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)

Surgical management of the conal (supracristal) ventricular septal defect differs significantly from the management of the perimembranous (infracristal) ventricular septal defect. The absence of a portion of the conal septum can lead to prolapse of the right cusp of the aortic valve, which predisposes these patients to aortic insufficiency. Between January 1980 and December 1989, 36 children with conal ventricular septal defect underwent intracardiac repair. Diagnosis was by echocardiography, cardiac catheterization, and intraoperative exploration. Preoperative evaluation showed that 26 patients (72%) had aortic valve prolapse and 16 (44%) had aortic insufficiency. Pulmonary-to-systemic flow ratios ranged from 1:1 to 3.5:1 (mean 2.0:1.0). Ten patients (27%) were believed to have clinical congestive heart failure. Age at the time of operation ranged from 2 weeks to 18 years (mean 5.5 years). Operative exposure was through the pulmonary artery (26), aorta (4), right ventricle (3), or right atrium (3). Simultaneous aortic valve suspension for aortic insufficiency was performed in four patients. Operative survival was 100%. Follow-up is complete in all patients and ranges from 0.5 to 9 years (mean 4.3 years). All patients are in normal sinus rhythm. No residual ventricular septal defects have been identified. Twenty-three of 36 patients (64%) have no evidence of aortic insufficiency; 12 of 36 (33%) have trivial or mild aortic insufficiency. One patient with initial severe aortic insufficiency underwent repeat aortic valvuloplasty 3 years after ventricular septal defect closure and aortic valve suspension. No patients have required aortic valve replacement. Surgical management of the conal ventricular septal defect differs from that of the perimembranous ventricular septal defect in two critical aspects. The operative approach should be through the pulmonary artery. This allows the best exposure of the remaining conal septum and the pulmonary and aortic valve leaflets, facilitating closure of the defect without injury to the valves or conduction system. Conal ventricular septal defects should undergo early closure, regardless of shunt volume, to prevent progressive aortic insufficiency.
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PMID:Surgical management of the conal (supracristal) ventricular septal defect. 186 2

The records of 362 patients of Ventricular Septal Defect (VSD) were analysed to find out the incidence of aortic regurgitation (AR) and their hemodynamic and angiographic features. Thirty-seven patients (10.2%) were found to have AR, whose mean age was 13.4 years (range: 2-45) and male to female ratio was 5:1. Of the 37 cases 31 (84%) had infracristal and 6 (16%) had supracristal VSD. In 31 patients with infracristal VSD the prolapsing cusp was Right Coronary Cusp (RCC) in 14 (48%), Noncoronary Cusp (NCC) in 12 (41%) and both RCC and NCC in 3 (11%). Of the 6 patients with supracristal VSD the prolapsing cusp was RCC in 5 (83%) and NCC in 1 (17%). In two patients the AR was due to bicuspid aortic valve. The pulmonary artery pressure was normal in 26 of 37 (70.2%) patients and the left to right shunt was 1.5:1 or less in 23 of 37 (62%) patients. Nineteen of the 37 patients (51.3%) had grade I or II AR and the remaining 18 (48.7%) had grade III or IV AR. There was no relationship between the severity of AR and the location of the VSD. In conclusion, in this series, the incidence of VSD+AR is relatively higher and that of supracristal VSD is lower. In majority of patients the left to right shunt is small and pulmonary artery pressure within normal limits. The prolapse of RCC is more common in supracristal VSD and there is no relation between the severity of AR and the location of the VSD.
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PMID:Ventricular septal defect with aortic regurgitation: a hemodynamic and angiographic profile in Indian subjects. 208 7

Aortic valve insufficiency with ventricular septal defect is usually treated by plication of the commissures. However, long-term deterioration is common. We propose a new technique that corrects the aortic annulus dilatation and the leaflet prolapse and reinforces the sinus of Valsalva. Two groups were compared: group I (29 patients) had plication of the prolapsed leaflet(s) and folding of the free edge; group II (26 patients) had triangular resection of the prolapse cusp, annuloplasty, and reinforcement of the aortic wall. The two groups were similar with regarding to preoperative clinical data. There was no perioperative mortality. Primary failure (aortic valve replacement) occurred in 8 patients in group I (28%) and in 2 patients in group II (8%). The rate of secondary failure was 31% in group I and 4% in group II. The actuarial rate of freedom from reoperation at 5 years is 55% in group I and 88% in group II (p less than 0.05). The late mortality was 6.5% in group I and 10.9% in group II (no difference). We conclude that aortic valve insufficiency with ventricular septal defect is a malformation of the aortic leaflets, the annulus, and the sinus of Valsalva, and that the proposed technique offers a better result than the usual methods in terms of residual aortic valve insufficiency.
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PMID:Ventricular septal defect associated with aortic valve incompetence: results of two surgical managements. 219 13

From 1946 to March 1989, 92 patients (33 women and 59 men) were seen with ventricular septal defect (VSD) and audible aortic regurgitation (AR). The VSD was subcristal in 62 patients, subpulmonary in 21 and unknown in the remaining 9. The median age of onset of AR was 5.3 years. The risk of developing AR was 2.5 times greater in those with a subpulmonary VSD. The aortic valve was tricuspid in 90% and bicuspid in 10%. Prolapse was seen in 90% of those with subcristal VSD and in all with subpulmonary VSD. Pulmonary stenosis was seen in 46% of the patients with gradients ranging from 10 to 55 mm Hg. The incidence of infective endocarditis was 15 episodes/1,000 patient years. Among 20 patients followed medically, for 297 patient years, 1 died (1959) and most have been stable, including 2 followed for greater than 30 years. In the 72 patients operated on, there were 15 perioperative and 5 late deaths. Operations consisted of VSD closure alone in 7, VSD closure and valvuloplasty in 50 and VSD closure and aortic valve replacement in the other 15. Valvuloplasty was more effective in those operated on under age 10 compared to those older than 15 years (46 vs 14%). The durability of the valvuloplasty was 76% at 12 years and 51% at 18 years.
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PMID:Long follow-up (to 43 years) of ventricular septal defect with audible aortic regurgitation. 236 80

This study using pulsed and continuous wave Doppler echocardiography was designed to achieve a cross-sectional echocardiographic categorization of the fibrous tissues in the environs of perimembranous ventricular septal defects, to determine the mechanism involved in its formation and for qualitative and quantitative evaluation of the anomalies associated with the entity. A total of 67 patients was studied, 23 presented cross-sectional echocardiographic evidence of perimembranous ventricular septal defect in isolation, 12 associated with tissue 'tags' and 32 combined with 'restrictive' tissue in the area of the defect. Four echocardiographic features of the 'restrictive' tissue were observed. In 23 of these 32 patients, it was possible to identify the exact anatomic origin of the 'restrictive' tissue (in seven complete and, in 15, partial involvement of the septal leaflet of the tricuspid valve; in one, prolapse of the aortic valve with a partial involvement of the tricuspid septal leaflet) while in nine the origin remained undetermined. In 20, the 'restrictive' tissue simultaneously protruded into the right atrium and ventricle; only in 12 did it extend exclusively into the right ventricle. Tricuspid insufficiency was detected by pulsed Doppler in 78% of the patients with 'restrictive' tissue and in 23% of the remaining patients. Tricuspid incompetence was severe in only two patients of the first group. Three patients with 'restrictive' tissue (9%) had obstruction to the outlet of the right ventricle and four (13%) patients presented aortic insufficiency. Five patients (16%) with 'restrictive' tissue closing the defect did not present pulsed Doppler evidence of a shunt at the ventricular level.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Restrictive tissue in the area of perimembranous ventricular septal defect. Cross-sectional and Doppler echocardiographic study. 237 95

The diameter of the aortic root was measured in 151 patients with small ventricular septal defect (Qp/Qs less than 2 and pulmonary vascular resistance less than 1.5 U.m-2) and correlated with the presence of absence of aortic regurgitation. In 26 cases the aortic juxtavalvular region (bulbus) was dilated above 2 SD compared to a control group. All patients with aortic regurgitation of with cusp prolapse belonged to this group of 26 cases. Aortic root dilatation seems to have a specificity of 89 p. 100, a sensitivity of 75 p. 100 and a negative predictive value of 98 p. 100 in relation to the occurrence of aortic insufficiency. The finding of an aortic root dilatation associated with a ventricular septal defect should incite to a closer supervision of non-operated patients should be used as an argument when discussing the surgical closure of the ventricular septal defect.
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PMID:[Dilation of the aortic root in ventricular septal defects. Risk factor of aortic insufficiency]. 250 Jan 4

68 cases with 76 left ventriculographies, including rheumatic mitral valvular disease, congestive and hypertrophic types of cardiomyopathy, endocardial cushion defect, atrial and ventricular septal defects, coronary heart disease and mitral valve prolapse were analyzed with respect to the morphological and functional changes of the mitral valve and its appendages. Dynamic study with cineradiographic technic was the chief method used in this investigation. Except for ventricular septal defect, all the above-mentioned disease entities showed one or several of the changes of the mitral valvular apparatus including stenosis, insufficiency, displacement, cleft, deformity, prolapse and functional disorder. Regurgitation associated with mitral insufficiency exhibiting specific manifestations in different conditions was analyzed and its method of grading discussed. Mitral valve prolapse with its suggested method grading and functional disorder of the mitral valve were also discussed in detail.
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PMID:[Angiographic diagnosis of lesions of the mitral valve and its appendages]. 252 46

A nineteen-year-old female who had history of infectious endocarditis underwent surgical repair for ruptured aneurysm of sinus of Valsalva with aortic regurgitation. Through aortotomy mild degree of prolapse of right coronary cusp and perforation of left coronary cusp sized 6 mm in diameter were recognized and the latter was thought to be the dominant lesion resulting in severe aortic regurgitation. Following direct closure of ruptured aneurysm and patch closure of ventricular septal defect, perforated left coronary cusp was repaired with autologous pericardium. Post operative course was uneventful and she is now doing well.
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PMID:[A case report of successful repair for ruptured aneurysm of sinus of Valsalva (I VSD) with aortic regurgitation]. 261 29

Interstitial deletions of 3q have, to our knowledge, been reported in only four patients. We present an additional patient with interstitial deletion of 3q, with breakpoints at 3q23 and 3q25. The patient was small for gestational age and had a multiple congenital anomalies (MCA) syndrome including microcephaly; unusual facial appearance with bilateral microphthalmia, blepharophimosis, and ptosis; ventricular septal defect; and bilateral clubfeet. Comparison between the clinical and cytogenetic findings of the present case and those of previously reported cases suggests that a recognizable phenotype may be associated with deletions of 3q2.
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PMID:Interstitial deletion of the long arm of chromosome 3: case report, review, and definition of a phenotype. 236 39

The clinical presentation, diagnosis, and surgical treatment of 63 patients with doubly committed subarterial ventricular septal defects (DCVSD) were analyzed retrospectively. The patients were divided into three groups. Thirty-one patients had severe congestive heart failure in infancy and presently have a large ventricular septal defect that has no tendency to close or to produce aortic valve regurgitation (Group 1). Ideally, these defects should be closed in infancy, and the transpulmonary approach is recommended to achieve closure. In the first group, there was one death in a patient with a hypoplastic right ventricle. In Group 2, nineteen patients had aortic valve prolapse or aortic valve regurgitation. The DCVSD were moderately large or small. A number of DCVSD in Group 2 patients had maintained the normal offsetting of the arterial valves. These defects must be closed by the time mild aortic valve regurgitation has occurred. In Group 2, there was one late death in a patient who developed subacute bacterial endocarditis. The two patients who had severe aortic valve regurgitation required aortic valve replacement and underwent multiple surgical procedures to replace calcified bioprostheses. Group 3 comprised 13 patients who were diagnosed with tetralogy of Fallot. All of these patients had a large DCVSD with aortic valve overriding. All Group 3 patients survived radical repair of the defect, which required a transannular patch in most cases (76%).
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PMID:Surgical management of doubly committed subarterial ventricular septal defects. 318 Apr 5


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