Biomedical subjects
Publications and source records attributed to S Ozme.
Partial anomalous pulmonary venous return (PAPVR) is a congenital anomaly in which one or more, but not all, of the pulmonary veins are connected to a systemic vein or to the right atrium directly. Its incidence is higher in autopsy series than in clinical series. We report 51 cases of PAPVR diagnosed by cardiac catheterization and evaluated from the aspects of age, sex, type and associated anomalies and diseases.
Eleven cases of intracardiac thrombi caused by different factors including protein-C deficiency are presented for discussion of the etiology and predisposing factors of intracardiac thrombi during infancy and childhood, and to stress the importance of protein-C deficiency as an etiological factor. Thrombi were localised in the left heart in five patients and right heart in five patients. One patient had both-sided thrombi. Four of our patients had dilated cardiomyopathy, one had mitral valve hypoplasia, and one had pulmonary valvar stenosis as the predisposing factors for thrombus formation. In three patients whose cardiac anatomies were completely normal, we determined protein-C deficiency as an etiological factor of thrombus formation. One of these had congenital protein-C deficiency and the other two had acquired temporary protein-C deficiency due to sepsis. In conclusion we recommend that protein-C deficiency should be investigated as an etiological factor in all cases of intracardiac thrombi irrespective of whether or not another predisposing factor is identified.
We prospectively evaluated 80 patients with syncope, between January 1991 and January 1992 to determine the causes of syncope in children. There were 35 male and 45 female patients, whose mean age was 10.5 years. A single syncopal attack had occurred in 30 patients and multiple attacks in 50. A cardiovascular cause was established in 22 (27.5%) patients and a noncardiovascular cause in 36 (45%). The cause remained unknown in 22 patients (27.5%). Vasovagal syncope was the leading cause of syncope in these patients with an incidence of 32.5%. These findings suggest that every patient who has even one syncopal attack should be promptly investigated since the underlying cause could be a life-threatening one.
The purpose of this paper is to present the authors' 3-yr experience of echocardiographic examination of patients with the clinical diagnosis of tetralogy of Fallot, and their evaluation for surgical treatment without prior cardiac catheterization. Among the patients with the clinical diagnosis of tetralogy of Fallot 227 had a definite diagnosis made by M-mode, two-dimensional, Doppler and contrast echocardiography. For the diagnosis of tetralogy of Fallot, ventricular septal defect, pulmonary stenosis, and overriding of the aorta were considered to be fundamental. Ventricular septal defect could be seen easily in the subaortic region by two-dimensional echocardiography. However, in some patients whose ventricular septal defect was not seen clearly, peripheral vein contrast echocardiography was performed. The diameters of pulmonary artery, and main branches at a few millimeters distal to their origin were measured. These parameters were correlated with the aortic diameter for evaluation as to whether they were able to accept the total cardiac output. In patients whose left ventricular end-diastolic dimension was small, shunt operation was preferred. In 115 patients the pediatric cardiologist performing the echocardiography thought that cardiac catheterization was necessary. In these cases the reliability of echocardiography in detecting important cardiac abnormalities was evaluated. Detection of ventricular septal defect, presence of pulmonary valve, detection of stenosis on the pulmonary bifurcation and/or main branches revealed a high sensitivity. Two-hundred-and-one patients diagnosed by echocardiography underwent total correction. In all cases except one the preoperative diagnosis was confirmed by surgery.(ABSTRACT TRUNCATED AT 250 WORDS)
This study evaluated diagnostic criteria, clinical observations, surgical indications and complications in 90 cases of fixed subvalvular aortic stenosis. Echocardiography, catheter angiography and surgical findings indicated that 60 (66.6%) patients had subvalvular membranous stenosis, 20 (22.2%) patients had fibromuscular stenosis, 4 (4.4%) patients had membranous and fibromuscular stenosis, and 6 (6.6%) had a tunnel-type obstruction. Forty-two patients had additional cardiac anomalies, such as PDA, valvular aortic stenosis and VSD. Fifty patients were treated surgically. Thirty-six patients were evaluated by two-dimensional and Doppler echocardiography at post-surgical intervals ranging from 7 days to 11 years (mean 3.6 years). There were pathological echo findings in 12 patients. The prognosis of this anomaly depends upon early diagnosis and treatment. The results of this study support the importance of the two-dimensional and Doppler echocardiography in early diagnosis and follow-up of the surgical results in treatment of this anomaly.
Eighteen children (10 females, 8 males; age range 5.5 to 17 years, median: 10 years) who suffered from recurrent syncope, drug refractory supraventricular tachycardia and atrioventricular block or bradycardia were evaluated by cardiac electrophysiologic study between August 1988-April 1990. During the study, basal intervals were measured and the conduction system and sinus node functions were investigated. In some patients the mechanism of the tachycardia was investigated and drug-electrophysiologic studies also performed. Two of 6 cases with recurrent syncope had positive electrophysiologic findings and adequate treatment resolved the symptoms. The others have been followed. The mechanism of the tachycardia was determined in 5 cases with drug refractory supraventricular tachycardia and drug-electrophysiologic studies were done in all of them. The site of block was determined in 7 patients with atrioventricular block and pacemaker implantation was done in 5 patients. The results emphasized the usefulness of cardiac electrophysiologic study in childhood arrhythmias.
Endomyocardial biopsy studies in adults have demonstrated the usefulness of this method. It is possible that studies will be more productive in determining the etiology and clinical status in patients with clinically diagnosed myocardial diseases. A prospective study conducted over 16 months included 17 children, aged 14 months to 18 years, with the diagnosis of dilated, restrictive cardiomyopathy and myocarditis. In 16 patients right, and in 1 patient left heart endomyocardial biopsies were performed. The specimens were evaluated by light and electron microscopy. There were no serious complications after the procedure. In 1 of 17 children histology showed no myocardial tissue. Electron microscopy evaluations were currently available in 9 patients. Endomyocardial biopsy findings were found to be diagnostic in 41.2%, helpful in 29.4% and of no help in 29.4% of patients. In conclusion, endomyocardial biopsy technique is highly sensitive in children with myocardial disorders. In future it will be the major diagnostic tool for invasive but safe detection of myocardial disease.
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Right-sided endocarditis is rare in children. Since the clinical picture is nonspecific and the frequency of sterile blood cultures is high, the clinical and laboratory diagnosis is difficult. Recent reports suggest that echocardiography is a useful method to detect the presence of right-sided endocarditis. We studied 8 patients with congenital heart disease and right-sided endocarditis detected by two-dimensional echocardiography. All the patients were shown to have one or more vegetations in the two-dimensional echocardiographic examination. The 4 patients who did not respond to antibiotic therapy underwent elective cardiac surgery. One of these patients additionally had recurrent emboli to the lungs. Another of these 4 patients died during surgery due to myocardial failure. Apart from these 4 cases, urgent surgical intervention had to be carried out in 2 cases because of very large vegetative masses. Surgical confirmation of the diagnosis was available in all 6 cases. According to these results, we can conclude that two-dimensional echocardiography assumes a specific diagnostic role in cases with right-sided endocarditis.
In this article, the clinical and epidemiological characteristics of 137 children with cardiomyopathy admitted to the Hacettepe Pediatric Cardiology Unit were studied and the prognosis was evaluated after a follow-up period of 24 +/- 5 months. It was found that the highest proportion of patients were residents of Ankara, followed by the Northern and the Central Anatolian regions, and consanguinity between their parents was more common than the proportion for Turkey as a whole. Most of the patients had dilated cardiomyopathy (78.9%) and the age at which symptoms appeared varied according to the type of cardiomyopathy. A high proportion of patients came to the hospital with complaints of dyspnea and a decrease in effort capacity. The most common findings on the physical examination were hepatomegaly and tachycardia. Electrocardiographic and echocardiographic evaluations were made for all patients and cardiac catheterization and endomyocardial biopsy were performed when necessary. The outcome was as follows: 34.5% improved, 45.7% remained stable, 9.5% deteriorated and 10.3% died.
Six patients with d-transposition of the great arteries and 1 patient with tricuspid atresia were diagnosed echocardiographically. Balloon atrial septostomy (BAS) was performed successfully in all cases with the aid of two-dimensional echocardiography. Echocardiography may be superior to fluoroscopy during BAS because it permits easy detection of spontaneous passage of the inflated catheter into the left ventricle spontaneously through the mitral valve (like a left atrial myxoma). With the visualization of related cardiac structures during BAS, obstruction of the IVC with the balloon was also prevented. Using this method, bedside application of BAS is possible.
This study was performed on 20 children with congenital heart defect and left-to-right shunt. These patients were divided into two groups. Group I consisted of 10 patients with normal pulmonary arterial pressure while group II consisted of 10 patients with pulmonary hypertension. Prostaglandin Like Materials (PLMs) in pulmonary venous and arterial blood which were taken during cardiac catheterization were extracted and biologically assayed for Prostaglandin-Like Activities (PGLA) on rat stomach fundus. In all patients of group I, the ratio of pulmonary venous to pulmonary arterial PGLA levels (PV/PA-PGLA) was found to be less than one. However in group II, this ratio was less than one in only two patients. In 8 out of the patients in group II, the ratio was either 1 or greater. These data confirmed the early concepts suggesting the role of prostaglandins (PG) in regulating normal pulmonary circulation. In addition these data have suggested that PGs may have an important role in the development of pulmonary hypertension in some cardiac patients.
A 10-year-old boy with aortico-left ventricular tunnel, a very unusual congenital cardiovascular malformation, is presented. Closure of both orifices of the tunnel by round teflon patches is recommended. We think this surgical technique is superior to closure of these openings by primary sutures only, which can result in stretching and distortion of the aortic ring and leaflets leading to postoperative residual aortic regurgitation.
A case of pulmonary artery sling in a 1-year-old boy, treated by resection of the left pulmonary artery (LPA) at its origin from the right pulmonary artery (RPA) and reanastomosis of the LPA to the main pulmonary artery (MPA), is reported. In this patient the LPA-MPA anastomosis was demonstrated to be patent in a follow-up pulmonary angiogram six months after the operation. Pulmonary artery sling should be kept in mine when assessing respiratory distesss syndrome in children.
Valvar heart disease is a rare complication of juvenile rheumatoid arthritis (JRA), the aortic valve being most commonly affected. Reported cases with symptomatic mitral involvement are rare. We describe a 13-year-old boy with seronegative, polyarticular onset of JRA in whom mitral and aortic valve insufficiency was diagnosed by clinical and laboratory investigations. Two-dimensional and continuous-wave Doppler echocardiography confirmed mild pericardial effusion with moderate mitral and mild aortic insufficiency. Cardiac assessment and echocardiographic follow-up are recommended in all patients with JRA.
Unexplained syncope may cause diagnostic and therapeutic problems in children. The head-up tilt test has been shown to be a useful tool for investigating unexplained syncope, especially for diagnosis of neurally mediated syncope. In this study 20 patients aged 9-18 years (12.0 +/- 2.5 years) with syncope of unknown origin and 10 healthy age-matched children were evaluated by head-up tilt to 60 degrees for 25 minutes. The test was considered positive if syncope or presyncope developed in association with hypotension, bradycardia, or both. If tilting alone did not induce symptoms (syncope or presyncope), isoproterenol infusion was administered with increasing doses (0.02-0.08 mu g/kg per minute). During the tilt test, symptoms were elicited in 15 (75%) of the patients with unexplained syncope but in only one (10%) of the control group (p < 0.001). The sensitivity of the test was 75% and its specificity 90%. Three patterns of response to upright tilt were observed in symptomatic patients: vasodepressor pattern with an abrupt fall in blood pressure in 67%; cardioinhibitory pattern with profound bradycardia in 6%; and mixed pattern in 27%. In patients with positive head-up tilt, there were sudden decreases in systolic blood pressure (from 130 +/- 15 to 61 +/- 33 mmHg) and in mean heart rate (from 147 +/- 26 to 90+/-38 beats per minute) (p < 0.001) during symptoms. Treatments with atenolol 25 mg/day has shown complete suppression of syncope in positive responders during a mean follow-up period of 18 +/- 6 months. The head-up tilt test is a noninvasive, sensitive, specific diagnostic tool for evaluating children with unexplained syncope.
A group of 67 children with cyanotic congenital heart disease (CCHD) were studied, and 35 were given iron treatment according to a regimen that gives iron to patients with a hematocrit (Hct) below 60%. The patients were categorized as iron-deficient and iron-sufficient according to their transferrin saturation and ferritin values. The pretreatment hemoglobin (Hb) and Hct values of the groups were similar. The mean Hct was nearly three times as much as the mean Hb in the iron-sufficient group and more than three times as much as the Hb in the iron-deficient group. Excessive erythrocytosis in the iron-deficient group was impressive. Mean corpuscular volume (MCV) values were below 72.7 fl in all of the iron-deficient patients. After treatment the Hb, Hct, transferrin saturation, and ferritin increased significantly in both groups, with the increments greater in the iron-deficient group. Increments in the erythrocyte (RBC) count were significant in the iron-sufficient group but insignificant in the iron-deficient one. Increments of MCV in the iron-deficient group were significant but insignificant in the iron-sufficient group. Our study demonstrated that prediction of Hb, RBC count, and MCV, measurements of which are easy and inexpensive and require little blood, can suffice for the diagnosis of iron deficiency in patients with CCHD without altering systemic perfusion.