After treatment, the pressure gradient decreased from 41 to 7 mmHg. This patient with hypertrophic cardiomyopathy suffered a rare isolated splenic infarction associated with paroxysmal atrial fibrillation.
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3/42. Intra-aortic balloon pump associated with dynamic left ventricular outflow tract obstruction after valve replacement for aortic stenosis.

    An unstable patient with critical aortic stenosis had an intra-aortic balloon pump placed preoperatively for hemodynamic support and alleviation of symptoms. After separation from cardiopulmonary bypass following aortic valve replacement, the patient was hypotensive with increased pulmonary artery pressures. Transesophageal echocardiography revealed left ventricular outflow tract obstruction associated with systolic anterior motion of the mitral valve and severe mitral regurgitation. This pathophysiology was present when ventricular systole was preceded by balloon counterpulsation, but was absent during unassisted systole. This case report demonstrates a potentially significant untoward effect of intra-aortic balloon pump augmentation after aortic valve replacement for aortic stenosis. The timely diagnosis of this iatrogenic condition in the operating room permitted the prompt implementation of appropriate management strategies and avoided unnecessary surgical intervention.
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4/42. mitral valve repair and septal myectomy for hypertrophic obstructive cardiomyopathy.

    Combined mitral valve repair using the sliding leaflet technique and septal myectomy were employed to successfully treat left ventricular outflow tract (LVOT) obstruction and mitral regurgitation due to hypertrophic obstructive cardiomyopathy (HOCM). A 46-year-old man was diagnosed with HOCM along with congestive heart failure and was treated medically. These symptoms, however, were resistant to medical treatments with a beta-blocker, a Ca-antagonist, and disopyramide, and he was referred to our hospital for surgery. Doppler echocardiography demonstrated an LVOT obstruction at rest with a peak pressure gradient of 138 mmHg. The interventricular septum thickness was 14 mm. Mitral regurgitation of 3 with severe SAM was also observed. Temporary dual chamber pacing was tried without significant improvement. Following these examinations, the patient underwent surgery. A transaortic septal myotomy-myectomy was performed first, and the mitral valve was then approached through the left atrium. mitral valve repair was performed with the sliding leaflet technique to reduce the height of the posterior leaflet from 2 cm to 1 cm. Postpump transesophageal echocardiography revealed no MR and a peak LVOT gradient of 15 mmHg. The patient recovered well except for a residual mild SAM, and MR2 . We therefore concluded that this surgical approach might provide results which are superior to those of myectomy alone.
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5/42. Double-chambered right ventricle presenting in adulthood.

    BACKGROUND: Double-chambered right ventricle is a form of right ventricular outflow tract obstruction that develops over time, often in patients with an abnormally short distance between the moderator band and pulmonary valve. This lesion typically presents in childhood or adolescence and is often accompanied by a ventricular septal defect. Only a handful of previous cases have been described in which double-chambered right ventricle occurred in adulthood. methods: Since 1992, three patients more than 30 years old (38, 43, and 66 years of age) have presented at our institution with unusual symptoms or a previous incorrect diagnosis. We reviewed the clinical data in these patients. RESULTS: Presenting symptoms included syncope, angina, and severe dyspnea resembling pulmonary hypertension. In 1 patient, disease was categorized as new york Heart association class IV, and in the other 2 as class III. Coexisting anomalies included a patent foramen ovale or secundum atrial septal defect in 2 patients, a small ventricular septal defect in 1 (with a probable history of ventricular septal defect in another), and mild aortic regurgitation in 1. All patients required urgent or emergent operations, with peak pressures in the proximal right ventricular chamber of 135 to 180 mm Hg and severely depressed left ventricular function in 1 patient. Resection of the anomalous right ventricular muscle bundles was achieved through a right atrial approach in all patients. All patients were alive with improved functional status at follow-up, which was between 15 and 40 months. CONCLUSIONS: Right ventricular outflow tract obstruction resulting from a double-chambered right ventricle is rare in adults, but when it does occur it can present with unusual symptoms. When evaluating the patient with signs or symptoms of primary right heart failure, cardiologists should make an effort to image the entire right heart complex. Subcostal echocardiography can facilitate adequate visualization of the right ventricle when it is difficult to distinguish the subpulmonary outflow tract from the parasternal and apical windows.
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6/42. Apical aneurysm and left ventricular hypertrophy.

    A 59-year-old woman presented with an embolic transient ischemic attack and a history of controlled hypertension for 16 years. Both echocardiogram and MRI showed severe biventricular hypertrophy and an apical aneurysm with a thrombus. The occurrence of an apical aneurysm in the presence of cardiac hypertrophy is a rare finding and has been described in patients with hypertrophic cardiomyopathy. However, it has not been reported in patients with systemic arterial hypertension. In this patient the lack of a relationship between the severity of the hypertrophy and the levels of blood pressure, together with the presence of histologic disorganization of myocardial cardiac muscle cells by endomyocardial biopsy suggested the diagnosis of hypertrophic cardiomyopathy.
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7/42. Sinoatrial nodal artery aneurysm with right ventricular outflow tract compression: report of a case.

    We described a 16-year-old boy with sinoatrial nodal (SAN) artery aneurysm that drained into right atrium and compressed right ventricular outflow tract. The patient was clinically asymptomatic. Hemodynamic study revealed a 15 mm Hg peak systolic pressure gradient at right ventricular outflow tract. The fistula was successfully excised without sequalae. Cathet. Cardiovasc. Intervent. 51:328-331, 2000.
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8/42. 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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9/42. Obstruction of the left ventricular outflow tract after mitral valvuloplasty with implantation of Gregori's ring.

    Mitral valvuloplasty is efficient for repairing mitral valve disease with few complications. In some cases, obstruction of the left ventricular outflow tract may occur due to systolic anterior motion of the mitral valve. We report the case of a patient with this complication and a pressure gradient between the left ventricle and the aorta of 130 mm Hg after mitral valvuloplasty with implantation of a Gregori's ring. The management was clinical with suspension of the vasoactive drugs and introduction of a beta-blocker. Two years after the surgery, the patient is asymptomatic and has a normal life.
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10/42. recurrence of left ventricular outflow tract obstruction after left anterior descending coronary artery revascularization in a patient with hypertrophic obstructive cardiomyopathy.

    A patient with known hypertrophic obstructive cardiomyopathy presented with an anteroseptal myocardial infarction which resulted in the disappearance of his subaortic pressure gradient. Surgical revascularization of his left anterior descending coronary artery after the viability of his myocardium had been documented led to the recurrence of his left ventricular outflow tract obstruction and subaortic pressure gradient.
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