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)

Three adults, 2 with tricuspid aortic valve and 1 with bicuspid valve, underwent valvuloplasty for aortic valve regurgitation resulting from cusp prolapse. Surgical procedures consisted of combined cusp plication by triangular cusp resection and subcommissural annuloplasty. Doppler echocardiography revealed trivial aortic valve regurgitation intraoperatively and less than I/IV at discharge in all cases. After mean follow-up of 15 months, 2 tricuspid aortic valve patients remain I/IV regurgitation and II/IV in the bicuspid patient. Although long-term results remain unclear, our results show that this procedure is feasible and beneficial in patients with aortic valve regurgitation due to cusp prolapse.
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PMID:Valvuloplasty for aortic valve regurgitation resulting from cusp prolapse. 1130 58

Reports of aortic regurgitation due to rupture of the aortic valve commissures are rare. Prompt surgical intervention is necessary, as the condition results in rapid, progressive heart failure and subsequent death. We report the case of a 78-year-old man who presented with aortic laceration and cardiac tamponade that was probably induced by prolapse of the bicuspid aortic valve. We speculate that prompt initial surgery may have prevented aortic laceration and cardiac tamponade in this patient. Thus, in order to optimize clinical outcome, clinicians must consider early, precautionary surgical management in patients who have sudden cardiac failure due to aortic regurgitation associated with prolapse of the bicuspid aortic valve.
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PMID:Aortic laceration due to prolapse of the bicuspid aortic valve: case report. 1138 Jan 2

Noonan's syndrome is characterized by craniofacial anomalies, i. e. ptosis, webbing of the neck and a deep nuchal hairline, as well as skeletal deformities such as short stature, clinodactyly, pectus carinatum and funnel chest and other organ anomalies, mainly cardiac valve disease, less often testicular retention or kidney malformations. Noonan syndrome presents with aspects similar to Ullrich-Turner syndrome, but can be found in both male and female patients. In about one half of the patients with Noonan's syndrome cardiovascular anomalies occur, mostly anomalies of the right heart (mainly valvular pulmonary stenosis). Aortic stenosis and coarctation of the aorta are rarely seen. We report on a patient with four sequential potentially stenosing and stenosed parts of the proximal systemic circulation: hypertrophic cardiomyopathy, bicuspid aortic valve, coarctation of the aorta and a hypoplastic aortic arch as a part of Noonan's syndrome. This patient presents with a unique combination of anomalies, as he also shows a Madelung like deformity of the wrist.
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PMID:[Noonan's syndrome with an unusual combination of hypertrophic cardiomyopathy, congenital bicuspid aortic valve, coarctation of the aorta and hypoplastic aortic arch]. 1508 76

A 32-year-old male patient with bicuspid aortic valve underwent valvuloplasty for the treatment of aortic regurgitation. The aortic regurgitation was estimated to be grade III based on the transthoracic echocardiography and aortography findings. Transesophageal echocardiography showed prolapse of the conjoined leaflet. At surgery, the valvuloplasty consisted of triangular resection and re-suture of the prolapsed larger leaflet, and subcommissural annuloplasty. The degree of the aortic regurgitation decreased to very slight degree after the repair. The peak pressure gradient across the repaired aortic valve was 8.8 mmHg. The patient was discharged without any complications. The bicuspid aortic valve demonstrating aortic regurgitation seems to be repairable. As a result, the valvuloplasty is indicated for such patients, especially for young patients.
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PMID:[Valvuloplasty for aortic regurgitation with bicuspid valve: report of a case]. 1551 Aug 23

We report the case of a Caucasian man with the unusual combination of an infundibular ventricular septal defect (VSD), an aneurysm of the sinus of Valsalva communicating with the right ventricle, and a bicuspid aortic valve. When aortic valve regurgitation associated with mild right aortic cusp prolapse appeared during follow-up examination, the patient, although totally asymptomatic, underwent surgical repair of the aortic sinus and closure of the VSD.
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PMID:Infundibular ventricular septal defect, aneurysm of the sinus of Valsalva, and bicuspid aortic valve in a caucasian male. 1574 18

Aortic valve replacement is the standard procedure in patients with aortic valve regurgitation (AR). Although long-term results for both biological and mechanical heart valves could be improved, a valve-sparing operation has several advantages especially in young patients. Alterations in the geometry of the aortic root, especially dilatation of the sinutubular junction, are the primary cause of AR in patients with aneurysms of the ascending aorta. In patients with a bicuspid aortic valve, AR is usually caused by a prolapse; with appropriate surgical experience, the prolapse can be corrected and the valve reconstructed. Isolated reconstruction of a tricuspid aortic valve such as decalcification, commissurotomy or plication of ring or leaflets are seldom indicated. The recontstructive techniques for aortic root aneurysms or type A dissection described by David and Yacoub have become routine procedures over the last decade. The long-term clinical results are excellent, and revision and thromboembolism rates are very low. A maximal diameter of the aortic root > 5 cm is indicative for performing the above procedures. This technique has been used in the own clinic since 1996 with excellent clinical and echocardiographic results. Only four of 101 patients operated had a moderate AR during the follow-up period. The reconstruction of a prolapse in a bicuspid aortic valve is possible by using an autologous, fixed pericardial patch, with very good long-term results. By using this surgical technique, two geometrically correct pockets with a broad coaptation zone can be constructed. In 36 of the patients operated in the own clinic, only four patients showed grade I AR. No patient had to undergo reoperation. An aortic valve reconstruction for an isolated leaflet perforation following a healed endocarditis is seldom indicated.In the authors' opinion, valve-sparing reconstruction in cases of aneurysms of the ascending aorta, and for bicuspid valves, represents a promising alternative to prosthetic valve replacement. With low surgical mortality and morbidity, excellent clinical and functional long-term results can be achieved. Furthermore, the lack of the necessity of anticoagulation as well as positive hemodynamic factors argue for a valve-sparing surgical technique. A final evaluation of the method is not possible, however, until long-term follow-up of up to 20 years is available and the positive results have been confirmed.
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PMID:[Indication, technique, and results of aortic valve and ascending aorta reconstruction]. 1707 82

In this study, we report on our first experience with the construction of a valve using autologous vena cava tissue for right ventricular outflow tract reconstruction. Simulating the clinical situation valves were built from tubular pieces of porcine inferior vena cava placed in a PTFE tube and investigated in a pulsatile flow simulator. Based on the given vena cava dimensions, conduits were constructed with diameters of 19 mm in bicuspid or tricuspid and 22 mm and 24 mm in bicuspid configuration. The lowest pressure gradients were observed in the 22 mm vena cava valves in bicuspid configuration (8.6+/-0.5 mmHg) compared to 24 mm valves (10.6+/-0.9 mmHg, P=0.0004) and 19 mm valves (13.4+/-1.5 mmHg, P=0.005). No differences could be found between 19 mm bicuspid and tricuspid valves. Concerning valve opening movements, a complete opening in the 19 mm and a nearly unhindered opening in 22 mm valves were registered. In 24 mm valves opening was incomplete. Leakage was increased in 19 mm bicuspid valves due to leaflet prolapse. In conclusion, construction of a valve mechanism from vena cava tissue is feasible. The in-vitro hemodynamic results are encouraging, animal experiments are ongoing to investigate the midterm function of these valves.
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PMID:Vena cava as autologous tissue for pulmonary valve substitute. 1871 54

We studied 11 adult patients with dextro-transposition and 5 adult patients with levo-transposition (corrected transposition) of the great arteries with real time two-dimensional (2DTTE) and live/real time three-dimensional transthoracic echocardiography (3DTTE). All patients with dextro-transposition underwent a Mustard or Senning procedure during infancy. Incremental findings provided by 3DTTE and not delineated by 2DTTE were (a) comprehensive examination of all three leaflets of the tricuspid valve including the detection and measurement of anatomic defects in the leaflets and the assessment of systolic noncoaptation and segmental prolapse; (b) en face viewing and measurement of vena contracta areas of the valvular regurgitation jets and the assessment of regurgitant volumes; (c) en face viewing of the intra-atrial baffle and localization and measurement of baffle defects as well as the measurement of vena contractas of the baffle leaks; (d) recognition of a bicuspid pulmonary valve; and (e) the quantitative assessment of left ventricular outflow tract obstruction. 3DTTE appears to be a useful noninvasive modality which could supplement 2DTTE in the comprehensive assessment of adult patients with transposition of the great arteries.
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PMID:Live/real time three-dimensional transthoracic echocardiographic assessment of transposition of the great arteries in the adult. 1984 77

We report a female infant with partial trisomy 8p (8p11.2-->pter) and deletion of 13q (13q32-->qter). She was born with mild hypotonia, intrauterine growth retardation, microcephaly, micrognathia, large low set ears, pectus excavatum, anteriorly placed anus, and bilateral clinodactyly. Echocardiography showed left ventricular hypertrophy, bicuspid aortic valve, dilatation of the aorta and pulmonary artery, and prolapse of atrio-venticular valve leaflets. Cytogenetic investigation of her sister and her father showed that the altered region resulted from a balanced translocation between the part of the long arm of chromosome 13 and short arm of chromosome 8. In partial trisomy 8p, the clinical picture of the patients comprises hypotonia, structural brain abnormalities, facial anomalies including a large mouth with a thin upper lip, a high arched palate, a broad nasal bridge, an abnormal maxilla or mandible, malformed, low set ears, and orthopedic anomalies. Although patients with proximal deletions of 13q that do not extend into band q32 have mild to moderate mental and growth delays with variable minor anomalies, patients with more distal deletions including at least part of band q32 usually have major malformations such as retinoblastoma, mental-motor growth retardation, malformation of brain and heart, anal atresia, and anomalies of the face and limbs. To our knowledge partial trisomy 8p and partial monosomy of 13q have not been reported previously in the same person.
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PMID:Partial trisomy 8p (8p11.2-->pTER) and deletion of 13q (13q32-->qTER): case report. 2161 86

We report two unbalanced translocations involving the short arms of chromosomes 7 and 18 due to a balanced translocation 7;18 in the mother. Karyotyping and fluorescence in situ hybridization analysis of the female fetus revealed an unbalanced subtelomeric translocation(karyotype 46,XX,der(18)t(7;18)(p22.3;p11.32)mat resulting in a partial trisomy 7p and a partial monosomy 18p.Array comparative genomic hybridization (CGH) detected a4.44-Mb heterozygous duplication at 7p22.3 to 7p22.1 and a0.178-Mb heterozygous deletion at 18p11.32. Clinical characteristics comprised a mildly stenotic bicuspid aortic valve and a small aortic arch without coarctation. The patient's older brother displayed a reciprocal version of her chromosomal aberration (46,XY,der(7)t(7;18)(p22;p11.32) resulting in a partial monosomy 7p and a partial trisomy 18p. Array CGH revealed a 4.75-Mb heterozygous deletion at 7p22.3p22.1 and a 0.579-Mb duplication at 18p11.32. He presented with tetralogy of Fallot, cleft palate, microcephalus without craniosynostosis, growth retardation, ptosis of the right eyelid, right-sided renal agenesis, unilateral cryptorchism,and mental retardation. In this report, we present the clinical phenotype in patients with aberrations of chromosomes 7p and 18p and reviewed the literature to summarize cardiovascular malformations in these patients.
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PMID:Cardiac malformation of partial trisomy 7p/monosomy 18p and partial trisomy 18p/monosomy 7p in siblings as a result of reciprocal unbalanced malsegregation--and review of the literature. 2230 61


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