Facioscapulohumeral syndrome with cardiomyopathy.
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Cardiomyopathies↗
Biomedical subjects
A Celiker
Publications and source records attributed to A Celiker.
Detection of right-sided endocarditis in children with congenital heart disease by two-dimensional echocardiography.
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.
Child↗
Cutaneous anthrax.
The case of a 9-year-old boy diagnosed and treated for cutaneous anthrax is presented. We discuss the clinical features and course of this disease, encountered today only rarely in less developed regions where humans are in contact with infected animals.
Anthrax↗
Head-up tilt test: a highly sensitive, specific test for children with unexplained syncope.
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.
Adolescent↗
Parameters of iron deficiency in children with cyanotic congenital heart disease.
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.
Anemia, Hypochromic↗
Isolated chylopericardium secondary to intrathymic lymphangiomatous malformation.
A case of chylopericardium associated with lymphangiomatous malformation of the thymus is presented. The specific diagnosis of chylopericardium was made by pericardiocentesis and analysis of the fluid. Despite the pericardiocentesis and pericardial tube drainage, pericardial effusion recurred and the patient underwent pericardiectomy. At operation the thymus, which contained a tumor and showed diffuse hyperplasia, was excised and pericardiectomy was performed. The 2-year-old patient recovered uneventfully without reaccumulation of the chyle. The pathologic diagnosis of the mass was intrathymic ectatic lymphangiomatous malformation.
Child, Preschool↗
Torsade de pointes associated with encephalitis.
Torsade de pointes is a polymorphic ventricular tachycardia. Causes of torsade de pointes are well described. Although intracranial disease can produce dramatic electrocardiographic (ECG) changes, we are not aware of previous cases with torsade de pointes and encephalitis. We report a case with encephalitis who developed torsade de pointes, and was treated with temporary ventricular pacing and magnesium infusion.
Child, Preschool↗
The angiocardiographic analysis of 73 patients with double-outlet right ventricle.
The purpose of this study was to determine the cardiac anatomy of patients with double-outlet right ventricle by angiocardiography. A total of 73 patients between the ages of one day and 11 years were examined. The aorta was on the right side of the pulmonary artery in 23 cases (32%), right anterior in 20 (27%) and right posterior in 17 cases (23%). Pulmonary stenosis was found in 53 patients (73%) and subaortic stenosis in six cases. Ventricular septal defect (VSD) was subaortic in 39 cases (52%), remote type in 17 (23%), doubly committed in 10 (13%), and subpulmonic in 9 (12%). Double VSD was noted in two patients. Pulmonary hypertension was more frequent in subpulmonic ventricular septal defect (78%). The most common associated anomalies were atrial septal defect (34%), anomalous coronary arteries (12%) and endocardial cushion defect (10%). Aortic root angiography was not satisfactory in half of the cases with coronary arterial anomaly. In conclusion, double-outlet right ventricle is a complex anomaly, all of whose cardiac features can be successfully demonstrated in detail by echocardiography and angiocardiography. However, in order to determine the anatomy of the coronary arteries, selective coronary angiography may be necessary in some patients.
Angiocardiography↗
Transtelephonic ECG versus electrophysiologic study in children with recurrent palpitation attacks.
Palpitation may be a terrifying event for children and pose diagnostic problems for pediatric cardiologists. Routine methods often fail to document episodic arrhythmia because the episodes may be brief or infrequent or both. The purpose of this study was to evaluate and compare values of non-invasive and invasive techniques. Twenty-three children (mean age 11.7 +/- 2 years, range 7-16 years) with recurrent palpitation attacks (> 2 times), who had normal physical examinations, ECG, echocardiography, 24 hour ambulatory ECG monitoring and treadmill exercise tests, were included in the study. Redline Model CG 4000 TTE recorders were given to patients for 10 or 20 days. We performed an intracardiac electrophysiologic study (EPS) on 14 patients. The mean number of palpitation attacks was 11.3 +/- 7.4 (median: 5), lasting 9.6 +/- 7 (median 3) minutes. The number of transmitting records was 2 +/- 1.4 (1-20). Of the 23 patients, only 15 (65%) transmitted during the palpitation attacks. Twenty-four-hour ambulatory ECG monitoring findings were normal in all patients. One patient had a wide QRS tachycardia attack in the TTE records. We stimulated ventricular tachycardia in the same patient in the EPS. Among the other patients who transmitted TTE records during palpitation attacks, we diagnosed two cases of concealed accessory pathway and eight cases of dual AV nodal pathway in the EPS. In conclusion, TTE is a sensitive and accurate method for diagnosis and follow-up of patients with cardiac arrhythmia. It can be used before invasive studies in children with recurrent palpitation attacks.
Adolescent↗
Adenosine-sensitive right ventricular tachycardia in children.
Right ventricular outflow tachycardia is a distinct subgroup of idiopathic ventricular tachycardia (VT) with characteristic clinical and electrophysiologic properties. This study was planned to evaluate the effects of adenosine on idiopathic right ventricular tachycardia in children, and to assess the long-term efficacy of verapamil treatment. Diagnostic tests including electrocardiogram, chest roentgenogram, echocardiogram, exercise stress test and 24-hour ambulatory electrocardiographic monitoring were performed in each patient. Adenosine was administered in increasing amounts until an effective dose or a maximal dose of 300 micrograms/kg was reached. In adenosine-sensitive patients verapamil was given orally (3-10 mg/kg/day) for long-term suppression of arrhythmia. Seven patients with a mean age of 10.2 +/- 4.7 years were enrolled in the study. There were five male and two female patients. Six patients had VT, and one patient had frequent ventricular ectopic bests. Arrhythmia originated from the right ventricle in all patients. Adenosine was effective in terminating the arrhythmia in all patients, with a mean effective dose of 183 +/- 75 micrograms/kg. Favorable long-term results (mean follow-up period 17 +/- 8 months) were obtained with verapamil treatment at an average dose of 6.3 +/- 2.2 mg/kg. In conclusion, adenosine can be used effectively for termination of VT originating from the right ventricular outflow tract in children. In these patients verapamil may be considered as the drug of choice for long-term therapy.
Adenosine↗
Analysis of 2017 Holter records in pediatric patients.
Holter monitoring (HM) is widely used in arrhythmic disorders of adult patients; however, studies in the pediatric age group are limited. This study aims to determine the value of HM in diagnosis and treatment of disorders related to arrhythmias in pediatric patients. We examined 2,017 Holter records of 1,500 children who applied to our institution between November 1994 and October 1998. The age ranged from 0-24 years (52% male, 48% female). The indications for HM were screening for arrhythmic symptoms (palpitation, chest pain, syncope) in 67 percent, monitoring dysrhythmic therapy in 17 percent, postoperative control in five percent, and pacemaker control in four percent. Palpitation is the leading presenting symptom, with more frequent findings of supraventricular extrasystole (SVE), supraventricular tachycardia (SVT), ventricular extrasystole (VE) and complete heart block (CHB) when compared to other symptoms. Only 5.3 percent of the patients had arrhythmic symptoms during monitoring and asymptomatic patients had more frequent arrhythmias. SVT, VE, and CHB are more frequent findings in the abnormal heart with previous cardiac operations. The diagnostic yield is low with arrhythmic symptoms in the pediatric age group; however, HM enables cumulative evaluation of heart rhythm and rhythm variability, which is important in diagnosing silent arrhythmias in high risk groups (abnormal heart, postoperative heart).
Adolescent↗
Dilatation of a restrictive interatrial communication using a balloon angioplasty catheter.
Balloon atrioseptostomy is a life-saving procedure palliating certain congenital heart defects like transposition of the great arteries, right or left atrioventricular valve atresia, hypoplastic left heart syndrome, and pulmonary hypertension. Occasionally the Rashkind balloon septostomy technique may be ineffective in creating an adequate interatrial communication. We performed balloon dilatation of a restricted atrial septal defect using a balloon angioplasty catheter in a three-month-old infant.
Angioplasty, Balloon↗
Congenital sick sinus syndrome with breath holding and severe syncope episodes during infancy. A case report.
Sick sinus syndrome is a rare cause of bradycardia in children without structural heart disease. A case of profound sinus bradycardia, sinus arrest with junctional escape, and pauses in a two-year-old infant with breath-holding and syncope episodes is presented. As a result of these clinical symptoms and electrocardiographic findings, the patient with sick sinus syndrome underwent implantation of transvenous ventricular pacemaker. He has been well and asymptomatic since the insertion of the pacemaker. In the differential diagnosis of an infant with breath-holding and syncope episodes, when these symptoms in particular cannot be explained by other common reasons, sick sinus syndrome should be kept in mind. This case also illustrates the importance of electrocardiographic studies for the diagnosis.
Apnea↗
Transcatheter closure of secundum atrial septal defects, a ventricular septal defect, and a patent arterial duct.
We report our clinical experience with the newly developed Amplatzer device in transcatheter closure of nine atrial septal defects (ASDs), one ventricular septal defect (VSD), and one patent arterial duct (PDA). Eleven patients with ASD (age range 2.5-18 years) selected according to the location and size of the defect by transesophageal echocardiography (TEE), a five-year-old patient with muscular VSD and a one-year-old patient with PDA were considered for transcatheter closure with Amplatzer devices. All procedures were performed under general anesthesia with fluoroscopic and TEE guidance, following a routine hemodynamic evaluation in the catheter laboratory. The optimal device size was selected after the balloon sizing of the ASDs. The sizes of the VSD and PDA were measured on TEE and angiography. The patients were discharged at 24 hours, after an evaluation with x-ray, electrocardiogram (ECG), and echocardiography; they were on 3-5 mg/kg/day aspirin and infective endocarditis prophylaxis for six months after the procedure. They were reassessed at six to eight weeks and Holter monitoring was done in addition. Devices were used for nine ASD patients, and for the VSD and the PDA patients. Mean ASD size was 14.3 +/- 5.3 mm at TEE and 18.3 +/- 4.3 mm at balloon sizing (p=0.02). The mean size of the device was 18.7 +/- 4.2 mm. The procedure time and the fluoroscopy time were 46.1 +/- 12.3 and 12.9 +/- 1.6 minutes, respectively. Immediately after the procedure, four patients (44%) had trivial shunts (TS). TS remained in only two during discharge, and no shunt was observed at second evaluation. The devices were similarly applied to VSD (12-7 mm) and PDA (8-6 mm) patients. Both cases had TS immediately, which disappeared at 24 hours. None of the patients had major complications. Junctional rhythm developed in one patient, and another patient had frequent supraventricular extrasystoles. Amplatzer is an effective and safe device for transcatheter closure of ASD, VSD, or PDA, especially in pediatric patients.
Adolescent↗
Endovascular stent implantation in congenital heart defects.
We report the immediate and short-term results of endovascular stent implantations from our center. We performed stent implantations in four patients (3, 12, 18 and 20 years old) with different stenoses or obstructions: right ventricular outflow conduit obstruction, left Blalock-Taussig obstruction, post-operative recoarctation and cavopulmonary anastomosis obstruction. Stent were implanted successfully. The mean diameters of stenoses were expanded from 4.5 +/- 3.5 (2-7) mm to 9 +/- 1. 2 (8-10) mm, and the complaints of patients were improved significantly by stent implantation. There was no complication related to the procedures. All patients are living except one who died from cerebrovascular event unrelated to the stent implantation. The mean follow-up period of three living patients is 6.3 +/- 5.5 (1-2) months. As of the last control, all have remained at the caliber achieved at original placement. In light of our limited experience and previous reported studies, we conclude that intravascular stents are safe and can be used effectively in selected patients with congenital heart defects.
Adolescent↗
Preliminary pediatric transesophageal echocardiography experiences.
With transesophageal echocardiography (TEE), a new echocardiographic window is obtained which enables cardiologists to explore the heart from the esophagus and stomach. However, the procedure, when first undertaken, may present certain difficulties for the cardiologist in interpreting the anatomical findings and approaching a diagnosis. We thus convey our first experiences and results of TEE in 107 pediatric patients. Transesophageal echocardiography (TEE) was performed in 107 pediatric patients at our institution between December 1998-February 2001, using the standard techniques and following the standard criteria suggested by the American heart Association. The mean age of 54 male (50.5%) and 53 female (49.5%) patients was 7.8 years. Intubation difficulty was experienced in four cases. In one case, while drawing the transducer back from the esophagus, it kinked at the hypopharynx. None of the cases had major hemorrhage or esophageal rupture, and only a few cases had minor pharyngeal injuries or hemorrhages. We used TEE in detecting vegetations in patients with possible endocarditis, and evaluating the prosthetic valves and abnormal pulmonary venous return. We also used TEE to clarify preooperative anatomical details, postoperative complications and residual defects of complex congenital cardiac anomalies. Transcattheter closure of 47 secundum atrial septal defects (ASD)'s and a muscular ventricular septal defect (VSD) (both during patient selection and during the procedure) were accomplished under TEE guidance. As the pediatric cardiologists gain more experience in performing TEE, this technique will have a wider and more effective use in the pediatric population.
Adolescent↗
Anomalous origin of one pulmonary artery branch from ascending aorta ("so-called hemitruncus"): report of an additional case treated surgically.
The aortic origin of one pulmonary artery branch, so-called hemitruncus, is a rare congenital anomaly with poor prognosis. In this report, an additional patient is presented. The patient, a 60-day-old male infant with the right pulmonary artery originating from the ascending aorta was operated successfully. Postoperative catheterization demonstrated normal flow to the right lung and pulmonary artery pressure decreased to normal level.
Cardiac Catheterization↗
Echocardiographic findings in endomyocardial fibrosis.
An 18-month-old infant diagnosed as having endomyocardial fibrosis by echocardiography is presented. Most patients with endomyocardial fibrosis reported in the literature are either older children or adults. To our knowledge, our patient was the youngest ever to have been reported. Echocardiographic studies showed obliteration of the left ventricular apex and increased echo reflectance at the left ventricular endocardium and subendocardium. The left atrium and right ventricle were significantly enlarged. Doppler echocardiography showed minimal mitral, but significant tricuspid regurgitation. In regard to the contribution of echocardiography in the diagnosis, we recommend this method for suspected cases. Contrary to the other patients reported, there was no thickening of the atrioventricular valves. Mitral valve insufficiency was related to the restriction of the ventricular filling rather than to valve involvement occurring with the disease.
Cineangiography↗