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)

Double-chambered right ventricle (DCRV) is commonly associated with ventricular septal defect (VSD). In this study, an assessment was made of the relevance of a malalignment-type VSD to hemodynamic and morphologic features in DCRV. During an 8.5-year period, 53 patients with DCRV were enrolled after study with echocardiography, catheterization, and angiography. Patients were divided into 2 groups: group I included 40 patients, aged 3.7 +/- 3.2 years, with a malalignment-type VSD; group II consisted of 13 patients, aged 8.6 +/- 2.7 years, without a malalignment-type VSD. History of congestive heart failure in infancy was present in 21 group I and 2 group II patients (53% vs 15%, respectively, p <0.05). The mean pulmonary-to-systemic flow ratio was significantly higher in group I than in group II (1.89 +/- 0.74 vs 1.14 +/- 0.21, respectively, p <0.05). The mean pressure gradient across the right ventricular outflow tract was lower in group I than in group II (41 +/- 16 vs, 73 +/- 33 mm Hg, respectively, p <0.05). Among 42 patients who had a series of echocardiograms recorded, progression of pressure gradient was evident in 35: 28 in group I and 7 in group II. A subaortic ridge was present exclusively in 29 group I patients (73%). Prolapse of the aortic valve was present in 26 (49%): 20 group I (50%) and 6 group II (46%) patients. Aneurysm formation of the septal defect was found in 17 (43%) and 7 (54%) group I and II patients, respectively. It can be concluded that a history of congestive heart failure was more common in DCRV patients with a malalignment-type VSD. Malalignment-type VSD is significantly associated with a larger pulmonary-to-systemic flow ratio and subaortic ridge.
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PMID:Malalignment-type ventricular septal defect in double-chambered right ventricle. 862 36

A 23-year-old man presented with progressive exercise-related dyspnea and easy fatigability. He gave a history of a murmur of aortic insufficiency since childhood. Cardiac catheterization demonstrated severe aortic insufficiency. At surgery the patient was found to have prolapse of the right coronary cusp into a significant supracristal ventricular septal defect. Repair was carried out with a Dacron patch closure of the septal defect and replacement of the valve with a 29-mm St. Jude valve. The patient recovered uneventfully and is fully active and employed 5 years later. Review of the literature documents that this is an uncommon lesion. The approaches to preoperative diagnosis include transesophageal echo. The management techniques have included ventricular septal defect closure, alone and with valvuloplasty, and septal defect closure with aortic valve replacement. The anatomic and patient characteristics guide selection of the most suitable management of these patients.
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PMID:Considerations in the surgical management of ventricular septal defect and aortic insufficiency--a case report. 955 36

Serial changes in patients with isolated VSD during adolescence have not previously been investigated. Hemodynamic status, diameter of the defect, and growth were studied yearly in 106 children with VSD. The mean duration of the follow-up was 13.16 years and ranged in 80% of subjects from 7 to 19 years (1395 patients years). The mean ages at pre- and postpuberty were 8.62 and 16.67, respectively. The presented longitudinal study, in which losses due to death and operation were minimal (4%), ideally reflected the natural history of VSD. Although weight showed retardation during prepuberty, this lag was caught up by the end of adolescence. Stature showed no retardation in pre- and postpuberty. Cardiothoracic ratio decreased significantly from a mean of 0.48 to 0.44 and showed normal variation. Although the mean defect diameter at prepuberty was 5.33 mm, this decreased to 2.7 postpubertally. The individual decrease (1.7 +/- 2.34 mm) was significant (t = 5.349, p < 1/10(5)). The defect closed spontaneously in 24 (22.6%). In the 75 patients without pulmonary hypertension and with mild left-to-right shunting, 52 remained in the same class and spontaneous closure was observed in 23. In the 24 patients with moderate to severe left-to-right shunt, this decreased in 23 and only one remained stable. The 2 patients (1.9%) with Eisenmenger syndrome remained stable and 1 died. Aortic regurgitation developed in 10 patients (9.4%); however, this was of mild degree in most of them. No infective endocarditis was observed. It is concluded that patients with VSD should be followed closely through adolescence, because the diameter of the defect, as well as left-to-right shunting, can decrease, and it is concluded that the spontaneous closure of the defect is to be expected in a considerable 23%, and aortic prolapse or mild regurgitation may develop in approximately 10%.
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PMID:The natural course of isolated ventricular septal defect during adolescence. 956 19

Congenital aneurysms of the sinus of Valsalva are rare lesions. Because the aortic root is central, the aneurysm can rupture into any cardiac chamber, and virtually all combinations of sinus and chamber fistulas have been described. Rupture into the pulmonary artery, however, is very rare. We encountered a 14-year-old boy with conal ventricular septal defect and right coronary cusp prolapse with an unruptured aneurysm of the sinus of Valsalva into the pulmonary artery.
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PMID:Unruptured aneurysm of the sinus of Valsalva into the pulmonary artery. 959 92

A seventy-year-old man was admitted at our hospital because of dyspnea. Echocardiogram and left ventriculogram showed an aneurysm formation of the membranous ventricular septum and small left-to-right shunt through ventricular septum defect and also severe mitral and tricuspid insufficiency. Operation was performed after medical therapy for congestive heart failure. During operation, mitral leaflets showed no organic lesions nor prolapse, but the annulus was dilated. The cause of mitral insufficiency, we thought, might be congenital, and the annulus dilatation was caused of mitral insufficiency, we thought, might be congenital, and the annulus dilatation was caused to produce tricuspid insufficiency secondary. The ventricular septal communication became small (diameter; 5 mm) and was associated with aneurysm formation of the remaining portion of the membranous septum. And the aneurysm, protruding to the septal leaflet of tricuspid valves, enhanced tricuspid insufficiency. It was reported by many authors that the aneurysm formation was related to spontaneous closure of ventricular septal defect. Patients with small ventricular septal defect, without any symptoms, must be followed intensively, or they might get cardiac complications, such as arrhythmia, right ventricular outflow obstruction, tricuspid insufficiency, and so on.
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PMID:[Aneurysm of the membranous ventricular septum with ventricular septal defect, mitral and tricuspid insufficiency]. 984 79

The MR features of two cases of supracristal ventricular septal defect are described. In both patients, axial SE T1-weighted images demonstrated a defect between the base of the aorta and the upper posterior aspect of the right ventricular infundibulum. Cine MRI in the two cases showed left-to-right shunting with a flow jet in the distal right ventricular outflow tract that propagated into the main pulmonary artery. Both patients had prolapse of the right sinus of Valsalva, and one had aortic insufficiency.
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PMID:MRI of supracristal ventricular septal defects. 1005 Jul 99

Oculo-facio-cardio-dental syndrome is a very rare condition. So far, only nine cases have been documented. We report on three additional female patients representing the same entity. The clinical findings were: congenital cataract, microphthalmia/microcornea, secondary glaucoma, vision impairment, ptosis, long narrow face, high nasal bridge, broad nasal tip with separated cartilages, long philtrum, cleft palate, atrial septal defect, ventricular septal defect, and skeletal anomalies. The following dental abnormalities were found: radiculomegaly, delayed dentition, oligodontia, root dilacerations (extension), and malocclusion. For the first time, fusion of teeth and hyperdontia of permanent upper teeth were seen. In addition, structural and morphological dental changes were noted. These findings expand the clinical spectrum of the syndrome.
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PMID:Rare dental abnormalities seen in oculo-facio-cardio-dental (OFCD) syndrome: three new cases and review of nine patients. 1006 16

We present a case report of a patient with conal septal hypoplasia (supracristal) ventricular septal defect (VSD) complicated by aortic insufficiency (AI) and subpulmonic stenosis from a fibrous membrane. The development of AI with VSD is a well-recognized problem. However, the association of VSD, AI, and significant right ventricular outflow tract obstruction (RVOTO) is less common. Mechanisms of RVOTO include prolapse of an aortic valve cusp across the VSD, as well as infundibular hypertrophy or muscle bundles. Technical echocardiographic issues can make the diagnosis of VSD, AI, and RVOTO challenging. The presence of a discrete fibrous subpulmonary membrane is uncommon in this setting.
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PMID:Membranous subpulmonic stenosis associated with ventricular septal defect and aortic insufficiency. 1062 32

We reviewed the Doppler echocardiographic findings of the aortic valve and associated aortic regurgitation (AR) in 55 patients who underwent patch closure of doubly committed subarterial ventricular septal defect (VSD). The maximal diameter of the VSD measured > or = 5 mm, whereas the postoperative follow-up interval was > or = 5 years. Twenty-three patients underwent closure before they developed aortic cusp prolapse or AR (group A). In 15 patients the VSD was closed when aortic cusp prolapse was recognized, but AR was absent (group B). Aortic cusp prolapse with AR was detected before closure in a further 15 patients (group C). Of 8 patients with no AR before closure, AR was detected during follow-up in 6 group A and in 2 group B patients. In group C, AR resolved after surgery in 4 patients, whereas AR grade improved in a further 8 patients and remained unchanged in 3. Although residual AR was more frequent in patients with aortic cusp prolapse and AR before closure, it was silent and asymptomatic.
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PMID:Evolution of aortic regurgitation following simple patch closure of doubly committed subarterial ventricular septal defect. 1100 73

Systemic lupus erythematosus is a disease in which inflammatory process provoked by different antibodies affects many organs and systems. The circulatory system is one of them. In patients with systemic lupus erythematosus cardiac disorders are generally known and well proved. It is known that this disease has heritage background. Thus, the offsprings of patients suffering from systemic lupus erythematosus belong to a risk group. Moreover, it is thought that maternal antibodies crossing transplancentally to the fetus cause damages of tissues including the heart. The aim of this study was to evaluate cardiological status of 38 children whose mothers suffered from systemic lupus erythematosus. In all sick mothers the diagnosis fulfilled criteria of the American Rheumatism Association. The mothers have been remaining under medical treatment while the children have been under control and simultaneously prophylaxis of lupus has been undertaken. The study was undertaken in 17 girls and 21 boys aged 3 to 18 years (average: 12 +/- 4.5 years). Physical development of presented children was satisfactory. During cardiological examination all subjects were in good general condition, without any clinical evidence of collagen disease and infection. Obtained results were compared with the ones found in control group of 38 children of healthy mothers, being at the same age. Study methods were: physical examination, arterial blood pressure measurement, standard and 24 hours according to Holter method ECG record, echocardiographic and Doppler examination, and physical performance test according to Bruce's protocol. In children of sick mothers examined laboratory parameters were within the normal limits excluding the presence of antinuclear antibodies (controlled by indirect immunofluorescence test), result of which was positive in 15 studied children (39%). In the group of the children of sick mothers the abnormalities of sinus node function were detected in 12 cases (32%), significantly more often than in the control. There were found abnormalities of atrio-ventricular and intraventricular conduction in 15 subjects (40%). The premature beats of ventricular origin were noticed in 3 cases (8%). These disturbances were significantly different from the control group. In addition, correlation between the presence of antinuclear antibodies and the cardiac abnormalities was taken into consideration. So, significant correlation between antinuclear antibodies and heart rhythm disorders was proved. During echocardiographic examination structural and functional abnormalities were found. They were: ventricular septal defect (muscular part) (1), pericarditis effusion (1), prolapse of the mitral valve anterior leaflet (2), mitral valve regurgitation of the second degree (2) and increased diameter of left atrium (8). One girl from the studied group, suffering from atrio-ventricular block of III* was operated on because of persistent ductus arteriosus still in the newborn's period. At the same time the permanent pace-maker was implanted. After 1 year of age this girl was operated on because of atrial septal defect (ASD II). In studied group of children echocardiographic global indices of left ventricular systolic function were normal. The subclinical impairment of diastolic left ventricle function was found in 8 children with increased left atrium-aorta index. Both the theoretical knowledge and the results of the studies suggest that the offsprings of mothers suffering from SLE need a careful cardiological observation.
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PMID:[Cardiological state of offsprings of the mothers suffering from systemic lupus erythematosus]. 1105 11


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