Biomedical subjects
Publications and source records attributed to S Ozkutlu.
A seven year old boy with seronegative systemic onset juvenile chronic arthritis who developed dilated cardiomyopathy, is presented. Treatment with methotrexate, prednisone, and digitalis has been successful in controlling the acute phase of the disease and congestive heart failure.
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.
Cor triatriatum (CT) is a rare congenital cardiac anomaly. A case is presented of subdivided left atrium, corrected surgically, which can be considered a type of CT not previously reported.
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Left atrioventricular (A-V) valve anomalies are very frequent in corrected transposition, but left A-V valve replacement has seldom been reported. These anomalies are an important cause of morbidity and mortality in these patients, and due to anatomical presentation valve replacement may be difficult. We present 4 cases of left A-V valve replacement with emphasis given to Ebstein's anomaly of the left A-V valve.
Cardiac involvement in Gaucher's disease has been reported in only a few patients, mostly adults with pericardial changes. We describe findings in two siblings with Gaucher's disease, aged 15 and 9 years respectively, in whom mitral and aortic valve lesions were evaluated by auscultation and echocardiography. In both cases the mitral and aortic valves were thick and restricted in motion. Continuous Doppler echocardiography revealed significant mitral regurgitation and mitral stenosis. At the aortic valve level there was a systolic pressure gradient. Echocardiographic investigation of patients with suspected cardiac involvement with Gaucher's disease is recommended.
Perigraft seroma is a specific clinicopathologic entity, which is a collection of clear, sterile fluid, confined within a nonsecretory fibrous pseudomembrane surrounding a vascular graft. Serous leaks and/or seroma formation from polytetrafluoroethylene grafts have been reported in peripheral arteriovenous anastomoses, arterial grafts and subclavian pulmonary anastomoses. We present a case of seroma formation which developed after a Blalock-Taussig shunt operation constructed with a polytetrafluoroethylene graft in a child with tricuspid atresia and which was diagnosed by echocardiography.
We present a rare complication of balloon atrial septostomy in an infant with complete transposition of the great arteries and review the management of this problem. The balloon could not be deflated after the eighth pass across the atrial septum. We punctured the balloon with a stylet which was passed through the circulation lumen of the double-lumen septostomy catheter. We suggest that this method should once be tried before other methods are employed.
We present a 3-yr-old girl with coarctation of aorta and patent ductus arteriosus in whom mycotic aneurysm and bacterial endarteritis developed postoperatively and was diagnosed by two-dimensional and Doppler echocardiography. Five weeks after the operation of ligation of ductus and resection of coarctated segment, the patient was readmitted with complaints of vomiting, fever and coughing. Bacterial endarteritis, empyema and septic arthritis were diagnosed. Suprasternal echocardiographic examination demonstrated an aneurysmatic appearance 60 x 65 mm in size at the location of coarctation. The patient died, most probably due to aortic rupture, before surgical treatment could be undertaken. Autopsy study confirmed our diagnosis.
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.
Blade atrial septostomy has been applied in 3 patients with transposition of the great arteries under two-dimensional echocardiography instead of fluoroscopy. The blade at the tip of the catheter was seen on an echo screen when it was in the left atrium. The blade was then extended and pulled slowly across the atrial septum from the left into the right atrium, under echocardiographic monitoring. The procedure was successful in all 3 patients. Interatrial communication measured echocardiographically was sufficient in size. Since the interatrial septum is clearly seen by means of echocardiography during blade atrial septostomy, the method is thought to be superior to fluoroscopy in the prevention of complications.
We present a patient with left ventricular thrombus diagnosed by two-dimensional echocardiography. Thrombosis was due to acquired transient protein C deficiency which was caused by impaired liver function due to hepatitis, sepsis and heart failure. With proper treatment the thrombus disappeared on the fourth day. Eighteen weeks later the protein C level returned to normal. We recommend echocardiographic evaluation and follow-up of suspected cases for intracardiac thrombus. The measurement of protein C level in such cases is proposed. This is the first case with left-sided cardiac thrombus associated with protein C deficiency in the medical literature.
Noninfective endocarditis may develop on heart valves in a wide variety of clinical conditions. Various events have been cited as possible etiologic factors. These lesions are clinically important because the vegetations frequently embolize and cause arterial obstruction and tissue infarction. Previously, the diagnosis of the disease had been made only at autopsies. Only a single case has been reported to have been diagnosed clinically in childhood. We present a patient with noninfective endocarditis, urgently operated on with the presumptive echocardiographic diagnosis of tricuspid valve myxoma, whose protein C level was found to be very low. It's known that in patients with homozygous congenital protein C deficiency venous thrombosis may develop. We think that the etiologic factor of the thrombosis on the tricuspid valve in the case presented is congenital protein C deficiency. With this case study we further emphasize the specific role of two-dimensional echocardiography in the diagnosis of noninfective endocarditis and recommend that protein C deficiency be investigated as an etiologic factor.
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.
Cardiac myxomas are extremely rare in infancy. We report a case of right atrial myxoma in a 35-day-old male infant (with cyanosis and convulsions). Echocardiography was carried out and a diagnosis of right atrial myxoma was made. Open heart surgery was performed using cardiopulmonary bypass and a 2.5 X 3.0 cm mass was removed. The patient's postoperative course was uneventful. To our knowledge there is no previously reported case of right atrial myxoma in such a young infant which was operated on successfully.
Primary cardiac tumors are quite rare in the newborn period. Prior to surgery, cardiac catheterization and angiocardiography have been performed to confirm the two-dimensional echocardiographic findings. In this report a 2-day-old baby with the clinical impression of severe cyanotic congenital heart disease diagnosed by two-dimensional echocardiography as multiple rhabdomyoma and confirmed by surgery is presented. It is emphasized that two-dimensional echocardiography is a very useful technique for the diagnosis of primary cardiac tumors. By means of this method severely symptomatic newborns may be taken directly to surgery without prior cardiac catheterization, thus minimizing complications.
Infections occurring after aortic valve surgery, whether valvuloplasty or replacement, commonly affect the valve itself. However infection of the aortic suture line alone is extremely rare. Such cases with endarteritis can be diagnosed at autopsy or by angiocardiography. In this report a patient with a vegetation at the aortic suture line which was diagnosed by echocardiography is presented. By two-dimensional echocardiography, a very mobile and echo-dense mass protruding from the ascending aorta was observed on the recordings through the suprasternal notch. This diagnosis was surgically confirmed and the patient was treated.