About unilateral absence of intrapericardial pulmonary artery.
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Blood Vessel Prosthesis Implantation↗
Biomedical subjects
S Ozkutlu
Publications and source records attributed to S Ozkutlu.
Thrombosis in children with cardiac pathology: frequency of factor V Leiden and prothrombin G20210A mutations.
This study analyzed 28 thrombotic children with various cardiac disorders. They comprise 17% of a total of 168 patients with thrombosis from a single center. Among the 28 patients, 21 had congenital heart disease and 7 were diagnosed with cardiomyopathy. The patients with thrombosis were evaluated for congenital and acquired thrombotic risk factors. In addition to cardiac disorders, two, three, or more risk factors were present in 61% of the children with thrombosis. Two common mutations, namely factor V Leiden and prothrombin G20210A mutations, were found in 6 patients (22%). Nine patients (32%) died of infection, congenital heart disease, cardiomyopathy, thrombosis, operation, or a combination of these; two patients required surgical intervention. Following cardiac angiography, due to necrosis, amputation of the right index finger and right lower extremity was performed on 1 patient. The second patient's index fingers had to be amputated and resection of the bowel was performed following the operation on coarctation of the aorta. This study indicates that congenital heart disease and cardiomyopathy are two common cardiac disorders that may lead to the development of thrombosis. The majority of thrombosis develops within the heart and/or its great vessels. The second predominant site for thromboembolic symptoms is in the brain, including sinovenous thrombosis.
Adolescent↗
Truncus arteriosus with a very small ventricular septal defect diagnosed by echocardiography.
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Child↗
A late complication with the CardioSEAL ASD occluder device and need for surgical revision.
A late complication of the CardioSEAL atrial septal defect (ASD) occluder is reported. Although left atrial umbrella was completely epithelialized and occluded ASD without residual defect, the right atrial umbrella protruded toward the center of right atrium after 18 months. We believe this may be associated with the structural abnormality of the device.
Adolescent↗
Can subclinical valvitis detected by echocardiography be accepted as evidence of carditis in the diagnosis of acute rheumatic fever?
AIM: Subclinical valvar insufficiency, or valvitis, has recently been identified using Doppler echocardiography in cases of acute rheumatic fever with isolated arthritis or chorea. The prognosis of such patients with acute rheumatic fever and subclinical valvitis is critical when determining the duration of antibiotic prophylaxis. We aimed, therefore, prospectively to investigate the association of silent valvitis in patients having rheumatic fever in the absence of clinical evidence of cardiac involvement, and to evaluate its prognosis. METHODS AND RESULTS: Between November 1998 and September 1999, we identified 26 consecutive patients with silent valvitis in presence of rheumatic fever but in the absence of clinical signs of carditis. The patients, eight female and 18 male, were aged from 6 to 16 years, with a mean of 9.9+/-2.7 years. Major findings were arthritis in 16, chorea in 7, and arthritis and erythema marginatum in 1 patient. Two cases had arthralgia with equivocal arthritic signs and Doppler echocardiographic findings of pathologic mitral regurgitation. Silent pathologic mitral regurgitation was found in 12 cases, and aortic regurgitation in 2 cases. All patients with arthritic findings were treated with acetylsalicylic acid with one exception, this patient receiving both prednisone and acetylsalicylic acid. No antiinflammatory treatment was given to patients with chorea. After a mean follow-up of 4.52 months, valvar regurgitation disappeared in 4 patients, including the one with migratory arthralgia and no other major criterions. All six patients with chorea and silent carditis still have mitral insufficiency. CONCLUSION: Acute rheumatic fever without clinical carditis is not a benign entity. Doppler echocardiographic findings of subclinical valvar insufficiency, therefore, should be considered as carditis when seeking to establish the diagnosis of acute rheumatic fever.
Acute Disease↗
Bidirectional Glenn shunt in an infant with prune-belly syndrome.
The prune-belly syndrome (PBS) usually is described as a deficiency of the anterior abdominal muscle involving bilateral cryptorchidism and urinary tract malformations. In this report, we will present an eleven-month-old boy with PBS associated with a complex cardiac anomaly. A bilateral bidirectional Glenn shunt was performed with the diagnosis of isolated dextrocardia, single ventricle, pulmonary atresia, incomplete A-V septal defect, hemiazygos continuity, persistent right superior vena cava, patent ductus arteriosus-dependent pulmonary blood flow. The patient required special consideration for postoperative pulmonary care.
Abdominal Muscles↗
Isolated cardiac metastasis of osteosarcoma.
Osteosarcoma metastasis to the heart caused by tumor thrombosis is very rare. A 7-year-old girl with an osteosarcoma of the right humerus, refused the treatment, at first, then, 1 year later, referred to the hospital with metastasis to the heart. The mass invading the pulmonary arteries was successfully removed with open-heart surgery.
Biopsy, Needle↗
Double outlet left ventricle with subpulmonary ventricular septal defect and pulmonary hypertension.
A two-month old male infant with the rare occurrence of double outlet left ventricle, subpulmonary ventricular septal defect and pulmonary hypertension is presented. The infant was managed temporarily with banding of the pulmonary trunk, with a favorable result, and is scheduled for definitive intraventricular repair.
Heart Septal Defects, Ventricular↗
Beneficial effects of 1-year captopril therapy in children with chronic aortic regurgitation who have no symptoms.
OBJECTIVE: This prospective study was performed to assess the effects of 1 year of angiotensin-converting enzyme inhibition with captopril in 20 children (mean age 14.3+/-2.3 years) with asymptomatic chronic aortic regurgitation. METHODS AND RESULTS: At 12 months patients receiving captopril had a significant reduction in left ventricular end-diastolic and end-systolic dimensions (57+/-9.3 vs 51+/-9.5 mm, p < 0.001; 35.4+/-6.1 vs 32+/-6.8 mm, p < 0.001), end-diastolic and end-systolic volume indexes (111+/-36 vs 94+/-29 ml/m2, p < 0.001; 35+/-13 vs 30+/-12 ml/m2, p < 0.001, respectively), and mass index (138+/-37 vs 109+/-32 gm/m2, p < 0.0001) determined by two-dimensional echocardiography. Meridian (p < 0.01) and circumferential (p < 0.0001) wall stresses also decreased significantly with therapy. Significant reduction (27.8%, p < 0.0001) was achieved in regurgitant fraction with captopril. CONCLUSIONS: These data show that the long-term therapy with angiotensin-converting enzyme inhibitors is able to reverse left ventricular dilation and hypertrophy and suggest that such therapy has the potential to favorably influence the natural history of the disease in children.
Administration, Oral↗
Posterior deviation of left ventricular outflow tract septal components without ventricular septal defect.
OBJECTIVE: To describe 11 patients with narrowing of the left ventricular outflow tract caused by angular posterior deviation of both the outlet septum and the upper part of trabecular septum, which was diagnosed by cross sectional echocardiography in all and confirmed by angiocardiography in seven. RESULTS: Four patients had a subaortic systolic pressure gradient ranging from 23 to 70 mm Hg by Doppler echocardiography; cardiac catheterisation showed a significant (60 and 104 mm Hg) systolic pressure gradient in two. In four cases aortic regurgitation and two tricuspid pouches were shown by Doppler echocardiography, angiocardiography, or both. Four cases had a ridge at the angulation point on echocardiographic examination. Three patients were operated on for systolic pressure gradients of the left ventricular outflow tract and one for severe aortic regurgitation. There was proliferation of collagen-rich fibrous tissue in the subendocardial region on histopathological examination of the myectomy material. A ventricular septal defect had been diagnosed previously by contrast echocardiography in one patient; thus ventricular septal defects may close spontaneously over a period of time including fetal life. A subaortic ridge was detected in one patient at follow up. CONCLUSIONS: Deviation of the outlet and trabecular septa should be considered as a cause of ventricular outflow tract obstruction even when no ventricular septal defect is present.
Adolescent↗
Unguarded tricuspid orifice: a rare malformation of tricuspid valve diagnosed by echocardiography. Report of two cases and review of the literature.
In this report we present two cases with unguarded tricuspid orifice, and review the literature. The complete absence of tricuspid valvular structures is a very rare anomaly coexisting with pulmonary atresia and intact ventricular septum, and is termed unguarded tricuspid orifice. Double tricuspid orifices and isolated dextrocardia were distinguishing features of our first case. The second patient of this report, a 5-year-old boy, is the first case in the literature with this malformation of the tricuspid valve as an isolated anomaly, in addition to being the first surviving case in the literature.
Child, Preschool↗
Effects of respiration on left ventricular diastolic function in healthy children.
Pulsed Doppler echocardiographic indices of mitral valve filling were measured in 20 healthy children, between 3 and 12.5 years old, in order to evaluate the effects of spontaneous respiration on left ventricular diastolic filling patterns. There were significant respiratory variations in four parameters of left ventricular diastolic function: The peak early filling velocity, the ratio of early to late peak filling velocity, and the ratio of early to late diastolic velocity-time integral decreased significantly during inspiration (mean decrease 7%, P < 0.05; 16%, P < 0.01; and 12%, P < 0.05, respectively). On the other hand there was a significant increase in late peak filling velocity with inspiration (10% increase, P < 0.05). Other variables of left ventricular diastolic filling were unchanged with inspiration. These results suggest that assessment of left ventricular diastolic function in children should be standardized with regard to respiratory phases in any clinical application.
Child↗
Subpulmonary and subaortic ridges in doubly committed subarterial ventricular septal defect: an echocardiographic study.
BACKGROUND: Certain conditions, including discrete subaortic stenosis and right ventricular outflow tract obstruction are well-known associations with ventricular septal defect. However, the association of discrete type subpulmonary and subaortic stenosis with ventricular septal defect has not been described to date. PATIENTS: In this report we present our experience in nine patients with doubly committed subarterial ventricular septal defect in whom associated discrete subpulmonary and subaortic stenosis were diagnosed by two-dimensional echocardiography. The mean age of the patients was 5.7 years, and eight of them were male. Two patients had additional hypertrophied anomalous muscle bundles with pressure gradients across the right ventricular outflow tract. Aortic valve prolapse was detected in two patients with one of them having mild aortic regurgitation. INTERVENTION: Cardiac catheterization was performed in four patients, and three underwent surgery. Doubly committed subarterial ventricular septal defect was closed, and subpulmonary and subaortic ridges were resected in each patient. Histological findings were similar for both ridges, which were composed of thin, short and irregularly arranged elastic fibres covered by endothelial cells. RESULTS: The finding of fibrous ridge on both sides of the doubly committed ventricular septal defect, where the turbulence is likely to be maximum, and the histopathological similarities of subpulmonary and subaortic ridges, may suggest a common mechanism for the development of fibrous ridges. CONCLUSION: Serial echocardiographic assessment of patients with doubly committed ventricular septal defect is recommended.
Adolescent↗
Heart disease in Friedreich's ataxia: a clinical and echocardiographic study.
Twelve patients with Friedreich's ataxia (FA) were evaluated clinically and echocardiographically for evidence of heart disease. Electrocardiographic and echocardiographic abnormal findings were discovered in eight (67%) and seven (58%) children, respectively. A high incidence of cardiac involvement is well known in FA cases. Although the patient number in the present study is small, the findings are consistent with those in the literature. The most common pathology was asymmetric septal hypertrophy (ASH), followed by concentric left ventricular hypertrophy (CLVH) and dilated cardiomyopathy (DC).
Adolescent↗
Double-chambered right ventricle: experience with 52 cases.
The presence of anomalous muscle bundles may produce a pressure gradient between the inflow and outflow portions of the right ventricle, thus resulting in double-chambered right ventricle bearing troublesome clinically in its diagnosis. The aim of the present study was to review the diagnostic criteria. Fifty-two patients with a double-chambered right ventricle were seen during an 8-year period. They ranged in age at the catheterization from 4 months to 17 years (mean 7.5 +/- 4.4 years). Diagnosis was confirmed in 51 patients at cardiac catheterization and in other one on operation. The majority of the patients had associated cardiac anomalies: there were 33 ventricular septal defect (63%), 21 pulmonary valve stenosis (40%), nine atrial septal defect (17%), and four double-outlet right ventricle. The electrocardiograms revealed upright T waves alone in right precordial leads suggesting right ventricular hypertrophy in 33% of the patients. At cardiac catheterization, there was a pressure gradient of 20-160 mmHg between the right ventricular inflow and outflow portions. Forty patients have had surgery and four have undergone balloon pulmonary valvuloplasty. Surgical treatment was planned for two patients and other six had no indication for treatment.
Adolescent↗
Adenosine induced torsades de pointes in a child with congenital long QT syndrome.
Torsades de pointes is a rare arrhythmia characterized by its bradycardia dependence and increased adrenergic discharge, whether it occurs as a congenital anomaly or as an acquired problem resulting from drug intoxication or other conditions. There are no reliable tests to assess the propensity toward torsades de pointes or evaluate the efficacy of treatment in these patients. Adenosine can result in marked slowing of sinus and ventricular rate and leads to increased sympathetic discharge when given intravenously. We induced torsades de pointes in a child with congenital long QT syndrome (Jervell-Lange-Nielsen syndrome) using 200 micrograms/kg IV adenosine bolus. Higher dosage of adenosine (600 micrograms/kg) did not lead to torsades de pointes after beta blockade. Adenosine may induce torsades de pointes in patients with the long QT syndrome and may be used as a test to reproduce the clinical arrhythmia. Whether adenosine proves to be useful for assessing the efficacy of treatment will require extensive investigation in larger series of patients.
Adenosine↗
Partial anomalous pulmonary venous return. Evaluation of 51 cases.
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.
Adolescent↗
Echocardiographic findings in some metabolic storage diseases.
This study was planned to determine the presence and extent of cardiac involvement in metabolic storage diseases, including types I and II glycogenoses, Gaucher and Neimann-Pick diseases, galactosialidosis and mucopolysaccharidosis. M-mode, 2-dimensional and Doppler echocardiographic studies were used for the determination of left ventricular wall thickness, systolic function, anatomic derangement, valvular dysfunction and left ventricular diastolic function in the patient and control groups. In 19.35% of the cases valvular involvement, and in 59.26% thickening of the left ventricular wall due to metabolic storage, was found. Left ventricular systolic function was in the normal range. The diastolic mitral flow patterns of the cases showed obstructive type changes. It can be concluded that in this study, besides previously described cardiovascular changes, some new findings have been detected by means of echocardiography in patients with metabolic storage diseases.
Adolescent↗