1/893. Deletion of 1q in a patient with acrofacial dysostosis. The Nager syndrome is the most common form of acrofacial dysostosis. Although autosomal dominant and recessive forms of acrofacial dysostosis have been described the molecular etiology of these disorders is unknown. We report on a child with acrofacial dysostosis, critical aortic stenosis, and a deletion of chromosome 1q involving the heterochromatic block and adjacent euchromatin. ( info) |
2/893. Anaesthetic management of a patient with williams syndrome undergoing aortoplasty for supravalvular aortic stenosis. PURPOSE: A case of a patient associated with williams syndrome undergoing aortoplasty for supravalvular aortic stenosis is presented. CLINICAL FEATURES: williams syndrome is a rare disease associated with a characteristic facies, supravalvular aortic stenosis, and mental retardation. A 15-yr-old girl with williams syndrome underwent aortoplasty for supravalvular aortic stenosis. Anaesthesia was induced with fentanyl and thiamylal, and maintained with nitrous oxide, oxygen, sevoflurane, and continuous intravenous infusion of fentanyl. Supravalvular aortic stenosis was evaluated using a multiplane transesophageal echocardiography (TEE) probe before and after repair. CONCLUSION: Multiplane TEE was found to be very useful for anaesthetic management in a patient with williams syndrome undergoing aortoplasty for supravalvular aortic stenosis. ( info) |
3/893. Aortic stenosis and coronary artery disease caused by alkaptonuria, a rare genetic metabolic syndrome. alkaptonuria is a rare metabolic disease in which homogentisic acid deposits occur in various body tissues. We present a case of alkaptonuria which resulted in aortic stenosis and coronary artery disease due to homogentisic acid deposition. ( info) |
4/893. An unusual combination of cardiovascular surgical disorders. A 53-year-year-old man presented with aortic regurgitation, subvalvular and supravalvular aortic stenoses, and aneurysms involving the ascending aorta, the arch, and the innominate, right subclavian, and left common carotid arteries. Surgery consisted of resection of the obstructive lesions, replacement of the aortic valve, graft replacement of the ascending aorta, and the arch resection of innominate and subclavian artery aneurysms and reconstruction with a side limb to which the right carotid artery was anastomosed. The patient has remained asymptomatic with full employment. ( info) |
5/893. 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. ( info) |
6/893. Discrete subaortic valvular stenosis: the clinical utility and limitations of transesophageal echocardiography. Transesophageal echocardiography (TEE) is a useful technique in the diagnosis and intraoperative assessment of discrete subaortic stenosis (DSS). It allows clear visualization of the subaortic membrane, which may be missed by transthoracic echocardiography, differentiates DSS from other causes of left ventricular outflow tract obstruction, and accurately detects the presence of associated aortic regurgitation and bacterial endocarditis. Limitations in its use include inadequate visualization of the left ventricular outflow tract by TEE in the presence of a prosthetic mitral valve or mitral annular calcification, and improper alignment of the Doppler cursor across the outflow tract. ( info) |
7/893. Iatrogenic left main coronary artery stenosis. Iatrogenic left main coronary artery stenosis is a potentially life-threatening complication of cardiac valve replacement surgery due to injury by perfusion cannulas. This requires prompt clinical recognition and diagnosis by repeat coronary angiography, and treatment by early coronary artery bypass grafting. We present 3 patients who had normal coronary arteries prior to valve replacement surgery, and who developed severe left main coronary artery stenosis after surgery. Accelerating angina and refractory ventricular arrhythmia were presenting clinical manifestations. coronary artery bypass grafting was successfully performed in all 3 patients. ( info) |
8/893. The Freestyle stentless bioprosthesis for prosthetic valve endocarditis. We report a case of methicillin-resistant staphylococcus aureus-induced prosthetic valve endocarditis, which was successfully treated with aortic valve replacement using the Freestyle stentless bioprosthesis. The total root and stentless design of this bioprosthesis allows for more radical removal of infected tissue and easier treatment for annular abscess, while requiring less prosthetic materials than a conventional prosthesis. This bioprosthesis thus seems to be a valuable option for active endocarditis. ( info) |
9/893. Combined aortic and mitral stenosis in mucopolysaccharidosis type I-S (Ullrich-Scheie syndrome). The genetic mucopolysaccharidosis syndromes (MPS) are autosomal recessive inborn errors of metabolism. Heart valve involvement in MPS is not uncommon but only a few case reports of successful cardiac surgery are available. In particular, reports of combined aortic and mitral stenosis associated with MPS type I-S are very rare. Both type I and type VI MPS are associated with significant left sided valvar heart disease that requires surgical valve replacement because of irregular valve thickening, fibrosis, and calcification. A 35 year old man had severe mitral valve stenosis after successful surgical replacement of a stenotic aortic valve. Valvar heart disease was investigated by cardiac ultrasound and left heart catheterisation. Histomorphological characterisation of the affected mitral valve was performed. The case illustrates typically associated clinical features of cardiac and extracardiac abnormalities found in MPS type I-S. ( info) |
| BACKGROUND AND AIM OF THE STUDY: In order to prevent prosthetic valve endocarditis (PVE), the implantation of a new silver-coated sewing ring has been introduced to provide peri- and postoperative protection against microbial infection. methods: A 56-year-old woman with aortic stenosis had elective replacement with a St. Jude Medical mechanical valve fitted with a silver-coated sewing ring (Silzone). The patient developed early PVE, which necessitated reoperation after one month. Despite a second Silzone prosthesis being implanted, the endocarditis recurred. During a third operation an aortic homograft was implanted, and after six months a fourth operation was performed for a pseudoaneurysm at the base of the homograft, in proximity to the anterior mitral valve leaflet. RESULTS: The diagnosis of PVE was confirmed by the presence of continuous fever, transesophageal echocardiography and growth of penicillin-resistant staphylococcus epidermidis from the valve prosthesis. CONCLUSION: The implantation of all prosthetic valves is encumbered with a risk of endocarditis. Although silver has bacteriostatic actions, the advantages of silver-coated prostheses in the treatment of this condition have yet to be assessed in clinical trials. ( info) |