Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033377 (prolapse)
 11,717 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical records and radiographs of 46 patients with a supracristal ventricular septal defect are reviewed to illustrate the spectrum of associated lesions and complications seen with this defect. The presence of a supracristal ventricular septal defect compromises the normal conal muscular support of the aortic valve, usually in the region of the right sinus of Valsalva, and, consequently, complications involving the aortic valve are frequently noted. Decreased sinus support may lead progressively to prolapse of the right aortic cusp through the supracristal ventricular septal defect into the right ventricular infundibulum effecting sequentially aortic regurgitation, right ventricular outflow obstruction, and ultimately right sinus of Valsalva-right ventricular fistula. This potential progression is an indication for early recognition and repair of the supracristal ventricular septal defect. A supracristal ventricular septal defect is also an integral component of the Taussig-Bing malformation, is present in the majority of patients with aortic arch interruption, and may be seen in as many as 25% of Oriental patients with tetralogy of Fallot.
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PMID:Supracristal ventricular septal defects: spectrum of associated lesions and complications. 40 94

Two patients (one with transposition of the great arteries and another with Taussig-Bing anomaly) underwent the Damus-Stansel-Kaye procedure (Group I). Significant aortic valve insufficiency developed postoperatively in both patients. In contrast, seven patients with a univentricular heart and subaortic stenosis from a variety of reasons underwent creation of an aortopulmonary window (Group II), a procedure very similar to the proximal main pulmonary artery-aortic root anastomosis of the Damus-Stansel-Kaye procedure. Aortic valve insufficiency had not developed after up to 7 years of follow-up in this group (average 43 months). Postoperative angiograms suggest that aortic valve incompetence in Group I may have been caused by prolapse of the aortic valve. The valvular structures are subjected to high systolic pressures and face a dilated, low-pressure right ventricle. Aortic root distortion may have contributed, as well. In Group II patients, the aortic valve structures face a small, thick-walled chamber. The orientation of the aortic valve vis-a-vis the right ventricle changed postoperatively in Group I but not in Group II patients. Our experience suggests that the aortic valve or subaortic valve region should be closed at the initial repair in patients with low pulmonary vascular resistance who are undergoing the Damus-Stansel-Kaye procedure, to minimize the need for reoperation for aortic valve insufficiency.
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PMID:The Damus-Stansel-Kaye procedure. Should the aortic valve or subaortic valve region be closed? 370 81