Evaluación del lipidograma y ciertos factores de riesgo de ateroesclerosis en niños cuyos padres tuvieron arteriopatía coronaria de inicio temprano / Assessment of lipid profile and some risk factors of atherosclerosis in children whose parents had early onset coronary artery disease

Antecedentes/Objetivo. El objetivo de nuestro estudio fue analizar el lipidograma y ciertos factores de riesgo de ateroesclerosis, tales como las lipoproteínas de baja densidad oxidadas (ox-LDL, por su sigla en inglés) y las lipoproteínas de baja densidad pequeñas y densas (sdLDL, por su sigla en inglés) en los hijos de pacientes con cardiopatía coronaria (CC) prematura. Población y métodos. Hijos de padres con CC de inicio temprano emparejados con pares de su misma edad y mismo sexo. Se analizaron las concentraciones de lípidos, apolipoproteínas (ApoA, B, E), ox-LDL, sdLDL y lipoproteína (a) [Lp(a)] en los niños de estudio y de referencia. Los datos se evaluaron con el programa SPSS, junto con la prueba t de Student y la prueba U de Mann-Whitney. Resultados. Los niños del grupo de estudio (n: 43) tenían niveles más elevados de LDL, Lp(a) y ox-LDL y cocientes mayores de CT/HDL, ApoB/ApoA, LDL/HDL y ox-LDL/HDL (p < 0,05) que los del grupo de referencia. Conclusión. Con base en estos hallazgos, se sugiere que la dislipidemia y las concentraciones elevadas de LDL, Lp(a) y ox-LDL son frecuentes en los hijos de pacientes con CC de inicio temprano y representan gran parte de la predisposición familiar a tener CC

Background/Aim: The objective of our study was to analyze the lipid profile and some risk factors of atherosclerosis such as oxidized-low density lipoprotein (ox-LDL), small dense LDL (sd LDL) in the offspring of patients with premature coronary heart disease (CHD). Population and Methods: Children whose parents had early onset CHD were matched with age and sex pairs. Study and controls were analyzed for lipid levels, apolipoproteins (Apo- A,B,E), ox-LDL, sd LDL and lipoprotein (a) [Lp(a)]. The data were evaluated with SPSS using "Student tand Mann-Whitney U" tests. Results: Thestudy group children (n: 43) had higher LDL, Lp(a) and ox-LDL levels, ratios of TC/HDL, Apo-B/A, LDL/HDL and ox-LDL/HDL (p<0.05) than control group. Conclusion: These findings suggest that dyslipidemia and increased LDL, Lp(a) and ox-LDL levels are common in the offspring of patients with early onset CHD and account largely for their familial predisposition for CHD.

Cardiovascular abnormalities in Williams syndrome: 20 years'experience in Istanbul.

AIMS: Williams syndrome (WS) is a microdeletion syndrome affecting cardiovascular and connective tissue as well as the endocrine and central nervous systems in 1 in 10,000 live births. This study aims to identify and evaluate cardiovascular abnormalities (CVAs) in 45 WS patients. PATIENTS AND METHODS: We retrospectively reviewed a cohort of WS patients who were followed at our institution from January 1, 1990 through December 31, 2010. WS was clinically diagnosed by an experienced medical geneticist and confimed by fluorescence in situ hybridization. CVAs were assessed using electrocardiography, echocardiography or cardiac catheterization. RESULTS: Twenty-seven patients (60%) were male; 18 were female (40%).The mean age at presentation was 4.6 +/- 3.1 years (3 months-13 years); the follow-up period was 6.9 +/- 4.4 years (6 months-18 years). CVAs were found in 86% of patients, the most common one being supravalvar aortic stenosis (SVAS) in 73% (isolated in 48%), peripheral pulmonary artery stenosis (PAS) in 42%, and mitral valve prolapse (MVP) in 22%. Less common were aortic insufficiency (15%), ventricular septal defect (11%), valvular pulmonary stenosis (11%), and aortic arch hypoplasia (8%) and coarctation (2%). Hypertension was present in 22% of patients. Surgical or catheter-based interventions were performed in 22% of cases. Two patients were lost in the postoperative period. CONCLUSION: CVAs were found in more than four out of five patients, the most common ones being SVAS and PAS. Although surgery was performed in more patients with SVAS than with PAS, SVAS was minimal or mild in most patients and improved in few cases.

[The comparison a 16-year follow-up results of balloon angioplasty for aortic coarctation in children of different age groups: a single-center experience]. / Aort koarktasyonu tanisiyla balon anjiyoplasti uygulanan farkli yas gruplarindaki çocuklarin 16 yillik izlem sonuçlarinin karsilastirilmasi: Tek merkez deneyimi.

OBJECTIVE: Pediatric patients with different age groups who underwent balloon angioplasty for aortic coarctation were evaluated for recoarctation, aneurysm, peripheral arterial injuries and concomitant diseases. METHODS: From January 1994 to 2010, 80 patients with aortic coarctation (native/recoarctation) were evaluated, retrospectively. According to age at angioplasty, patients were divided into three groups: Group A (0-3 months, n=29, 25 male/4 female, average weight 4±1.2 kg), Group B (3-12 months, n=20, 15 male/5 girls, average weight 6.5±1.9 kg) and Group C (> 1 year, n= 31, 15 male/16 girls, average weight 22.8±16 kg). The patients were followed with echocardiography and clinical signs. The data of the native and recoarcted patients and also those in three different age groups were analyzed by using Chi-square, Kruskal-Wallis, and Student t tests. RESULTS: Peak systolic pressure gradient was reduced from 42±17 mmHg to 6.2±6 mmHg after balloon angioplasty (p<0.001) [n=80, 56 (70%) native, 24 (30%) recoarctation]. There was no difference between groups for early success. None of them did require immediate surgery. There were ventricular septal defect in 23 (28.7%), bicuspid aorta in 18 (22.5%), patent ductus arteriosus in 11 (13.7%) patients. Two patients had Turner's syndrome. Mean follow-up period was 74±56 months. While recoarctation developed in 20 (25%), [12 (60%) in Group A, 5 (25%) in Group B and 3 (15%) in group C, incidence was higher in the 0-3 months age group (p=0.018). Femoral artery occlusion and aneurysm were developed in 6 (7.5%) and 4 (5%) patients, respectively, and all of them were under 1-year-old. CONCLUSION: Balloon angioplasty can be used a method in treatment of native aortic coarctation and postoperative restenosis. Especially, children under 3 months should be monitored closely after the procedure for recoarctation, aneurysms and peripheral artery problems.

Therapeutic combination of sildenafil and iloprost in a preterm neonate with pulmonary hypertension.

While new pharmacological approaches have been demonstrated to effectively manage PH in adults, few reports have addressed PH treatment in neonates and infants. This case report describes the successful management of severe PH secondary to bronchopulmonary dysplasia, respiratory syncytial virus infection, and hypoxia in a preterm 4-month-old with the long-term use of orally administered sildenafil and inhaled iloprost.

Cardiac surgery of premature and low birthweight newborns: is a change of fate possible?

Low birthweight (LBW) continues to be a high-risk factor in surgery for congenital heart disease. This risk is particularly very high in very low birthweight infants under 1500g and extremely LBW infants under 1000g. From January 2005 to December 2008, 33 consecutive LBW neonates underwent cardiac surgery in our clinic in keeping with the criteria for choice of surgery. Their weight range was between 800 and 1900g. Nine of them were under 1000g. Cardiopulmonary bypass (CPB) was used in 17 patients (39.5%) and pulsatile perfusion mode was applied to patients in the CPB group. The same surgical team operated to achieve palliation (8 patients, 24.2%) or full repair (25 patients, 75.8%). Median gestational age was 36 weeks with 12 (36.4%) premature babies (≤37 weeks). Median age at operation was 5 days. Pathologies were single ventricle (n=3), pulmonary atresia-ventricular septal defect (n=3), aortic coarctation (n=10), aorticopulmonary window and interrupted aortic arch combination (n=6), patent arterial duct (n=11), critical aortic stenosis (n=8), and tetralogy of Fallot with pulmonary atresia (n=2). One infant had VATER syndrome. Selective cerebral perfusion technique was used in complex arch pathologies for cerebral protection. Median follow-up was 14 months. There were four early postoperative deaths. None of the cases showed a need for early reoperation. The acceptable early- and midterm mortality rates in this group suggest that these operations can be successfully performed. There is a need for further multicenter studies to evaluate these high-risk groups.

An evaluation of children with Kawasaki disease in Istanbul: a retrospective follow-up study

BACKGROUND: Kawasaki disease (KD) is an acute, self-limiting vasculitis of unknown etiology. The incidence of KD is increasing world wide. However, the epidemiological data for KD in Turkey has not been well described. OBJECTIVE: To describe the demographic, clinical, and laboratory features of children with KD who were diagnosed and managed in the American Hospital, Istanbul, Turkey. METHOD: Patients with KD were retrospectively identified from the hospital discharge records between 2002 and 2010. Atypical cases of KD were excluded. A standardized form was used to collect demographic data, clinical information, echocardiography and laboratory results. RESULTS: Thirty-five patients with KD, with a mean age of 2.5 + 1.9 years, were identified. Eighty-five point seven per cent of patients were under 5 years of age. A seasonal pattern favouring the winter months was noticed. In addition to fever and bilateral conjunctival injection, changes in the oral cavity and lips were the most commonly detected clinical signs in our cases. Coronary artery abnormalities were detected in nine patients. The majority of our patients had started treatment with intravenous immunoglobulin in the first 10 days of the onset of fever, and only one patient required systemic steroids for intravenous immunoglobulin-resistant KD. The coronary artery abnormalities resolved in all nine patients within 8 months. CONCLUSION: This study is the most comprehensive series of children from Turkey with KD included in Medline. As adult-onset ischemic heart disease may be due to KD in childhood, further prospective clinical investigations are needed to understand the epidemiology, management and long-term follow-up of the disease.

Congenital heart disease in children with Down's syndrome: Turkish experience of 13 years.

BACKGROUND: Down's syndrome (DS) is the most common chromosomal abnormality due to a trisomy of chromosome 21 commonly associated with congenital heart defects (CHDs). This study aimed to evaluate the frequency and types of CHD patterns in Turkish children with DS. METHOD: The data relate to paediatric patients with DS who underwent cardiologic screening between 1994 and 2007 and were reviewed in our Paediatric Cardiology unit. RESULTS: Four hundred and twenty-one out of the 1042 paediatric patients with DS studied over a 13-year period had associated CHD. Of these, 320 (77.6%) had a single cardiac lesion, while the remaining 92 patients (22.4%) had multiple defects. The most common single defect was an atrioventricular septal defect (AVSD) found in 141 patients (34.2%), followed by 69 patients (16.7%) showing secundum type atrial septal defect, and ventricular septal defect in 68 patients (16.5%). AVSDs were the leading type, isolated or combined with other cardiac anomalies with an overall occurrence of 19.8% of paediatric patients with DS, and 49.2% of paediatric patients with both DS and CHD. CONCLUSION: This is the first study concerning the frequency and type of CHD observed in Turkish children with DS. The high frequency of AVSD in Turkish children with DS implied that early screening for CHDs by echocardiography is crucial. The correction of AVSDs in paediatric patients with DS should be performed in the first 6 months of life to avoid irreversible haemodynamic consequences of the defect.

The effects of surgical repair on P-wave dispersion in children with secundum atrial septal defect.

INTRODUCTION: Atrial septal defect (ASD) is one of the most common congenital heart diseases in children. P-wave dispersion has been reported to be associated with non-homogeneous propagation of sinus impulses. The heterogeneity of atrial conduction time may predispose the atria to arrhythmias. The aim of this study was to determine the impact of surgical repair on P-wave indices in children with isolated secundum ASD. METHODS: Children with isolated secundum ASD undergoing surgical repair (n=50; mean age, 7.0+/-3.0 years) and healthy controls (n=51; mean age, 7.6+/-2.7 years) were compared. Maximum P-wave duration (Pmax), shortest duration (Pmin) and P-wave dispersion (Pd) were measured using 12-lead surface electrocardiography. RESULTS: Mean Pmax was found to be significantly higher in children with ASD compared with controls (95.2+/-10.8 vs 84.1+/-9.2 msec; P<0.001), and Pd before surgery was significantly higher compared with controls (47.4+/-12.0 vs 38.8+/-9.7 msec; P<0.001). Both P-wave indices were significantly decreased within the first year after surgical closure - the values decreased to those comparable to healthy controls (Pmax, 86.2+/-9.7 msec; Pd, 39.8+/-10.7 msec; P>0.05). CONCLUSION: Surgical closure of ASD in children decreases Pmax and P-wave conduction time. We speculate that earlier closure of the defect may play an important role in avoiding permanent changes in the atrial myocardium and atrial fibrillation in adulthood.

Fish oil supplementation improves left ventricular function in children with idiopathic dilated cardiomyopathy.

Fish oil has a cardioprotective effect in adults with ischemic heart disease. The authors examined the effects of fish oil in children with idiopathic dilated cardiomyopathy (DCM). Eighteen DCM patients (group I) and 12 healthy children (group III) were given fish oil (10 mL/d). Their cardiac findings were compared with those of 11 patients with DCM who did not receive fish oil (group II). After 6.62+/-1.70 months, left ventricular ejection fraction had increased by 8.44%+/-3.80% (P<.05), in group I; 2.48%+/-3.85% (not statistically significant) in group II; and 0.84%+/-2.34% (not statistically significant) in group III. Left ventricular internal diastolic diameter (mm) was reduced by 4.36+/-4.86 (P=.001) in group I and 1.92+/-5.37 (P=.263) in group II, but increased by 0.22+/-2.54 (not statistically significant) in group III. The results suggest that fish oil leads to accelerated improvement of left ventricular function. The authors believe that if these results are confirmed in larger studies, fish oil should be added to the standard anticongestive therapy of children with DCM.