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[Relative valve area stenosis after replacement of a Starr-Edwards mitral valve (model 6520) for corrected TGA (S, L, L): report of a case].

The patient was a 38-year-old man who had undergone mitral valve replacement with a Starr-Edwards disc valve (model 6520, 3 M, phi 30 mm, functional valve area: 2.85 cm2) at the age of 15 years for TR combined with corrected TGA. He developed dyspnea on exertion and palpitations at the age of 30 years, and paroxysmal supraventricular tachycardia appeared at the age of 35 years. Cardiac catheterization revealed that the PCWP was 30 mmHg and the TVA was 1.1 cm2, and a diagnosis of stenosis of the prosthetic valve was made. Thus, replacement of the Starr-Edwards disc valve using a BICARBON double leaflet valve (phi 27 mm, functional valve area: 2.85 cm2) was performed. The operative findings of the Starr-Edwards disc valve revealed a normal appearance and no pannus growth. Postoperative echocardiography and cardiac catheterization found that the TVA was 3.7 cm2 and the PCWP was 13 mmHg. The actual valve area of the disc valve sewn on the ventrical, which was anatomically RV construction might have been smaller than the ideal area; however, the double leaflet valve proved efficient in our patient. This case report serves to demonstrate that because of the postoperative difference in measurement between the actual valve area and the prostheticin ball or disc valve, patients undergoing mitral valve replacement must be followed up carefully.

Adult↗

Autosomal dominant secundum atrial septal defect with various cardiac and noncardiac defects: a new midline disorder.

We report on a Lebanese family in which 12 persons had an atrial septal defect and various cardiac and noncardiac anomalies. Cardiac anomalies are left axis deviation of QRS, right bundle branch block, atrial fibrillation, Wolff-Parkinson-White syndrome, nodal atrioventricular rhythm, aortic stenosis, pulmonic valve stenosis, mitral stenosis (Lutembacher syndrome), and low implantation of the tricuspid valve (Ebstein disease). Noncardiac abnormalities consisted specially of the presence of hypertelorism, cleft lip, and pectus excavatum. This combination appears to constitute a hitherto undescribed autosomal dominant midline disorder of the heart and upper half of the body with almost full penetrance and variable expressivity. The mutation does not map to any known locus involved in atrial septal defect or conduction block.

Abnormalities, Multiple↗

Stenosis of a mechanical mitral prosthetic valve in a patient with systemic lupus erythematosus.

A young woman with systemic lupus erythematosus (SLE) developed progressive heart failure several years after mitral valve replacement with a Björk-Shiley prosthesis for treating mitral stenosis due to Libman-Sacks endocarditis. She was admitted to the hospital with pulmonary oedema. Transoesophageal echocardiography revealed stenosis of the mitral prosthesis, which was covered by fibrous tissue. Replacement of the prosthesis was done but the patient died from cerebral haemorrhage three days later. Although three cases of prosthetic valve dysfunction in SLE have been documented so far, this is to our knowledge the first report of a SLE recurrence on a tilting disc mechanical valve.

Adult↗