Osman Yilmaz

Ventricular non-compaction in children: clinical characteristics and course

Isolated left ventricular non-compaction (LVNC) is a rare cardiomyopathy characterized by prominent trabeculations and deep intratrabecular recesses. In this study, we aimed to identify the clinical characteristics of children with ventricular non-compaction and determine the factors affecting prognosis. We retrospectively evaluated 29 children with LVNC followed at Dr. Sami Ulus Children Hospital Pediatric Cardiology Department from December 2004 to November 2009. There were 13 females (45%) and 16 males (55%) and the mean age at presentation was 4.8±4.6 years (one month-15 years). Although there was no statistical significance; early presentation age and high left ventricular end-diastolic diameter at the diagnosis were associated with poorer prognosis.

An evaluation of heart rate variability and its modifying factors in children with type 1 diabetes

OBJECTIVE To evaluate heart rate variability by Holter monitoring in type 1 diabetic children compared with a healthy control group and determine the factors modifying heart rate variability. METHODS This was designed as a prospective study comparing 28 patients, diagnosed with type 1 diabetes and under follow-up, with 27 healthy control group subjects. RESULTS The patients were aged 9.9 ± 4.2 years in the diabetic group, including 13 (46.5%) girls and 15 (53.5%) boys. The healthy control group comprised 20 (74%) girls and seven boys (26%) with an average age of 8.6 ± 3.7 years. The search for factors modifying heart rate variability yielded the following correlations: for the time-dependent variables, negative between age and both average and maximal heart rate (r = -0.263 and -0.460, respectively), negative between haemoglobin A1c and percentage of differences between adjacent RR intervals >50 ms, positive between diabetes duration and square root of the mean of the sum of squares of differences between adjacent NN intervals. The average heart rate and percentage of differences between adjacent RR intervals >50 ms was significantly higher in the girls than the boys in all groups. With regard to the frequency-dependent factors affecting heart rate variability, correlations were found between haemoglobin A1c level and both total power and very low frequency (r = -0.751 and -0.644) and between very low frequency and diabetes duration. CONCLUSION A reduction in heart rate variability parameters was observed in type 1 diabetes mellitus patients who had a long disease duration or were poorly controlled, as compared with healthy controls.

Assessment of left ventricular functions with tissue Doppler, strain, and strain rate echocardiography in patients with familial Mediterranean fever.

Objective: This study assessed the early changes in regional and global systolic and diastolic myocardial functions in patients with familial Mediterranean fever without any cardiovascular symptoms using tissue Doppler and strain and strain rate echocardiography and compared them to the results of a control group. Methods: This study has a cross-sectional and observational design. FMF patients with normal left ventricular function were included in the study. We excluded patients who had arrhythmia, acquired/congenital heart disease, pericarditis, or acute attack. We compared 45 children with familial Mediterranean fever on colchicine therapy and 45 age- and sex-matched healthy children. Results: The 45 patients with familial Mediterranean fever included 24 (55.3%) girls and 21 (46.7%) boys with a mean age of 11.3±3.7 (range 2-18) years. The mean disease duration was 4.6±2.4 (range 0.5-10) years. In the patient group, the homozygous M694V mutation was the most common (64.4%) mutation. The patients with familial Mediterranean fever had statistically lower longitudinal global strain, radial global strain, and strain rates (-14.44±4.77%, 14.80±6.29%, and 0.59±0.24 s, respectively) than the controls (-17.40±1.79%, 17.53±4.63%, and 0.83±0.51 s) (p<0.05). The circumferential global strain did not differ significantly between the groups. Conclusion: Patients with familial Mediterranean fever who are subclinical from a cardiac aspect might have normal left ventricular function as measured by conventional echocardiography. However, the disease affects their myocardial tissue, and these patients should be followed with conventional, strain, and strain rate echocardiography techniques regularly.

Congenital Right Pulmonary Artery Agenesis with Atrial Septal Defect and Pulmonary Hypertension

Unilateral pulmonary artery agenesis is a rare congenital anomaly caused by a backward displacement of the conical artery of the truncus arteriosus. It is commonly associated with additional cardiovascular abnormalities. A 7-year-old girl was admitted to our clinic with the complaint of shortness of breath upon exertion. Chest radiography revealed a hypoplastic right lung. Absence of the right pulmonary artery with atrial septal defect and pulmonary hypertension was demonstrated by echocardiography, computed tomography, and cardiac catheterization. Bosentan is effectively used to treat pulmonary arterial hypertension.

Kalbin Sol Tarafinin Multipl Obstrüktif Doğumsal Lezyonlari

Congenital multiple obstructive lesions of the left heart Objective: To investigate the localization, type, associations, other accompanying abnormalities and treatment methods. Material and Methods: The study included 24 cases presenting to the Pediatric Cardiology Unit of our Hospital with more than one left heart lesion, evaluated with two-dimensional color Doppler echocardiography (Vivid 7 Pro); 18 underwent cardiac catheterization and angiography. Complaints at admission, lesion site, type and associations, additional abnormalities and treatment were evaluated. Results: Multiple obstructive left heart lesions were more frequent (79%) in males, at an average age of 6.3. Most frequent symptoms at admission were dyspnea, fatigability and cyanosis. The most frequent associations of obstructive abnormalities were: mitral and aortic stenosis in 29%, mitral stenosis with aortic coarctation in 17% and aortic valve stenosis with aortic coarctation in 37%. Mitral stenosis associated to both aortic valve stenosis and aortic coarctation was seen in 17%. The most frequent associated non-obstructive abnormality was a bicuspid aortic valve (62%). Following diagnosis, 5 of the patients did not show up for follow-up, while the decision for one patient was observation. The remaining 18 cases underwent cardiac catheterization and angiography. Six patients had successful coarctation angioplasty and one angioplasty plus aortic valvuloplasty. We lost 2 of the patients who had undergone coarctation angioplasty. Conclusion: It was observed that the lesion most significantly contributing to mortality due to congenital obstructive lesions of left heart outflow are mitral valve obstruction and pulmonary hypertension, and that early invasive intervention is life-saving.

OP-136 CLINICAL ANALYSIS OF 204 CASES WITH ACUTE RHEUMATIC FEVER

Objective: This retrospective study aimed to identify clinical findings, etiologies, diagnosis, treatments and prognosis of 316 patients (age range:1 month-18 years) with pericarditis. Methods: The patients with echocardiographically detected pericarditis were admitted to our center between 1996 and 2008. These patients were reviewed according to physical examinations, blood chemistry results, microbiological, radiological and cardiological studies. Results: The mean age was 7.5±4.3 years. There were 156 (49.3%) boys and 160 (50.7%) girls. Although the most common symptoms were dyspnea (58.8%), fever (51.2%), chest pain (39.8%) and cough (35.1%), the physical examination revealed tachypnea (50.9%), hepatomegaly (44.9%), fever (39.5%), murmur (38.9%), tachycardia (34.1%), peripheral edema (21.8%), distant heart sounds (16.7%), ascites (15.2%) and frotman (5.6%). The most frequent causes of the pericarditis were viral/idiopathic (22.2%), malignancy (18.3%) and cardiac diseases (15.5%). Anemia was detected in 54.1% of patients. Cardiomegaly was seen in 55.7% of the cases.Echocardiographic study showed mild pericardial effusion in 45.9% of the patients. Twenty-seven (45%) of 60 patients (19%) who underwent pericardiosentesis had purulent pericarditis. In addition to medical treatment, dialysis, pericardial tube drainage and pericardiectomy were performed in 35, 25, and 7 patients,respectively. Ten patients developed constrictive pericarditis. Conclusions: We emphasized that early diagnosis and treatment is very important in patients who have signs and symptoms of pericarditis.Also echocardiography is essential for the diagnosis. Our study demonstrated that the viral/idiopatic etiology is the most common cause of pericarditis. We considered that improvement of socioeconomic status, preventive health care services, and effective antibiotherapy can decrease the prevalence of the pericarditis.