Cases reported "Aortic Valve Stenosis"

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1/96. Stenting of the aorta for recurrent, long stenosis due to Takayasu's arteritis in a child.

    Balloon angioplasty followed by stenting of the thoracic aorta is reported in a 5-year-old girl with Takayasu's arteritis, who presented with severe hypertension and congestive heart failure. Her aortogram showed severe long segment stenosis of the descending thoracic aorta, which was successfully treated by balloon angioplasty on two occasions, but developed recurrences after 6 and 7 months of angioplasty. Balloon dilatation of the stenosed aorta, followed by sequential implantation of three 30-mm long Palmaz stents in tandem, bridging the full segment of the angioplastied aorta were performed successfully without any complications. The aortic diameter increased from 3.3 mm to 7.7 mm and the peak systolic pressure gradient decreased from 75 mmHg to 3 mmHg. There was no recurrence on clinical follow-up of 13 months.
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2/96. 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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3/96. Alkaptonuric aortic stenosis: a case report.

    alkaptonuria is a rare disease of phenylalanine, aromatic amino acids, and tyrosine metabolism. Because of a genetic deficiency of the enzyme homogentisic acid oxidase, an accumulation of homogentisic acid causes ochronotic pigment deposition. The most common clinical manifestations are arthropathy, urinary calculi and discoloration, cutaneous and cartilaginous pigmentation, and cardiac valvular disease. Arthropathy and aortic stenosis are the most debilitating manifestations of the disease. A case of alkaptonuric aortic stenosis is described. A 75-year-old woman with a history of alkaptonuria presented in the emergency department with complaints of progressive dyspnea. Upon examination, the patient was hypertensive, tachypneic, and tachycardic with premature ventricular contractions. She had pitting edema of the lower extremities and complaints of generalized weakness. Chest x-rays revealed congestive heart failure and pulmonary edema. diuretics were administered, and a continuous nitroglycerin infusion was initiated in the emergency department. The patient was admitted for further evaluation. The patient's respiratory status continued to decline. She was intubated endotracheally 1 day after admission. Subsequent cardiac evaluation revealed an ejection fraction of 35%, severe aortic stenosis, mild coronary artery disease, ischemic cardiomyopathy, and anteroapical akinesis. A dobutamine infusion was instituted for persistent hypotension, and renal dose dopamine was initiated for oliguric renal failure. The patient underwent an emergency operation for an aortic valve replacement with a Dacron patch 10 days after admission. cardiopulmonary bypass and mild hypothermia were used during the procedure. The patient's hemodynamic status remained tenuous throughout the procedure. Although the first attempt to wean off cardiopulmonary bypass failed, the second attempt was successful with the aid of an intra-aortic balloon pump, inotropic support, and atrioventricular pacing. These measures were maintained during transport to the surgical intensive care unit. In the intensive care unit, the patient did not have an audible blood pressure or a palpable pulse without the support of the intra-aortic balloon pump and atrioventricular pacing. Coarse atrial fibrillation was the underlying electrocardiogram rhythm in the absence of atrioventricular pacing. sodium bicarbonate was given without improvement. After discussion with the family, all life support measures were discontinued. The patient died 10 minutes after her arrival in the intensive care unit. alkaptonuria's pathogenesis is manifested as both local and systemic in nature. collagen vascular diseases share a similar pattern of multisystem involvement. Despite the negative outcome for the patient described, valuable insight can be obtained by studying this case and noting the anesthetic considerations specific to collagen vascular diseases in general.
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4/96. Pulmonary vein atresia with Shone's anomaly in an infant: a case report.

    We report a case of individual pulmonary vein atresia associated with multiple levels of left heart obstruction, including aortic coarctation, valvular aortic stenosis, and parachute mitral valves with stenosis. The diagnosis of pulmonary vein obstruction is likely to be missed in patients who also have other left heart obstructive diseases, since the latter usually dominates the clinical presentation. We diagnosed the existence of individual pulmonary vein atresia preoperatively via cardiac catheterization. The pulmonary artery angiograms revealed back and forth motion of the dye with no visualization of either a capillary or venous phase on the lesion side. The pulmonary capillary wedge pressure was unevenly elevated and highest on the lesion side. The results were later confirmed by operation and autopsy. Thus, selective pulmonary artery catheterization and angiography remains a good diagnostic tool to rule out the existence of pulmonary vein obstruction in cases which have multiple levels of left heart obstruction.
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5/96. Living related donor liver transplantation in a patient with severe aortic stenosis.

    We report the successful anaesthetic management of a young girl with Alagille's syndrome and severe aortic stenosis (resting pressure gradient 88 mm Hg) undergoing living related donor liver transplantation (LRDLT). The patient had end-stage liver disease and LRDLT was performed before replacement of the aortic valve. Anaesthesia was conducted uneventfully with the aid of a pulmonary artery catheter. intra-aortic balloon pumping was used in the perioperative period for protection against myocardial ischaemia. Total clamping of the inferior vena cava was avoided during surgery and volume administration was guided by the pulmonary artery pressure. A stable circulation was maintained in the reperfusion period. The patient was discharged from hospital on day 54 after operation with normal liver function. Two years later her aortic valve was replaced successfully.
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6/96. Anterograde double-balloon valvoplasty for treatment of severe valvar aortic stenosis in a preterm baby weighing 1400 grams.

    We describe our treatment of a premature baby born weighing 1400 g with severe aortic stenosis, with a gradient of 80 mmHg across the valve. Efforts to advance a 6 mm angioplasty catheter into the stenotic aortic valve via the left ventricle failed. Anterograde angioplasty, instead, was performed using two 4 mm coronary angioplasty catheters. Six months subsequent to the intervention, the pressure gradient measured 25 mmHg, and there was no hemodynamically significant aortic insufficiency.
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7/96. 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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8/96. A novel use of Amplatzer duct occluder.

    This report describes the use of the Amplatzer patent ductus arteriosus occluder to close a left ventricle to descending aorta conduit. The patient was a 10-year-old male who was born with critical aortic stenosis and left ventricular outflow tract obstruction. After initial valvotomy, he underwent left ventricular to descending aorta conduit placement. At the age of 10, he had a Konno procedure to enlarge the left ventricular outflow tract and 21-mm St. Jude aortic valve placement. Closure of the conduit was not addressed because it was inaccessable from median sternotomy. Postoperatively, echocardiogram revealed significant flow through the conduit with a wide pulse pressure. cardiac catheterization was performed with the premise to close the conduit with an Amplatzer patent ductus arteriosus occluder device.
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9/96. Symptomatic calcific stenosis of a Toronto stentless porcine valve.

    We describe the calcific structural failure of a Toronto stentless porcine valve (TSPV) which had been used to replace a calcified bicuspid aortic valve in a 46-year-old man. Against expectations, left ventricular hypertrophy persisted and the transvalvular pressure gradient rose to 125 mmHg by 6 years with the patient becoming symptomatic and requiring redo surgery. On removal the TSPV showed atypical calcification of the leaflet hinges and wall. To our knowledge this is the first case reported and it may have implications for long term durability and future surgery using this prosthesis.
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10/96. Ultrasonic aortic valve decalcification.

    We report a case of calcific aortic stenosis in a 79-year-old man who had undergone aortocoronary bypass. Since ordinary valve replacement was problematic because of severe annular calcification, a small annular diameter, and three patent vein grafts, we performed ultrasonic aortic valve decalcification. As a result, the pressure gradient across the aortic valve decreased from 100 mmHg to 25 mmHg, and the patient is doing well to date at two years after the operation. Although long-term results of ultrasonic aortic valve decalcification are still undetermined, it appears worth considering in cases in which ordinary valve replacement could be extremely difficult.
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