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Biomedical subjects

J Ovseyevitz

Publications and source records attributed to J Ovseyevitz.

At least 19 recordsLinked to original sources
PubMed1988

Duplication 11q and deletion 5p syndromes due to a reciprocal translocation segregating in four generations.

We report on 2 relatives with duplication 11q and deletion 5p, resulting from an adjacent-1 segregation of a balanced reciprocal translocation 5p15;11q23, segregating in 4 generations of this family. Twelve out of 16 at-risk relatives of inheriting the translocation were shown to be carriers, giving a significant (p less than .05) 3:1 ratio of carriers/noncarriers. The breakpoint on chromosome 11 at q23 is a folate sensitive fragile site into where the proto-oncogene c-ets has been mapped.

Abnormalities, Multiple↗
PubMed1987

Congenitally corrected transposition with mirror-image atrial arrangement.

Congenitally corrected transposition (discordant atrioventricular and ventriculoarterial connexions) with mirror-image atrial arrangement is much less frequent than the same chamber combinations in the setting of usual atrial arrangement. This scarcity of cases has made their analysis difficult. In this study we have compared the anomalies found in 19 patients with congenitally corrected transposition in mirror-image arrangement with those in 39 patients having usual atrial arrangement. Absolute, relative and attributable risks were calculated for the presence of subvalvar pulmonary stenosis, ventricular septal defect, tricuspid regurgitation, atrioventricular block or the absence of these anomalies for each of the two groups. A greater absolute risk was found for both subvalvar pulmonary stenosis (68.4 vs. 43.6%, respectively) and ventricular septal defect (63.2 vs. 58.9%, respectively). The absolute risk for tricuspid regurgitation was 25.6% in those with usual arrangement as against 21.1% in those with mirror-image arrangement. Atrioventricular block was also more frequent in those with usual arrangement (25.6 vs. 10.5%) as was absence of associated cardiac defects (17.9 vs. 10.5%, respectively). The relative risks were greater for subvalvar pulmonary stenosis and ventricular septal defect in the patients with mirror-image as opposed to usual atrial arrangement (1.57: 1.00 and 1.07: 1.00, respectively), contrasting with greater relative risk for tricuspid regurgitation (1.26: 1.00), atrioventricular block (2.43: 1.00) and absence of associated anomalies (1.70: 1.00) in the patients with usual arrangement. The attributable risk for subvalvar pulmonary stenosis was estimated to be 24.8% and ventricular septal defect 4.3% for those having mirror-image atrial arrangement.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiomyopathy, Hypertrophic↗
PubMed1986

Surgical results in subaortic stenosis.

Since 1965, 46 patients aged 4 to 42 years, underwent cardiac surgery for subaortic stenosis. Resection of the subvalvar obstruction without myomectomy was performed in all cases. Three patients died during the operation, another one after 6 months due to infective endocarditis and one more suddenly 11 years after treatment. One patient was lost to follow-up but 41 were available after at least 1 year of follow-up. Before surgery, 21 cases were in NYHA class I, 17 in class II and 8 in class III. One year after surgery 36 were in class I, 4 in class II and only one in class III. Actuarial survival rate was 91% from 1 to 12 years and 79% from 13 to 18 years. Event-free survival was 45% up to 18 years. The mean preoperative peak systolic gradient was 93.15 +/- 35.57 mm Hg. The first postoperative peak systolic gradient was 21.61 +/- 17.91 mm Hg (P = 0.001). Cases with adverse postoperative events such as aortic regurgitation (13 cases), restenosis (13 cases), death (2 cases) and infective endocarditis (2 cases) had a mean peak systolic gradient of 55.78 +/- 35.97 mm Hg, while in the event-free patients the gradient was 14.61 +/- 13.34 mm Hg (P = 0.001). Recurrent obstruction was observed in seven patients and an increase in the residual gradient in six. The initial mean postoperative peak systolic gradient in these patients had been 18.23 +/- 17.32 mm Hg and the second postoperative cardiac catheterisation showed a mean gradient of 59.23 +/- 37.78 mm Hg (P = 0.001). We conclude that long-term follow-up following removal of subaortic stenosis is mandatory in order to detect and treat adverse events.

Adolescent↗
PubMed1986

Late results of mitral valve replacement with the Björk-Shiley prosthesis in children under 16 years of age.

The late results of isolated mitral valve replacement were evaluated in 37 children under 16 years of age receiving a Björk-Shiley prosthesis for the treatment of rheumatic mitral lesions. Three patients died, one during the operation and the others 2 months postoperatively. Of the latter two, one had a cerebral hemorrhage and the second had septicemia. The survivors were followed up for a mean of 4.7 years (range 2.8 to 8.9 years). After the operation, all patients were placed on a strict anticoagulant regimen with acenocoumarol. The actuarial survival rate was 92% at the end of the follow-up period. Before valve replacement two patients were in New York Heart Association Class I, 15 in Class II, 18 in Class III, and two in Class IV. After treatment 33 were in Class I and one in Class II. No instances of thromboembolism or infective endocarditis were observed in the survivors. Twenty-one patients underwent cardiac catheterization 2 to 7 years after the operation for evaluation of surgical results. The mean pulmonary artery systolic pressure decreased significantly after operation (p = 0.001), and the mean pulmonary artery wedge pressure decreased to normal values (p = 0.001). A mild mean diastolic gradient across the mitral valve at rest was found after the operation (4.9 +/- 2.4 mm Hg). During isometric exercise this gradient increased to 6.5 +/- 4.6 mm Hg. In two patients a discrete paravalvular leak was demonstrated by cineangiography, but the pulmonary wedge pressure was normal in both. The overall results with the Björk-Shiley prosthesis are encouraging in patients in whom reconstructive operations cannot be performed.

Adolescent↗
PubMed1985

The clinical features and diagnosis of a discordant atrioventricular connexion.

Seventy-three patients were studied with a discordant atrioventricular connexion (ages 3 months to 46 years). In 58 the diagnosis was proved by angiography and in the other 15 at necropsy. Forty-eight had usual atrial arrangement and 25 mirror-image atria. Fifty-two patients had ventriculo-arterial discordance, 13 double outlet right ventricle, 4 ventriculoarterial concordance, 3 single outlet of the heart and 1 double outlet left ventricle. Nine with ventriculoarterial discordance had no other associated defects. The spatial relationship of the ventricles was variable, but in usual atrial arrangement the morphologically left ventricle tended to be on the right, and in mirror-image arrangement to the left of the morphologically right ventricle. The spatial relationship between the arterial valves was also variable, but in ventriculoarterial discordance and double outlet right ventricle the aortic valve was anterior and either to the right or left of the pulmonary valve in all but 3 cases. There is no typical clinical picture for these malformations. Symptoms depend upon the associated anomalies or the presence of atrioventricular dissociation. The diagnosis of a discordant atrioventricular connexion is best achieved by the echocardiographic and haemodynamic studies, but the electrocardiogram, chest radiograph and nuclear medicine studies may suggest its presence. Most patients are asymptomatic and progress normally to adult life. Only the symptomatic patients require surgical correction. Postoperative follow-up in the survivors is excellent, only 2 of 14 patients dying after surgery.

Adolescent↗
PubMed1983

Congenital absence of pulmonary valve leaflets.

Congenital absence of pulmonary valve leaflets is an uncommon condition usually associated with ventricular septal defect and an obstructive pulmonary valve ring. Twenty-one patients with these malformations are described. Twenty had an associated ventricular septal defect with ventriculoarterial concordance, and one also had transposition of the great arteries, ventricular septal defect, and obstructive pulmonary valve ring. The clinical features, cardiac catheterisation findings, and angiocardiographic results are presented. Twelve patients underwent cardiac surgery. Three patients died, one in the early, and the other two in the late postoperative period. The results, according to the surgical technique employed and postoperative cardiac catheterisation findings, showed that patients in whom the bioprostheses were implanted in the pulmonary position had a better late follow-up.

Adolescent↗
PubMed1982

Congenital absence of the pulmonary valve leaflets associated with complete transposition of the great arteries.

We describe the clinical and surgical findings of a patient with absence of the pulmonary valve leaflets and complete transposition of the great arteries. The main clinical features were a loud, single second heart sound, a loud pansystolic murmur over the entire precordial area, but no diastolic murmurs were heard. The electrocardiogram suggested right ventricular hypertrophy and right bundle branch block. The roentgenographic study showed cardiomegaly with dilatation of both branches of the pulmonary artery. Cardiac catheterization and angiocardiography showed systemic arterial desaturation, discordant ventriculo-arterial connections and absence of the pulmonary valve leaflets. The patient underwent surgical correction by the Rastelli technique with a favorable clinical course.

Blood Pressure↗
PubMed1980

Crossed atrioventricular connections.

Four cases of crossed atrioventricular connections are described. All of them were diagnosed at cardiac catheterization by angiocardiography and one was examined pathologically. Two possessed situs solitus, one with concordant connections and the other with discordant connections; the other had two situs inversus, both of them with concordant connections. Two had double-outlet right ventricle, one had transposition of the great arteries, and the other had normally related and connected great arteries. These cases have been interpreted as representing abnormal rotation of the ventricles following sepatation. A review of 36 cases previously reported on and our own cases, suggests that most patients have concordant atrioventricular connections. There are many types of ventriculo-arterial connections, the most frequent being transposition of the great arteries. There has not been any case reported with persistent truncus arteriosus. On the basis of atrioventriculo-arterial connections, we propose a classification for this malformation. We discuss the importance of the bulboventricular loop in the type of atrioventricular connections, some clinical implications for the diagnosis, and analyze the value of the rules to localize the ventricles by means of the position of the great arteries.

Adolescent↗
PubMed1980

Angiographic studies of atrioventricular discordance.

The results and value of conventional and axial angiocardiography were studied in 26 cases of atrioventricular discordance (AVD). The angiographic anatomy of this malformation was analyzed to determine the precise characteristics of atrioventricular and ventriculoarterial connections. The left and right ventricles in atrial situs solitus and levocardia show angiographic features that differ from those seen in atrial situs solitus and dextrocardia. The same differences are found in atrial situs inversus. Regardless of the type of ventriculoarterial connections, the ventricles maintain the same angiographic characteristics. The ventricular septum in AVD in situs solitus and levocardia and situs inversus and dextrocardia has a peculiar orientation. The use of axial angiocardiography facilitates detection and localization of the ventricular septal defect. In cases of malposition of the heart, the conventional frontal view allows visualization of the ventricular septum in all of its length. The axial projection is useful in diagnosing left ventricular outflow tract obstruction.

Adolescent↗
PubMedDate not supplied

[Aortic atresia. Presentation of 7 cases].

The clinical and anatomic findings of 7 patients with aortic atresia were studied. All cases had usual atrial arrangement, atrioventricular and ventriculoarterial concordance. All but one had an intact ventricular septum. All cases presented a hypoplastic left ventricle, and in one, a mitral atresia was found. The clinical diagnosis was made by means of cardiac catheterization and angiocardiography. The presence of a patent ductus arteriosus, the size of the atrial and ventricular septal defects, the diameter of the ascending aorta, pulmonary vascular resistances and right ventricular function are all factors that influence the survival in the patients with aortic atresia.

Abnormalities, Multiple↗
PubMedDate not supplied

[Malposition of the great arteries. Is this term a valid one?].

We present four cases of a congenital heart malformation classically known as malposition of the great arteries. This term means there are concordant ventriculo-arterial connections with abnormal spatial relations of the aortic and the pulmonary arteries, in which the former is anterior relative to the latter, and either to the right or to the left, according to the position of the corresponding ventricle. This anomaly can be found in any type of atrial situs, notwithstanding the type or mode of atrioventricular connection, as in the cases we studied. If we consider that this anomaly depends on the associated lesions in order to produce physiological heart alterations, we think that the term of malposition of the great arteries is not valid, since in the anatomic diagnosis of congenital heart disease it is important to consider the connections between atria, ventricles and arteries, the spatial relations between them being of second importance.

Adolescent↗
PubMedDate not supplied

[Double inlet left ventricle].

Twenty-five cases of double-inlet left ventricle, 23 in atrial situs solitus and 2 with dextroisomerism are described. Twenty had both atrioventricular valves and in 3 a common atrioventricular valve connected to the left ventricle. In rest, the left atrioventricular valve was straddling over the left-sided right ventricle in about 15%. The position of the rudimentary right ventricle varied from superior, anterior and to the right of left ventricle (6 with transposition and 9 with concordant ventriculo arterial connection), to superior anterior and to the left of left ventricle (10 cases, all with transposition). Five cases had stenosis of the left atrioventricular valve and 2 stenosis of the right one. In 11, the clinical presentation was dominated by cyanosis, reduced pulmonary blood flow and right to left shunt. The rest had cyanosis and congestive heart failure. Five patients with right ventricle on the left had complete atrioventricular block, 9 right AQRS orientation and all of them findings of left ventricle hypertrophy. Three cases showed initial Q wave in VI and one left bundle branch block. Cases with right ventricle on the right, had left QRS orientation, left ventricle hypertrophy and 2 complete atrioventricular block. Cross-sectional echoes showed in 13, two atrioventricular valves committed to the left ventricle; in 3 of them the right ventricle was visualized. In 22 cases angiography determined the type of atrioventricular connection. Double inlet left ventricle is one of the most challenging diagnosis in congenital heart disease. Considering its lack of clinical specificity and according to our results, the electrocardiogram, echocardiogram and angiographic findings are of great help for its identification.

Adolescent↗
PubMedDate not supplied

[Double outlet right ventricle with discordant atrioventricular connection. Clinical study].

The clinical and anatomic findings were reviewed in 17 patients with double-outlet right ventricle and atrioventricular discordance. Ten cases had atrial situs solitus, seven with right-sided heart three with left-sided heart. Seven cases had atrial situs inversus, five with left-sided heart and two with right-sided heart. All cases presented ventricular septal defect, 13 subvalvar pulmonary stenosis, two tricuspid regurgitation and two complete atrioventricular block. The spatial relationship between the arterial valves are variable. Most cases in atrial situs solitus had a left-sided and anterior aorta and all patients in atrial situs inversus had a right-sided and anterior aorta. In this study we compared the anomalies found in our cases with double outlet right ventricle with those in 58 patients with corrected transposition. Absolute, relative and attributable risks were calculated for the presence of subvalvular pulmonary stenosis, ventricular septal defect, tricuspid regurgitation and atrioventricular block for each the two groups. We concluded that patients with double-outlet right ventricle are more prone to present ventricular septal defect and subvalvar pulmonary stenosis, while those with corrected transposition have a greater likelihood of presenting with tricuspid regurgitation and atrioventricular block. There is no typical clinical picture for the malformations. Symptoms depend upon the associated anomalies. The final diagnosis is best achieved by the echocardiographic and angiocardiographic studies, but electrocardiogram and chest radiograph may suggest the presence of a discordant atrioventricular connection.

Adolescent↗
PubMedDate not supplied

[Anomalies of the urinary tract associated with congenital heart diseases].

Due to the frequent association of congenital heart disease and urinary tract malformations we studied 434 patients undergoing angiocardiograms for diagnosis of their congenital heart disease. In every patient a plain abdominal X-ray film was made in order to observe urinary elimination of the contrast material. We found 70 patients with urinary tract malformations, the most frequent of which was pyelo-chaliceal dilatation, accounting for 47.1% of all the malformations found. A double pyelo-chaliceal system was found in 25.7%, kidney hypoplasia in 10% and in lower frequencies double urethers, kidney agenesia, pelvic kidneys, horseshoe-shaped kidney and others. Patients were usually urologically asymptomatic. In view of the frequent association of congenital heart disease and urinary tract malformations a plain abdominal X-ray film should always be performed postangiographically. In some cases a contrast urography is recommended.

Adolescent↗