Cases reported "Heart Valve Diseases"

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1/16. Bilateral diaphragmatic paralysis after open heart surgery.

    The purpose of the present case report is to present a case of bilateral diaphragmatic paralysis as a complication of open-heart surgery. A 47-year-old male was operated for aortic and mitral valve replacement. After discontinuation of sedation, bilateral diaphragmatic paralysis as well as motor and sensitive dysfunction in the four extremities was observed. The patient remained with mechanical ventilation support for twenty months. Two years after the operation a complete normalisation of the diaphragmatic motion was observed. Although uncommon, bilateral diaphragmatic paralysis after open-heart surgery could take place, being necessary long term mechanical ventilation support until recovery.
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2/16. Pseudo-false-positive exercise treadmill testing caused by systolic anterior motion of the anterior mitral valve leaflet.

    Subendocardial ischemia as indicated by electrocardiography during exercise, in association with severe systolic anterior motion of the anterior mitral valve leaflet without left ventricular hypertrophy, has not been well described. We report the case of a 42-year-old man who presented with symptoms of exertional angina and 2-mm ST depression on treadmill electrocardiography but had a normal perfusion scan and coronary angiogram. Initially the negative angiographic results caused us to regard the treadmill results as false-positive. Subsequently, low-dose dobutamine echocardiography showed severe systolic anterior motion of the anterior mitral valve leaflet with a >144-mmHg left ventricular outflow tract gradient; we then recognized the original treadmill results to be pseudo-false-positive. Electrocardiographic changes in association with the above-described motion of the anterior mitral valve leaflet and increased left ventricular outflow tract gradient were verified by use of treadmill and supine bicycle stress echocardiography.
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3/16. Infective endocarditis with perivalvular pseudoaneurysm.

    A 16-year-old Japanese woman with infective endocarditis was admitted to hospital. An echocardiography exhibited a perivalvular pseudoaneurysm just under the mitral valve. Left ventriculography demonstrated that the aneurysm had a small neck and dyskinetic motion with oppression to the coronary sinus and right coronary artery.
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4/16. Successful thrombolysis of st. Jude medical aortic prosthesis with tissue-type plasminogen activator in a pregnant woman: a case report.

    Mechanical valve thrombosis is a life-threatening event. pregnancy is associated with a hypercoagulable state that further emphasizes the importance of adequate anticoagulation. This is associated with a therapeutic dilemma. Continued anticoagulation with warfarin throughout the first trimester can result in fetopathic effects, while replacement of warfarin by heparin between 6 and 12 weeks of gestation does not completely prevent the risk of valve thrombosis. There are a small number of reported cases of pregnant women with prosthetic heart valve thrombosis under low molecular weight heparin and consecutive lytic therapy. The authors report a 33-year-old pregnant woman with a St. Jude Medical aortic prosthesis, anticoagulated with a therapeutic dosage of low molecular weight heparin from 6 weeks of gestation, who developed prosthetic heart valve thrombosis at 17 weeks of gestation. A thrombolysis with recombinant tissue-type plasminogen activator (50 mg for 2 hours) was performed. Under thrombolysis, ST-segment elevation in leads II, III, aVF, V5, and V6 developed electrocardiographically with a maximal creatine kinase (CK) of 349 U/L (CK-MB isoenzyme of 48 U/L). echocardiography revealed normal function of the St. Jude Medical aortic prosthesis 2 hours after thrombolysis and normal wall motions. Short-course thrombolytic therapy appears to be an effective alternative to surgical intervention for the treatment of thrombotic dysfunction of valve prostheses in pregnancy.
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5/16. Echocardiographic manifestations of aortic cusp rupture in a myxomatous aortic valve.

    A 16-year-old pregnant black girl who had spontaneous perforation of the aortic valve associated with myxomatous aortic valvular degeneration is presented. The echocardiogram revealed chaotic systolic motion of one of the aortic cusps, diastolic aortic valvular fluttering, and abnormal diastolic echoes in the left ventricular outflow tract. The report illustrates that the echocardiographic features associated with valvular vegetations are not specific for infectious endocarditis.
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6/16. Valve adaptation as a cause of disc opening reduction in mechanical heart valves: the case of the Lillehei-Kaster valve.

    At fluoroscopy a decreased disc motion in mechanical heart prostheses is often a sign of valve thrombosis. On occasion, however, despite an exhaustive diagnostic work-up, common causes of prosthetic valve thrombosis are not found. In these cases the valve disc abnormalities are thought to be due to functional changes. We here report our experience with 5 consecutive patients carrying the Lillehei-Kaster prosthesis who had this fluoroscopic finding that was lately attributed to "valve physiologic adaptation". The time of onset, differential diagnosis and clinical/hemodynamic impact of valve adaptation are discussed.
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7/16. Echocardiographic evidence of outflow tract obstruction in Pompe's disease (glycogen storage disease of the heart).

    A 7 month old black female infant with the clinical findings of Pompe's disease is presented. The diagnosis of an infiltrative myocardiopathy with left ventricular outflow tract obstruction presenting with a pronounced systolic anterior motion of the anterior mitral valve leaflet was made by echocardiography. This diagnosis was confirmed by cardiac catheterization and angiocardiography. Pathologic findings were consistent with Pompe's disease (type II glycogen storage disease). The presence of systolic anterior motion of the mitral valve in this patient suggests that this finding is not pathognomonic of idiopathic hypertrophic subaortic stenosis.
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8/16. prenatal diagnosis of transposition-like double-outlet right ventricle with mitral valve atresia in heterotaxy syndrome.

    We present a case of heterotaxy syndrome with right isomerism detected in a twin fetus, with the sonographic findings that established this diagnosis. At 24 weeks' menstrual age, ultrasound detected anomalies in the heart suggestive of heterotaxy syndrome. Intrauterine fetal echocardiography demonstrated that the heart of the affected twin was located on the left side with a normal cardiac axis. The right ventricle was much larger than the left ventricle. The appearance of the mitral valve was dysplastic with restricted motion. The ascending aorta exited from the right ventricle, anterior to, right to, and parallel to the smaller pulmonary trunk, which also arose from the right ventricle. Double-outlet right ventricle (DORV) was clearly demonstrated. cesarean section was performed at 38 weeks' menstrual age. Both twins were healthy and survived. Postnatal echocardiography and MRI confirmed the prenatal findings. At the age of 4 months, the affected infant had no evidence of cardiac decompensation. This report shows that conventional ultrasound can be used to detect heterotaxy syndrome and that intrauterine fetal echocardiography can delineate the cardiac complex in heterotaxy syndrome. Postnatal surgery with complex Fontan procedures was planned for the affected twin.
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9/16. Abnormal left ventricular catheter motion: an ancillary angiographic sign of left atrial myxoma.

    The normal motion of a left ventricular catheter parallels that of the aortic root; it moves anterior during systole and posterior during diastole. In contrast, a prolapsing left atrial myxoma causes paradoxical motion of the catheter; posterior during systole and anterior during diastole. Paradoxical motion was found in each of five cases of prolapsing left atrial myxoma (no false negatives), and in six out of 61 controls (six false positives). In the false positive cases, the catheter was not positioned on the ventricular floor and usually only minor degrees of abnormal motion were present. Paradoxical motion of the left ventricular catheter is an ancillary angiographic finding in prolapsing left atrial tumor.
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10/16. Sudden development of intraoperative left ventricular outflow obstruction: differential and mechanism. An intraoperative two-dimensional echocardiographic study.

    Systolic anterior motion (SAM) of the mitral valve, once considered to be pathognomonic of hypertrophic cardiomyopathy, has been reported in the absence of asymmetric septal hypertrophy. Of the 1,000 open heart operations performed with intraoperative two-dimensional epicardial echocardiography monitoring, four patients developed intraoperative dynamic left ventricular outflow obstruction associated with systolic anterior motion of the mitral valve that was not present preoperatively: three cases of mitral valve annuloplasty with Carpentier ring insertion and one of coronary artery bypass grafting. Though no patient had asymmetric septal hypertrophy or echocardiographic evidence of outflow obstruction by either preoperative cardiac catheterization or echocardiography, intraoperative two-dimensional epicardial echocardiography revealed SAM, and hyperdynamic left ventricles with three of these patients having documented left ventricular outflow tract gradients causing hemodynamic compromise. (Case 4 was hemodynamically stable following mitral valve repair, but had SAM and significant residual mitral regurgitation [MR] requiring reinstitution of cardiopulmonary bypass and re-repair). Measurement of mitral annular dimension demonstrated a normal decrease in size from diastole to systole in control operative subjects but not in the patients who developed outflow obstruction. The pathophysiology, treatment, and role of intraoperative echocardiography of dynamic left ventricular outflow tract obstruction are discussed.
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